A pineal tumour with features of ?Pineal anlage tumour?

Abstract

A primitive tumour of the pineal region in a 9 month-old boy caused hydrocephalus, ataxic gait and paraplegia leading to death after 9 months of evolution. Histological and histochemical examination revealed a pinealoblastoma with melanotic cells as well as cartilaginous formations. The leptomeningeal seeding of the tumour showed neuroblastic and neuronal differentiation. A diagnosis of pineoblastoma with neuroepithelial and ectomesenchymal differentiation was made. This tumour belongs to a recently defined category of pineal tumours, called pineal anlage tumours.

A rare case of a pineoblastoma with a rhabdomyoblastic component

“…In the cerebellum, medullomyoblastoma (MMb) is recognized as a subtype of medulloblastoma and is characterized by the presence of a rhabdomyoblastic component . PAT is known as a PB subtype with pathological features characterized by the presence of both PB and ectomesenchymal component with melanin and cartilaginous tissue and/or rhabdomyodifferentiation . This case had pathological features similar to MMb and somewhat different from previously reported PAT cases.…”

“…Most PAT cases are reported as poorly differentiated pineal parenchymal tumors with ectomesenchymal components., although two of the nine reported PAT cases did not have an immature neoplastic component and had a good prognosis (Table ) . The outcome of the present case could not be determined because the patient was transferred.…”

Histopathology of Tumors of the Pineal Region

“…Melanin pigment may be present in rare PBs [45,68,[70][71][72]. This pigment has been found in the human fetal and post-natal pineal gland [16,73,74].…”

“…Neoplastic differentiation may recall some features present during the developmental stages of the pineal gland. The designation of pineal anlage tumor is proposed for tumors in which, in addition to pineoblastomatous components, mesenchymal elements differentiating along striated muscle or chondroblastic lines are observed [71,72,75].…”

Pineal anlage tumour - a rare entity with divergent histology

“…Since then only five patients have been reported. [1][2][3][4][5] Pineal anlage tumour is a rare tumor that is not listed in the 2007 World Health Organization classification of tumours of the central nervous system. 6 2.…”