Giant Sporadic Low Grade Malignant Peripheral Nerve Sheath (MPNST) of Left Thigh

Abstract

Malignant Peripheral Nerve Sheath Tumours (MPNST) are rare spindle-cell sarcomas which are derived from the schwann cells or the pleuripotent cells of the neural crest. MPNSTs are usually located in the peripheral nerve trunk roots, extremities and in the head and neck region. These tumours may arise

Malignant Peripheral Nerve Sheath Tumor of the Forehead

“…They supposed that gene expression profiling was a possible biomarker and/or a therapeutic target for the management of MPNSTs and that it could be used as a main analytic instrument. 1,13 In the present case, a morphodiagnosis of an MPNST was made with the help of the hypocellular and hypercellular areas with the characteristic nerve-like whorls of the tumor tissues and fascicles and also the nuclear criteria of the tumor cells. The particular mitotic figures and the tumor necrosis favored its low-grade nature, and the size of the tumor suggested its giant type.…”

“…In excess of 50% necrosis with exceeding 5 mitotic figures each 10 highpower field has been thought as indicative of a high-grade tumor. 1,[9][10][11] In our patient, the MPNST was of low grade considering, the 1 to 3 mitotic figures with 10% of tumor necrosis.…”

“…[1][2][3][4][5] The estimated frequency of MPNSTs in the population is 0.001%; on the other hand, it can increase from 2 to 5% to 42% in patients with neurofibromatosis type 1 (NF1) with an aggressive course. 3 The highest incidences of these tumors occur about the ages of 20 to 50 years.…”

“…3 The highest incidences of these tumors occur about the ages of 20 to 50 years. 1 Malignant peripheral nerve sheath tumors derive from the peripheral nerve root trunk, extremities, and the head and neck area. 6 Malignant peripheral nerve sheath tumor is extremely occasional tumor with an incidence of 1 per 100,000 population, which is between 3% and 10% of all the soft tissue sarcomas.…”

“…So, this entity is often managed as a subgroup of soft tissue sarcomas. 1,7 Here, we are reporting a case of a sporadic, malignant, peripheral nerve sheath tumor of the forehead in a 65-year-old man. In our case, there was no clinical sign of NF1, and NF1-independent MPNSTs are extremely infrequent.…”