Author(s)
Hiromu Nakamura, MD
Yasuhiro Tada, MD, PhD
Shigeyuki Murono, MD, PhD
Affiliation(s)
Fukushima Medical University;
Abstract:
Angiomyolipoma (AML) is a rare benign mesenchymal tumor composed of variable amounts of smooth muscle, adipose tissue and thick-walled blood vessels, and usually named PEComas (perivascular epithelioid cell tumors). The tumor often develops with tuberous sclerosis patients and located in the kidneys. In the head and neck location, AML presentation is extremely rare. Based on our knowledge, only 3 cases have been described in the larynx. We have reported a case of AML in the larynx and discussed the difference between laryngeal AML and renal AML. A 69-year-old man who was pointed to a laryngeal tumor with an upper gastrointestinal endoscopy was admitted to our department for detailed examination. The patient did not appear any history of laryngeal symptoms. Flexible fiber opticendoscopic examination revealed a mass covered by a smooth mucosa and attached loosely on the right plica aryepiglottica. The mass was totally excised by endolaryngeal microsurgery. Histopathologically, the tumor was composed of smooth muscle, mature fat, and thick-walled blood vessels. Immunohistochemically, tumor cells were positive for SMA and Desmin, but Melan-A and HMB-45, characteristic marker expressed in PEComa, are negative. The diagnosis of Angiomyolipoma was made. The patient was followed for 9 months and the patient had no sign of local recurrence. In conclusion, we present a laryngeal AML, which indicated the difference immunohistochemical features from renal AML. The clinical and morphologicala features of the tumor were similar to the group of mucocutaneous AMLs.