Berkala Ilmu Kedokteran Indrawati et al, Pancreatoblastoma : A case report

Vol. 39, No. 1, Maret 2007: 59-63

Pancreatoblastoma : A case report

Indrawati1, Rahmayani1, Susi Hariyati2, Soeripto1
1
Department of Anatomic Pathology, Faculty of Medicine, Gadjah Mada University
2
Department of Pediatric, Sardjito General Hospital, Jogjakarta

ABSTRACT
Indrawati, Rahmayani, Susi Haryati, Soeripto - Pancreoblastoma : A case report

A 2.5 year old female child was admitted to Dr. Sardjito Hospital with complaint of enlarging
abdomen since 6 months. She was apparently well until 3 months prior to admission when she had
anorexia and weight lost. Physical examination revealed a solid intrabdominal mass (15x13 cm) with
smooth surface and no tenderness. USG of the abdomen showed a large mass in paraaortal region and
pushed the aorta. Operation was done and during exploration the mass was located as high as the stomach
level and extended into the pancreas. Gross examination showed the tumor was 11x11x8 cm, encapsulated,
nodular, white cut surface, some parts were brown and most of them were fragile. The diagnosis of
pancreoblastoma was established by immunohistochemical examination.
The presenting features of pancreoblastoma are generally nonspecific and clinically difficult to distinguish
from other intraabdominal tumors such as neuroblastoma, non-Hodgkin lymphoma, Wilms tumor,
hepatoblastoma and desmoblastoma. Some clinical tests might suggest these tumors, i.e. multiorgan
involvement for non-Hodgkin lymphoma; renal origin, the propensity for venous invasion and for the
pulmonary metastasis for Wilms tumor. a–feto protein that is positive in either hepatoblastoma or
pancreoblastoma cannot differ both tumors. Another intrabdominal tumor that should be considered is
desmoblastoma that positive vimentin stain. The positive CAM5.2. as well as cytokeratin and the negative
vimentin in immunohistochemical examination confirmed the diagnosis of pancreoblastoma.

Keywords : pancreoblastoma – child – intraabdominal tumors – immunohistochemical examination

ABSTRAK
Indrawati, Rahmayani, Susi Haryati, Soeripto - Pancreoblastoma : Laporan kasus

Seorang anak perempuan berumur 2.5 tahun dirawat di Rumah Sakit Dr. Sardjito dengan keluhan
perut semakin membesar seajk 6 bulan. Sebelumnya anak tampak sehat sampai 3 bulan sebelum masuk
rumah sakit, waktu itu dia mulai anoreksia dan berat badannya menurun. Pemeriksaan fisik menunjukkan
adanya massa intraabdominal padat (15x13 cm) dengan permukaan rata dan tidak nyeri tekan. Pemeriksaan
USG abdomen menemukan satu massa yang besar, di regio para-aorta dan mendesak aorta. Tindakan
bedah dilakukan, dan pada waktuj eksplorasi ternyata massa itu terletak setinggi lambung dan meluas ke
dalam pancreas. Pemeriksaan luar menunjukkan tumor berukuran 11x11x8 cm, berkapsul, nodular,
permukaan potongan berwarna putih, beberapa bagian coklat dan kebanyakan rapuh. Diagnosis
pancreoplastoma ditegakkan dengan pemeriksaan immunohistokimiawi.
Gambaran klinis pancreoblastoma secara umum tidak khas dan klinis sukar dibedakan dengan tumor
intraabdominal lainnya seperti neuroblastoma, limfoma non-Hodgkin, tumor Wilms, hepatoblastoma dan
desmoblastoma. Beberapa uji klinis mungkin mengarah ke dugaan tumor-tumor tersebut, misalnya keterlibatan
multiorgan untuk non-Hodgkin lymphoma, kecenderungan untuk invasi vena dan metastasis di paru untuk
tumor Wilms. a–feto protein positif pada hepatoblastoma maupun pancreoblastoma sehingga tidak dapat
membedakan kedua tumor itu. Tumor intraabdominal lain yang juga perlu dipertimbangkan adalah
desmoblastoma, tumor ini positif pada pemulasan vimentin. CAM5.2. cytokeratin yang positif serta vimentin
yang negatif pada pemeriksaan immunohistokimiawi memastikan diagnosis pancreoblastoma.

Indrawati, Rahmayani, Soeripto, Department of Anatomic Pathology,

Faculty of Medicine, Gadjah Mada University.
Susi Hariyati, Department of Pediatric, Sardjito General Hospital, Yogyakarta

59
Berkala Ilmu Kedokteran, Volume 39, No. 1, Maret 2007: 59-63

INTRODUCTION is uncertain.12 These tumors tend to have a good

prognosis, accounting only for 0.2% of deaths for
Pancreatoblastoma is a rare pancreatic tumor
malignancies in this age13, although poor results and
with distinct acinar and squamoid cell differentiation
occasional distant metastases (lung, liver or lymph
that generally affected infants and young children. This
nodes) 14 have been reported. Considering the
tumor accounts for approximately 0.5 % of pancreatic
favourable prognosis, pancreatoblastoma should be
non endocrine tumors and just over 50 cases have
differentiated from other aggressive tumors in
been reported in the literature till 1997.1
children
Pancreatoblastoma has been first reported in the
A case of pancreatoblastoma is reported and
surgery literature by Becker in 1957. The earliest
its differential diagnosis will be discussed in this
histopathologic descriptionwas published by Frable et
article
al. in 1971. Although the tumor was initially referred
to as “infantile carcinoma of the pancreas,”its histologic
resemblance to fetal pancreatic tissue at approximately CASE
7 weeks gestation led Horie et al., in 1977, to propose Clinical history and examination
that the original name be replaced with pancreato-
A 2.5 year old child male was admitted to Dr.
blastoma. Pancreatoblastoma has since become the
Sardjito Hospital with chief complaint of an enlarging
accepted term and is used interchangeably with
abdominal mass. Since six months prior to referral,
pancreaticoblastoma.2,3
she had a mass in her abdomen which became
Pancreatoblastoma usually affects patients
larger rapidly. She was apparently well until three
between the age of 1 and 8 years, but it has been
months prior to referral when she had anorexia and
reported in neonates and in the elderly.4,5 The
weight lost.
congenital form is associated with Beckwith-
Clinical examination revealed a solid intra-
Wiedemann syndrome and has been described as
abdominal mass (15 x 13 cm), with smooth surface
being cystic. The tumor is slightly more common in
and no tenderness. USG of the abdomen revealed
male patients, and half of all cases reported in the
a large mass which cannot be reached by trans-
literature occurred in Asians.5,6
ducer in para aorta region and pushed the aorta.
The etiology of pancreatoblastoma is unknown
During exploration, the mass was revealed located
and the head of the gland is affected in about 50% of
as high as the stomach level and extended into the
cases7 The clinical presentation of pancreatoblastoma
pancreas. There was no abnormality of the kidney
was variable. Typically, pancreatoblastoma is slow-
and liver.
growing, clinically occult,and quite large at the time of
Gross examination : Tissue 11x11x8 cm,
diagnosis.8 Symptoms, when present, are usually
encapsulated, nodular and fragile with white cut
related to the mass effect and include early satiety,
surface, some parts were brown and most of them
vomiting, constipation, and pain. Cushing syndrome
were fragile (FIGURE 1).
and the syndrome of inappropriate antidiuretic
Five slides from different areas were made
hormone secretion have been reported.These
for microscopic examination by routine
endocrine syndromes are postulated to be the result
Hematoxylin-eosin staining and one slide was
of adrenocorticotrophic hormone secretion by the
examined by PAS staining. Three blocks of
tumor.9 Laboratory values may reveal elevated
parrafine embedded tissue were sent to The
serum levels of a-fetoprotein, a-antitrypsin, or
Department of Pathology, AMC, The Netherlands,
lactate dehydrogenase .10 Local spread, including
for confirmation of the diagnosis and immuno-
omental and peritoneal disease and vascular
histochemical examination.
invasion, has been described. The liver is the most
Microscopic examination : Tumor with variety
common site for metastasis. Other sites include the
of cellular histological pictures. Several areas
lung, bone and posterior mediastinum. 11
showed irregular fields of epitheloid cells surrounded
Therapy of pancreatoblastoma consists of
by desmoplastic stroma and some areas revealed
surgical resection of the localized tumor. The role
the impression of tubular/glandular differentiation
of adjuvant chemotherapy for a resectable tumor

60
 Indrawati et al, Pancreatoblastoma : A case report

and rosette-like formation. The tumor cells were

small, round and oval with vesicular round nuclei
and scanty cytoplasm. The other solid area
consisted of tumor cells with fusiform and
hyperchromatic nuclei. Several areas showed
marked necrosis. Infiltration of tumor cells into
fibrous capsul tissue and to some vessels were also
noted (FIGURE 2-3). Part of tumor cells showed
PAS positive staining.
Immunohistochemical examination showed
expression of CAM5.2 and in some of the tubular
structures showed expression of cytokeratin 7.
Vimentin, desmin and neuroendocrine markers were FIGURE 3. Microscopic feature of pancreatoblastoma
negative (FIGURE 4-5). (HE staining, 400X)

CONCLUSSION
Intraabdominal mass : Pancreatoblastoma

FIGURE 4. Positive expression of cytokeratin shows

brown cytoplasmic staining

FIGURE 1. Macroscopic feature of pancreatoblastoma

FIGURE 2. Microscopic feature of pancreatoblastoma FIGURE 5. Vimentin staining shows negative expression of
(HE staining, 100X) tumor cells

61
Berkala Ilmu Kedokteran, Volume 39, No. 1, Maret 2007: 59-63

DISCUSSION been associated with improved prognosis in patients

with advanced disease. In completely resected tumors,
The presenting feature of pancreatoblastoma
pure fetal histology confers a better prognosis, while
are generally nonspesific. Especially in the pediatric
small-cell undifferentiated histologyis associated with
age group, many patients present with an inciden-
a poor prognosis. 17,18,19 The distinction of pancrea-
tally detected abdominal mass. Clinically,
toblastoma from hepatoblastoma may be difficult as
distinguishing pancreatoblastoma from other
both tumors may occur with an elevated level of a-
pediatric abdominal masses might be difficult,
fetoprotein. In addition, histopathologhical feature of
particularly when the tumor is large and when its
hepatoblastoma especially mixed epithelial/mesen-
origin is uncertain. In such cases, the differential
chymal type may resemble with pancreatoblastoma
diagnosis in a child includes a consideration of any
in this reported case. However, in contras to pan-
large intra- or retroperitoneal mass, such as a
creatoblastoma, immunohistochemical examination
neuroblastoma, non-Hodgkin lymphoma, or Wilms
of vimentin was weakly positive in those hepato-
tumor.2 Neuroblastoma often appears as a large,
blastomas where mesenchymal tissue was present
heterogeneous, calcified abdominal mass. The
in the tumor.20,21
distinction from pancreatoblastoma can be made
The other important histopathological differential
clinically on the basis of the predilection for
diagnosis of pancreatoblastoma is desmoplastic
neuroblastoma in very young patients, the
small round cell tumor.22 Desmoplastic small round
characteristic paraneoplastic syndromes and the
cell tumor is a recently recognized rare cancer of
presence of urine catecholamines. Non-Hodgkin
uncertain histogenesis. This tumor is a primitive
lymphoma may present as a large, abdominal soft-
sarcoma with distinctive histopathological features
tissue mass or masses. The high incidence of
that suggest a multilineage origin .23,24 Most patients
multiorgan involvement at presentation, may help
with this tumor are 15-35 years of age, although
to distinguish non-Hodgkin lymphoma from
patients as young as 5 years have been reported.25
pancreatoblastoma. A Wilms tumor is another
The patients present with a large abdominal and/
potentially large, heterogeneous, retroperitoneal
or pelvic mass with extensive peritoneal invol-
tumor that occurs in young children. However, its
vement, usually without an identifiable visceral site
renal origin,its propensity for venous invasion, and
of origin. The most common complaint is abdominal
its pulmonary metastases help to distinguish it from
distension, often associated with pain and consti-
pancreatoblastoma. 2,15
pation.26 Desmoplastic small round cell tumor is a
When the tumor appears to arise from the liver,
highly aggressive neoplasm with an extremely poor
pancreatoblastoma may resemble other primary
prognosis.27. It has been shown to be sensitive to
pediatric hepatic neoplasms, such as hepatoblastoma.2
alkylating agents and is dose-responsive. Multi-
Hepatoblastoma is the most common malignant
modality treatment with high-dose chemotherapy,
tumor of the liver in children. Nearly 90 % of
aggressive debulking surgery, radiotherapy, and
hepatoblastoma are seen in the first 5 year of life,
myeloablative chemotherapy with stem cell rescue
with 60% presenting in the first 2 years.16 Most patients
may improve progression-free survival. Although
present with an enlarging abdominal mass. The right
such treatment offers good palliation and may
lobe is involved three times more commonly than the
prolongsurvival in isolated cases, it is not curative.28
left, with bilobar involvement seen in 20%-30%, and
Two basic morphologic features of desmoplastic
multicentric involvement in 15%. Less common
small round cell tumor include small round cells and
symptoms are anorexia, weight loss, and pain.
a fibrosclerotic stroma. The tumor cells appear
Hepatoblastoma is classified by histology as epithelial
undifferentiated and have small hyperchromatic
(56%) or mixed epithelial/mesenchymal (44%).17
nuclei with inconspicuous nucleoli and scant amount
Epithelial hepatoblastoma is further broken down to
of eosinophilic cytoplasm.25 This histopathological
pure fetal (31%), embryonal (19%), macrotrabecular
feature may resemble with pancreatoblastoma.
(3%) and small-cell undifferentiated (3%) . The most
However, in contrast with pancreatoblastoma,
common mesenchymal elements are osteoid and
desmoplastic small round cell tumor is characterized
cartilage.The presence of mesenchymal elements has

62
 Indrawati et al, Pancreatoblastoma : A case report

by a polyphenotypic profile with expression of 12. Dhebri AR, Connor S, Campbell F. Diagnosis, treatment
epithelial, mesenchymal and neural markers. All and outcome of pancreatoblastoma. Pancreatology 2004;
4: 441-51
tumors stain for epithelial markers, including 13. Tsukimoto I, watanabe K, Lin J. Pancreatic carcinoma in
cytokeratins and epithelial membrane antigen. In children in Japan. Cancer 1973 : 53 ; 963-69
addition, although the most useful diagnostic marker 14. Buchino JJ, Castello FM, Nagari HS. Pancreatoblastoma: a
is desmin, virtually all desmoplastic small round cell histochemical and ultrastructural analysis. Cancer 1984;
tumors stain for vimentin. 29 The positivity of 53:963-69.
15. Friedman AC, Edmonds PR. Rare pancreatic malignancies.
vimentin can differentiate desmoplastic small round Radiol Clin North Am 1989; 27:177-90
cell tumor from pancreatoblastoma. 16. Stocker J T. Hepatic Tumors in Children in Suchy FJ,
Sokol FJ, Balistreri WF. Editors. Liver Disease in Children.
2th ed. Lippincott Williams & Wilkins, 2001 : 927-35
CONCLUSION 17. Cynthia E. Herzog, Richard J. Andrassy, Farzin Eftekhari
A case of pancreatoblastoma was reported. Childhood Cancers: Hepatoblastoma The Oncologist ; 5 :
445-53
The positivity of CAM5.2, cytokeratin and
18. Stocker JT. Hepatoblastoma. Semin Diagn Pathol
negativity of vimentin examined by immuno- 1994;11:136-43.
histochemical staining can differentiate pancreato- 19. Haas JE, Muczynski KA, Krailo M et al. Histo-pathology
blastoma from hepatoblastoma and desmoplastic and prognosis in childhood hepatoblastoma and
small round cell tumor . hepatocarcinoma. Cancer 1989;64:1082-95.
20. O’Brien WJ, Finlay JL, Gilbert-Barness EF. Patterns of
antigen expression in hepatoblastoma and hepatocellular
REFERENCE carcinoma in childhood. Pediatr hematol Oncol 1989; 6:
361-65
1. Chun Y, Kim W, Park K, Lee S, Jung S. Pancreatoblastoma.
21. Kimura N, Yonekura H, Okamoto H, Nagura H. Expression
J Pediatr Surg 1997 ; 32 : 1612-65
of human regenerating gene mRNA and its product in normal
2. Montemarano H, Lonergan GJ, Dorothy I. Bulas DI, Selby
and neolastic human pancreas. Cancer 1992 ; 70 : 1857-63
DM. Pancreatoblastoma: Imaging Findings in 10 Patients
22. Bismar TA, Basturk O, Grald WL, Schwarz K, Adsay NV.
and Review of the Literature. Radiology 2000; 214:476-82
Desmoplastic small cell tumor in the pancreas. Am J Surg
3. Horie A, Yano Y, Kotoo Y. Morphogenesis of
Pathol 2004 ; 28 : 808-12
pancreatoblastoma, infantile carcinoma of the pancreas:
23. Basade MM, Vege DS, Nair CN. Intra-abdominal
report of two cases. Cancer 1977; 39: 247-54
desmoplastic small round cell tumor in children : a
4. Levey JM, Banner BF. Adult pancreatoblastoma: a case
clinicopathologic study. Pediatr Hematol Oncol 1996; 13 :
report and review of the literature. Am J Gastroenterol
95
1996; 91:1841-44.
24. Gerald WL, Miller HK, Battifora H. Intra-abdominal
5. Klimstra DS, Adair CF, Hefess CS. Pancreatoblastoma: a
desmoplastic small round cell tumor: Report of 19 cases of
clinicopathologic study and review of the literature. Am J
a distinctive type of high-grade poly-phenotypic
Surg Pathol 1987; 11:855-65.
malignancy affecting young individuals. Am J Surg Pathol
6. Drut R, Jones MC. Congenital pancreatoblastoma in
1991 ;15: 499–513
Beckwith-Wiedemann syndrome: an emerging association.
25. Weiss SW, Goldblum JR. Enzingers and Weiss Soft Tissue
Pediatr Pathol 1988; 8:331-39.
Tumors. 4th ed. Mosby Company, 2001, 1538-45
7. Hamilton SR, Aaltonen LA. Pathology and genetics of
26. Gerald WL, Ladanyi M, Alava E. Clinical, pathologic, and
Tumors of The Digestive System. WHO Classification of
molecular spectrum of tumors associated with t (11;22)
Tumors, IARC 2001 ; 244-45
(p13;q12): Desmoplastic small round-cell tumor and its
8. Mergo PJ, Helmberger TK, Buetow PC, et al. Pancreatic
variants. J Clin Oncol 1998 ; 16: 3028–36.
neoplasms: MR imaging and pathologic correlation.
27. Schwartz RE, Gerald WL, Kushner BH. Desmoplastic
RadioGraphics 1997; 17:281-301.
small round cell tumors: Prognostic indicators and results
9. Passmore SJ, Berry PJ, Oakhill A. Recurrent pancreato-
of surgical management. Ann Surg Oncol 1998 ; 5:416–22
blastoma with inappropriate adrenocorticotropic hormone
28. Kushner BH, LaQuaglia MP, Wollner N. Desmoplastic
secretion. Arch Dis Child 1988; 63:1494-96.
small round cell tumor: Prolonged progression free survival
10. Willnow U, Willberg B, Schwamborn D. Pancreatoblastoma
with aggressive multimodality therapy. J Clin Oncol 1996;
in children: case report and review of the literature. Eur J
14:1526–31
Pediatr Surg 1996; 6:369-72.
29. Ordonez NG. Desmoplastic small round cell tumor. An
11. Ohaki Y, Misugi K, Hirose M. Pancreatic carcinoma of
Ultrastructural and immunohistochemical study with
childhood: report of an autopsy and review of the literature.
emphasis on new immunohistochemical markers. Am J
Acta Pathol Jpn 1985; 35:1543-54.
Surg Pathol 1998 : 22 ; 1314

63