Cutaneous Embryonal Rhabdomyosarcoma Presenting as a Nodule on Cheek; A Case Report and Review of Literature

Abstract

We report a case of primary cutaneous rhabdomyosarcoma, solid embryonal type, presenting as a rapidly enlarging nodule on the right cheek of a 7-year-old boy. This lesion had begun as a pea-sized nodule 8 months previously, and, with suspected abscess, had been incised. It recurred 2 months later; at that time, incisional biopsy was consistent with malignant round cell tumor. Wide local excision of the tumor was then completed. Subsequent immunohistochemical staining with desmin and myoglobin confirmed embryonal rhabdomyosarcoma. The patient underwent radiation therapy followed by chemotherapy and continues to be disease free at 14 months after his wide local excision. Rhabdomyosarcoma presenting as a dermal nodule is rare. It usually presents as an asymptomatic papule without distinctive clinical features and therefore may result in delayed diagnosis unless a biopsy is performed.

Progressive Left Periorbital Swelling

“…1,2 Rhabdomyosarcomas originate from the embryonic mesenchymal precursor of striated muscle, and the diagnosis is largely based on immunohistochemical staining. 3 Histologically, rhabdomyosarcomas are divided into 5 groups: embryonic (58%), alveolar (31%), botryoid (6%), pleomorphic (4%), and undifferentiated (1%). 4 In our patient, computed tomographic images showed a 2.7 × 2.1 × 2.2-cm round, soft-tissue mass with bony erosion of the left zygoma and orbit.…”

Rabdomiossarcoma alveolar cutâneo primário em paciente pediátrico

“…Factors indicative of good prognosis include early age at diagnosis, primary site in the genitourinary tract or orbital cavity, and embryonic or botryoid histological types. 1,5 Despite the young age at which rhabdomyosarcomas affect patients, lesions that are completely resected are associated with a 5-year survival rate of 90%, which highlights the need for prompt diagnosis and treatment.…”

“…Combined therapy has increased survival by five years in recent decades from 25% in 1975 to 70% in 1991. 4,5,7 Prognosis varies in accordance with the site of origin, the tumor size, clinical staging, the patient's age and the histological type. Factors indicative of good prognosis include early age at diagnosis, primary site in the genitourinary tract or orbital cavity, and embryonic or botryoid histological types.…”

“…[1][2][3]5 Differential diagnosis of the skin lesions on the head and neck include: hemangioma, lymphoma, lymphangioma, cutaneous leukemia, angiofibroma, neuroblastoma, hematoma, cutaneous myofibromatosis, glioma, cellulitis, abscess, mastoiditis and other sarcomas. 2,5,6 Histopathology is characterized by a dense dermal infiltrate composed of small, round, blue cells of clear cytoplasm with mitotic figures. These characteristics may be present in various pathologies such as Ewing's sarcoma, lymphoma and neuroblastoma; therefore, immunohistochemistry is mandatory in order to define diagnosis.…”

Congenital Primary Cutaneous Rhabdomyosarcoma of the Perineum with Mixed Histopathological Features and Unusual Expression of Smooth Muscle Actin: Report of a Case

“…While conventional RMS has a slight male predominance, PC-RMS tends to be more common in females [11]. Among pediatrics, the face tends to be the most frequently affected site with PC-RMS [1,2,9,[12][13][14][15]. Other rarely involved sites include the hand [16], chest [17], feet [12], anus [18,19], and the perineum [20].…”