Embryonal rhabdomyosarcoma of the auricle in a child

Abstract

We describe the diagnosis and management of a child with embryonal rhabdomyosarcoma of the auricle and emphasize both clinical and radiological findings of this rare condition. A nine-year-old boy presented for evaluation of a slowly enlarging left auricle mass. The mass was nodular, violaceous, semi-translucent, and hyperpigmented with an overlying pseudo-vesicular plaque. The mass appeared to involve the left cavum concha, root of the helix, superior aspect of the external auditory canal, the tragus and extend to a deep preauricular component. MR imaging documented a lobulated soft tissue mass surrounding the external auditory canal with superficial involvement of the pinna. Incisional biopsy of the mass suggested embryonal rhabdomyosarcoma. The tumor was completely removed by total auriculectomy and lateral temporal bone resection. The final diagnosis was embryonal rhabdomyosarcoma. Although rare, otolaryngologists, pediatricians, and radiologists need to consider rhabdomyosarcoma in the differential diagnosis of auricle mass in children.

Management of neonatal retro-auricular embryonal rhabdomyosarcoma – Case report

“…With an atypical aspect, clinical presentation and unusual location, benign tumors, such as glomus tumor, hemangioma, pilomatrixoma, or malignant tumors, such as liposarcoma, chondrosarcoma, neuroepithelioma, extra skeletal Ewing’s sarcoma, juvenile fibromatosis or malignant lymphoma can be suspected [ 6 , 7 ]. RMS is a congenital tumor.…”

“…MRI provides a better assessment of extension, lesion size or lymph node involvement. Imaging has a key role in the initial staging, the preoperative planning and allows early detection of recurrence during the follow up [ 6 ].…”

Sclerosing Spindle Cell Preauricular Rhabdomyosarcoma in a 7-Year-Old Female

“…The spindle type consists of cells that have spindle morphology and is most commonly located in the orbital and para-testicular areas. Lastly the rare pleomorphic subtype is most commonly seen in extremity skeletal muscle in patient over the age of 45 years [2,11]. Microscopically, they are 1) round cells with eccentric eosinophilic cytoplasm, 2) spindle cell myoblasts with tapered fibrillary eosinophilic cytoplasm, and 3) anaplastic [1].…”

“…RMS is occasionally seen in patients with other genetic diseases such as Li-Fraumeni syndrome, Neurofibromatosis, Beckwith-Wiedemann syndrome and Costello syndrome [9,10,11]. Symptoms and signs depend on tumor location [2]. There are primarily two histological types of RMS with genetic mutations that play a role in pathogenesis of this malignancy, the alveolar subtype and the embryonal subtype.…”

“…Rhabdomyosarcoma (RMS) is a primary malignancy in children and adolescents that arises from embryonic mesenchymal cells which develop into skeletal muscle [1]. RMS comprises over 50% of all soft tissue sarcomas in children, and is thus the most common soft tissue sarcoma [2]. RMS has different locations and types based on the age group.…”