Primary cerebellopontine angle melanoma: case report and review

Abstract

Primary melanomas at the cerebellopontine (CP) angle are extremely rare and considered a diagnostic dilemma. With only 16 prior cases reported so far, there is not enough material in world literature. We report a 29-year-old male who presented with vertigo, headache and features of lower cranial nerve involvement with MRI revealing a melanotic lesion at CP angle. The patient underwent right suboccipital craniectomy and only subtotal excision of the tumor was possible. Histopathologically it turned out to be malignant melanoma. Since a thorough search for any possible primary was futile, it was considered a primary melanoma. Patient was given adjuvant radiotherapy. Ten months later, he presented with weakness and multiple spinal metastases and two months later he succumbed to his disease. We did a comprehensive review of literature about various melanocytic tumors at the CP angle. Pathologically, melanocytic tumors range from benign melanocytomas to malignant melanoma with variable prognosis. The clinical presentation depends upon whether the lesions are diffuse or discrete. Surgery is the primary modality of treatment and the amount of tumor excised is related to the prognosis. We conclude the discussion with a novel classification for melanotic tumors of CNS and the current diagnostic pathway for a melanotic lesion at the CP angle.KeywoRds: Cerebellopontine angle melanoma, Intracranial melanoma, Primary melanoma ÖZ Serebellopontin (CP) açıda primer melanomlar çok nadirdir ve tanısal bir ikilem olarak değerlendirilir. Şu ana kadar sadece 16 vaka bildirilmiştir ve henüz dünya literatüründe yeterli bilgi yoktur. Baş dönmesi, başağrısı ve alt kraniyal sinir tutulumu bulguları ile gelen ve MRG'nin CP açıda bir melanotik lezyon gösterdiği 29 yaşında bir erkek hasta sunuyoruz. Hastaya sağ suboksipital kraniektomi yapıldı ve tümör ancak subtotal olarak eksize edilebildi. Histopatolojik olarak malign melanom olduğu saptandı. Herhangi bir olası primer açısından kapsamlı bir araştırma yapmak anlamsız olduğundan primer melanom kabul edildi. Hastaya adjuvan radyoterapi verildi. Hasta on ay sonra zayıflık ve çok sayıda spinal metastaz ile geldi ve iki ay sonra hastalığı nedeniyle öldü. CP açıda çeşitli melanostik tümörler hakkında literatürü kapsamlı olarak araştırdık. Patolojik olarak melanostik tümörler benign melanositomlardan değişken prognozlu malign melanoma kadar değişmektedir. Klinik sunum lezyonların difüz veya tek tek olmasına göre değişir. Cerrahi temel tedavi modalitesidir ve eksize edilen tümör miktarı prognozla ilişkilidir. Tartışmayı MSS melanotik tümörleri için yeni bir sınıflandırma ve CP açıda bir melanotik lezyon için mevcut diagnostik yaklaşımı sunarak bitiriyoruz.ANAHTAR sÖZCÜKLeR: Serebellopontin açı melanomu, İntrakraniyal melanom, Primer melanom

Intracranial malignant melanoma: A report of 7 cases

“…In conclusion, the prognosis of intracranial malignant melanoma is determined by the following factors: i) The type of lesion; ii) the involvement of the leptomeninges; iii) the extent of tumor excised; and iv) the molecular immunology borstel number 1 (MIB 1) antibody index, which is the most relevant factor for prognosis in this type of cancer ( 1 ).…”

“…It is particularly rare in the brain compared with other areas of the CNS and accounts for 0.07–0.17% of all intracranial neoplasms. Depending on the behavior of the tumor, brain melanomas are classified into the following categories: i) Well-differentiated melanocytoma; ii) intermediate grade melanocytoma; and iii) primary malignant melanoma ( 1 ). CNS primary malignant melanoma only accounts for ~1% of all cases of melanoma ( 2 ).…”

Melanoma maligno primario del sistema nervioso central: un reto diagnóstico

“…Se presenta en un rango de edad entre 35 a 50 años 7 . Bhandari et al 2 refieren que fue Virchow quien, en 1859, describió el primer caso de melanoma intracraneal primario difuso, y Oogle reportó en 1899 el primer caso de melanoma primario solitario intracraneal 7,8 . Estos tumores surgen de melanocitos presentes en las leptomeninges de la convexidad cerebral, de la base del cráneo, fosa posterior, canal cervical espinal, la piamadre que recubre los vasos, formación reticular del puente y médula, sustancia negra y locus coerulleus 7 .…”

Primary Malignant Melanoma of Brainstem Medulla Mimicking as Cavernoma – Case Report

“…Primary malignant melanoma of central nervous system is very rare and accounts for 0.07% of all brain tumors [1]. It has very low incidence, estimated at 0.9 per 10 million inhabitants [2]. Primary CNS melanoma arises from melanocytes which have been developed from melanoblasts in the neural crest.…”