Neurocutaneous melanosis with an intracranial cystic-solid meningeal melanoma in an adult:A case report and review of literature

BACKGROUND Neurocutaneous melanosis(NCM)is a rare congenital,nonhereditary neurocutaneous syndrome that mainly occurs in children;adult NCM is very rare.Due to its rarity,the clinical features and treatment strategies for NCM remain unclear.The purpose of this study was to explore the clinical features,diagnosis,treatment and prognosis of NCM in adults.Most intracranial meningeal melanomas are solid masses,and cystic-solid malignant melanomas are very rare.Due to the lack of data,the cause of cystic changes and the effect on prognosis are unknown.CASE SUMMARY A 41-year-old woman was admitted to the hospital with intermittent headache for 1 mo.Magnetic resonance imaging(MRI)showed a 4.7 cm×3.6 cm cystic-solid mass in the left temporal lobe with peritumoral edema.The entire mass was removed,and postoperative pathology indicated malignant melanoma.CONCLUSION MRI is the first-choice imaging approach for diagnosing central nervous system diseases in NCM patients,although cerebrospinal fluid may also be used.At present,there is no optimal treatment plan;gross total resection combined with BRAF inhibitors and MEK inhibitors might be the most beneficial treatment.

Neurocutaneous melanosis with intraspinal malignant melanoma in adults—an extremely rare clinical entity: A case report with review of literature

Objectives: 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(range19–65 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.

A Case of Coexistence of Aplasia Cutis Congenita and Giant Congenital Melanocytic Nevus:Coexistence of Two Rare Skin Diseases

Aplasia cutis congenita(ACC)is a rare disease that is characterized by complete or partial absence of skin at birth,either in a localized or widespread region.Melanocytic nevi refers to tumor-like malformations of the skin or mucous membrane caused by benign proliferation of melanocytes.It is classified as a giant congenital melanocytic nevus(GCMN)when the diameter of the largest nevus exceeds 20 cm.The co-occurrence of ACC and GCMN is extremely rare,to the best of our knowledge.We report a case of coexistence of ACC and GCMN of infancy in a 2-month-old male infant.The lesions consisted of a large hyperpigmented plaque occupying most of the trunk and pelvic region,and smaller hyperpigmented plaques on the trunk,head,and extremities.Additionally,there were large,sharply marginated,triangular,depressed atrophic plaques covered by thin,translucent,glistening epithelial membranes in the center of the GCMN on the back.The presumptive diagnosis was coexistence of GCMN and ACC.This could be a manifestation of SCALP syndrome,a rare neuro-cutaneous condition characterized by the presence of Sebaceous nevus,Central nervous system(CNS)malformations,Aplasia cutis congenita,Limbal dermoid and Pigmented(giant melanocytic)nevus.