Snoring as main symptoms of peripheral primitive neuroectodermal tumor in oropharynx:a case report with review of literature

Abstract IntroductionIn children, snoring is often associated with hypertrophied tonsils and adenoids. There was a case in a girl who did not suffer from hypertrophy adenoids, but from a peripheral primitive neuroectodermal tumor (pPNET) in the oropharynx. pPNET often occurs in the deep soft tissues of the trunk, paravertebral, lower limbs and retroperitoneum. It is rarely located in children's oropharynx and makes snoring.Patient concernsWe report a case of a 4-years-old girl who complained of slurred speech and snoring. Her serum showed normal results, computed tomography (CT) and magnetic resonance imaging (MRI) revealed a mass lesion in oropharynx. By using histology and immunohistochemistry, cluster of differentiation 99 (CD99), vimentin, friend leukemia integration 1 (FLI-1), integrase interactor-1 and α-smooth muscle actin were positive, but epithelial membrane antigen, erythroblast transformation-specific (ETS) related gene (ERG), NK homeobox 2.2, Wilms’ tumor 1 and S100-protein were negative. And the rearrangement of the Ewing sarcoma breakpoint region 1 (EWSR1) gene was proved in the test of fluorescent.DiagnosisShe was diagnosed as a peripheral primitive neuroectodermal tumor.Interventions and outcomeThe girl had been treated with surgery and chemotherapy. She has been well for 24 months without any signs of disease.ConclusionIn children who snore, we should not only think of hypertrophied adenoids, but also the possibility of pPNETs in oropharynx. CT scan or MRI should be conducted in time, as well as histology and immunohistochemistry to avoid misdiagnosis.

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A case of peripheral primitive neuroectodermal tumor of the ovary

International Journal of Gynecological Cancer ◽

10.1136/ijgc-00009577-200403000-00027 ◽

2004 ◽

Vol 14 (2) ◽

pp. 370-372 ◽

Cited By ~ 8

Author(s):

K.-J. Kim ◽

B.-W. Jang ◽

S.-K. Lee ◽

B.-K. Kim ◽

S.-L. Nam

Keyword(s):

Primitive Neuroectodermal Tumor ◽

Soft Tissues ◽

Case Reports ◽

The Body ◽

Neuroectodermal Tumor ◽

Pelvic Cavity ◽

Age Group ◽

Peripheral Primitive Neuroectodermal Tumor ◽

Children And Young Adults ◽

The Right

Peripheral primitive neuroectodermal tumor (PNET) belongs to the PNET/Ewing's sarcoma family. PNET is a small round cell tumor of putative neuroectoderm origin and is the second most common sarcoma among children and young adults. It may occur anywhere in the body and within any age group; however, it is most likely to occur in the bone and soft tissues. There have been a small number of case reports of PNET arising in the ovary. We presented a case of PNET arising in the right ovary of an 18-year-old woman. The tumor was metastased to the lymph nodes of the pelvis and para-aorta at surgical staging. We had persecuted Taxol/carboplatin chemotherapy, pelvic cavity radiotherapy, and Vincristine/Actinomycink, Cyclophosphamide/Doxorubicin (VACA). She died after 10 months due to septic shock.

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Peripheral primitive neuroectodermal tumor of the small bowel mesentery: Report of a case

Srpski arhiv za celokupno lekarstvo ◽

10.2298/sarh1510619m ◽

2015 ◽

Vol 143 (9-10) ◽

pp. 619-622

Author(s):

Helena Maric ◽

Radovan Cvijanovic ◽

Igor Ivanov ◽

Ljiljana Gvozdenovic ◽

Dejan Ivanov ◽

...

Keyword(s):

Young Adults ◽

Small Intestine ◽

Primitive Neuroectodermal Tumor ◽

Soft Tissues ◽

Clinical Presentation ◽

Neuroectodermal Tumor ◽

Small Bowel Mesentery ◽

Peripheral Primitive Neuroectodermal Tumor ◽

Neural Origin ◽

Children And Young Adults

Introduction. Primitive neuroectodermal tumor or Ewing?s sarcoma is a tumor of undifferentiated small round cells that arise from the soft tissues, and is believed to be of neural origin. It occurs most often in children, followed by adolescents and young adults. Case Outline. A case of a 24-year-old patient with ulcerostenosans Ewing?s sarcoma of the initial part of the small intestine is presented in our paper. Reviewing the literature and using as an example the case of a female patient with signs of sideropenic anemia caused by primitive neuroectodermal tumor of the small intestine, an attempt was made to clarify the etiology, clinical presentation, diagnosis and therapy with the aim of its rapid detection and treatment. Conclusion. Mesenteric primitive neuroectodermal tumor is a rare neoplasm in adults, while it usually occurs in children and young adults. Surgical resection of the lesions with the application of chemotherapy is the main form of treatment of patients suffering from this disease.

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