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.