Primitive Neuroectodermal Tumor/Ewing Sarcoma of the Retina

An 11-year-old boy underwent enucleation of his left eye for an intraocular tumor. Examination showed a small, round blue cell tumor arising in the peripheral retina near the ciliary body. Immunohistochemical stain results were positive for neuron-specific enolase, synaptophysin, cluster of differentiation 99 (CD99), Friend leukemia integration 1, and CD56. Ultrastructural findings included occasional intracytoplasmic dense core granules. Polymerase chain reaction of the tumor showed a Ewing sarcoma/Friend leukemia integration gene fusion product. The tumor was classified as a primitive neuroectodermal tumor/Ewing sarcoma of the retina and should be distinguished from retinoblastoma. To our knowledge, this is the first case of primary primitive neuroectodermal tumor of the retina.

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Primitive Neuroectodermal Tumor of the Stomach

Archives of Pathology & Laboratory Medicine ◽

10.5858/2005-129-107-pntots ◽

2005 ◽

Vol 129 (1) ◽

pp. 107-110 ◽

Cited By ~ 5

Author(s):

Raoulin Soulard ◽

Valère Claude ◽

Philippe Camparo ◽

Jean-Philippe Dufau ◽

Patrick Saint-Blancard ◽

...

Keyword(s):

Polymerase Chain Reaction ◽

Young Adults ◽

Soft Tissue ◽

Ewing Sarcoma ◽

Primitive Neuroectodermal Tumor ◽

Neuron Specific Enolase ◽

Neuroectodermal Tumor ◽

Chain Reaction ◽

Children And Young Adults ◽

Polymerase Chain

Abstract Ewing sarcoma/primitive neuroectodermal tumor is classically a tumor of the soft tissue or bone in children and young adults, but several cases have been described in patients of all ages. Within the last decade, the clinicopathologic spectrum of Ewing sarcoma/primitive neuroectodermal tumor has been markedly expanded by recognition that the tumor may also have a visceral origin. We describe a case of primitive neuroectodermal tumor arising in the stomach of a 66-year-old woman. The neoplasm was excised using a radical surgical procedure. Microscopically, the tumor was made up of solid nests and sheets of round cells. Immunohistochemically, the tumor cells showed immunoreactivity for CD99, S100, neuron-specific enolase, and vimentin. A multiplex real-time polymerase chain reaction assay detected an EWS-ERG fusion. To our knowledge, this is the first description of a primitive neuroectodermal tumor arising in the stomach.

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Primary Vulvar Ewing Sarcoma/Primitive Neuroectodermal Tumor: A Report of 2 Cases and Review of the Literature

International Journal of Gynecological Cancer ◽

10.1111/igc.0b013e3181acae33 ◽

2009 ◽

Vol 19 (6) ◽

pp. 1131-1136 ◽

Cited By ~ 17

Author(s):

Handan Çetiner ◽

Gözde Kr ◽

Edward P. Gelmann ◽

Metin Ozdemirli

Keyword(s):

Ewing Sarcoma ◽

Primitive Neuroectodermal Tumor ◽

Correct Diagnosis ◽

Polymerase Chain Reaction Analysis ◽

Neuroectodermal Tumor ◽

Diagnostic Tools ◽

Fusion Product ◽

Molecular Tests ◽

S 100

Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) family of tumor is a very aggressive malignant round cell tumor characterized by translocations involving EWS-FLI1 genes. They are increasingly recognized in extraosseous sites as a result of improvements in diagnostic tools. In this paper, we report 2 additional cases arising in vulva of young adults who have been treated aggressively and have survived fore more than 7 and 4 years successively. Histologic examination showed small round (blue) cell morphology in both cases. The tumor cells contained glycogen and were positive for CD99 and vimentin and negative for keratins, lymphoid markers, S-100, synaptophysin, chromogranin, and desmin. Reverse transcriptase polymerase chain reaction analysis from paraffin-embedded tissue revealed EWS-FLI1 fusion product in 1 case. Collectively, 13 cases of vulvar ES/PNET have been reported in the literature. Only 8 cases have detailed follow-up information with an average follow-up data of 28 months. Ewing sarcoma/PNET should be considered in the differential diagnosis of any undifferentiated tumors involving the lower gynecologic tract and all axillary tests including molecular tests should be performed for correct diagnosis because prolonged survival is possible for this dreadful disease after complete surgical resection, followed by adjuvant therapy.

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Primitive neuroectodermal tumor of the median nerve Case report with cytogenetic analysis

Journal of Neurosurgery ◽

10.3171/jns.1996.85.1.0163 ◽

1996 ◽

Vol 85 (1) ◽

pp. 163-169 ◽

Cited By ~ 10

Author(s):

Edward W. Akeyson ◽

Ian E. McCutcheon ◽

Mark A. Pershouse ◽

Peter A. Steck ◽

Gregory N. Fuller

Keyword(s):

Peripheral Nerve ◽

Median Nerve ◽

Cytogenetic Analysis ◽

Primitive Neuroectodermal Tumor ◽

Soft Tissues ◽

Neuron Specific Enolase ◽

Neuroectodermal Tumor ◽

Elderly Adult ◽

Content Type ◽

Dense Core Granules

✓ The authors describe a malignant peripheral primitive neuroectodermal tumor (PNET) that originated in the median nerve in an elderly adult. After the diagnosis was made by biopsy, the patient underwent radical local resection with interpositional vein grafting of the brachial artery. The tumor had the typical appearance of a primitive neural tumor with small, round cells forming rosettes. It stained positively for both the Ewing's sarcoma/peripheral PNET antigen (HBA-71) and neuron-specific enolase, confirming its neural origin. Ultrastructural examination revealed dense core granules and suggested neural differentiation of the neoplasm. Cytogenetic analysis suggested a chromosome (11;22) translocation typical of peripheral PNET. Early reports consisted of tumors arising solely in peripheral nerves, but recent series have focused mainly on tumors arising in the soft tissues other than nerves. There are no other cases of true PNET of peripheral nerve in the modern literature that have been fully characterized by immunohistochemical, ultrastructural, and cytogenetic criteria. Although peripheral PNETs occur more commonly in children, this unusual neoplasm should be considered in the differential diagnosis of peripheral nerve neoplasms in adults. Early diagnosis is desirable because of its aggressive nature and poor outcome.

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