|Year : 2015 | Volume
| Issue : 2 | Page : 113-115
Solid cystic papillary tumor of the pancreas in an 18-year-old female
Veenu Jain1, Rani Bansal2, Tarun Agarwal3, Yogesh Kumar Yadav1
1 Department of Pathology, Integral Institute of Medical Sciences and Research, Lucknow, Uttar Pradesh, India
2 Department of Pathology, Subharti Institute of Medical Sciences, Meerut, Uttar Pradesh, India
3 Department of Surgery, Career Institute of Medical Sciences and Hospital, Lucknow, Uttar Pradesh, India
|Date of Web Publication||17-Nov-2015|
Yogesh Kumar Yadav
Department of Pathology, 145, A-Block, South City, Rae Barely Road, Lucknow - 226 025, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
Solid cystic papillary tumor is a rare tumor of the pancreas. These are seen more commonly in young females. Preoperative diagnosis is difficult to make due to its similarity with other cystic pancreatic lesions. These tumors carry a favorable prognosis because of their low malignant potential, and have a good prognostic outcome after surgery. Here we report a case of an 18-year-old female patient who presented with a history of pain and lump in epigastrium for 7 months. Diagnostic laparotomy was done that revealed a well-defined cystic mass attached to the body of pancreas. The mass was excised along with distal pancreatic tissue. Splenectomy was also done in the patient. She had no evidence of recurrence for the last 1 year after complete excision of the tumor.
Keywords: Pancreatic tumor, papillary, solid cystic
|How to cite this article:|
Jain V, Bansal R, Agarwal T, Yadav YK. Solid cystic papillary tumor of the pancreas in an 18-year-old female. J Clin Sci 2015;12:113-5
| Introduction|| |
Solid cystic papillary tumor of pancreas was first described by Frantz in 1959. It is also known as solid pseudopapillary neoplasm, solid cystic or papillary cystic tumor, and solid and papillary epithelial neoplasm (SPENP). It is a rare tumor of low malignant potential accounting for 0.17-2.7% of all nonendocrine tumors of pancreas. It is most commonly seen in young females especially those who are in 2nd and 3rd decades of life suggesting role of hormones in its pathogenesis, but only few studies showed estrogen and progesterone receptor positivity of the tumor.
These tumors are misdiagnosed as pancreatic pseudocysts both clinically and radiologically. Hence, all cystic lesions arising from pancreas should be investigated thoroughly.
| Case Report|| |
An 18-year-old female presented with a history of pain and lump in epigastrium for 7 months. The mass had gradually increased in size. On per abdomen examination a tender mass was found moving with change of posture. It was firm-to-hard in consistency, mobile, and disappeared in lying posture. Clinically it was not possible to ascertain the origin of mass. Complete hemogram, liver function test, and renal function test were within normal limits. Ultrasound of the patient revealed a cystic mass arising from pancreas. The patient also underwent exploratory laparotomy. A well-encapsulated mass was seen originating from the body of pancreas. There was no hepatosplenomegaly, free fluid or any other evidence of metastasis. Distal pancreatectomy and splenectomy were done. Proximal pancreatic tissue was buried with suture and adequate hemostasis achieved. Closed abdominal drain was placed and abdomen was closed in layers. The postoperative recovery period of the patient was uneventful.
Specimen consisted of a globular mass of 6 cm × 5 cm × 5 cm with smooth outer surface and a normal looking spleen. Mass was encapsulated and showed areas of congestion. Cut surface showed variegated appearance exhibiting solid brownish hemorrhagic areas, gray white tissue with golden pigment, and central area of cystic degeneration [Figure 1].
|Figure 1: Cut surface of tumor mass is variegated with areas of cystic degeneration|
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Sections revealed encapsulated tumor tissue having large areas of hemorrhage, necrosis, and cystic degeneration. Tumor showed solid, papillary, and cord-like structures of almost uniform small polyhedral cells having round to oval vesicular nuclei and well-defined cytoplasmic boundaries situated around fibrovascular cores [Figure 2]. Intermingled with these were many clusters of large foamy cells. Mitosis was infrequent. Inflammatory response was sparse. Strip of normal pancreatic tissue was seen attached outside tumor capsule. Spleen was unremarkable except for mild red pulp hyperplasia.
|Figure 2: Section reveals papillary structures with definite vascular cores (H and E, 400X)|
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Routine formalin fixed, paraffin embedded sections were also stained by peroxidase technique. Monoclonal antibody to estrogen and progesterone receptor and cytokeratin was used after heat retrieval in pressure cooker in Tris-EDTA (TE) buffer. Positive staining was observed for all three antibodies. For CD10 sections were sent outside and the results obtained were negative.
| Discussion|| |
SPENP is a rare tumor of low malignant potential and accounts only for about 2% of all exocrine pancreatic tumors and 9.3% of cystic pancreatic neoplasms., This tumor is most commonly seen in females with overall average age of 27 years. About 10% of the cases occur in men. The most common clinical symptoms are pain, abdominal fullness, or mass. Our case is that of an 18-year-old female who presented with similar complaints. On radiological examination, it is often difficult to differentiate these tumors from pancreatic pseudocysts. In our case, the presence of pancreatic cyst was confirmed by ultrasonography (USG). On histopathology, SPENP can be differentiated from other cystic pancreatic lesions. Differential diagnoses are intraductal papillary mucinous neoplasm (IPMN), mucinous cystic neoplasm, serous cystic adenoma (SCA), ductal adenocarcinoma, and neuroendocrine tumor. IPMN is very commonly seen in elderly males. These mainly involve the ducts. Cysts are filled with thick mucinous material and are lined by tall columnar cells. Mucinous cystic neoplasm occurs in middle-aged females. The cysts contain thick tenacious mucin and are lined by mucus secreting columnar epithelial cells with ovarian type of stroma. SCA occurs in middle and elderly females. Grossly, cyst contains watery straw colored fluid and are lined by low cuboidal cells. Ductal adenocarcinoma is more common in males and is rarely seen in patients of less than 40 years. These are fast growing tumors that usually present with widespread metastasis having a bad prognosis. Tumor is not well demarcated and cysts are lined by atypical cuboidal cells. Neuroendocrine tumors occur in females of any age group. Here cystic degeneration and hemorrhage are rare., In our case, the tumor was well encapsulated with solid, cystic, and hemorrhagic areas. Cysts contained hemorrhagic and necrotic debris and were lined by monomorphic small polyhedral cells. Typical clinical presentation and histological features of the present case were sufficient to exclude other possible cystic lesions and to reach the final diagnosis of solid cystic papillary tumor of the pancreas.
There is discrepancy in results of immunohistochemical staining of SPENP in past studies. Some authors suggest that these tumors express CD10, ά1-antitrypsin, vimentin, and abnormalities in β-catenin expression. But expression of pancreatic enzymes (like chymotrypsin and islet cell marker), cytokeratin, estrogen receptor (ER), progesterone receptor (PR), CD56, and neuroendocrine markers remain debatable. So line of differentiation of these tumors also remain uncertain. The cell of origin is unknown but some suggest that it is derived from primordial pancreatic cells and thus classified as tumor of indeterminate type. Others suggest that these tumors are hormone dependent so might be derived from genital ridge/ovarian anlage related cells that are attached to pancreatic tissue during early embryogenesis. In the present case, positivity for ER/PR and cytokeratin was seen which supports the theory of hormonal dependence of these tumors.
Surgery remains the mainstay of treatment with excellent outcome after aggressive approach. Aggressive approach is needed as any lesser procedure may result in invasion to porta hepatis, liver, major vascular structures, and viscera. Vascular invasion is indicative of poor prognosis. Commonest site of metastasis is liver. Rarely, lymph nodal and peritoneal spread may occur. In our case, aggressive surgery was done and patient is well till now.
As this case is difficult to diagnose clinically and radiologically, a greater awareness of diagnostic and surgical options for treatment are required for this rare pancreatic tumor, which has a good prognosis if completely excised. Studies suggest that magnetic resonance imaging (MRI) is superior to computed tomography (CT) and other imaging modalities for the characterization of solid cystic papillary tumors of pancreas. Surgery remains the mainstay of treatment even in big size tumors but there are reports that claim the efficacy of chemotherapy and radiotherapy in treatment of these tumors.,,
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[Figure 1], [Figure 2]