Neurocutaneous melanosis with intraspinal malignant melanoma in adults-an extremely rare clinical entity: A case report with review of literature
Dongkang Liu1,2, Yujun Wei1, James Wang2, Guihuai Wang2
1. Medical Center, Tsinghua University, Beijing, 100084, China;
2. Department of Neurosurgery, Beijing Tsinghua Changgung Hospital, Medical Center, Tsinghua University, Beijing 102218, China
Neurocutaneous melanosis with intraspinal malignant melanoma in adults-an extremely rare clinical entity: A case report with review of literature
Dongkang Liu1,2, Yujun Wei1, James Wang2, Guihuai Wang2
1. Medical Center, Tsinghua University, Beijing, 100084, China;
2. Department of Neurosurgery, Beijing Tsinghua Changgung Hospital, Medical Center, Tsinghua University, Beijing 102218, China
摘要 Objective: Neurocutaneous melanosis (NCM) in adult patients was rare, and few cases had been reported. In this report, we summarized the features of NCM in adult patients for providing useful information about this rare clinical entity. Methods: In this report, we present a case of a 41-year-old woman, who was eventually diagnosed with NCM with intraspinal malignant melanoma. The clinical features, treatments, and prognosis of cases of NCM in adults were thoroughly reviewed and discussed. Results: A 41-year-old woman was eventually diagnosed with NCM with intraspinal malignant melanoma. The post-operative course was uneventful and her symptoms significantly improved (VAS: 3 points). However, she developed symptoms of intracranial hypertension and deteriorated rapidly after 11 months of operation, and died 1 month later. To date, only 15 cases of NCM in adults have been reported in the English literature. Analyses showed that the average age was 33.8 years (range 19-65 years), and 11 patients (68.8%) were between 20 and 40 years. A slight male predominance (M:F/11:5) was found. For the 12 cases which detailed follow-up information was available, 9 patients (75%) died, with a survival time ranging from 1 day to 29 months post-operation. Death occurred within the first month of operation in 6 cases (46.2%). The median survival time was 3 months. Conclusions: We presented a rare case of NCM with intraspinal malignant melanoma in an adult patient. Although rare, this clinical entity causes significant mortality and has poor prognosis.
Abstract: Objective: Neurocutaneous melanosis (NCM) in adult patients was rare, and few cases had been reported. In this report, we summarized the features of NCM in adult patients for providing useful information about this rare clinical entity. Methods: In this report, we present a case of a 41-year-old woman, who was eventually diagnosed with NCM with intraspinal malignant melanoma. The clinical features, treatments, and prognosis of cases of NCM in adults were thoroughly reviewed and discussed. Results: A 41-year-old woman was eventually diagnosed with NCM with intraspinal malignant melanoma. The post-operative course was uneventful and her symptoms significantly improved (VAS: 3 points). However, she developed symptoms of intracranial hypertension and deteriorated rapidly after 11 months of operation, and died 1 month later. To date, only 15 cases of NCM in adults have been reported in the English literature. Analyses showed that the average age was 33.8 years (range 19-65 years), and 11 patients (68.8%) were between 20 and 40 years. A slight male predominance (M:F/11:5) was found. For the 12 cases which detailed follow-up information was available, 9 patients (75%) died, with a survival time ranging from 1 day to 29 months post-operation. Death occurred within the first month of operation in 6 cases (46.2%). The median survival time was 3 months. Conclusions: We presented a rare case of NCM with intraspinal malignant melanoma in an adult patient. Although rare, this clinical entity causes significant mortality and has poor prognosis.
Dongkang Liu, Yujun Wei, James Wang, Guihuai Wang. Neurocutaneous melanosis with intraspinal malignant melanoma in adults-an extremely rare clinical entity: A case report with review of literature[J]. 临床转化神经科学, 2016, 2(4): 236-240.
Dongkang Liu, Yujun Wei, James Wang, Guihuai Wang. Neurocutaneous melanosis with intraspinal malignant melanoma in adults-an extremely rare clinical entity: A case report with review of literature. Translational Neuroscience and Clinics, 2016, 2(4): 236-240.
20170308101928 Figure 1 (a) A giant congenital blue nevus in the patient’s back. (b) The spinal cord and cauda equina surrounded by dark black soft tissues.
20170308102006 Figure 2 Sagittal magnetic resonance (MR) images. Multi-nodules of T6 through L1, hyperintense on the T1 weighted image (a), hypointense on the T2 weighted image (b), and homogenous enhancement of the lesions and linear enhancement of the spinal pia mater on T1-weighted images (c).
20170308102059 Figure 3 Hematoxylin and Eosin (H&E) staining, ×400. Variable amounts of melanin with areas of necrosis and foci of parenchymal infiltration and nuclear pleomorphism with occasional mitotic figures are observed.
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2016, Vol. 2 
