| Pancreatoblastoma. JOP.
2007 Jan 9;8(1):55-63.
Pancreatoblastoma
(PB), or infantile pancreatic carcinoma, is an extremely rare
pancreatic tumor in childhood, comprising 0.5% of pancreatic
non-endocrine tumors. Although PB mainly presents during childhood but
can also occur in adults. PB tend to be less aggressive in infants and
children compared to adults. Children with PB usually present late
with upper abdominal pain and many have a palpable mass in the
epigastrium. Mechanical obstruction of the upper duodenum and gastric
outlet by tumor in the head of the pancreas may be associated with
vomiting, jaundice and gastrointestinal bleeding. Histologically, PB
is characterized with distinct acinar and squamoid cell
differentiation. PB has been associated with alterations in the Wnt
signaling pathway and chromosome 11p loss of heterozygosity (LOH),
Beckwith-Wiedemann syndrome and familial adenomatous polyposis. The
majority of these tumors arise in the head of the pancreas.
Alpha-fetoprotein may be elevated in up to 68% of patients with PB.
Ultrasound and CT scan may be useful but preoperative diagnosis is
often quite difficult. The treatment of choice is complete resection,
that may often be curative. The role of adjuvant chemotherapy or
radiotherapy is still under discussion due to small number of patients
treated as yet. Chemotherapy regimens consisting of cyclophosphamide,
etoposide, doxorubicin, and cisplatin have been used in neoadjuvant
setting with anecdotal benefit. Prognosis of this rare tumor is good,
when resected completely. Prognosis is poorer, when there is
metastasis or when it is inoperable. On the whole, PB is regarded to
be a curable tumor; hence the clinical diagnosis should be made early.
Awareness of this rare tumor of pancreas is essential for early
detection and proper management. The author review the clinical
presentation, etiology, diagnosis, treatment and prognosis of PB in
this presentation.
Pancreatoblastoma. A case report with special emphasis on squamoid
corpuscles with optically clear nuclei rich in biotin.Acta
Cytol. 2003 Jul-Aug;47(4):679-84.
BACKGROUND:
Pancreatoblastoma (PBL) is a rare neoplasm that generally occurs in
the pediatric age group and shows unique histopathology, including
squamoid corpuscles that may contain tumor cells with optically clear
nuclei (OCN) rich in biotin. In the English-language literature there
have been two reports on the cytology of PBL, but neither of them
refers to the cytologic features of squamoid corpuscles. CASE: A
3-year-old boy with nausea and general fatigue was referred to our
center. Imaging studies showed an approximately 7.5-cm, left-sided
abdominal mass and multiple metastases in the lung. The abdominal mass
was biopsied, and its histology showed solid cellular nests with
occasional acinar differentiation and squamoid corpuscles. Imprint
cytology of the biopsied sample displayed cellular epithelial nests
with focal acinar structures and foci composed of larger cells with a
low nuclear/cytoplasmic ratio. These foci contained a few tumor cells
with biotin-rich OCN and were determined to be squamoid corpuscles.
CONCLUSION: Detection of occasional squamoid corpuscles with
biotin-rich OCN can be useful in making a diagnosis of PBL on
cytologic samples.
Significance of aberrant (cytoplasmic/nuclear) expression of beta-catenin
in pancreatoblastoma.
J Pathol. 2003 Feb;199(2):185-90.
This study
concerns the significance of aberrant (nuclear/cytoplasmic) expression
of beta-catenin in pancreatoblastoma (PBL). On immunohistochemistry,
all seven PBLs examined showed nuclear/cytoplasmic expression of beta-catenin,
predominantly in the squamoid corpuscles (SCs). In areas with acinar/ductular
differentiation, few tumour cells displayed nuclear/cytoplasmic
expression of beta-catenin and more than half of the tumour cells
showed membranous expression. Two out of five (40%) tumours examined
showed missense mutations in codons 33 and 37 of exon 3 of the beta-catenin
gene. No mutation of the adenomatous polyposis coli (APC) gene was
detected in two of the remaining three tumours. Amplifiable DNA for
APC analysis was not obtained from the one other tumour.
Immunoreactivity for cyclin D1, one of the nuclear targets of beta-catenin,
was found predominantly in the SCs of the seven tumours. In contrast,
the Ki-67 labelling index was 2-4% (median 3%) in the SCs and 8-18%
(median 12%) in the other areas, indicating a negative correlation
with nuclear cyclin D1 reactivity. These results imply that in PBLs,
nuclear/cytoplasmic accumulation of beta-catenin and overexpression of
its target gene cyclin D1 are not associated with the induction of
tumour cell proliferation. Nuclear/cytoplasmic accumulation of beta-catenin
may be related to the morphogenesis of the SCs that are considered
most characteristic for PBL.
Prolonged survival after resection of pancreatoblastoma and
synchronous liver metastases in an adult.
Hepatogastroenterology.
2001 Sep-Oct;48(41):1340-2.
Pancreatoblastoma
is an uncommon pediatric neoplasm with distinct acinar and squamoid
cell differentiation. Pancreatoblastoma is exceedingly rare in adults
with only ten reported cases. Pancreatoblastoma in adults has a poor
prognosis and no survival without recurrence exceeding 30 months has
been reported. We report the first adult case of pancreatoblastoma
revealed by gastric bleeding due to segmental hypertension. On
computed tomography scan, the tumor appeared lobulated and extended
from the splenic hilum to the portal vein. Two hypervascular
centimetric hepatic metastases were observed in segments III and VII.
The patient was operated and a distal pancreatectomy with splenectomy
associated with two hepatic wedge resections was performed. The
diagnosis of pancreatoblastoma was made on immunohistochemical
examination. The patient received 6 cycles of adjuvant therapy. After
three years of follow-up, the patient was well with no sign of
recurrence on computed tomography scan. This case suggests that in the
presence of pancreatic tumor of unknown origin, aggressive management
including complete surgical resection and adjuvant chemotherapy should
be attempted even in the presence of synchronous liver metastases.
Alpha-fetoprotein variants in a case of pancreatoblastoma.
Ann Clin Biochem. 2000 Sep;37 ( Pt
5):681-5.
We describe a
6-year-old boy with histologically confirmed pancreatoblastoma and a
markedly elevated serum alpha-fetoprotein (AFP) concentration. Due to
local tumour invasion, cytotoxic chemotherapy was given to debulk the
tumour before attempting surgical resection. Serial serum AFP
concentrations were measured on this patient. During chemotherapy
there was a > 95% fall in total AFP. Tumour-specific variants of AFP,
detected by isoelectric focusing, also disappeared during chemotherapy
but recurred when chemotherapy was withdrawn. It is suggested that
although there was no change in overall size of the tumour, as
assessed by various imaging techniques, the changes in serological
markers may indicate that the treatment did in fact cause considerable
tumour necrosis, AFP and its variants may be useful markers of tumour
response in patients with pancreatoblastoma. The expression of AFP and
its variants in pancreatoblastoma may be related to the embryonic
origin of the pancreas.
Pancreatoblastoma. Three original cases and review of the
literature.Acta
Radiol. 2000 Jul;41(4):334-7.
PURPOSE: To
describe the characteristics of pancreatoblastoma. MATERIAL AND
METHODS: We studied 3 cases of pancretoblastoma and reviewed another
59 cases. Parameters analyzed were tumor site, hemorrhage, capsule
formation, necrosis, vascularity, production of alpha-fetoprotein (AFP),
cystic changes and calcification. RESULTS: The diagnostic findings
were as follows: pancreatic head origin (24/54, 44%), pancreatic
body and tail origin (30/54, 56%), hemorrhage (16/17, 94%), capsule
formation (24/26, 92%), necrosis (28/31, 90%), hypervascularity
(10/14, 71%), production of AFP (19/28, 68%), cystic changes (11/16,
69%), and calcification (10/21, 48%). All neonatal cases
demonstrated cystic changes. Three of them were patients with
Beckwith-Wiedmann syndrome. The incidence of capsule formation and
calcification was not related to the origin of the tumor.
CONCLUSION: The most common features of pancreatoblastoma are
hemorrhage, capsule formation and necrosis.
Pancreatoblastoma:
imaging findings in 10 patients and review of the literature.Radiology.
2000 Feb;214(2):476-82.
PURPOSE: To
describe the features of pancreatoblastoma at magnetic resonance (MR)
imaging, computed tomography (CT), and ultrasonography (US). MATERIALS
AND METHODS: Imaging and surgical findings in 10 patients (age range,
2-20 years; mean age, 6.8 years) with pathologically proved
pancreatoblastoma were reviewed for tumor size, organ of origin,
definition and quality of tumor margins, tumor heterogeneity,
calcification, enhancement, ascites, biliary and/or pancreatic ductal
dilatation, local invasion, adenopathy, vascular invasion, vascular
encasement, metastases, and signal intensity on MR images. Results
from 10 CT, seven US, and three MR imaging examinations were reviewed.
RESULTS: Five of the 10 tumors were pancreatic; four others appeared
to be pancreatic or hepatic. Most had well-defined margins (nine of
10), were heterogeneous (nine of 10), and enhanced (10 of 10). Other
findings included calcification (two of 10), biliary and pancreatic
ductal dilatation (one of 10), and ascites (three of 10). Hepatic (two
patients) and pelvic (two patients) metastases were present.
Adenopathy (two patients) and vascular invasion (one patient) were not
identified radiologically. Tumors had low to intermediate signal
intensity on T1-weighted images and high signal intensity on
T2-weighted images. CONCLUSION: Pancreatoblastoma is typically a
heterogeneous tumor with well-defined margins that may appear to arise
from the pancreas or liver. It may behave aggressively, with localized
vascular or bowel invasion or with widespread metastatic disease.
Although it is rare, it should be considered in the differential
diagnosis of an upper abdominal mass in a child.
Pancreatoblastoma: optically clear nuclei in squamoid corpuscles
are rich in biotin.Mod
Pathol. 1998 Oct;11(10):945-9.
Pancreatoblastoma
is a rare pancreatic neoplasm that generally occurs in children and
demonstrates unique histopathologic features, including squamoid
corpuscles. We encountered five cases of pancreatoblastoma and
unexpectedly found the presence of optically clear nuclei (OCN) only
in tumor cells of squamoid corpuscles. Recent studies showed that OCN
seen in some types of neoplastic and non-neoplastic tissues were rich
in biotin and revealed false-positive immunostaining by the avidin-biotin
peroxidase complex method. We conducted an investigation to see if the
same were true for pancreatoblastoma. As a result, the OCN were
positively stained when the tissue section was incubated with
peroxidase-labeled avidin or streptavidin and developed in a chromogen
solution. The reaction was completely blocked by pretreatment with
free avidin. An identical nuclear staining pattern was observed by the
indirect immunoperoxidase method with the antibiotin antibody.
Ultrastructurally, the OCN were occupied by filamentous substructures
of chromatin measuring approximately 10 to 12 nm in diameter. These
results suggested to us that the OCN in pancreatoblastomas were part
of the unique nuclear change in which abundant biotin accumulated in
association with the characteristically arranged chromatin
substructure. When immunostaining with biotin as the linkage agent is
performed in pancreatoblastomas, the false positivity in the OCN
should be kept in mind to avoid any misinterpretation of the results.
Pancreatoblastoma.
J Pediatr Surg. 1997 Nov;32(11):1612-5.
BACKGROUND:
Pancreatoblastoma is a rare pancreatic tumor with distinct acinar and
squamoid cell differentiation that generally affects infants and young
children. Just over 50 cases have been reported in the literature.
METHODS: Five cases of pathologically proven pancreatoblastoma treated
at Seoul National University Hospital from 1984 to 1994 were reviewed.
There were three girls and two boys who were 2 years to 5 years of
age. All cases came to medical attention because of an abdominal mass.
RESULTS: Abdominal pain was observed in one case and anorexia,
vomiting, and weight loss in one case. There was marked elevation of
serum alpha-fetoprotein (27,000 ng/mL) in one case of liver
metastases. Complete excision was performed in two cases in which the
tumors were located in the tail of the pancreas. Partial excision was
performed in two patients who had unresectable tumors of the head of
the pancreas. One patient had an unresectable tumor at diagnosis and
needle aspiration biopsy was carried out under ultrasound guidance.
Electron microscopy was performed on pathological specimens of three
cases and showed zymogen granules but not neuroendocrine granules.
Immunocytochemical studies for alpha-fetoprotein, insulin, glucagon
and somatostatin were performed in one patient, and results were all
negative. Of two patients who underwent complete excision, one patient
presented with liver metastases 4 months after operation and received
chemotherapy, but died of tumor 6 months after operation. The other
patient had local recurrence 1 year after operation. Reoperation and
chemotherapy were performed, and the child is now alive without
evidence of disease for 32 months. All three patients who had
unresectable tumor died of tumor despite adjuvant radiotherapy and
chemotherapy. CONCLUSIONS: The authors emphasize that the diagnosis of
pancreatoblastoma in childhood should be suspected with palpation of
an abdominal mass, and the chance for cure may be determined by
complete excision of the tumor.
Pancreatoblastoma in children. Case report and review of the
literature.
Eur J Pediatr Surg. 1996 Dec;6(6):369-72.
Pancreatoblastomas are rare embryonal malignancies in childhood. We
report a 3-year-old girl with a tumor of the head of pancreas. Staging
by bone scintigraphy and CT scans of abdomen and chest did not show
evidence of metastatic disease. Tumor markers showed elevated levels
of alpha-1 fetoprotein (64 ng/ml; normal 0-10 ng/ml) and lactate
dehydrogenase (423 U/l; normal range below 300 U/l). The tumor was
macroscopically completely removed by local resection. Postoperative
tumor grading was pT1, NO, MO. The child recovered very soon after
surgery without severe complications. Tumor markers dropped to normal
values, indicating complete remission (follow-up time 12 months).
According to the biological growth characteristics of
pancreatoblastomas and to the literature, localized and non-metastatic
tumors should be completely resected without radical
pancreatoduodenectomy and without adjuvant chemotherapy. This is the
most conservative therapy with a good prognosis. However, metastatic
disease, primarily inoperable conditions or local relapses are
indications for chemotherapy combined with radiotherapy and followed
by resection of the tumor. At present, the prognosis of such cases is
rather poor.
Pancreatoblastoma--a rare pancreatic malignancy in childhood.
Langenbecks Arch Chir Suppl Kongressbd.
1996;113:1040-2.
Between 1885 and
1991 only 71 cases of malignant pancreatic tumours in childhood and
adolescence have been reported in literature; the majority of these
were pancreatoblastomas. The symptoms, pathology and therapy of this
rare tumour are demonstrated in the case of a 17-year-old girl. The
tumour is believed to develop at an early stage of pancreatic cell
differentiation. Usually it is composed of both exocrine and endocrine
cell types. The treatment of choice is radical resection. In contrast
to pancreatic neoplasms in adult patients the pancreatoblastoma has a
favourable prognosis. The role of adjuvant chemotherapy or
radiotherapy is still under discussion due to the small number of
patients treated as yet.
Pancreatoblastoma: a histochemical and immunohistochemical
analysis.J
Clin Pathol. 1996 Nov;49(11):952-4.
A case of
pancreatoblastoma arising in a five year old girl was analysed using
histochemical and immunohistochemical methods. The tumour was composed
of tubular gland-like structures, squamoid components and some small
round cells surrounding tubular structures. The cytoplasm of the small
round cells and a few of the squamoid cells was positive on staining
with Grimelius argyrophil stain. Immunohistochemically, tumour tissue
was positive for neurone specific enolase. The cytoplasm of some of
the small round cells was positive for insulin, glucagon, somatostatin,
pancreatin polypeptide, thyroid stimulating hormone, follicle
stimulating hormone, and neurotensin. These results suggest that this
tumour arose from primitive multipotential stem cells, showing
exocrine and neuroendocrine differentiation.
Mixed-type pancreatoblastoma. Report of a clinical case.
Minerva Chir. 1996;51(9):729-35.
Pancreatoblastoma
is a very rare tumor of the pancreas, affecting children in the first
decade of life. Ultrasound and CT scan may be useful but pre-operative
diagnosis is often quite difficult. High level of seric
alpha-fetoprotein could be suggestive of pancreatoblastoma. The
definitive diagnosis is however made by histologists when an organoid
structure, well encapsulated, consisting of acinar cells with zymogen-like
granules and squamoid corpuscle is demonstrated, sometimes associated
with mesenchymal tissue (mixed-type pancreatoblastoma).
Immunohistochemical studies may help in differentiating
pancreatoblastoma from papillary cystic tumors, acinar cell carcinomas
and endocrine pancreatic tumors of the pancreas. We report a case of
mixed-type pancreatoblastoma in a 12-year-old female patient, located
in the head of the pancreas. The treatment has been surgical,
consisting of a duodeno-cephalo-pancreasectomy. Neither radiotherapy
nor chemotherapy has been performed. The patient is alive, without
recurrence, four months after the operation. Diagnostic, therapeutic
and prognostic aspects are discussed.
Pancreatoblastoma. A clinicopathologic study and review of the
literature.Am
J Surg Pathol. 1995 Dec;19(12):1371-89.
Pancreatoblastoma
is a rare pancreatic tumor with a distinctive histologic appearance
that generally affects infants and young children. We have studied 14
cases of pancreatoblastoma and reviewed 41 previously reported
examples. Nine of our cases occurred in children (from newborn to 4
years old; mean, 2.4), and five affected adults (from 19 to 56 years
old; mean, 40). There were 8 male cases and 6 female cases. Most
patients presented with incidental abdominal masses, although pain,
weight loss, and obstructive jaundice were present, but rarely. The
tumors were very cellular microscopically, with cytologically uniform
epithelial cells arranged in sheets and nests. Well-formed acinar
structures were a consistent feature, and several cases contained
ectatic ductular formations, rarely exhibiting intracellular mucin.
Consistently present were squamoid corpuscles: circumscribed, whorled
nests of plump spindle cells with a squamous appearance and occasional
keratinization. The stroma was moderate to abundant and frequently
quite cellular (especially in the pediatric cases). By
immunohistochemistry, the tumors exhibited acinar, endocrine, and
ductal differentiation, with positivity for pancreatic enzymes (100%),
endocrine markers (82%), and carcinoembryonic antigen (85%).
Ultrastructural examination most commonly revealed acinar
differentiation, although mucigen granules and neurosecretory granules
were also occasionally found. The behavior was variable: 36% of
patients developed metastases, especially to the liver. The adult
patients did poorly: three of five died of tumor (mean survival, 18
months), and two were alive at 5 and 15 months, respectively. In
contrast, five of the six evaluable pediatric patients were alive from
22 months to 22 years after diagnosis, and only one died of tumor
after 16 months. Good responses to chemotherapy were noted in the
pediatric group.
Pancreatoblastoma: a case report.
Zhonghua Yi Xue Za Zhi (Taipei).
1994;54(3):198-203.
Primary neoplasms of the pancreas are rare in
children. One variant of these tumors is pancreatoblastoma, comprising
0.5% of epithelial tumors of the pancreas. It usually affects children
at 1-8 years of age, with quite equal sex ratio. The tumor can be
found at any site of pancreas, but it most commonly arises in the head
of pancreas. Histopathologically, pancreatoblastoma is an encapsulated
tumor with distinct organoid structures and sometimes squamoid
corpuscles. Acinar cells with zymogen granules are occasionally found.
The tumor has favorable prognosis. We present a 14-year-old female who
was admitted due to prolonged jaundice for about 2 months. Physical
examination revealed pale conjunctiva, yellowish skin color and
hepatosplenomegaly. Abdominal sonography and CT scan showed dilated
common bile duct and a tumor mass about 2 x 2 cm in dimension located
a pancreatic head. She received surgical operation to relieve
obstructive jaundice and later Whipple's operation for radical
resection. Six months after operation, the patient received the
examination of abdominal sonography, CT scan and gallium tumor scan
but there was no evidence of local recurrence or distant metastasis.
Now the patient is living well for more than one year. Because of its
rare occurrence, we demonstrate this case and review the literature.
Pancreatoblastoma in an older adult.
Arch Pathol Lab Med. 1995;119(6):547-51.
We present a case of pancreatoblastoma in a
61-year-old man. To our knowledge, this is the oldest patient with
this tumor to be reported. Originally regarded as a
microadenocarcinoma, the tumor was composed of small cells, sometimes
in acinar groups with clusters of squamoid cells. It also showed
acinar and neuroendocrine features by immunohistochemistry. Staining
for trypsin and several neuroendocrine markers was widespread and
intense in the smaller, majority-type cells. There was faint staining
for some neuroendocrine markers in the larger squamoid cells. By
electron microscopy both exocrine secretory and neurosecretory dense
core granules were found. Modern methods of immunohistochemistry and
greater understanding of rare tumor types allowed reclassification of
this tumor.
Adult metastatic
pancreaticoblastoma detected with Tc-99m MDP bone scan.
Clin Nucl Med.
2005 Aug;30(8):577-8.
Osteotropic
radiopharmaceutical uptake has been reported in a wide variety of
benign and malignant soft tissue tumors. We present an unusual case of
pancreaticoblastoma with mesenteric and omental metastases detected by
bone scan in a 69-year-old man who presented with fever, weight loss,
and renal insufficiency. Pancreaticoblastoma is a rare childhood tumor
that may occur in adults, although only two cases of adults with
metastatic disease have been described.
Pancreatoblastoma
in an adult: its separation from acinar cell carcinoma.Virchows
Arch. 1994;424(5):485-90.
Pancreatoblastomas are rare tumours, which usually occur in childhood.
Here we describe a pancreatoblastoma in a 39-year-old woman. The
tumour was located in the tail of the pancreas and consisted of cells
forming well-differentiated acinar structures and scattered solid
components ("squamoid corpuscles"). Immunocytochemically, the acinar
components were positive for pancreatic enzymes and pancreatic stone
protein, while the cells of the "squamoid corpuscles" lacked these
markers. There was no p53 overexpression nor any mutation at codon 12
of the Ki-ras oncogene. The main differential diagnosis of this tumour
was acinar cell carcinoma, because both tumours have a number of
features in common (scattered solid components, positivity for
pancreatic enzymes, lack of p53 overexpression and of Ki-ras
mutation). Findings which distinguished the pancreatoblastoma and
separated it from acinar cell carcinoma were the negativity of the
solid components ("squamoid corpuscles") for neuroendocrine markers
and their very weak keratin positivity. As the patient is alive and
well 30 months after tumour resection, this pancreatoblastoma also
differs in biology from the usual acinar cell carcinoma.
Pancreatoblastoma:
report of one case.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi.
1992 Sep-Oct;33(5):388-96.
Pancreatoblastoma
is an extremely rare pancreatic tumor in childhood, comprising 0.5% of
pancreatic non-endocrine tumors. It mostly affects children of under 8
years old without special sexual predilection. Abdominal mass is the
dominant clinical feature and abdominal X-ray, sonography, UGI series,
CT scan are of assistance in establishing diagnosis. The presence of
acinar cells with zymogen granules, squamous metaplasia and endocrine
components makes the diagnosis. We report a case of 4-year-old girl,
who suffered from abdominal pain for two months. Abdominal mass was
found at our OPD and abdominal sonography, UGI series, CT scan all
indicated a parapancreatic tumor. AFP was 4700 ng/ml. Laparotomy
confirmed a pancreatic tumor and only partial excision was performed
due to tumor invasion and adhesion to major vessels. Diagnosis of
pancreatoblastoma was made by pathohistology, cytochemical special
stains and electronic microscopic examination of the tumor. Neither
chemotherapy nor radiotherapy was performed due to family refusal.
Then patient followed up at NTUH OPD regularly. Unfortunately patient
expired because of the regrowth of residual tumor eight months later.
Pancreatoblastoma--histopathological and ultrastructural analysis of
two cases.J
Korean Med Sci. 1992 Jun;7(2):184-8.
Pancreatoblastoma
has been described in children and characterized by unique histologic
features and excellent clinical course. Ultrastructural and
immunohistochemical studies of pancreatoblastoma reveal either
exocrine alone or both endocrine and exocrine differentiation. We
present two cases of pancreatoblastoma in children in which
immunohistochemical and ultrastructural examination failed to
demonstrate features of either enzyme or hormone production and which
became worse in clinical course. We assume that pancreatoblastomas are
tumors which differentiate more toward acinar or ductal elements than
toward islet cell.
Pancreatoblastoma with
marked elevation of serum alpha-fetoprotein. An autopsy case report
with immunocytochemical study.Virchows
Arch A Pathol Anat Histopathol. 1990;416(3):265-70.
The autopsy
findings in a pancreatoblastoma in a 7-year-old Japanese girl is
reported. The tumour was in the head and body of the pancreas, and was
associated with diffuse carcinomatous peritonitis and hepatic and
pulmonary metastases. There was marked elevation (more than 10,000 ng/ml)
of serum alpha-fetoprotein (AFP). Histopathologically the tumour was
composed of solid epithelial elements with fibrous stroma, showing
acinar arrangement, squamoid clusters and tubular structures. The
epithelial elements contained numerous fine PAS positive granules in
the cytoplasm. Immunocytochemical results suggested epithelial
differentiation with positivity to alpha-1-antitrypsin (AAT), keratin,
CA19-9, and AFP. No endocrine elements were recognized. Characteristic
feature of this tumour are discussed and compared with previous
reports.
Fine needle aspiration cytology of pancreatoblastoma with
immunocytochemical and ultrastructural studies.Acta
Cytol. 1990 Sep-Oct;34(5):632-40.
The cytologic
features of a pancreatoblastoma (infantile adenocarcinoma), a rare
pancreatic neoplasm of childhood, are described. Fine needle
aspiration (FNA) under ultrasound guidance produced a hypercellular
specimen consisting of numerous oval-to-cuboidal cells that had a
moderate amount of granular cytoplasm. Spindle-shaped, elongated and
triangular-shaped epithelial cells were also seen, along with smaller
cells that had a higher nuclear/cytoplasmic ratio and a denser
cytoplasm. In addition, there were abundant fragments of stroma
present, including some surrounded by epithelial cells.
Immunoperoxidase studies performed on the aspirated material revealed
positive staining of the epithelial cells for cytokeratin (AE1/3),
including high and low molecular weight cytokeratin, carcinoembryonic
antigen, neuron-specific enolase and alpha-1-antitrypsin.
Ultrastructural examination demonstrated epithelial cells containing
either large electron-dense zymogen granules in the range of 400 nm to
600 nm or small dense neuroendocrine granules measuring from 100 nm to
200 nm. This finding, in concert with the immunocytochemical studies,
supported a "blastemal" cell origin with bidirectional differentiation
for this unusual pancreatic neoplasm and enabled a specific
preoperative diagnosis of pancreatoblastoma to be made. The
differential diagnosis of pancreatoblastoma from other pediatric
neoplasms involving the pancreas, including neuroendocrine tumors and
neoplasms of acinar cell derivation, is presented. We believe that the
FNA cytologic findings can lead to a correct diagnosis of
pancreatoblastoma, especially when coupled with immunocytochemical and
ultrastructural studies performed on the aspirated material.
Pancreatoblastoma.
A histochemical and ultrastructural analysis.Cancer.
1984 Feb 15;53(4) : 963-9.
A case of
pancreatoblastoma in a 4-year-old boy is presented. This tumor was
studied by histochemical and ultrastructural techniques and was found
to have both exocrine and endocrine components. This analysis and a
review of the literature helps to further define this entity, which
has a significantly better prognosis than other pancreatic
malignancies.
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