Embryonal Rhabdomyosarcoma (RMS) of Retroperitoneum in Young Child

Abstract

A 5-year-old female child was brought by parents to General Surgery department of Krishna hospital with complaints of fullness in left lower abdomen and swelling over left thigh since last 15 days. Since 15 days patient had fullness in left iliac fossa which rapidly increased in size and was associated with pain. Gradually patient had developed swelling over left thigh [Table/Fig-1]. On general examination patient was well built and nourished and apatite was not reduced only pallor was present. On examination of abdomen there was a palpable mass of 15 X 5 cm in left iliac fossa which was fixed to underlying tissues, hard in consistency. The swelling on the left thigh also was fixed to underlying tissues and hard in consistency. All peripheral pulses were palpable. Based on above findings our clinical diagnosis was mass arising from abdomen and extending into the left thigh. Ultrasonography of abdomen and pelvis suggestive of a large illdefined, isoechoic, solid, retroperitoneal mass [Table/Fig-2] of indeterminate origin in pelvis, posterior to bladder measuring about 112 x 60 x 88 mm in size with fixity to adjacent pelvic vasculature. Few retroperitoneal nodes were enlarged. Computed tomography scan of abdomen suggestive of large, ill defined, lobulated pelvic mass extending to left thigh [Table/Fig-3] causing mass effect with lymphadenopathy. USG guided tru-cut biopsy from abdominal and left thigh mass done which showed malignant small blue round cell tumour [Table/Fig-4]. Immunohistochemistry performed which showed that tumour cells exhibit immunopositivity for myogenin/desmin [Table/Fig-5,​,6]6] and are immunonegative for TdT/CD99 [Table/Fig-7,​,8]8] which confirmed the diagnosis of Embryonal Rahabdomyosarcoma. As mass was fixed to pelvic vasculatures, it was impossible to operate so patient was refered for chemotherapy and Radiotherapy. Chemotherapy regime VAC (Vincristine, Actinomycine-D and Cyclophosphamide) was started along with Radiotherapy. Last follow up was done after one month and patient was doing well. [Table/Fig-1]: Clinical photograph of mass in lower abdomen extending into left thigh [Table/Fig-2]: Showing Ultrasonographic image of mass in lower abdomen and left thigh [Table/Fig-3]: Showing Coronal section of computerized tomography scan showing mass in pelvis extending into left thigh [Table/Fig-4]: Showing microphotograph of biopsy from mass showing multiple blue round tumour cells [Table/Fig-5 & 6]: Microphotograph of IHC with myogenin and desmin stain shows immunopositivity to tumour cells [Table/Fig-7 & 8]: Showing ultrasonographic image of mass in lower abdomen and left thigh