Korean J Urol.  2006 Nov;47(11):1240-1242. 10.4111/kju.2006.47.11.1240.

Malignant Peripheral Nerve Sheath Tumor of the Retroperitoneum in Patient with Neurofibromatosis

• Kang IS
• Choi SH
• Moon HB
• Se IY

• KMID: 2294286
• DOI: http://doi.org/10.4111/kju.2006.47.11.1240

Abstract

A malignant peripheral nerve sheath tumor (MPNST), also known as a malignant neurilemmoma or malignant schwannoma, is significantly associated with neurofibromatosis type 1 (NF 1). NF 1 is characterized by multiple dermal neurofibromas, cafe-au-lait spots, MPNST and other abnormalities. MPNST is often metastatic, and is detected in the extremities, frequently in the head and neck, but rarely in the retroperitoneal and pelvic spaces. We experienced a case of MPNST in the retroperitoneal space of a 38-year-old male with NF 1, who also had metastatic lesions of the lung and liver.

Keyword

Malignant peripheral nerve sheath tumors; Retroperitoneal space; Neurofibromatosis

MeSH Terms

Adult
Cafe-au-Lait Spots
Extremities
Head
Humans
Liver
Lung
Male
Neck
Neurilemmoma
Neurofibroma
Neurofibromatoses*
Neurofibromatosis 1
Peripheral Nerves*
Retroperitoneal Space

Figure

• 

  Fig. 1 (A) Computed tomography image of the lung shows a mass in the right lower lung. (B) Contrast-enhanced computed tomography scan of the abdomen shows a large mass in the retroperitoneal space.

• 

  Fig. 2 (A) Gross finding of the tumor specimen shows a white and yellow brown soft mass (15×10cm), with focal necrosis and hemorrhage. (B) A microphotograph shows a fascicular arrangement of the anaplastic spindle cells and hypocellular areas (H&E stain, ×200).

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