Melanotic Schwannoma of the Vagina: A Report of a Very Rare Tumor and Review of the Literature.

Kofi Effah,Stefan Seidl,Patrick Kafui Akakpo,Edith Gorges

doi:10.1155/2019/8521834

Abstract

Melanotic schwannoma (MS) is a rare nerve sheath tumor with fewer than 200 cases reported. MS has uncertain malignant potential and comprises 1% of all nerve sheath tumors with a predilection for the spinal nerve roots. An even rarer location for this tumor is the vagina. Up to 55% of MSs that contain psammoma bodies are associated with the Carney complex, an autosomal dominant syndrome. Criteria for malignancy in MS are still not well established and long term follow-up of patients is recommended. A 26-year-old woman presented with a bleeding vaginal tumor which was diagnosed as MS following excision. The clinical, histopathological, and immunohistochemical features of this tumor are discussed.

Highlights

Melanotic schwannoma (MS) is a rare nerve sheath tumor composed of Schwann cells, some of which are pigmented [1, 2]
We were unable to find a report of vaginal MS in the English literature after a search of PubMed central and Google Scholar, though cases of regular schwannoma have been reported in the vagina [4]
Due to the rare nature of this tumor, postsurgical management is controversial and large tumors, incompletely resected tumors, and tumors showing aggressive features may require radiation and chemotherapy in conjunction with surgery [1, 8,9,10,11,12]. This is the first case of MS of the vagina that has been reported

Summary

Background

Melanotic schwannoma (MS) is a rare nerve sheath tumor composed of Schwann cells, some of which are pigmented [1, 2]. Fewer than 200 cases of MS have been reported worldwide and they account for less than 1% of all nerve sheath tumors [1]. MS has a predilection for intracranial structures and spinal nerve roots [1, 2]. Up to 50% of MSs contain psammomatous calcification with 50% of these psammomatous MSs being linked to the Carney complex. 50% of these tumors are associated with Carney complex (an autosomal dominant syndrome) and patients have characteristic patchy skin pigmentation, myxomas, and endocrine tumors of the adrenal cortex, pituitary, testis, and thyroid [1,2,3]. A rarer case of MS of the vagina is presented and the literature reviewed

Case Presentation

Histopathological Examination

Discussion

Conclusion

Findings

Conflicts of Interest