Extramedullary malignant melanotic schwannoma of the spine: Case report and an up to date systematic review of the literature

Solomou, Georgios; Silva, Adikarige Haritha Dulanka; Wong, Adrianna; Pohl, Ute; Tzerakis, Nikolaos

doi:10.1016/j.amsu.2020.10.003

Cited by 18 publications

(20 citation statements)

References 53 publications

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“…Approximately 200 cases have been reported in the literature since the first identified case published in 1931 by Millar, who described the lesion as a “malignant melanotic tumor of the ganglion cells,”[ 4 ] with fewer than 70 of these lesions localized to the spine. [ 5 ] These lesions may be separated into psammomatous and non-psammomatous subtypes. Non-psammomatous lesions appear to arise sporadically, while psammomatous subtypes are considered to be part of the Carney complex, characterized by the presence of cutaneous lesions, endocrine tumors, and cardiac myxomas.…”

Section: Discussion and Literature Reviewmentioning

confidence: 99%

“…Malignant melanotic nerve sheath tumors (MMNSTs) of the spine are rare, and fewer than 70 cases are described in the literature. [ 5 ] According to the 5 th Edition of the WHO classification of soft tissue and bone lesions, these most commonly affect the dorsal nerve roots but can also impact the entire neuroaxis and the gastrointestinal tract. Despite a relatively benign histological appearance, they exhibit high local recurrence rates and/or metastases.…”

Section: Introductionmentioning

confidence: 99%

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Leptomeningeal dissemination of a malignant melanotic nerve sheath tumor: A case report and review of the literature

Shui

Davey

Scholsem

2022

Surgical Neurology International

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Background: Malignant melanotic nerve sheath tumors (MMNSTs) are rare tumors of presumed neural crest origin. Here, we present a 21-year-old female with a left L5/S1 MMNST along with a review of approximately 70 spinal cases reported in the literature, the majority of which were either local recurrences or metastases. Case Description: A 21-year-old female presented with 3 months of severe left L5 distribution radicular leg pain and sensory loss. The MR revealed a dumbbell-shaped, heterogenously enhancing lesion centered on the left L5/S1 foramen; the intracanalicular component displaced the thecal sac to the right, while the extraforaminal portion of tumor extended anteriorly into the retroperitoneal space. Gross total resection was performed after a L5/S1 facetectomy. In the immediate postoperative period there were no complications, and the patient had full lower limb power. Four months later, the patient experienced generalized seizures, headache, and multiple cranial nerve palsies due to local and diffuse CNS dissemination. The MRI of the brain and whole spine revealed diffuse leptomeningeal enhancement along the full length of the spinal cord into the brainstem and cerebrum along with a focally recurrent epidural soft-tissue lesion located posterolaterally on the left at the L4/5 level (i.e., measuring 12 mm × 10 mm). An external ventricular drain and subsequent ventriculoperitoneal shunt were inserted, followed by craniospinal irradiation. She was discharged 3 months later with residual distal lower limb weakness. Conclusion: This case illustrates the rapid disease progression of MMNST despite gross total resection. Further such lesions should be aggressively treated locally, and followed by adjuvant radiotherapy and systemic chemotherapy/immunotherapy.

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Section: Discussion and Literature Reviewmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Leptomeningeal dissemination of a malignant melanotic nerve sheath tumor: A case report and review of the literature

Shui

Davey

Scholsem

2022

Surgical Neurology International

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“…Solomou et al reported the use of multiple immunotherapeutics in the management of a patient with advanced melanotic spinal schwannoma. 39 They described a case of a dumbbell-shaped lesion with intradural and extradural components in the cervical spine along the C6 nerve root. The patient underwent 2 surgical procedures and received combination immunotherapy, including nivolumab (programmed cell death protein 1 [PD-1] death-ligand receptor), ipilimumab (cytotoxic T-lymphocyte antigen 4 [CTLA4]), and denosumab (receptor activator of nuclear factor kappa b ligand antibodies), as these therapies have previously shown efficacy in treatment of primary melanoma.…”

Section: Schwannomasmentioning

confidence: 99%

“…The patient underwent 2 surgical procedures and received combination immunotherapy, including nivolumab (programmed cell death protein 1 [PD-1] death-ligand receptor), ipilimumab (cytotoxic T-lymphocyte antigen 4 [CTLA4]), and denosumab (receptor activator of nuclear factor kappa b ligand antibodies), as these therapies have previously shown efficacy in treatment of primary melanoma. 39 Unfortunately, this aggressive immunotherapy combination was ineffective, and the patient was subsequently readmitted with respiratory complications and died. 39 Another report by Sahay et al described 7 cases of melanotic spinal schwannoma occurring in the cranium or spinal cord.…”

Section: Schwannomasmentioning

confidence: 99%

“…39 Unfortunately, this aggressive immunotherapy combination was ineffective, and the patient was subsequently readmitted with respiratory complications and died. 39 Another report by Sahay et al described 7 cases of melanotic spinal schwannoma occurring in the cranium or spinal cord. 40 Four of these cases originated in the spine, in which 1 patient received immunotherapy following resection of a lumbar tumor.…”

Section: Schwannomasmentioning

confidence: 99%

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Immunotherapeutic treatments for spinal and peripheral nerve tumors: a primer

Bryant

Govindarajan

et al. 2022

Neurosurgical Focus

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OBJECTIVE Spinal and peripheral nerve tumors are a heterogeneous group of neoplasms that can be associated with significant morbidity and mortality despite the current standard of care. Immunotherapy is an emerging therapeutic option to improve the prognoses of these tumors. Therefore, the authors sought to present an updated and unifying review on the use of immunotherapy in treating tumors of the spinal cord and peripheral nerves, including a discussion on mechanism of action, drug delivery, current treatment techniques, and preclinical and clinical studies. METHODS Current data in the literature regarding immunotherapy were collated and summarized. Targeted tumors included primary and secondary spinal tumors, as well as peripheral nerve tumors. RESULTS Four primary modalities of immunotherapy (CAR T cell, monoclonal antibody, viral, and cytokine) have been reported to target spine and peripheral nerve tumors. Of the primary spinal tumors, spinal cord astrocytomas had the most preclinical evidence supporting immunotherapy success with CAR T-cell therapy targeting the H3K27M mutation, whereas spinal schwannomas and ependymomas had the most evidence reported for monoclonal antibody therapy preclinically. Of the secondary spinal tumors, primary CNS lymphomas demonstrated some clinical response to immunotherapy, whereas multiple myeloma and bone tumor experiences with immunotherapy were largely limited to concept only. Within peripheral nerve tumors, the use of immunotherapy to treat neurofibromas in the setting of syndromes has been suggested in theory, and possible immunotherapeutic targets have been identified in malignant peripheral nerve tumors. To date, there have been 2 clinical trials involving spine tumors and 2 clinical trials involving peripheral nerve tumors that have reported results, all of which are promising but require validation. CONCLUSIONS Immunotherapy to treat spinal and peripheral nerve tumors has become an emerging area of research and interest. A large amount of preclinical data supporting the translation of this therapy into practice, aimed at ameliorating the poor prognoses of specific tumors, have been reported. Future clinical studies for translation will focus on the optimal therapy type and administration route to best target these tumors, which often preclude total surgical resection given their proximity to the neural and vascular elements of the spine.

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