Malignant tumors of the parotid gland are rare in children, with sarcomas representing less than 1.5% of these malignancies. Among sarcomas, rhabdomyosarcoma (RMS) is one of the more common types found in the parotid region during childhood and adolescence, although it is still infrequent overall.[1-3]

The parotid gland is the most common site for this tumor, followed by the submandibular gland, and rarely, accessory salivary glands. Rhabdomyosarcoma is a high-grade malignancy, known for its aggressive nature and its ability to metastasize, leading to a poor prognosis. It typically grows locally and can spread to surrounding tissues and regional lymph nodes. Due to its potential for distant metastasis, early detection and treatment are crucial. 

Despite treatment options like surgery, radiation, and chemotherapy, Rhabdomyosarcoma remains a challenging and often unfavorable diagnosis due to its aggressive behavior and tendency to metastasize. 

A 15 year old female child presented in ENT OPD with a painless swelling in front of right ear for past 3 months which was insidious in onset, gradually progressive and persistent.

ON GENERAL PHYSICAL EXAMINATION : Child was afebrile, vitals were stable. 

ON LOCAL EXAMINATION :There was multilobulated swelling in right preauricular region of size approximately 5x3 cm, extending to right temporal region. It was non tender ,non mobile , firm to hard on palpation. Color of the overlying skin was normal.Paresis of zygomatic and temporal branch of facial nerve was also present.Grade 1 trismus was present. Rest of the head and neck examination was unremarkable. There was no cervical lymphadenopathy .There were multiple firm to hard nodules on chest and back. Ocular examination was normal.

Diagnostic work up

Routine blood investigastions were found to be within normal limits with raised serum LDH. Ultrasonography of right parotid region showed necrotic conglomerated right intraparotid lymph nodes with provisional diagnosios of Pleomorphic adenoma 

A Contrast enhanced computed tomography (CECT) base of skull to T4 showed  Multiple peripherally enhancing conglomerated lesions in the subcutaneous plane on the right side temporal , intratemporal , masticator, parotid , retromaxillary space with round to oval eccentric lesion abutting the intraorbital part of left optic nerve.Lytic lesions in the L3 and sclerotic foci in the C5 vertebral body. 

Magnetic resonace imaging (MRI) Brain showed a heterogenously enhancing altered signal intensity lesion in right parotid and masseter space showing intense diffusion restriction with contrast enhancement and an altered signal intensity  lesion was seen  along inferior aspect of left optic nerve. There were Multiple lesions in subcutaneous plane in supra and infraclavicular region suggestive of neurofibromatosis 1. 

PET CT was also done which showed hypermetabolic mass lesion involving right parotid and masticator space with cervical to inguinal lymphadenopathy and lesions involving subcutaneous soft tissue ,muscles ,breast and abdomen suggestive of lymphomatous involvement. 

FNAC was taken from parotid swelling,chest swelling which was suggestive of non hodgkin’s lymphoma. Incisional biopsy of the swelling was done which was suggestive of round blue cell tumor On immunohistochemistry Embryonalrhabdomyosarcoma with ALK-1 expression was diagnosed.Patient was attached to department of hematooncology where vincristin,actinomycin,cyclophosphamide (VAC) regimen was started.

Patient improved clinically with notable reduction of swelling. On 9 months follow up patient presented with recurrence along with orbital involvement. Patient underwent orbital exenteration for orbital involvement.

Salivary gland carcinomas are rare in childhood. Parotid rhabdomyosarcoma usually presents as painful swelling of the parotid gland that rapidly increases in size. Facial paralysis is a frequent sign, testifying to the aggressiveness and degree of tumor extension. CT scans and magnetic resonance imaging (MRI) of the parotid are an essential contribution in terms of exploration of the parotid gland and evaluation of tumor extension to neighboring structures.Because of the aggressive nature of this tumor, treatment combines surgery, radiation therapy, and chemotherapy. Salivary rhabdomyosarcomas have a tendency of local recurrence, the time to onset is on average nine months [4]

Rhabdomyosarcoma  is one of the more common sarcomas of the parotid region occurring during childhood and adolescence, but overall, its incidence remains rare and often have an unfavorable prognosis. Improving results requires an early diagnosis and multidisciplinary management based on the results of multicenter trials. The inclusion of these patients in trials must be systematic due to the rarity of cases.

Conflict of Interest: The authors declare that they have no conflict of interest

Funding: No funding sources

Ethical approval: The study was approved by theAIIMS, Rishikesh, Uttrakhand.

1. Luna, M. A., Tortoledo, E., Ordonez, N. G., et al. "Primary Sarcomas of the Major Salivary Glands." Archives of Otolaryngology - Head & Neck Surgery 117 (1991): 302–306.

2. Rogers, D. A., Rao, B. N., Bowman, L., et al. "Primary Malignancy of the Salivary Gland in Children." Journal of Pediatric Surgery 29 (1994): 44–47.

3. Enzinger, F., and S. Weiss. Soft Tissue Tumors. 3rd ed. St. Louis: Mosby, 1995. Chapter 22.

4. Flamant, F., Rodary, C., Rey, A., et al. "Treatment of NonmetastaticRhabdomyosarcoma in Childhood and Adolescents: Results of the Second Study of the International Society of Pediatric Oncology (MMT)." European Journal of Cancer 34 (1984): 1050–1062