INTRODUCTION

Pancreatic tumor is a type of tumor that attacks pancreas and affects its exocrine,
endocrine and other supporting tissues. It can be benign or malignant. Pancreatic tumor is
quite common on people over 60-year old age, but rarely occur on people under the age of
30. Occurrence on children is quite rare with an incidence rate of only 0.46 per one million
population. Solid pseudopapillary neoplasma on children is even rarer with an incidence rate
of only 0.2 to 2.7% of all pancreatic tumors. Most patients are female within the second or
third decade of life with only a small minority concerning in children. In this report, we
present a case of a 10-year old girl with a pancreatic distal tumor which had been operated
with open distal pancreatectomy.

CASE REPORT

A 11 years old girl, without any past medical or surgical history came to our
emergency department with an enlarging abdomen mass in the upper left region and
vomitting. She denied having any abdominal pain, weight loss, diarrhea, lethargy and
jaundice. From physical examination, a mass was present on the left upper region abdomen
with solid consistency, uncircumscribed and no tenderness.
CT scan showed the presence of a mass arising from the tail of the pancreas with size
of 8.88cm x 7.28 cm x 8.75 cm, and is homogeneous with solid component. From laboratory
result, it was found a slight decrease of erythrocytes around 4.26 million/mm3. Other routine
blood investigations was within normal limits. No evidence of abnormal liver function and
cholestasis was found. Alpha fetoprotein (AFP) tumor marker and human chorionic
gonadotropin (B-Hcg) were all within normal. No examination was performed on CEA and
CA 19-9 tumor markers.
Approximately two weeks later, the patient underwent an elective surgery
(pancreatotectomy and splenectomy). It was found there was an encapsulated solid mass in
the pancreatic tail, with size of (…x… ), invades the splenic artery and vein and no potential
metastatic to the other organ. . Histopathology result showed lobules and nests of uniform
cells with eosinophilic to granular ctyoplasm, separated by dense fibrous stroma.
(Figure 1. CT scan showed the presence of a solid mass)
 (Figure 2 : Resection of tumor)

(Figure 3 : Gross pathologic specimen showed tumor of distal pancreas)

 DISCUSSION

Pancreatoblastoma also called pancreaticoblastoma or infantile type carcinoma of

pancreas, is the most common pancreatic tumor of young children. Like all pancreatic tumors
in children, pancreatoblastoma is rare, accounting for only 0.2 to 2.7% of all pancreatic
tumors. More than half of reported cases are in Asians. Patients most commonly present with
an asymptomatic, large abdominal mass. Those with symptoms usually have nonspecific
complaints such as abdominal pain, fatigue, lethargy, weight loss, anorexia, diarrhea, or
vomiting. Symptoms due to compression to gaster such as vomiting are possibility , as
reported to our patient. Jaundice is uncommon. Blood test are usually within normal range
and no spesific tumor markers are known to diagnosed pancreatoblastoma.
Histopathology result showed an encapsulated tumor composed of cystic, solid, and
hemorrhagic components. Degenerative changes lead to extensive fibrosis, hemorrhage, focal
calcification, and occasional ossification. According to WHO criteria SPNs are considered
malignant in the presence of pancreatic parenchyma, perineural and/or angiovascular
invasion, none of which were found in our case.
The examination of tumor markers are typically within the normal range. Serum AFP
levels may be used to monitor clinical response to therapy or reccurance in those patients
whose tumor produce it. Other examinations for tumor markers such as CEA, CA 19-9 were
not performed and do not show any significant correlation.
The radiological features of solid papillary neoplasma (SPN) a solid well-defined
tumor, usually heterogeneous and with solid and cystic component. A pseudocapsule may be
present and evidenced by peripheral contrast enhancement.
Resection is the primary treatment and is feasible depending on the location of the
tumor. Distal pancreatectomy involves surgical resection of the body and tail of the pancreas
to the left of the superior mesenteric vein (SMV )- portal vein confluence with or without
splenectomy. Pancreaticoduodenectomy is procedure removal tumor that located in the head
of pancreas. Complete resection ranges from 66-80% but reccurence after resection remains
as the main adverse outcome factor. Distal pancreatectomy with splenectomy indicates for
tumor that affects the splenic artery and vein as reported in this case. Since there are different
clinical outcomes, surgery remains the only standard treatment. The role of chemotherapy is
for unresectable, metastatic, or recurrent disease.
 CONCLUSION

Pancreoblastoma is a rare clinical phenomenon especially in children. Prognosis of

this rare tumor is good, when resected completely. Prognosis is poorer, when there is
metastasis or when it is inoperable. Early diagnosis and management, including urgent
evacuation of the tumor and chemotherapy can lead to a full recovery and positive clinical
outcomes.

DAFTAR PUSTAKA

Ellen M, Mark D, Richard M. Pancreatic Tumors in Children : Radiologic-Pathologic

Correlation. AFIF 2006, Vol 26 (4).
Tabusso FY, Placencia, RF. Two Cases of Adult Pancreatoblastoma : Infrequent
Differential Diagnosis. JOP 2017, vol 18 (3): p283-285.
Cao G, Mendez J. Pancreatoblastoma in a Pediatric Patient: Anatomo-pathological
Aspects of a Case with Multiple Hepatic Metastases. Ecancermedicalscience 2018, vol 12.
Branco C, Vilaca S, Falcao J. Solid Pseudopappilary Neoplasm- Case Report of a
Rare Pancreatic Tumor. IJSCR 2017, vol 33: p148-150.
Singh AP et al. Pancreatoblastoma in a Child. Formosan Journal of Surgery 2017, vol
50(50); p 227-230.