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GCIG RARE TUMOR CONSENSUS REVIEW &

191GUIDELINES

Isabelle Ray-Coquard, MD, PhD, Jonathan A. Ledermann, MD, Philipp Harter, MD
Michael Friedlander, MD, PhD, Frederic Amant, MD, PhD, Aikou Okamoto, MD, PhD

Eva Gómez-García, MD, Charlie Gourley, MD, PhD, Martee Hensley, MD
Patricia Pautier, MD, Keiichi Fujiwara, MD, PhD Domenica Lorusso, MD, Satoru Sagae, MD

Akila N. Viswanathan, MD, MPH, Mario M. Leitao Jr, MD, Toyomi Satoh, MD, PhD
Nick Reed, MD, Jubilee Brown, MD, Dominique Berton Rigaud, MD

Rosalind Glasspool, MBBS, PhD, FRCP, Eric Pujade Lauraine, MD, PhD

Introduction present and future clinical trials involving rare cancers;
3) to identify national and international barriers for trials
This paper devoted to the consensus review on rare dedicated to rare gynaecologic cancers; 4) to summarize
malignant gynaecologic tumors, is a summary of an and prioritize key issues for research and agree on a new
original and unique initiative of the Gynecologic Cancer set of trial concepts addressing the key issues in several
InterGroup (GCIG). The primary objective of the GCIG rare tumors; and, 5) to prioritize and design international
is to facilitate interaction amongst cooperative groups initiatives in rare gynaecologic cancers. Are summarized
conducting Gynecologic Cancer clinical research. To in this chapter all the 20 guidelines dedicated to the most
date twenty-seven (27) cooperative groups from twenty frequent rare gynaecologic cancers, where unmet needs
(20) countries have met the requirements for GCIG are identified.
membership; that includes being a network of several
sites having performed at least one randomized phase Malignant ovarian germ cell tumors
III therapeutic clinical trial in Gynecologic Cancer with (Brown et al., 2014)
Good Clinical Practice (GCP) compliance and sufficient
data management and statistical support. Most women diagnosed with malignant ovarian germ cell
tumors (MOGCT) have curable disease and experience
With 16.1/100 000 new EU annual cases, the sum of limited treatment-associated morbidity. Success is related
all rare gynaecological tumors (RGT), including ovarian, both to tumor biology and improvements in treatment
fallopian, uterine, cervix, vaginal, and vulvar, represent over the last 4 decades. MOGCT occur predominantly
more than 50% of all gynaecologic cancers (Gatta et in girls, adolescents, and young women and are often
al., 2011). As it has not been feasible to conduct large- unilateral tumors of early stage, although advanced-
scale randomized trials in rare gynaecologic cancers, stage disease occurs in approximately 30% of patients.
treatments have been developed based on expert opinion Tumors are usually chemosensitive, thereby allowing
or by drawing on experience from therapeutic advances fertility-sparing surgery (oophorectomy) in most women
made in treating other similar tumors. A global approach yielding a high cure rate and excellent survival after long-
is needed to share information and collect research data. term follow-up. Outcomes are equivalent to patients
In spite of the fact that oncologists and gynecologists undergoing hysterectomy with bilateral salpingo-
managing RGT are usually well-organized at a national oophorectomy. Based on efficacy and tolerability, BEP
level, there are no specific structured collaborations has become the standard of care when chemotherapy is
that exist internationally. The past 5 years have seen an warranted. Patients with FIGO Stage I dysgerminoma
explosion in our understanding of the heterogeneity of and Stage IA grade 1 immature teratoma may be safely
gynaecological cancer. To make use of this knowledge observed postoperatively without chemotherapy. Current
there needs to be a change in the way clinical studies research aims to find other low risk patients in whom
are designed in the case of rare tumors. Consequently, chemotherapy may be avoided. Poor prognostic factors
in 2014 the GCIG published guidelines reviews to include age > 45, advanced stage, elevated hCG and AFP,
promote research and clinical trials for those not so rare incomplete surgical resection, and yolk sac histology. In
cancers at the international level of GCIG cooperation. the rare patient with chemoresistant disease, treatment
The main objectives of the project were: 1) to define failures are categorized as platinum-resistant or platinum-
current recommendations for the management of rare sensitive (recurrence beyond 6 weeks of initial therapy.
gynaecologic tumors; 2) to define control arms for

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