Extraskeletal myxoid chondrosarcoma metastasis to a Meckel's diverticulum adenocarcinoma.

Extraskeletal myxoid chondrosarcoma is a rare soft tissue tumour with a high local and distant metastasis rate and limited response to chemotherapy. Meckel's diverticulum is the most frequent congenital anomaly, and it is associated with a considerable risk of malignant transformation. In this case report, we describe a 50-year-old female patient with a history of extraskeletal myxoid chondrosarcoma of the lower limb and metastasis to the forearm who went to the emergency department with abdominal pain. The investigations revealed a caecal volvulus. A lesion in the middle third of the ileum was incidentally discovered and removed during surgery. Pathology examination revealed a Meckel's diverticulum adenocarcinoma, with metastasis of extraskeletal myxoid chondrosarcoma. Resection was complete; however, the patient had diffuse metastatic pulmonary disease and died eight months later due to disease progression. This mechanism of tumour-to-tumour metastasis is described in other locations, but, regarding the Meckel's diverticulum, this is a unique situation, previously unreported in the literature.

Embryonal Sarcoma of the Liver in the Adult: Challenges in the Diagnosis of a Rare Entity.

Introduction: Embryonal sarcoma of the liver (ESL) is a rare neoplasm of the liver occurring mainly in paediatric ages. Making the correct diagnosis can be challenging as the laboratory and radiological findings that are often nonspecific, and the tumour immunophenotype is poorly defined and even somewhat variable. Case Presentation: A large epigastric mass was detected in a computerized tomography scan of a 43-year-old woman presenting with abdominal pain and bloating. The mass was biopsied and submitted to histopathological study. Microscopically the tumour had sarcomatoid features and showed multinucleated cells with periodic acid-Schiff (PAS)-positive globules. Immunostaining revealed positivity for vimentin, CD10, glypican-3, and α1-antitrypsin and negativity for keratins, muscle, adipocytic, and melanocytic differentiation markers. The patient was then submitted to a left hepatectomy with similar histological findings. Discussion: ESL in adults is a rarity and its diagnosis requires the exclusion of other entities. While some microscopic features are very common, they remain nonspecific. The main feature is the presence of multinucleated cells with PAS-positive hyaline globules. While ancillary testing is key, the immunophenotype also lacks specificity and ESL may have variable staining for glypican-3 and epithelial or muscle differentiation markers. Although it has been described for more than 3 decades, the prognosis and optimal treatment are still not well defined, but surgery has yielded favourable results.

Introdução: O sarcoma embrionario do figado (SEF) e uma neoplasia rara do figado que ocorre principalmente em idades pediatricas. Fazer o diagnostico correto pode ser um desafio, uma vez que os achados laboratoriais e radiologicos sao muitas vezes inespecificos e o imunofenotipo desta entidade e mal definido e algo variavel. Apresentação do caso: Foi detetada em tomografia computorizada (CT) abdominal uma massa epigastrica volu-mosa numa mulher de 43 anos apresentando dor abdominal e distensao abdominal. A massa foi biopsada e submetida a estudo histopatologico. Microscopicamente, o tumor tinha caracteristicas sarcomatoides e apresentava celulas multinucleadas com globulos hialinos com positividade para acido periodico Schiff (APS). O estudo imunohistoquimico revelou positividade para vimentina, CD10, glipicano-3 e α1-antitripsina e negatividade para queratinas e marcadores de diferenciacao muscular, adipocitica e melanocitica. Discussão/Conclusão: O SEF no adulto e uma raridade e o seu diagnostico requer a exclusao de outras entidades. Embora algumas caracteristicas microscopicas sejam muito comuns, estas permanecem inespecificas. A principal caracteristica e a presenca de celulas multinucleadas com globulos hialinos positivos para APS. Ainda que o estudo imunohistoquimico seja fundamental, o imunofenotipo tambem carece de especificidade e o SEF pode ter marcacao variavel para glipicano− 3 e marcadores de diferenciacao epitelial ou muscular. Apesar de ter sido descrito ha mais de tres decadas, o prognostico e o tratamento ideal ainda nao estao bem definidos, mas a cirurgia tem apresentado resultados favoraveis.