Primary primitive neuroectodermal tumor of the orbit

Das, Dipankar; Kuri, Ganesh Chandra; Deka, Panna; Bhattacharjee, Kasturi; Bhattacharjee, Harsha; Deka, Anil

doi:10.4103/0301-4738.55071

Cited by 15 publications

(18 citation statements)

References 5 publications

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“…PNET is a small round cell malignancy of primitive, neuroectodermal tissue or pluripotential, migratory neural crest cells that arises from the soft tissue or bone, commonly affecting older children and adults. [ 7 ]…”

Section: Ewing's Sarcoma and Primitive Neuroectodermal Tumormentioning

confidence: 99%

Round cell tumors: Classification and immunohistochemistry

Sharma

Kamala²,

Nair

et al. 2017

Indian J Med Paediatr Oncol

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Round cell tumors as the name suggest are comprised round cells with increased nuclear-cytoplasmic ratio. This group of tumor includes entities such as peripheral neuroectodermal tumor, rhabdomyosarcoma, synovial sarcoma, non-Hodgkin's lymphoma, neuroblastoma, hepatoblastoma, Wilms' tumor, and desmoplastic small round cell tumor. These round cells tumors are characterized by typical histological pattern, immunohistochemical, and electron microscopic features that can help in differential diagnosis. The present article describes the classification and explains the histopathology and immunohistochemistry of some important round cell tumors.

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Section: Ewing's Sarcoma and Primitive Neuroectodermal Tumormentioning

confidence: 99%

Round cell tumors: Classification and immunohistochemistry

Sharma

Kamala²,

Nair

et al. 2017

Indian J Med Paediatr Oncol

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“…In both humans and animal species, primary PNET tumors can arise within the peripheral and central nervous systems, as well as the eye . In humans, the majority of primary ocular PNET tumors are reported in the pediatric population, and most commonly include retinoblastomas and medulloepitheliomas . Although both originate from the primitive neuroepithelial cells of the optic cup, retinoblastomas are malignant tumors arising from the primitive neuroepithelium of the immature retina, while medulloepitheliomas, arise from the primitive medullary epithelium of the ciliary body .…”

Section: Introductionmentioning

confidence: 99%

“…1,[3][4][5][6] In humans, the majority of primary ocular PNET tumors are reported in the pediatric population, and most commonly include retinoblastomas and medulloepitheliomas. [7][8][9] Although both originate from the primitive neuroepithelial cells of the optic cup, retinoblastomas are malignant tumors arising from the primitive neuroepithelium of the immature retina, while medulloepitheliomas, arise from the primitive medullary epithelium of the ciliary body. 7,[10][11][12] Of these ocular primitive neuroectodermal tumors, retinoblastomas are the most frequently occurring, due in part because they are the most common primary intraocular neoplasm of children, with a reported incidence ranging from 1:17 000 to 1:24 000 live births.…”

Section: Introductionmentioning

confidence: 99%

Primary primitive neuroectodermal tumors of the retina and ciliary body in dogs

Regan

Dubielzig

Zeiss

et al. 2013

Veterinary Ophthalmology

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We describe the clinical, histological, and immunohistochemical features of primary intraocular primitive neuroectodermal tumors in eight dogs. Four of eight tumors exhibited histological features similar to human retinoblastomas characterized by Flexner-Wintersteiner rosettes, and fleurettes, and demonstrated variable immunoreactivity for retinal markers opsin, S-antigen (S-Ag) and interphotoreceptor retinoid-binding protein (IRBP). All dogs with tumors displaying histological and immunohistochemical features of retinal differentiation were ≤2 years of age. All tumors diagnosed as medulloepitheliomas (n = 4) did not display histological and immunohistochemical features of retinal differentiation and were present in dogs 7 years or older. Age of onset, in conjunction with immunohistochemistry for opsin, S-Ag, and IRBP, is an important aid in the differentiation of primary, primitive neuroectodermal tumors arising within the canine ciliary body, retina, and optic papilla.

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“…At times, it becomes difficult to differen-tiate peripheral PNET from other small, round cell tumors because they have some microscopic features and genetic abnormalities in common. 11 Small, round cells, hyperchromatic nuclei, and Homer-Wright rosettes are suggestive but not diagnostic of peripheral PNET. Peripheral PNET shows a varying degree of neural differentiation beginning with neuron-specific enolase expressivity, followed by Homer-Wright rosette formation, phenotypic ganglion cell differentiation, and neurofilament protein expression.…”

Section: Discussionmentioning

confidence: 99%