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

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.

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Hemorrhagic spinal melanotic schwannoma presenting as acute chest pain: A case report and literature review

Surgical Neurology International ◽

10.25259/sni_786_2020 ◽

2021 ◽

Vol 12 ◽

pp. 164

Author(s):

Dallas J. Soyland ◽

Dylan R. Goehner ◽

Kayla M. Hoerschgen ◽

Troy D. Gust ◽

Shawn M. Vuong

Keyword(s):

Myocardial Infarction ◽

Chest Pain ◽

Acute Chest Pain ◽

Carney Complex ◽

Spinal Nerve ◽

Nerve Sheath Tumor ◽

Melanotic Schwannoma ◽

Nerve Sheath ◽

Ct Angiogram ◽

Intradural Extramedullary

Background: Melanotic schwannoma (MS) is a rare variant of peripheral nerve sheath tumor. MS commonly arises along the spinal nerve sheath. Patients most often experience pain along the dermatome of the affected nerve root. Symptoms development is usually insidious. About half of MS cases are associated with Carney complex, a multi-neoplastic disorder. The remaining cases arise spontaneously. About 10–44% of these tumors undergo malignant transformation. Case Description: We describe a case of hemorrhagic MS presenting as acute chest pain mimicking myocardial infarction, a presentation which has not yet been described in the literature. Neurologic examination did not reveal any abnormalities. Myocardial infarction was ruled out in the ER, and a chest CT angiogram was ordered for evaluation of PE or aortic dissection which revealed an intradural extramedullary dumbbell-shaped mass extending through the left vertebral foramen at the level of T8. MRI revealed a heterogenous mass that was hyperintense with T2 and hypointense with T1-weighted imaging. The patient underwent an open laminectomy of the left T8 and T9 vertebrae and gross total resection (GTR) of a hemorrhagic black tumor. Microscopic examination showed fascicles and nests of plump spindle cells with variable intracellular melanin. Immunohistochemistry showed the cells to be positive for S100, SOX10, HMB-45, and MART-1, confirming diagnosis of MS. Two months after the operation, the patient was doing well and is free of recurrence. Conclusion: GTR is considered the optimal treatment for MS; radiotherapy and chemotherapy may be considered but have not been shown to improve patient outcomes.

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Clinical Characteristics of Spinal Nerve Sheath Tumors: Analysis of 149 Cases

Neurosurgery ◽

10.1227/01.neu.0000153752.59565.bb ◽

2005 ◽

Vol 56 (3) ◽

pp. 510-515 ◽

Cited By ~ 121

Author(s):

Takahiro Jinnai ◽

Minoru Hoshimaru ◽

Tsunemaro Koyama

Keyword(s):

Nerve Root ◽

Spinal Nerve ◽

Nerve Sheath Tumor ◽

Spinal Nerve Root ◽

Intervertebral Foramen ◽

Nerve Roots ◽

Nerve Sheath Tumors ◽

Nerve Sheath ◽

Caudal Portion ◽

Rostral Portion

Abstract OBJECTIVE: Spinal nerve sheath tumors arise from the spinal nerve root and grow along it. There are two sites at which the growth of a tumor is restricted: the dural aperture for the spinal nerve root and the intervertebral foramen. This article describes the growth pattern of a spinal nerve sheath tumor along the spinal nerve root at various spinal levels. METHODS: We retrospectively reviewed the records for 149 patients with spinal nerve sheath tumors who were treated between 1980 and 2001. Of these, 176 resected tumors were classified into five groups according to the relationship to the dura mater and/or the intervertebral foramen. RESULTS: Strictly intradural tumors compose 8% of nerve sheath tumors of the first two cervical nerve roots. The percentage of these tumors increased gradually from the high cervical region to the thoracolumbar region, where it was more than 80%. In contrast, the percentage of strictly extradural tumors gradually decreased from the rostral portion to the caudal portion. Similarly, a percentage of tumors extending outside the spinal canal decreased from the rostral portion to the caudal portion. These changes of the growth pattern may be explained by the anatomic features of the spinal nerve roots, which have a longer intradural component at the more caudal portion of the spinal axis. CONCLUSION: The anatomic relationship of a nerve sheath tumor with the dura mater and the intervertebral foramen varies depending on the level of the tumor. This knowledge may help us to create a strategy for total resection of a nerve sheath tumor.

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Extrathecal intraradicular nerve sheath tumor

Journal of Neurosurgery Spine ◽

10.3171/spi.2005.3.1.0001 ◽

2005 ◽

Vol 3 (1) ◽

pp. 1-11 ◽

Cited By ~ 10

Author(s):

Paolo Celli ◽

Giuseppe Trillò ◽

Luigi Ferrante

Keyword(s):

Nerve Root ◽

Spinal Nerve ◽

Nerve Sheath Tumor ◽

En Bloc ◽

Content Type ◽

Nerve Sheath Tumors ◽

Nerve Sheath ◽

Radical Removal ◽

Fine Print

Object. The purpose of this study was to analyze the clinical profile of patients harboring extrathecal and intraradicular nerve sheath tumors (NSTs), located inside the sleeve of an extrathecal nerve root and very often within the proximal portion of the spinal nerve, and to evaluate the incidence of long-term dysfunction of the tumor-affected roots if resected. These tumors have not received particular attention in the literature. Methods. A single-institution series of 16 patients who had undergone surgery for intraradicular NSTs during a 50-year period was selected retrospectively. Data pertaining to clinical features, tumor characteristics, and results of surgery were analyzed. Conclusions. Extrathecal and intraradicular neurofibromas or schwannomas more frequently affect the lumbar and S-1 nerve roots, often producing root pain only. Selective en bloc enucleation sparing at least part of the motor rootlets is possible for small schwannomas of the extrathecal—preganglion segment of the radix, whereas total resection of the affected root is generally required for radical removal of neurofibromas and large schwannomas. In the authors' experience, neither deafferentation pain nor severe radicular weakness occurs after division of the nerve root harboring the tumor.

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Pathology of Melanotic Schwannoma

Archives of Pathology & Laboratory Medicine ◽

10.5858/arpa.2017-0162-ra ◽

2018 ◽

Vol 142 (12) ◽

pp. 1517-1523 ◽

Cited By ~ 19

Author(s):

Borislav A. Alexiev ◽

Pauline M. Chou ◽

Lawrence J. Jennings

Keyword(s):

Correct Diagnosis ◽

Molecular Genetic ◽

Metastatic Potential ◽

Carney Complex ◽

Spinal Nerve ◽

Current Status ◽

Nerve Sheath Tumor ◽

High Tendency ◽

Melanotic Schwannoma ◽

Nerve Sheath

Context.— Melanotic schwannoma (MS) is a nerve sheath tumor with a uniform composition of variably melanin-producing Schwann cells and metastatic potential. The MS is an uncommon neoplasm, accounting for less than 1% of all nerve sheath tumors, with a predilection for spinal nerve involvement. Microscopically, the tumors are characterized by spindle and epithelioid cells arranged in interlacing fascicles, with marked accumulation of melanin in neoplastic cells and associated melanophages. The MSs are frequently associated with Carney complex, showing features of psammoma bodies and adipose-like cells. Strict criteria of malignancy in MS are not well developed, although a combination of worrisome histologic features (large, vesicular nuclei, with macronucleoli, brisk mitotic activity, and necrosis) raises concern for aggressive behavior. Objective.— To review the current status of the MS literature, discussing putative etiology, histopathology, current genetics, and differential diagnoses, including overlap with other pigmented tumors. Data Sources.— Search of PubMed (National Center for Biotechnology Information, Bethesda, Maryland) and the authors' own experiences. Conclusions.— The occurrence of MS at several unusual anatomic sites and its spectrum of morphologic patterns can result in significant diagnostic difficulty, and correct diagnosis is particularly important because of its high tendency to recur locally and to metastasize, which highlights the importance of diagnostic recognition, ancillary molecular genetic testing, and close clinical follow-up of patients with MS.

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Complete removal of the spinal nerve sheath tumors. Surgical technics and results from a series of 30 patients

Arquivos de Neuro-Psiquiatria ◽

10.1590/0004-282x20140008 ◽

2014 ◽

Vol 72 (4) ◽

pp. 312-317 ◽

Cited By ~ 7

Author(s):

Rudi Lenck Fernandes ◽

José Carlos Lynch ◽

Leonardo Welling ◽

Mariangela Gonçalves ◽

Rodrigo Tragante ◽

...

Keyword(s):

Great Majority ◽

Complete Removal ◽

Spinal Nerve ◽

Surgical Mortality ◽

Total Removal ◽

Recurrence Rates ◽

Nerve Sheath Tumors ◽

Nerve Sheath ◽

Recurrence Method

Objective: Observe whether a microsurgical gross total removal (GTR) of a spinal nerve sheath tumors (SNSTs) is safe and decreases the tumor recurrence. Method: We identify 30 patients with 44 SNSTs. Results: We operated upon 15 males and 15 females patients; mean age 40 years. GTR was achieved in 29 (96.6%) instances. Surgical mortality was 3.3% and the recurrence rate was 3.3%. The median follow-up time was 6.2 years. Conclusion: The surgical approach used in this group of patients afford that the great majority of tumors could be totally removed with low mortality and low recurrence rates, proving to be safe and effective.

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Dumbbell Nerve Sheath Tumors

10.1093/med/9780190617127.003.0019 ◽

2018 ◽

Author(s):

Hussam Abou-Al-Shaar ◽

Mark A. Mahan

Keyword(s):

Imaging Modality ◽

Spinal Nerve ◽

Surgical Approaches ◽

Nerve Sheath Tumor ◽

Imaging Features ◽

Neural Foramen ◽

Dumbbell Tumor ◽

Nerve Sheath Tumors ◽

Nerve Sheath ◽

The Individual

A dumbbell tumor is a nerve sheath tumor that arises from a spinal nerve in the neural foramen and grows as a dumbbell-shaped mass. The differential diagnosis for a dumbbell tumor includes schwannoma, neurofibroma, malignant peripheral nerve sheath tumor, and metastases, among others. MR imaging is considered the gold-standard imaging modality for diagnosis of dumbbell tumors. Surgical approaches that are tailored to the individual patient’s case can be utilized. The chapter reviews dumbbell tumors, including a case example and covers the incidence, clinical presentation, imaging features, decision-making strategy, surgical approaches, outcomes, and potential complications associated with their management.

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Nerve Sheath Tumors—Schwannomas and Neurofibromas

10.1093/med/9780190617127.003.0018 ◽

2018 ◽

Author(s):

Christian Heinen ◽

Thomas Kretschmer

Keyword(s):

Peripheral Nerve ◽

Nerve Sheath Tumor ◽

Surgical Strategies ◽

Treatment Timing ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Tumor ◽

Nerve Sheath ◽

Contrast Enhanced

A benign peripheral nerve sheath tumor is illustrated in a case presentation of a painful mass in the medial thigh, with paresthesias radiating along the course of the saphenous nerve. The presenting features, appropriate workup, treatment timing, surgical strategies, follow-up, and results for nerve-associated masses are outlined. Specific imaging findings for peripheral nerve sheath tumors on contrast-enhanced MR imaging and the merits of high-resolution ultrasound are detailed. The typical features of a well-defined and noninvasive peripheral nerve tumor, the principles of exploration, and microsurgical enucleation technique are highlighted. Other nerve tumor entities that should be considered in the differential diagnosis, as well as their respective features, are discussed.

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Melanotic schwannoma of the lumbar spine: a case report and literature review

Coluna/Columna ◽

10.1590/s1808-185120151402122972 ◽

2015 ◽

Vol 14 (2) ◽

pp. 149-151

Author(s):

João Bernardo Sancio Rocha Rodrigues ◽

Nathália Ambrozim Santos Saleme ◽

Charbel Jacob Junior ◽

José Lucas Batista Junior ◽

Igor Machado Cardoso ◽

...

Keyword(s):

Lumbar Spine ◽

Carney Complex ◽

Spinal Nerve ◽

Benign Tumors ◽

Nerve Roots ◽

Melanotic Schwannoma ◽

Immunohistochemical Studies ◽

Weighted Sequences ◽

Slow Growing ◽

Usual Development

<p>Schwannomas are benign tumors, usually solitary, encapsulated, slow-growing, which have their origin in differentiated neoplastic Schwann cells with extramedullary intradural usual development related to nerve roots. The melanotic schwannoma is a variant of these tumors whose location in almost one third of cases is on the posterior spinal nerve root, with a nonspecific clinical presentation. Magnetic resonance imaging is the most widely used test for the diagnosis, revealing hyperintense T1-weighted sequences and hypointense T2-weighted sequences. Diagnostic confirmation is obtained by histological and immunohistochemical studies, in which there is intense cytoplasmatic pigmentation. There are two distinct types of melanotic schwannomas: sporadic and psammomatous, the latter related to the called Carney complex, a form of multiple endocrine neoplasm with familiar character. In literature we found few cases of these neoplasms, the largest series consisting of five cases. The objective of this study is to report a rare case of melanotic schwannoma of the lumbar spine of the sporadic type of extramedullary location. We also present a brief review of the literature containing the main characteristics of the tumor, including its different forms, differential diagnoses, data from histological and immunohistochemical studies as well as the currently recommended approach in order to contribute to a better understanding of this neoplasm.</p>

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