Journal List > Korean J Urol > v.47(11) > 1069797

TOOLS
Kang, Choi, Moon, and Seo: Malignant Peripheral Nerve Sheath Tumor of the Retroperitoneum in Patient with Neurofibromatosis
Case Report
Korean Journal of Urology

Korean Journal of Urology 2006; 47(11): 1240-1242.

Published online: 30 November 2006

DOI: https://doi.org/10.4111/kju.2006.47.11.1240

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

Il Sang Kang, Seung Hwa Choi, Hyung Bae Moon1, Ill Young Seo

Department of Urology, Wonkwang University School of Medicine, Iksan, Korea.

1Department of Pathology, Wonkwang University School of Medicine, Iksan, Korea.

Corresponding author (seraph@wonkwang.ac.kr)

Received 3 July 2006       Accepted 8 August 2006

Copyright © 2006 The Korean Urological Association

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.

Keywords: Malignant peripheral nerve sheath tumors, Retroperitoneal space, Neurofibromatosis

Figures and Tables

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.
kju-47-1240-g001
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).
kju-47-1240-g002

References

1. Pantuck AJ, Barone JG, Amenta PS, Smilow PC, Cummings KB. Diagnosis and management of malignant perirenal schwannoma. Am Surg. 1996. 62:1024–1027.
2. Riccardi VM, Powell PP. Neurofibrosarcoma as a complication of von recklinghausen neurofibromatosis. Neurofibromatosis. 1989. 2:152–165.
3. Peters KM, Gonzalez JA. Malignant peripheral nerve sheath tumor of the scrotum: a case report. J Urol. 1996. 155:649–650.
4. Evans DG, Baser ME, McGaughran J, Sharif S, Howard E, Moran A. Malignant peripheral nerve sheath tumours in neurofibromatosis 1. J Med Genet. 2002. 39:311–314.
5. Ducatman BS, Scheithauer BW, Piepgras DG, Reiman HM, Ilstrup DM. Malignant peripheral nerve sheath tumors. a clinicopathologic study of 120 cases. Cancer. 1986. 57:2006–2021.
6. Enzinger FM, Weiss SW. Soft tissue tumors. 1995. 3rd ed. St. Louis: Mosby;889–928.
7. Das Gupta TK, Brasfield RD. Solitary malignant schwannoma. Ann Surg. 1970. 171:419–428.
8. Wick MR, Swanson PE, Scheithauer BW, Manivel JC. Malignant peripheral nerve sheath tumor: an immunohistochemical study of 62 cases. Am J Clin Pathol. 1987. 87:425–433.
9. Goldman RL, Jones SE, Heusinkveld RS. Combination chemotherapy of metastatic malignant schwannoma with vincristine, adriamycin, cyclophosphamide and imidazole carboxamide: a case report. Cancer. 1977. 39:1955–1958.
10. Rames RA, Smith MT. Malignant peripheral nerve sheath tumor of the prostate: a rare manifestation of neurofibromatosis type I. J Urol. 1999. 162:165–166.
TOOLS
Similar articles