Abstract
Introduction. Rhabdomyomas of the head and neck are exceptionally rare benign mesenchymal tumors. Rare cases have been reported to involve other sites of the body including the head and neck regions. Case Presentation. We report a case of voluminous extracardiac adult rhabdomyoma affecting adult patients and initially seen as slowly growing, indolent neoplasms. The patient is a seventy-year old male Italian patient. Conclusion. Adult extracardiac rhabdomyoma is a rare benign tumor that may present with symptoms that vary from aerodigestive tract obstruction to remaining asymptomatic for many years. Although histology is very characteristic, several differential diagnoses have to be considered. To our knowledge, this is the first case of voluminous adult-type symptomless rhabdomyoma.
Highlights
Rhabdomyomas of the head and neck are exceptionally rare benign mesenchymal tumors
We report a case of voluminous extracardiac adult rhabdomyoma affecting adult patients and initially seen as slowly growing, indolent neoplasms
Adult extracardiac rhabdomyoma is a rare benign tumor that may present with symptoms that vary from aerodigestive tract obstruction to remaining asymptomatic for many years
Summary
Rhabdomyomas are benign mesenchymal tumours with skeletal muscle differentiation They can be divided into extracardiac-and intracardiac-type depending on the localization. The extracardiac type is extremely rare, with a propensity for occurrence in the head and the neck and for multiple localization (3–10%, synchronous or asynchronous); recurrences may be observed after complete excision (10 to 40%) They are virtually never associated with other malformations; on the other hand intracardiac rhabdomyomas are often associated with tuberous sclerosis (up to 86% of patients with TS in series with echocardiographical evaluation) or other genetical disorders. The AR presents polygonal cells, eosinophilic granular cytoplasm, cross-striations, and spider cells; the expression of desmin, muscle-specific actin, myoglobin, and myogenin can be immunohistochemically demonstrated [1]. Antiarrhythmic drugs can be helpful if arrhythmias are the presenting symptom [3]
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