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GCIG Rare Tumor Consensus Review & Guidelines 1525
(and called high grade endometrial sarcomas). Median or neoplasia (GTN). GTN often arises after molar
age of the patients is between 55 and 60 years. The most (CHM/PHM) pregnancies but can also occur after any
common symptoms are vaginal bleeding, abdominal gestation including miscarriages and term pregnancies.
pain, and increasing abdominal girth. Disease is usually GTN arising after evacuation of a molar pregnancy
advanced with approximately 70% of the patients staged is most commonly diagnosed biochemically as all
III to IV according to the International Federation of patients should be regularly monitored with serum
Gynecology and Obstetrics classification. Preferential HCG assessments. Hydatidiform moles are removed
metastatic locations include peritoneum, lungs, intra- by suction and curettage ideally under ultrasound
abdominal lymph nodes, and bone. Median progression- control to ensure adequate evacuation and avoid uterine
free survival ranged from 7 to 10 months, and median perforation. Medical inductions of labor or hysterotomy
overall survival ranged from 11 to 23 months. There is are not recommended for several reasons including an
no clear prognostic factor identified for HGUS, not even increased risk of developing post molar GTN requiring
stage. The standard management for HGUS consists of chemotherapy. The FIGO criteria for commencing
total hysterectomy and bilateral salpingooophorectomy. chemotherapy for post-mole GTN are well defined. The
Systematic lymphadenectomy is not recommended. commonest reason is a rising or plateaued HCG level. An
Adjuvant therapies, such as chemotherapy and elevated but falling hCG 6 months post molar evacuation
radiotherapy, have to be discussed in multidisciplinary is no longer an absolute requirement for commencing
staff meetings. First line therapy in metastatic setting is chemotherapy since patients undergoing continued
doxorubicin based therapy. surveillance all appear to spontaneously normalize
their hCG levels. Other criteria for chemotherapy in
Mullerian Adenosarcomas of some institutions include a serum HCG level greater
The Female Genital Tract than 20000 IU/L more than four weeks after evacuation
(Friedlander et al., 2014) because of the risk of uterine perforation and uterine or
intra-abdominal hemorrhage requiring transfusion. For
Mullerian adenosarcomas of the female genital tract are post-partum GTN, chemotherapy is indicated either
rare malignancies, originally described in the uterus, when a histological diagnosis of choriocarcinoma is
which is the most common site of origin, but they can established or when there are worrying clinical features
also arise in extrauterine sites. Uterine adenosarcomas such as unexplained widespread metastasis in a woman of
make up 5% of uterine sarcomas and tend to occur in childbearing age with elevated HCG values. For women
postmenopausal women. They are usually low-grade who become refractory/resistant to first line single agent
tumors and are characterized by a benign epithelial chemotherapy, poly chemotherapy can be administered.
component with a malignant mesenchymal component,
which is typically a low-grade endometrial stromal Vulvo Vaginal Melanoma
sarcoma, but can also be a high-grade sarcoma. Tumors (Leitao, Jr. et al., 2014)
that exhibit a high-grade sarcomatous overgrowth have
a worse outcome. Adenosarcomas have been described Vulvovaginal melanomas are rare tumors that account
as being midway along the spectrum between benign for a small fraction of all vulvovaginal cancers.
adenofibromas and carcinosarcomas. They generally Biologically, they seem to be similar to mucosal and
have a good prognosis with the exception of deeply acral melanomas of other sites. There are limited
invasive tumors or those with high-grade sarcomatous data specific to vulvovaginal melanomas, especially
overgrowth. The mainstay of treatment for uterine regarding systemic therapies. The majority of treatment
adenosarcomas is hysterectomy and bilateral salpingo- decisions are based on extrapolation from data regarding
oophorectomy. In view of their rarity, there have not cutaneous melanomas of other sites. Surgery is the
been any clinical trials in mullerian adenosarcomas preferred primary treatment for vulvovaginal melanoma
and relatively little research. Management of metastatic if feasible and not requiring an exenterative procedure. A
adenosarcoma with ESS should be similar to management wide local excision of the primary tumor with a clinically
of ESS with hormonal therapy. The management of negative margin should be attempted. The exact minimal
undifferentiated/heterologous sarcomas is based on the excision margin for melanomas is not entirely proven,
principles of management of other high grade uterine but it is recommended that a clinical gross 1 cm margin
sarcomas. circumferentially be achieved for a melanoma with a
Breslow thickness of less than or equal to 2 mm and 2 cm
Gestational Trophoblastic Disease for a Breslow thickness greater than 2 mm. SLN mapping
(Mangili et al., 2014) should be considered in all patients undergoing a wide
local excision for newly diagnosed vulvar melanoma. The
The malignant forms of GTD are also collectively majority of published randomized trials do not support a
known as gestational trophoblastic tumors (GTT) survival advantage for the use of adjuvant radiotherapy,
