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E-IJD CORRESPONDENCE
Year : 2014  |  Volume : 59  |  Issue : 6  |  Page : 635
Lymph node metastasis of a malignant peripheral nerve sheath tumor without distant metastasis


Department of Dermatology, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, Kyoto, Japan

Date of Web Publication30-Oct-2014

Correspondence Address:
Jun Asai
Department of Dermatology, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, Kyoto
Japan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.143604

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How to cite this article:
Morimoto A, Asai J, Wakabayashi Y, Tashima S, Wada M, Iida S, Komori S, Hanada K, Takenaka H, Katoh N. Lymph node metastasis of a malignant peripheral nerve sheath tumor without distant metastasis. Indian J Dermatol 2014;59:635

How to cite this URL:
Morimoto A, Asai J, Wakabayashi Y, Tashima S, Wada M, Iida S, Komori S, Hanada K, Takenaka H, Katoh N. Lymph node metastasis of a malignant peripheral nerve sheath tumor without distant metastasis. Indian J Dermatol [serial online] 2014 [cited 2020 Aug 15];59:635. Available from: http://www.e-ijd.org/text.asp?2014/59/6/635/143604


Sir,

Malignant peripheral nerve sheath tumors (MPNSTs) are thought to arise from Schwann cells in large peripheral nerves. [1] MPNSTs typically spread via perineural invasion or through hematogenous routes. [2] Consequently, lymph node metastasis is rare, occurring in less than 10% of patients, and is primarily seen in conjunction with widespread metastasis. [1] We report here an extremely rare case of MPNST with lymph node metastasis in the absence of distant metastasis.

A 58-year-old woman with a history of neurofibromatosis type I presented with a cutaneous nodule on her left upper back. Gross examination revealed a 13 × 12 cm firm nodule with ulceration [Figure 1]a. Computed tomography scans revealed no evidence of lymph node swelling or distant metastases. A primary cutaneous MPNST was highly suspected, and a nodule was excised with a 5-cm margin. On microscopic examination, a hypercellular neoplasm consisting of irregular, buckle-shaped spindle cells was located extending from the dermis to the subcutaneous adipose tissue [Figure 1]b.

Mitoses were observed in 9 per 10 high-power fields. The MIB-1 index was 16.2%. The cells were focally positive for S-100 [Figure 1]c. At a peripheral area of the nodule, a hypocellular spindle-cell neoplasm with no mitotic figures was observed, consistent with neurofibroma. A diagnosis of MPNST arising from a neurofibroma was made. The surgical margin was negative for MPNST.
Figure 1: Clinical presentation of the primary lesion (a) A reddish brown, dome-shaped, 13 × 12 cm fi rm nodule with erosion was observed on the right back (b) The cells were hypercellular, irregular, buckled, and spindle-shaped (H and E, ×200) (c) The tumor cells were positive for S-100 protein (Streptavidin-peroxidase; original magnifi cation, ×200)

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Eight months after the operation, the patient presented with a 7 × 4 cm subcutaneous nodule on the left axilla and underwent surgical excision. Microscopic examination revealed a subcutaneous nodule composed of hypercellular, spindle-shaped malignant cells similar to those of the previous tumor in the back. Three years later, the patient presented with 3 × 2 cm subcutaneous nodule on the right axilla and underwent surgical excision. Microscopic examination revealed a similar malignant neoplasm within a lymph node [Figure 2]a and b.
Figure 2: (a) Metastatic tumor widely observed in the lymph node (b) Cells are similar to those of the primary malignant peripheral nerve sheath tumor [H and E, ×20 (a), ×200 (b)]

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The cells were positive for S-100. Collectively, these observations were consistent with lymph node metastasis of MPNST. Computed tomography scans revealed no evidence for local recurrence or metastases 14 months after the last operation.

Lymph node metastasis of MPNST in the absence of widespread disease is thought to be extremely rare. To our knowledge, the current report is only the second one in the literature to report its occurrence, [3] and this indicates that our case of MPNST with regional lymph node metastasis is quite uncommon. Generally, the prognosis of MPNST with distant metastasis is poor. [4] However, in the present case, no distant metastasis has developed in the 6 years since the first operation, suggesting the prognosis of isolated lymph node metastasis from MPNST should be compared to that of distant metastasis. In conclusion, this case may present a new clinical subtype of MPNST. To further our knowledge, the reporting of similar cases is desirable.

 
   References Top

1.Weiss SW, Goldblum JR. Enzinger and Weiss′s Soft Tissue Tumors. 5 th ed. St. Louis: Mosby; 2008. p. 903-7.  Back to cited text no. 1
    
2.Stone JA, Cooper H, Castillo M, Mukherji SK. Malignant schwannoma of the trigeminal nerve. AJNR Am J Neuroradiol 2001;22:505-7.  Back to cited text no. 2
    
3.Godfrey GJ, Farghaly H. Lymph node metastasis of malignant peripheral nerve sheath tumor in the absence of widespread disease five year after: A rare finding. Int J Clin Exp Pathol 2010;3:812-4.  Back to cited text no. 3
    
4.Wong WW, Hirose T, Sceithauer BW, Schild SE, Gunderson LL. Malignant peripheral nerve sheath tumor: Analysis of treatment outcome. Int J Radiat Oncol Biol Phys 1998;42:351-60.  Back to cited text no. 4
    


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