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Int J Clin Exp Pathol 2010;3(8):812-814

Case Report
Lymph node metastasis of malignant peripheral nerve sheath tumor in the absence of
widespread disease five years after diagnosis: a rare finding

Gwendolyn J. Godfrey, Hanan Farghaly

Department of Pathology, University of Louisville, Louisville, KY 40202, USA.

Received August 25, 2010; accepted September 21, 2010; available online October 2, 2010

Abstract: Malignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue neoplasm that accounts for less than 10 percent of all soft
tissue sarcomas.  These tumors can arise spontaneously or in association with a neurofibroma in patients with neurofibromatosis type I (NFI).  
Lymph node metastasis from MPNST is rarely seen and has only been reported in the setting of widespread metastasis.  Therefore, regional
lymph node dissections are not routinely performed in the management of MPNST.  To our knowledge, this case is the first reported case of
cutaneous MPNST metastatic to a regional lymph node in the absence of widespread disease.  We report the case of a 49 year old male with a
history of NFI and MPNST involving only the right elbow which was excised with clear margins.  Five years later, he presented with an isolated
11.5 x 9.0 x 7.0 centimeter right axillary mass which was completely excised.   Microscopic examination revealed metastatic MPNST with lymph
node involvement.  Although isolated lymph node metastasis from¬¬ MPNST is rare, this case expands our knowledge of the clinical behavior
of MPNST. (IJCEP1008002).

Keywords: Malignant peripheral nerve sheath tumor, spindle cell neoplasm, Lymph node metastasis, S-100

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Address all correspondence to:
Hanan Farghaly, M.D.
Department of Pathology  
University of Louisville Hospital
530 South Jackson Street, Room COF 19
Louisville, KY 40202
Phone: 502-852-1764
Fax: 502-852-1761
email:
h0farg01@louisville.edu