abstract: Clinical syndromes associated with ovarian neoplasms: a comprehensive review

Radiographics. 2010 Jul-Aug;30(4):903-19.

Functional ovarian neoplasms have unique clinical manifestations related to hormone overproduction and may give rise to a broad spectrum of clinical syndromes.

Sex cord-stromal tumors, the most common functional ovarian neoplasms, are associated with either hyperestrogenism (as in granulosa cell tumor and thecoma) or hyperandrogenism (as in Sertoli-Leydig cell tumor and Leydig cell tumor). Other, less common ovarian neoplasms that may have endocrine or nonendocrine syndromic manifestations include germ cell tumors associated with the excessive production of human chorionic gonadotropin (eg, choriocarcinoma, dysgerminoma), monodermal teratomas (eg, carcinoid tumor, struma ovarii) associated with carcinoid syndrome and hyperthyroidism, and primary epithelial ovarian cancers associated with paraneoplastic syndromes.

The application of diagnostic algorithms based on patient demographic information, clinical manifestations, laboratory findings, and cross-sectional imaging features may help identify ovarian neoplasms in complex clinical settings.

Management of rare ovarian cancers: The experience of the French website «Observatory for rare malignant tumours of the ovaries» by the GINECO group

Background

Non-epithelial ovarian cancers are rare; their natural history is poorly understood and prognostic factors remain unclear. A French website (www.ovaire-rare.org) was developed to collect clinical cases and tumour samples in order to better define prognostic factors and develop specific trials. We report the results of the first 100 patients with germ cell (GCT) and sex cord-stromal (SCT) tumours.

 Conclusions

This online observatory allows assessing medical practice for GCT and SCT in France. Histological discrepancies between diagnosis and second opinion confirm the need for systematic review before treatment. Extension to other rare gynaecologic malignancies is on-going.

Immunohistochemical profile of steroid cell tumor of the ovary: a study of 14 cases and a review of the literature (pathology)

"Immunohistochemistry helps in the distinction between SCTs of the ovary and other primary or metastatic ovarian neoplasms with eosinophilic and clear-cell histology. In addition, immunohistochemistry can confirm the presence of recurrent SCT, if no sufficient clinical history is provided. As some SCTs can be positive for epithelial markers and histologically similar epithelial tumors can be positive for sex cord stromal markers, the use of multiple immunohistochemical stains is recommended."

free full text: Non-epithelial ovarian cancer: ESMO Clinical Recommendations for diagnosis, treatment and follow-up

Note: the papers from ESMO (ovarian cancer) requires registration/access is free 1) Classification of germ cell ovarian tumors 2) Classification of sex cord stromal ovarian tumors "This set of recommendations applies to invasive epithelial ovarian carcinoma; the management of tumors of low malignant potential (‘borderline’) is not covered here."

Targeted therapies for rare gynaecological cancers : The Lancet Oncology

The Lancet Oncology, Early Online Publication, 1 April 2010

Targeted therapies for rare gynaecological cancers

Summary

Some gynaecological cancers are uncommon, such as sex cord-stromal tumours, malignant germ-cell tumours, vulvar carcinoma, melanoma of the female genital tract, clear-cell carcinoma of the ovary and endometrium, neuroendocrine tumours of the cervix, and gestational trophoblastic neoplasia.

All these cancers have different clinicopathological characteristics, suggesting different molecular biological pathogeneses. Despite aggressive treatment, some cancers recur or respond poorly to therapy. Comprehensive knowledge of the molecular biology of each cancer might help with development of novel treatments that maximise efficacy and minimise toxic effects. Targeted therapy is a new treatment strategy that has been investigated in various tumours in clinical and laboratory settings.

Since these cancers are rare and large clinical trials are difficult to do, molecular biological techniques might allow rapid proof-of-principle experiments in few patients. Novel targeted agents either alone or in combination with other treatments offer promising therapeutic options.