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Journal of Indian Association of Pediatric Surgeons
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Table of Contents   
CASE REPORT
Year : 2022  |  Volume : 27  |  Issue : 6  |  Page : 747-750
 

Successful outcome of metastatic pancreatoblastoma in an adolescent: A case report and review of the literature


1 Department of Pediatrics, Division of Pediatric Hemato-Oncology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
2 Department of Pediatrics, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
3 Department of Surgical Oncology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
4 Department of Pathology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
5 Department of Radio Diagnosis, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India

Date of Submission11-Jan-2022
Date of Decision13-Mar-2022
Date of Acceptance30-Apr-2022
Date of Web Publication11-Nov-2022

Correspondence Address:
Dhaarani Jayaraman
Department of Pediatrics, Division of Pediatric Hemato-Oncology, Sri Ramachandra Institute of Higher Education and Research, No. 1, Ramachandra Nagar, Porur, Chennai - 600 116, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaps.jiaps_4_22

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   Abstract 


Pancreatoblastoma, an orphan disease, is the most common malignant epithelial neoplasm of the pancreas in children. With vague clinical features, diagnosis is made by radiological suggestions and histopathology. The presence of metastatic disease and inoperable/incomplete excision remains the poor prognostic markers. We present a rare instance of an adolescent who has survived metastatic pancreatoblastoma after neoadjuvant chemoreduction/complete surgical excision.


Keywords: Pancreatic malignancies, pancreatoblastoma, PLADO chemotherapy, serum alpha-fetoprotein, squamoid nests


How to cite this article:
Jayaraman D, John A, Bose JC, Venkitaraman B, Shalini S, Murali A. Successful outcome of metastatic pancreatoblastoma in an adolescent: A case report and review of the literature. J Indian Assoc Pediatr Surg 2022;27:747-50

How to cite this URL:
Jayaraman D, John A, Bose JC, Venkitaraman B, Shalini S, Murali A. Successful outcome of metastatic pancreatoblastoma in an adolescent: A case report and review of the literature. J Indian Assoc Pediatr Surg [serial online] 2022 [cited 2022 Nov 30];27:747-50. Available from: https://www.jiaps.com/text.asp?2022/27/6/747/360959





   Introduction Top


Pancreatic tumors in children are extremely rare with an incidence of 0.004/1,00,000 population. Due to nonspecific signs and symptoms, imaging and histopathology remain the mainstay of diagnosis. Metastasis at diagnosis portends a poor prognosis. We report an adolescent boy successfully managed with chemotherapy and complete surgical excision of metastatic pancreatoblastoma.


   Case Report Top


A 17-year-old boy presented with abdominal pain for 3 weeks. The clinical examination revealed a firm ill-defined, nontender abdominal mass palpable in the epigastric region.

Ultrasonogram (USG) of the abdomen revealed a hypoechoic and heterogeneous solid mass measuring 13 cm × 8 cm × 8 cm seen in the epigastric region. Complete blood counts and renal and liver function tests were normal; he had elevated lactate dehydrogenase and hyperuricemia of 11 mg/dl. Serum alpha-fetoprotein (AFP) and CA19.9 were normal.

Computed tomography (CT) with contrast showed a heterogeneously enhancing soft-tissue density mass of approximately (14.6 cm × 9.8 cm × 11.5 cm) in retroperitoneum involving tail of pancreas and hypoechoic liver nodules with mass effect over adjacent bowel loops.

USG-guided biopsy from the largest liver nodule showed infiltrating tumor cells arranged in nests, cords, and glandular patterns lined by polygonal cells with a scanty amount of eosinophilic cytoplasm, hyperchromatic nuclei, and inconspicuous nucleoli with surrounded desmoplastic stroma. Immunohistochemistry showed positivity for cytokeratin, CK7, CK19, CDX2 (suggestive of glandular structures) and CD10, CYCLIN-D1, and beta-catenin [Figure 1] consistent with metastatic malignant tumor with acinar and squamoid features, suggesting pancreatoblastoma.
Figure 1: Infiltrating tumor cells arranged in nests, cords, and glandular pattern lined by polygonal cells with scant amount of eosinophilic cytoplasm, hyperchromatic nuclei, and inconspicuous nucleoli with surrounded desmoplastic stroma (40×- high power view on left 100× - on right side)

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Staging positron emission tomography-CT (PET-CT) scan showed fluorodeoxyglucose avid neoplastic lesion in the distal body and tail of the pancreas with peripancreatic, perigastric lymph nodal spread, multiple hepatic metastases, and possible peritoneal metastases [Figure 2].
Figure 2: Primary neoplastic lesions on distal body and tail of pancreas with peripancreatic and perigastric lymph nodal spread, multiple hepatic metastases, and possible peritoneal metastases

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As the lesion was inoperable, we started neoadjuvant platinum (cisplatin) doxorubicin chemotherapy (cisplatin and doxorubicin) similar to the SIOPEL-hepatoblastoma protocol. Repeat PET-CT after four cycles of neoadjuvant chemotherapy showed near-complete metabolic response to therapy. Serum uric acid remained high between 8 and 10 mg/dL until surgical resection; however, renal function was normal throughout.

The patient underwent diagnostic laparoscopy before laparotomy which showed no definite lesions in the liver, and only postchemotherapy scars were made out. Intraoperative ultrasound of the liver did not reveal any parenchymal lesion. Frozen section biopsy of scar lesion was negative for malignancy. Distal pancreaticospleenectomy was done with adequate hemostasis for a better surgical clearance of microscopic residual disease [Figure 3]. The postoperative stay was uneventful without major complications.
Figure 3: Liver showing scar, confirmation with intraoperative ultrasound; forceps indicating the location of tumor in the distal pancreas, pancreas divided at the junction of the body and tail using staplers and pancreatic remnant postresection

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Histopathology of excised specimen showed completely necrotic tumor with negative margins. He was treated with two cycles of adjuvant chemotherapy, is well on follow-up for the past 3.5 years and in remission as screened by ultrasonography of the abdomen once in 3 months.


   Discussion Top


Pancreatoblastoma remains the most common pancreatic tumor in children. According to the RARECAREnet database by the European Union Joint Action on Rare Cancers, pancreatoblastoma fits in very rare pediatric cancers, defined as those with an annual incidence <2/1,000,000 (orphan disease).[1]

Pancreatoblastoma usually occurs in children aged <10 years with a median age of 4–5 years. Boys are more commonly affected than girls.[1] Pancreatoblastoma is an aggressive tumor and 35%–50% of them present with locally advanced or metastatic disease.[2] Sites of metastasis commonly include the regional lymph nodes, omentum, liver, and lungs.

The higher incidence of metastasis at diagnosis is partly due to the aggressive nature of the disease, extreme rarity, and nonspecific signs and symptoms. Vague abdomen pain and mass in the epigastric region are commonly noted. Diagnosis is often suspected based on imaging finding of a mass arising from the pancreas and is confirmed with histopathology.

Common association with familial adenomatosis polyposis has been described, and rarely, an association with Beckwith–Wiedemann syndrome, similar to that of hepatoblastoma. Allelic loss of chromosome 11p is noted in more than 80% of cases.[2],[3]

Some genetic similarities have been noted between pancreatoblastoma and hepatoblastoma (i.e., loss of heterozygosity at 11p15.5, overexpression of IGF2, and beta-catenin mutations).[2] Hence, serum AFP remains a tool for diagnosis, monitoring response to treatment, and also to predict relapse. However, cases of pancreatoblastoma with normal AFP levels have also been described, similar to our patient. Poor prognosis related to low AFP as in hepatoblastoma, however, is not highlighted in pancreatoblastoma.

Apart from CA19.9, a rare instance of secretion of antidiuretic hormone and cortisol by pancreatoblastoma has been described.[4] Hyperuricemia is possible in all malignancies; however, the strikingly high value of 11 mg% which was persistent in our child until surgery without any renal failure, was not reported as a significant event in the literature.

The patient was staged as stage 4 in view of metastatic lesions to the liver as per the tumor staging proposed by the Italian Tumori Rari in Età Pediatrica group.[4]

Macroscopically, the lesion is lobed and solid with a heterogeneous, necrotic, hemorrhagic cut surface. Microscopically, it is characterized by being cellular, cytologically uniform organization of cells in nests and islets, with a tendency to form acinar structures. The formation of whorled nests of scaly spindle cells called squamoid corpuscles is characteristic.

Immunohistochemistry reveals diffuse expression of cytokeratin membrane (AE1–AE3) and epithelial membrane antigen and AFP in secreting tumors.[3],[5]

Surgery remains the mainstay of management and complete surgical excision is the most consistent prognostic factor having significantly better outcomes.

Cases of partial hepatectomy and liver transplant in children with liver metastasis have been described;[5] however, in our patient, as he had complete metabolic response and nonviable tissue in the nodules after chemotherapy, extreme resections were not necessary; however, due to infiltration of splenic hilum and nodes, splenectomy was mandatory for complete tumor clearance.

The role of chemotherapy or radiotherapy is significant in inoperable or metastatic cases. Although various chemotherapeutic agents such as vincristine, doxorubicin, etoposide, cyclophosphamide, cisplatin, and adriamycin have been implicated to be effective, there are no standard regimens

PLADO regimen is used with good results due to biological similarities to hepatoblastoma.[4]

In our patient, metastatic lesions necessitated neoadjuvant chemotherapy.

A long-term follow-up for pancreatic exocrine and endocrine insufficiency is indicated based on the extent of surgical excision.


   Conclusion Top


Pancreatoblastoma is the most common pancreatic malignant neoplasms. Similarities in histology, treatment, and rise in serum AFP between hepatoblastoma and pancreatoblastoma can be explained by the similar embryonic origin. Prognosis depends on the feasibility of complete resection, response to therapy, and distant metastasis at diagnosis. Judicious use of neoadjuvant chemotherapy to optimize surgical excision in locally invasive/metastatic lesions will improve the outcomes even in advanced-stage diseases.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Ferrari A, Brecht IB, Gatta G, Schneider DT, Orbach D, Cecchetto G, et al. Defining and listing very rare cancers of paediatric age: Consensus of the Joint Action on Rare Cancers in cooperation with the European Cooperative Study Group for Pediatric Rare Tumors. Eur J Cancer 2019;110:120-6.  Back to cited text no. 1
    
2.
Abraham SC, Wu TT, Klimstra DS, Finn LS, Lee JH, Yeo CJ, et al. Distinctive molecular genetic alterations in sporadic and familial adenomatous polyposis-associated pancreatoblastomas: Frequent alterations in the APC/beta-catenin pathway and chromosome 11p. Am J Pathol 2001;159:1619-27.  Back to cited text no. 2
    
3.
Muguerza R, Rodriguez A, Formigo E, Montero M, Vázquez JL, Páramo C, et al. Pancreatoblastoma associated with incomplete Beckwith-Wiedemann syndrome: Case report and review of the literature. J Pediatr Surg 2005;40:1341-4.  Back to cited text no. 3
    
4.
Dall'igna P, Cecchetto G, Bisogno G, Conte M, Chiesa PL, D'Angelo P, et al. Pancreatic tumors in children and adolescents: The Italian TREP project experience. Pediatr Blood Cancer 2010;54:675-80.  Back to cited text no. 4
    
5.
Ghaffarian AA, Book L, Meyers RL. Liver transplant for metastatic pancreatoblastoma: 7-year event-free survival after chemotherapy, pancreatectomy, complete hepatectomy, and liver transplant. Pediatr Transplant 2018;22:10.1111/petr.13098.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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