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Adult pancreatic hemangioma in pregnancy - Concerns and considerations of a rare case


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Background: Pancreatic tumors in pregnancy are rare but clinically challenging. Careful diagnostic workup, including appropriate imaging examinations, should be performed to evaluate surgery indications and timing . In the present case a diagnosis of an adult pancreatic hemangioma was made. We were not able to identify a similar case in the very sparse literature on this rare disease. Case presentation: A 30-year-old woman at 12 weeks of gestation was diagnosed with a large pancreatic tumor having a cystic pattern based on imaging. Although the preoperative diagnosis was uncertain, patient preference and clinical symptoms and signs suggested surgery. Open distal pancreatic resection including splenectomy was performed, and complete resection of the large cystic tumor was successfully achieved, with no postoperative complications. Although a solid pseudopapillary epithelial neoplasm (SPEN) was suspected, specimen morphology, including immunohistochemistry, supported the diagnosis of an adult benign pancreatic hemangioma. Conclusion: Although mucinous cystic neoplasm (MCN) and adenocarcinoma are the most common pancreatic tumors during pregnancy, various other malignant and benign lesions can be encountered. This report adds to the very small number of pancreatic hemangiomas reported in the literature and involves the first patient diagnosed with this rare condition during pregnancy. Careful clinical considerations regarding diagnostic workup and treatments are required to ensure that mother and child receive the best possible care.
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C A S E R E P O R T Open Access
Adult pancreatic hemangioma in pregnancy
concerns and considerations of a rare case
Jon Arne Søreide
, Ole Jakob Greve
and Einar Gudlaugsson
Background: Pancreatic tumors in pregnancy are rare but clinically challenging. Careful diagnostic workup,
including appropriate imaging examinations, should be performed to evaluate surgery indications and timing . In
the present case a diagnosis of an adult pancreatic hemangioma was made. We were not able to identify a similar
case in the very sparse literature on this rare disease.
Case presentation: A 30-year-old woman at 12 weeks of gestation was diagnosed with a large pancreatic tumor
having a cystic pattern based on imaging. Although the preoperative diagnosis was uncertain, patient preference
and clinical symptoms and signs suggested surgery. Open distal pancreatic resection including splenectomy was
performed, and complete resection of the large cystic tumor was successfully achieved, with no postoperative
complications. Although a solid pseudopapillary epithelial neoplasm (SPEN) was suspected, specimen morphology,
including immunohistochemistry, supported the diagnosis of an adult benign pancreatic hemangioma.
Conclusion: Although mucinous cystic neoplasm (MCN) and adenocarcinoma are the most common pancreatic
tumors during pregnancy, various other malignant and benign lesions can be encountered. This report adds to the
very small number of pancreatic hemangiomas reported in the literature and involves the first patient diagnosed
with this rare condition during pregnancy. Careful clinical considerations regarding diagnostic workup and
treatments are required to ensure that mother and child receive the best possible care.
Keywords: Cystic lesion, Hemangioma, Pancreas, Pregnancy
In pregnant women, the acute abdomen is often a chal-
lenge. Careful clinical evaluation, close cooperation be-
tween the surgeon and the gynecologist as needed, and
the application of appropriate diagnostic tools and edu-
cated judgment remain the cornerstones of standard
care [16]. Although the diagnostic workup can be
demanding in these cases, a responsible surgeon might
regard appropriate surgical treatment as stressful for the
relatively common diseases encountered during preg-
nancy such as appendicitis [7, 8] and acute cholecystitis
[9, 10]. A surgeon is confronted by even greater responsi-
bility for a mother and her child in clinical settings involv-
ing rare acute abdominal conditions of pregnancy, which
include acute severe pancreatitis [11], gastrointestinal
bleeding [12, 13], and suspected tumors in various loca-
tions [1419]. In cases involving a cystic lesion, the nature
of the lesion, including its malignant potential and the risk
of a spontaneous rupture, must be considered when dis-
cussing indications for and the timing of surgery [15, 20].
In this situation, the surgical approach, which varies
depending on the pregnancy trimester is a particularly
concerning issue.
In this report, we discuss clinical considerations re-
lated to the unexpected finding of a large cystic lesion
that appears to be related to the pancreas in a pregnant
woman with acute abdomen.
Written informed consent was obtained from the patient
for publication of this Case report and any accompany-
ing images. A copy of the written consent is available for
review by the Editor of this journal.
* Correspondence:
Department of Gastrointestinal Surgery, Stavanger University Hospital,
N-4068 Stavanger, Norway
Department of Clinical Medicine, University of Bergen, Bergen, Norway
Full list of author information is available at the end of the article
© 2015 Søreide et al. Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0
International License (, which permits unrestricted use, distribution, and
reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to
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( applies to the data made available in this article, unless otherwise stated.
Søreide et al. BMC Surgery (2015) 15:119
DOI 10.1186/s12893-015-0106-1
Content courtesy of Springer Nature, terms of use apply. Rights reserved.
Case presentation
An otherwise healthy, non-obese 30-year-old woman,
para 0, at 12 weeks gestation was admitted to our hospi-
tals Department of Gastrointestinal Surgery with a
history of 3 weeks of varying but increasing pain in the
left upper quadrant of her abdomen and nausea without
vomiting. A palpable left subcostal mass was detected by
clinical examination, which revealed no other notable
findings except for her pregnant status. Ultrasound (US)
examination (Fig. 1) revealed a large cystic lesion (size,
17 × 10 cm) with septa related to the tail of the pancreas
and spleen. Magnetic resonance imaging (MRI) depicted a
multicystic lesion related to the pancreatic tail, between
the spleen and left kidney, with moderate dislocation of
both the kidney and spleen (Fig. 2). Routine biochemistry
was normal, and tumor markers carcinoembryomic anti-
gen (CEA = 4 μg/l), cancer antigen 125 (CA125 = 42kU/l),
carbohydrate antigen 199 (CA199 < 5 kU/l), and chro-
mogranin A(CgA= 2.8 nmol/l) were all within normal
ranges. The preoperative diagnosis was uncertain; how-
ever, based on imaging findings and the patientsyoung
age, a solid pseudopapillary epithelial neoplasm (SPEN)
was considered. A gynecologist evaluated the patients
pregnancy as normal. Relevant factors when considering
indications for surgery included the presence of increasing
clinical symptoms in a pregnant woman, the uncertain
nature of the rather large cystic pancreatic tumor, and the
possible risk and undesirable consequences of a rupture of
this tumor during the second or third trimester. In
accordance with the patients preferences, surgical treat-
ment was recommended. Open resection of the most dis-
tal portion of the pancreas, including the large cystic
tumor and spleen, was performed. Frozen sections of the
pancreas resection border confirmed that the resected
tissue was benign. The patients postoperative course was
uneventful, and she was able to leave the hospital on the
postoperative day without complications.
The multicystic tumor (Fig. 3) measured 19.5 × 10 × 7 cm
and exhibited close adherence to the distal pancreas but
without infiltration in the pancreas parenchyma or any
communication with the pancreatic ducts. Microscopically,
benign pancreatic tissue was confirmed, and the tumor was
multicystic with extremely thin septa, fibrosis and a
pattern of chronic inflammation, but no epithelial
tissue indicating that SPEN was unlikely. Additional
immunohistochemistry (IHC) with CD34 and CD 31
(Fig. 4) provided information to support a large-vessel
hemangioma lined with single endothelial layer without
cytologic atypia, and with focal degenerative changes in
septa and the cystic wall. This endothelial lining was
negative for cytokeratins and calretinin, excluding epi-
tehelial and mestothelial nature of the lesion Taken
together, the tumor morphological findings were char-
acteristic of a truly benign lesion most consistent with
adult pancreatic hemangioma, which was resected com-
pletely without any ruptures.
After surgery, the patients pregnancy proceeded un-
eventfully, and she spontaneously delivered a healthy child
Fig. 1 Ultrasound showed a large cystic lesion with septa and some sedimentation. Minimal Doppler signal of the large tumor (18 × 10 cm).
Spleen, pancreas and left kidney without focal lesions
Søreide et al. BMC Surgery (2015) 15:119 Page 2 of 6
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at the calculated gestational time. During follow-up of at
least 12 postoperative months, the patient has expressed
no complaints or concerns related to her treatment.
While hemangiomas are rarely found in the pancreas,
they are very common in the liver. Mundinger et al.
reviewed the literature and found only 9 cases with con-
firmed adult pancreatic hemangiomas reported between
1939 and 2009 [21]. The lesions were most commonly
located in the head, or head/body of the gland, with a
size varying from 3 cm to 20 cm in diameter. Recently,
Bursics and coworkers [22] reported on another patient
(a 72-year-old man) surgically treated for a confirmed
adult hemangioma of the pancreas, and presented rele-
vant data on the 12 reported cases, including their own,
published until 2013. Male:female ratio was found to be
2:1, and the median age was 61 (range, 3079) years. As
demonstrated by these 12 reported cases in the world
literature [21, 22], pain was the most common clinical
symptom which was also the case in our patient. The
pattern of a suggested cystic lesion by imaging was
Fig. 2 Coronal (a) and transversal (b) MRI T2 (FIESTA) view. Large tumor (T) medial to the spleen (S) and adjacent to the pancreatic tail dislocating the
left kidney (K) medial and cranial. Mostly high signal intensity at T2 and intermediate to high at T1, indicating cystic components with protein or blood. A
smaller part of the lesion had lower signal and diffusion restriction indicating more solid parts. (P) is the pregnant uterus. A solid pseudopapillaryepithel
neoplasia (SPEN) of the pancreas was suspected in this young woman with an encapsulated lesion with cystic, solid and hemorrhagic components
Fig. 3 Operative specimen with the cystic tumor and the spleen seen from the ventral (left) and the dorsal (right) side. The cystic tumor
measured 19.5 × 10 × 7 cm
Søreide et al. BMC Surgery (2015) 15:119 Page 3 of 6
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found in most patients with available information in
this regard. Notably, the CT patterns of a pancreatic
hemangioma are different from the CT signs of a liver
hemangioma, as a typical early peripheral contrast-
enhancement during the arterial phase is missing in the
former [23]. Therefore, this imaging modality is inef-
fective for ruling out pancreatic hemangioma.
Our patient was definitely in the younger range of age,
and importantly, we report on the first pregnant patient
diagnosed with an adult pancreas hemangioma. Although
the final morphologic diagnosis of this rare condition can
be challenging, CD 31 and CD 34 immunohistochemical
labeling adds valuable support to the diagnosis of a neo-
plasm of vascular origin [21].
Both benign and malignant pancreatic neoplasms are
rarely diagnosed during pregnancy; in particular, pancre-
atic cancer during pregnancy is extremely rare, with
fewer than 10 described cases to date [15, 16]. However,
a number of dilemmas can be encountered when
attempting to determine an accurate diagnosis to enable
appropriate treatment. Within this context, both US and
MRI are reliable and useful imaging modalities [5, 20]
without known health risks for the fetus [24]. When in-
dicated, CT can also contribute to imaging findings. Of
note, the risk that the fetus will develop congenital ab-
normalities due to the side effects of radiation exposure
(via repeat and/or multiphase CT scans) is considered to
be extremely low [25], nonetheless, due to concerns
regarding the potential for deleterious side effects, radi-
ation and various contrast agents should be limited and
used with care [24, 25].
Del Chiaro et al. [26] recently demonstrated that the
overall accuracy of preoperative diagnoses of cystic pan-
creatic lesions is only approximately 60 %, with similar
accuracies for asymptomatic and symptomatic lesions.
Thus, inaccurate preoperative assessments of pancreatic
cystic lesions are common; however, diagnostic errors
are clinically relevant in less than 10 % of these cases.
Data regarding lesion size, patient gender, and the
patients prior history (with respect to pancreatitis or
other relevant diseases) could be valuable information for
reaching appropriate decisions and thereby preventing the
overutilization of operative resection in patients with these
lesions [27]. For non-pregnant patients, additional im-
aging (e.g., contrast CT scans) and endoscopic ultrasound
(EUS)-guided aspiration of the cystic lesion for analyses of
DNA mutations and proteins within pancreatic cyst fluid
can contribute to the diagnostic workup [20, 28]. How-
ever, due to potential risks for a biopsy related bleeding,
rupture of the lesion or peritoneal seeding of biopsy
material, we did not regard our symptomatic pregnant pa-
tient with a large pancreatic lesion as a candidate for this
Fig. 4 aLarge dilated vascular structures lined by endothelial cells filled with red blood cells. The thickened vessel walls are composed of fibrous or
spindle cell stroma with mild chronic inflammation. (H and E; original magnification: ×10). bLarge dilated vascular structures lined by endothelial cells
filled with red blood cells. The thickened vessel walls are composed of fibrous or spindle cell stroma with mild chronic inflammation and hemosiderin
loaded macrophages (arrow) (H and E; original magnification: ×20). cMicroscopy showing large dilated vascular structure lined by endothelial cells.
The wall is infiltrated with mild chronic inflammation. (H and E; original magnification: ×100). d. Immunohistochemical expression of CD31 (brown)
depicting the endothelial lining of larger vascular spaces and small vessels in the stroma (original magnification, ×20)
Søreide et al. BMC Surgery (2015) 15:119 Page 4 of 6
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diagnostic approach. Her subjective and increasing com-
plaints, the uncertain nature of the lesion, and the risk of
tumor rupture with undesired side effects or complica-
tions later during her pregnancy, were all important con-
siderations for the timing of treatment. The preoperative
diagnosis of a possible SPEN was not definitively deter-
mined. As recently suggested [29] SPENs are rare cystic
lesions that frequently occur in young women and pa-
tients with these lesions exhibit good prognoses if radical
surgical resection can be achieved. Despite the challenging
nature of a definite diagnosis, the only feature of the de-
scribed case that could be associated with malignancy was
a large tumor size at diagnosis [20, 29]. Thus fare, malig-
nancy has not been reported for pancreatic hemangiomas.
Based on the rarity of this condition, the lack of reliable
follow-up data, and the sparse literature on this topic, an
understanding of the pathophysiology and natural history
of these lesions remains at an early stage [21].
Although reports have described a number of anecdotes
involving worrisome histories of pregnant women with
malignant tumors, including carcinomas of the pancreas
[16, 30, 31], pancreatic neuroendocrine tumors [14], and
bleeding neoplasms [32], or spontaneous SPEN rupture
[33], these cases are generally exceptions rather than the
rule. Nevertheless, given the objective of providing opti-
mal care to both mother and child, the determination of
an appropriate treatment schedule for any young pregnant
woman requires the consideration of a wide range of
diagnoses and a number of important factors. Although
the second trimester is considered to be the most favor-
able time for surgical intervention for pancreatic tumors
[15], multidisciplinary consultation should occur with
respect to a pregnant patients diagnosis, indications and
timing to ensure that the best possible outcomes for both
the mother and the child are achieved [15, 18, 19, 22, 26].
CT: Computer tomography; EUS: Endoscopic ultrasound;
IHC: Immunohistochemistry; MCN: Mucinous cystic neoplasm; MRI: Magnetic
resonance imaging; SPEN: Solid pseudopapillary epithelial neoplasm.
Competing interests
The authors declare they have no competing interests.
Study conception and design: JAS. Data acquision: JAS, OJG, EG. Data
interpretation: JAS, OJG, EG. Drafting of the manuscript: JAS. Manuscript
editing and final approval: JAS, OJG, EG.
The authors appreciate the patients consent to present this case.
Author details
Department of Gastrointestinal Surgery, Stavanger University Hospital,
N-4068 Stavanger, Norway.
Department of Radiology, Stavanger University
Hospital, Stavanger, Norway.
Department of Pathology, Stavanger University
Hospital, Stavanger, Norway.
Department of Clinical Medicine, University of
Bergen, Bergen, Norway.
Received: 6 July 2015 Accepted: 19 October 2015
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... 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. ...
... Clinical characteristics of all cases are summarized in Table 1. According to these cases, just as other hemangiomas, the pancreatic hemangioma is also more commonly found in women (23 in this series, 77%), sometimes during pregnancy (15,23), indicating the importance of excess of female sex hormones in these tumors (30 (2,10,17,25), one found at autopsy (2), while the other three found in incidental imaging examinations. Excluding the one found at autopsy, other asymptomatic cases are all suspicious of malignancy. ...
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.
... 5 Though biopsy is an important tumor diagnostic tool, some authors do not recommend it during pregnancy because of the potential risks of biopsy related bleeding, rupture of the lesion, or peritoneal seeding of the biopsy material. 12 Acute hemoperitoneum is a rare obstetrical emergency, and its presence in combination with an epigastric mass is rarer. The differentials are few. ...
Full-text available
Introduction: Hemoperitoneum resulting from the rupture of pancreatic tumors is a rare condition, especially during pregnancy. Case presentation: We report a case of a 21-year-old gravida 2, para 1, at 25+5 weeks of gestation, who presented to the hospital with severe epigastric pain and decreased fetal movement. Ultrasonography showed intrauterine fetal death, a retroperitoneal mass in the epigastric region, and hemoperitoneum. Computed tomography scan revealed a heterogeneously enhancing pancreatic mass suggestive of pancreatic neoplasm. However, the late diagnosis and the delay in treatment resulted in a deterioration of maternal status with eventual mortality. Conclusion: Diagnostic difficulties occur because of the rarity of the condition and vague clinical presentations. In case of a pregnancy complicated by hemoperitoneum, prompt effort to stop the intraperitoneal bleeding is imperative.
... At the same time, the majority of pancreatic hemangiomas were described as multilocular cystic lesions [10,11,12,13]. ...
Abstract Pancreatic hemangiomas are benign tumors extremely rare in adults, rarer than in children. Although were reported a small number of pancreatic hemangiomas, mostly were proved to be hyper vascular tumors. We present the case of a sixty-four years old male who had acute upper abdominal pain, and who was admitted, with the suspicion of acute pancreatitis. Imagistic examinations detected a tissular nodule in the body of the pancreas, with well- defined margins, without capsule, with heterogeneous structure, and with maximum size of 1.9 cm. Also, it was discovered a similar lesion, regarding the imagistic appearance, in the fourth segment of the liver, and a hemangioma in the sixth segment of the liver. There were no lymphadenopathies in ultrasound and computed tomography exams. Although was suspected a pancreatic adenocarcinoma with liver metastasis, the patient was planned for surgery, knowing that this type of neoplasia is extremely aggressive with a poor prognosis if is not surgically removed. Histopathological exam indicate that the pancreatic mass was a hemangioma.
Background: Mucinous cystic neoplasms and solid pseudopapillary neoplasms are the most common pancreatic tumors occurring in women of fertile age and in pregnant women. The aim of this study is to provide an updated literature review on this association and to present a fully laparoscopic resection of a pregnancy-associated pancreatic cystic neoplasm. Materials and methods: A systematic literature review was performed using PubMed (MEDLINE), Scopus, Ovid, ISI Web of Science, and Google Scholar for searching. The syntax was (pancr*) AND (cyst*) AND (pregn*) AND (tumor). Only English-language articles describing pancreatic surgical resections were included. Results: Forty-seven case reports were included. The mean age of the patients was 29.6±5.3. Nine patients (20%) required emergency surgery, 4 (9%) due to cyst rupture, and 5 (11%) due to hemorrhage. Four patients (9%) suffered a miscarriage, and 2 (5%) opted for pregnancy termination; the rest of the women delivered a healthy newborn (86%, n=36). Thirty percent (n=14) of the resected neoplasms were malignant, and among mucinous cystic lesions, this raised to 45% (n=11). All patients diagnosed during the third trimester were resected postpartum, whereas 26/34 (76%) of patients diagnosed during the first 2 trimesters underwent surgery before delivery. Conclusions: The most worrisome complications in pregnancy-associated pancreatic cysts are bleeding or rupture. Mucinous cystic neoplasm has a tendency to grow during pregnancy. A postpartum resection was generally preferred when the cystic neoplasm was diagnosed during the third trimester. This report is the first to describe a fully laparoscopic pancreatic resection.
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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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.
Pancreatic haemangiomas are benign vascular tumours very rare in adults. Twenty-two cases are described in the literature. The symptoms are non-specific, and therefore rarely clinically suspected, and the vast majority are incidental findings in imaging tests such as ultrasound, CT, angiography or MRI. They appear on CT as a cystic lesion with contrast enhancement in the arterial phase. We present the case of a 36-year-old male patient with no history of disease, referred with lumbar pain and suspected renal calculus after tomography showing hypervascular enhancement in the pancreatic body and infiltrative lesion (possible neuroendocrine neoplasia) on MRI and biliopancreatic echoendoscopy. He was submitted to laparotomy with subtotal pancreatectomy and splenectomy and satisfactory evolution.
There are a few entities that account for most solid and cystic masses of the pancreas. The pancreas harbors a wide array of diseases, including adenocarcinoma, and its variants, such as anaplastic and adenosquamous carcinoma. Other neoplasms include acinar cell carcinoma, solid pseudopapillary tumor, and sarcomas. Benign lesions include hamartomas, hemangiomas, lymphangioma, and plasmacytoma. Isolated metastases include renal cell carcinoma, melanoma, and other carcinomas. Benign inflammatory conditions, such as autoimmune pancreatitis and groove pancreatitis can also mimic solid neoplasms of the pancreas.
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This is a case of a pregnant lady at 8 weeks of gestation, who presented with acute abdomen. She was initially diagnosed with ruptured ectopic pregnancy and ruptured corpus luteal cyst as the differential diagnosis. However she then, was finally diagnosed as acute hemorrhagic pancreatitis with spontaneous complete miscarriage. This is followed by review of literature on this topic. Acute pancreatitis in pregnancy is not uncommon. The emphasis on high index of suspicion of acute pancreatitis in women who presented with acute abdomen in pregnancy is highlighted. Early diagnosis and good supportive care by multidisciplinary team are crucial to ensure good maternal and fetal outcomes.
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An acute presentation after pregnancy of coeliac disease (CD) in the puerperium is a rare condition which has been described mostly in primigravidae in patients highly suspicious of latent CD. We report the case of a 37-year-old woman who was referred to our Hospital because of refractory watery diarrhea and malnutrition syndrome. Endoscopy of the upper gastrointestinal tract revealed the classic visual features of CD and in addition, some duodenal ulcers negative for Helicobacter pylori, which seems to be another clinical feature in patients with CD. The diagnosis of acute onset of fulminant postpartal CD (Marsh score stage 3c) was confirmed histologically. Remarkably, simultaneous well-differentiated neuroendocrine non-functioning pancreatic neuroendocrine carcinoma (PNET) was diagnosed on radiological abdominal imaging which was performed since serum gastrin was remarkably high, treated by distal pancreatectomy and splenectomy. This report is, to our knowledge, the first description of the two entities, CD and PNET occurring together. Since results of antral histological studies showed diffuse hyperplasia of G-cells, probably in response to hypergastrinaemia, enterochromaffin cell carcinogenesis might have served as a possible link between both diseases.
This chapter on the gastrointestinal and hepatic systems in pregnancy focusses on those conditions that are frequent and troublesome (gastro-oesophageal reflux and constipation), distressing (hyperemesis gravidarum) or potentially fatal (obstetric cholestasis, acute fatty liver of pregnancy and HELLP (haemolysis, elevated liver enzymes, low platelets) syndrome). It also highlights the clinical challenge obstetricians may face in managing rare conditions such as the Budd-Chiari syndrome, liver transplantation, primary biliary cirrhosis and Wilson disease. The clinical presentation of liver and gastrointestinal dysfunction in pregnancy is not specific, and certain 'abnormalities' may represent physiological changes of pregnancy. Diagnosis and management are often difficult because of atypical symptoms, a reluctance to use invasive investigations and concerns about the teratogenicity of the medications. The best available evidence to manage these conditions is discussed in the chapter.
Background: Spontaneous rupture of solid pseudopapillary neoplasm of the pancreas is an unusual complication during pregnancy. Case: At 19 weeks of gestation, a 29-year-old woman presented with a pancreatic mass and upper abdominal pain radiating to the back. On the third day of admission, shock and peritoneal signs developed. Exploratory laparotomy and subsequent subtotal pancreatectomy were performed for a bleeding tumor. Solid pseudopapillary neoplasm was confirmed by pathological examination. The patient delivered a healthy full-term girl vaginally. Eight months postoperatively, the clinical courses of both mother and infant have been uneventful. Conclusion: Ruptured solid pseudopapillary neoplasms can cause an acute abdomen during pregnancy. The expression of progesterone receptors in solid pseudopapillary neoplasm is a possible cause of this potentially devastating event.
About 20000 gastrointestinal endoscopies are performed annually in America in pregnant women. Gastrointestinal endoscopy during pregnancy raises the critical issue of fetal safety in addition to patient safety. Endoscopic medications may be potentially abortifacient or teratogenic. Generally, Food and Drug Administration category B or C drugs should be used for endoscopy. Esophagogastroduodenoscopy (EGD) seems to be relatively safe for both mother and fetus based on two retrospective studies of 83 and 60 pregnant patients. The diagnostic yield is about 95% when EGD is performed for gastrointestinal bleeding. EGD indications during pregnancy include acute gastrointestinal bleeding, dysphagia > 1 wk, or endoscopic therapy. Therapeutic EGD is experimental due to scant data, but should be strongly considered for urgent indications such as active bleeding. One study of 48 sigmoidoscopies performed during pregnancy showed relatively favorable fetal outcomes, rare bad fetal outcomes, and bad outcomes linked to very sick mothers. Sigmoidoscopy should be strongly considered for strong indications, including significant acute lower gastrointestinal bleeding, chronic diarrhea, distal colonic stricture, suspected inflammatory bowel disease flare, and potential colonic malignancy. Data on colonoscopy during pregnancy are limited. One study of 20 pregnant patients showed rare poor fetal outcomes. Colonoscopy is generally experimental during pregnancy, but can be considered for strong indications: known colonic mass/stricture, active lower gastrointestinal bleeding, or colonoscopic therapy. Endoscopic retrograde cholangiopancreatography (ERCP) entails fetal risks from fetal radiation exposure. ERCP risks to mother and fetus appear to be acceptable when performed for ERCP therapy, as demonstrated by analysis of nearly 350 cases during pregnancy. Justifiable indications include symptomatic or complicated choledocholithiasis, manifested by jaundice, cholangitis, gallstone pancreatitis, or dilated choledochus. ERCP should be performed by an expert endoscopist, with informed consent about fetal radiation risks, minimizing fetal radiation exposure, and using an attending anesthesiologist. Endoscopy is likely most safe during the second trimester of pregnancy.
Bleeding and pain are experienced by 20% of women during the first trimester of pregnancy. Although most pregnancies complicated by pain and bleeding tend to progress normally, these symptoms are distressing for woman, and they are also associated with an increased risk of miscarriage and ectopic pregnancy. Ultrasound is the first and often the only diagnostic modality that is used to determine location of early pregnancy and to assess its health. Ultrasound is an accurate, safe, painless and relatively inexpensive diagnostic tool, which all contributed to its widespread use in early pregnancy. Pain and bleeding in early pregnancy are sometimes caused by concomitant gynaecological, gastrointestinal, and urological problems, which could also be detected on ultrasound scan. In women with suspected intra-abdominal bleeding, ultrasound scan can be used to detect the presence of blood and provide information about the extent of bleeding. In this chapter, we comprehensively review the use of ultrasound in the diagnosis and management of early pregnancy complications. We include information about the diagnosis of gynaecological and other pelvic abnormalities, which could cause pain or bleeding in pregnancy. We also provide a summary of the current views on the safety of ultrasound in early pregnancy.
Acute cholecystitis is defined as inflammation of the gallbladder and is usually caused by obstruction of the cystic duct. Cholescintigraphy is the most sensitive imaging modality for cholecystitis. The gold standard treatment of acute cholecystitis is laparoscopic cholecystectomy. Operating early in the disease course decreases overall hospital stay and avoids increased complications, conversion to open procedures, and mortality. Cholecystitis during pregnancy is a challenging problem for surgeons. Operative intervention is generally safe for both mother and fetus, given the improved morbidity of the laparoscopic approach compared with open, although increased caution should be exercised in women with gallstone pancreatitis.
Acute appendicitis is one of the most common acute surgical presentations. However investigation and management is sometimes confounded in a pregnant patient. Appendicitis in pregnancy is often managed jointly by both the surgical and obstetric teams, which can lead to discrepant pathways, which may be detrimental to the patient. This review sets out to identify the normal physiological changes of pregnancy that pose diagnostic and therapeutic difficulties to the clinician, assess the more common differential diagnoses and review the current evidence to assist achieving a swift diagnosis and appropriate treatment. A literature review of the investigation and management of suspected appendicitis in pregnancy was undertaken. Guidelines by the relevant surgical, obstetric and radiological societies were also reviewed. There remains no consensus on the best diagnostic pathway for appendicitis in pregnancy; which is unsurprising given that appendicitis in non-pregnant patients can yield diagnostic conundrums. However this review identifies a role for MRI scanning as a useful adjunct in these patients. The increasing role of laparoscopy in these patients is also becoming more apparent. Appendicitis in pregnancy remains a complex problem necessitating a close working relationship between various specialties to achieve the best outcome for mother and fetus.
Diagnostic errors in the evaluation of cystic neoplasms of the pancreas (PCNs) are quite common. Few data are available regarding the impact of these errors on clinical management. The aim of this study was to determine the accuracy of a pancreatic multidisciplinary conference in diagnosing PCNs, to assess the potential risk of misdiagnosis, and to evaluate the clinical impact of these errors. A retrospective consecutive series of patients undergoing surgery for PCNs at Karolinska University Hospital between 2004 and 2012 was analyzed. During the study period, a total of 141 patients had undergone pancreatic resection for PCN. The overall accuracy of the preoperative diagnosis was 60.9 %. The rate of concordance between preoperative diagnosis and histology was similar for asymptomatic and symptomatic lesions (62.8 vs. 59.1 %; p = NS). The rate of correct diagnosis increased over time (54.5 % in 2004-2006, 61.7 % in 2007-2012, 63.5 % in 2010-2012). Univariate analysis identified the location of the lesion (diffuse pancreatic involvement) and a mucinous nature of the lesion as factors conducive to a correct diagnosis. Reevaluation of the original indication for surgery in light of the exact diagnosis showed that a surgical procedure should not have been performed in 12 patients (8.5 %). This study confirms that diagnostic errors are fairly common in the preoperative assessment of PCNs, but the errors are clinically relevant in <10 % of patients.