Primitive Neuroectodermal Tumor of the Gallbladder

Abstract Ewing sarcoma/primitive neuroectodermal tumor (ES/ PNET) most commonly occurs in the soft tissues of the extremities and the paravertebral areas. To our knowledge, no case of PNET has been reported in the gallbladder to date. We report a case of PNET of the gallbladder occurring in a 53-year-old woman. Microscopically, the monotonous small round cells with a formation of Homer-Wright rosettes showed intense membranous positive immunoreactivity for MIC2/CD99, as well as for neuron-specific enolase and synaptophysin. Other small round cell tumors, such as lymphoma, rhabdomyosarcoma, or melanoma, were excluded in light of negative immunoreactivity for leukocyte common antigen, desmin, and S100. The diagnosis of PNET was rendered based on the characteristic morphology and immunohistochemical findings, despite an absence of chromosomal translocations, such as t(11; 22)(q24;q12) and t(21;22)(q22;q12).

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Pelvic primitive neuroectodermal tumor associated with a cluster of small round cell tumors: case report and review of current literature☆

Gynecologic Oncology ◽

10.1016/s0090-8258(03)00416-5 ◽

2003 ◽

Vol 91 (1) ◽

pp. 247-253 ◽

Cited By ~ 2

Author(s):

Bobbie S Gostout ◽

Noralane M Lindor ◽

Connie S DiMarco ◽

Prema P Peethambaram ◽

Amy C Clayton

Keyword(s):

Case Report ◽

Current Literature ◽

Primitive Neuroectodermal Tumor ◽

Neuroectodermal Tumor ◽

Round Cell ◽

Small Round Cell ◽

Round Cell Tumors

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EWSR1 Rearrangement and CD99 Expression as Diagnostic Biomarkers for Ewing/PNET Sarcomas in a Moroccan Population

Disease Markers ◽

10.1155/2018/7971019 ◽

2018 ◽

Vol 2018 ◽

pp. 1-5 ◽

Cited By ~ 1

Author(s):

Sara Louati ◽

Nadia Senhaji ◽

Laila Chbani ◽

Sanae Bennis

Keyword(s):

Differential Diagnosis ◽

Sensitivity And Specificity ◽

Ewing Sarcoma ◽

Primitive Neuroectodermal Tumor ◽

Neuroectodermal Tumor ◽

Fish Analysis ◽

Round Cell ◽

Diagnostic Biomarkers ◽

Moroccan Population ◽

Round Cell Tumors

Ewing sarcoma/primitive neuroectodermal tumor (Ewing/PNET sarcomas or EPS) are a group of round cell tumors. Malignant round cell tumors form a large and diverse group that includes rhabdomyosarcoma, synovial sarcoma, non-Hodgkin’s lymphoma, neuroblastoma, hepatoblastoma, Wilm’s tumor, desmoplastic small round cell tumor, and other morphologically similar entities. Differential diagnosis of Ewing sarcoma/primitive neuroectodermal tumor (Ewing/PNET sarcomas or EPS) is difficult. In addition to morphology and immunohistochemistry (IHC), differential diagnosis of these tumors is based on molecular analysis of the EWSR1 gene rearrangement using fluorescent in situ hybridization (FISH) technique. We investigated the diagnostic value of combined CD99 immunostaining and EWSR1 t(22q12) alteration using a dual-color, break-apart rearrangement probe in forty-one formalin-fixed paraffin-embedded (FFPE) tissue samples from pediatric and adult patients diagnosed with EPS. IHC was performed in all cases using the CD99 antibody and showed a positivity of 92.7% in the enrolled cases (38/41) followed by FISH analysis where 48.8% of the cases (20/41) were rearranged. Sensitivity and specificity for IHC assays were 88% and 58%, respectively. Notably, FISH had a sensitivity of 100% and a specificity of 87%. In addition, CD99 positivity was found to correlate with EWSR1 rearrangement (p<0.05). This report shows that FISH has better sensitivity and specificity than IHC in the Moroccan population, and supports its combination with CD99 immunostaining as diagnostic biomarkers for this rare malignant entity.”

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A Retroperitoneal/omental tumor with characteristics of Ewing’s sarcoma/peripheral primitive neuroectodermal tumor (EWS/pPNET) and desmoplastic small round cell tumor (DSRCT) with unique pathologic and immunohistochemical features, presented as a renal tumor

The Internet Journal of Pathology ◽

10.5580/efd ◽

2009 ◽

Vol 8 (1) ◽

Keyword(s):

Primitive Neuroectodermal Tumor ◽

Renal Tumor ◽

Cell Tumor ◽

Neuroectodermal Tumor ◽

Round Cell ◽

Peripheral Primitive Neuroectodermal Tumor ◽

Round Cell Tumor ◽

Small Round Cell Tumor ◽

Small Round Cell ◽

Omental Tumor

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Extraskeletal Ewing's Sarcoma/Primitive Neuroectodermal Tumor of the Posterior Mediastinum with t(11;22)(q24;q12)

Tumori Journal ◽

10.1177/030089160809400623 ◽

2008 ◽

Vol 94 (6) ◽

pp. 888-891 ◽

Cited By ~ 16

Author(s):

Marosh Manduch ◽

David F Dexter ◽

Peter M Ellis ◽

Kenneth Reid ◽

Phillip A Isotalo

Keyword(s):

Primitive Neuroectodermal Tumor ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Posterior Mediastinum ◽

Neuroectodermal Tumor ◽

Malignant Neoplasms ◽

Round Cell ◽

Mediastinal Tumors ◽

Small Round Cell ◽

Extraskeletal Ewing’S Sarcoma

Ewing's sarcoma/primitive neuroectodermal tumor family of tumors is part of a rare group of malignant neoplasms with small round-cell morphology. We describe a 24-year-old woman who presented with non-specific back pain. A chest radiograph and magnetic resonance imaging demonstrated an extraosseous, dumbbell-shaped mass of the posterior mediastinum with extension into the spinal canal. The patient underwent a left posterolateral thoracotomy and a T3–5 laminectomy with subsequent multi-agent chemotherapy. Histopathologic examination of the tumor demonstrated sheets of primitive small round malignant cells that showed no visible differentiation. Neoplastic cells were strongly immunoreactive for CD99 and vimentin and were negative for chromogranin, synaptophysin, CD31, CD34, calcitonin, desmin, low-molecular weight cytokeratins, wide-spectrum cytokeratins, leukocyte common antigen, S-100 protein, and thyroid transcription factor-1. The neoplasm was diagnosed as a Ewing's sarcoma/primitive neuroectodermal tumor, and cytogenetic studies confirmed a t(11;22)(q24;q12) chromosomal translocation and an associated trisomy of chromosome 2, supporting the histologic diagnosis. Extraskeletal Ewing's sarcoma/primitive neuroectodermal tumors are rare neoplasms that should be distinguished from other small round-cell tumors by morphology and ancillary laboratory techniques. Although rare, they need to be considered in the differential diagnosis of primary mediastinal tumors.

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Transplantation of congenital primitive neuroectodermal tumor of fetus to the uterus of mother: application of biotin-labeled chromosome-specific probes

International Journal of Gynecological Cancer ◽

10.1046/j.1525-1438.1995.05060459.x ◽

1995 ◽

Vol 5 (6) ◽

pp. 459-464 ◽

Cited By ~ 5

Author(s):

M. E. Nath ◽

A. Kanbour ◽

J. Hu ◽

U. Surti ◽

A. Kunschner ◽

...

Keyword(s):

Y Chromosome ◽

Primitive Neuroectodermal Tumor ◽

Male Infant ◽

Neuroectodermal Tumor ◽

White Female ◽

Round Cell ◽

Small Round Cell ◽

Dilation And Curettage ◽

Normal Placenta

We report a 38-year-old gravida 3, para 3, white female with an unremarkable history and normal amniocentesis, who delivered a male infant with a large pedunculated and ulcerated tumor by vaginal delivery. A portion of the tumor was sheared and expelled with the normal placenta at the time of delivery. The biopsy of the infant mass and the expelled tissue showed histologic features of a small round cell tumor with immunohistochemical features of a primitive neuroectodermal tumor (PNET). The infant eventually died at the age of 4 weeks as a result of extensive dissemination of tumor. The mother had postpartum bleeding, and fractional dilation and curettage procedures at 6 weeks and 4 months after delivery revealed tumor similar to that of the infant. The mother underwent radical hysterectomy and bilateral salpingo-oophorectomy, which revealed a neoplasm at the junction of the lower uterine segment and cervix. Fluorescentin situhybridization (FISH) study of both the infant's tumor and the mother's uterine tumor showed positivity for the Y chromosome by using a classical α-satellite Y chromosome-specific probe. These findings support the hypothesis that the tumor was transferred from the fetus to the mother.

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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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Thoracic Primitive Neuroectodermal Tumor: An Unusual Case and Literature Review

Case Reports in Pulmonology ◽

10.1155/2013/326871 ◽

2013 ◽

Vol 2013 ◽

pp. 1-4

Author(s):

Kubra Erol Kalkan ◽

Ahmet Bilici ◽

Fatih Selcukbiricik ◽

Nurcan Unver ◽

Mahmut Yuksel

Keyword(s):

Needle Biopsy ◽

Rare Case ◽

Primitive Neuroectodermal Tumor ◽

Unusual Case ◽

Immunohistochemical Analysis ◽

Fine Needle Biopsy ◽

Anterior Mediastinum ◽

Neuroectodermal Tumor ◽

Round Cell ◽

Small Round Cell

We describe herein a rare case of a primary primitive neuroectodermal tumor (PNET) in the mediastinum of a 75-year-old man. Grossly, the tumor was located in the left upper anterior mediastinum. Transcutaneous fine-needle biopsy (TCNB) revealed small round-cell proliferation. The expression immunohistochemical analysis was confirmed the diagnosis of PNET. He was successfully treated with chemotherapy and is alive with no sign of recurrence for 17 months after the diagnosis.

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