An Unusual Variant of Anlage Tumor of Pineal Region in an Infant

Abstract

A 9-month-old male child was brought with complaints of increasing head size for 2 months, increasing lethargy and vomiting for the last 2 days. Radiology revealed a heterogeneously enhancing, globular lesion in the pineal region with hydrocephalus. Near total excision of the tumor was carried out. The histopathological examination of the lesion showed heterogenous elements in the form of mature neuroepithelial and ectomesenchymal tissue. The pathology and radiology of this unusual lesion is discussed with relevant review of literature.

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., 2,3,5,7-10 although two of the nine reported PAT cases did not have an immature neoplastic component and had a good prognosis (Table 2). 4,6 The outcome of the present case could not be determined because the patient was transferred. However, the histopathological features and high MIB-1 labeling index suggest that he might have a poor prognosis.…”

“…Tumors that occur in the pineal region are of three major types, namely germ‐cell, pineal parenchymal and glial‐cell tumors. PB have a prevalence between 24% and 50% of pineal parenchymal tumors and have the worst prognosis . PATs constitutes a rare subtype of PBs…”

“…Pineal region neoplasms are rare, with a prevalence between 0.4% and 1.0% of all primary brain tumors. 6,11 Tumors that occur in the pineal region are of three major types, namely germ-cell, pineal parenchymal and glial-cell tumors. PB have a prevalence between 24% and 50% of pineal parenchymal tumors and have the worst prognosis.…”

“…PB have a prevalence between 24% and 50% of pineal parenchymal tumors and have the worst prognosis. 6,11 PATs constitutes a rare subtype of PBs. 1,2 PATs were first described by Schmidbauer et al 2 and are considered as PBs with melanin production as well as cartilaginous and rhabdomyoblastic differentiation, 1 or primitive pineal tumors with neuroepithelial and ectomesenchymal differentiation.…”