| Huge primitive neuroectodermal tumor of the pancreas:
report of a case and review of the literature. World J Gastroenterol. 2006;12(37):6070-3.
Primitive neuroectodermal tumor (PNET) of the
pancreas is an extremely rare tumor that usually occurs in children or
young adults. We report a case of a 33-year-old male patient with an
18 cm multiply 18 cm multiply 16 cm mass arising from the pancreatic
body and tail with a one-day history of abdominal pain. Initial CT
scan showed no signs of metastatic tumor spread. The tumor caused
intrabdominal bleeding and the patient underwent primary tumor
resection including partial gastrectomy, left pancreatic resection and
splenectomy. Diagnosis of PNET was confirmed by histology,
immunohistochemistry and FISH analysis. All neoplastic cells were
stained positive for MIC2-protein (CD99). Approximately one month
after surgery, several liver metastases were observed and the patient
underwent chemotherapy according to the Euro-Ewing protocol.
Subsequent relaparotomy excluded any residual hepatic or extrahepatic
abdominal metastases. Although PNET in the pancreas is an extremely
rare entity, it should be considered in the differential diagnosis of
pancreatic masses, especially in young patients. This alarming case
particularly illustrates that PNET in the pancreas although in an
advanced stage can present with only a short history of mild symptoms.
Diagnosis and treatment of primitive
neuroectodermal tumors of pancreas.Zhongguo
Yi Xue Ke Xue Yuan Xue Bao. 2006 Apr;28(2):191-5.
OBJECTIVE: To improve the diagnosis and treatment
of primitive neuroectodermal tumors (PNET) of the pancreas. METHODS:
One patient with PNET of the pancreas was reported in this article.
The corresponding literatures on the diagnosis and treatment was
reviewed. RESULTS: The patient was diagnosed as pancreatic PNET by her
clinical, microscopic, and immunohistochemical features as well as
cytogenetic analysis after the resection of the tumor located in the
uncinate process in PUMC Hospital. Radiochemotherapy was given after
the operation for 8 months and no recurrence was observed. Since PNET
of pancreas have no specific clinical symptoms and most patients have
jaundice and/or abdominal pain, the diagnosis depended on the
immunohistochemical features of positive P30/32(MIC2) and at least two
of the neural markers. The cytogenetic analysis showed translocation
mainly harbored the characteristic t (11; 22) (q24; q12). Since
pancreatic PNET were highly aggressive, early chemotherapy, close
follow-up, and immediate surgical interventions were required as early
as possible. CONCLUSION: PNET can occur in pancreas, and diagnosis and
treatment should be made as early as possible to improve the outcome.
Primitive neuroectodermal tumor of the pancreas. A case report of
an extremely rare tumor.Pancreatology.
2003;3(4):352-6.
Primitive neuroectodermal tumor (PNET) of the pancreas is extremely
rare. Although the diagnosis of PNET is suggested by the light
microscopic appearance of the tumor, it should be confirmed by the
immunohistochemical evaluation of the c-myc expression and if
possible, further determination of the particular chromosome
translocation, t(11;22)(q24,q12). In this report, we present a male
patient with pancreatic PPNET who had been followed up for 50 months.
The related literature is also reviewed. In our case, the pathologic
diagnosis was based on the positive immunoreactivity for CD99 in many
of the tumor cells. The complementary cytogenetic studies were not
possible in the private setting of the patient's treatment. The
patient was 31 years old when first operated. Within 4 months of the
first operation he had local recurrence. In the third year of his
follow-up he had been discovered to have pulmonary metastases and
another metastatic tumor in his lung was diagnosed the year after. The
metastatic foci were primarily treated by surgical resections. He had
chemotherapy after each resection of pulmonary metastatic foci. After
50 months of the initial surgical intervention, he succumbed to
widespread thoracic and bone metastases. Because of the extreme rarity
of PPNET in the pancreas, and its rather protracted course, we think
our case may further contribute to the ever expanding database for
this particular entity.
Primitive neuroectodermal tumors of the pancreas: a report of seven
cases of a rare neoplasm.Am
J Surg Pathol. 2002 Aug;26(8):1040-7.
Primitive neuroectodermal tumors (PNETs) have
rarely been described in solid organs. We report a series of seven
PNETs of the pancreas. The clinical, gross, microscopic, and
immunohistochemical features of these seven PNETs of the pancreas are
described, as are the genetic analyses in five cases. The patients
ranged in age from 6 to 25 years (mean 18 years). Four of the patients
were male. All of the patients presented with jaundice and/or
abdominal pain. All of the tumors were located in the head of the
pancreas, and they ranged in size from 3.5 to 9.0 cm. Light microscopy
revealed the typical morphologic features of PNETs. By
immunohistochemistry the neoplastic cells in all seven cases expressed
O13 (CD99, p30/32MIC2). In five of six tested cases, the neoplastic
cells also expressed cytokeratin. All of the tumors expressed neural-neuroendocrine
markers. Two of the three cases examined ultrastructurally showed
prominent epithelial features. There was cytogenetic or molecular
genetic evidence of the t(11;22)(q24;q12) in four of five cases
examined. Clinical follow-up was available in five cases. Two of the
patients were alive with no evidence of disease at 33 and 43 months.
One patient was alive with disease at 27 months. One patient died of
postoperative complications. Another patient died of disease 4 years
after diagnosis. PNET can sometimes arise as a primary neoplasm of the
pancreas. Like PNETs arising in more conventional sites, pancreatic
PNETs occur in the pediatric and adolescent population, show the
characteristic staining with O13, and typically harbor the
t(11;22)(q24;q12) chromosomal translocation. PNETs should be included
in the differential diagnosis of poorly differentiated small round
cell tumors of the pancreas. Moreover, they should not be confused
with pancreatic endocrine tumors, which also demonstrate dual
epithelial and neuroendocrine differentiation by immunohistochemistry
and express O13 in 30% of cases.
Primitive neuroectodermal tumor of the pancreas. An extremely rare
tumor. Case report and review of the literature.Klin
Padiatr. 2000 Jul-Aug;212(4):185-8.
Peripheral primitive neuroectodermal tumor (PPNET)
is a malignant neoplasm of the peripheral nervous system and soft
tissues. Representing the fourth case published we herein report a
PPNET arising in the pancreas of a six year old girl. She presented
with severe anemia due to ulcerative tumor growth and hemorrhage into
the duodenum. From the first biopsy pancreatoblastoma was considered
as histological diagnosis. Therefore pancreato-duodenectomy was
successfully performed. Immunohistochemically, the tumor cells were
positive for cytokeratines and several neuronal markers. Due to focal
membranous staining for MIC-2 gene product and rosettes in one lymph
node metastasis the diagnosis had to be altered into PPNET. This was
confirmed by cytogenetic analysis. We conclude that the interpretation
of histologic sample excisions from pediatric pancreatic neoplasms may
be difficult and that PPNET should be included in the differential
diagnosis.
Primitive neuroectodermal tumor arising in the pancreas. Mod
Pathol. 1994 Feb;7(2):200-4.
Peripheral primitive neuroectodermal tumors (PNETs)
are extra cranial primitive small round blue cell tumors showing
histologic, immunohistochemical or electron microscopic evidence of
neuroectodermal differentiation. They are most commonly encountered in
the soft tissue or bone in children and young adults. We report an
unusual case of a PNET arising in the pancreas. A 17-yr-old male
presented with a pancreatic mass and underwent a pancreatoduodenectomy.
Histologically, the neoplasm was composed of sheets of small round
cells that stained for cytokeratin, neuron specific enolase, and 12E7
but not muscle specific action, desmin, common leukocyte antigen,
chromogranin, synaptophysin, or other islet cell markers. The
diagnosis of PNET in this unusual location was confirmed by
cytogenetic analysis which showed the chromosomal translocation
characteristics of PNETs, t(11;12)(q24;q12). This case highlights the
difficulty in the diagnosis of PNET when it is present in visceral
organs where other neuroendocrine neoplasms and adenocarcinomas are
more common.
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