Ann Dermatol.  2009 Nov;21(4):402-405. 10.5021/ad.2009.21.4.402.

Cutaneous Plexiform Schwannomas in a Patient with Neurofibromatosis Type 2

• Ko JY
• Kim JE
• Kim YH
• Ro YS

• KMID: 2156506
• DOI: http://doi.org/10.5021/ad.2009.21.4.402

Abstract

Plexiform schwannoma is a rare benign neoplasm of the neural sheath characterized by a multinodular plexiform growth pattern. The tumor usually occurs as an isolated finding, although rare cases have been reported in association with neurofibromatosis type 2 (NF2). A 25-year-old man was admitted for foot drop. He had an asymptomatic skin-colored nodule on his neck that had been present for 10 years. His medical history included local excision of a plexiform schwannoma on his left leg in our dermatology clinic 6 years prior. A histopathological examination of the skin-colored nodule also showed the typical microscopic features of a plexiform schwannoma, including the characteristic Antoni type A areas showing frequent nuclear palisading and Verocay bodies. Magnetic resonance imaging revealed a meningioma and a vestibular schwannoma in the cranium and multiple neurofibromas on the spinal cord. Herein we report a rare case of cutaneous plexiform schwannomas in a patient with NF2.

Keyword

Neurofibromatosis type 2; Plexiform; Schwannoma

MeSH Terms

Adult
Dermatology
Foot
Humans
Leg
Magnetic Resonance Imaging
Meningioma
Neck
Neurilemmoma
Neurofibromatoses
Neurofibromatosis 2
Neuroma, Acoustic
Skull
Spinal Cord

Figure

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  Fig. 1 (A) Skin-colored movable nodule on the right side of the neck, (B) a brown tumor on the left calf.

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  Fig. 2 (A) Multiple lobulated nodules in the dermis (H&E, Scanning view), (B) Antoni type A areas showing frequent nuclear palisading and Verocay bodies (H&E, ×400), (C) tumor nodules strongly positive for S-100 protein (S-100, ×40), (D) tumor nodules negative for neurofilament (Neurofilament, ×40).

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  Fig. 3 MRI view, (A) vestibular schwannoma in the left internal acoustic canal, (B) meningioma involving the vertex of the superior sagittal sinus, (C) multiple neurofibromas from vertebral levels T11 to L4.

Reference

1. Kao GF, Laskin WB, Olsen TG. Solitary cutaneous plexiform neurilemmoma (schwannoma): a clinicopathologic, immunohistochemical, and ultrastructural study of 11 cases. Mod Pathol. 1989. 2:20–26.

2. Sheikh S, Gomes M, Montgomery E. Multiple plexiform schwannomas in a patient with neurofibromatosis. J Thorac Cardiovasc Surg. 1998. 115:240–242.

3. Lim HS, Jung J, Chung KY. Neurofibromatosis type 2 with multiple plexiform schwannomas. Int J Dermatol. 2004. 43:336–340.

4. Woodruff JM, Marshall ML, Godwin TA, Funkhouser JW, Thompson NJ, Erlandson RA. Plexiform (multinodular) schwannoma. A tumor simulating the plexiform neurofibroma. Am J Surg Pathol. 1983. 7:691–697.

5. Ishida T, Kuroda M, Motoi T, Oka T, Imamura T, Machinami R. Phenotypic diversity of neurofibromatosis 2: association with plexiform schwannoma. Histopathology. 1998. 32:264–270.

6. Reith JD, Goldblum JR. Multiple cutaneous plexiform schwannomas. Report of a case and review of the literature with particular reference to the association with types 1 and 2 neurofibromatosis and schwannomatosis. Arch Pathol Lab Med. 1996. 120:399–401.

7. Val-Bernal JF, Figols J, Vazquez-Barquero A. Cutaneous plexiform schwannoma associated with neurofibromatosis type 2. Cancer. 1995. 76:1181–1186.

8. Miyakawa T, Kamada N, Kobayashi T, Hirano K, Fujii K, Sasahara Y, et al. Neurofibromatosis type 2 in an infant with multiple plexiform schwannomas as first symptom. J Dermatol. 2007. 34:60–64.

9. Harkin JC, Arrinton HJ, Reed RJ. Benign plexiform schwannoma, a lesion distinct from plexiform neurofibroma [abstract]. J Neuropathol Exp Neurol. 1978. 37:622.

10. Fletcher CD, Davies SE. Benign plexiform (multinodular) schwannoma: a rare tumour unassociated with neurofibromatosis. Histopathology. 1986. 10:971–980.

11. Honda M, Arai E, Sawada S, Ohta A, Niimura M. Neurofibromatosis 2 and neurilemmomatosis gene are identical. J Invest Dermatol. 1995. 104:74–77.

12. Trofatter JA, MacCollin MM, Rutter JL, Murrell JR, Duyao MP, Parry DM, et al. A novel moesin-, ezrin-, radixin-like gene is a candidate for the neurofibromatosis 2 tumor suppressor. Cell. 1993. 72:791–800.

13. Evans DG, Baser ME, O'Reilly B, Rowe J, Gleeson M, Saeed S, et al. Management of the patient and family with neurofibromatosis 2: a consensus conference statement. Br J Neurosurg. 2005. 19:5–12.

14. Evans DG, Sainio M, Baser ME. Neurofibromatosis type 2. J Med Genet. 2000. 37:897–904.