|Year : 2015 | Volume
| Issue : 6 | Page : 789-791
Solid-pseudopapillary tumor of pancreas in a male child: A nuclear feature at light microscopy that can aid in its diagnosis in cases where papillary architecture is not very apparent
Sonali R Saraf, Patil V Milind, Manisha S Khare, Alka D Kalgutkar
Department of Pathology, Lokmanya Tilak Municipal Medical College, Mumbai, Maharashtra, India
|Date of Web Publication||19-Nov-2015|
Sonali R Saraf
Department of Pathology, Lokmanya Tilak Municipal Medical College, Mumbai, Maharashtra
Source of Support: None, Conflict of Interest: None
Solid-pseudopapillary tumor (SPPT) of the pancreas is an uncommon low-grade exocrine pancreatic malignancy. An 11-year-old male child presented with pain in the abdomen. He had a history of fall 1-week back for which a computerized tomography (CT) scan was advised. A pancreatic mass was incidentally detected on CT scan and diagnosed as pancreatic pseudocyst. Explorative surgery was performed that revealed a solid-cystic tumor in the tail of the pancreas. The histopathological examination reported the features of pancreatic SPPT which was also the final diagnosis.
Keywords: Male child; pancreatic pseudocyst; solid pseudopapillary tumor
|How to cite this article:|
Saraf SR, Milind PV, Khare MS, Kalgutkar AD. Solid-pseudopapillary tumor of pancreas in a male child: A nuclear feature at light microscopy that can aid in its diagnosis in cases where papillary architecture is not very apparent. Med J DY Patil Univ 2015;8:789-91
|How to cite this URL:|
Saraf SR, Milind PV, Khare MS, Kalgutkar AD. Solid-pseudopapillary tumor of pancreas in a male child: A nuclear feature at light microscopy that can aid in its diagnosis in cases where papillary architecture is not very apparent. Med J DY Patil Univ [serial online] 2015 [cited 2020 Jan 24];8:789-91. Available from: http://www.mjdrdypu.org/text.asp?2015/8/6/789/169935
| Introduction|| |
There is a variety of cystic neoplasms in the pancreas. These include benign serous cystic neoplasms, benign and malignant mucinous cystic neoplasm, and also benign and malignant forms of intraductal papillary-mucinous neoplasms. On radio-imaging, it is important not to assume that all fluid-filled pancreatic abnormalities represent pseudocysts or that a dilated pancreatic duct represents only chronic pancreatitis. The presence of a solid component in a cystic lesion, septations within the cyst, and the absence of a clinical history of pancreatitis are factors that should alert the surgeon to the possible presence of a neoplasm.
An 11-year-old boy presented with a solid-cystic mass in the pancreas which was later diagnosed as solid-pseudopapillary tumor (SPPT) of pancreas (SPTP). This tumor has a preponderance in adult women and is rarely seen in young males. 
| Case Report|| |
An 11-year-old male child came with complaints of pain in abdomen since 2 days. He had a history of fall 1-week back. Computerized tomography scan of abdomen showed a partially fluid-filled mass in the tail of pancreas-benign tumor of the pancreatic pseudocyst. There was no organomegaly. His laboratory investigations were within normal limits. A partial pancreatectomy with total splenectomy was performed. The cut surface of the pancreas showed a well-defined capsulated mass measuring 6 cm × 5 cm × 4 cm in the tail of the pancreas [Figure 1]. The mass was solid-cystic with areas of hemorrhage and necrosis. The mass was limited to the pancreas. At microscopy, the tumor exhibited a solid monomorphic pattern of cells arranged in sheets and occasional papillae. The tumor cells had eosinophilic cytoplasm with indented nuclei and inconspicuous nucleoli. Considering the age and sex of the proband, the differential diagnosis considered were: Solid-cystic papillary epithelial neoplasm and pancreatic endocrine neoplasm. The tumor cells were positive for CD10, CD56, and vimentin and negative for chromogranin and synaptophysin. Thus, solid-cystic papillary epithelial neoplasm of the pancreas was considered as the final diagnosis. The histopathological features are illustrated below [Figure 2], [Figure 3], [Figure 4] and [Figure 5].
|Figure 1: A well-defined capsulated solid-cystic mass in the tail of pancreas with friable and papillary appearance on the cut surface|
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|Figure 2: H and E section from the tumor showing tumor cells arranged in solid sheets and papillae|
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|Figure 3: H and E section from the tumor showing the bland tumor cells in sheets and fine papillae|
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|Figure 4: H and E section from the tumor showing nuclear grooves and indentations in most of the cells|
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| Discussion|| |
SPPT is the preferred term for a distinctive type of pancreatic tumor also known as papillary and solid epithelial neoplasm, papillary-cystic neoplasm, and cystic-solid papillary carcinoma. SPTP has a tendency to predominantly affect young women aged between 25 and 35 years. It rarely affects males. 
Review of the literature revealed some 24 cases of SPTP reported in children with an average age of 10.8 years (range: 8-16 years) and a male/female ratio of 1:4.75.  Four cases, three girls and a boy of 13-16 years, presented acutely following blunt abdominal trauma in manner similar to our case ],[],[],[ upon whom Whipple procedure was performed in some cases.  Followup of all these cases was uneventful, and the patients did not require any adjuvant chemotherapy.
The differential diagnosis of SPTP includes any solid or cystic pancreatic disease entity such as mucinous cystic tumor, microcystic adenoma, islet cell tumor, cystadenocarcinoma, acinar cell carcinoma, inflammatory pseudocyst, mucus secreting tumor, pancreatoblastoma, and a vascular tumor-like hemangioma. The first four are usually seen in older patients and have no particular gender preponderance.  Pancreatoblastoma is usually found in younger individuals of either sex. Radiologically, a linear sunburst pattern of calcification is the usual finding in microcystic adenoma. A hypervascular pattern on angiography is suggestive of islet cell tumor rather than SPPT. In the present case, the bland monotonous appearance of cells arranged in solid sheets led to the differential diagnosis of pancreatic endocrine neoplasm and SPPT. The papillary arrangement was not very distinct. The round to oval nuclei had finely dispersed chromatin and were often grooved or indented. Mitoses were rare in such cases wherein the papillary architecture is not very apparent, the nuclear grooves and indentations are very characteristic which are seen in SPPT and not in pancreatic endocrine tumor.  This feature is of help in places where immunohistochemistry is not routinely performed. The tumor was negative for chromogranin and synaptophysin (which are specific for pancreatic endocrine tumor) but was positive for CD10, CD56, and vimentin, and thus confirming the diagnosis of SPPT.
Surgery is the mainstay of treatment that is usually curative for localized disease. There are evidence for prolonged survival after adequate surgical resection even with metastases. Even if the disease is extensive at the time of presentation, surgical debulking favors prolonged survival. Intra-operative frozen section may be helpful to ascertain the adequacy of the resection margins. There have been only a few reports regarding the use of radiotherapy or chemotherapy,  so it is difficult to judge the value of such measures.
| Conclusion|| |
SPTP is a rare indolent neoplasm with an unclear origin that typically occurs in young females. The diagnosis depends on histological confirmation, but its appearance on imaging is fairly characteristic. Surgical resection has generally been curative, but close follow-up is recommended, particularly when the histological appearance suggests a more aggressive tumor which is characterized by unequivocal perineural invasion, venous invasion, invasion into surrounding soft tissue, a high nuclear grade, a high mitotic count, and metastasis.  In general, solid-pseudopapillary neoplasm is regarded as a low-grade malignancy which can be completely cured by surgery.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]