The chemotherapy effect on embryonal rhabdomyosarcoma in a 2-year-old girl in a development country: A rare case (original) (raw)

2021, Indian Journal of Case Reports

R habdomyosarcoma (RMS) is a malignant tumor originating from mesenchymal tissue which is suspected to arise from striated muscle cells. RMS is the most common soft-tissue sarcoma in childhood and accounts for 3.5% of childhood cancers. More than 50% of tumors occur in the first decade of life [1]. It is a highly malignant tumor with local tissue invasion and lymphatic and hematogenous metastases. Based on the 2013 World Health Organization classification, RMS is divided histologically into four types, namely, embryonal, alveolar, spindle, and pleomorphic. Embryonal RMS and alveolar RMS are the most common types which account for 70-80% of all cases [2,3]. More than half of the RMS cases are embryonal RMS characterized by the presence of primitive spindle cells, often against a myxoid background [4,5]. The main treatment for RMS is complete resection with a combination of chemotherapy and radiotherapy. However, in some cases, especially in difficultto-access areas, complete resection is difficult, thus affecting the prognosis [6]. CASE REPORT A 2-year-old girl came with her parents to the Surgical Oncology Polyclinic General Hospital with a complaint of noisy breathing (Stridor). According to her parents, she also began to have difficulty swallowing coarse-textured food and could only swallow softtextured drinks and foods such as porridge. Within 2 weeks, a lump quickly grew on her upper neck. No signs of distant metastases were found. The history of radiation exposure was denied. Intraoral examination showed an oval mass sized 3 × 3 × 4 cm at the base of the tongue that almost closed the airway, felt soft, and had a bump. A computed tomography (CT) scan of the neck was performed (Fig. 1) and found a solid mass of 3.5 × 3.9 × 5.5 cm with a firm border and an irregular border on the posterior lingua. The patient underwent an incisional biopsy. Histopathological results obtained a final diagnosis of embryonal RMS (Fig. 2). The histopathological results were confirmed by immunohistochemical results with positive Myogen obtained according to embryonal RMS. Because the tumor mass was clinically unresectable, 2 weeks after the biopsy, the patient decided to perform neoadjuvant chemotherapy. The neoadjuvant chemotherapy given were vincristine, epirubicin, and etoposide. Evaluation after 2 times chemotherapy showed that the mass size was clinically reduced to 2 × 2 × 3 cm. DISCUSSION The most common areas of RMS are in genitourinary (29%), parameningeal (24%), extremities (15%), retroperitoneal (13%),