Epithelioid sarcoma of the upper limb with nine years of evolution

Bittencourt, Maraya de Jesus Semblano; Lima, Caren dos Santos; Dias, Aline de Lima; Neri, Camilla Corrêa

doi:10.1016/j.abd.2020.06.007

Cited by 1 publication

(1 citation statement)

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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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Section: Introductionmentioning

confidence: 99%