Sarcoma epitelióide: aspectos clínicos, fatores prognósticos e sobrevida

Nunes, Luiz Fernando; Fiod, Nelson José Jabour; Vasconcelos, Roberto André Torres de; Meohas, Walter; Rezende, José Francisco Neto

doi:10.1590/s0100-69912010000400002

Cited by 6 publications

(3 citation statements)

References 15 publications

(22 reference statements)

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“…É um tumor frequentemente multifocal, com taxas de recidiva que podem atingir os 50% e taxas de metastização de 40%, sendo que as metástases podem aparecer até 20 anos depois do diagnóstico inicial; a metastização pleuro-pulmonar é a mais frequente mas também pode existir envolvimento dos gânglios linfáticos e cerebral. A sobrevida aos 5 anos é de aproximadamente 60-70% 5,56 .…”

Section: Sarcoma Epitelióideunclassified

Sarcomas Cutâneos – Do Diagnóstico Ao Tratamento

Fernandes

Pinto

Moura³

et al. 2013

SPDV

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O diagnóstico dos sarcomas cutâneos é desafiante, devido à complexidade e heterogeneidade deste gru- po de lesões, cuja apresentação nos tecidos superficiais (pele e tecido celular subcutâneo) impõe diagnóstico diferen- cial obrigatório com tumores benignos e processos inflamatórios. Apesar de serem tumores pouco frequentes alguns tipos assumem grande importância por serem localmente invasivos, com elevada morbi-mortalidade associada, taxas de recidiva local e metastização significativas. Os autores apresentam cinco casos clínico-patológicos de sarcomas cutâneos – sarcoma de Kaposi, dermatofibros- sarcoma protuberans, fibroxantoma atípico, leiomiossarcoma e angiossarcoma – e fazem uma breve revisão da literatura sobre os tipos de sarcoma cutâneo com maior interesse na Dermatologia, salientando os aspectos fisiopa- tológicos, clínicos, histopatológicos e terapêuticos particulares.PALAVRAS-CHAVE – Neoplasias da pele; Sarcoma, Sarcoma de Kaposi; Dermatofibrossarcoma protuberans; Fibro- xantoma atípico, Leiomiossarcoma; Angiossarcoma; Lipossarcoma, Neoplasia da bainha do nervo periférico.

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Section: Sarcoma Epitelióideunclassified

Sarcomas Cutâneos – Do Diagnóstico Ao Tratamento

Fernandes

Pinto

Moura³

et al. 2013

SPDV

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“…It predominantly affects the limbs of young individuals and affects the dermis, subcutaneous tissue, or deeper soft tissues. The exact cause of ES is still unknown [3][4][5] . Due to the scarcity of reported cases, limited knowledge exists regarding the clinical presentation, imaging characteristics and optimal management strategies for ES in the context of NF1.…”

Section: Introductionmentioning

confidence: 99%

Uncommon thigh mass in neurofibromatosis type 1: unveiling aggressive epithelioid sarcoma

Gharbi,

Limaiem,

Romdhane

et al. 2024

European Journal of Case Reports in Internal Medicine

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Background: Patients with neurofibromatosis type I (NF1) have an increased risk of developing soft-tissue sarcomas, particularly those related to the nervous system. Epithelioid sarcoma (ES) is an exceptionally rare subtype of soft-tissue sarcoma, with limited knowledge about its clinical presentation and optimal management in NF1. This report aims to provide insights into the characteristics and outcomes of ES in NF1 patients. Case description: A 37-year-old man with a history of NF1 presented with a progressively worsening mass on his right inner thigh. An MRI scan revealed a well-defined tissue mass originating from the adductor magnus muscle, later confirmed as ES through histopathology and immunohistochemistry. Considering poor local and general prognosis, the multidisciplinary team recommended salvage hip disarticulation, however the patient refused and opted for palliative marginal resection to reduce the tumour size. The patient’s condition declined rapidly, and he succumbed six days after the surgery. Conclusion: This case highlights the rarity of ES in NF1 patients and underscores the potential for malignant tumour development in this population. Further research is needed to improve our understanding and management of sarcomas in the context of NF1.

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“…It is reported that about 15%–75% local recurrence and 30%–50% distant metastasis rates, usually to regional lymph nodes and lungs. 5 – 11 …”

Section: Introductionmentioning

confidence: 99%

Epithelioid sarcoma of the parapharyngeal space: A case report

Luo

Zhang

2021

Rare Tumors

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Epithelioid sarcoma (ES) was first described by Enzinger in 1970. It is a rare variant of soft tissue sarcoma with a 5-year overall survival (OS) rate of 50%. Here, we reported a case of epithelioid sarcoma in the parapharyngeal space of an adult, resulting in a favorable prognosis after chemotherapy and radiation therapy. A 34-year-old female who complained of pharynx pain and discomfort was suspected of having a tumor in the right parapharyngeal space by CT scan. Excision biopsy suggested epithelioid sarcoma. Clinical and radiological studies did not reveal tumor distant metastasis in the patient. After excisional biopsy, the patient underwent chemotherapy and external beam radiation treatment. She has remained alive for 2 years and 7 months without recurrence since her last treatment. In this paper, we also provide a detailed review of the role of radiotherapy in the treatment of epithelioid sarcoma in previously reported cases.

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Sarcoma epitelióide: aspectos clínicos, fatores prognósticos e sobrevida

Cited by 6 publications

References 15 publications

Sarcomas Cutâneos – Do Diagnóstico Ao Tratamento

Sarcomas Cutâneos – Do Diagnóstico Ao Tratamento

Uncommon thigh mass in neurofibromatosis type 1: unveiling aggressive epithelioid sarcoma

Epithelioid sarcoma of the parapharyngeal space: A case report

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