Melanotic Schwannomas Are Rarely Seen Pigmented Tumors with Unpredictable Prognosis and Challenging Diagnosis

Melanotic Schwannoma (MS) is rarely seen and potentially malignant neoplasm that is categorized as a variant of Schwannoma. MS most frequently involves intracranial structures followed by posterior nerve roots in the spinal canal. Approximately 50% of the cases with MS have psammomatous calcificatio...

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Main Authors: Elif Keskin, Sumeyye Ekmekci, Ozgur Oztekin, Gulden Diniz
Format: Article
Language:English
Published: Hindawi Limited 2017-01-01
Series:Case Reports in Pathology
Online Access:http://dx.doi.org/10.1155/2017/1807879
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spelling doaj-d80f2f2d7cc94138ba069e005726e73c2020-11-24T22:52:06ZengHindawi LimitedCase Reports in Pathology2090-67812090-679X2017-01-01201710.1155/2017/18078791807879Melanotic Schwannomas Are Rarely Seen Pigmented Tumors with Unpredictable Prognosis and Challenging DiagnosisElif Keskin0Sumeyye Ekmekci1Ozgur Oztekin2Gulden Diniz3Tepecik Education and Research Hospital, Izmir, TurkeyTepecik Education and Research Hospital, Izmir, TurkeyTepecik Education and Research Hospital, Izmir, TurkeyTepecik Education and Research Hospital, Izmir, TurkeyMelanotic Schwannoma (MS) is rarely seen and potentially malignant neoplasm that is categorized as a variant of Schwannoma. MS most frequently involves intracranial structures followed by posterior nerve roots in the spinal canal. Approximately 50% of the cases with MS have psammomatous calcifications and this type of MS is related to Carney complex with autosomal dominant inheritance. Most cases of MS are benign, though 10% of them are malignant with metastatic potential. MS mimics melanoma and the differential diagnosis should be made excluding other melanin producing neoplasms especially melanoma. Case 1. A 42-year-old hypertensive male presented for checkup. He had a well-defined extraspinal oval lesion measuring 3.5 × 2.5 cm near right adrenal. Case 2. A 22-year-old female presented with neurofibromatosis-2, bilateral acoustic schwannomas and café au lait lesions on sacrococcygeal region. She had an intradural extramedullary lesion measuring 6.1 × 2.0 cm at L1-2 level. MS is a rare neoplasm composed of Schwann cells and melanin pigment. These tumors are usually benign but they may become aggressive. The biologic behavior of MS is difficult to predict; the patients have to be followed up for a longer period due to its malignant potential.http://dx.doi.org/10.1155/2017/1807879
collection DOAJ
language English
format Article
sources DOAJ
author Elif Keskin
Sumeyye Ekmekci
Ozgur Oztekin
Gulden Diniz
spellingShingle Elif Keskin
Sumeyye Ekmekci
Ozgur Oztekin
Gulden Diniz
Melanotic Schwannomas Are Rarely Seen Pigmented Tumors with Unpredictable Prognosis and Challenging Diagnosis
Case Reports in Pathology
author_facet Elif Keskin
Sumeyye Ekmekci
Ozgur Oztekin
Gulden Diniz
author_sort Elif Keskin
title Melanotic Schwannomas Are Rarely Seen Pigmented Tumors with Unpredictable Prognosis and Challenging Diagnosis
title_short Melanotic Schwannomas Are Rarely Seen Pigmented Tumors with Unpredictable Prognosis and Challenging Diagnosis
title_full Melanotic Schwannomas Are Rarely Seen Pigmented Tumors with Unpredictable Prognosis and Challenging Diagnosis
title_fullStr Melanotic Schwannomas Are Rarely Seen Pigmented Tumors with Unpredictable Prognosis and Challenging Diagnosis
title_full_unstemmed Melanotic Schwannomas Are Rarely Seen Pigmented Tumors with Unpredictable Prognosis and Challenging Diagnosis
title_sort melanotic schwannomas are rarely seen pigmented tumors with unpredictable prognosis and challenging diagnosis
publisher Hindawi Limited
series Case Reports in Pathology
issn 2090-6781
2090-679X
publishDate 2017-01-01
description Melanotic Schwannoma (MS) is rarely seen and potentially malignant neoplasm that is categorized as a variant of Schwannoma. MS most frequently involves intracranial structures followed by posterior nerve roots in the spinal canal. Approximately 50% of the cases with MS have psammomatous calcifications and this type of MS is related to Carney complex with autosomal dominant inheritance. Most cases of MS are benign, though 10% of them are malignant with metastatic potential. MS mimics melanoma and the differential diagnosis should be made excluding other melanin producing neoplasms especially melanoma. Case 1. A 42-year-old hypertensive male presented for checkup. He had a well-defined extraspinal oval lesion measuring 3.5 × 2.5 cm near right adrenal. Case 2. A 22-year-old female presented with neurofibromatosis-2, bilateral acoustic schwannomas and café au lait lesions on sacrococcygeal region. She had an intradural extramedullary lesion measuring 6.1 × 2.0 cm at L1-2 level. MS is a rare neoplasm composed of Schwann cells and melanin pigment. These tumors are usually benign but they may become aggressive. The biologic behavior of MS is difficult to predict; the patients have to be followed up for a longer period due to its malignant potential.
url http://dx.doi.org/10.1155/2017/1807879
work_keys_str_mv AT elifkeskin melanoticschwannomasarerarelyseenpigmentedtumorswithunpredictableprognosisandchallengingdiagnosis
AT sumeyyeekmekci melanoticschwannomasarerarelyseenpigmentedtumorswithunpredictableprognosisandchallengingdiagnosis
AT ozguroztekin melanoticschwannomasarerarelyseenpigmentedtumorswithunpredictableprognosisandchallengingdiagnosis
AT guldendiniz melanoticschwannomasarerarelyseenpigmentedtumorswithunpredictableprognosisandchallengingdiagnosis
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