Yeungnam Univ J Med.  2019 Jan;36(1):63-66. 10.12701/yujm.2019.00031.

Superficial malignant peripheral nerve sheath tumor from recurrent neurofibroma in the abdominal wall of a patient without neurofibromatosis type 1

• Jung CY
• Bae JM
• Choi JH
• Jung KH

• KMID: 2434102
• DOI: http://doi.org/10.12701/yujm.2019.00031

Abstract

Malignant peripheral nerve sheath tumor (MPNST) is rare, accounting for 5-10% of all soft tissue sarcomas. MPNST is characteristically aggressive and has a poor prognosis. Fifty percent of patients with MPNST have neurofibromatosis type 1 (NF1). NF-associated MPNST occurs more often at younger ages than sporadic MPNST, but the survival difference is controversial. Superficial MPNST from a recurrent neurofibroma is extremely rare and only a limited number of cases have been reported in the literature. Herein, we report an unusual case of superficial MPNST from a recurrent neurofibroma in a patient without NF1.

Keyword

Nerve sheath neoplasm; Neurofibroma; Neurofibrosarcoma; Recurrent neurofibroma; Superficial MPNST

MeSH Terms

Abdominal Wall*
Humans
Nerve Sheath Neoplasms
Neurilemmoma
Neurofibroma*
Neurofibromatoses*
Neurofibromatosis 1*
Neurofibrosarcoma
Peripheral Nerves*
Prognosis
Sarcoma

Figure

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  Fig. 1. Clinical photographs. (A, B) A mass with an old operation scar is present in the abdominal wall.

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  Fig. 2. A computed tomography scan shows and abdominal wall mass (in red circle).

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  Fig. 3. Microscopic findings. The transformation from neurofibroma (left area) to malignant peripheral nerve sheath tumor (right area) is present (hematoxylin and eosin stain, ×100).

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  Fig. 4. Immunochemical findings. The tumor cells are weakly positive for S100 protein (A) and negative for H3K27me3 (B) (immunohistochemial stain, ×200)

Reference

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