Case report of a sarcoma botryoides of the uterine cervix in fertile age and literature review

Abstract

Although the combined modality approach has given, if not constant, acceptable outcomes, a larger investigation on all the cases of sarcoma botryoides of the cervix uteri is expected.

Embryonal rhabdomyosarcoma of the uterine cervix: a rare case report

“…5 Furthermore, information regarding this tumour is difficult to obtain from the literature because the primary place of origin is not always known or described. 4 Usually, it looks like a polypoid fragile mass, measuring around 2-9.5 cm of diameter and is always associated with vaginal bleeding and sometimes a feeling of a mass in the vaginal introitus. Like sarcoma botryoides occurring at other mucosal sites, the tumour may form soft, grape-like clusters, present as single or multiple polyps.…”

“…However, a precise diagnosis is sometimes difficult, because it macroscopically resembles a benign polyp and, in about 40% of cases, it can histologically appear as a few cell mass with an edematous and laxus stroma, being confusing for pathologists. 4 Several other lesions enter into the pathologic differential diagnosis, including adenosarcoma, rhabdomyoma, edematous cervical mesodermal polyps (pseudosarcoma botryoides), and other "small, round, blue cell" tumours. 6 Adenosarcomas, a strong option that was considered in our case as a differential diagnosis, can also be found in young female, but histologically can be distinguished from sarcoma botryoides by their fibrous stroma and leaf-like pattern of glands.…”

“…Also, immunohistochemical markers that usually label ERMS are desmin, with a positivity rate of 75-100%, vimentin and myogenin, which is considered the most sensible and specific marker (70-100% of cases). 4,6 Before 1970, radical surgery was considered the gold standard. However, after consecutive trials, the IRSG has modified the treatment protocols with the addition of multiple chemotherapeutic agents to surgery, greatly improving the prognosis and survival of thistumour and also allowed more conservative fertility-sparing surgeries in early stages of the disease.…”

“…The embryonal subtype is the most common, accounting for 68% of all ERMS cases, with classic, botryoid and spindle cell variants embracing 49, 6 and 3%, respectively. 1,3,4 Sarcoma botryoides of the cervix uteri rarely occurs in fertile age since it only accounts for 0.2% of all malignant tumours of the uterus. 4 This sarcoma generally presents as soft nodules that fill and sometimes protrude from the vagina, resembling a bunch of grapes.…”

“…1,3,4 Sarcoma botryoides of the cervix uteri rarely occurs in fertile age since it only accounts for 0.2% of all malignant tumours of the uterus. 4 This sarcoma generally presents as soft nodules that fill and sometimes protrude from the vagina, resembling a bunch of grapes. 1,3 This histological subtype is associated with a very favourable outcome.…”