TITLE:
Malignant Peripheral Nerve Sheath Tumor of the Thigh Invading the Superficial Femoral Artery, with Necrotic Lung Metastases as Presenting Symptoms
AUTHORS:
Patrick Mailleux, François Buche, Geoffrey Colin
KEYWORDS:
Necrotic Metastasis, Malignant Peripheral Nerve Sheath Tumor, NF1, Lung Adenocarcinoma, 18F-FDG PET/CT, Unknown Primary Tumor
JOURNAL NAME:
Advances in Lung Cancer,
Vol.9 No.2,
May
6,
2020
ABSTRACT: A NF1 (neurofibromatosis 1) patient developed multiple necrotic lung metastasesfrom a sciatic malignant peripheral nerve sheath tumor (MPNST) invading the superficial femoral artery. The first diagnosis was metastases of a non-small-cell adenocarcinoma because the right calf MPNST was not clinically noticeable ant that the chest/abdomen PET/CT did not include the region of the legs. When the MPNST was diagnosed, new histological analysis on the metastases changed the diagnosis to that of epithelioid undifferentiated sarcoma.The article deals with the sometimes-delayed diagnosis in those NF1 patients with large palpable masses and chronic pain pre-existing the malignant transformation, and discusses the difficulty of the biopsy of necrotic metastases.