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1524 GCIG Rare Tumor Consensus Review & Guidelines
based on current evidence for advanced and metastatic
disease. At present, the same chemotherapy regimen
might be recommended for both AC and SCC: paclitaxel
plus cisplatin plus bevacizumab as standard treatment
and paclitaxel and carboplatin as alternative treatment
for patient not able to receive bevacizumab (JCOG0505).
Prospective clinical trials are needed to help develop
treatment guidelines and explore the role of neo adjuvant
chemotherapy or exact sensisitivity to radiation therapy
for such histological subgroup.
Cervix Small Cell Carcinoma
(Satoh et al., 2014)
Small cell carcinoma of the cervix (SCCC) is a rare
histological entity of uterine cervical cancer. Compared
with other common histological types, squamous cell
carcinoma or adenocarcinoma, the outcome of SCCC
is poor because of the high incidence of nodal and/
or distant metastasis even with early stage. There are
insufficient data to provide clear guidance on the optimal
management strategy, though multimodal therapy with
surgery, chemotherapy (such as cisplatin and etoposide)
and radiation therapy is often recommended.
Uterine Leiomyosarcoma
(Hensley et al., 2014)
Uterine sarcomas represent about 8% of uterine cancers,
with an incidence of about 0.4 per 100,000 women.
Leiomyosarcomas are the most common subtype; most
are high grade malignancies with a high risk for recurrence
and progression. Overall survival is dependent on stage
with 5-year survival estimates of stage I: 76%, stage II:
60%, stage III: 45%, and stage IV: disease 29% . Uterine
leiomyosarcomas are staged using the FIGO 2009 uterine
sarcoma staging system. Other factors that have been
evaluated for their potential prognostic impact include
tumor morcellation , mitotic index and tumor grade.
For patients whose disease appears limited to the uterus,
hysterectomy of the intact uterus, without morcellation,
is recommended. Although it is recognized that the
risk for recurrence after resection of uterus-limited
high grade LMS exceeds 50%, no adjuvant intervention
has been shown to improve progression-free or overall
survival outcomes. Standard management after complete
resection of uterus-limited disease is observation. For
patients with locally advanced, completely resected
uterine LMS, there are no prospective data upon which
to base management recommendations. Choices may
include observation, adjuvant radiation, adjuvant
hormone blockade, or adjuvant chemotherapy. For
metastatic uterine LMS, systemic treatment is the
standard of care. There is no established superior first line with a very bad prognosis (undifferenciated sarcomas) or
chemotherapy regimen. Treatment recommendations for
an individual patient should take into consideration the
patient’s preferences for the treatment schedule, drug side
effects, venous access, co-morbidities, disease burden,
disease pace, and organ function. Reasonable regimens
to consider for first-line therapy include gemcitabine plus
docetaxel, doxorubicin, doxorubicin plus ifosfamide, and
gemcitabine. Other treatment options, used as second-
line therapy or after, include trabectedin, ifosfamide,
pazopanib, dacarbazine or temozolomide, and eribulin.
For patients with indolent disease and low tumor burden
whose tumors express ER or PR aromatase inhibition
may be considered. For patients with single-site or
oligometastatic disease and a long disease-free interval
metastatectomy may be considered.
Endometrial Stromal Sarcoma
(Amant et al., 2014)
Endometrial stromal sarcoma (ESS) accounts for
approximately 20% of all uterine sarcomas and presents,
at a mean age, around 50 years of age. Half of the patients
are premenopausal. ESS often manifests as an endometrial
polyp and 60% of cases present with FIGO stage I disease.
The natural history is one of slow growing indolent
disease. Typical microscopic findings include a uniform
population of endometrial stromal-type cells invading the
myometrium and myometrial vessels. Imaging studies
cannot reliably diagnose ESS preoperatively, so surgical
resection for a presumed fibroid is a common scenario.
Hysterectomy is the cornerstone of treatment for localized
ESS, but morcellation should be avoided. Systematic
lymphadenectomy in ESS does not improve the outcome.
Leaving the ovaries in situ does not worsen survival and
this is of importance especially for young women. The
data support the current practice to administer adjuvant
hormonal treatment, although several questions remain,
such as optimal doses, regimens (progestins or aromatase
inhibitors) and duration of therapy. Repeat surgery for
recurrent disease that is indolent and hormone sensitive
appears to be an acceptable approach. Systemic treatment
for recurrent disease is mainly hormonal.
High-Grade Undifferentiated Sarcomas
(Pautier et al., 2014)
High-grade undifferentiated sarcomas (HGUSs) are
rare uterine malignancies arising from the endometrial
stroma. They are poorly differentiated sarcomas
composed of cells that do not resemble proliferative-
phase endometrial stroma. High-grade undifferentiated
sarcomas are characterized by aggressive behavior and
poor prognosis. They can have complex karyotypic
modifications but lack a unifying tumor-specific
alteration with no cyclin D1 nor ER and PR expression
be associated with an YWHAE-FAM22 rearrangement
and cyclin D1 expression (sometimes ER/PR positivity)