Primitive neuroectodermal tumor of the cervix uteri: A case report Changing concepts in therapy

Erasmus Universiteit Rotterdam, Rotterdam, South Holland, Netherlands
Gynecologic Oncology (Impact Factor: 3.77). 10/2005; 98(3):516-9. DOI: 10.1016/j.ygyno.2005.05.020
Source: PubMed


Peripheral primitive neuroectodermal tumor (PNET) of the cervix uteri is extremely rare. Between 1987 and 2002, there have been eight cases described in the English literature. The treatment policies in these eight cases differed considerably, partly due to the rarity of the disease and to differing time periods of diagnosis and treatment.
At the end of 2002, a 21-year-old woman presented with a PNET of the cervix uteri at our institute, the Erasmus Medical Center. For the appropriate treatment in this case, we reviewed the literature and decided that the treatment should be different from the local surgical treatment followed by additional treatments as most of the earlier reports describe.
In view of the current knowledge of PNET belonging to the family of Ewing's sarcoma, and the improvement of treatment outcome in these tumors due to dose-intensive neo-adjuvant chemotherapy, patients with PNET of the cervix should be treated in accordance to the protocol for bony Ewing's sarcoma with multimodality therapy by means of induction chemotherapy, surgery, and consolidation chemotherapy.

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    • "Horn et al6 used cisplatin and fluorouracil for 3 years after the initial therapy (for pulmonary metastasis). Snijders-Keilholz et al10 used six cycles of doxorubicin, ifosfamide, mesna, and etoposide preoperatively and vincristine, ifosfamide, and actinomycin for five cycles. Tsao et al13 used cyclophosphamide, doxorubicin (Adriamycin® Pfizer, New York, NY, USA), and vincristine and ifosfamide and etoposide for two cycles before surgery and two cycles after surgery. "
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    ABSTRACT: Primary primitive neuroectodermal tumors (PNETs) are rare and high-grade malignant tumors that mostly occur in children and young adults. The most common sites are the trunk, limbs, and retroperitoneum. Herein, we present a case of a PNET involving the cervix uteri in a 27-year-old woman. The lesion showed characteristic histologic features of a PNET and was positive for the immunohistochemical markers cluster of differentiation (CD) 99, vimentin, neuron-specific enolase, neural cell adhesion molecule 1 (CD56), and CD117 (c-kit), further defining the tumor while helping to confirm PNET. The clinical Stage IIIB tumor was treated with chemotherapy and radiotherapy.
    OncoTargets and Therapy 06/2013; 6:707-11. DOI:10.2147/OTT.S45889 · 2.31 Impact Factor
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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]. "
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    ABSTRACT: 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.
    Human Mutation 12/2011; 32(12):1381-4. DOI:10.1002/humu.21600 · 5.14 Impact Factor
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    • "It might be considered that neoadjuvant chemotherapy is an overtreatment for the management of PNET. However, as Snijders-Keilholz et al. [9] mention, up to now duration of follow-up of the reported cases is too short (five to 42 months) to prove or reject this discussion. As with Ewing's sarcoma of other sites, we showed that neoadjuvant chemotherapy in metastatic cases could change an inoperable presentation to an operable state for successful local and/or regional treatment. "
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    ABSTRACT: Peripheral primitive neuroectodermal tumor of the cervix uteri is extremely rare. Between 1987 and 2010, there were only nine cases reported in the English literature, with considerably different management policies. A 45-year-old Iranian woman presented to our facility with a primitive neuroectodermal tumor of the cervix uteri. Her clinical stage IB2 tumor was treated successfully with chemotherapy. Our patient underwent radical hysterectomy. There was no trace of the tumor after four years of follow-up. According to current knowledge, primitive neuroectodermal tumors belong to the Ewing's sarcoma family, and the improvement of treatment outcome in our patient was due to dose-intensive neoadjuvant chemotherapy, surgery and consolidation chemotherapy in accordance with the protocol for bony Ewing's sarcoma.
    Journal of Medical Case Reports 09/2011; 5(1):489. DOI:10.1186/1752-1947-5-489
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