Fetal-Type Rhabdomyoma of the Cheek: A Conservative Management

Abstract

Extracardiac rhabdomyomas are rare benign mesenchymal tumors diagnosed upon radiological and hystologic investigations and the treatment of choice is surgical exertion. There aren’t any similar cases managed conservatively reported in literature as in our case, to the best of our knowledge. We present a rare case of fetal cheek rhabdomyoma diagnosed in a healthy 2 months-old boy, with asymptomatic mass over the left masseter. The lesion could not be removed, due to the size and dimensions and the young age of the patient. However, the lesion did not show signs of spreading or progression over a 36 months follow-up. Fetal rhabdomyoma is a benign tumor, often located in the head and neck district, where surgery, especially in very young children, is associated with a high risk of complications and long-term sequelae. Our case report demonstrates the possibility to manage these lesions conservatively in the first years of life with close sonographic and clinical follow-up.

Fetal rhabdomyoma in a Thoroughbred filly

“…In human medicine, two forms of fetal rhabdomyoma are described: myxoid (classic) and intermediate (juvenile) (Hansen & Katenkamp, 2005 ). While the myxoid type is typical of young children and is characterised by a uniform population of oval or spindle cells in an abundant myxoid stroma, the intermediate type affects the adults, shows less myxoid matrix and consists of more differentiated muscle fibres with cross striations (Hansen & Katenkamp, 2005 ; Troisi et al., 2023 ). Myxoid stroma was not a feature of the equine rhabdomyoma reported here, but no similar categorisations have been attempted yet for the equine fetal rhabdomyomas.…”

“…reported no recurrence or complications at one year following discharge from the hospital. In the human literature, surgical excision is considered the gold standard treatment for fetal rhabdomyoma, and the procedure is curative in most cases (Aaronson et al., 2015 ; Cai et al., 2019 ; Leboulanger et al., 2010 ; Troisi et al., 2023 ). Persistence, or recurrence of the mass following surgical removal is extremely rare however possible, particularly when the mass is incompletely excised (Valdez et al., 2006 ; Walsh & Hurt, 2008 ).…”

“…These tumours are derived from pluripotent mesenchymal cells and, in human medicine, are classified as cardiac or extracardiac, with the latter accounting for less than 2% of all neoplasms showing striated muscle differentiation (Hansen & Katenkamp, 2005 ; Keane et al., 2016 ; Troisi et al., 2023 ). While the cardiac form occurs in hearts of infants and young children, the extracardiac form is histologically classified into three subtypes, based on the morphology and differentiation stages of the neoplastic cells (Hansen & Katenkamp, 2005 ; Walsh & Hurt, 2008 ): the adult type, which commonly occurs in the upper aerodigestive tract of male adults; the genital type, which usually affects the vagina and vulva of middle‐aged women; and the fetal type (Keane et al., 2016 ; Troisi et al., 2023 ; Valdez et al., 2006 ). Fetal rhabdomyoma is the least common of all rhabdomyomas and it generally occurs in newborns and young children (Troisi et al., 2023 ; Valdez et al., 2006 ; Walsh & Hurt, 2008 ).…”

“…While the cardiac form occurs in hearts of infants and young children, the extracardiac form is histologically classified into three subtypes, based on the morphology and differentiation stages of the neoplastic cells (Hansen & Katenkamp, 2005 ; Walsh & Hurt, 2008 ): the adult type, which commonly occurs in the upper aerodigestive tract of male adults; the genital type, which usually affects the vagina and vulva of middle‐aged women; and the fetal type (Keane et al., 2016 ; Troisi et al., 2023 ; Valdez et al., 2006 ). Fetal rhabdomyoma is the least common of all rhabdomyomas and it generally occurs in newborns and young children (Troisi et al., 2023 ; Valdez et al., 2006 ; Walsh & Hurt, 2008 ). Approximately 25% of these lesions are congenital and therefore present at birth (Troisi et al., 2023 ; Valdez et al., 2006 ).…”

“…Fetal rhabdomyoma is the least common of all rhabdomyomas and it generally occurs in newborns and young children (Troisi et al., 2023 ; Valdez et al., 2006 ; Walsh & Hurt, 2008 ). Approximately 25% of these lesions are congenital and therefore present at birth (Troisi et al., 2023 ; Valdez et al., 2006 ).…”