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CASE REPORT
Year : 2017  |  Volume : 50  |  Issue : 6  |  Page : 227-230

Pancreatoblastoma in a child


Department of Pediatric Surgery, SMS Medical College, Jaipur, Rajasthan, India

Date of Submission30-Mar-2017
Date of Decision23-Apr-2017
Date of Acceptance12-May-2017
Date of Web Publication08-Dec-2017

Correspondence Address:
Dr. Aditya Pratap Singh
Near the Mali Hostel, Main Bali Road, Falna, Pali, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/fjs.fjs_53_17

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  Abstract 


Pancreatoblastoma (PB) is a rare pancreatic tumor in children. Its biologic behavior is aggressive with frequent local invasion, recurrence, and metastasis, for which there has been no standard treatment regimen. Complete surgical resection has been considered for long-term survival of patients with PB. We present here a case of PB in a 3-year-old male child.

Keywords: Child, pancreatic neoplasms, pancreatoblastoma


How to cite this article:
Singh AP, Shukla R, Tanger R, Gupta AK. Pancreatoblastoma in a child. Formos J Surg 2017;50:227-30

How to cite this URL:
Singh AP, Shukla R, Tanger R, Gupta AK. Pancreatoblastoma in a child. Formos J Surg [serial online] 2017 [cited 2020 Sep 23];50:227-30. Available from: http://www.e-fjs.org/text.asp?2017/50/6/227/220349




  Introduction Top


Pancreatoblastoma (PB) is an unusual malignant exocrine tumor arising in the pancreas. PB is reported with an annual incidence of around 0.004 new cases per 100,000. PB is frequently seen in childhood, but it may occur at any stage of life; nevertheless, adults and fetuses appear to be free from this disease.[1],[2] Although this tumor was formerly known as “infantile carcinoma of the pancreas,” Horie et al.[3] named the tumor PB in 1977. Complete surgical resection is the key to good prognosis in the absence of metastatic disease. Chemotherapy and radiotherapy are recommended for recurrent, unresectable, or metastatic cases, but there has been no standard regimen so far.


  Case Report Top


A 3-year-old boy suffering from abdominal pain was referred to our institute for further examination and treatment. Three days earlier, he had been examined for abdominal pain elsewhere, and abdominal ultrasonography (US) had identified a well-defined, hypoechoic, heterogeneous solid mass measuring 6 cm × 5 cm × 9 cm seen in the upper abdominal region with few microcalcifications, likely neuroblastoma. Clinical examination was normal in our hospital except for a palpable abdominal mass. He had no comorbidities and his medical history revealed nothing of significance. Laboratory tests showed that hemoglobin was 8.9 g/dl, hematocrit was 28.9%, and lactic dehydrogenase was 930 U/L. Other routine blood investigations including renal function test, liver function test, serum electrolytes, serum alpha fetoprotein (AFP), and 24 h urinary vanillylmandelic acid level were within normal limits. Abdominal Doppler US showed a large solid mass noted in the midline which is pushing the stomach, the portal vein anteriorly with few collaterals at porta, and encasing celiac artery and its branches showing tiny internal foci suggestive of extra-adrenal neuroblastoma.

Computed tomography identified a heterogeneously enhancing soft-tissue density mass of approximately 7 cm × 8 cm × 9 cm in retroperitoneum involving the body of pancreas with mass effect over the adjacent bowel loops [Figure 1]. The tumor was partially encasing the splenic vein, portal veins, hepatic artery, and abutting hepatic parenchyma and gastric wall with no evidence of their infiltrations. No distant metastatic tumor was identified in the preoperative imaging studies. A tru-cut biopsy was planned for the patient. Tru-cut biopsy showed biphasic population including thin spindle cells and epithelial differentiation with the formation of gland-like structure, suggestive of undifferentiated malignant neoplasm and a possibility of Wilms' tumor. Immunohistochemistry report of the tru-cut biopsy showed positive for neuron-specific enolase and suggested small round cell tumor, likely neuroblastoma. Neoadjuvant chemotherapy was started for neuroblastoma. After four cycles of the chemotherapy, abdominal Doppler US and contrast-enhanced computed tomography (CECT) abdomen were repeated. Abdominal Doppler US showed a midline, lobulated, mixed heterogeneous solid mass (95 mm × 48 mm) noted in the left-side retroperitoneum containing few cystic areas with few tiny calcific foci crossing midline suggestive of pancreatic space-occupying lesion. There was no change in the size of the mass on CECT abdomen with the same findings. Hence, surgical exploration of the mass was planned for the patient. Surgery revealed that the primary lesion was arising from the body of pancreas. It was not firmly adhered to the vessels and adjacent viscera with well-defined margin but partially encasing the vital structures including the major vessels. The bile duct, portal vein, and the inferior vena cava were also compressed but patent. It was firmly adherent at the body of pancreas so it was removed subtotally, leaving few parts behind to save major vascular structures and viscera. Excised specimen was sent for histopathology and showed lobules and nests of uniform cells with eosinophilic to granular cytoplasm, separated by dense fibrous stroma. The cells are arranged in solid nests, acinar and glandular pattern with scattered squamous corpuscles, suggestive of PB. Immunohistochemistry showed positive for pan cytokeratin, patchy positive for cytokeratin 7, and carcinoembryonic antigen while negative for synaptophysin, chromogranin A, and vimentin, suggestive of PB. After surgery, the patient was treated with adjuvant chemotherapy with PLADO (a combination of cisplatin and doxorubicin).
Figure 1: Contrast-enhanced computed tomography abdomen images (red and blue arrow showing mass)

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  Discussion Top


There have been approximately 200 cases in literature since Becker described PB in 1957.[4] PB accounts for 0.5% of exocrine tumors of the pancreas. There is a male predominance, and more than half of the reported cases are in Asians.[5],[6] The tumor may arise from any portion of the pancreas.

This tumor was divided into two categories by Horie et al.[3] as right-side tumors and left-side tumors. According to Horie et al.,[3] the right-side tumors arise from the primordial ventral pancreas, do not contain calcification and islet cells, are usually well encapsulated, and have a better prognosis. However, the left-side tumors arise from the primordial dorsal pancreas, contain calcification and islet cells, have no encapsulation, and indicate a poorer prognosis than right-side tumors.[3],[7] The tumor in our patient has the features of a left-side tumor because it arose from the body of pancreas with calcification.

The mass is often so large at presentation, and therefore making a determination of the organ of origin is quite difficult. In the series of Montemarano et al.,[8] in only half of the cases did the imaging appearance suggest the pancreas as the organ of origin. These large tumors typically compress the surrounding structures without appearing to invade them, although local invasion may be evident at surgical resection.[8],[9]

The liver is the most common site of metastases; however, regional lymph nodes, peritoneal, bone, pulmonary, and mediastinal metastases have also been described.[10],[11] Vascular invasion is uncommon and ascites may be an indicator of tumor spread.[8] In early childhood, the spectrum of differential diagnosis is wide when it cannot be clearly seen that the tumor arises from the pancreas. The tumors that must be considered first are Wilms' tumor, neuroblastoma, hepatoblastoma, and other primary liver tumors.[5],[12] Squamoid corpuscles are typical in PB, but they do not exist in acinar cell carcinoma.[5] Although there are certain characteristic imaging findings, the diagnosis of PB relies mainly on pathology.

The pathologic features of PB are well described in literature, but few series describe the radiologic features of PB. The radiological features are usually those of well-defined, lobulated, heterogeneous masses, with necrosis and/or calcification, usually arising from the body and/or tail of the pancreas or involving the entire organ, rather than being universally located in the pancreatic head.[13]

The tumor in our case was diagnosed as neuroblastoma with the marker study of the tru-cut biopsy specimen. On complete biopsy specimen, tumor had squamous corpuscles in the histopathology and with different marker study diagnosed as PB. In our view, it was due to scanty tissue in the tru-cut biopsy specimen and may be due to technical errors.

The production of AFP by PB has repeatedly been noted in the literature, both in the form of serum elevation and immunohistochemical detection. AFP may be useful for diagnosis and used as a tumor marker in follow-up after surgery and during chemotherapy.[14]

Complete surgical resection is the treatment most commonly associated with long-term survival of patients with PB. PB, however, is often unresectable at diagnosis.[15] Various regimens of neoadjuvant chemotherapy have been used, aiming at converting an unresectable tumor into a resectable one, but it is unknown whether neoadjuvant chemotherapy for PB ultimately results in an improved survival rate.[16] Currently, there is no proven indication for adjuvant chemotherapy in successfully resected cases of PB, whether primary or recurrent.[16] The chemotherapeutic regimen that was recommended by Défachelles et al.[17] as a neoadjuvant chemotherapy to reduce tumor volume in unresectable cases of PB was cisplatin and doxorubicin. Gemcitabine was reported to be effective in decreasing the size of primary and metastatic tumors of PB to achieve the complete resection.[18] Gemcitabine may be one of the promising second-line chemotherapy regimens for PB.

There are different surgeries to deal with PB in children, but it needs further research for the best surgical technique. The clinical, histological, and therapeutic characteristics of this tumor are hardly known by most pediatric surgeons and oncologists.[20] The ability to resect depends on the local extent of the disease, its location, and obviously the existence or absence of metastatic disease. Resection may involve distal pancreatectomy with or without splenectomy for lesions of the body and tail. Lesions of the head may sometimes require pancreaticoduodenectomy (Whipple procedure) and pancreaticoduodenectomy with portal venous reconstruction using a venous conduit.[19],[21] We left behind some part of the tumor to save the major vascular structures and visceral injuries. We do not have any frozen biopsy facilities at our institute, so we did not perform frozen biopsy at the time of surgery.

We planned to monitor the child closely by history, physical examination, and serial imaging. In addition, serial monitoring of AFP levels may be useful to screen for recurrence. There are no set protocols for follow-up at this time. We have been followed up this patient for 4 months without evidence of regrowth of the residual tumor and any recurrence.


  Conclusion Top


When a large, well-defined, lobulated, heterogeneous mass, with necrosis and/or calcification, is identified in the pancreas of children, the diagnosis of PB should be considered. We report on this rare disease to emphasize its clinical features, radiological findings, laboratory findings, diagnosis, and management.

Acknowledgment

The authors would like to thank Dr. Annu Bhandari, radiologist, senior professor, SMS Medical College, Jaipur, for helping in this manuscript.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
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Horie A, Yano Y, Kotoo Y, Miwa A. Morphogenesis of pancreatoblastoma, infantile carcinoma of the pancreas: Report of two cases. Cancer 1977;39:247-54.  Back to cited text no. 3
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Montemarano H, Lonergan GJ, Bulas DI, Selby DM. Pancreatoblastoma: Imaging findings in 10 patients and review of the literature. Radiology 2000;214:476-82.  Back to cited text no. 8
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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. 12
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Roebuck DJ, Yuen MK, Wong YC, Shing MK, Lee CW, Li CK. Imaging features of pancreatoblastoma. Pediatr Radiol 2001;31:501-6.  Back to cited text no. 13
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Ogawa B, Okinaga K, Obana K, Nakamura K, Hattori T, Ito T, et al. Pancreatoblastoma treated by delayed operation after effective chemotherapy. J Pediatr Surg 2000;35:1663-5.  Back to cited text no. 14
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Klimstra DS, Wenig BM, Adair CF, Heffess CS. Pancreatoblastoma. A clinicopathologic study and review of the literature. Am J Surg Pathol 1995;19:1371-89.  Back to cited text no. 15
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Défachelles AS, Martin De Lassalle E, Boutard P, Nelken B, Schneider P, Patte C. Pancreatoblastoma in childhood: Clinical course and therapeutic management of seven patients. Med Pediatr Oncol 2001;37:47-52.  Back to cited text no. 17
    
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Belletrutti MJ, Bigam D, Bhargava R, Grundy P. Use of gemcitabine with multi-stage surgical resection as successful second-line treatment of metastatic pancreatoblastoma. J Pediatr Hematol Oncol 2013;35:e7-10.  Back to cited text no. 18
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Tanaka H, Kitano Y, Takayasu H, Matuda S, Yamada W. Pancreatoblastoma with portal vein involvement in a child: A case report. 2013;3:44-49.  Back to cited text no. 21
    


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