A Case of Malignant Peripheral Nerve Sheath Tumor with Neurofibromatosis Type 1

Choi, Sang Kyu; Kim, Cheol Keun; Kim, Soon Heum; Jo, Dong In

doi:10.15596/arms.2017.26.1.23

Cited by 1 publication

(1 citation statement)

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“…Choi et al presented a case of a patient aged 54 years with a history of NF1 with an MPNST of the upper arm and forearm. Debulking surgery was done because the tumour was not encapsulated, and the remaining defect was reconstructed with local advancement flap 10…”

Section: Discussionmentioning

confidence: 99%

Arm malignant peripheral nerve sheath tumour: a rare clinical presentation

Nascimento¹,

Valença‐Filipe

Horta

et al. 2019

BMJ Case Rep

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Soft tissue sarcomas account for about 1% of malignant tumours. More than 50 subtypes of these tumours have been described with some being extremely rare, namely malignant peripheral nerve sheath tumours (MPNST). The authors present a case of a man aged 81 years with a medical history of an adenocarcinoma of the rectum, which was referred to our clinic due to a growing painless mass on the right arm. An MRI showed a well-delimited encapsulated mass involving the long head of the biceps muscle. Biopsy findings revealed a spindle cell tumour with cytonuclear pleomorphism. The patient underwent wide tumour excision and was successfully reconstructed with a latissimus dorsi functional muscle transfer. The presence of two pulmonary nodules on CT scan staging implied a lung biopsy that showed rectum primary tumour metastases. With these additional findings, the pathology department reassessed the case and reclassified the arm tumour as an MPNST, synchronous with pulmonary adenocarcinoma metastases of the rectum.

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Section: Discussionmentioning

confidence: 99%