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Cavernous hemangioma of adult pancreas: A case report and literature review

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Pancreatic hemangioma is a rare type of benign vascular tumor. Low clinical suspicion and inability of current cross sectional imaging techniques to differentiate it from other pancreatic lesions, contribute to the difficulty in making the correct diagnosis. Without a definitive diagnosis, and due to concern for malignancy, in many instances, surgery is performed. We report a case of pancreas cavernous hemangioma in an 18-year-old female. The patient presented with three-month history of epigastric pain. Physical examination and routine blood tests were normal. Abdominal Computed Tomography scan revealed a 5 cm × 6 cm complex non-enhancing cystic mass in the head of pancreas. Magnetic resonance imaging, endoscopic ultrasonography (EUS) and EUS guided fine needle aspiration cytology were non-diagnostic. Because of uncontrolled symptoms, the patient underwent surgical resection. Histopathology and Immunohistochemical staining confirmed the diagnosis of cavernous hemangioma of pancreas.
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Cavernous hemangioma of adult pancreas: A case report
and literature review
Utpal Mondal, Nichole Henkes, David Henkes, Laura Rosenkranz
Utpal Mondal, Laura Rosenkranz, Division of Gastroenterology
and Nutrition, School of Medicine, University of Texas Health
Science Center at San Antonio, San Antonio, TX 78229, United
States
Nichole Henkes, David Henkes, Department of Pathology and
Laboratory, CHRISTUS Santa Rosa Hospital, San Antonio, TX
78240, United States
Author contributions: Mondal U designed the paper, did the
acquisition, analysis and interpretation of data, reviewed literature
and wrote the paper; Henkes N did literature search and proof
reading; Henkes D was responsible for cytopathology and
pathology; and Rosenkranz L provided clinical care including
endoscopy and made critical revision of the manuscript; all authors
approved the final version.
Institutional review board statement: The case study was
reviewed and approved by the Office of the Institutional Review
Board of the UT Health Science Center San Antonio.
Informed consent statement: Patient provided informed
written consent for this publication.
Conflict-of-interest statement: None of the authors has any
disclosure to make.
Open-Access: This article is an open-access article which was
selected by an in-house editor and fully peer-reviewed by external
reviewers. It is distributed in accordance with the Creative
Commons Attribution Non Commercial (CC BY-NC 4.0) license,
which permits others to distribute, remix, adapt, build upon this
work non-commercially, and license their derivative works on
different terms, provided the original work is properly cited and
the use is non-commercial. See: http://creativecommons.org/
licenses/by-nc/4.0/
Correspondence to: Utpal Mondal, MD, Division of Gastro-
enterology and Nutrition, School of Medicine, University of
Texas Health Science Center at San Antonio, 7703 Floyd Curl
Drive, San Antonio, TX 78229,
United States. mondalu@uthscsa.edu
Telephone: +1-210-5674880
Fax: +1-210-5671976
Received: April 5, 2015
Peer-review started: April 7, 2015
First decision: April 23, 2015
Revised: May 29, 2015
Accepted: June 26, 2015
Article in press: June 26, 2015
Published online: September 7, 2015
Abstract
Pancreatic hemangioma is a rare type of benign vascular
tumor. Low clinical suspicion and inability of current
cross sectional imaging techniques to differentiate it
from other pancreatic lesions, contribute to the difficulty
in making the correct diagnosis. Without a definitive
diagnosis, and due to concern for malignancy, in many
instances, surgery is performed. We report a case
of pancreas cavernous hemangioma in an 18-year-
old female. The patient presented with three-month
history of epigastric pain. Physical examination and
routine blood tests were normal. Abdominal Computed
Tomography scan revealed a 5 cm × 6 cm complex non-
enhancing cystic mass in the head of pancreas. Magnetic
resonance imaging, endoscopic ultrasonography (EUS)
and EUS guided fine needle aspiration cytology were
non-diagnostic. Because of uncontrolled symptoms, the
patient underwent surgical resection. Histopathology and
Immunohistochemical staining confirmed the diagnosis
of cavernous hemangioma of pancreas.
Key words: Hemangioma; Endo scopic ultrasound;
Endoscopic ultrasonography; Fine needle aspiration;
Pancreas
© The Author(s) 2015. Published by Baishideng Publishing
Group Inc. All rights reserved.
Core tip: Pancreas hemangioma is a very rare patho-
logical finding in adulthood. It may mimic other more
CASE REPORT
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DOI: 10.3748/wjg.v21.i33.9793
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World J Gastroenterol 2015 September 7; 21(33): 9793-9802
ISSN 1007-9327 (print) ISSN 2219-2840 (online)
© 2015 Baishideng Publishing Group Inc. All rights reserved.
common pancreatic lesions. Diagnosis is difficult, due
to its rarity, and the absence of typical radiological
findings of hemangioma. It should be considered in the
differential diagnosis of cystic, mixed and solid pancreatic
lesions. Surgical resection is the effective treatment in
symptomatic cases, while providing a final diagnosis,
however, surgery may not be necessary at all.
Mondal U, Henkes N, Henkes D, Rosenkranz L. Cavernous
hemangioma of adult pancreas: A case report and literature
review. World J Gastroenterol 2015; 21(33): 9793-9802
Available from: URL: http://www.wjgnet.com/1007-9327/full/
v21/i33/9793.htm DOI: http://dx.doi.org/10.3748/wjg.v21.
i33.9793
INTRODUCTION
Pancreatic cavernous hemangioma in adulthood is
a very rare condition. To date, only 20 cases have
been reported in the literature. Hemangioma mimics
common pancreatic lesions, and modern imaging may
not adequately characterize this benign condition.
The following case is that of an 18-year-old female
who underwent surgical resection of a large head of
pancreas lesion, with a final diagnosis of cavernous
hemangioma. A review of clinicopathological and
radiographic features of all cases reported in the
English literature to date is included.
CASE REPORT
An 18-year-old Hispanic female was referred to our
Institution by her primary care physician, for the
evaluation of a “pancreatic cystic lesion” found on
abdominal computed tomography (CT) scan, after
she presented with complaints of worsening epigastric
pain for three months. The pain was described as
stabbing and severe, radiating to the back, occurring
postprandial. Nausea and emesis were associated with
pain. Analgesia was only partially achieved with the use
of prescription oral narcotics.
The patient’s medical history included childhood
treated pulmonary tuberculosis. There was no history of
acute pancreatitis or abdominal trauma. Patient had no
prior surgeries. She was on no medications, particularly
has never been prescribed oral contraceptives, nor did
she ever use over the counter drugs. Family history was
non-contributory. Physical examination was normal.
Complete blood counts, complete metabolic panel,
serum amylase and lipase, coagulation panel, fasting
lipid profile, serum CA 19-9 and carcinoembryonic
antigen (CEA) levels were normal.
CT revealed a well-defined cystic mass with solid
components in the head of pancreas. The lesion did
not show contrast enhancement (Figure 1). Magnetic
resonance imaging (MRI) with and without intravenous
gadolinium showed a 5.3 cm × 6.2 cm, heterogeneous,
multi-septated, predominantly cystic mass in the head
and uncinate process of the pancreas, with variable
T1 and T2 signal intensity (Figure 2). Foci of T1
hyperintensity were thought to be due to hemorrhagic
or proteinaceous contents. The mass exhibited septal
enhancement with maximum septal wall thickness of 2
mm. Post-contrast dynamic images showed peripheral
hyperintensity with some indication of progressive
centripetal enhancement. There was no ductal,
vascular or nodal involvement. Magnetic resonance
cholangiopancreatography (MRCP) demonstrated no
ductal dilation (Figure 3).
Endoscopic ultrasonography (EUS) showed a well-
circumscribed complex structure with fluid and solid
components arising from the uncinate process and
extending to the head of pancreas (Figure 4). The
lesion was devoid of Doppler signal. The surrounding
parenchyma appeared normal. There was no ductal or
vascular involvement. No peri-pancreatic lymph nodes
were seen. The liver, gall bladder, spleen, left adrenal
and kidneys appeared normal. Fine needle aspiration
(FNA) using a 22G ProCore Cook Needle (Cook Medical
Inc., Winston-Salem, NC, United States) was performed,
and a total of five passes with adequate sampling
was obtained in the presence of a cytopathologist. No
bleeding occurred during or after the procedure, and
patient had an uneventful post-procedure recovery.
Microscopy of the direct smear from FNA showed
abundant red blood cells and a few clusters of cells
that had a cohesive nature, but no atypia (Figure 5).
Some areas had increased lymphocytes, suggesting
an inflammatory process. The cell block showed
clusters of histiocytes, some of which had light brown
material in their cytoplasm, suggestive of hemosiderin
deposition. Focal fibrotic fragments of tissue were
seen, without distinct vascular structures being
identified. No granulomatous features were seen.
Cyst fluid carcinoembryonic antigen (CEA) level was
< 0.5 ng/mL, and amylase level was 13 U/L. Gram
stain, acid-fast bacilli, and alpha 1 antitrypsin stains
were negative. CAM 5.2 was negative. Vimentin was
positive in macrophages, but no epithelioid cells were
identied. Overall, the ne needle aspiration ndings
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Figure 1 Contrast computed tomography scan showed a non-enhancing
cystic lesion in the head of the pancreas.
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Figure 2 Magnetic resonance imaging. Variable intensity in T1 weighted (A) and T2 weighted (B) imaging. Overall, the hemangioma had hypo-intensity in T1W and
hyper-intensity in T2W imaging. Peripheral enhancement after gadolinium injection with gradual centripetal lling-in (C, D: Arterial phase; E: Venous; and F: Delayed
venous phase) was noticed. Intensity was parallel to that of aorta. A central cystic area remained hypo-intense.
A B
C D
E F
Figure 3 Magnetic resonance cholangiopancreatography demonstrated
no ductal dilation.
Figure 4 Endoscopic ultrasound: Complex cyst at the head of pancreas,
devoid of Doppler signals, with normal surrounding parenchyma and
without any ductal or vascular abnormality.
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was found to be inseparable from the duodenum.
Pylorus preserving pancreaticoduodenectomy with
cholecystectomy and resection of 17 lymph nodes
were performed.
The gross pathology revealed a pink-brown, partly
hemorrhagic, multi-loculated cystic mass, 6 cm × 4
cm × 3.5 cm in size, with intracystic hemorrhage and
no mucinous component (Figure 6). On microscopic
examination (Figure 7), a single layer of flattened
cells lined the blood filled cystic spaces, and had
uniform nuclei and no atypia. The vessels lumina
contained macrophages, some with hemosiderin
within. Aggregates of chronic inammatory cells were
present in the surrounding brous tissue. One vessel
had an organizing thrombus, also containing fibrin
and foamy macrophages. On immunohistochemical
suggested a benign process most consistent with an
inflammatory benign cyst. Due to the presence of a
few cohesive clusters of cells, a neoplastic cystic lesion
(serous cystadenoma or solid cystic papillary tumor)
could not entirely be ruled out.
The case was reviewed in the multidisciplinary
Pancreatic Tumor Board. A recommendation was made
to continue close clinical monitoring, and a consensus
from pathologists, radiologists, gastroenterologists, and
surgeons reiterated the benign etiology of the lesion.
Over the next few weeks, the patient experienced
worsening nausea, vomiting and abdominal pain,
refractory to narcotics and antiemetics. Subsequently,
the patient limited her oral intake and lost 20 pounds
of body weight. The patient was eventually taken
to surgery. Intra-operatively, the pancreatic lesion
Figure 5 Fine needle aspiration cytology. Direct smear showed few clusters of cells without atypia (A, B) and increased lymphocytes (C); Cell block showed
clusters of histiocytes, some of which had light brown material in their cytoplasm, suggestive of hemosiderin (D).
A B
C D
Figure 6 Gross pathology showing head of pancreas lesion (A) and cystic lesion with thick septa and recent hemorrhage evident on cross section (B).
A B
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(IHC) staining, the lining was positive for CD31, focally
positive for CD34, and negative for cytokeratin (AE1/
AE3) supporting the diagnosis of hemangioma. All 17
lymph nodes had normal histology.
Postoperatively, the patient was monitored in
the Intensive Care unit for two days. By second day,
she was tolerating oral intake of liquids. She was
discharged home on the 3rd post-operative day. Six
months after surgery, she remained symptom free.
DISCUSSION
Vascular tumors (hemangioma, lymphangioma, he-
molymphangioma, hemangioendothelioma, hemangio-
pericytoma, hemangioblastoma and angiosarcoma)
constitute only 0.1% of the pancreatic tumors[1].
Hemangiomas are vascular tumors composed of blood
vessels lined by endothelial cells. Visceral hemangiomas
can be found in various organs including the brain,
parotid, thorax, liver, spleen, adrenal, retroperiton-
eum and gastrointestinal tract, in isolation, or with
synchronous cutaneous lesions[2]. Hemangioma is the
most common benign tumor of the liver, however,
pancreas hemangiomas are extremely rare, both in
adult and pediatric population.
A PubMed and Google Scholar search of the lite-
rature using the keywords “pancreas hemangiomawas
performed, and pertinent articles and their references
were reviewed. Since 1960, a total of twenty-one
cases have been reported in nineteen articles (Table
1)[3-19]. Mundinger et al[20] in their 2009 article, stated
that a number of nine cases have been described in
the medical literature prior to 1939. In our review,
cases of hemolymphangioma, lymphangioma and
vascular malformations were excluded. Hemangiomas
with retroperitoneal origin involving the pancreas were
also excluded. The aim of the present review is to
provide a summary of the epidemiological and clinical
characteristics of this rare tumor.
The average age of the patients at diagnosis is 48.3
years (range: 18-79 years). Our patient is the youngest
adult case reported. Infantile hemangiomas of pancreas
are very rare, but they involute by early childhood[21].
Male to female ratio is 1:3. Other pancreatic tumors e.g.,
mucinous cystic neoplasm (MCN), intraductal papillary
mucinous neoplasm (IPMN) and solid pseudopapillary
neoplasm (SPN) also show female predominance.
Male to female ratio of 1:5 is reported in cavernous
hemangioma of the liver. Although an association
between the proliferation of hepatic cavernous
hemangiomas and increased levels of estrogen (from
contraceptives, pregnancy) is reported, no definite
causal relationship has been proven[22].
The most common presenting symptom is epi-
gastic pain (47%). Nausea, abdominal distention,
jaundice, fever, episodic dizziness and palpitations
and gastrointestinal bleeding may be associated to
pain. No reported case had episodes of pancreatitis or
family history of pancreas diseases. Rarely, especially
in young patients, cavernous hemangiomas of the
pancreas may be observed in the setting of von
Hippel-Lindau disease[23]. Abnormalities of the serum
Figure 7 Histopathology. A: The hemangioma displaced the exocrine pancreas; B: It had blood lled ectatic vessels with thin endothelium lining large cavernous
spaces and aggregates of chronic inammatory cells in the surrounding brous tissue; C: The lumina of the vessels contained macrophages, some containing
hemosiderin; D: One vessel with organizing thrombus containing brin and foamy macrophages.
A B
C D
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liver tests and thrombocytopenia may be present
but no correlation between the size or of the lesions
location and symptomatology was noted. In fact, these
tumors can be as large as 20 cm and arising from the
head of pancreas, yet they do not cause much pain
or obstructive features. On the contrary, two of the
smallest reported lesions, presented with obstructive
jaundice (No. 8, 9). Average reported tumor size is
8.9 cm (range: 3-20 cm), with the majority (70%)
involving the head of the pancreas. Hemangiomas
are indolent (No. 13, 14) and do not have malignant
potential. Why our patient presented with rather
short duration of symptoms is unknown. Whether
hemorrhage, thrombosis or necrosis within the cyst
occurred and led to symptoms is not known.
Ultrasonography
Transabdominal ultrasonography (US) is reported to
detect hemangiomas in 9 cases. All these lesions were
more than 5 cm in size. Lesions were well demarcated,
appeared overall hyper- or iso-echogenic to rest of
the pancreas, and had mixed echogenicity. A 20 cm
mass in the head of pancreas was diagnosed as a
giant hemangioma on US (No. 6). The absence of
a fine speckled internal echo pattern may suggest
a vascular tumor as opposed to a mucinous cystic
tumor[24]. Intra-operative US was helpful in diagnosing
pancreatic hemangioma in one case and prevented
pancreatectomy (No. 9). Our Endoscopic ultrasound
findings were similar to those described by Lee
(No. 14). We identified a cystic mass with thick
septations and solid components. Doppler signal was
absent within the lesion. The overall sonographic
appearance was suggestive of an inflammatory or
mucinous cyst. Willamson (No. 18) made a diagnosis
of pancreas hemangioma based on endosonographic
characteristics, which allowed a conservative approach
to the management of his reported case, and surgery
was avoided. Hemorrhage, thrombosis, necrosis,
shunting and vascularity of the solid component
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Table 1 Previously reported pancreas hemangiomas in literature
No. Year Country Author Age (yr)/
sex
Site/size (cm) Preoperative diagnosis Treatment Follow up
1 1939 - Ranstrom 61/F Head/7 Found at autopsy -
2 1960 France Ringoir - - - Surgery
3 1961 France Ringoir 71/F Head/15 - Retrocolic gastroenterostomy,
vagotomy
4 1972 France Colardyn 42/F Body tail/- - Fat free diet, anticholinergic1
5 1985 France Mangin 62/F Head to
tail/20
Multicystic heterogenous
structure
Laparotomy with observation1
6 1991 Japan Kobayashi 30/M Head/20 Cavernous hemangioma
suggested on US/MRI
Pancreatoduodenectomy
7 1991 Germany Dageforde 79/F Body tail/6 Observation1
8 2003 Taiwan Chang 70/F Body tail/4 Heterogenous hypervascular
solid mass. Serous/cystic
adenoma/adenocarcinoma?
Subtotal pancreatectomy Healthy at 14
mo
9 2006 Austria Plank 36/M Head/3 Hypervascular mass.
Nonfunctioning
neuroendocrine tumor
Hemangioma suspected during
laparotomy, conrmed by
intraoperative US. Not resected1
10 2008 China Xu - - Three cases reported -
11 2009 United States Mundinger 45/F Head/5.5 Hypodensity mass in CT.
Benign (duplication cyst,
paraganglioma, cystic GIST)
cyst
Frozen section: benign cyst.
PPPD performed
12 2010 - Jarboui 60/F - - -
13 2011 Israel Weidenfeld 73/F Head/5 Cyst with solid part Whipple’s procedure
14 2011 Malaysia Lee 49/F Neck/5 Non-enhancing septated cyst.
Mucious cyst with malignant
features
Central partial pancreatectomy
and gastrostomy
Symptom free
at 6 mo
15 2012 Germany Kersting 53/M Head/8 Non-enhancing mass.
Adenocarcinoma?
Extirpation of the tumor
16 2013 China Zhi-hua 23/F Head/5.4 Non enhancing multilocular
cyst with uid uid levels
Subtotal pancreatectomy Healthy, no
recurrence at 1
yr
17 2013 United
Kingdom
Malik 70/F Head/8 Giant hemangioma suggested
on CT
PPPD Fine at 6 wk
18 2014 United
Kingdom
Williamson 78/F Head/4 Hemangioma per EUS Observation1
19 2014 Japan Naito 40/F Body tail/10 Multilocular septated cystic
mass
Pancreatectomy No recurrence
at 6 yr
20 2015 United States Mondal 18/F Head/6 Benign cyst PPPD Fine at 6 mo
1Cases that were preoperatively suspected to be hemangioma and/conservatively managed. PPPD: Pylorus preserving pancreatoduodenectomy.
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within, may account for poor Doppler signal in most
pancreatic hemangiomas[9].
CT scan
Typically, hemangiomas are strongly contrast enhancing
in the arterial phase of conventional contrast-enhanced
CT imaging[7]. Peripheral irregular enhancement
with central non-enhancement in venous phase,
and progressive filling-in during the delayed phases
of dynamic CT scan is typically described in hepatic
hemangiomas. Based on these features, preoperative
diagnosis of pancreatic giant hemangioma was
suggested only in one case (No. 17). A non-enhancing
three-phase contrast CT scan does not exclude
hemangioma. Of the cases that reported pre-contrast
CT findings, five lesions (No. 8, 9, 11, 17, 19) were
heterogeneous (with both, high and low attenuation),
multi-loculated cysts and ve (No. 13, 14, 16, 18, 20)
were hypo-attenuating “cystswith solid components
and peripheral micro- or speckled calcifications. Post-
contrast CT showed poorly enhanced or unenhanced,
hypo-vascular, multi-loculated, cystic tumors in
some cases (No. 6, 11, 14, 15, 16, 20) and hyper-
enhancement in others (No. 8, 9, 13, 17, 18, 19).
MRI
Hemangioma on MRI appears as a lobulated, hypo-
intense mass in T1 -weighted images, and shows
moderate hyperintensity signal in T2 -weighted images,
while after intravenous gadolinium administration, there
is marked enhancement[25,26]. Four articles (No. 6, 9, 16,
19) reported MRI findings of pancreas hemangiomas.
Low T1W signal attenuation in (No. 6, 9, 16) and high
T2W signal attenuation in (No. 16, 19) were seen on
non-contrast MRI. MRI was able to diagnose a giant
hemangioma of pancreas (No. 6) without further work-
up. In our case, in the post-contrast dynamic imaging,
there was some evidence of centripetal filling-in. No
dilatation of the pancreatic and the common bile ducts
was seen on MRCP in our patient.
Fine needle aspiration cytology
Since radiological imaging cannot characterize these
lesions, almost all complex cystic lesions of the
pancreas undergo endoscopic or per-cutaneous fine
needle aspiration (FNA) to establish a diagnosis.
Hemangiomas can be highly vascular. Fatality related
to biopsy of hepatic hemangioma has been reported[27],
therefore concern regarding FNA sampling of these
lesions should rise. If the radiographic characteristics
of hemangioma are absent on imaging, the risk
of bleeding remains concerning following lesion
sampling or during surgery. On the other hand, the
article reporting 38 patients with sampled hepatic
hemangiomas, along with several other small series,
revealed needle biopsy to be safe[2 8]. FNA was
performed in pancreas hemangiomas (No. 14, 17, 18),
and there was no reported bleeding. We performed
EUS guided FNA using a 22G ProCore Cook Needle
(Cook Medical Inc., Winston-Salem, NC, United States)
with 5 passes without associated bleeding.
Other imaging methods
Angiography performed in cases of pancreatic
hemangiomas showed either a poorly vascular (No. 6)
or a hypervascular (No. 8) tumor without encasement
of surrounding vascular structures. Arteriography does
not provide additional useful information beyond CT/
MRI, and is not routinely performed, unless selective
embolization of feeding vessels is being considered
as treatment. Technetium (Tc)-99 isotope study and
positron-emission tomography (PET) scan have been
found useful in diagnosing hemangiomas in other
organs, but none has been reported in pancreas he-
mangioma cases.
Differential diagnoses
As hemangiomas are essentially benign tumors, correct
preoperative differentiation from other tumors could
prevent surgical resection. On imaging, hemangiomas
elicit characteristics that overlap with radiographic
phenotypes of other common lesions of the pancreas.
The list of radiological differential diagnoses for cystic-
solid and cystic pancreatic lesions is broad (Table 2).
Most cases proceeded to surgery without conrmatory
diagnosis. The correct pre-operative or intra-operative
diagnosis of hemangioma was made only in 5 out of
20 cases (No. 4, 5, 7, 9, 18), thus avoiding need for
surgical resection.
Histopathology
Microscopically, hemangiomas are composed of an
increased number of blood-filled ectatic capillaries
lined by flat endothelium and separated by scant
fibrous connective tissue stroma. Depending on
the size of vascular spaces, they can be capillary or
cavernous type. Vascular endothelial marker (CD31,
CD34 and factor -related antigen) positivity, along
with negative results for cytokeratin (AE1/AE3, D2-40,
CAM 5.2, MNF 116) immunohistochemical staining
indicates hemangioma, lymphangioma and other
benign vascular tumors. Lymphangioma may be easily
excluded, as they consist of lymphatics (instead of
blood vessels) filled or infiltrated with lymphocytes.
Using electron microscopy (EM), lymphangioma was
diagnosed in a resected pancreas tumor[29], however,
EM has not been described in hemangioma. A precise
histopathological diagnosis of hemangioma is very
important, as other vascular tumors may require
wider surgical margin at resection, adjunctive therapy
and closer post-resection follow up. For instance, in
hemolymphangioma, 10%-27% and 50%-100%
recurrences are reported after complete and partial
resection, respectively[30].
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Treatment of hemangioma
Unlike pediatric hemangiomas that mostly involute, adult
pancreatic hemangiomas do not. The natural history
of adult pancreas hemangiomas is unknown. They
appear to be very slow growing and have no malignant
potential. Over a 7 years period, a hemangioma grew
from 2 cm to 5 cm (No. 14). Pancreatic hemangiomas,
even w hen large, rarel y obstruct or infiltrate
adjacent structures. Although the possibility of future
complications (spontaneous or traumatic rupture,
bleeding, pancreatitis, infection, etc.) is present, a
conservative approach can be considered, if confident
diagnosis is made preoperatively. Five cases are reported
to be managed by observation strategy. However, details
regarding surveillance and outcomes are not known.
Propranolol, corticosteroid, interferon alpha, vincristine,
cyclophosphamide and radiotherapy have been used to
successfully treat infantile hemangiomas, but their role
in adult hemangioma treatment is not known.
Surgical excision seems to be a reasonable strategy
for large hemangiomas when diagnostic uncertainty
remains, for uncontrolled symptoms with failed con-
servative management, or to address complications
from mass effect. All resected pancreas hemangioma
cases had good outcome, with resolution of symptoms
and no tumor recurrence. Naito (No. 19) reported
surgical removal of a giant pancreatic hemangioma
without recurrence at 6-year follow up. Preoperative
suspicion, use of intra-operative ultrasound and frozen
sections may help in determining the best surgical
method, e.g., enucleation, other more conservative
surgical techniques or an extensive resection.
Current imaging techniques cannot reliably diagnose
pancreas hemangioma from other cystic neoplasm.
Table 2 Differential diagnoses of cystic and cystic-solid lesions of pancreas
Cystic lesions Sex/age/size/site Imaging, cytology features
Pseudocysts Any Unilocular
Serous cystic
neoplasms
F/60s/large/body or
tail
Serous and mucinous cystadenoma similar on CT
Central brous scar with calcication
Septation
Multiple cysts lined by glycogen-rich epithelial cells that are positive for periodic acid Schiff
and express cytokeratins
Mucinous cystic
neoplasms
F/50-60s/> 10 cm/body
or tail
Single multilocular cysts that do not communicate with the ductal system
Smooth shape, even wall, with or without (ne) septa with potential small nodules
Peripheral egg shell calcication
Cyst lined by columnar mucin-producing epithelial cells set within an ovarian-like stroma
Dysplasia and malignant potential
Express keratin, carcinoembryonic antigen and CA19-9, while CK20 and CDX2
(markers of intestinal differentiation) are negative
IPMN M = F/70-80s/head Intraductal proliferation of mucinous cells usually showing papillary projections
Dysplasia, invasion and malignant potential
The epithelium of IPMNs expresses keratins, CEA and CA19-9, with variable expression of
MUC
Solid pseudopapillary
neoplasms
Young F/> 10 cm/tail,
head
Solid or cystic-solid hypervascular tumors, solid part enhances
Show pseudopapillae and pseudocysts
Stain for vimentin and beta catenin with partial reactivity to keratin
CD56 and synaptophysin stains may be positive, chromogranin negative
Cystic change of
solid tumors
PDAC is rare < 50,
M = F
Recognition of adjacent solid lesion (carcinoma) is the key to correct diagnosis of ductal
adenocarcinoma
PDAC are brotic, hypo-vascular, ill dened border, early inltration in peri-pancreatic fat,
invasion of vascular structures and ducts
NET and Islet cell tumors are hyper-vascular and hyper-enhancing with positive staining
for chromogranin and synaptophysin
Lymphoepithelial cysts M/50-60s/ body or tail Lined by squamous epithelium and surrounded by dense lymphoid tissue, possibly
showing germinal centers
Vascular tumors
(Lymphangiomas,
hemangiomas,
hemolymphangioma,
hemangioendothelioma,
angiosarcoma
F > M/large Cystic-solid, encapsulated, multi-loculated, micro-cystic portions appear solid and
enhancing
Vascular markers CD31, CD34 and factor are positive in endothelial cells
Cytokeratins neg
Lymphatic marker D2-40 neg in hemangioma, positive in lymphangioma
Angiosarcomas, highly aggressive, a vascular channel lined by variably atypical endothelial
cells
Metastatic from renal cell cancer Hypervascular, invasive
Accessory splenic tissue Tail hypervascular
Others Rare solid tumors of the pancreas also include: mature teratoma, hamartoma, sarcoidosis,
yolk sac tumor, acinar cell carcinoma, lymphoplasmatic sclerosing pancreatitis, primary
pancreatic lymphoma., duodenal tumor, duplication cysts, paraganglioma, cystic GIST,
glomus tumor, etc.
IPMN: Intraductal papillary mucinous neoplasms; PDAC: Pancreatic ductal adenocarcinoma.
Mondal U
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. Cavernous hemangioma of pancreas
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Newer techniques like contrast enhanced US, PET scan,
elastography, etc. may be tried in future. Early suspicion
by clinicians and radiologists is crucial. Although rare,
pancreatic hemangioma should be considered in the
differential diagnosis.
COMMENTS
Case characteristics
An 18-year-old female with nausea, vomiting and epigastric pain for 3 mo and a
5 cm x 6 cm non-enhancing cystic mass in the head of pancreas on computed
tomography scan.
Clinical diagnosis
Epigastric tenderness but no mass on examination.
Differential diagnosis
Pancreatic neoplasm, pancreatic cyst
Laboratory diagnosis
Complete blood counts, complete metabolic panel, serum amylase and lipase,
coagulation panel, fasting lipid prole, serum CA 19-9 and carcinoembryonic
antigen levels were normal.
Imaging diagnosis
Magnetic resonance imaging showed a 5.3 cm x 6.2 cm, heterogeneous, multi-
septated, predominantly cystic mass in the head of the pancreas, with variable
T1W and T2W signal intensity and differential diagnoses included: serous
cystadenoma, solid pseudopapillary neoplasm, inammatory cyst and mucinous
cyst.
Pathological diagnosis
Endoscopic ultrasonography guided fine needle aspiration cytology was
negative for malignancy; post resection histopathology (blood-lled cavernous
spaces lined by flat endothelium) and immunohistochemistry (CD31, CD34
positive and AE1/AE3, CAM 5.2 negative) confirmed the diagnosis of
hemangioma.
Treatment
Pylorus preserving pancreatoduodenectomy.
Related reports
Cavernous hemangioma of pancreas is a rare condition and preoperative
diagnosis is difficult. Cases reported in English literature were reviewed to
identify clinicopathological and radiological characteristics of these tumors.
Experiences and lessons
Pancreas hemangiomas are rare benign tumors that mimic other more
common lesions of pancreas. Condent preoperative diagnosis is challenging.
Symptomatic patients need resection that also establishes the diagnosis.
Peer-review
This article describes the clinical, radiological and pathological features of a
case of cavernous hemangioma of pancreas and also provides a literature
review of the reported cases.
REFERENCES
1 England RJ, Woodley H, Cullinane C, McClean P, Walker J,
Stringer MD. Pediatric pancreatic hemangioma: a case report and
literature review. JOP 2006; 7: 496-501 [PMID: 16998249]
2 Schulz AS, Urban J, Gessler P, Behnisch W, Kohne E, Heymer B.
Anaemia, thrombocytopenia and coagulopathy due to occult diffuse
infantile haemangiomatosis of spleen and pancreas. Eur J Pediatr
1999; 158: 379-383 [PMID: 10333119]
3 Derom F, Ringoir S, Marlier R. [Two cases of intraabdominal
hemangioma: liver and pancreas]. Acta Chir Belg 1960; 59: 172-182
[PMID: 13816057]
4 Ringoir S, Derom F, Colle R, Mortier G. Hemangioma of the
pancreas. Report of a case. Gastroenterology 1961; 41: 43-45 [PMID:
13741765]
5 Colardyn F, Elewaut A, Van de Velde E, Barbier F. Hemangioma
of the pancreas. Tijdschr Gastroenterol 1972; 15: 260-267 [PMID:
4642101]
6 Mangin P, Perret M, Ronjon A. [Hemangioma of the pancreas]. J
Radiol 1985; 66: 381-384 [PMID: 4032348]
7 Kobayashi H, Itoh T, Murata R, Tanabe M. Pancreatic cavernous
hema ngioma: CT, MRI , US, and angiography characteristics.
Gastrointest Radiol 1991; 16: 307-310 [PMID: 1936772 DOI:
10.1007/BF01887375]
8 Dageförde J, Gmelin E, Otte M. [Hemangioma of the pancreas].
Rofo 1991; 154: 332-333 [PMID: 1849305]
9 Chang WT, Lee KT, Yang SF. Cavernous hemangioma of the
pancreas: report of a case. Pancreas 2003; 26: 310-312 [PMID:
12657961 DOI: 10.1097/00006676-200304000-00018]
10 Plank C, Niederle B, Ba-Ssalamah A, Schima W. Pancreatic
hemangioma: imaging features with contrast-enhanced CT and with
gadolinium- and mangafodipir-enhanced MRI. Eur J Radiol 2006;
57: 59-62 [DOI: 10.1016/j.ejrex.2005.11.008]
11 Xu Q, Wang CF, Zhao P, Shan Y, Zhao DB, Liu Q. [The diagnosis
and treatment of pancreatic cavernous hemangioma]. Zhonghua Yi
Xue Za Zhi 2008; 88: 28-30 [PMID: 18346376]
12 Jarboui S, Salem A, Gherib BS, Ben Moussa M, Rajhi H, Mnif N,
Zaouche A. Hemangioma of the pancreas in a 60-year-old woman:
a report of a new case. Gastroenterol Clin Biol 2010; 34: 569-571
[PMID: 20609542 DOI: 10.1016/j.gcb.2010.06.001]
13 Weidenfeld J, Zakai BB, Faermann R, Barshack I, Aviel-Ronen S.
Hemangioma of pancreas: a rare tumor of adulthood. Isr Med Assoc
J 2011; 13: 512-514 [PMID: 21910381]
14 Lee J, Raman K, Sachithanandan S. Pancreatic hemangioma
mimicking a malignant pancreatic cyst. Gastrointest Endosc 2011;
73: 174-176 [PMID: 20932519 DOI: 10.1016/j.gie.2010.07.038]
15 Kers ting S, Janot MS, Munding J, Suelberg D, Tannapfel A,
Chromik AM, Uhl W, Bergmann U. Rare solid tumors of the
pancreas as differential diagnosis of pancreatic adenocarcinoma.
JOP 2012; 13: 268-277 [PMID: 22572130]
16 Lu ZH, Wu M. Unusual features in an adult pancreatic hemangioma:
CT and MRI demonstration. Korean J Radiol 2013; 14: 781-785
[PMID: 24043972 DOI: 10.3348/kjr.2013.14.5.781]
17 Malik M, Ahmed I, Kurban L. Pancreatic Hemangioma - A case
Report. Gastroenterol Hepatol Res 2013; 2: 545-548 [DOI: 10.6051/
j.issn.2224-3992.2013.02.236]
18 Williamson JM, Finch-Jones M, Pope I. Endoscopic ultrasonography
allowing expectant management of pancreatic haemangioma. Ann R
Coll Surg Engl 2014; 96: e1-e2 [PMID: 24780776 DOI: 10.1308/003
588414X13814021678231]
19 Naito Y, Nishida N, Nakamura Y, Torii Y, Yoshikai H, Kawano H,
Akiyama T, Sakai T, Taniwaki S, Tanaka M, Kuroda H, Higaki K.
Adult pancreatic hemangioma: A case report. Oncol Lett 2014; 8:
642-644 [PMID: 25013478 DOI: 10.3892/ol.2014.2206]
20 Mundinger GS, Gust S, Micchelli ST, Fishman EK, Hruban RH,
Wolfgang CL. Adult pancreatic hemangioma: case report and
literature review. Gastroenterol Res Pract 2009; 2009: 839730
[PMID: 19421421 DOI: 10.1155/2009/839730]
21 Takahashi K, Mulliken JB, Kozakewich HP, Rogers RA, Folkman
J, Ezekowitz RA. Cellular markers that distinguish the phases of
hemangioma during infancy and childhood. J Clin Invest 1994; 93:
2357-2364 [PMID: 7911127 DOI: 10.1172/jci117241]
22 Glinkova V, Shevah O, Boaz M, Levine A, Shirin H. Hepatic
haemangiomas: possible association with female sex hormones.
Gut 2004; 53: 1352-1355 [PMID: 15306599 DOI: 10.1136/
gut.2003.038646]
23 Hammel P, Beigelman C, Chauveau D, Resche F, Bougerolles E,
Mondal U
et al
. Cavernous hemangioma of pancreas
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Flejou JF, Bernades P, Delchier JC, Richard S. [Variety of pancreatic
lesions observed in von Hippel-Lindau disease. Apropos of 8 cases].
Gastroenterol Clin Biol 1995; 19: 1011-1017 [PMID: 8729413]
24 Balderramo DC, Di Tada C, de Ditter AB, Mondino JC. Hemo-
lymphangioma of the pancreas: case report and review of the
literature. Pancreas 2003; 27: 197-199 [PMID: 12883270 DOI:
10.1097/00006676-200308000-00014]
25 Semelka RC, Brown ED, Ascher SM, Patt RH, Bagley AS, Li
W, Edelman RR, Shoenut JP, Brown JJ. Hepatic hemangiomas: a
multi-institutional study of appearance on T2-weighted and serial
gadolinium-enhanced gradient-echo MR images. Radiology 1994;
192: 401-406 [PMID: 8029404]
26 Goshima S, Kanematsu M, Kondo H, Yokoyama R, Kajita K, Tsuge
Y, Shiratori Y, Onozuka M, Moriyama N. Hepatic hemangioma:
correlation of enhancement types with diffusion-weighted MR
findings and apparent diffusion coefficients. Eur J Radiol 2009; 70:
325-330 [PMID: 18321673 DOI: 10.1016/j.ejrad.2008.01.035]
27 Terriff BA, Gibney RG, Scudamore CH. Fatality from fine-
needle aspiration biopsy of a hepatic hemangioma. AJR Am J
Roentgenol 1990; 154: 203-204 [PMID: 2104717 DOI: 10.2214/
ajr.154.1.2104717]
28 Tung GA, Cronan JJ. Percutaneous needle biopsy of hepatic
cavernous hemangioma. J Clin Gastroenterol 1993; 16: 117-122
[PMID: 8463614 DOI: 10.1097/00004836-199303000-00008]
29 Murao T, Toda K, Tomiyama Y. Lymphangioma of the pancreas. A
case report with electron microscopic observations. Acta Pathol Jpn
1987; 37: 503-510 [PMID: 3618222 DOI: 10.1111/j.1440-1827.1987.
tb00384.x]
30 Fang YF, Qiu LF, Du Y, Jiang ZN, Gao M. Small intestinal hemo-
lymphangioma with bleeding: a case report. World J Gastroenterol
2012; 18: 2145-2146 [PMID: 22563205 DOI: 10.3748/wjg.v18.
i17.2145]
P- Reviewer: Cosen-Binker L, Sakata N, Sumi S S- Editor: Ma YJ
L- Editor: A E- Editor: Wang CH
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... As a congenital malformation of the vascular system, cavernous hemangioma is an uncommon type of primary cystic neoplasm. The occurrence of pancreatic hemangiomas in adults is extremely rarely reported , especially that of cavernous hemangioma [9,15,21]. Adult pancreatic hemangiomas often manifest as large cystic lesions in middle-aged females, and in many cases, the patients exhibit abdominal pain but no evidence of malignancy [11,13,16,19]. The major symptoms of cavernous hemangioma are abdominal pain and distension associated with the enlarged tumor [1,7,11]. ...
... As a benign neoplastic lesion with slow growth and rarely malignant changes, these lesions are commonly found in the skin, eyes, liver, brain, spleen and other organs, while cavernous hemangioma occurring in the pancreas is extremely rare [22]. Pancreatic cavernous hemangioma is more common in women than in men (the male to female ratio is 1:3), the exact cause of the disease is unknown, and it is mostly recognized as a congenital disease [11,15]. The age of the patients at diagnosis Fig. 1 The tumor was a multilocular cyst with septa and fluid-fluid levels. ...
... Gross pathology showed a cystic lesion with thick septa ranges from 18 to 79 years, and it occurs more frequently in childhood than adulthood . It progresses slowly to the stage of degeneration after hyperplasia in infancy and gradually disappears after many years, leaving traces of fiber and fat in adulthood [11,15,23]. Adult pancreatic hemangioma is very rare, and its pathological nature is completely different from that of childhood pancreatic hemangioma [11,15,23]. ...
Article
Full-text available
Background: Pancreatic cavernous hemangioma is an extremely rare benign tumor that is difficult to diagnose on an imaging examination, and its histopathological examination has rarely been reported. Case presentation: Herein, we present the case of a 63-year-old man who was admitted to the hospital due to left upper abdominal pain and defecation unformed for more than 2 years. None of the positive results obtained from the physical examination could explain his symptoms. The imaging examination indicated a multilocular cyst with septa in the head of the pancreas. The patient underwent a pancreaticoduodenectomy, and the pathologic diagnosis was pancreatic cavernous hemangioma. The histopathological examination showed that the lesion was positive for benign vascular markers, such as CD31, CD34 and F8, and negative for lymphocyte markers, such as D2-40. Moreover, it was also positive for ERG and cytokeratin markers, CAM5.2 and AE1/AE3, indicating the complexity of its components, and Ki-67 negativity revealed its benign nature. Conclusions: Pancreatic cavernous hemangioma has a complex composition that may be reflected not only in the imaging examination but also in the immunohistochemical detection, and it may achieve a good outcome by surgical excision.
... Although pancreatic hemangioma is a benign tumor, it can be difficult to differentiate them from epithelial tumors of the pancreas, also because of the risk of bleeding [27], surgical excision is still the best treatment in adults. Most patients who underwent pancreas hemangioma resection have good prognosis [10,20,21]. ...
... According to the location of the tumor in pancreas, different resection methods could be choose. Tumors located in the head of the pancreas have been treated by pancreaticoduodenectomy in most cases [6,10,11,15,17,21,27,29]. Tumors located in the body and tail of the pancreas [8,12,16,22,24,25] have been treated by local resection with spleen preservation or partial excision. ...
... Thus, it is necessary to undertake both CT and MRI for a more reliable diagnosis. However, it is still difficult to make a definite diagnosis in some cases (11,17,21,22,27) though both CT and MRI were undertaken. Endoscopicultrasound guided fine needle aspiration (EUS-FNAB) was undertaken in 3 reports (13,18,22), which can give some hints and exclude the malignancy of the lesion. ...
... However, it is still difficult to make a definite diagnosis in some cases (11,17,21,22,27) though both CT and MRI were undertaken. Endoscopicultrasound guided fine needle aspiration (EUS-FNAB) was undertaken in 3 reports (13,18,22), which can give some hints and exclude the malignancy of the lesion. It is worth doing if conditional. ...
Article
Adult pancreatic hemangioma is an especially rare benign tumor that is hard to diagnose through imaging examinations, meanwhile its histopathological and immunohistochemical studies have rarely been reported. At this time, only 29 cases in 27 publications have been reported. We report a new case as well as review these literatures. A 71-year-old woman came to our hospital with slight pain on left upper abdomen for three days. But the results obtained from the physical or laboratory examination were all negative so that her symptoms could not be explained clearly. The imaging examinations including ultrasonography and computed tomography both showed a mixed mass in the neck of the pancreas. The preoperative diagnosis of cystadenoma or adenocarcinoma was made, with high suspicious of malignancy. The patient underwent a central pancreatectomy with pancreatojejunostomy, but the pathologic diagnosis was pancreatic hemangioma. The immunohistochemical examination found the positive benign vascular markers (CD31, CD34) and negative lymphocyte markers (D2-40). Moreover, Ki-67 negativity also revealed its benign nature. After 36 months of follow-up, the patient has no complaints for abdominal pain. In conclusion, adult pancreatic hemangioma is extremely rare without any specific clinical manifestations. It is usually diagnosed postoperatively by histological examination and immunohistochemical studies. Imaging examinations, including computed tomography and magnetic resonance imaging, can't give definite conclusion. Endoscopic-ultrasound guided fine needle aspiration is conditionally worth doing, which can give some hints and exclude the malignancy of the lesion. If malignancy can be safely ruled out, the surgical decision must be made according to risk-benefit analysist. Maybe close observation and regular follow-up are more beneficial options.
... Adult pancreatic hemangioma is an extremely rare type of benign vascular tumor. To date, about 20 cases have been reported in the English literature [1]. Adult patients with pancreatic hemangiomas usually have no specific symptoms, particularly in early stages. ...
... EUS is another method often used to diagnose adult pancreatic hemangioma, which was applied in four patients. The tumors were generally devoid of a obvious vascular flow on Doppler imaging [1]. Endoscopic ultrasonography-guided fine needle aspiration (EUS-FNA) or core biopsy provides an effective way to identify pancreatic hemangiomas preoperatively. ...
Article
Full-text available
Background: Adult pancreatic hemangioma is an extremely rare type of benign vascular tumor. To date, about 20 cases have been reported in the English literature. Adult patients with pancreatic hemangiomas usually have no specific symptoms, particularly in early stages. Therefore, it is difficult to detect and diagnose these lesions, which usually are identified during cross sectional imaging for an apparently unrelated causes or when biliary obstruction occurs because of compression by a tumor. Case presentation: This study presents the case of a 52-year-old female with a chief complaint of epigastric pain. Contrast-enhanced computed tomography revealed a well-defined mass with mildly inhomogeneous enhancement in the body of the pancreas. Endoscopic ultrasonography showed calcifications in the lesion, and a few small vessels were detected by Doppler imaging. The patient received a central pancreatectomy, and pathological examination confirmed the diagnosis of pancreatic hemangioma. Conclusion: In this report, we reviewed the clinical manifestations, radiologic features, preoperative diagnosis, pathologic characteristics, and surgical treatment of adult pancreatic hemangioma.
... In rare instances, AVM has been reported to cause acute abdominal pain. In the literature, there are few cases where hemorrhage caused by AVM can present as acute abdomen and all the cases were diagnosed postoperatively [4][5][6][7]. ...
Article
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Arteriovenous and lymphatic malformations are a common phenomenon and can present in various clinical forms. They rarely present as a cause of acute abdomen, and in the literature, there are only a few cases where intra-abdominal arteriovenous malformation (AVM) presented as acute appendicitis in the adult population. Most of the cases are diagnosed post-operatively on histology but the previous reporting in the literature has made it possible for the radiologists to look for these anomalies and consider them as a probable cause of acute abdomen when no other obvious cause is found. We are presenting an interesting case of congenital vascular malformation mimicking acute appendicitis in a young female. She had a previous history of complications related to AVM and her complicated urological history was initially focussed causing a delay in referral to surgeons but finally imaging was done which helped in final diagnosis. A cute appendicitis is the most common cause of acute abdominal pain with an incidence of 5.7-57/100,000 population [1]. Arteriovenous malformations (AVM) are congenital in nature and usually found sporadically. While in only 2% of cases they are multiple, most of them are solitary [2]. According to a study, only12% of AVM become symptomatic in the course of life [3]. In rare instances, AVM has been reported to cause acute abdominal pain. In the literature, there are few cases where hemorrhage caused by AVM can present as acute abdomen and all the cases were diagnosed postoperatively [4-7]. The case we are presenting is unique as hemorrhage caused by an AVM in common iliac vein and external iliac artery caused inflammation around the appendix and adjacent area presented clinically as acute appendicitis. The patient was diagnosed preoperatively by imaging and not subjected to surgery, as the episode proved to be self-limiting. CASE REPORT A 23-year-old young girl presented with 3 days history of lower abdominal pain which started suddenly and then radiated to her right groin which she described in her own words felt like a 'stitch'. There were no aggravating or relieving factors for the pain. Her pain symptoms were accompanied by urinary frequency and offensive smell in her urine for the last three days. She recently had cough and coryza which settled on its own. The pain progressively worsened and forced the patient to seek medical attention. Her past medical history included congenital AQ1 Figure 1: CT scan coronal plane image of the abdomen and pelvis showing (a) AVM/Lymphatic malformation in spleen and pelvis and (b) extensive AVM and soft tissue stranding in the pelvis around Iliac vessels and RIF close to appendix.
Chapter
Rare pancreatic tumors include uncommon lesions, which are rarely seen even by specialists in high-volume centers. Consequently, data on these lesions are limited. Their diagnosis and clinical management are therefore highly challenging and warrant an interdisciplinary approach integrating all available information, including clinical, laboratory, imaging data and pathological evaluation. The group includes rare epithelial neoplasms, ranging from potentially malignant lesions like solid-pseudopapillary neoplasms, to malignant entities such as pancreatoblastomas or acinar cell carcinomas, as well as rare non-epithelial neoplasms, which may be benign (e.g. haemangiomas or lymphangiomas), potentially malignant (e.g. PEComas or teratomas) or frankly malignant (e.g. sarcomas). Rare tumor-like lesions of the pancreas, such lymphoepithelial cysts, parasitic cysts, pseudolymphomas, among others, complete the spectrum of these rare entities.
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Pancreatic haemangiomas are a rare cause of pancreatic lesions in adults. Diagnosis is challenging as they are seldom suspected and difficult to differentiate on imaging. Historically, pancreatic haemangiomas have been managed surgically despite their benign nature, largely due to diagnostic uncertainty. We present the case of a 69-year-old woman who, through combination of radiological, biochemical and endoscopic investigations, was diagnosed with pancreatic haemangioma and managed conservatively, avoiding the morbidity and mortality associated with surgical resection of a benign lesion.
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Adult pancreatic hemangioma is an extremely rare disease, with only 22 cases reported since 1939. Pancreatic hemangioma has no specific symptoms, diagnostic imaging, or laboratory findings, making it difficult to be clinically suspected and diagnosed. The majority are confirmed after surgery. In this report, a 61-year-old woman presented with melena and showed multiple small hyper-vascular lesions in the pancreas. A pancreatic neuroendocrine tumor was suspected, and the patient underwent a distal pancreatectomy. The pathology examination and immunohistochemical study revealed a pancreatic hemangioma.
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Background/aim: Hemangiomas are benign neoplastic proliferations of blood vessels. Cytogenetic information on hemangiomas is limited to four tumors with abnormal karyotypes. We report here a solitary chromosomal translocation and its molecular consequence in a hemangioma. Materials and methods: A cavernous hemangioma was extirpated from the foot of a 62 years old man and genetically studied with cytogenetic and molecular genetic methodologies. Results: G-Banding analysis of short-term cultured tumor cells yielded the karyotype 46,Y,t(X;15)(q22;q26)[4]/46,XY[12]. RNA sequencing detected fusion of the collagen type IV alpha 5 chain gene (COL4A5 on Xq22.3) with intronic sequences of nuclear receptor subfamily 2 group F member 2 antisense RNA 1 (NR2F2-AS1 on 15q26.2) resulting in a putative COL4A5 truncated protein. The fusion was verified by RT-PCR together with Sanger sequencing and FISH analyses. Conclusion: The involvement of COL4A5 indicates that some hemangiomas have pathogenetic similarities with other benign tumors such as leiomyomas and subungual exostosis.
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Vascular neoplasms of the pancreas are extremely rare and usually manifest as symptomatic, cystic lesions. This study presents a case that includes the clinicopathologic information used to discriminate pancreatic hemangioma from other types of cystic lesion of the pancreas. A 40yearold female visited hospital with a chief complaint of abdominal pain. The serum CEA and CA199 levels of the patient were within the normal limits. An abdominal computed tomography scan and magnetic resonance imaging showed a 100mm mass lesion in the body and tail of the pancreas, and the tumor extended toward the retroperitoneum and surrounded the splenic vein. The lesion was subsequently resected. Macroscopically, it was a multiloculated cyst with intracystic hemorrhage. Microscopically, the lesion was composed of numerous, heterogeneous cysts lined by a flattened single layer of cells without significant atypia. Notably, numerous neoplastic vessels extended into the interlobular septa of the pancreas and surrounded the main pancreatic duct. Immunohistochemical analysis showed that the lining cells expressed CD31 and CD34. The lesion was diagnosed as adult pancreatic hemangioma. Surgical treatment may be required when a direct contact between the lesion and the pancreatic tissue is demonstrated using imaging.
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Hemangiomas in the pancreas are very rare and only a few cases in adulthood have been reported in the literature. We describe a case of pancreatic hemangiomas in an adult with unique imaging findings. A 23-year-old woman visited the hospital for an incidentally detected pancreatic mass. CT and MRI revealed a multilocular cyst with fluid-fluid levels and no obvious enhancement. The patient underwent surgery and the mass was confirmed as a pancreatic hemangioma. The radiological features and differential diagnosis of this rare lesion are discussed.
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Rare solid tumors of the pancreas can be misinterpreted as primary pancreatic cancer. The aim of this study was to report our experience in the treatment of patients with rare tumor lesions of the pancreas and to discuss clinical and pathological characteristics in the context of the role of surgery. Data from patients of our prospective data-base with rare benign and malignant tumors of the pancreas, treated in our division from January 2004 to August 2010, were analyzed retrospectively. One-thousand and ninety-eight patients with solid tumors of the pancreas underwent pancreatic surgery. In 19 patients (10 women, 9 men) with a mean age of 57 years (range: 20-74 years) rare pancreatic tumors (metastasis, solid pseudopapillary tumor, teratoma, hemangioma, accessory spleen, lymphoepithelial cyst, hamartoma, sarcoidosis, yolk sac tumor) were the reason for surgical intervention. If rare benign and malignant pancreatic tumors, intrapancreatic metastasis, as well as pancreatic malformations or other abnormalities, present themselves as solid masses of the pancreas, they constitute an important differential diagnosis to primary pancreatic neoplasia, e.g. pancreatic ductal adenocarcinoma. Clinical imaging techniques cannot always rule out malignancy, thus operative exploration often remains the treatment of choice to provide the correct diagnosis and initiate adequate surgical therapy.
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Pancreatic haemangiomas are rare benign tumours that can affect both adults and children. They have an unknown incidence and only 15 adult cases have been reported, all from histological examination. Patients present with vague symptoms relating to tumour mass or they are detected incidentally. Cross-sectional imaging is the mainstay of investigation and may reveal arterially enhancing cystic lesions but in the case presented here, it was non-diagnostic. The use of endoscopic ultrasonography confirmed the nature of the benign lesion, allowing a conservative approach as opposed to operative resection.
Article
A case of lymphangioma of the pancreas In a 49-year-old woman is presented. The patient noticed a left hypochondral mass, which was clinically diagnosed as a multicystic tumor of the pancreas. A partial pancreatectomy and splenectomy were performed. The surgical specimen showed a multicystic mass measuring 13×9×5 cm which occupied the anterior region of the body and tail of the pancreas. From the light and electron microscopic findings, the multicystic mass was diagnosed as a lymphangioma with cystic and cavernous features. A small glucagonoma was incidentally found in the tail of the resected pancreas. The previous literature of pancreatic lymphangiomas is reviewed and the clinico-pathologic characteristics of this tumor are discussed.
Article
A large pancreatic cavernous hemangioma was found in a 30-year-old man with abdominal distention. Plain and contrast-enhanced computed tomography (CT), magnetic resonance imaging (MRI), ultrasonography (US), and angiography were performed prior to operation. Contrast-enhanced CT and angiography showed a large poorly enhanced hypovascular tumor at the head of the pancreas. But MRI and US disclosed findings compatible with a cavernous hemangioma.
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Small intestinal hemolymphangioma is a very rare benign tumor. There was only one report of a hemolymphangioma of the pancreas invading to the duodenum until March 2011. Here we describe the first case of small intestinal hemolymphangioma with bleeding in a 57-year-old woman. She presented with persistent gastrointestinal bleeding and endoscopy revealed a small intestinal tumor. Partial resection of the small intestine was thus performed and the final pathological diagnosis was hemolymphangioma. We also highlight the difficultly in making an accurate preoperative diagnosis in spite of modern imaging techniques. To arrive at a definitive diagnosis and exclude malignancy, partial resection of the small intestine was considered to be the required treatment.
Article
Pancreatic hemangioma is an extremely rare tumor, with only a very few cases reported in the literature. We present the case of a 36-year-old man admitted to the hospital with jaundice and abdominal pain. Contrast-enhanced CT showed a hypervascular mass with large vessels in the pancreatic head. The mass appeared moderately hypervascular on gadolinium-enhanced MRI, and, on mangafodipir-enhanced MRI, no contrast agent uptake into the mass could be detected. Despite the size of the lesion, no dilatation of the pancreatic and the common bile duct were seen. Pancreatic hemangioma should be included in the list of differential diagnoses in case of a hypervascular pancreatic lesion without signs of mass effect.