Leptomeningeal melanoma in childhood

Abstract

The rate of incidence of MM melanoma in the U.K. is increasing, and it will represent an increasing proportion of the pediatric oncologist's workload. A consistent approach to the therapy of patients with metastatic MM and NCM is needed if we are to have any hope of offering more than palliative therapy to these children in the future.

Asymptomatic neurocutaneous melanocytosis in patients with large congenital melanocytic nevi: A study of cases from an Internet-based registry

“…Combination chemotherapy and/or radiotherapy have been attempted, but have been unsuccessful. 12,15,16 Interferon alfa-and IL-2-based immunotherapy have been ineffective for treating CNS melanoma. 6,[17][18][19][20] New treatments under exploration that may prove to be beneficial include temozolomide, an oral alkylating agent, which has the ability to cross the blood-brain barrier, and has shown activity in trials for melanoma brain metastases.…”

Childhood malignant blue nevus of the ear associated with two intracranial melanocytic tumors—metastases or neurocutaneous melanosis?

“…Although sporadic leptomeningeal melanomas are more common in adults, those associated with neurocutaneous melanosis usually develop in children. 8 Pathologically, 2 types of primary leptomeningeal melanoma can be distinguished: diffuse and nodular, with both types marked by cellular pleomorphism, mitoses, necroses, and hemorrhage. 7,8 In either form, primary leptomeningeal melanoma is usually a highly aggressive tumor, with metastases also being described outside the central nervous system, for example, to liver and bones.…”

“…8 Pathologically, 2 types of primary leptomeningeal melanoma can be distinguished: diffuse and nodular, with both types marked by cellular pleomorphism, mitoses, necroses, and hemorrhage. 7,8 In either form, primary leptomeningeal melanoma is usually a highly aggressive tumor, with metastases also being described outside the central nervous system, for example, to liver and bones. 9 Neurocutaneous melanosis is a rare congenital neuroectodermal dysplasia affecting melanocytes that migrate to the skin and pia mater.…”

Congenital melanocytic nevi: Where are we now?

“…135,150 Symptomatic patients have a poor prognosis with a high mortality rate (secondary to CNS melanoma or mechanical obstruction caused by nonmalignant melanocytes). 136,139,145,153,154 In addition, the Dandye Walker malformation has been described with NCM and suggests an even more worrisome prognosis. [155][156][157][158][159][160] Other CNS abnormalities have been reported, including lissencephaly and corpus callosum agenesis.…”

“…Patients who develop neurologic symptoms (eg, seizures, behavioral disturbances, hemiplegia, headache, vomiting, cranial nerve palsies, papilledema, lethargy, bulging fontanelles, sensorimotor deficits, bowel and bladder problems, or DandyeWalker syndrome) or fail to meet developmental guidelines should be urgently evaluated by a specialist and with imaging if indicated. 135,154,166,[178][179][180][181][182] The risk of TSC with lumbosacral CMN remains to be defined. Unlike NCM, an effective treatment exists and delayed diagnosis can result in permanent sequelae.…”