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

ArticleinGynecologic Oncology 98(3):516-9 · October 2005with11 Reads
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.
    • "Currently there is no uniformity of treatment, owing to the rarity of this neoplasm. Snijders-Keilholz et al. recommended a multidisciplinary approach similar to that used in osseous PNETs with induction chemotherapy , surgery, adjuvant chemotherapy, and radiation [17]. When surgery is feasible, wide excision performed at a sarcoma center is preferable. "
    [Show abstract] [Hide abstract] ABSTRACT: Introduction Ewing’s sarcoma belongs to a spectrum of neoplastic diseases known as Ewing’s family of tumors. This family of tumors is usually seen in osseous sites. Ewing’s sarcoma of the cervix is extremely rare, with only 18 cases reported in the English literature. The immunohistochemical profile of Ewing’s sarcoma overlaps with other malignancies like small cell carcinoma. The rarity and complex pathologic picture of Ewing’s sarcoma of the cervix creates the potential for misdiagnosis. Hence, we believe this case needs to be reported to add to the available literature. Case presentation A 49-year-old white Caucasian woman presented with vaginal bleeding. A pelvic examination revealed a cystic lesion arising from her cervix. Examination of a biopsy specimen revealed a poorly differentiated neoplasm, with sheets of small hyperchromatic cells, staining weakly for neuroendocrine markers. She was diagnosed with small cell carcinoma and started on concurrent chemotherapy and radiation. However, additional positive immunostaining for CD99 was strongly suggestive of Ewing’s sarcoma. Fluorescence in situ hybridization revealed ESWR1 gene rearrangement, confirming Ewing’s sarcoma. Our patient underwent surgery, which confirmed stage IIB Ewing’s sarcoma. She received adjuvant chemotherapy but died from progressive metastatic disease after four cycles. Conclusion With early diagnosis and appropriate treatment, Ewing’s sarcoma of the cervix can be a potentially curable disease. However, owing to overlapping clinical and histopathological features, the diagnosis poses a challenge to oncologists and pathologists. This article guides pathologists to consider Ewing’s sarcoma in the differential diagnosis of small cell carcinoma with weak staining for neuroendocrine markers. This literature review will benefit oncologists encountering this rare entity.
    Full-text · Article · Dec 2015
    • "Primitive neuroectodermal tumors (PNETs) are rare neoplasms that can occur in the female genital tract, among other visceral sites. The most common location within the female genital tract is the ovary, followed by the uterine corpus (Euscher et al., 2008), vagina, and cervix (Snijders-Keilhotlz et al., 2005). PNETs can present in pure form or admixed with other components, including endometrioid adenocarcinoma, adenosarcoma, carcinosarcoma (metaplastic carcinoma), and heterologous sarcomas (Nogales, 2003; Bartosch et al., 2011). "
    [Show abstract] [Hide abstract] ABSTRACT: Highlights • Bevacizumab was an effective agent in one case of advanced uterine PNET. •VEGF was expressed by the tumor, supporting a mechanism for effectiveness. •Cisplatin/etoposide/bevacizumab should be further studied in clinical trials. •Patient remains disease-free forty-eight months following intervention.
    Full-text · Article · Sep 2015
    • "Both YAP and cIAP1 accelerated tumorigenesis and were required to sustain rapid growth of amplicon-containing tumors [43]. The YAP gene was also reported to be amplified and overexpressed in other human cancers, such as oral squamous cell carcinoma, primary intracranial ependymomas, malignant pleural mesotheliomas, and oral cancer444546. YAP has been implicated as an oncogene and is altered in different kinds of human digestive system cancers ( [22] staining, while 95% of normal liver tissue samples showed very weak staining, suggesting a significant difference in YAP protein levels between normal and cancerous tissues. Other researchers examined the expression level in an HCC cohort in China and found that both the YAP protein and mRNA transcription levels were significantly elevated in the majority of HCC tumorous tissue when compared with adjacent nontumor tissue by 62% to 9%, respectively474849. "
    [Show abstract] [Hide abstract] ABSTRACT: Hepatocellular carcinoma (HCC) is one of the most common malignancies worldwide and the third leading cause of cancer mortality. Despite continuing development of new therapies, prognosis for patients with HCC remains extremely poor. In recent years, control of organ size becomes a hot topic in HCC development. The Hippo signaling pathway has been delineated and shown to be critical in controlling organ size in both Drosophila and mammals. The Hippo kinase cascade, a singling pathway that antagonizes the transcriptional coactivator Yes-associated protein (YAP), plays an important role in animal organ size control by regulating cell proliferation and apoptosis rates. During HCC development, this pathway is likely inactivated in tumor initiated cells that escape suppressive constrain exerted by the surrounding normal tissue, thus allowing clonal expansion and tumor development. We have reviewed evolutionary changes in YAP as well as other components of the Hippo pathway and described the relationships between YAP genes and HCC. We also discuss regulation of transcription factors that are up- and downstream of YAP in liver cancer development.
    Full-text · Article · Aug 2013
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