GYNOCARE Update: Modern Strategies to Improve Diagnosis and Treatment of Rare Gynecologic Tumors—Current Challenges and Future Directions

More than 50% of all gynecologic tumors can be classified as rare (defined as an incidence of ≤6 per 100,000 women) and usually have a poor prognosis owing to delayed diagnosis and treatment. In contrast to almost all other common solid tumors, the treatment of rare gynecologic tumors (RGT) is often based on expert opinion, retrospective studies, or extrapolation from other tumor sites with similar histology, leading to difficulty in developing guidelines for clinical practice. Currently, gynecologic cancer research, due to distinct scientific and technological challenges, is lagging behind. Moreover, the overall efforts for addressing these challenges are fragmented across different European countries and indeed, worldwide. The GYNOCARE, COST Action CA18117 (European Network for Gynecological Rare Cancer Research) programme aims to address these challenges through the creation of a unique network between key stakeholders covering distinct domains from concept to cure: basic research on RGT, biobanking, bridging with industry, and setting up the legal and regulatory requirements for international innovative clinical trials. On this basis, members of this COST Action, (Working Group 1, “Basic and Translational Research on Rare Gynecological Cancer”) have decided to focus their future efforts on the development of new approaches to improve the diagnosis and treatment of RGT. Here, we provide a brief overview of the current state-of-the-art and describe the goals of this COST Action and its future challenges with the aim to stimulate discussion and promote synergy across scientists engaged in the fight against this rare cancer worldwide

Gingival overgrowth as the initial paraneoplastic manifestation of Hodgkin's Lymphoma in a child. A case report

Background: The purpose of this paper is to present the first case of gingival overgrowth, premature root resorption, and alveolar bone loss, which preceded the diagnosis of a stage IVB Hodgkin's lymphoma (HL) in a 9-year-old boy. Methods: The child presented complaining of gingival pain which first appeared 3 months prior. Clinical examination revealed inflamed, hyperplastic gingivae, while x-ray showed premature root resorption and alveolar bone loss. Medical work-up was significant for cervical lymphadenopathy. Gingival biopsy, followed by lymph node resection, was performed twice. Results: Histological examination of both gingival biopsies disclosed a mixed inflammatory infiltrate, while classical Hodgkin's lymphoma of the nodular sclerosis type was diagnosed from the second lymph node biopsy. Chemotherapy was instituted with mustard-vincristine-procarbazine-prednizone and adriamycine-bleomycine-vinblastine-dacarbazine. Remission of the lymphoma was observed with concomitant regression of the gingival overgrowth. Conclusions: The inflammatory gingival overgrowth, premature root resorption of deciduous teeth, and alveolar bone loss in this case, in conjunction with the regression of gingival overgrowth which followed the completion of chemotherapy, are strongly indicative of a paraneoplastic manifestation of HL The postulated mechanism for the development of the manifestation is the constitutive activation of the transcription factor NF-kB. The gingival inflammatory reaction was probably further aggravated by the bacterial-stimulated cytokine secretion released by monocytes