Clinical investigation of expansive retroperitoneal lesion suggestive of Frantz's tumor: case report and review of literature/Investigacao clinica de lesao expansiva retroperitoneal sugestiva de Tumor de Frantz: relato de caso e revisao de literature

• Published in: Revista de medicina (São Paulo, Brazil) Vol. 100; no. 5; p. 508
• Main Authors: Oliveira Dupin, Thais, Fontes Lelis, Paula, Chaves Moreira, Weber, Oliveira Silveira, Andre Luis de, Castro Nunes, Alan de, Almeida Barbosa, Cirenio de
• Format: Journal Article
• Language: Portuguese
• Published: Universidade de Sao Paulo. Departamento Cientifico da Faculdade de Medicina 01-09-2021
• Subjects: Brasil
• ISSN: 0034-8554
• DOI: 10.11606/issn.1679-9836.v100i5p508-513

Introduction: Pancreatic neoplasms represents 2% of tumors diagnosed in Brazil and are responsible for 4% of deaths due to neoplasms. About 10 to 15% of cases are cystic pancreatic neoplasms. Objective: To present a case of expansive retroperitoneal lesion suggestive of Frantz's Tumor which anatomopathology report showed Mucinous Cystadenocarcinoma. Case report: Female, 30 years old, complaining of representative weight loss and obstructive gastrointestinal symptoms manifested progressively within 6 months. There were no comorbidities. Laboratory tests showed nothing abnormal. The computed tomography showed a complex retroperitoneal cystic expansive lesion in mesogastrium and left hypochondrium, with intimal relationship with body/tail of pancreas. She was submitted to tumor resection and Distal Pancreatectomy. The patient had good postoperative evolution with improvement in gastrointestinal symptoms. Anatomopathological analysis evidenced histology compatible with pancreatic mucinous cystic neoplasm, with mild and moderate epithelial dysplasia and areas of mucinous cystadenocarcinoma, moderately differentiated, with high-grade pleomorphic components. Discussion: The most common subtype of cystic pancreatic neoplasms is the Mucinous Cystadenocarcinoma, which can either be malignant at the moment of diagnosis or benign, with the possibility of malignization. There are other types as Serous Cystadenoma, Mucinous Cystadenoma, Intraductal Papillary Mucinous Neoplasm and Frantz Tumor. This last type presents with slowly growing and unspecific clinical manifestations, being diagnosed frequently when it has bigger dimensions. Conclusion: It was a case of Invasive Mucinous Cystadenocarcinoma with atypical presentation, similar to Frantz tumor. The diagnosis was possible due to the anatomopathological study, that showed to be a fundamental instrument for the differential diagnosis. Keywords: Mucinous cystadenocarcinoma; Pancreatic neoplasms, Retroperitoneal space. Introducao: As neoplasias pancreaticas representam 2% dos canceres diagnosticados no Brasil e sao responsaveis por 4% das mortes por neoplasia. Cerca de 10% a 15% dos casos sao representados por neoplasias pancreaticas cisticas. Objetivo: E apresentado um caso de lesao expansiva retroperitoneal sugestiva de tumor de Frantz cujo laudo anatomopatologico mostrou cistadenocarcinoma mucinoso. Relato de caso: Mulher, 30 anos, com queixa de perda de peso importante e sintomas obstrutivos do trato gastrointestinal com evolujao progressiva em 6 meses. Nao apresentava nenhuma comorbidade. Exames laboratoriais sem alterares. A tomografia computadorizada evidenciou lesao expansiva cistica complexa retroperitoneal no mesogastrio/hipocondrio esquerdo, em intima relajao com o corpo/cauda do pancreas. Foi submetida a ressecjao do tumor com pancreatectomia distal. Paciente apresentou boa evolujao pos-operatoria, com melhora dos sintomas gastrointestinais. A analise anatomopatologica evidenciou histologia compativel com neoplasia cistica mucinosa pancreatica, com displasia epitelial leve a acentuada e areas de cistadenocarcinoma mucinoso, moderadamente diferenciado, de alto grau. Discussao: Das neoplasias pancreaticas cisticas, o subtipo mais comum e o cistoadenocarcinoma mucinoso, que pode ser maligno ou benigno na avaliajao diagnostica e, as vezes, sugerir possibilidade de malignizajao. Existem outros tipos como o cistoadenoma seroso, cistoadenoma mucinoso, neoplasia intraductal mucinosa papilifera e o tumor de Frantz. Esse ultimo apresenta crescimento lento e manifestares clinicas inespecificas, sendo diagnosticado frequentemente quando possui grandes dimensoes. Conclusao: Tratava-se de um caso de cistadenocarcinoma mucinoso invasor de pancreas com apresentajao atipica, semelhante ao Tumor de Frantz. O diagnostico tornou-se possivel atraves do estudo anatomopatologico, mostrando-se fundamental para o diagnostico diferencial. Descritores: Cistadenocarcinoma mucinoso; Neoplasias pancreaticas; Espajo retroperitoneal.