Rhabdomyosarcoma of the clitoris

Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 1

“…48,50 Early onset is typical of RMS, frequently before the age of 5 years, with aggressive behavior and predominantly affecting the male gender. 51 Forty percent of the cases are located in the head and neck, and only 25% of all RMS are located on the genitalia -vulva, vagina, bladder, or cervix. 48,51 Although vulvar location is rare, it is associated with a more favorable prognosis.…”

“…51 Forty percent of the cases are located in the head and neck, and only 25% of all RMS are located on the genitalia -vulva, vagina, bladder, or cervix. 48,51 Although vulvar location is rare, it is associated with a more favorable prognosis. 48,52 Most of the cases occur sporadically, but genetic factors have been found in about 30% of patients, and they appear to be a risk factor for the development of RMS.…”

“…63 The diagnosis of RMS is based on the histological features and additional diagnostic methods such as fine needle aspiration biopsy with subsequent cytological analysis and immunocytochemical staining for markers of myogenic differentiation. 51,64 Expression of nuclear transcription factor myogenin (myf4) and MyoD1, myogenic transcriptional regulatory proteins that are highly specific markers for the identification of skeletal muscle differentiation, is characteristic of these tumors. 51 It should be borne in mind that, in rare cases, focal immunoreactivity to myogenin can be seen in fibroepithelial polyps, which can lead to diagnostic mistakes.…”

“…51,64 Expression of nuclear transcription factor myogenin (myf4) and MyoD1, myogenic transcriptional regulatory proteins that are highly specific markers for the identification of skeletal muscle differentiation, is characteristic of these tumors. 51 It should be borne in mind that, in rare cases, focal immunoreactivity to myogenin can be seen in fibroepithelial polyps, which can lead to diagnostic mistakes. 60 Carcinocythemia, representing the leukemic phase of a solid tumor, has been reported in isolated cases in association with alveolar rhabdomyosarcoma.…”

“…65 Local invasion, regional lymph node spread, and therapeutic response are important prognostic markers in RMS. 51,61 Factors associated with a favorable prognosis are embryonal/botryoid histology, tumor size < or = 5 cm, with no metastases and age below 10 years at the time of diagnosis. 66 ).…”