TY - JOUR
T1 - A small intraneural epithelioid malignant peripheral nerve sheath tumour of the median nerve simulating a benign lesion. Description of a case and review of the literature
AU - Corradi, Domenico
AU - Alquati, Sara
AU - Bertoni, Franco
AU - Bartoli, Veronica
AU - Dei Tos, Angelo Paolo
AU - Wenger, Doris
AU - Giannini, Caterina
N1 - Copyright:
Copyright 2012 Elsevier B.V., All rights reserved.
PY - 2012/1
Y1 - 2012/1
N2 - The epithelioid variant of malignant peripheral nerve sheath tumours (MPNSTs) is a very rare malignancy. We describe the case of a 30-year-old man complaining of acute pain in his right elbow, mild distal paraesthesias, and some motor deficiencies. He was discovered as having a small fusiform swelling of the median nerve. In view of its very small size, shape, and nonspecific MRI signal, it had initially suggested a benign lesion. The diagnosis of epithelioid MPNST was made only at the histopathological examination. This malignant neoplasm recurred locally fourteen months after surgery. In addition to describe the above very rare case, we have reviewed the literature on epithelioid MPNSTs clearly involving deep major nerve trunks. This case serves as a warning that, even in major nerve trunks, tiny lesions may in reality be early intraneural MPNSTs which, due to their deep location, must be treated adequately with wide margin surgery since the resection margin status represents one of the major parameters influencing the local control of disease and its clinical outcome.
AB - The epithelioid variant of malignant peripheral nerve sheath tumours (MPNSTs) is a very rare malignancy. We describe the case of a 30-year-old man complaining of acute pain in his right elbow, mild distal paraesthesias, and some motor deficiencies. He was discovered as having a small fusiform swelling of the median nerve. In view of its very small size, shape, and nonspecific MRI signal, it had initially suggested a benign lesion. The diagnosis of epithelioid MPNST was made only at the histopathological examination. This malignant neoplasm recurred locally fourteen months after surgery. In addition to describe the above very rare case, we have reviewed the literature on epithelioid MPNSTs clearly involving deep major nerve trunks. This case serves as a warning that, even in major nerve trunks, tiny lesions may in reality be early intraneural MPNSTs which, due to their deep location, must be treated adequately with wide margin surgery since the resection margin status represents one of the major parameters influencing the local control of disease and its clinical outcome.
KW - Epithelioid variant
KW - Immunohistochemistry
KW - Malignant peripheral nerve sheath tumour
KW - Outcome
KW - Surgical margins
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U2 - 10.1007/s12253-010-9348-5
DO - 10.1007/s12253-010-9348-5
M3 - Article
C2 - 21197591
AN - SCOPUS:84855268369
SN - 1219-4956
VL - 18
SP - 101
EP - 106
JO - Pathology and Oncology Research
JF - Pathology and Oncology Research
IS - 1
ER -