Intracranial melanocytic meningeal tumours and melanosis oculi: case report and literature review

BMC Cancer

Case Report

Gelareh Zadeh¹  Pasquale De Bonis²  Nicola Montano²  Giulio Maira²  Roberto Pallini²  Francesco Doglietto³  Libero Lauriola⁴  Cesare Colosimo⁵  Mario Balducci⁶ 

[1] Division of Neurosurgery, Toronto Western Hospital, Toronto, ON, Canada;Institute of Neurosurgery, Catholic University School of Medicine, Rome, Italy;Institute of Neurosurgery, Catholic University School of Medicine, Rome, Italy;Catholic University School of Medicine, Institute of Neurosurgery, Largo A. Gemelli, 8 00168, Roma, Italy;Institute of Pathology, Catholic University School of Medicine, Rome, Italy;Institute of Radiology, Catholic University School of Medicine, Rome, Italy;Institute of Radiotherapy, Catholic University School of Medicine, Rome, Italy;

关键词: Melanocytoma;    Melanocytic meningeal tumour;    Intracranial melanocytosis;    Melanosis oculi;    Cavernous sinus;   

DOI  :  10.1186/1471-2407-12-220

 received in 2011-12-27, accepted in 2012-06-06,  发布年份 2012

来源: Springer

【 摘 要 】

BackgroundMelanocytic meningeal tumours are rare extra-axial neoplasms of the nervous system, with only three reported cases in the cavernous sinus. Herein we describe for the first time the association of ocular melanosis and multiple intracranial melanocytic meningeal tumours, with the presenting lesion being in the cavernous sinus. The importance of this association is discussed together with the diagnostic and therapeutic challenges of the case.Case presentationA 20-year-old man presented with a left sixth cranial nerve deficit; general examination documented only congenital melanosis of the homolateral eye. MRI examination showed a space occupying lesion in the left cavernous sinus, which was followed conservatively for 2 years, until a new space occupying lesion was evident at the level of the right frontal convexity: both lesions presented with neuroradiological characteristics suggestive of melanin content.The frontal convexity lesion was removed: intraoperatively the dura was markedly and diffusely melanotic. Histological examination documented a melanocytic meningeal tumour, with a proliferative index of 3 %. The patient underwent 3D-Conformal Radiation Therapy on the lesion of the cavernous sinus (total dose 5040 cGy), with initial tumour reduction. Three years later, due to a symptomatic growth, he underwent partial removal of the lesion in the cavernous sinus. Histological examination was unchanged. He then received adjuvant Temozolomide with Low Dose Fractionated Radiation Therapy (LD-FRT). Due to further disease progression cisplatin plus fotemustine were administered, concomitant with LD-FRT: after two cycles MRI documented significant disease regression. After a period of apparent disease control, the patient presented with persistent cough and evidence of multiple thoracic metastases, which lead to his death, seven years after presentation.ConclusionsIntracranial melanocytic meningeal tumours are challenging lesions, both from a diagnostic and therapeutic point of view; though rare, the possible association with ocular melanosis should be recognized and might facilitate an early diagnosis. Surgery remains the best possible option when feasible. In the event of partial resection, this “benign” disease might be clinically aggressive.

【 授权许可】

CC BY   
© Doglietto et al.; licensee BioMed Central Ltd. 2012