Korean J Dermatol.  2006 Jan;44(1):75-78.

A Case of Malignant Peripheral Nerve Sheath Tumor Arising from Neurofibromatosis during Pregnancy

• Kim HD
• Shin HJ
• Park YL
• Whang KU
• Baik SH
• Park JS

Abstract

Malignant Peripheral Nerve Sheath Tumor is a neoplasm that most often arises in anatomically discernible peripheral nerves or neurofibroma. This rare tumor usually has a strong association with neurofibromatosis type 1. Nearly half of these malignant tumors arise in individuals affected with neurofibromatosis type 1. Neurofibromas often first appear around the time of puberty, increase in size and number during pregnancy, and shrink after giving birth. Because growth hormone (GH) concentration increases during puberty and pregnancy, it is possible that GH influences the growth of these tumors. A 33-year-old pregnant woman presented with a 4-month history of a painful, huge mass growing on the right breast. She suffered from neurofibromatosis type 1, with multiple cafe au lait spots and neurofibromas. The clinical and histopathological findings showed a malignant peripheral nerve sheath tumor. We report a case of malignant peripheral nerve sheath tumor on the right breast in a pregnant woman with neurofibromatosis type 1.

Keyword

Malignant peripheral nerve sheath tumor; Pregnancy; Neurofibromatosis type 1

MeSH Terms

Adolescent
Adult
Breast
Cafe-au-Lait Spots
Female
Growth Hormone
Humans
Neurofibroma
Neurofibromatoses*
Neurofibromatosis 1
Parturition
Peripheral Nerves*
Pregnancy*
Pregnant Women
Puberty
Growth Hormone