Sarcoma botryoides
Encyclopedia
Sarcoma botryoides or botryoid sarcoma or botryoid rhabdomyosarcoma is a subtype of embryonal rhabdomyosarcoma
, that can be observed in the walls of hollow, mucosa lined structures such as the nasopharynx, common bile duct, urinary bladder
of infants and young children or the vagina
in females, typically younger than age 8. The name comes from the gross appearance of "grape bunches" (botryoid in Greek).
one can see rhabdomyoblasts that may contain cross-striations. Tumor
cells are crowded in a distinct layer beneath the vaginal epithelium
( cambium layer).
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.
s occurs at age 3 years (38.3 months) on average. Cases of older women with this condition have also been reported.
. New multidrug chemotherapy
regimens with or without radiation therapy
are now used in combination with less radical surgery with good results, although outcome data are not yet available.
Embryonal rhabdomyosarcoma
Embryonal rhabdomyosarcoma is a rare histological form of cancer of connective tissue wherein the mesenchymally-derived malignant cells resemble the primitive developing skeletal muscle of the embryo...
, that can be observed in the walls of hollow, mucosa lined structures such as the nasopharynx, common bile duct, urinary bladder
Urinary bladder
The urinary bladder is the organ that collects urine excreted by the kidneys before disposal by urination. A hollow muscular, and distensible organ, the bladder sits on the pelvic floor...
of infants and young children or the vagina
Vagina
The vagina is a fibromuscular tubular tract leading from the uterus to the exterior of the body in female placental mammals and marsupials, or to the cloaca in female birds, monotremes, and some reptiles. Female insects and other invertebrates also have a vagina, which is the terminal part of the...
in females, typically younger than age 8. The name comes from the gross appearance of "grape bunches" (botryoid in Greek).
Histology
Under the microscopeMicroscope
A microscope is an instrument used to see objects that are too small for the naked eye. The science of investigating small objects using such an instrument is called microscopy...
one can see rhabdomyoblasts that may contain cross-striations. Tumor
Tumor
A tumor or tumour is commonly used as a synonym for a neoplasm that appears enlarged in size. Tumor is not synonymous with cancer...
cells are crowded in a distinct layer beneath the vaginal epithelium
Epithelium
Epithelium is one of the four basic types of animal tissue, along with connective tissue, muscle tissue and nervous tissue. Epithelial tissues line the cavities and surfaces of structures throughout the body, and also form many glands. Functions of epithelial cells include secretion, selective...
( cambium layer).
Clinical characteristics
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 cancerVaginal cancer
Vaginal cancer is any type of cancer that forms in the tissues of the vagina. Primary vaginal cancer is rare in the general population of women and is usually a squamous carcinoma. Metastases are more common. Vaginal cancer occurs more often in women over age 50, but can occur at any age, even in...
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.
Epidemiology
Sarcoma botryoides normally is found in children under 8 years of age. Onset of symptomSymptom
A symptom is a departure from normal function or feeling which is noticed by a patient, indicating the presence of disease or abnormality...
s occurs at age 3 years (38.3 months) on average. Cases of older women with this condition have also been reported.
Treatment and prognosis
The disease used to be uniformly fatal, with a 5-year survival rate between 10 to 35%. As a result, treatment was radical surgeryRadical surgery
Radical surgery, also called radical dissection, refers to the removal of blood supply, lymph nodes and sometimes adjacent structures of a diseased organ or tumor during surgery. In surgical oncology, radical surgery typically describes the removal of a tumor or mass and ancillary lymph nodes that...
. New multidrug chemotherapy
Chemotherapy
Chemotherapy is the treatment of cancer with an antineoplastic drug or with a combination of such drugs into a standardized treatment regimen....
regimens with or without radiation therapy
Radiation therapy
Radiation therapy , radiation oncology, or radiotherapy , sometimes abbreviated to XRT or DXT, is the medical use of ionizing radiation, generally as part of cancer treatment to control malignant cells.Radiation therapy is commonly applied to the cancerous tumor because of its ability to control...
are now used in combination with less radical surgery with good results, although outcome data are not yet available.