Primitive neuroectodermal tumor of the cervix uteri: A case report

Snijders-Keilholz, A.; Ewing, Patricia C.; Seynaeve, C.; Burger, Curt W.

doi:10.1016/j.ygyno.2005.05.020

Cited by 55 publications

(35 citation statements)

References 6 publications

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“…cPNETs are exceptionally rare and often aggressive tumors, with no known etiological factors. As of 2005, only nine cases had been reported [Snijders-Keilholz et al, 2005] and no etiological factors have been identified. Some authors consider them to be Ewing sarcomas [Fletcher et al, 2002].…”

Section: Introductionmentioning

confidence: 99%

Extending the phenotypes associated withDICER1mutations

Foulkes¹,

Bahubeshi²,

Hamel³

et al. 2011

Hum. Mutat.

181

127

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DICER1 is crucial for embryogenesis and early development. Forty different heterozygous germline DICER1 mutations have been reported worldwide in 42 probands that developed as children or young adults, pleuropulmonary blastoma (PPB), cystic nephroma (CN), ovarian sex cord-stromal tumors (especially Sertoli-Leydig cell tumor [SLCT]), and/or multinodular goiter (MNG). We report DICER1 mutations in seven additional families that manifested uterine cervix embryonal rhabdomyosarcoma (cERMS, four cases) and primitive neuroectodermal tumor (cPNET, one case), Wilms tumor (WT, three cases), pulmonary sequestration (PS, one case), and juvenile intestinal polyp (one case). One carrier developed (age 25 years) a pleomorphic sarcoma of the thigh; another carrier had transposition of great arteries (TGA). These observations show that cERMS, cPNET, WT, PS, and juvenile polyps fall within the spectrum of DICER1-related diseases. DICER1 appears to be the first gene implicated in the etiology of cERMS, cPNET, and PS. Young adulthood sarcomas and perhaps congenital malformations such as TGA may also be associated.

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Section: Introductionmentioning

confidence: 99%

Extending the phenotypes associated withDICER1mutations

Foulkes¹,

Bahubeshi²,

Hamel³

et al. 2011

Hum. Mutat.

181

127

View full text Add to dashboard Cite

show abstract

“…It might be considered that neoadjuvant chemotherapy is an overtreatment of PNET. However, as Snijders-Keilholz et al 7 mention, upto now duration of follow -up of the reported cases is too short (5-42 months) to prove or reject this discussion. Farah Farzaneh et al 2 reported that for Ewing's sarcoma of other sites, neoadjuvant chemotherapy in metastatic cases could change inoperable presentation to operable state for successful local/or regional treatment.…”

Section: Discussionmentioning

confidence: 89%

Ewing’s Sarcoma or Primitive Neuroectodermal Tumour of Cervix: A Case Report

Deshpande¹,

Yelikar²

2014

jemds

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“…EES/PNET of the female genital tract (FGT) is very unusual, and reported most common sites in FGT were the ovaries [5] followed by the uterine body [6] and less commonly the cervix [7], vagina and vulva [8,9].…”

Section: Discussionmentioning

confidence: 99%

Primary Ewing Sarcoma of Vulva: A Case Report and a Review of Literature

Kakoti

Sharma

Kataki

et al. 2017

Indian J Gynecol Oncolog

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Introduction Ewing sarcoma family of tumour represents a group of bone and soft-tissue tumours with variable neuroectodermal differentiation. Extraosseous Ewing sarcoma (EES) commonly arises in soft tissues of trunk or extremities with a few reported rare sites include larynx, nasal cavity, neck, lung, retroperitoneum, mediastinum and genital tract. Till now 28 cases of extraosseous Ewing sarcoma of vulva and vagina have been reported in the literature. Case Report We report 29th case of Ewing sarcoma vulva in sixth youngest, 16-year-old patient with third largest size tumour. Her all routine blood reports were found to be normal, and MRI showed highly vascular vulval mass with terminal urethral involvement. Tru-cut biopsy revealed uniform sheets of small round cells with geographical necrosis and perivascular clusters of tumour cells. Immunohistochemical staining demonstrated that the cells were focally positive for CK and vimentin and showed membranous positivity for CD99 and nuclear positivity for FLI1, whereas all other IHC markers to rule out differentials of small round cell tumour were negative. Based on HPE and IHC, which is polyimmunophenotypic, has confirmed the diagnosis of an ES/PNET. After ruling out for the metastatic lesions, 14 cycles of chemotherapy with compressed VDC/IE were planned, but after receiving her 1st cycle itself, she expired on 10th day of postchemotherapy. Conclusion EES is a rare fast growing aggressive tumour requiring ancillary techniques for exact diagnosis and multimodality treatment for better survival.

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Primitive neuroectodermal tumor of the cervix uteri: A case report

Cited by 55 publications

References 6 publications

Extending the phenotypes associated withDICER1mutations

Extending the phenotypes associated withDICER1mutations

Ewing’s Sarcoma or Primitive Neuroectodermal Tumour of Cervix: A Case Report

Primary Ewing Sarcoma of Vulva: A Case Report and a Review of Literature

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