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GCIG Rare Tumor Consensus Review & Guidelines 1521
Over 50% of patients with recurrent platinum-sensitive Small Cell Carcinomas of The Ovary
testicular cancer are salvaged with VeIP (vinblastine, (Reed et al., 2014b)
ifosfamide, and cisplatin) or TIP (paclitaxel, ifosfamide,
and cisplatin), with or without high dose chemotherapy, Small cell carcinomas of the ovary are uncommon
but data are limited regarding treatment of resistant and account for less than 1% of ovarian cancers.
MOGCT patients. They were first recognized in 1979, and a number
of reports appeared during the next 2 decades. Two
Sex Cord Stromal Tumors types are identified, Small cell carcinoma of ovary of
(Ray-Coquard et al., 2014) with hypercalcaemic type (SCCOHT) and small cell
carcnoma of ovary of pulmonary type (SCCOPT). The
Sex cord tumors (SCSTs) are rare cancers of the ovarian former are now considered to be malignant ovarian
area in adults. They constitute a heterogeneous group rhabdoid tumours. They are highly aggressive tumors
of tumors (Granulosa cell tumors, Sertoli Leydig Cell and usually carry a poor prognosis, although this may
tumors and pure stromal tumal including fibrosarcoma) reflect that most are diagnosed at advanced stage;
that develop from the sex cords and the ovarian stroma. however, those diagnosed as stage 1A have only 30% to
These tumors are detected typically at an early stage, 40% of long-term survivors. Treatment requires a multi-
and they may recur as late as 30 years after the initial disciplinary approach and usually requires surgery for
treatment. FOXL2 & DICER1 are the new identified early stage disease followed by chemotherapy and pelvic
molecular mutated genes involved in the oncogenic radiation. For more advanced tumours, neo-adjuvant
process of adult Granulosa cell tumor and Sertoli chemotherapy followed by surgery should be considered
Leydig tumor respectively. Because 70% of the patients followed by further chemotherapy thereafter. Prognosis
present with stage I tumors, surgery represents the major remains poor for cases beyond stage 1 with few surviving
therapeutic arm. There are no data to support any kind of long term but most seroes show a 30-40% 5-year survival
postoperative adjuvant treatment for patients with stage for early stage. SMARCA4 has been reported as a new
I sex-cord stromal tumors, given the indolent nature of molecular marker but to date no candidate drug has been
these neoplasms and the overall good prognosis (except identified for therapy. New treatments are required to
for poorly differentiated Sertoli Leydig tumor). The improve the outlook.
long natural history of the disease may lead to repeated
surgical procedure should a relapse occurs. Platinum- Ovarian Squamous Cell Carcinoma
based chemotherapy is currently used for patients with (Glasspool et al., 2014)
advanced stage sex-cord stromal tumors or recurrent
disease, with an overall response rate of 63-80%. The Squamous cell carcinoma of the ovary is a rare
indolent nature of SCSTs with the tendency for late complication of mature cystic teratoma (MCT). The
recurrence requires long-term follow up. prognosis for stage 1a disease is good but falls rapidly
with advancing stage with a 5YS of 76%, 34%, 21% and
Ovarian Neuroendocrine Tumors 0% in stage I, II, III and IV respectively. Abdominal
(Reed et al., 2014a) pain, swelling or a palpable mass are the most common
presenting symptoms but it is often an unexpected
Neuroendocrine tumors (NETs) are a heterogeneous finding at surgery. Preoperative diagnosis increases the
group of neoplasms most commonly occurring in chance of complete resection without tumour spillage.
the gastrointestinal tract or the lungs, but over the Hence there is considerable interest in identifying factors
past 30 years, there have been reported around 200 that might predict for malignancy. There are insufficient
cases of ovarian NETs. Neuroendocrine tumors in the data to provide clear guidance on management. Surgical
gynaecologic tract are uncommon and account for resection with the aim of complete cytoreduction,
about 2% of all gynaecologic malignancies but may also including total abdominal hysterectomy, bilateral
be metastatic from other sites, therefore they require salpingo-oophorectomy and omentectomy should be
exclude a metastatic origin. The ovarian NETs requiere performed. There are reports of rapid progression of
a multimodality therapeutic approach determined by the disseminated tumour after spillage of cyst contents
extent of disease and the primary organ of involvement. and hence laparascopic surgery should be avoided. The
Pathological diagnosis is critical to guide therapy. benefits of adjuvant treatment with chemotherapy and
Surgery is the cornerstone of treatment for localized radiotherapy are unclear. There are reports of responses
disease. There have been many new developments for to chemotherapy in advanced disease, with or without
treatment of advanced NETs including somatostatin radiation, but these appear to be the exception and in
analogs, hepatic artery embolization, chemotherapy, all series the outcome for those with advanced disease
interferons, mammalian target of rapamycin inhibitors or recurrent disease is very poor regardless of therapy.
and radiolabeled somatostatin analogs. There is no universally accepted regimen but platinum
