Embryonal rhabdomyosarcoma: A rare oral tumor

Abstract

Rhabdomyosarcoma is the malignant neoplasm of striated muscle and a relatively uncommon tumor of the oral cavity. Embryonal variety is the most common subtype, observed in children below 10 years of age but occasionally seen in adolescents and young adults. The present report describes a case of embryonal rhabdomyosarcoma in the left posterior buccal mucosa, with extension in the adjacent alveolus, soft palate, oropharynx and nasopharynx of a 17-year-old female.

Rhabdomyosarcoma involving maxilla mimicking gingival enlargement: A diagnostic challenge

“…RMS mainly affects children in the first two decades of life, the highest incidence being reported in the age group of 2–6 years 1 4–6 11. It accounts for <1% of all malignancies in adults, and is rare in individuals above 45 years of age 2 3 5 7 11 25 26.…”

“…The head and neck region accounts for 35%–40% of all RMS cases and the areas involved are broadly categorised into three main groups (i) the parameningeal area (which includes the nasal cavity, nasopharynx, paranasal sinuses, middle ear and skull base), (ii) the orbit and (iii) the non-orbital–non-parameningeal regions (involving deeper areas like the soft tissues of the neck, salivary glands, larynx, middle and lower pharynx, thyroid and superficial sites such as scalp, pinna and face) 6 8 9 25. About 10%–12% cases of head and neck RMS involve the oral cavity, mainly affecting the palate, tongue, lips and buccal mucosa 1 3 7 11–13. A few cases of intraosseous RMS have also been reported where the involvement of the maxilla is two times more common than the mandible 1 7.…”

“…Management of RMS of head and neck requires a multidisciplinary approach. Radical surgical excision along with reconstruction, followed by administration of multiagent chemotherapy, with or without radiotherapy remains the mainstay of treatment 1 5 23. The patient under discussion was referred to the department of oral and maxillofacial surgery where, she underwent local surgical excision of the tumour mass including the entire maxilla, along with hard palate and left inferior turbinate under general anaesthesia (figure 6A,B).…”

“…Rhabdomyosarcoma (RMS) is a rare, rapidly growing, malignant soft tissue neoplasm, originating from primitive embryonic mesenchymal cells, that retain their ability to differentiate into skeletal muscle. However, it may also originate at locations where skeletal muscle tissue is normally absent or is minimal 1 2…”

“…The head and neck region is commonly involved in children, whereas in adults, the trunk and extremities are mainly affected 7 9 10. Areas involved in the head and neck region are divided into three main groups, cranial parameningeal, orbital and non-orbital–non-parameningeal which accounts for 35%–40% of all RMS cases, of which 10%–12% cases involve the oral cavity, with an affinity for the palate, tongue, lips and buccal mucosa 1 3 7 8 11–13. RMS exhibits more aggressive behaviour and an overall worse survival rate in adults, than in children 5.…”