Dataset Information

Rare ovarian tumours. Other treatments for ovarian cancer.

ABSTRACT:

Aim

The description of rare malignant ovarian tumours and the most suitable treatments. Alternative therapies different from intravenous chemotherapy are also explained.

Methods

Literature review and ongoing trial information have been used to elaborate this guide.

Results

Each ovarian cancer type must be identified and treated properly from diagnostic to surgery, adjuvant treatment and metastatic disease. Hormonotherapy can be useful as an alternative treatment, especially in low-grade ovarian cancer and endometrioid subtype. Tumour characterisation is appropriated for treatment selection when targeted therapy is indicated. MEK inhibitors, tyrosine-kinase inhibitors, EGFR inhibitors, therapies against integrins, antibody-drug conjugates and other strategies are described. Antiangiogenics, PARP inhibitors and immunotherapy are discussed in other parts of this publication.

Conclusion

Different ovarian cancer types must receive the appropriated treatment. Alternative therapies may be evaluated beyond the standard therapy, frequently in a clinical trial, and an individualised molecular study may help to find the best treatment.

SUBMITTER: Gil-Martin M

PROVIDER: S-EPMC7573466 | biostudies-literature | 2020 Aug

REPOSITORIES: biostudies-literature

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Publications

Rare ovarian tumours. Other treatments for ovarian cancer.

Gil-Martin Marta M Pardo Beatriz B Barretina-Ginesta Maria-Pilar MP

EJC supplements : EJC : official journal of EORTC, European Organization for Research and Treatment of Cancer ... [et al.] 20200822

<h4>Aim</h4>The description of rare malignant ovarian tumours and the most suitable treatments. Alternative therapies different from intravenous chemotherapy are also explained.<h4>Methods</h4>Literature review and ongoing trial information have been used to elaborate this guide.<h4>Results</h4>Each ovarian cancer type must be identified and treated properly from diagnostic to surgery, adjuvant treatment and metastatic disease. Hormonotherapy can be useful as an alternative treatment, especially ...[more]

PMID: 33240448

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Project description:This manuscript reports the consensus statements on designing clinical trials in rare ovarian tumours reached at the fifth Ovarian Cancer Consensus Conference (OCCC) held in Tokyo, November 2015. Three important questions were identified concerning rare ovarian tumours (rare epithelial ovarian cancers (eOC), sex-cord stromal tumours (SCST) and germ cell tumours (GCT)): (i) What are the research and trial issues that are unique to rare ovarian tumours? There is a lack of randomised phase III data defining standards of care which makes it difficult to define control arms, but identifies unmet needs that merit investigation. Internationally agreed upon diagnostic criteria, expert pathological review and translational research are crucial. (ii) What should be investigated in rare eOC, GCT and SCST? Trials dedicated to each rare ovarian tumour should be encouraged. Nonetheless, where the question is relevant, rare eOC can be included in eOC trials but with rigorous stratification. Although there is emerging evidence suggesting that rare eOC have different molecular profiles, trials are needed to define new type-specific standards for each rare eOC (clear cell, low grade serous and mucinous). For GCTs, a priority is reducing toxicities from treatment while maintaining cure rates. Both a robust prognostic scoring system and more effective treatments for de novo poor prognosis and relapsed GCTs are needed. For SCSTs, validated prognostic markers as well as alternatives to the current standard of bleomycin/etoposide/cisplatin (BEP) should be identified. (iii) Are randomised trials feasible? Randomised controlled trials (RCT) should be feasible in any of the rare tumours through international collaboration. Ongoing trials have already demonstrated the feasibility of RCT in rare eOC and SCST. Mucinous OC may be considered for inclusion, stratified, into RCTs of non-gynaecological mucinous tumours, while RCTs in high risk or relapsed GCT may be carried out as a subset of male and/or paediatric germ cell studies.

Project description:BACKGROUND: Epstein-Barr Virus (EBV) associated lymphoproliferative disorders are encountered in immunodeficiency states. Amongst these, primary CNS lymphoma in HIV/AIDS patients is relatively rare and represents a therapeutic challenge. MATERIALS AND METHODS: This report was generated from patient's medical records throughout hospitalizations and outpatient visits. RESULTS: A 20-year-old female with congenitally acquired HIV and poor medication compliance presented with severe headaches and mild cranial nerve deficits. HIV viral load at presentation was 89,976/ml with CD4 count of 4/uL. Head MRI revealed 3-cm thick-walled ring-enhancing lesion in right basal ganglia. Serum titers for toxoplasmosis were negative. EBV DNA was detected by PCR in CSF without peripheral viremia. Imaging with thallium scan and MRI findings in combination with EBV PCR positive CSF allowed for the presumptive diagnosis of EBV-associated primary CNS lymphoma. Highly active anti-retroviral therapy (HAART) was reinitiated. Karnofsky score was 90%, thus chemotherapy was offered to optimize chance of cure. Agents of choice were high-dose Methotrexate for CNS penetration/anti-lymphoma activity and Rituximab. Within the next 5 months she received 8 cycles of alternating Methotrexate (4000 mg/m2/dose on Day 1) and Rituximab (375 mg/m2/dose on Day 8). Supportive care included leucovorin rescue and urine alkalinization. Therapy was tolerated well without sepsis episodes during neutropenia. Her headaches and neurological deficits resolved after the first cycle of therapy. Serial imaging demonstrated substantial and continuous regression of the lymphoma lesion. No adjunctive radiotherapy was administered. Patient remains in remission 18 months after diagnosis, despite fluctuating viral loads and CD4 counts due to the poor HAART compliance. No chronic hypogammaglobulinemia resulted from therapy to date. CONCLUSION: Given the observed results and acceptable toxicity profile, methotrexate and rituximab combination may be effective and should be considered in addition to HAART for treating EBV-associated CNS lymphoma in young patients with minimal comorbidities and underlying HIV/AIDS. INTRODUCTION: Pediatric pineal parenchymal tumors of intermediate differentiation (PPTID, WHO grade II or III) are extremely uncommon. Appropriate therapy has not been defined. We present three new cases of pediatric PPTID and 8 cases in patients under age 45 identified in a literature review. METHODS: This study utilized the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) (http://www.prisma-statement.org.). RESULTS: We diagnosed two children (female age 5 y, male age 14 y) with PPTID in the pineal region and negative work-up for metastases. Both underwent gross total resection (GTR). Pathologic evaluation was consistent with PPTID grade II in each. Both were treated with local radiation therapy via protons to 50.4 CGE. The first patient (case 1) is now 4 years since diagnosis with no evidence of disease (NED) and the second (case 2) is 8 months after surgery and NED. Our third case (male age 15 y) was diagnosed with PPTID grade III with drop metastases in the thecal sac (case 3). He was treated with craniospinal and boost radiation and multiagent chemotherapy but relapsed with leptomeningeal disease near the conus 2 years after diagnosis. Our literature review identified 8 cases of PPTID in patients under age 45, described in Table 1 (cases 4-11). There were broad treatment strategies from GTR only to craniospinal radiation and multiagent chemotherapy. While the therapy varied, the outcome for those with non-metastatic grade II tumors was good. Grade III tumors were associated with metastatic disease and a worse outcome. DISCUSSION: With very few cases, definitive recommendations for therapy are impossible. For PPTID grade II, GTR alone may be sufficient. PPTID grade III should be considered for more aggressive therapy.

Dataset Information

Rare ovarian tumours. Other treatments for ovarian cancer.

Aim

Methods

Results

Conclusion

Publications

Rare ovarian tumours. Other treatments for ovarian cancer.

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