Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Solid pseudopapillary tumor of the pancreas was studied in a 20-year-old woman and a 54-year-old woman. In the younger patient, the tumor had metastasized to the liver 8 years after distal pancreatectomy. In both neoplasms, the distinct histologic pattern of solid, pseudopapillary, and degenerative cystic areas was present. Analysis by means of immunohistochemistry revealed a diffuse expression for vimentin, neuron-specific enolase, and a focal positivity for al-antitrypsin, whereas epithelial markers were negative in the tumor of the older patient and only focally expressed in the tumor of the younger patient. Immunohistochemical analysis of cell cycle-associated proteins provided an overexpression of cyclin D1 and cyclin D3 in both tumors, although to varying degrees. In addition, the cyclin-dependent kinase inhibitors p21, and to a lesser extent p27, were up-regulated just as mdm2. There was no accumulation of p53 protein, and Ki67-positive cells were extremely scarce. Analysis of the liver metastases showed an immunoreactive profile similar to that of the primary tumor. The results show a deregulation of the cell cycle with overexpression of cell cycle-activating proteins D1 and D3 and a probably counterbalancing upregulation of the cyclin-dependent kinase inhibitors p21 and p27. The findings may explain the low pool of Ki67-reactive tumor cells and the generally good clinical outcome of these tumors. Whether a more profound dysbalance of the cell cycle regulation is responsible for the development of metastatic disease remains to be clarified.
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PMID:Deregulated expression of cell cycle-associated proteins in solid pseudopapillary tumor of the pancreas. 1123 5

Solid pseudopapillary tumor is a rare, indolent neoplasm almost exclusively seen in the pancreas. We describe an unusual case of solid pseudopapillary tumor arising in the greater omentum of a 45-year-old man with subsequent multiple liver metastases and peritoneal dissemination. The patient underwent a total of ten laparotomies and died of unresectable disease 8 years after the initial presentation. Microscopically, the primary tumor and the relapsed tumors consistently exhibited identical growth patterns, which were characterized by solid sheets intermingling with pseudopapillary arrangements of uniformly small cells. Immunohistochemical staining was diffusely positive for vimentin and focally positive for alpha-1-antitrypsin. These features were compatible with those of conventional pancreatic solid pseudopapillary tumors. We also performed quantitative evaluation of Ki-67 immunoreactivity and mitotic figures, which indicated malignant transformation of this extremely rare tumor. This is the first detailed report of solid pseudopapillary tumor arising outside the pancreas complicated by repetitive liver metastases and peritoneal carcinomatosis, suggesting the existence of a more lethal subgroup of tumors.
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PMID:A solid pseudopapillary tumor arising from the greater omentum followed by multiple metastases with increasing malignant potential. 1669 62

Solid pseudopapillary tumor is an unusual primary tumor of the pancreas with a low potential for malignancy and unknown cell origin, seen mostly in young women. Although it is discussed among pancreatic epithelial tumors, many cases do not express cytokeratin but show neuroendocrine differentiation. Three cases (2 female, 1 male, aged 24, 45 and 50 years, respectively) of solid pseudopapillary tumor localized in the pancreas are presented. All cases displayed a well-circumscribed tumor, with an average diameter of 6 cm and a red-brown colored, hemorrhagic, cystic cut surface. Microscopically they were encapsulated with large areas composed of thin papillary formations and solid areas focally. Tumor cells were dyscohesive with small, round- to-oval, central nuclei, and vacuolated, clear or eosinophilic cytoplasm without mitotic activity. NSE, vimentin, synaptophysin, ER, PR, Ki-67, S-100, Pan CK, a1-antitrypsin, a2-antichymotrypsin, and antibodies were used in the immunohistochemical study. Vimentin, synaptophysin, NSE, PR, and a1-antitrypsin showed expression in all cases, while Pan-CK was expressed in two cases. Ki-67 expression was below 1% in all cases. Morphologic features of solid pseudopapillary tumor may be confused with pancreatic endocrine neoplasm and ductal adenocarcinoma. All cases showed features of histiocytic and neuroendocrine differentiation. Epithelial differentiation was identified in two cases. We conclude that immunohistochemistry is incapable of giving additional information for the diagnosis of solid pseudopapillary tumor due to different lines of differentiation of tumor cells. We believe that macroscopic and microscopic features (using hematoxylin and eosin stain) are more important for the diagnosis and differential diagnosis of this tumor.
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PMID:Solid pseudopapillary tumor of the pancreas: emphasis on differential diagnosis from aggressive tumors of the pancreas. 1694 Dec 59

Solid pseudopapillary tumor (SPT) is rare primary tumor of the pancreas with low malignant potential affecting adolescent or young women. Radical surgical resection is the definitive treatment for long-term survival even in the patients with metastases. We report a case of 14-year-old girl who presented with unresectable SPT of the pancreas. She received preoperative chemotherapy with cisplatinum, ifosfamide, etoposide, and vincristine followed by intraoperative radiofrequency ablation of metastatic liver lesions with surgical resection of the primary tumor successfully. We demonstrate that all attempts should be made to resect or ablate the primary as well as the metastatic lesions for long-term survival.
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PMID:Preoperative chemotherapy and intraoperative radiofrequency ablation for unresectable solid pseudopapillary tumor of the pancreas. 1809 Sep 37