Adult Laryngeal Rhabdomyosarcoma: Report of a Case and Literature Review

Rhabdomyosarcoma is relatively seen in the pediatric age group with the head and neck region as the commonest site. To the best of our knowledge, few cases of laryngeal involvement in adult have been described in the literature. Biologically, rhabdomyosarcoma is different from squamous cell carcinoma, which is the commonest tumor of the larynx. A previously healthy non-smoker 77-year-old lady presented to the ENT outpatient with a six weeks history of intermittent alteration of voice quality. She had no history of sore throat, or any symptoms suggesting laryngo-pharyngeal reflux. Examination showed asymmetry of the left arytenoid cartilage and aryepiglottic fold. She subsequently had a direct laryngoscopy and biopsy. Histology and immunohistochemistry examination suggested the diagnosis of mesenchymal neoplasm. Following discussion at MDT she subsequently had a total laryngectomy. Histology confirmed a completely excised laryngeal rhabdomyosarcoma. Rhabdomyosarcoma of larynx in adult is a rare disease. Surgical treatment with or without adjuvant radiotherapy is currently the treatment of choice for this disease.Le Rhabdomyosarcome est relativement connu dans le groupe d'\ue2ge p\ue9diatrique et les r\ue9gions les plus touch\ue9es sont la t\ueate et le cou. A notre connaissance, peu de cas de l'implication du larynx chez des adultes ont \ue9t\ue9 cit\ue9s dans la litt\ue9rature. Biologiquement, rhabdomyosarcome est diff\ue9rent de la cellule squameuse carcinome, qui est la tumeur de larynx la plus ordinaire. Une femme non-fumeuse, \ue2g\ue9e de 77 ans d'une tr\ue8s bonne sant\ue9 au d\ue9part, s'est pr\ue9sent\ue9e \ue0 l'ORL ambulatoire avec une p\ue9riode de six semaines histoires de l'alt\ue9ration r\ue9p\ue9t\ue9e de la qualit\ue9 de la voix. Elle n'avait pas d'ant\ue9c\ue9dente de maux de gorge ou aucun sympt\uf4mes suggestifs le reflux laryngo-pharyngien. L'examen a montr\ue9 l'asym\ue9trie de la gauche aryt\ue9no\uefde cartilage aryepiglottique pli. Elle a par la suite eu une laryngoscopie directe et la biopsie. L'examen histologique et immunohistochimie a sugg\ue9r\ue9 le diagnostique des tumeurs mesenchymteuses, n\ue9oplasme. A la suite d'une discussion au MDT elle a ensuite eu une laryngectomie totale. L'histologie a confirm\ue9 une excis\ue9e larynx chez des adultes qui est une maladie rare. Le traitement chirurgical avec ou sans radioth\ue9rapie adjuvante est actuellement le traitement du choix pour cette maladie

Laryngeal embryonal rhabdomyosarcoma in an adult - A case presentation in the eyes of geneticists and clinicians

<p>1. Abstract</p> <p>Background</p> <p>Rhabdomyosarcoma is a solid tumor, resulting from dysregulation of the skeletal myogenesis program. For rhabdomyosarcomas (RMS) with a predilection for the head and neck, genitourinary tract, extremities, trunk, retroperitoneum, the larynx is still an unusual site. Till now only several cases of this laryngeal tumor have been described in world literature in the adult population. The entire spectrum of genetic factors underlying RMS development and progression is unclear until today. Multiple signaling pathways seem to be involved in ERMS development and progression.</p> <p>Case presentation</p> <p>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.</p> <p>Conclusions</p> <p>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.</p

Dysplastic bone disease mimicking exostoses of the ear canal

No abstract available

Adult Laryngeal Rhabdomyosarcoma: Report of a Case and Literature Review

Rhabdomyosarcoma is relatively seen in the pediatric age group with the head and neck region as the commonest site. To the best of our knowledge, few cases of laryngeal involvement in adult have been described in the literature. Biologically, rhabdomyosarcoma is different from squamous cell carcinoma, which is the commonest tumor of the larynx. A previously healthy non-smoker 77-year-old lady presented to the ENT outpatient with a six weeks history of intermittent alteration of voice quality. She had no history of sore throat, or any symptoms suggesting laryngo-pharyngeal reflux. Examination showed asymmetry of the left arytenoid cartilage and aryepiglottic fold. She subsequently had a direct laryngoscopy and biopsy. Histology and immunohistochemistry examination suggested the diagnosis of mesenchymal neoplasm. Following discussion at MDT she subsequently had a total laryngectomy. Histology confirmed a completely excised laryngeal rhabdomyosarcoma. Rhabdomyosarcoma of larynx in adult is a rare disease. Surgical treatment with or without adjuvant radiotherapy is currently the treatment of choice for this disease.Le Rhabdomyosarcome est relativement connu dans le groupe d&apos;âge pédiatrique et les régions les plus touchées sont la tête et le cou. A notre connaissance, peu de cas de l&apos;implication du larynx chez des adultes ont été cités dans la littérature. Biologiquement, rhabdomyosarcome est différent de la cellule squameuse carcinome, qui est la tumeur de larynx la plus ordinaire. Une femme non-fumeuse, âgée de 77 ans d&apos;une très bonne santé au départ, s&apos;est présentée à l&apos;ORL ambulatoire avec une période de six semaines histoires de l&apos;altération répétée de la qualité de la voix. Elle n&apos;avait pas d&apos;antécédente de maux de gorge ou aucun symptômes suggestifs le reflux laryngo-pharyngien. L&apos;examen a montré l&apos;asymétrie de la gauche aryténoïde cartilage aryepiglottique pli. Elle a par la suite eu une laryngoscopie directe et la biopsie. L&apos;examen histologique et immunohistochimie a suggéré le diagnostique des tumeurs mesenchymteuses, néoplasme. A la suite d&apos;une discussion au MDT elle a ensuite eu une laryngectomie totale. L&apos;histologie a confirmé une excisée larynx chez des adultes qui est une maladie rare. Le traitement chirurgical avec ou sans radiothérapie adjuvante est actuellement le traitement du choix pour cette maladie