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diffuse leptomeningeale melanocytose (aandoening)
diffuse leptomeningeale melanocytose
DLM
Diffuse leptomeningeal melanocytosis
A rare tumor of meninges arising from leptomeningeal melanocytes, characterized by diffuse infiltration of the leptomeninges (pia mater and arachnoidea) anywhere in the central nervous system. Clinical features may include stillbirth, intracranial hypertension and hydrocephalus, seizure, ataxia, syringomyelia, cranial nerve palsy, intracranial hemorrhage, sphincter dysfunction and neuropsychiatric symptoms. Transformation into malignant melanoma of the central nervous system was reported. It may be associated with congenital nevi, as a part of neurocutaneous melanosis.
Id770628008
StatusPrimitive
Associated morphologydiffuse melanocytose
Finding sitestructuur van leptomeninx
Associated morphologymelanose
Finding sitestructuur van meningen
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TargetD42.9
TermNeoplasma met onzeker of onbekend gedrag van meningen, niet gespecificeerd
SNOMED CT to Orphanet simple map252031
SNOMED CT to ICD-10 extended map
TargetD42.9
RuleTRUE
AdviceALWAYS D42.9 | POSSIBLE REQUIREMENT FOR MORPHOLOGY CODE
CorrelationSNOMED CT source code to target map code correlation not specified