Multifocal Adult Rhabdomyoma of the Head and Neck Manifestation in 7 Locations and Review of the Literature

Abstract

Background. Adult rhabdomyoma is a rare benign tumour with the differentiation of striated muscle tissue, which mainly occurs in the head and neck region. Twenty-six cases of multifocal adult rhabdomyoma are documented in the literature. Method. We report a 55-year-old male with simultaneous diagnosis of 7 adult rhabdomyomas and review the literature of multifocal adult rhabdomyoma. Result. Review of the literature revealed 26 cases of multifocal adult rhabdomyoma, of which only 7 presented with more than 2 lesions. Mean age at diagnosis was 65 years with a male to female ratio of 5.5 : 1. Common localizations were the parapharyngeal space (36%), larynx (15%), submandibular (14%), paratracheal region (12%), tongue (11%), and floor of mouth (9%). Besides the known radiological features of adult rhabdomyoma, our case showed FDG-uptake in (18) F-FDG PET/CT. Conclusion. This is the first case of multifocal adult rhabdomyoma published, with as many as 7 simultaneous adult rhabdomyomas of the head and neck.

Clinical and mutational spectrum of highly differentiated, paired box 3:forkhead box protein o1 fusion–negative rhabdomyosarcoma: A report from the Children's Oncology Group

“…In our series described here, two thirds of PF- D3 RMS tumors arose at head/neck or genitourinary sites, and overall survival was substantially better than expected in children with RMS 1 . Interestingly, head/neck or genitourinary locations also are known to be common sites of origin of rhabdomyomas, which never metastasize and have excellent outcomes, but exhibit locally aggressive growth and occasional multifocality 24–26 . As previously suggested by Kodet 12, 15 and ourselves 9, 11 , highly differentiated RMS tumors and rhabdomyomas might form a continuous spectrum of tumors.…”

Pyriform Sinus Soft-Tissue Mass Recurring in Esophagus After Excision

“…Occasionally, the second site becomes evident only years after the initial diagnosis. 7 After excision, the local recurrence rate is estimated to be 42% and is presumed to be due to incomplete excision. 4 To our knowledge, only 4 cases of esophageal ARM can be found in the literature.…”

“…5 Macroscopically, these tumors are soft, well-circumscribed, lobulated, and tan-gray lesions. 4,5,7 Histologically, they are composed of well-demarcated, unencapsulated lobules with closely packed, large polygonal cells and scant stroma. The tumor cells have a small, round, centrally or peripherally located nucleus with prominent nucleoli and abundant, eosinophilic, granular, or vacuolated cytoplasm.…”

Case report Extracardiac rhabdomyomas – presentation of two cases with analysis of AKT/mTOR and ERK1/2 signaling

“…However, RM can recur even many years after the primary excision, but without malignant transformation and metastases. The pathogenesis of extracardiac RM is unknown, while in few cases, SHH pathway activation and association with Gorlin's syndrome has been proved [5,6].…”

“…Usually the lesion is solitary, less frequently polinodular or multifocal. Histological differential diagnosis includes granular cell tumor, hibernoma, paraganglioma, alveolar soft part sarcoma and reticulohistiocytoma [6,9]. The fetal type RM divided into the classic, juvenile and fetal subtype, occurs mainly in the children, involving head and neck, with a preference for the postauricular region.…”