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Introduction
Solid-pseudopapillary tumor (SPT) is a very rare primary neoplasm of the pancreas. Franz first described it in 1959. It is usually seen in young females. In spite of possible histological findings of malignancy, SPPT typically shows a benign clinical course and a low malignant potential. The pathogenesis of these tumors is still controversial. It has been suggested that it might originate from ductal and acinar pancreatic cells, endocrine cells or pluripotential stem cells.
Case Report
A 19-year-old female presented with intermittent left abdominal fullness and pain for 50 days. Physical examination revealed an abdominal mass about 11 cm × 9 cm in the left upper quadrant. The mass was smooth-surfaced, firm and non-tender.
Abdominal ultrasonography (USG) and multi-slice computed tomography (MSCT) showed a mass of body and tail of the pancreas. Enhancement CT and 3D reconstruction disclosed a solid mass containing cystic and calcified areas (10 cm × 8 cm× 9 cm in diameter) located on the body and tail of the pancreas. The edge of the solid part in the intracavity was irregular and partly enhanced. The diagnosis showed it was mucinous cystadenoma of the pancreas (Fig. 1, 2).
A solid mass containing cystic and calcified areas (10 cm × 8 cm × 9 cm) located on the body and tail of the pancreas. The edge of the solid part in the intracavity was irregular and partly enhanced.
During the operation, it was clear that a large mass with the appearance similar to a sphere pushed the stomach to the front and left. The mass measuring 14 cm × 12 cm × 12 cm was found attached to the body and tail of the pancreas, which adhered to the posterior surface of the stomach, mesentery blood vessels, spleen and posterior wall of the abdomen (Fig. 3).
It is a round mass. The mass measuring 14 cm × 12 cm × 12 cm was found attached to the body and tail of the pancreas.
From inter-operative frozen sections, there was an indication of papillary-like branching suggestive of a solid-pseudopapillary tumor. On immunohi-stochemical staining, the tumor was strongly positive for α-antichymotrypsin, neuron-specific-enolase, and vimentin, and negative for chromogranin and cytokeratin (indicating a low potential malignancy). A distal pancreatectomy and splenectomy were performed (Fig. 4, 5).
There was an indication of papillary like branching suggestive of a solid pseudopapillary tumor. The tumor was strongly positive for α-antichymotrypsin, neuron-specific-enolase, and vimentin, and negative for chromogranin and cytokeratin (indicating a low potential malignancy).
Discussion
A SPT is a very rare entity that was first described by Frantz in 1959. SPT has been classified as a borderline tumor of the pancreas by WHO, 1996. It has a reported incidence of 0.2% to 2.7% of all pancreatic tumors[1]. It was defined as the solid tumor having pseudopapilla with a malignant potential in the year 2000. About 90% of the patients are females with a mean age of 22 years. This tumor can be found at any pancreatic site but is mostly the tail of the body of pancreas. It rarely occurs in sites other than the pancreas[2]. The diameter of the tumor usually is between 0.5 cm to 34.5 cm. Clinically, patients with SPT of the pancreas usually are asymptomatic or gradually develop abdominal symptoms with a feeling of fullness discomfort, or pain[3]. With swelling of the tumor, a symptom of a compressed stomach and intestine may ensue. Some of the patients will be anaemic as result of a large tumor with intra-abdominal hemorrhage. Also, an acute abdomen or shock may develop and a mass may be palpable on the upper abdomen[4]. Some cases had metastasis[5]. Abdominal ultrasound and CT show a well encapsulated, complex mass with both solid and cystic components and displacement of nearby structures. There may be calcifications at the periphery of the mass and intravenous contrast enhancement inside the mass suggesting hemorrhagic necrosis. Although the clinical location can be ascertained, it is difficult to produce a qualitative diagnosis. Abdominal B-ultrasound, CT and endoscopic ultrasound and tumor biopsy have gradually become the effective methods for preoperative diagnosis of a pancreatic tumor. There is a significant difference in the cytopathologic results between SPT and intra-operative fine needle aspirations. The differential diagnosis can be deemed the reliable evidence for diagnosing this pancreatic tumor.
Surgery is the treatment of choice. Complete resection of the tumor with distal pancreatectomy or pancreaticoduodenectomy is usually achieved. It is unnecessary to extensively scavenge the lymph nodes. There has been an uncertainty concerning the efficiency of radiotherapy or chemotherapy[6,7].
- Received August 25, 2008.
- Accepted January 7, 2009.
- Copyright © 2009 by Tianjin Medical University Cancer Institute & Hospital and Springer
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