Sarcoma botryoides

Other names: Botryoid rhabdomyosarcoma
a-c)Contrast-enhanced computed tomography of abdomen
Specialty| Oncology

Sarcoma botryoides or botryoid sarcoma[1] 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).

## Contents

* 1 Signs and symptoms
* 2 Histology
* 3 Diagnosis
* 3.1 Differential diagnosis
* 4 Treatment
* 5 Epidemiology
* 6 References
* 7 External links

## Signs and symptoms[edit | edit source]

For botryoid rhabdomyosarcoma of the vagina, the most common clinical finding is vaginal bleeding[2] but vaginal bleeding is not specific for sarcoma botryoides: other vaginal cancers 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.[citation needed]

## Histology[edit | edit source]

Under the microscope one can see rhabdomyoblasts that may contain cross-striations. Tumor cells are crowded in a distinct layer beneath the vaginal epithelium (cambium layer). Spindle-shaped tumor cells that are desmin positive.[citation needed]

## Diagnosis[edit | edit source]

### Differential diagnosis[edit | edit source]

The DDx for Sarcoma botryoides is as follows:[3]

* Fibroepithelial polyp
* Mullerian papilloma
* Rhabdomyoma

## Treatment[edit | edit source]

The disease used to be uniformly fatal, with a 5-year survival rate between 10 and 35%.[4] As a result, treatment was radical surgery. 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.[5]

## Epidemiology[edit | edit source]

Sarcoma botryoides normally is found in children under 8 years of age. Onset of symptoms occurs at age 3 years (38.3 months) on average.[6] Cases of older women with this condition have also been reported.[7]

## References[edit | edit source]

1. ↑ "botryoid sarcoma" at Dorland's Medical Dictionary
2. ↑ Rahaman, J and Cohen, CJ. Gynecologic Sarcomas. in Holland-Frei Cancer Medicine - 6th Ed. Kufe, DW et al. editors. BC Decker Inc., Hamilton, Ontario, 2003.
3. ↑ Wu, Maoxin; Deligdisch, Liane; Llorca, Frederique Penault; Schlosshauer, Peter W.; Altchek, Albert (22 December 2008). Early Pathologic Diagnosis Of Gynecologic Cancer Including A Clinician's View. World Scientific Publishing Company. p. 99\. ISBN 978-981-310-727-4. Archived from the original on 17 October 2022. Retrieved 15 October 2022.
4. ↑ Piver M, Rose P (1988). "Long-term follow-up and complications of infants with vulvovaginal embryonal rhabdomyosarcoma treated with surgery, radiation therapy, and chemotherapy". Obstet Gynecol. 71 (3 Pt 2): 435–7. PMID 3347430.
5. ↑ Rotmensch, J and Yamada, SD. Neoplasms of the Vulva and Vagina. in Holland-Frei Cancer Medicine - 6th Ed. Kufe, DW et al. editors. BC Decker Inc., Hamilton, Ontario, 2003.
6. ↑ Hilgers R (1975). "Pelvic exenteration for vaginal embryonal rhabdomyosarcoma: a review". Obstet Gynecol. 45 (2): 175–80. PMID 1090863.
7. ↑ Reynolds E, Logani S, Moller K, Horowitz I (2006). "Embryonal rhabdomyosarcoma of the uterus in a postmenopausal woman. Case report and review of the literature". Gynecol Oncol. 103 (2): 736–9. doi:10.1016/j.ygyno.2006.03.033. PMID 16684558.

## External links[edit | edit source]

* humpath.com #12369 Archived 2021-07-29 at the Wayback Machine

Classification|

* ICD-O: 8910/3
* MeSH: D018233
* DiseasesDB: 1557
* SNOMED CT: 404052009

|

* v
* t
* e

Connective/soft tissue tumors and sarcomas

Not otherwise specified|

* Soft-tissue sarcoma
* Desmoplastic small-round-cell tumor

Connective tissue neoplasm|

| Fibromatous|

| Fibroma/fibrosarcoma:|

* Dermatofibrosarcoma protuberans
* Desmoplastic fibroma

|

Fibroma/fibromatosis:|

* Aggressive infantile fibromatosis
* Aponeurotic fibroma
* Collagenous fibroma
* Diffuse infantile fibromatosis
* Familial myxovascular fibromas
* Fibroma of tendon sheath
* Fibromatosis colli
* Infantile digital fibromatosis
* Juvenile hyaline fibromatosis
* Plantar fibromatosis
* Pleomorphic fibroma
* Oral submucous fibrosis

Histiocytoma/histiocytic sarcoma:|

* Benign fibrous histiocytoma
* Malignant fibrous histiocytoma
* Atypical fibroxanthoma
* Solitary fibrous tumor

Myxomatous|

* Myxoma/myxosarcoma
* Cutaneous myxoma
* Superficial acral fibromyxoma
* Angiomyxoma
* Ossifying fibromyxoid tumour

Fibroepithelial|

* Brenner tumour
* Fibroadenoma
* Phyllodes tumor

Synovial-like|

* Synovial sarcoma
* Clear-cell sarcoma

Lipomatous|

* Lipoma/liposarcoma
* Myelolipoma
* Myxoid liposarcoma
* PEComa
* Angiomyolipoma

* Chondroid lipoma
* Intradermal spindle cell lipoma
* Pleomorphic lipoma
* Lipoblastomatosis
* Spindle cell lipoma
* Hibernoma

Myomatous|

| general:|

* Myoma/myosarcoma

|

smooth muscle:|

* Leiomyoma/leiomyosarcoma

skeletal muscle:|

* Rhabdomyoma/rhabdomyosarcoma: Embryonal rhabdomyosarcoma
* Sarcoma botryoides
* Alveolar rhabdomyosarcoma

* Leiomyoma
* Angioleiomyoma
* Angiolipoleiomyoma
* Genital leiomyoma
* Leiomyosarcoma
* Multiple cutaneous and uterine leiomyomatosis syndrome
* Multiple cutaneous leiomyoma
* Neural fibrolipoma
* Solitary cutaneous leiomyoma
* STUMP

Complex mixed and stromal|

* Adenomyoma
* Pleomorphic adenoma
* Mixed Müllerian tumor
* Mesoblastic nephroma
* Wilms' tumor
* Malignant rhabdoid tumour
* Clear-cell sarcoma of the kidney
* Hepatoblastoma
* Pancreatoblastoma
* Carcinosarcoma

Mesothelial|

* Mesothelioma
* Adenomatoid tumor

* v
* t
* e

Tumors of the female urogenital system

Adnexa|

| Ovaries|

| Glandular and epithelial/
surface epithelial-
stromal tumor|

| CMS:|

* Ovarian serous cystadenoma
* Mucinous cystadenoma
* Cystadenocarcinoma
* Papillary serous cystadenocarcinoma
* Krukenberg tumor

|

* Endometrioid tumor
* Clear-cell ovarian carcinoma
* Brenner tumour

Sex cord–gonadal stromal|

* Leydig cell tumour
* Sertoli cell tumour
* Sertoli–Leydig cell tumour
* Thecoma
* Granulosa cell tumour
* Luteoma
* Sex cord tumour with annular tubules

Germ cell|

* Dysgerminoma
* Nongerminomatous
* Embryonal carcinoma
* Endodermal sinus tumor
* Gonadoblastoma
* Teratoma/Struma ovarii
* Choriocarcinoma

Fibroma|

* Meigs' syndrome

Fallopian tube|

* Adenomatoid tumor

Uterus|

| Myometrium|

* Uterine fibroids/leiomyoma
* Leiomyosarcoma
* Adenomyoma

|

Endometrium|

* Endometrioid tumor
* Uterine papillary serous carcinoma
* Endometrial intraepithelial neoplasia
* Uterine clear-cell carcinoma

Cervix|

* Cervical intraepithelial neoplasia
* Clear-cell carcinoma
* SCC
* Glassy cell carcinoma
* Villoglandular adenocarcinoma

Placenta|

* Choriocarcinoma
* Gestational trophoblastic disease

General|

* Uterine sarcoma
* Mixed Müllerian tumor

Vagina|

* Squamous-cell carcinoma of the vagina
* Botryoid rhabdomyosarcoma
* Clear-cell adenocarcinoma of the vagina
* Vaginal intraepithelial neoplasia
* Vaginal cysts

Vulva|

* SCC
* Melanoma
* Papillary hidradenoma
* Extramammary Paget's disease
* Vulvar intraepithelial neoplasia
* Bartholin gland carcinoma

*[v]: View this template
*[t]: Discuss this template
*[e]: Edit this template