Rhabdomyoma is an uncommon benign mesenchymal tumor with skeletal muscle differentiation that may occur either in the heart or in extra-cardiac sites. Even though the head and neck region is the most common area of extra-cardiac rhabdomyoma, the larynx is rarely involved. We present the case of an 85-year-old woman who reported a 10-day history of breathing difficulties, dysphagia and dysphonia. A computed tomography scan of the head and neck showed a contrast-enhanced, solid hypopharyngeal-laryngeal neoplasm with well-defined margins causing subtotal obliteration of the right pyriform sinus and a reduction in air lumen of the laryngeal vestibule. The patient underwent complete endoscopic removal of the lesion; histologic examination revealed an adult- type rhabdomyoma based on the histologic features and the immunoreactivity of the neoplastic cells for desmin, myoglobin and muscle-specific actin but not for cytokeratin, S-100, CD68R, chromogranin-A and synaptophysin. Since clinical and imaging features are not specific for rhabdomyoma, histologic examination and immunohistochemical analyses play a central role in the differential diagnosis of the adult-type rhabdomyoma from other laryngeal neoplasms. A correct diagnosis is mandatory to avoid inappropriate treatment.

Adult-type rhabdomyoma of the larynx: clinicopathologic study of an uncommon tumor in a rare location / Altissimi, Giancarlo; Ralli, Massimo; Sementilli, Giulio; FIORENTINO, FRANCESCO; Ciofalo, Andrea; Greco, Antonio; de Vincentiis, Marco; Corsi, Alessandro; Cianfrone, Giancarlo. - In: CASE REPORTS IN OTOLARYNGOLOGY. - ISSN 2090-6773. - ELETTRONICO. - 2017(2017), pp. 1-6. [10.1155/2017/7186768]

Adult-type rhabdomyoma of the larynx: clinicopathologic study of an uncommon tumor in a rare location

Altissimi, Giancarlo
Primo
;
Ralli, Massimo
Secondo
;
Sementilli, Giulio;FIORENTINO, FRANCESCO;Ciofalo, Andrea;Greco, Antonio;de Vincentiis, Marco;Corsi, Alessandro
Penultimo
;
Cianfrone, Giancarlo
Ultimo
2017

Abstract

Rhabdomyoma is an uncommon benign mesenchymal tumor with skeletal muscle differentiation that may occur either in the heart or in extra-cardiac sites. Even though the head and neck region is the most common area of extra-cardiac rhabdomyoma, the larynx is rarely involved. We present the case of an 85-year-old woman who reported a 10-day history of breathing difficulties, dysphagia and dysphonia. A computed tomography scan of the head and neck showed a contrast-enhanced, solid hypopharyngeal-laryngeal neoplasm with well-defined margins causing subtotal obliteration of the right pyriform sinus and a reduction in air lumen of the laryngeal vestibule. The patient underwent complete endoscopic removal of the lesion; histologic examination revealed an adult- type rhabdomyoma based on the histologic features and the immunoreactivity of the neoplastic cells for desmin, myoglobin and muscle-specific actin but not for cytokeratin, S-100, CD68R, chromogranin-A and synaptophysin. Since clinical and imaging features are not specific for rhabdomyoma, histologic examination and immunohistochemical analyses play a central role in the differential diagnosis of the adult-type rhabdomyoma from other laryngeal neoplasms. A correct diagnosis is mandatory to avoid inappropriate treatment.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/1044601
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