Korean J Pain.  2013 Apr;26(2):160-163. 10.3344/kjp.2013.26.2.160.

Concurrence of Malignant Peripheral Nerve Sheath Tumor at the Site of Complex Regional Pain Syndrome Type 1: A Case Report

• Jeong YH
• Choi EJ
• Nahm FS

• KMID: 1978661
• DOI: http://doi.org/10.3344/kjp.2013.26.2.160

Abstract

Malignant peripheral nerve sheath tumors (MPNSTs) are very rare sarcomas derived from various cells in the peripheral nerve sheath. Malignant peripheral nerve sheath tumors have a known association with neurofibromatosis type 1. Diagnosis of MPNSTs is difficult in patients with chronic pain, when MPNST occurs at an overlapping area of chronic pain. Therefore, the diagnosis can be missed unless clinicians pay attention to the possibility of this disease. Here in, we report a case of concurrent malignant peripheral nerve sheath tumor with complex regional pain syndrome type 1. A 44-year female patient, who was diagnosed with complex regional pain syndrome (CRPS) type 1 in her left ankle, visited our clinic because of aggravated pain. The cause of the aggravated pain was revealed as concurrent MPNST in the left common peroneal nerve territory, which overlapped the site of pain from CRPS.

Keyword

complex regional pain syndrome; nerve sheath neoplasm; neurofibromatosis

MeSH Terms

Animals
Ankle
Chronic Pain
Female
Humans
Nerve Sheath Neoplasms
Neurofibromatoses
Neurofibromatosis 1
Peripheral Nerves
Peroneal Nerve
Sarcoma

Figure

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  Fig. 1 Ultrasonographic images: short axis view (A) and long axis view (B). A mass with heterogeneous echogenicity can be found.

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  Fig. 2 Contrast enhanced T1 weighted magnetic resonance images: axial view (A) and coronal view. (B) About 3.0 × 3.1 × 5.2 cm sized mass along the common peroneal nerve, on the distal femur level, can be found.

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  Fig. 3 A 9.5 × 6.0 × 3.5 cm sized surgical specimen consisted of a solid mass measuring 4.0 × 3.5 × 3.5 cm.

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