Sarcoma botryoides or botryoid sarcoma is a subtype of

embryonal rhabdomyosarcoma Embryonal rhabdomyosarcoma (EMRS) is a rare histological form of cancer in the connective tissue wherein the mesenchymally-derived malignant cells resemble the primitive developing skeletal muscle of the embryo. It is the most common soft tissue s ...

, that can be observed in the walls of hollow, mucosa lined structures such as the nasopharynx, common bile duct,

urinary bladder The urinary bladder, or simply bladder, is a hollow organ in humans and other vertebrates that stores urine from the kidneys before disposal by urination. In humans the bladder is a distensible organ that sits on the pelvic floor. Urine enters ...

of infants and young children or the

vagina In mammals, the vagina is the elastic, muscular part of the female genital tract. In humans, it extends from the vestibule to the cervix. The outer vaginal opening is normally partly covered by a thin layer of mucosal tissue called the hymen ...

in females, typically younger than age 8. The name comes from the gross appearance of "grape bunches" (''botryoid'' in Greek).

Presentation

For botryoid rhabdomyosarcoma of the vagina, the most common clinical finding is vaginal bleeding but vaginal bleeding is not specific for sarcoma botryoides: other

vaginal cancer Vaginal cancer is an extraordinarily rare form of cancer that develops in the tissue of the vagina. Primary vaginal cancer originates from the vaginal tissue – most frequently squamous cell carcinoma, but primary vaginal adenocarcinoma, sarcoma ...

s are possible. They may appear as a polypoid mass, somewhat yellow in color and are friable: thus, they (possibly) may break off, leading to vaginal bleeding or infections.

Histology

Under the

microscope A microscope () is a laboratory instrument used to examine objects that are too small to be seen by the naked eye. Microscopy is the science of investigating small objects and structures using a microscope. Microscopic means being invisibl ...

one can see

rhabdomyoblast A rhabdomyoblast is a cell type which is found in some rhabdomyosarcomas. When found histologically, a rhabdomyoblast aids the diagnosis of embryonal, alveolar, spindle cell/sclerosing, and pleomorphic rhabdomyosarcomas; however, in a tumor, express ...

s that may contain cross-striations.

Tumor A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...

cells are crowded in a distinct layer beneath the

vaginal epithelium The vaginal epithelium is the inner lining of the vagina consisting of multiple layers of (squamous) cells. The basal membrane provides the support for the first layer of the epithelium-the basal layer. The intermediate layers lie upon the basal ...

( cambium layer). Spindle-shaped tumor cells that are

desmin Desmin is a protein that in humans is encoded by the ''DES'' gene. Desmin is a muscle-specific, type III intermediate filament that integrates the sarcolemma, Z disk, and nuclear membrane in sarcomeres and regulates sarcomere architecture. Str ...

positive.

Treatment

The disease used to be uniformly fatal, with a 5-year survival rate between 10 and 35%. As a result, treatment was

radical surgery Radical surgery, also called radical dissection, is surgery that is more extensive than "conservative" surgery. In surgical oncology, radical surgery is surgery intended to remove both a tumor and any metastases thereof, for diagnostic and/or tre ...

. New multidrug

chemotherapy Chemotherapy (often abbreviated to chemo and sometimes CTX or CTx) is a type of cancer treatment that uses one or more anti-cancer drugs (chemotherapeutic agents or alkylating agents) as part of a standardized chemotherapy regimen. Chemotherap ...

regimens with or without

radiation therapy Radiation therapy or radiotherapy, often abbreviated RT, RTx, or XRT, is a therapy using ionizing radiation, generally provided as part of cancer treatment to control or kill malignant cells and normally delivered by a linear accelerator. Radia ...

are now used in combination with less radical surgery with good results, although outcome data are not yet available.

Epidemiology

Sarcoma botryoides normally is found in children under 8 years of age. Onset of

symptom Signs and symptoms are the observed or detectable signs, and experienced symptoms of an illness, injury, or condition. A sign for example may be a higher or lower temperature than normal, raised or lowered blood pressure or an abnormality showin ...

s occurs at age 3 years (38.3 months) on average. Cases of older women with this condition have also been reported.

References

External links

humpath.com #12369
{{DEFAULTSORT:Sarcoma Botryoides Gynaecological cancer Pediatric cancers Rare cancers Vagina