Esophageal Hemangiopericytoma or Hemangiopericytoma-Like Tumor: A Case Report and Review of the Literature

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Abstract
Hemangiopericytoma is a rare malignant vascular tumor deriving from pericytes. It usually occurs in the extremities and retroperitoneum, and extremely rarely in the gastrointestinal tract. A case of hemangiopericytoma in the gastroesophageal region is reported. A 56-year-old man was treated with H2-receptor antagonist for dyspeptic symptoms that had occurred three months before. Therapy failed to prove successful and gastroendoscopy was performed in March 2001 to reveal slightly reddish ventricular mucosa with normal rugal folds and a sessile, polypoid, grayish mass of rough surface located at the gastroesophageal junction. Histopathologic examination of polypoid mass samples revealed a vascular tumor covered with squamous epithelium. The hallmarks of the tumor were numerous branching, slit-like capillary channels with "staghorn" appearance, and large gaping sinusoidal spaces enclosed within nests of ovoid cells. Vascular invasion, mitotic activity, giant cells and necrosis were not found. Immunohistochemical analysis showed diffuse and strong reactivity for vimentin and focal, low or moderate reactivity for actin, S-100 and smooth muscle actin. Proliferation index measured by PCNA and Ki-67 ranged from 2% to 13% (median 11.2%) and from 5% to 18% (median 7.6%), respectively. A diagnosis of hemangiopericytoma with low malignant potential was made. Almost two years of the intervention, the patient is alive and free from any signs of tumor spread.
D. Balièeviæ
et al. Esophageal hemangiopericytoma or hemangiopericytoma-like tumor
Acta clin Croat, Vol. 43, No. 3, 2004 293
Acta Clin Croat 2002; 43: Case Report
ESOPHAGEAL HEMANGIOPERICYTOMA OR
HEMANGIOPERICYTOMA-LIKE TUMOR: A CASE REPORT
AND REVIEW OF THE LITERATURE
Drinko Balièeviæ
1
, Irena Novosel
2
, Davor Tomas
1
, Davor Hrabar
3
, Ahmed Pirkiæ
1
and Mladen Belicza
1
1
Ljudevit Jurak University Department of Pathology, Sestre milosrdnice University Hospital, Zagreb;
2
Department of Pathology,
Dr. Ivo Pediiæ County Hospital, Sisak;
3
Department of Gastroenterology, Sestre milosrdnice University Hospital, Zagreb, Croatia
SUMMARY  Hemangiopericytoma is a rare malignant vascular tumor deriving from pericytes. It usually
occurs in the extremities and retroperitoneum, and extremely rarely in the gastrointestinal tract. A case of
hemangiopericytoma in the gastroesophageal region is reported. A 56-year-old man was treated with H2-
receptor antagonist for dyspeptic symptoms that had occurred three months before. Therapy failed to
prove successful and gastroendoscopy was performed in March 2001 to reveal slightly reddish ventricular
mucosa with normal rugal folds and a sessile, polypoid, grayish mass of rough surface located at the gastroe-
sophageal junction. Histopathologic examination of polypoid mass samples revealed a vascular tumor cov-
ered with squamous epithelium. The hallmarks of the tumor were numerous branching, slit-like capillary
channels with staghorn appearance, and large gaping sinusoidal spaces enclosed within nests of ovoid cells.
Vascular invasion, mitotic activity, giant cells and necrosis were not found. Immunohistochemical analysis
showed diffuse and strong reactivity for vimentin and focal, low or moderate reactivity for actin, S-100 and
smooth muscle actin. Proliferation index measured by PCNA and Ki-67 ranged from 2% to 13% (median
11.2%) and from 5% to 18% (median 7.6%), respectively. A diagnosis of hemangiopericytoma with low
malignant potential was made. Almost two years of the intervention, the patient is alive and free from any
signs of tumor spread.
Key words: Esophageal neoplasms  pathology; Hemangiopericytoma  pathology; Hemangiopericytoma  immunochem-
istry
Correspondence to: Drinko Balièeviæ, M.D., Ph.D., Ljudevit Jurak Univer-
sity Department of Pathology, Sestre milosrdnice University Hospital,
Vinogradska c. 29, HR-10000 Zagreb, Croatia
Received March 5, 2003, accepted in revised form May 28, 2004
Introduction
Hemangiopericytoma (HP) is a rare malignant vas-
cular tumor deriving from Zimmermanns pericytes
1
. It
is almost always a solitary and solid tumor with smooth
surface, its color ranging from grayish-white to reddish-
brown. In three fourths of cases, the tumor is well cir-
cumscribed and encapsulated. In spite of that, surgical
excision may be difficult because of profuse bleeding,
and re-exploration is occasionally needed. HP metasta-
sizes to the lungs, bone and liver in approximately 50%
of patients. It is a tumor of adults, median age 45, and
rarely affects infants and children. The tumor shows an
equal sex involvement
1,2
.
HP may occur as a slowly growing, deep seated, pain-
less mass at any anatomic site, however, lower extrem-
ities, pelvic fossa, retroperitoneum, head and neck,
trunk and upper extremities are most commonly affect-
ed. It can also be found in subcutaneous tissue. Rare
cases of lipomatous HP, meningeal HP, HP of the nasal
passages and paranasal sinuses, orbital HP, and HP of
miscellaneous sites are on record
1,2
. HP is extremely rare
in the gastrointestinal tract, and only three cases of
esophageal HP have been reported in recent literature
3-5
.
The most common localization of HP in the gastrointes-
tinal tract was stomach, and only few isolated cases of
ileum, colon and rectum involvement have been de-
scribed
6-12
.
D. Balièeviæ
et al. Esophageal hemangiopericytoma or hemangiopericytoma-like tumor
294 Acta clin Croat, Vol. 43, No. 3, 2004
HP was first described and named by Stout and Mur-
ray, who also postulated its origin from pericytes
13
. Pericytes
were first identified by Rouget in 1873 and further defined
by Zimmermann in 1923. Pericytes are contractile, arboriz-
ing cells arranged ubiquitously along capillaries and
venules, and encircle the vasculature. Pericytes are thought
to represent modified smooth muscle cells or resting mes-
enchymal stem cells capable of undergoing myoid, fibro-
histiocytic and endothelial differentiation. These cells
have no readily identifiable light microscopic and immu-
nohistochemical features but are recognized ultrastructur-
ally by their topographic relation to small blood vessels and
close association with the endothelial basal lamina
1,2,13
.
Stout has reported on a series of 25 cases seven years
after the first description of HP
5
. In spite of the solidified
histopathology of the tumor, established in 1955, the con-
troversy about the true existence of HP still exists and it
seems that with the extensive use of immunohistochem-
istry it is becoming even more doubtful. The current ap-
proach applied by Stout and Enzinger is primarily to ex-
clude any other diagnosis before HP is confirmed
1,5
.
The diagnosis of HP is traditionally based on a micro-
scopic pattern characterized by the branching appearance
of small and large vessels. This vascular pattern can be seen
in a variety of other benign and malignant tumors, and may
lead directly to a suggestion that HP does not exist as an
entity
1,2,5
.
Grossly, HP is a solitary, well to fairly well circumscribed
mass covered by a thin vascular pseudocapsule measuring
4-8 cm in diameter. On cut section, the color ranges from
gray-white to red-brown, with a variable number of dilat-
ed vascular spaces. Hemorrhagic areas and cystic degen-
eration are frequently observed. Necrosis is common in
malignant forms of HP. The diagnosis of classic HP de-
pends on identification of the typical architectural pattern
in association with a population of relatively bland mesen-
chymal cells that display no discernible differentiation
under the light microscope. Characteristically, the tumor
consists of tightly packed round to fusiform cells with in-
distinct cytoplasmic borders that are arranged around the
elaborate vasculature. The ramifying vascular network
exhibits striking caliber variation
1,2,5,13
.
The diagnosis of malignant HP is sometimes problem-
atic. A combination of mitotic activity, cellularity, hemor-
rhage and necrosis is important to estimate malignancy
1,2,5
.
Recent studies recommend proliferation index as a stan-
dard procedure to demonstrate malignancy in HP
14,15
.
Patient and Methods
Patient
A 56-year-old man was treated with H2-receptor an-
tagonist for dyspeptic symptoms that had occurred three
months before. Therapy failed to prove successful, and
gastroendoscopy was performed in March 2001 to reveal
slightly reddish ventricular mucosa with normal rugal folds
and a sessile, polypoid, grayish mass of rough surface lo-
cated at the gastroesophageal junction. During the inter-
vention, biopsy of the gastric mucosa and polypectomy
were performed. Control gastroscopy showed no residual
pathologic changes.
Methods
Three gastric mucosa samples and five polypoid spec-
imens from the gastroesophageal region, measuring up to
3 mm were obtained for histopathologic analysis. The spec-
imens were fixed in 10% buffered formaldehyde, paraffin
embedded, cut at 5-mm thickness, and routinely stained
with hematoxylin and eosin. The method of Giemsa stain-
ing was used to determine colonization with Helicobacter
pylori. Gomori method and immunohistochemistry were
used to analyze specimens from the gastroesophageal re-
gion. Deparaffinization and immunohistochemical stain-
ing were performed following the Microwave Streptavidin
ImmunoPeroxidase (MSIP) protocol on a DAKO Tech-
Mate Horizon automated immunostainer. We used pri-
mary antibodies to epithelial membrane antigen (EMA),
S-100, smooth muscle actin (SMA), actin, CD34, pancy-
tokeratin, Factor VIII, vimentin, CD-117, proliferating cell
nuclear antigen (PCNA), and Ki-67 (Table 1). Appropri-
ate positive and negative controls were used throughout
Table 1. Antibody source and dilution
Antibody Clone Manufacturer Dilution
EMA E29 Dako 1:100
S-100 Z0311 Dako 1:200
Actin 1A4 Dako 1:50
(smooth muscle)
Actin HHF35 Dako 1:75
CD34 BI-3C5 Dako 1:50
Pancytokeratin AE1/AE3 Dako 1:100
Factor VIII F8/86 Dako 1:50
Vimentin Vim 3B4 Dako 1:50
CD-117 A4502 Dako 1:400
PCNA PC10 Dako 1:200
Ki-67 Ki-S5 Dako 1:50
D. Balièeviæ
et al. Esophageal hemangiopericytoma or hemangiopericytoma-like tumor
Acta clin Croat, Vol. 43, No. 3, 2004 295
the analysis. Mitotic activity and proliferation index were
counted under high power magnification (X400).
Results
Histopathology
Histopathologic examination of the gastric mucosa
confirmed chronic gastritis with moderate colonization
with Helicobacter pylori, whereas examination of polypoid
specimens from the gastroesophageal region revealed a
vascular tumor covered with squamous epithelium. The
hallmarks of the tumor were numerous branching, slit-like
capillary channels with a staghorn appearance, and large
gaping sinusoidal spaces enclosed within nests of ovoid
cells. These cells formed a distinctly tufted, woven pat-
tern (Fig. 1). Gomori method showed abundant reticulin
fibers outlining the vessels and forming an intricate mesh-
work around individual tumor cells (Fig. 2). The tumor
showed a predominantly solid pattern consisting of mod-
erately polymorphic cells without mitotic activity. Vascu-
lar invasion, giant cells and necrosis were not found.
Immunohistochemistry
Immunohistochemical analysis showed diffuse and
strong reactivity for vimentin (Fig. 3) and focal, low or
moderate reactivity for actin, S-100 and SMA. Immunore-
activity for pancytokeratin, EMA, CD-117 and Factor VIII
Table 2. Immunohistochemistry results
Antibody Vimentin EMA CK S-100 SMA Actin CD34 CD-117 Factor VIII
Reaction +++ + + ++
(-) negative reaction;
(+) mild and focal reaction;
(++) moderate reaction;
(+++) diffuse and strong reaction
Fig. 1. Hemangiopericytoma (H&E, X200). Numerous small and
large branching vessels surrounded by nests and cords of tumor cells.
Fig. 2. Hemangiopericytoma (Gomori, X200). Gomori method
showed abundant reticulin fibers outlining vessels and forming a
meshwork around individual tumor cells.
Fig. 3. Hemangiopericytoma (vimentin, X400). Tumor cells were
strongly positive for vimentin.
D. Balièeviæ
et al. Esophageal hemangiopericytoma or hemangiopericytoma-like tumor
296 Acta clin Croat, Vol. 43, No. 3, 2004
was negative. CD34 was only positive in endothelial cells
(Table 2, Fig. 4). Proliferation index was analyzed in ten
different fields under high magnification (X400). The
proliferation index measured by PCNA and Ki-67 ranged
from 2% to 13% (median 11.2%) and from 5% to 18%
(median 7.6%), respectively (Table 3, Fig. 5).
Discussion
Primary mesenchymal tumors of the esophagus, and
especially HP, are rare
1,2
. To our knowledge, only three
cases of primary esophageal HP have been reported in the
English-language medical literature
3-5
. Also, HP rarely af-
fects other parts of the gastrointestinal tract. In recent lit-
erature, 21 cases of HP in the stomach, four in the ileum
and jejunum, three in the colon and only one in the rec-
tum have been reported
6-12
. Most of these cases were ac-
companied by unexpected and unusual metabolic and even
obscure symptoms mimicking other clinical and histo-
pathologic entities
16,17
.
HP is an uncommon tumor with a histologic pattern
similar to many other tumors, and the diagnosis of HP is
usually based on exclusion
1,2,5
. The following morphologic
features should be evaluated for diagnostic purposes:
vasculature, histologic pattern (solid, myxoid, trabecular,
alveolar), and features that correlate with aggressive be-
havior such as cellular pleomorphism, necrosis, mitotic ac-
tivity, appearance of giant cells, and recently described pro-
liferation index
1,2,5-7,14,15
.
Necrosis is more frequent in recurring or metastasiz-
ing HP. Together with mitotic figures, necrosis is the most
reliable histologic criterion for predicting malignancy or
suggesting an uncertain biologic potential
1,2
.
The staghorn or outlet-like configuration of vessels is
the most prominent histologic finding. Dilated and branch-
ing vessels communicate with small or minute vessels that
may be partly compressed and obscured by the surround-
ing cellular proliferation. Broad zones of hyalinization are
much less common in HP than in solitary fibrous tumors
(SFT). Focal spindle cell areas may be present but are
never arranged in long bundles or fascicles as in SFT. Fo-
cal solid cellular area or focal palisading reminiscent of a
neural tumor may be present. Pericytes are considered to
be pluripotential cells. Abundant stromal collagen separat-
ing tumor cells could be the consequence of the process
of repair of necrotic regions found in the tumor, or could
be attributed to infarction, especially if the tumor is pe-
dunculated or intraluminal
7,18,19
.
Hypoglycemia and obscure clinical symptoms have
been noted in association with both HP and SFT, partic-
ularly if localized in the pelvis and retroperitoneum. These
tumors are often large and slow growing. The symptoms
may even require glucose infusion and abate with tumor
removal
16,17
. They are mediated through the production of
insulin-like growth factors (IGFs) by the tumor. Inter-
estingly, IGFs and IGF receptor (IGF-R) can be identified
Fig. 4. Hemangiopericytoma (CD34, X400).
Immunoreaction to CD34 was only observed in vessels.
Fig. 5. Hemangiopericytoma (PCNA, X400). Proliferation index
measured by PCNA ranged between 2% and 13%.
Table 3. Proliferation indices
PCNA Ki-67
5%-18% 2%-13%
(median 11.2%) (median 7.6%)
D. Balièeviæ
et al. Esophageal hemangiopericytoma or hemangiopericytoma-like tumor
Acta clin Croat, Vol. 43, No. 3, 2004 297
in tumor cells even in the absence of clinical hypoglyce-
mia. IGF-IR belongs to the family of transmembrane ty-
rosine kinase receptors. Its absolute requirement for the
establishment and maintenance of the transformed phe-
notype proves its central role in the mechanism of trans-
formation. In addition to these, IGFs stimulate tumor cell
proliferation through an autocrine loop that can be an-
nounced when the receptors are inactivated. Therefore,
IGF and especially IGF-IR could be responsible not only
for malignant transformation but also for hypoglycemia in
some patients
10-23
. Our patient was without symptoms as-
sociated with hypoglycemia, and glucose level in periph-
eral blood was normal.
The exact genetic background of malignant HP is still
unknown, although chromosome 12 has been most often
implicated
1,2
. Paveliæ et al. report on progressive accumu-
lation of multiple genetic lesions in the development of
HP, stressing the activation of myc, fos, jun, ras, IGFI, IG-
FII and IGF-IR genes, and simultaneous inactivation of the
p53 gene
20-22
.
The existence of hemangiopericytoma as a distinct
tumor entity remains a controversial concept despite all
advances in immunohistochemistry. Table 4 shows the
results of immunohistochemical staining of HP in several
recent studies
14,24-27
.
The immunohistochemical analysis recommended by
Enzinger and Stout includes vimentin, desmin, actin,
CD34, CD31 and Factor VIII
1,5,7
. Tumor cells are usually
immunoreactive for vimentin, whereas actin and desmin
are only focally present. CD34 is positive in approximate-
ly two thirds of HP cases, which is less than in SFT. Vas-
cular antigens are absent, except for endothelial cells lin-
ing the vascular spaces
1,5,7
. Enzingers panel of immuno-
histochemistry tests to confirm HP is a standard basis,
which several studies tried to improve with CD34 and
introduction of proliferative markers (PCNA, MIB, Ki-67)
in order to indicate the aggressiveness of HP. The signif-
icance of CD34 is still unclear
28,29
. Middleton et al. used
CD31, CD34, vimentin, actin, cytokeratin, S-100 and SMA
in a study including 17 cases of HP. The study showed
uniform reactivity for CD34 and vimentin, whereas CD31,
cytokeratin, CD31 and S-100 showed uniformly negative
results. Actin and SMA were focally positive in one case
each
14
. Saleh and Haapaniemi used vimentin, cytokeratin,
CD34, Factor VIII, S-100 and HHF35. Immunoreactivity
was recorded for vimentin, CD34 and HHF35, whereas
other markers showed no positive reaction
24
. Flores-Sta-
dler et al. additionally used desmin, alpha
1
-antitrypsin and
Factor XIIa, of which only desmin showed clearly negative
reaction
25
. In their study of four tumors, Folpe et al. record-
ed positive immunoreactivity for Factor XIIa and collagen
IV, whereas CD34 and SMA were positive in only two cas-
es, and the reaction for desmin, actin and S-100 was neg-
ative in all cases
26
. Of all authors found in the literature,
Guillou et al. used the most extensive immunohistochem-
istry panel. Their report of 13 cases shows interesting and
Table 4. Immunohistochemistry analysis of hemangiopericytoma reported in the literature
Author(s) Vimen- Cytoke- CD CD Factor Desmin Actin S-100 SMA EMA CD
tin ratin 31 34 VIII 117
Saleh et al.
+ +
(n=1)
Flores-Stadler
+ +
et al. (n=1)
Middleton + + + +
et al. (n=17) (17/17) (17/17) (17/17) (17/17) (17/17) (17/17)
Folpe et al. + +
(n=4) (2/4) (4/4) (4/4) (4/4) (2/4)
Gouillou et al. + + 
(n=13) (13/13) (13/13) (12/12) (10/13) (13/13) (11/13) (10/13) (7/13) (9/13) (12/13)
1ND 1ND 1ND 1ND 1ND
(+) positive reaction;
() negative reaction;
ND  not done
D. Balièeviæ
et al. Esophageal hemangiopericytoma or hemangiopericytoma-like tumor
298 Acta clin Croat, Vol. 43, No. 3, 2004
almost controversial results. All tumors showed immunore-
activity for vimentin, cytokeratin, CD31 and desmin.
CD99 was positive in 12, bcl-2 in 7, CD34 in 10, actin in
one, S-100 in 3, SMA in 5, and EMA in 4 cases. Inhibin
and CD-117 were negative in all cases
27
.
In our patient, strong reactivity was observed for vimen-
tin, moderate for actin, and focal for SMA and S-100. CD34
was only positive in endothelial cells, whereas other study
markers were negative, which is consistent with literature
data
1,14,24-27
(Table 2).
Differential diagnosis of HP is usually difficult because
many other tumors, especially fibrous histiocytoma, syn-
ovial sarcoma, mesenchymal chondrosarcoma, juxtaglom-
erular tumors and SFT may share similar histologic and
immunohistochemical patterns
1,2
. Differential diagnosis
between HP and SFT is especially difficult. In their study,
Guillou et al. pointed to a new problem in the differential
diagnosis between HP and SFT. After extensive investi-
gation of 13 cases, which included sex, age, site, size, orig-
inal pathologic diagnosis, treatment, surgical margins, fol-
low-up, light microscopy findings, immunohistochemistry
and electron microscopy findings, Gulliou et al. concluded
that lipomatous HP (L-HP) and SFT were the same eti-
ologic but not the same pathologic entity, and considered
L-HP as a variant of SFT with lipocytes
27
.
Clinical behavior of HP is unpredictable, and the met-
astatic rate varies from 10% to 60% in different studies
1,2
.
Few authors have reported different criteria for assessment
of HP malignancy. Enzinger and Smith claim large size (>5
cm in diameter), increased mitotic rate (4 mitoses per 10
HPF  high malignant potential; 1-3 mitoses per 10 HPF
 low malignant potential), increased cellularity, presence
of immature and pleomorphic tumor cells, foci of hemor-
rhage and necrosis as ominous signs
7
. A study conducted
at Mayo Clinic connected malignant behavior with a low
degree of anaplasia and 1 mitosis per 10 HPF or a moder-
ate degree of cellular anaplasia and 2 mitoses per 20 HPF
2
.
Middleton et al. demonstrated that tumors with a trabec-
ular pattern, necrosis, mitoses, vascular invasion and cel-
lular atypia more frequently had recurrences and metastas-
es. In the same study, proliferation index measured with
MIB1 (Ki-67) was between 2% and 40%. Tumors with a
more aggressive course showed higher proliferating rate
(>15%) as compared with tumors without recurrence and
metastases
14
. Similar studies have been reported by Yu et
al.
30
and Kowalski et al.
15
. It seems that proliferation index
correlates better with tumor behavior than mitotic index;
proliferation index over 10% usually implies higher malig-
nant potential
14,15
. Controversially, Goldman et al. in their
study of 17 cases concluded that there was no appropriate
method to determine malignant potential of any particu-
lar HP
31
.
Surgical excision is the treatment of choice for any
tumor site. The malignant HP response to chemotherapy
and radiotherapy is variable and unpredictable. The 5-year
disease-free survival is about 50%. Longterm follow-up is
needed because 10% of tumors recur after 5 years
19
.
In our patient, the median proliferation index was up
to 10% measured by Ki-67, however, mitotic activity, tra-
becular pattern, necrosis and giant cells were not observed.
Therefore, we considered this tumor as a HP with low
malignant potential. Almost two years after the interven-
tion, the patient is alive and free from any signs of tumor
spread.
In conclusion, HP is not a clearly delineated entity with
characteristic histologic and immunohistochemical pat-
tern. Its diagnosis is mainly based on exclusion, and its clin-
ical behavior is unpredictable.
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Saetak
HEMANGIOPERICITOM ILI HEMANGIOPERICITOMU NALIK TUMOR JEDNJAKA: PRIKAZ SLUÈAJA I
PREGLED LITERATURE
D. Balièeviæ, I. Novosel, D. Tomas, D. Hrabar, A. Pirkiæ i M. Belicza
Hemangiopericitom je rijedak maligni vaskularni tumor koji potjeèe od pericita. Najèeæe je javlja na ekstremiteti-
ma, te u podruèju peritoneuma, a iznimno rijetko ga se nalazi u probavnom sustavu. Opisuje se sluèaj hemangioperic-
itoma naðenog u gastroezofagusnom podruèju. Bolesnik star 56 godina lijeèen je tri mjeseca antagonistima H-2 recep-
tora zbog dispeptiènih problema. Preporuèena terapija nije pokazala oèekivani rezultat, pa je uèinjena gastroskopija.
Naðena je sesilna polipozna tvorba velièine novèiæa, sivkaste boje i neravne povrine u gastroezofagusnom podruèju.
Histopatoloki je odstranjena tvorba bila graðena od razgranatih kapilarnih prostora tipa staghorn, okruenih gnijezdi-
ma ovalnih stanica koje su tvorile solidan uzorak. Nekroze, vaskularna invazija, velike stanice i mitoze nisu naðene.
Tumorske stanice su pokazivale difuznu, izrazito pozitivnu imunoreakciju na vimentin, dok je reakcija na aktin, S-100
i glatkomiiæni aktin bila arina i slabog intenziteta. Proliferacijska aktivnost tumora iznosila je izmeðu 2% i 13% (medijan
11,2%) mjerena pomoæu PCNA, te izmeðu 5% i 18% (medijan 7,6%) mjerena pomoæu Ki-67. Postavljena je dijagnoza
hemangiopericitoma niskog malignog potencijala. Skoro dvije godine nakon postavljene dijagnoze bolesnik je bez zna-
kova bolesti.
Kljuène rijeèi: Neoplazme jednjaka  patologija; Hemangiopericitom  patologija; Hemangiopericitom  imunokemija
  • [Show abstract] [Hide abstract] ABSTRACT: Hemangiopericytoma (HPC) of the head and neck is a rare neoplasm whose biologic behavior is difficult to predict by means of conventional histologic parameters. H & E-stained sections from 12 cases of HPC were reviewed. Proliferation index was assessed using an immunoperoxidase stain for MIB-1 (Ki-67). The study group consisted of 4 adult men, 5 adult women, and 1 infant male. Necrosis, hypercellularity, and pleomorphism were found in 1, 5, and 6 case(s), respectively. The mitotic index per 10 high power fields varied from 0-1 to 15. Proliferation indices using MIB-1 ranged from 2.6% to 52.5%. Clinical follow-up revealed 3 cases with recurrence all possessing proliferation indices of approximately 10%. Standard histomorphologic features may be inadequate predictors of clinical outcome. A proliferation index of 10% or greater may indicate a more aggressive subset of HPC of the head and neck.
    Article · Jun 2001
  • [Show abstract] [Hide abstract] ABSTRACT: Hemangiopericytoma (HPC) is an uncommon vascular neoplasm thought to be derived from pericytes. Prediction of patient outcome is difficult based what is currently known about these tumors and histological parameters alone. We compiled 27 cases of HPC and evaluated the spectrum of histological features to investigate whether there was any correlation between histology, immunostaining, prognostic markers, and patient outcome. The following parameters were evaluated: vasculature, histological pattern (solid, myxoid, trabecular, alveolar), degree of cellular pleomorphism, necrosis, mitoses, and giant cell content. Immunohistochemistry was performed to determine the reactivity for CD 31, CD34, vimentin, actin, cytokeratin, S100, actin, and SMA. Proliferative rate was analyzed using antibodies to PCNA and MIB1. Patient's age ranged from 8 months to 75 years (mean, 35; median, 31). Twenty of 27 cases were located in the extremities. The tumors were grossly described as lobulated and well circumscribed (n=12) and nonencapsulated (n=15). By histology, the characteristic ramifying or staghorn vasculature pattern was seen in all cases. A solid histological pattern was mixed with an alveolar pattern in three cases, trabecular pattern in six cases, and myxoid pattern in two cases. Tumor cells were uniform, polygonal to spindle-shaped, often with vesicular nuclei. Tumor giant cells were present in 9 of 27 cases; necrosis, in 11 of 27. Mitoses ranged from 0 to 14 per 10 high-power fields (HPF). Cellular pleomorphism was 1+ in nine cases, 2+ in 12 cases, and 3+ in six cases. Immunohistochemistry showed reactivity for CD34 and vimentin in all cases. Actin was focally positive in one case, and SMA was focally positive in another. CD 31, cytokeratin, and S100 were uniformly nonreactive. Proliferative index measured by PCNA and MIBI ranged between less than 1% and 40% of tumor cells. Follow-up was available in 22 cases and ranged from 1 year to 15 years. Seven patients had metastases, and two recurred locally. Thirteen patients had no evidence of disease at last checkup. Parameters associated with recurrences or metastases include a trabecular pattern, the presence of necrosis, mitoses, vascular invasion, and cellular pleomorphism. Features associated with an aggressive biological behavior can be identified histologically. There was some, but not total, correlation between proliferative markers and tumor aggressiveness.
    Article · Jul 1998
  • [Show abstract] [Hide abstract] ABSTRACT: Hemangiopericytoma (HP) is an uncommon, slowly growing tumor that originates in "pericyte" cells. Biologically it can be benign or malignant; however, it is difficult to predict tumor behavior based on histomorphology alone. The cytomorphology of this tumor has been described only rarely. Other spindle cell mesenchymal tumors can mimic HP on fine needle aspiration biopsy (FNAB). A 60-year-old man presented with cough and a left lung mass on chest roentgenography and multiple smaller bilateral lung nodules on computed tomographic scan. FNAB of the dominant left lung mass showed a moderately cellular aspirate with clusters and single spindle-shaped cells morphologically similar to those of hemangiopericytoma excised from the posterior part of the neck 11 years previously. With immunocytochemical studies, the tumor cells were negative for cytokeratin, factor VIII-related antigen, S-100 protein and HHF35 but positive for vimentin and CD34. FNAB is a valuable tool in evaluating nonepithelial metastatic lung tumors. In the appropriate clinical setting, it is possible to render a diagnosis of HP on FNAB in patients with previously documented HP based on morphologic comparison and ancillary studies, especially immunocytochemical stains.
    Article · Jul 1997
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