Clinico-epidemiological analysis of orofacial rhabdomyosarcoma in a Nigerian population

Sinonasal Alveolar Rhabdomyosarcoma in an Adult Patient: A Case Report and Review of the Literature

“…[5] The annual incidence of RMS in the USA is 4.6 per million in people under 20 years of age. RMS may occur in all age groups but is more prevalent in the first and second decades of life with a peak between 2 and 6 years of age, [6] It represents approximately 4 -8% of all pediatric cancers. [7] Although tumors of head and neck are rare in children [8], approximately 60% of pediatric RMS cases occur in the head and neck.…”

“…[9] Four different histopathological types have been described: embryonic, alveolar, pleomorphic and undifferentiated. [6] The two most common histopathological types described in childhood are embryonic and alveolar. [11] The embryonic type represents 70% of cases and it is mainly seen in children under the age of 12 and carries the best prognosis.…”

“…[12] The pleomorphic variety is less frequent and occurs more often in an older population. [6] Anatomically, RMS is classified as parameningeal, orbital and non-parameningeal non-orbital. Approximately 40% of newly diagnosed RMS arises in the head and neck structures including parameningeal sites (16% of all cases, and almost half of all head and neck cases), the orbit or eyelid (10% of all cases), and other non-orbit, non-parameningeal sites (10% of all cases).…”

“…The parameningeal tumors carry the worst prognosis. [1,6] The parameningeal sites include nose, nasopharynx, paranasal sinuses, middle ear, mastoid, infratemporal fossa and pterygopalatine fossa. Soderberg described the first case of an aggressive RMS in the middle ear.…”

“…The tongue, palate and cheeks are the most common oral sites. [6] Of the 35% of RMS that occur in the head and neck, 10-12% present in the oral cavity. RMS rarely occurs in the salivary glands.…”