Embryonal Rhabdomyosarcoma of the Larynx: A rare adult neoplasm

Adult laryngeal rhabdomyosarcoma, is a very rare diagnosis, commonly found affecting the younger age group1. This frequently arises in the head and neck and genitourinary tract, with larynx being an unusual site2. About 22 cases have been reported so far with first being in 1922, because of this scarce data is available to guide the appropriate management. This makes it the very first case to be reported from Pakistan. Our case is of a 63 years old man who presented with a 4-month history of gradually worsening hoarseness, his work-up comprising flexible laryngoscopy, an exophytic growth appeared to be arising from pharyngeal surface of epiglottis, looked like a sub mucosal mass with protrusion in the glottis, due to which vocal cord and sub glottis was not visualized. CT scan was done, which demonstrated a well-defined necrotic mass lesion arising from posterior aspect of epiglottis resulting in luminal narrowing and mild effacement of vallecula and pyriform sinus, measuring 2.9x1.3x2.9 cm. Bi lateral level IA, level IB and II lymph nodes were also noted, a provisional diagnosis of squamous cell carcinoma was made and he underwent biopsy. The report revealed it to be Embryonal Rhabdomyosarcoma. The specimen stained positive for desmin and myogenin, characteristic markers for rhabdomyosarcoma3. The patient was then informed about the diagnosis and the rarity of it and was referred for and is undergoing chemotherapy. He is currently under treatment and follow up on a regular basis. The current studies and the limited literature suggests treatment should be based on a multidisciplinary approach, such that surgery followed by chemo radiation should be opted, but in under developed countries it still remains a challenge to timely diagnose and implement treatment. Laryngeal Rhabdomyosarcoma is a rare disease and this case will help increasing the available literature on this topic.

Author(s): Farah Hafiz Yusuf

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