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Gold J, Hernandez NR, Wong T, Patel N, Weiner J, Hanft S. Primary Spinal Malignant Melanoma Mimicking a Cervical Nerve Root Schwannoma: Case Report and Literature Review. Asian J Neurosurg 2024; 19:540-550. [PMID: 39205889 PMCID: PMC11349406 DOI: 10.1055/s-0044-1787081] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 09/04/2024] Open
Abstract
Primary spinal malignant melanoma (PSMM) is a rare cancer of the central nervous system (CNS), and PSMM of the spinal nerve root is even more extraordinary. PSMM of a nerve root can mimic the radiographic appearance of benign nerve sheath tumors, thus resulting in misdiagnosis until tissue diagnosis can be made. A 53-year-old African American woman presented with pain primarily involving the left aspect of her neck and shoulder for 2 years. Magnetic resonance imaging (MRI) of the cervical spine demonstrated a T1-hyperintense, T2-hypointense, homogenously enhancing, dumbbell-shaped, intradural extramedullary mass extending out through the left C2-3 foramen. A midline incision was used to perform a C2 and C3 laminectomy, and the mass was removed from the cavity. The histopathologic profile was consistent with the diagnosis of malignant melanoma. The present case report adds to the 110 cases of PSMM and the 20 cases of PSMM of the spinal nerve root in the existing body of literature. Radiographic and clinical features resemble that of the much more common schwannoma or neurofibroma requiring immunohistochemical analysis for definitive diagnosis. The optimal treatment for PSMM has not yet been defined due to its rarity and it is therefore important to report such cases in order to share our clinical experiences and provide data to other clinicians treating this uncommon disease.
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Affiliation(s)
- Justin Gold
- Department of Neurological Surgery, Cooper Medical of Rowan University, Camden, New Jersey, United States
| | - Nick R. Hernandez
- Department of Neurosurgery, Robert Wood Johnson Hospital, New Brunswick, New Jersey, United States
| | - Timothy Wong
- Department of Neurosurgery, Robert Wood Johnson Hospital, New Brunswick, New Jersey, United States
| | - Nitesh Patel
- Department of Neurosurgery, Robert Wood Johnson Hospital, New Brunswick, New Jersey, United States
| | - Joseph Weiner
- Department of Radiation Oncology, Robert Wood Johnson Hospital, New Brunswick, New Jersey, United States
| | - Simon Hanft
- Department of Neurological Surgery, Westchester Medical Center, New York Medical College, Valhalla, New York, United States
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2
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Abbas T, Batool S, Muzaffar B, Ahsan Z, Hamid FB, Hameed A, Tariq MR. Primary Spinal Intradural Melanocytoma of the Thoracic Region: A Rare Case. Cureus 2023; 15:e41019. [PMID: 37519491 PMCID: PMC10372852 DOI: 10.7759/cureus.41019] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 06/27/2023] [Indexed: 08/01/2023] Open
Abstract
While the presence of metastatic melanocytoma in the central nervous system (CNS) is relatively common, primary spinal melanocytoma (PSM) is an extremely rare entity. Only 70 cases have been reported, and its usual position is the cervical region. We report a case of a 35-year-old male with primary spinal intramedullary melanocytoma with a dorsal exophytic component. The tumor was first opened in the periphery and was closed without being operated upon due to it being an uncommon pathology.
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Affiliation(s)
- Talha Abbas
- Department of Neurosurgery, Sir Ganga Ram Hospital, Lahore, PAK
| | - Sakina Batool
- Department of Internal Medicine, Sir Ganga Ram Hospital, Lahore, PAK
| | - Bireera Muzaffar
- Department of Internal Medicine, Sir Ganga Ram Hospital, Lahore, PAK
| | - Zainab Ahsan
- Department of Internal Medicine, Combined Military Hospital (CMH) Lahore Medical College and Institute of Dentistry, Lahore, PAK
| | - Fahad B Hamid
- Department of Medicine, Combined Military Hospital (CMH) Lahore Medical College and Institute of Dentistry, Lahore, PAK
| | - Abdul Hameed
- Department of Neurosurgery, Sir Ganga Ram Hospital, Lahore, PAK
| | - Mah R Tariq
- Department of Medicine, Combined Military Hospital (CMH) Lahore Medical College and Institute of Dentistry, Lahore, PAK
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3
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Shi YF, Chen YQ, Chen HF, Hu X. An atypical primary malignant melanoma arising from the cervical nerve root: A case report and review of literture. World J Clin Cases 2022; 10:381-387. [PMID: 35071542 PMCID: PMC8727269 DOI: 10.12998/wjcc.v10.i1.381] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/21/2021] [Revised: 10/29/2021] [Accepted: 11/30/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Primary melanomas affecting the central nervous system are very rare, and melanomas originating in the spinal canal or origin of the spinal nerve root are even rarer. As a consequence, not much is known about this.
CASE SUMMARY Here we report a case of primary malignant melanoma originating in the cervical spinal cord nerve root. A 64-year-old woman presented with symptoms of numbness in the right side of the neck, pain, and hypoesthesia in the right upper limb which persisted for 1 year. Neurological examination showed that the superficial sensation in the right upper limb had decreased with muscle strength of grade 4. Magnetic resonance imaging examination revealed a mass (approximately 2.5 cm × 1.4 cm × 1 cm) in the right side of the spinal canal in the C-2 plane. Based on findings obtained during operation, perioperative examination, pathological diagnosis, and the diagnostic criteria of primary central melanoma proposed by Hayward, the mass was confirmed to be a melanoma of intraspinal nerve root origin.
CONCLUSION This is the first case of primary malignant melanoma originating from cervical spinal cord nerve roots and spread along the inside and outside of the spinal canal. The clinical relevance of this case is discussed to provide new insights into the differential diagnosis of intraspinal tumours. Further studies are needed to better understand the mechanisms driving the growth pattern and development of this type of tumour.
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Affiliation(s)
- Yi-Feng Shi
- Department of Neurosurgery, West China Hospital, Chengdu 610041, Sichuan Province, China
| | - Yu-Qi Chen
- Department of Neurosurgery, West China Hospital, Chengdu 610041, Sichuan Province, China
| | - Hai-Feng Chen
- Department of Neurosurgery, West China Hospital, Chengdu 610041, Sichuan Province, China
| | - Xin Hu
- Department of Neurosurgery, West China Hospital, Chengdu 610041, Sichuan Province, China
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4
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Haberfellner E, Elbaroody M, Alkhamees AF, Alaosta A, Eaton S, Quint E, Shahab S, O'Connor A, Im J, Khan A, El-Gohary Y, Lotfy M, Sawan M, Shamisa A, Soliman MAR. Primary Spinal Melanoma: Case Report and Systematic Review. Clin Neurol Neurosurg 2021; 205:106649. [PMID: 33932774 DOI: 10.1016/j.clineuro.2021.106649] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/02/2021] [Revised: 03/15/2021] [Accepted: 04/10/2021] [Indexed: 11/21/2022]
Abstract
INTRODUCTION Primary malignant melanoma of the spinal cord (PSM) is a rare condition with limited evidence regarding its diagnosis (clinical and radiographic), management, and prognosis. Our aim was to report an extremely rare two cases of primary malignant melanoma of the spine one of them is sacral melanoma which represents the second reported case in the literature and to conduct a systematic review of the relevant literature. METHODS The diagnosis and management of these cases were retrospectively reviewed. Using the PRISMA guideline, we conducted a systematic review of the literature to analyze different management strategies and the prognosis of such pathology. RESULTS All two patients were operated on, and received gross total removal of their tumors, with extended follow up for tumor recurrences. One of the cases involved a sacral tumor, which was resected without adjuvant therapy. The other one was seen by oncology and received post-operative chemo- and radio- therapy. In addition to the aforementioned cases, we present a comprehensive review of the literature on PSM from 1950 to the present, demonstrating that PSM is a very rare tumor, with a limited counted number of cases reported worldwide. CONCLUSION In conclusion, we report an exceedingly rare two cases of primary malignant melanoma of the spine. Early surgical intervention is key to the management of these rare and aggressive tumors. GTR should be attempted if possible.
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Affiliation(s)
- Erika Haberfellner
- Schulich School of Medicine and Dentistry, Western University, Ontario, Canada
| | | | - Abdullah F Alkhamees
- Schulich School of Medicine and Dentistry, Western University, Ontario, Canada; Neurosurgery Department, Qassim University, Kingdom of Saudi Arabia
| | - Abdelfatah Alaosta
- Schulich School of Medicine and Dentistry, Western University, Ontario, Canada; Neurosurgery Department, Sirte University, Libya
| | - Sydney Eaton
- Schulich School of Medicine and Dentistry, Western University, Ontario, Canada
| | - Elise Quint
- Schulich School of Medicine and Dentistry, Western University, Ontario, Canada
| | - Saba Shahab
- Schulich School of Medicine and Dentistry, Western University, Ontario, Canada
| | - Avalon O'Connor
- Schulich School of Medicine and Dentistry, Western University, Ontario, Canada
| | - Jacob Im
- Schulich School of Medicine and Dentistry, Western University, Ontario, Canada
| | - Asham Khan
- Department of Neurosurgery, Jacobs School of Medicine and Biomedical Sciences at University at Buffalo, United States
| | - Yasser El-Gohary
- Pathology Department, Windsor Regional Hospital, Western University, Canada
| | | | | | - Abdalla Shamisa
- Neurosurgery Department, Windsor Regional Hospital, Western University, Canada
| | - Mohamed A R Soliman
- Schulich School of Medicine and Dentistry, Western University, Ontario, Canada; Neurosurgery Department, Cairo University, Egypt.
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5
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Saleem N, Saleem R, Asghar H, Zubair M, Farooque U. Primary Spinal Melanoma With Intra- and Extradural Extensions: A Rare Case. Cureus 2021; 13:e12855. [PMID: 33633887 PMCID: PMC7899273 DOI: 10.7759/cureus.12855] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022] Open
Abstract
Primary spinal melanoma (PSM) is a rare primary central nervous system melanoma with limited literature. A 30-year-old male presented with one year of progressive bilateral leg weakness and back pain. Physical examination revealed slightly decreased power and deep tendon reflexes of the lower extremities, decreased sensation at the level of T10, and normal anal sphincter and plantar reflexes. Magnetic resonance imaging (MRI) scan of the thoracolumbar spine revealed a hypointense lesion on T2-weighted and a hyperintense lesion on T1-weighted imaging at the level of T10 with mild extensions. The lesion was causing a mass effect on the spinal cord. The patient underwent laminectomy and near-total excision which showed a black, firm-to-hard, scarcely vascular extradural tumor extending from T10 to T11 that adhered to nerve roots. Histopathological examination and immunostaining with S-100 and Melan-A stains confirmed the diagnosis of malignant melanoma. Other imaging studies like brain computed tomography (CT) and positron emission tomography/computed tomography (PET/CT) scans, and chest X-ray were normal. On follow-up, the patient reported improvement in the power of his lower limbs with intact sensory function and sphincters. The first radiotherapy session was scheduled for six weeks postoperatively. There was no recurrence at a two-year follow-up. The possibility of a melanocytic tumor should be considered for a spinal lesion with paramagnetic properties as early surgical intervention is important for diagnosis and improved survival.
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Affiliation(s)
- Noman Saleem
- Forensic Medicine, Sahiwal Medical College, Sahiwal, PAK
| | - Rabia Saleem
- Neurosurgery, Punjab Institute of Neurosciences, Lahore, PAK
| | - Hannan Asghar
- Internal Medicine, Orange Park Medical Center, Orange Park, USA
| | | | - Umar Farooque
- Neurology, Dow University of Health Sciences, Karachi, PAK
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6
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Fortin Ensign S, Bollin K, Millis SZ, Hinds BR, Kosty M, Uchiyama C. Genomic analysis reveals low tumor mutation burden which may be associated with GNAQ/11 alteration in a series of primary leptomeningeal melanomas. Pigment Cell Melanoma Res 2019; 33:458-465. [PMID: 31663661 DOI: 10.1111/pcmr.12839] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/02/2019] [Revised: 10/16/2019] [Accepted: 10/27/2019] [Indexed: 12/30/2022]
Abstract
Primary central nervous system melanoma is rare and characterized by a variable prognosis, and no current treatment guidelines exist. We describe the clinical course of a 70-year-old female patient diagnosed with primary leptomeningeal melanoma (LMN) whose case represents the diagnostic and management challenges of this tumor. Targeted genomic sequencing of 315 genes from this tumor revealed GNAQ Q209L mutation and low (4 mutations/Megabase) tumor mutation burden (TMB). Wild-type NRAS, KIT, and BRAF were also observed. A cohort of 4,787 melanomas was subsequently analyzed to identify additional primary central nervous system melanomas, of which 10 additional tumors met pathologic criteria (0.21% of total melanoma cohort). These tumors were genomically assessed according to the same targeted sequencing panel, and 6 of the tumors were also found to harbor a GNAQ mutation. All 10 tumors had low (less than or equal to 2 mutations/Megabase) TMB indicating a potential trend between G-protein-coupled receptor (GPCR) alterations and low TMB in LMNs. GPCR alterations were found to significantly correlate with TMB across the cohort of 4,787 melanomas, supporting this potential finding in the limited LMN subset.
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Affiliation(s)
- Shannon Fortin Ensign
- Scripps Translational Science Institute, The Scripps Research Institute, La Jolla, California.,Department of Internal Medicine, Scripps Clinic/Scripps Green Hospital, La Jolla, California
| | - Kathryn Bollin
- Department of Medical Oncology, Scripps Clinic/Scripps Green Hospital, La Jolla, California
| | | | - Brian R Hinds
- Department of Dermatology, University of California San Diego, La Jolla, California
| | - Michael Kosty
- Department of Medical Oncology, Scripps Clinic/Scripps Green Hospital, La Jolla, California
| | - Christopher Uchiyama
- Department of Neurosurgery, Scripps Clinic/Scripps Green Hospital, La Jolla, California
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7
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Sharma A, Sinha VD. Primary Spinal Cord Melanoma of Intradural Extramedullary Origin. J Neurosci Rural Pract 2019; 10:522-525. [PMID: 31595127 PMCID: PMC6779565 DOI: 10.1055/s-0039-1697559] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022] Open
Abstract
Primary malignant melanoma is the very rare entity of the central nervous system. It accounts only 1% of all melanoma cases. A 67-year-old patient presented to us with lower back pain radiating to the left lower limb and progressive weakness of both lower limbs. She underwent magnetic resonance imaging scan of lumbosacral region that suggested T1 hyperintense, T2 hypointense, extramedullary altered signal intensity lesion at L1 and L2 vertebral level compressing terminal spinal cord, and cauda equine region. On histopathological examination, findings were suggestive of malignant melanoma that was confirmed with immunohistochemistry examination (positive for S-100, Melan-A, and HMB-45). Here, we are discussing an unusual case of primary malignant melanoma of conus and cauda equine region with comprehensive review regarding management and prognosis of the tumor.
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Affiliation(s)
- Ashish Sharma
- Department of Neurosurgery, Sawai Man Singh Medical College, Jaipur, Rajasthan, India
| | - Virendra Deo Sinha
- Department of Neurosurgery, Sawai Man Singh Medical College, Jaipur, Rajasthan, India
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8
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Hironaka K, Tateyama K, Tsukiyama A, Adachi K, Morita A. Hydrocephalus Secondary to Intradural Extramedullary Malignant Melanoma of Spinal Cord. World Neurosurg 2019; 130:222-226. [PMID: 31302270 DOI: 10.1016/j.wneu.2019.07.046] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/06/2019] [Revised: 07/02/2019] [Accepted: 07/03/2019] [Indexed: 01/07/2023]
Abstract
BACKGROUND Hydrocephalus secondary to spinal cord tumors is rare. CASE DESCRIPTION We present a 39-year-old male with gradual-onset headache whose initial diagnosis was cerebral aneurysm and communicating hydrocephalus. The correct diagnosis was primary intradural extramedullary malignant melanoma of the spinal cord. Initial brain magnetic resonance imaging demonstrated slight dilation of cerebral ventricles and a 3-mm unruptured anterior communicating artery aneurysm. He was placed under observation therapy. Two months later he was seen again due to severe headache. There was no intracranial hemorrhage on brain computed tomography scans. As we suspected rupture of the aneurysm, we operated on him for surgical clipping; however, there was no aneurysmal rupture. We found no lesions responsible for hydrocephalus, so we placed a ventriculoperitoneal shunt. His headache subsequently resolved. Nine months later he developed gait disturbance; a large volume of ascites was observed. Gadolinium-enhanced lumbar magnetic resonance imaging revealed an intradural extramedullary mass at the L-1 to S-5 level. Cytology and immunohistochemistry of the cerebrospinal fluid and ascites identified a few atypical cells positive for HMB-45, S-100 protein, and Melan-A. Whole-body examinations detected no primary lesions outside the central nervous system. Our final diagnosis was primary intradural extramedullary malignant melanoma of the spinal cord with cerebrospinal fluid dissemination. CONCLUSIONS Our findings indicate that communicating hydrocephalus may be due to primary malignant melanoma of the spinal cord.
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Affiliation(s)
- Kohei Hironaka
- Department of Neurological Surgery, Nippon Medical School Musashi Kosugi Hospital, Kawasaki, Kanagawa, Japan.
| | - Kojiro Tateyama
- Department of Neurological Surgery, Nippon Medical School Musashi Kosugi Hospital, Kawasaki, Kanagawa, Japan
| | - Atsushi Tsukiyama
- Department of Neurological Surgery, Nippon Medical School Hospital, Kawasaki, Kanagawa, Japan
| | - Koji Adachi
- Department of Neurological Surgery, Nippon Medical School Musashi Kosugi Hospital, Kawasaki, Kanagawa, Japan
| | - Akio Morita
- Department of Neurological Surgery, Nippon Medical School Hospital, Kawasaki, Kanagawa, Japan
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9
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Zhang M, Liu R, Xiang Y, Mao J, Li G, Ma R, Sun Z. Primary Spinal Cord Melanoma: A Case Report and a Systemic Review of Overall Survival. World Neurosurg 2018; 114:408-420. [PMID: 29614357 DOI: 10.1016/j.wneu.2018.03.169] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/01/2018] [Revised: 03/22/2018] [Accepted: 03/23/2018] [Indexed: 11/28/2022]
Abstract
BACKGROUND The incidence of primary spinal cord melanoma (PSCM) is rare. Several case series and case reports have been published in the literature. However, the predictive factors of PSCM survival and management options are not discussed in detail. METHODS We present a case of PSCM; total resection was achieved and chemotherapy was given postoperatively. A comprehensive search was performed on PubMed's electronic database using the words "primary spinal cord melanoma." Survival rates with various gender, location, treatment, and metastasis condition were collected from the published articles and analyzed. RESULTS Fifty nine cases were eligible for the survival analysis; 54% were male and 46% were female. Patient sex did not influence overall survival. The most common location was the thorax. Patient sex and tumor location did not influence overall survival. The major presenting symptoms were weakness and paresthesia of the extremities. Metastasis or dissemination was noted in 45.16% of 31 patients. In the Kaplan-Meier survival analysis, patients who had metastasis had the worst prognosis. Extent of resection was not related to mortality. Patients who received surgery and surgery with adjuvant therapy had a better median survival than did those who had adjuvant therapy alone. Prognosis was worst in those patients who underwent only adjuvant therapy without surgery (5 months). CONCLUSIONS Surgery is the first treatment of choice in treating PSCM. The goal of tumor resection is to reduce symptoms. Adjuvant therapy after surgery had a beneficial effect on limiting the metastasis.
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Affiliation(s)
- Mingzhe Zhang
- Department of Neurosurgery, Harrison International Peace Hospital, Hebei Medical University, Hebei, China
| | - Raynald Liu
- Beijing Neurosurgical Institute, Capital Medical University, Beijing, China
| | - Yi Xiang
- Department of Neurosurgery, Harrison International Peace Hospital, Hebei Medical University, Hebei, China
| | - Jianhui Mao
- Department of Neurosurgery, Harrison International Peace Hospital, Hebei Medical University, Hebei, China
| | - Guangjie Li
- Department of Neurosurgery, Harrison International Peace Hospital, Hebei Medical University, Hebei, China
| | - Ronghua Ma
- Department of Neurosurgery, Harrison International Peace Hospital, Hebei Medical University, Hebei, China
| | - Zhaosheng Sun
- Department of Neurosurgery, Harrison International Peace Hospital, Hebei Medical University, Hebei, China.
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10
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Wuerdeman M, Douglass S, Abda RB, Krasnokutsky M. A rare case of primary spinal cord melanoma. Radiol Case Rep 2018; 13:424-426. [PMID: 29904488 PMCID: PMC6000063 DOI: 10.1016/j.radcr.2018.01.009] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/10/2017] [Accepted: 01/04/2018] [Indexed: 12/26/2022] Open
Abstract
Primary central nervous system melanoma is rare, accounting for approximately 1% of total melanoma cases (1-3). Primary central nervous system melanoma localized to the spinal cord is even rarer. The cervical and thoracic levels are most frequently involved. There are no pathognomonic imaging characteristics and diagnosis must be confirmed immunohistologically. The preferred treatment is gross total resection. Utilization of adjuvant radiotherapy and chemotherapy may improve disease-free survival. The prognosis for primary central nervous system melanoma is generally better than that of metastatic and cutaneous melanoma, although there are relatively few cases from which to draw conclusions. We report a case of a 64-year-old woman diagnosed with primary spinal melanoma of the thoracic spine treated with subtotal surgical resection followed by adjuvant radiation therapy.
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11
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Primary Extradural Melanoma Arising in Cervical Spinal Nerve Root. World Neurosurg 2017; 111:211-215. [PMID: 29288844 DOI: 10.1016/j.wneu.2017.12.117] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/14/2017] [Revised: 12/15/2017] [Accepted: 12/18/2017] [Indexed: 11/22/2022]
Abstract
BACKGROUND Primary cervical melanoma is rare; this is especially true of extradural melanoma arising in the nerve root. Characterizing these cases can provide a basis for improved melanoma management. CASE DESCRIPTION A 42-year-old female patient with numbness and pain in the right shoulder and arm persisting for 5 years was admitted. Preoperative magnetic resonance imaging revealed an epidural ladle-shaped mass shape beyond the C7-T1 intervertebral foramen that resembled a nerve sheath tumor. Histopathologic analysis following complete resection confirmed the lesion as malignant melanoma. Radiotherapy and temozolomide and cisplatin chemotherapy were administered in an accompanying hospital with regular follow-ups at our institution. After 2 months of postoperative adjuvant therapy, hepatic metastatic lesions developed. The chemotherapy regimen was changed to carboplatin, nab-paclitaxel, and recombinant human endostatin injections for a 6-month period. Follow-up 8 months after chemotherapy (16 months post surgery) indicated a good prognosis. CONCLUSIONS In the past 10 years only 1 case of primary extradural spinal melanoma in cervical intervertebral foramen has been described, with no reported cases of hepatic metastasis. As radiographic results are not unequivocal, a diagnosis of spinal melanoma must be based on postoperative histologic confirmation. However, to date there is no standard postoperative adjuvant therapy for these patients. The chemotherapy regimen described in this report has broader implications for melanoma treatment.
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12
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Primary Intradural Extramedullary Spinal Melanoma in the Lower Thoracic Spine. Case Rep Oncol Med 2016; 2016:3815280. [PMID: 27127667 PMCID: PMC4835629 DOI: 10.1155/2016/3815280] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/22/2016] [Accepted: 03/21/2016] [Indexed: 11/17/2022] Open
Abstract
Background Context. Up to date, only four cases of primary intradural extramedullary spinal cord melanoma (PIEM) have been reported. No previous reports have described a case of PIEM located in the lower thoracic spine with long-term follow-up. Purpose. Demonstrating an unusual, extremely rare case of melanoma manifestation. Study Design. Case report. Methods. We report a case of a 57-year-old female suffering from increasing lower extremity pain, left-sided paresis, and paraesthesia due to spinal cord compression caused by PIEM in the lower thoracic spine. Results. Extensive investigation excluded other possible primary melanoma sites and metastases. For spinal cord decompression, the tumor at level T12 was resected, yet incompletely. Adjuvant radiotherapy was administered two weeks after surgery. The patient was recurrence-free at 104 weeks after radiotherapy but presents with unchanged neurological symptoms. Conclusion. Primary intradural extramedullary melanoma (PIEM) is extremely rare and its clinical course is unpredictable.
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13
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Liu QY, Liu AM, Li HG, Guan YB. Primary spinal melanoma of extramedullary origin: a report of three cases and systematic review of the literature. Spinal Cord Ser Cases 2015; 1:15003. [PMID: 28053708 DOI: 10.1038/scsandc.2015.3] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/05/2014] [Revised: 01/12/2015] [Accepted: 01/14/2015] [Indexed: 02/06/2023] Open
Abstract
STUDY DESIGN A review of the literature and three case reports. OBJECTIVES Primary spinal melanoma (PSM) of extramedullary origin is a rare malignant condition with limited current literature in regards to its clinical course, magnetic resonance imaging (MRI) findings, treatment strategies and prognosis. We reported here three cases of PSM of extramedullary origin. SETTING China, Guangzhou. METHODS We report three cases of PSM of extramedullary origin. The clinical and radiological findings of these cases were retrospectively analyzed. RESULTS The three cases were all of males aged 39, 47 and 76 years, respectively. The duration of their symptoms was 3 weeks, 2 months and 11 months respectively. The extramedullary tumors were all well-defined solitary tumors and were located at C4-5, L2-3 and T9-10, respectively. In one case, involvement of the intervertebral foramen was found. Preoperative MRI showed hyperintense T1W signals and hypointense T2W signals in all three cases and all tumors were clinically misdiagnosed as schwannomas. The patients received total or subtotal resection surgery without radiotherapy or chemotherapy. Patients were alive at 18 months, 27 months and 36 months postoperative follow-up, respectively. CONCLUSION PSM of extramedullary origin is a rare malignant tumor that shows characteristic findings on MRI. Surgical resection is the preferred treatment strategy.
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Affiliation(s)
- Q-Y Liu
- Department of Radiology, Zengcheng People's Hospital, Zengcheng, Guangdong Province, China; Department of Radiology, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, Guangdong Province, China
| | - A-M Liu
- Division of Neurosurgery, Department of Surgery, Sun Yat-sen Memorial Hospital, Sun Yat-sen University , Guangzhou, Guangdong Province, China
| | - H-G Li
- Department of Pathology, Sun Yat-sen Memorial Hospital, Sun Yat-sen University , Guangzhou, Guangdong Province, China
| | - Y-B Guan
- Department of Radiology, First Affiliated Hospital of Guangzhou Medical University , Guangzhou City, Guangdong Province, China
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14
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Tregnago AC, Furlan MV, Bezerra SM, Porto GCLM, Mendes GG, Henklain JVR, Pinto CAL, Kowalski LP, de Carvalho GB, Costa FD. Orbital melanocytoma completely resected with conservative surgery in association with ipsilateral nevus of Ota: report of a case and review of the literature. Head Neck 2014; 37:E49-55. [PMID: 24989678 DOI: 10.1002/hed.23828] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 06/29/2014] [Indexed: 12/17/2022] Open
Abstract
BACKGROUND Melanocytomas are rare pigmented primary lesions of the central nervous system arising from melanocytes of leptomeninges. They occur most frequently in the posterior fossa, Meckel's cave, or along the cervical and thoracic spinal cord. Orbital melanocytomas have been rarely reported. Nevus of Ota is a melanocytic lesion that can be associated with cutaneous and meningeal melanocytic neoplasms. METHODS AND RESULTS We describe a case of an orbital melanocytoma associated with ipsilateral Nevus of Ota. A 28-year-old man presented with proptosis and an ipsilateral congenital facial melanocytic lesion (Nevus of Ota). After imaging evaluation, a retro-orbital mass was discovered. A needle biopsy was performed and the diagnosis of melanocytoma rendered. The patient underwent complete surgical excision of the lesion. CONCLUSION In order to make the correct diagnosis and to choose the appropriate therapy, it is important to be aware of this rare presentation and its association with Nevus of Ota.
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Affiliation(s)
- Aline C Tregnago
- Department of Anatomic Pathology, A. C. Camargo Cancer Center, São Paulo, Brazil
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Cicuendez M, Paredes I, Munarriz PM, Hilario A, Cabello A, Lagares A. Primary melanoma of the cauda equina: Case report and review of the literature. Neurocirugia (Astur) 2012; 23:112-5. [PMID: 22561234 DOI: 10.1016/j.neucir.2012.02.003] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/16/2011] [Accepted: 02/02/2012] [Indexed: 02/07/2023]
Abstract
The authors report the case of an 82 year-old woman with a primary malignant melanoma of the cauda equina resembling lumbar schwannoma in the MRI study. Melanocytic neoplasms are very rare but they should be included in the differential diagnosis of lesions involving the spinal nerves. The treatment of choice for these lesions is complete resection followed by radiotherapy. The outcomes reported in the literature are variable and are associated with the age of presentation, histopathological findings, extent of surgical resection and absence of metastatic lesions.
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Affiliation(s)
- Marta Cicuendez
- Department of Neurosurgery, Hospital 12 de Octubre, Madrid, Spain
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Liubinas SV, Maartens N, Drummond KJ. Primary melanocytic neoplasms of the central nervous system. J Clin Neurosci 2010; 17:1227-32. [PMID: 20558070 DOI: 10.1016/j.jocn.2010.01.017] [Citation(s) in RCA: 121] [Impact Index Per Article: 8.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/20/2009] [Revised: 01/10/2010] [Accepted: 01/17/2010] [Indexed: 12/12/2022]
Abstract
Primary melanocytic neoplasms of the central nervous system (CNS) are rare lesions arising from melanocytes of the leptomeninges. They include diffuse leptomeningeal melanocytosis or melanomatosis, melanocytoma and primary malignant melanoma. We have reviewed the English literature regarding these lesions, which consists of case reports and a small number of larger case series. The presenting features, radiological, surgical and histological findings are reviewed, as are current management options and prognosis. We also present illustrative case reports of diffuse leptomeningeal melanocytosis and primary melanoma of the CNS.
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Affiliation(s)
- Simon V Liubinas
- Department of Neurosurgery, The Royal Melbourne Hospital, Grattan Street, Parkville, Victoria 3050, Australia.
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Abstract
STUDY DESIGN Case report. OBJECTIVE We report a case of primary intradural extramedullary melanoma of the cervical spinal cord in a nonwhite patient. SUMMARY OF BACKGROUND DATA Melanoma occurs most commonly in white populations and is rare in Asian populations. Primary malignant melanoma of the spinal cord is a rare disease entity that predominately affects the middle or lower thoracic spine. METHODS A 39-year-old man presented with a tingling sensation in the upper extremities accompanied by motor weakness of the lower extremities. Preoperative magnetic resonance imaging (MRI) of the cervical spine suggested a multiloculated subdural hematoma in the subacute stage that spread from the level of C1 to the level of C6. RESULTS A standard posterior midline approach was used under the impression that the subacute subdural hematoma was caused by a hidden vascular anomaly or a rare, intradural, pigmented tumor. A dark black-colored, hard, intradural mass was found, and gross total removal was performed. Histopathological investigation confirmed malignant melanoma. No hypermetabolic lesions were noted on whole-body FDG-PET. Additional dermatologic and ophthalmologic examinations did not reveal any other foci of primary melanoma. The patient was discharged with improvement of motor weakness. Subsequent radiotherapy and chemotherapy were administered. CONCLUSION Unlike most cases of primary intradural melanoma, this patient presented with unusual radiologic findings in the cervical spinal cord. The case described in the present study illustrates that primary spinal cord melanoma is rare and must be diagnosed with caution due to its variable clinical and radiologic presentation.
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