Clinical Course, Genetic, and Immunohistochemical Characterization of Solid Pseudopapillary Tumor of the Pancreas (Frantz Tumors) in a Brazilian Cohort

被引:4
作者
Tostes, Francinne T. [1 ,2 ]
Dutra Cabral de Carvalho, Parisina Fraga [3 ]
Araujo, Raphael L. C. [1 ]
Ribeiro, Rodrigo Chaves [4 ,5 ]
Apodaca-Torrez, Franz Robert [3 ]
Lobo, Edson Jose [3 ]
Diniz Gomes, Diogo Bugano [1 ]
Callegaro-Filho, Donato [1 ]
Schvartsman, Gustavo [1 ]
Moura, Fernando [1 ,2 ]
Schraibman, Vladimir [1 ]
Goldenberg, Alberto [1 ,3 ]
de Lima, Fernanda Teresa [2 ]
Segatelli, Vanderlei [6 ]
Serrano Uson Junior, Pedro Luiz [1 ,2 ]
机构
[1] Hosp Israelita Albert Einstein, Dept Oncol, BR-05652900 Sao Paulo, Brazil
[2] Hosp Israelita Albert Einstein, Ctr Personalized Med, BR-05652900 Sao Paulo, Brazil
[3] Univ Fed Med Estado Sao Paulo, Dept Surg, BR-04023062 Sao Paulo, Brazil
[4] Hosp Canc Barretos Fundacao PIO XII, Dept Pediat Surg, BR-14784370 Barretos, Brazil
[5] Barretos Sch Hlth Sci Dr Paulo Prata FACISB, BR-14784370 Barretos, Brazil
[6] Hosp Israelita Albert Einstein, Dept Pathol, BR-05652900 Sao Paulo, Brazil
关键词
Frantz tumor; solid pseudopapillary pancreatic neoplasm; pancreatic tumor; Li-Fraumeni syndrome; LIVER-TRANSPLANTATION; CASE SERIES; NEOPLASM;
D O I
10.3390/genes13101809
中图分类号
Q3 [遗传学];
学科分类号
071007 ; 090102 ;
摘要
Frantz tumors or solid pseudopapillary pancreatic neoplasm (SPN) are rare exocrine neoplasms that carry a favorable prognosis; they represent up to 3% of all tumors located in the region of the pancreas and have specific age and gender predispositions. In recent years, the rising curve of diagnosis is entitled to the evolution and access of diagnostic imaging. In this paper, we have retrospectively reviewed and described the clinical course of 40 patients with SPN from three institutions in Brazil, who had their diagnosis between 2005 and 2020, and analyzed the clinicopathological, genetic, and surgical aspects of these individuals. In accordance with the literature, most patients were women, 60% with unspecified symptoms at diagnosis, with tumors mainly located in the body and tail of the pancreas, of whom 70% underwent a distal pancreatectomy with sparing splenectomy as a standard procedure, and none of the cases have experienced recurrence to date. Surgery still remains the mainstay of treatment given the low metastatic potential, but more conservative approaches as observed in this cohort are evolving to become the standard of care. Herein, we present an in-depth analysis of cases focusing on the latest literature and report some of the smallest tumor cases in the literature. To our knowledge, this is the first report evaluating germline genetic testing and presenting a case of detected Li-Fraumeni syndrome.
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页数:10
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