scholarly journals Pathology of Melanotic Schwannoma

2018 ◽  
Vol 142 (12) ◽  
pp. 1517-1523 ◽  
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.

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

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.

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

2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Kofi Effah ◽  
Stefan Seidl ◽  
Edith Gorges ◽  
Patrick Kafui Akakpo
Keyword(s):  
Carney Complex ◽  
Spinal Nerve ◽  
Nerve Sheath Tumor ◽  
Nerve Roots ◽  
Melanotic Schwannoma ◽  
Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Long Term Follow Up

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.

Malignant Peripheral Nerve Sheath Tumor With Rhabdomyosarcomatous Differentiation (Malignant Triton Tumor)

2006 ◽  
Vol 130 (12) ◽  
pp. 1878-1881 ◽  
Author(s):  
Christopher J. Stasik ◽  
Ossama Tawfik
Keyword(s):  
Peripheral Nerve ◽  
Correct Diagnosis ◽  
Strong Association ◽  
Clinical History ◽  
Nerve Sheath Tumor ◽  
Malignant Triton Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Prognostic Features ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

Abstract Malignant peripheral nerve sheath tumors arise from Schwann cells or within existing neurofibromas and have a strong association with type 1 neurofibromatosis. These tumors are histologically diverse and may contain malignant areas of divergent mesenchymal differentiation, the most common of which is skeletal muscle (rhabdomyosarcoma). Malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation is also known as malignant triton tumor. Malignant triton tumor has a worse prognosis than classic malignant peripheral nerve sheath tumor does, and the correct diagnosis requires attention to the clinical history and knowledge of the complexities regarding its differential diagnosis. In this review we discuss the clinical, histopathological, immunohistochemical, and prognostic features of this rare neoplasm.

Diverse Characteristics of Spinal Nerve Sheath Tumor on Magnetic Resonance Images

2009 ◽  
Vol 16 (1) ◽  
pp. 38 ◽  
Author(s):  
Jae-Yoon Chung ◽  
Hyun-Jong Kim ◽  
Hyung-Yeon Seo ◽  
Jae-Joon Lee
Keyword(s):  
Magnetic Resonance ◽  
Spinal Nerve ◽  
Magnetic Resonance Images ◽  
Nerve Sheath Tumor ◽  
Nerve Sheath

Dumbbell Nerve Sheath Tumors

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.

MELANOTIC NONPSAMMOMATOUS TRIGEMINAL SCHWANNOMA AS THE FIRST MANIFESTATION OF CARNEY COMPLEX

Neurosurgery ◽  
2006 ◽  
Vol 59 (6) ◽  
pp. E1334-E1335 ◽  
Author(s):  
Carmen A. Carrasco ◽  
David Rojas-Salazar ◽  
Renato Chiorino ◽  
Juan C. Venega ◽  
Nelson Wohllk
Keyword(s):  
Diagnostic Criteria ◽  
Cranial Nerves ◽  
Cardiac Myxoma ◽  
Carney Complex ◽  
Regulatory Subunit ◽  
Nerve Sheath Tumor ◽  
Psammoma Bodies ◽  
Middle Fossa Approach ◽  
Melanotic Schwannoma ◽  
The Right

Abstract OBJECTIVE Melanotic schwannoma is a rare neoplasm, classifiable as a peripheral nerve sheath tumor, and differentiated from a typical schwannoma by heavy pigmentation. Psammoma bodies can be visualized in more than 50% of melanotic schwannomas. Half of patients with such “psammomatous melanotic schwannomas” have Carney complex, a dominantly transmitted autosomal disorder. Most recently, the tumor suppressor gene, PRKAR1A, coding for the Type 1α regulatory subunit of protein kinase A was found to be mutated in approximately half of the known Carney complex families. Although cranial schwannomas have been described in patients with Carney complex, their numbers are too small to be considered a definite part of the syndrome. Furthermore, only melanotic schwannomas with psammoma bodies are included as diagnostic criteria for Carney complex. The objective of this report is to communicate a case of trigeminal nonpsammomatous melanotic schwannoma as the first manifestation of Carney complex. CLINICAL PRESENTATION A 34-year-old woman presented with odontalgia, right V3hypoesthesia, V2paresthesia, and diplopia. Magnetic resonance imaging scans of the brain revealed a small tumor with homogenous contrast in the right trigeminal pathway. INTERVENTION We performed an extradural approach to the right cavernous sinus by a middle fossa approach. The lateral wall was opened between the cranial nerves, and a soft and black tumor was resected in a piecemeal fashion. Histology and immunohistochemical analysis of the tumor were compatible with melanotic schwannoma, but no psammomatous bodies were identified. Endocrine evaluation showed that this patient's symptoms fulfilled the diagnostic criteria of Carney complex, with lentiginosis, multiple breast ductal adenomas, multiple hypoechoic nodules on thyroid ultrasonography, and a 4 × 5-cm asymptomatic atrial cardiac myxoma, which was removed 15 days after the neurosurgery. Three months later, a recurrence of melanotic schwannoma was identified. Molecular analyses of genomic and somatic deoxyribonucleic acid from the patient found a 578 to 579delTG mutation of PRKAR1A. CONCLUSION We present the unusual case of a nonpsammomatous trigeminal melanotic schwannoma associated with Carney complex, with confirmed PRKAR1Agene mutation. Our case highlights that neurosurgeons, in the presence of a melanotic schwannoma, should be aware of the features of the Carney complex because, in such cases, pre- and postoperative management is significantly affected. We also postulate that the absence of psammoma bodies or cranial localization do not exclude this diagnosis.

scholarly journals Malignant nerve sheath tumor involving glossopharyngeal, vagus and spinal nerve with intracranial-extracranial extension and systemic metastases in a patient with type 1 neurofibromatosis: A case report

2016 ◽  
Vol 29 ◽  
pp. 196-200 ◽  
Author(s):  
José Raúl Guerra-Mora ◽  
Juan D. Del Castillo-Calcáneo ◽  
María Elena Córdoba-Mosqueda ◽  
Jorge Yáñez-Castro ◽  
Ulises García-González ◽  
...  
Keyword(s):  
Case Report ◽  
Spinal Nerve ◽  
Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Type 1 Neurofibromatosis

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

Neurosurgery ◽  
2005 ◽  
Vol 56 (3) ◽  
pp. 510-515 ◽  
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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