WNT‐activated medulloblastoma with melanotic and myogenic differentiation: Report of a rare case. Issue 4 (12th February 2016)

• Record Type: Journal Article
• Authors: Rajeshwari, Madhu; Kakkar, Aanchal; Nalwa, Aasma; Suri, Vaishali; Sarkar, Chitra; Satyarthee, Guru Dutta; Garg, Ajay; Sharma, Mehar Chand
• Is Part Of: Neuropathology. Volume 36:Issue 4(2016:Aug.)
• Page Start: 372
• Page End: 375
• Publication Date: 2016-02-12
• Subjects: medulloblastoma -- melanotic medullomyoblastoma -- myogenic -- pigmented -- WNT; Nervous system -- Diseases -- Periodicals; Nervous system -- Pathophysiology -- Periodicals; 616.8047
• DOI: 10.1111/neup.12281
• Languages: English
• ISSNs: 0919-6544

Abstract

Abstract : Medulloblastoma (MB) with melanotic and myogenic differentiation, previously known as melanotic medullomyoblastoma, is an extremely rare histological variant of MB showing melanocytic as well as skeletal muscle differentiation. Only 10 cases of this rare tumor have been reported in the literature to date. We report this case of a 2‐year‐old male child who presented with a midline cerebellar mass, which on histopathological examination showed classic MB intermixed with cells containing melanin pigment, along with rhabdomyoblasts, spindle cells and occasional strap cells, which corresponded to WNT subgroup on molecular classification. The cell of origin of this MB variant is likely to be neural crest‐derived stem cells which are capable of multilineage differentiation. Significant findings from previous reports and important differential diagnoses are discussed. Documentation of these tumors is important to characterize the clinical behaviour and to identify distinct genetic features, if any.