%0 Journal Article
%T Laryngeal embryonal rhabdomyosarcoma in an adult - A case presentation in the eyes of geneticists and clinicians
%A Wojciech Kukwa
%A Piotr Wojtowicz
%A Beata Jagielska
%A Grzegorz Sobczyk
%A Andrzej Kukwa
%A Anna M Czarnecka
%J BMC Cancer
%D 2011
%I BioMed Central
%R 10.1186/1471-2407-11-166
%X In this paper we report an interesting RMS case in which the disease was located within the glottic region. We report an embryonal rhabdomyosarcoma of the larynx in 33 year-old man. After unsuccessful chemotherapy hemilaryngectomy was performed. In follow up CT no signs of recurrence were found. Recently patient is recurrence free for 62 months.Considering the histological diagnosis and the highly aggressive nature of the lesion for optimal diagnosis positron electron tomography (PET) and computerized tomography (CT) of the neck and thorax should be performed. At this time surgical treatment with adjuvant radiotherapy seems to be the treatment of choice for this disease. Rhabdomyosarcoma of the larynx has a better prognosis than elsewhere in the body, probably because of its earlier recognition and accessibility to radical surgery.More than 95% of laryngeal tumors in adults are squamous cell carcinoma. Laryngeal rhabdomyosarcomas are very rare, but extremely malignant tumors. Rhabdomyosarcoma must certainly rank close to, if not the least common of the laryngeal sarcomas. Most of reported cases have occurred in children [1]. Rhabdomyosarcoma arises from undifferentiated mesodermal tissue. It accounts for 40% of sarcomas found in the head and neck region. Among sarcomas arising in the larynx, rhabdomyosarcoma is one of the rarest. The first case of rhabdomyosarcoma of the larynx was reported by Glick in 1944 [2]. On gross pathology, rhabdomyosarcomas are soft, reddish-brown and lobulated. There are four principal histological varieties of rhabdomyosarcoma: embryonal, alveolar, pleomorphic and botryoid according to their degree of cellular differentiation and maturity. Adult rhabdomyosarcomas are usually composed of closely packed round cells with peripherally located nuclei. The cells have glycogen-rich, eosinophilic, vacuolated cytoplasm with cross-striations. Nevertheless they may have present cytological variability and resemble different stages of skeletal muscle
%U http://www.biomedcentral.com/1471-2407/11/166