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GCIG Rare Tumor Consensus Review & Guidelines 1523
and when confined within the ovary, the prognosis is
good. However, advanced disease is associated with a
very poor prognosis and resistance to standard treatment.
Cytoreductive surgery should be performed for patients
with stage II, III, or IV disease. In a first OCCC-specific combination of carboplatin-paclitaxel is the most
international phase III study to compare irinotecan/
cisplatin (CPT-P) and paclitaxel/ carboplatin (TC) as
first line chemotherapy for stage I-IV OCCC, a survival
benefit was not observed by CPT-P (GCIG/ JGOG3017).
Considering the frequent PIK3CA mutation in CCC, dual
inhibitors targeting PI3K, AKT in the mTOR pathway,
are promising. Performing these trials and generating
the evidence will require considerable international
collaboration
Uterine Clear Cell Carcinomas
(Hasegawa et al., 2014)
Clear cell carcinomas of the uterine corpus and cervix
are rare gynaecological cancers with limited information
regarding the pathogenesis and biology. Association with
the exposure to prenatal diethylstilbestrol and increased
risk of its occurance has been suggested. At present, the
approach to management is the same as for patients with
the more common histological subtypes of endometrioid
endometrial cancer and adenocarcinoma of the cervix.
Surgical resection is the standard treatment for patients
with early-stage disease, but there is no evidence-based
approach to direct the management of patients with
more advanced-stage disease at presentation or with
recurrent disease. CCAC is refractory to chemotherapy
and radiation therapy. Patients who could not complete
the optimal resection have extremely poor prognosis.
Uterine & Ovarian Carcinosarcomas
(Berton-Rigaud et al., 2014)
Carcinosarcomas (also known as malignant mixed
mullerian tumors) are rare and aggressive epithelial
malignancies that contain both malignant sarcomatous
and carcinomatous elements. Uterine carcinosarcomas
(UCs) are uncommon with approximately more than
35% presenting with extra uterine disease at diagnosis.
Up to 90% ovarian carcinosarcomas (OCs) will have
disease that has spread beyond the ovary. Prognosis for
localized stage disease is poor with a high risk of local
and distant recurrences, occurring within 1 year. No
improvement in survival rates has been observed in the
past decades with an overall median survival of less than
2 years. Currently, there is no clear evidence to establish cm in size, and negative lymphovascular space invasion,
consensus guidelines for therapeutic management
of carcinosarcomas. Until recently, gynaecological
carcinosarcomas were considered as a subtype of sarcoma
and treated as such. However, carcinosarcomas are now
known to be metaplastic carcinomas and so should be
treated as gynaecological high-risk carcinomas. For
UCs, a complete surgical staging followed by systemic
chemotherapy is recommended for patients with both
early and advanced stage disease. Active agents include
paraplatin, cisplatin, ifosfamide, and paclitaxel. The
commonly used regimen. Adjuvant radiotherapy (external
beam irradiation and/or vaginal brachytherapy) has not
shown any overall survival benefit but has been reported
to decrease local recurrences. For OCs ,the mainstay of
treatment remains cytoreductive surgery followed, even
in early stage, by platinum-based chemotherapy, usually
carboplatin-paclitaxel.
Uterine Serous Carcinoma
(Sagae et al., 2014)
Uterine serous carcinoma (USC) represents a rare and
aggressive histologic subtype of endometrial cancer,
associated with a poor prognosis. Both USC and
approximately 25% of high-grade endometrioid tumors
represent extensive copy number alterations, few DNA
methylation changes, low estrogen and progesterone
levels, and frequent P53mutations. Uterine serous
carcinoma shares molecular characteristics with ovarian
serous and basal-like breast carcinomas. In addition
to optimal surgery, platinum- and taxane-based
chemotherapy should be considered in the treatment of
both early- and advanced-stage disease. The combination
of radiation and chemotherapy appears to be associated
with the highest survival rates. The role of radiation
therapy in the management of this disease, with a high
propensity for distant failures, remains elusive. Uterine
serous carcinoma is a unique and biologically aggressive
subtype of endometrial cancer and should be studied
as a distinct entity. Futures studies should identify
the optimized chemotherapy and radiation regimens,
sequence of therapy and schedule, and the role of targeted
biologic therapy.
Cervical Adenocarcinoma
(Fujiwara et al., 2014)
Cervical adenocarcinoma is known to be less common
than squamous cell carcinoma of the cervix comprising
approximately 25% of all cervical carcinomas.
Differences in associated human papillomavirus types,
patterns of spread, and prognosis call for treatments that
are not always like those for squamous cancers. Radical
hysterectomy or CCRT for patients with small tumors, <2
the survival difference between AC and SCC is negligible,
so the treatment strategy for these AC patients of should
be same as that for SCC patients. In patients with tumor
sizes >4 cm and progressively advanced disease, CCRT is
the primary treatment. Consideration of systemic therapy
with cisplatin/carboplatin and paclitaxel is reasonable