Long-term survival in a child with a central nervous system medulloepithelioma

Moftakhar, Parham; Fan, Xuemo; Hurvitz, Carole H.; Black, Keith L.; Danielpour, Moise

doi:10.3171/ped.2008.2.11.349

Cited by 21 publications

(55 citation statements)

References 40 publications

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“…[25] However, their occurrence beyond this age range has also been reported. [34] This tumor was initially classified as the most primitive neoplasm of the Central Nervous System (CNS) in 1926, by Bailey and Cushing, and later as a medulloepithelioma not otherwise specified, or a medulloepithelioma with differentiation by Rorke et al .…”

Section: Discussionmentioning

confidence: 99%

“…[34] This tumor was initially classified as the most primitive neoplasm of the Central Nervous System (CNS) in 1926, by Bailey and Cushing, and later as a medulloepithelioma not otherwise specified, or a medulloepithelioma with differentiation by Rorke et al . [2] The 2007 World Health Organization (WHO) Classification of Tumors of the CNS reclassified embryonal tumors as Medulloblastoma, CNS Primitive Neuroectodermal Tumor (PNET), and Atypical Teratoid/Rhabdoid Tumor (AT/RT). CNS PNET includes CNS Neuroblastoma, CNS Ganglioneuroblastoma, Medulloepithelioma, and Ependymoblastoma (Supratentorial PNET).…”

Section: Discussionmentioning

confidence: 99%

“…Most children die within five months, and only three cases have survived beyond five years. [12679] There are only two previously reported cases in adults, aged 23 and 20 years, located in the fronto-temporal and temporal lobes. [34]…”

Section: Discussionmentioning

confidence: 99%

“…[1] These are rare tumors with only 36 cases recorded in literature with the peak incidence between six months to five years of age. [2] Medulloepithelioma presenting in the older age group is extremely rare, and only two cases have been reported. [34] We report a rare case of supratentorial medulloepithelioma in a 17-year-old girl, the first case to be reported in late adolescence.…”

Section: Introductionmentioning

confidence: 99%

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Unusual occurrence of supratentorial medulloepithelioma in a young female

Ramesh

Mahadevan

Saini

et al. 2014

J Neurosci Rural Pract

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Medulloepithelioma is an extremely rare PNET in late adolescence and adults with only two cases noted in literature. These are WHO grade IV tumors with dismal prognosis. Only few cases survived beyond 5 months. We report a rare case of supratentorial medulloepithelioma in a 17 year old girl. She had presented with right sided weakness, headache and vomiting. Imaging showed an enhancing mass lesion in left parietal region which undergone gross total resection. After surgery, her headache, vomiting and right sided weakness improved. On histopathology, the tumor had characteristic trabecular, ribbon and palisaded arrangement with brisk mitotic activity, necrosis and calcification. Immuno-histochemistry revealed positivity for Synaptophysin, Vimentin and EMA while GFAP was negative. MIB-1 labeling was very high. Patient received postoperative radiotherapy. On follow up after 14 months, she was clinically asymptomatic with no recurrence on imaging.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Unusual occurrence of supratentorial medulloepithelioma in a young female

Ramesh

Mahadevan

Saini

et al. 2014

J Neurosci Rural Pract

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“…In a recent review, Moftakhar et al [19] considered supratentorial tumor location as a characteristic predictive of a favorable outcome.…”

Section: Discussionmentioning

confidence: 99%

Curative treatment for central nervous system medulloepithelioma despite residual disease after resection

et al. 2011

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Medulloepithelioma of the central nervous system (CNS) is an uncommon primitive neuroectodermal tumor (PNET) usually occurring in early childhood. It is characterized by highly malignant behavior with a propensity for progression, recurrence, and dissemination despite intensive therapy. Due to its rarity, the optimal management is still unknown. However, gross total resection (GTR) has been considered crucial to achieve cure. In this article, the authors report on 2 cases of CNS medulloepithelioma in which long-term survival (more than 6 years) could be achieved despite evidence of, or suspected postoperative residual disease with an otherwise dismal prognosis.The patients were treated according to different strata of the protocol for primitive neuroectodermal tumors (PNET) of the German-Austrian multicenter trial of the German Society for Pediatric Oncology and Hematology (GPOH) for childhood brain tumors (HIT 2000). Treatment included postoperative hyperfractionated radiotherapy of the craniospinal axis followed by a boost to the tumor site in combination with chemotherapy. A review of the 2 reported and 37 previously published cases confirmed GTR and older age as positive prognostic factors.

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