Superficial malignant peripheral nerve sheath tumor with overlying intradermal melanocytic nevus mimicking spindle cell melanoma
Christopher R. Jackson
Department of Pathology, VCU School of Medicine, Richmond, VA, USA
Search for more papers by this authorEugen C. Minca
Robert J. Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH, USA
Search for more papers by this authorJyoti P. Kapil
Department of Pathology, VCU School of Medicine, Richmond, VA, USA
Search for more papers by this authorCorresponding Author
Steven Christopher Smith
Department of Pathology, VCU School of Medicine, Richmond, VA, USA
Steven Christopher Smith, MD, PhD,
Department of Pathology, Virginia Commonwealth University School of Medicine, 1200 E Marshall Street Gateway 6–205, PO Box 980662, Richmond, VA 23226, USA
Tel: +804 828 4918
Fax: +804 828 8733
e-mail: [email protected]
Search for more papers by this authorSteven D. Billings
Robert J. Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH, USA
Search for more papers by this authorChristopher R. Jackson
Department of Pathology, VCU School of Medicine, Richmond, VA, USA
Search for more papers by this authorEugen C. Minca
Robert J. Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH, USA
Search for more papers by this authorJyoti P. Kapil
Department of Pathology, VCU School of Medicine, Richmond, VA, USA
Search for more papers by this authorCorresponding Author
Steven Christopher Smith
Department of Pathology, VCU School of Medicine, Richmond, VA, USA
Steven Christopher Smith, MD, PhD,
Department of Pathology, Virginia Commonwealth University School of Medicine, 1200 E Marshall Street Gateway 6–205, PO Box 980662, Richmond, VA 23226, USA
Tel: +804 828 4918
Fax: +804 828 8733
e-mail: [email protected]
Search for more papers by this authorSteven D. Billings
Robert J. Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH, USA
Search for more papers by this authorAbstract
Malignant peripheral nerve sheath tumors are rare soft tissue sarcomas with histological and immunohistochemical similarities to spindle cell melanoma. Although spindle cell melanoma is significantly more common, both tumors may express S100 and lack staining for HMB-45, Melan-A or MITF. Here we present a case of superficial malignant peripheral nerve sheath tumor with diffuse S100 positivity arising in a subtle neurofibroma in close proximity to an intradermal melanocytic nevus. This configuration had led to prior misdiagnosis as a desmoplastic melanoma arising in the nevus and to sentinel lymph node biopsy. Identification of the background neurofibroma, as well as CD34 positivity raised consideration of a low grade malignant peripheral nerve sheath tumor, which was confirmed via observation of Schwannian differentiation on electron microscopy. The importance of distinguishing these two tumors is stressed owing to the difference in management.
References
- 1Pizzo PA, Poplack DG. Principles and Practice of Pediatric Oncology, 6th ed. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins, 2010.
- 2Ducatman BS, Scheithauer BW, Piepgras DG, Reiman HM, Ilstrup DM. Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases. Cancer 1986; 57: 2006.
10.1002/1097-0142(19860515)57:10<2006::AID-CNCR2820571022>3.0.CO;2-6 CAS PubMed Web of Science® Google Scholar
- 3Panigrahi S, Mishra SS, Das S, Dhir MK. Primary malignant peripheral nerve sheath tumor at unusual location. J Neurosci Rural Pract 2013; 4(Suppl 1): S83.
- 4Woodruff JM. Pathology of tumors of the peripheral nerve sheath in type 1 neurofibromatosis. Am J Med Genet 1999; 89: 23.
10.1002/(SICI)1096-8628(19990326)89:1<23::AID-AJMG6>3.0.CO;2-# CAS PubMed Web of Science® Google Scholar
- 5Carroll S. Molecular mechanisms promoting the pathogenesis of Schwann cell neoplasms. Acta Neuropathol 2012; 123: 321.
- 6Rodriguez FJ, Folpe AL, Giannini C, Perry A. Pathology of peripheral nerve sheath tumors: diagnostic overview and update on selected diagnostic problems. Acta Neuropathol 2012; 123: 295.
- 7Guo A, Liu A, Wei L, Song X. Malignant peripheral nerve sheath tumors: differentiation patterns and immunohistochemical features - a mini-review and our new findings. J Cancer 2012; 3: 303.
- 8Allison KH, Patel RM, Goldblum JR, Rubin BP. Superficial malignant peripheral nerve sheath tumor a rare and challenging diagnosis. Am J Clin Pathol 2005; 124: 685.
- 9George E, Swanson PE, Wick MR. Malignant peripheral nerve sheath tumors of the skin. Am J Dermatopathol 1989; 11: 213.
- 10Carlson JA, Dickersin GR, Sober AJ, Barnhill RL. Desmoplastic neurotropic melanoma. A clinicopathologic analysis of 28 cases. Cancer 1995; 75: 478.
10.1002/1097-0142(19950115)75:2<478::AID-CNCR2820750211>3.0.CO;2-O CAS PubMed Web of Science® Google Scholar
- 11Busam KJ, Mujumdar U, Hummer AJ, et al. Cutaneous desmoplastic melanoma: reappraisal of morphologic heterogeneity and prognostic factors. Am J Surg Pathol 2004; 28: 1518.
- 12Hornick JL. Practical Soft Tissue Pathology: A Diagnostic Approach. A Volume in the Pattern Recognition Series. Philadelphia: Elsevier Health Sciences, 2013.
- 13Luzar B, Shanesmith R, Ramakrishnan R, Fisher C, Calonje E. Cutaneous epithelioid malignant peripheral nerve sheath tumour: a clinicopathological analysis of 11 cases. Histopathology 2016; 68: 286.
- 14Jo VY, Fletcher CD. Epithelioid malignant peripheral nerve sheath tumor: clinicopathologic analysis of 63 cases. Am J Surg Pathol 2015; 39: 673.
- 15Laskin WB, Weiss SW, Bratthauer GL. Epithelioid variant of malignant peripheral nerve sheath tumor (malignant epithelioid schwannoma). Am J Surg Pathol 1991; 15: 1136.
- 16Schaefer IM, Fletcher CD. Malignant peripheral nerve sheath tumor (MPNST) arising in diffuse-type neurofibroma: clinicopathologic characterization in a series of 9 cases. Am J Surg Pathol 2015; 39: 1234.
- 17Yeh I, McCalmont TH. Distinguishing neurofibroma from desmoplastic melanoma: the value of the CD34 fingerprint. J Cutan Pathol 2011; 38: 625.
- 18Mohamed A, Gonzalez RS, Lawson D, Wang J, Cohen C. SOX10 expression in malignant melanoma, carcinoma, and normal tissues. Appl Immunohistochem Mol Morphol 2013; 21: 506.
- 19Kang Y, Pekmezci M, Folpe AL, Ersen A, Horvai AE. Diagnostic utility of SOX10 to distinguish malignant peripheral nerve sheath tumor from synovial sarcoma, including intraneural synovial sarcoma. Mod Pathol 2014; 27: 55.
- 20Schaefer I-M, Fletcher CD, Hornick JL. Loss of H3K27 trimethylation distinguishes malignant peripheral nerve sheath tumors from histologic mimics. Mod Pathol 2016; 29: 4.
- 21Lan H, Thanh T, Chen SJ, et al. Expression of the p40 isoform of p63 has high specificity for cutaneous sarcomatoid squamous cell carcinoma. J Cutan Pathol 2014; 41: 831.
- 22McCalmont TH, Emanuel B, Fung M, Ruben BS. Perineuriomatous melanocytic nevi. J Cutan Pathol 2011; 38: 940.
- 23Pasquali S, Haydu LE, Scolyer RA, et al. The importance of adequate primary tumor excision margins and sentinel node biopsy in achieving optimal locoregional control for patients with thick primary melanomas. Ann Surg 2013; 258: 152.
- 24Weissinger SE, Keil P, Silvers DN, et al. A diagnostic algorithm to distinguish desmoplastic from spindle cell melanoma. Mod Pathol 2014; 27: 524.
- 25Kaplan HG. Vemurafenib treatment of BRAF V600E-mutated malignant peripheral nerve sheath tumor. J Natl Compr Canc Netw 2013; 11: 1466.
- 26Feng Z, Wu X, Chen V, Velie E, Zhang Z. Incidence and survival of desmoplastic melanoma in the United States, 1992–2007. J Cutan Pathol 2011; 38: 616.
- 27Dabski C, Reiman HM Jr, Muller SA. Neurofibrosarcoma of skin and subcutaneous tissues. Mayo Clin Proc 1990; 65: 164.
- 28Feng C-J, Ma H, Liao W-C. Superficial or cutaneous malignant peripheral nerve sheath tumor—clinical experience at Taipei veterans general hospital. Ann Plast Surg 2015; 74: S85.