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Neurocutaneous melanosis (NCM) is a rare phakomatosis that may be associated with intracerebral masses. The differential diagnosis of intracerebral masses in NCM is often challenging and should include pigmented and nonpigmented lesions.

management of acute type B aortic intramural hematoma (AIH) still represents a challenging issue. Although most resolve spontaneously or with conservative therapy, several cases of AIH may complicate into classic aortic dissection with subsequent risk of aortic rupture and visceral malperfusion, thus needing urgent or preemptive thoracic endovascular aneurysm repair (TEVAR). Despite the long-term aorta-related survival, TEVAR might lead to graft obstruction, migration, infection, stroke/paraplegia, visceral ischemia, endoleak and, last but not least, retrograde aortic dissection (AD), frequent in the acute phase and associated with a high mortality risk. In order to highlight such a close relationship between AIH and AD and the possibility to perform endovascular treatment, we report the experience of an adult female patient with an aortic intramural hematoma evolving into a classic aortic dissection. Despite successful thoracic endovascular aneurysm repair (TEVAR), our patient developed an aortic dissection type A at one month with subsequent indication for cardiac surgery, still representing the elective approach in case of pathologies including the ascending aorta. Thus, the aim of our discussion is to create a debate on the most appropriate management for the treatment of descending AIH.

Background: Neurocutaneous melanosis (NCM) is a rare nonfamilial phakomatosis characterized by the presence of congenital melanocytic nevi and abnormal melanocytes infiltration of the leptomeninges. Objective & Methods: This paper shows the importance of early diagnosis and the most important imaging features of the disease on CT and MR scans. PubMed database was searched from January 1972 to September 2020. Papers including imaging findings of NCM, clinical, follow-up, and treatment features were collected, selecting only 89 studies. Discussion: NCM is a term used for the first time by van Bogaert in 1948. It refers to a condition caused by an error during morphogenesis and migration leading to leptomeningeal melanocytic accumulation. Although histological findings are the gold standard for diagnosis confirmation, neuroimaging and clinical features strongly support the suspect of NCM. Localization and extension of the lesions are predictive of neurological manifestations related to increased intracranial pressure, mass lesions, or spinal cord compression. CT demonstrates sites of increased density in the anterior temporal lobe - mainly the amygdala - thalami, cerebellum, and frontal lobes base. However, MRI is the best imaging method to diagnose central nervous system lesions, often appearing as T1-short signal areas of the cerebral parenchyma, indicative of central nervous system melanosis. MRI can also reveal associated intracranial and intraspinal abnormalities. Conclusion: Early imaging, when available, is helpful if NCM suspect is raised and may be of guidance in comparing later studies. NCM requires a multidisciplinary approach since it is a multisystem disease with a genetic component.