|Year : 2013 | Volume
| Issue : 3 | Page : 507-510
Solid pseudo-papillary tumor of pancreas: A rare case report and review of Indian literature
Kanchan Murhekar1, A Seshadri Ramakrishnan2, Balaji Ramani2, B Jayanand Sunil2, Urmila Majhi1
1 Department of Pathology, Cancer Institute (WIA), Adyar, Chennai, India
2 Department of Surgical Oncology, Cancer Institute (WIA), Adyar, Chennai, India
|Date of Web Publication||8-Oct-2013|
Department of Pathology, Cancer Institute (WIA), Adyar, Chennai
Source of Support: None, Conflict of Interest: None
Solid pseudo-papillary tumor (SPT) of the pancreas is a rare pancreatic tumor, well known for its predilection for young women. As the tumor has favorable prognosis, differentiating it from other pancreatic tumors with aggressive behavior is necessary. We present a rare case of SPT and review the literature about SPT cases published from India.
Keywords: Immune-histochemistry, solid pseudo-papillary neoplasms, pancreas
|How to cite this article:|
Murhekar K, Ramakrishnan A S, Ramani B, Sunil B J, Majhi U. Solid pseudo-papillary tumor of pancreas: A rare case report and review of Indian literature. J Can Res Ther 2013;9:507-10
|How to cite this URL:|
Murhekar K, Ramakrishnan A S, Ramani B, Sunil B J, Majhi U. Solid pseudo-papillary tumor of pancreas: A rare case report and review of Indian literature. J Can Res Ther [serial online] 2013 [cited 2020 Feb 26];9:507-10. Available from: http://www.cancerjournal.net/text.asp?2013/9/3/507/119364
| > Introduction|| |
Solid pseudo-papillary tumor (SPT) of the pancreas is a rare exocrine pancreatic tumor, well known for its predilection for young women.  As the tumor has a favorable prognosis, differentiating it from other pancreatic tumors with aggressive behavior is essential. As SPTs are rare, they are often associated with diagnostic and therapeutic challenges. In India, less than 30 cases have been reported in published literature. We present a rare case of SPT and review the literature highlighting the cases published from India.
| > Case Report|| |
A 16-year-old girl was referred to our institute with a history of abdominal pain. On examination, a mass was felt in the epigastric region. Computed tomography (CT) scan showed a tumor arising from the head of pancreas/duodenum. Patient underwent ultrasound-guided biopsy elsewhere, which was reported as "desmoplastic small round cell tumor".
CT angiogram with vascular reconstruction at our institute revealed a large enhancing hetero-dense mass in sub-hepatic portion, probably arising from the head of pancreas. The mass was encircling the duodenum causing mass effect with complete loss of fat planes. The tumor was in close contact with the portal vein/superior mesenteric vein (SMV) for <180°. However, there was no infiltration. The superior mesenteric artery was free. Tumor was also abutting the renal segment of inferior vena cava (IVC), causing severe compression and luminal narrowing in the renal segment of IVC. The mass was supplied by branches arising from the celiac, right, and left hepatic arteries [Figure 1] and [Figure 2].
|Figure 1: Computed tomography abdomen showing hetero-dense mass arising from the head of pancreas|
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Fine-needle aspiration from the mass revealed cells arranged predominantly in pseudo-papillary pattern. The cells were round to cuboidal with characteristic nuclear grooving [Figure 3]. Based on the findings of fine-needle aspiration cytology (FNAC), the diagnosis of solid pseudo-papillary neoplasm of pancreas was made. Patient underwent Whipples pacreatico-duodenectomy. Intra-operatively, there was no evidence of ascites, liver metastasis, para-aortic lymphadenopathy, or peritoneal disease. A well-encapsulated mass was found to arise from the head of the pancreas. The tumor was carefully dissected free from the SMV. Mass was resected in toto. Post-operative histopathological examination was performed and diagnosis of solid pseudo-papillary neoplasm was confirmed [Figure 3] and [Figure 4]. No capsular or vascular invasion was seen and no mitotic figures were noted after extensive study of the specimen. Immunohistochemical study showed positivity for vimentin, progesterone receptor, CD10, and CD56. The MIB1 index was 2-3%.
|Figure 3: (a, b) Tumor showing cells with pseudo-papillary arrangement (H and E, ×20). (c) Smear showing round cells arranged in pseudo-papillary pattern. (d) Tumor cells showing positivity for vimentin|
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|Figure 4: Immuno-histochemistry: Tumor cells showing positivity for (a) CD56, (b) CD10, (c) progesteron receptor, and (d) and KI-67|
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| > Discussion|| |
SPTs are rare tumors with unclear origin and typically occur in young females. Patient often presents with pain in abdomen. Most of these tumors are benign in nature.  However, a small proportion of the cases have malignant potential. According to the WHO classification, SPTs with clear criteria of malignancy such as vascular and nerve sheath invasion, lymph node, or liver metastasis are designated as solid pseudo-papillary carcinomas.  As the splenic vein running across posterosuperior aspect of pancreas is vulnerable for compression or infiltration by the tumor, in rare instances, the tumor causes isolated splenic venous thrombosis resulting in extra-heaptic portal hypertension.
CT scans usually demonstrate a well-encapsulated and circumscribed retroperitoneal mass, hypodense, with various solid and cystic components. Because of its superior contrast resolution, magnetic resonance imaging (MRI) is better than CT for distinguishing certain tissue characteristics, such as hemorrhage, cystic degeneration, or the presence of a capsule.  Angiography usually demonstrates an avascular or hypovascular pancreatic tumor, and may help delineate the mass from adjacent structures. Several researchers advocate pre-operative endoscopic ultrasonography-guided fine-needle aspiration biopsy for pre-operative detection of the tumor.  The characteristic features of the smear include numerous papillary tissue fragments with slender branching fibrovascular stalks, pseudo-rosette formations, and nuclear folds or grooves. However, pre-operative diagnosis is difficult because of similarity of findings among cystic lesions as well as possibility of spread of tumor cells, and many authors do not recommend it.
The histopathological diagnosis of SPT is based on the well-defined solid and cystic structure and characteristic pseudo-papillary features. On gross examination, the tumor is encapsulated. The microscopic features of SPT are solid areas, which alternate with a pseudo-papillary pattern composed of a fibrovascular stalk surrounded by several layers of epithelial cells. Immuno-histochemical studies are useful in confirming the diagnosis. SPTs are typically positive for vimentin, and antitrypsin and are negative for trypsin and chymotrypsin. They may also show focal positivity for neuron-specific enolase (NSE) and cytokeratin. 
The misdiagnosis rate of SPTs is high.  The differential diagnosis of SPT includes adrenal mass, pancreatic endocrine tumor, liver cyst or tumor, or a pseudocyst. We made the diagnosis of SPT on the basis of (1) the typical presentation in a young girl; (2) findings of CT abdomen with angiogram of abdominal aorta, which revealed a hetero-dense mass in sub-hepatic portion probably arising from the head of pancreas; (3) smear cytology, which showed cells arranged predominantly in pseudo-papillary pattern with characteristic nuclear grooving; (4) histopathological examination of the tumor, which showed characteristic pseudo-papillary pattern; and (5) immune-histochemical reaction of tumor cells showing strong positivity to vimentin, NSE, and CD10 and negative reaction to cytokeratin and epithelial membrane antigen (EMA).
Although there has been a steady increase in the number of diagnosed SPT cases globally, in India, only seven case reports describing 26 cases of SPTs are published [Table 1]. ,,,,,, All the cases reported were females, aged between 13 and 50 years. Pain in abdomen and painless abdominal lump were the commonest presenting symptoms. Pre-operative FNAC was conducted among eight cases. Diagnosis of SPT, based on the characteristic smear findings, was made in five cases. All the cases underwent exploratory surgery and the tumor was resected in all but six cases as the tumor mass was infiltrating the IVC. The prognosis of patients who underwent surgery was good.
|Table 1: Clinical and pathological details of the cases of solid pseudo-papillary tumor of pancreas published from India|
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| > Conclusion|| |
Accurate diagnosis of SPT is important as surgical resection of these tumors is curative and has good prognosis. A possibility of SPT should be considered when young female patient presents with a pancreatic mass. CT scan and endosonography are valuable pointers to the pre-operative diagnosis. FNAC is of value in the specific diagnosis of SPT.
| > References|| |
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]