Rare Case Botryoid Rhabdomyosarcomas of the Genital Tract. About a case in a 30-month-old child

Rhabdomyosarcomas are the most common soft tissue sarcomas developed in children under 15 years of age. These tumours respond to multidisciplinary management: conservative surgery (Figure1), multi-chemotherapy and radiotherapy. Although the prognosis is always dire, a signifi cant proportion of children treated in this way do not metastasize [1]. We present a case of Sarcoma botryoides to illustrate the diffi culties encountered by pediatric surgeons in the management of this malignant and aggressive tumor.


Introduction
Rhabdomyosarcomas are the most common soft tissue sarcomas developed in children under 15 years of age. These tumours respond to multidisciplinary management: conservative surgery (Figure1), multi-chemotherapy and radiotherapy. Although the prognosis is always dire, a signifi cant proportion of children treated in this way do not metastasize [1]. We present a case of Sarcoma botryoides to illustrate the diffi culties encountered by pediatric surgeons in the management of this malignant and aggressive tumor.

Case repport
We report the observation of an 30-month-old girl with multiple whitish-looking polyploid formations, delivered at the vulva. Radiological studies, pelvic computerized tomographic scan (CT) with contrast revealed a solid, cystic mass 5cm3, located anterior to the rectum, and at the posterior-superior aspect of the bladder (Figure 2). There was no extension of the tumor beyond the vagina. Abdominal palpation did not reveal any abnormality, as did the preoperative laboratory workup. Removal of 12 polyps was performed. The polyps were pedicled, implanted on all vaginal walls, about three centimeters from the vulval orifi ce. Removal was performed vaginoscopically.
Anatomopathological examination of the specimen showed that it was a polypoid (grape-like) tissue bordered by a slightly hyperplastic squamous epithelium. Beneath this epithelium, within a myxoid layer, a proliferation of disseminated tumour cells was observed with an inconspicuous cytoplasm, a hyperchromatic, rounded or elongated nucleus, sometimes presenting cytonuclear atypia with monstrosity

Abstract
Rhabdomyosarcomas are the most common soft tissue sarcomas developed in children under 15 years of age. The reported fi nding is a vaginal tumour developed in a 30-month-old granddaughter. This was a typical botryoid rhabdomyosarcoma that usually occurs in the hollow organs lined with mucous membrane. Rhabdomyosarcomas have multiple aspects that vary according to the degree of cellular differentiation. The majority of these tumours can be classifi ed into four histological categories: embryonic, botryoid, alveolar or pleomorphic. Treatment involves excisional surgery combined with radiotherapy and chemotherapy. The prognosis remains bleak despite therapeutic advances in recent years.

Discussion
The clinical signs of botryoid rhabdomyosarcomas in boys are urinary signs due to bladder or prostate involvement. In the girl, the same signs can be found but, most often, it is the appearance of a "polyp" or cystic formation at the vulva, as was the case in our observation. In other cases, it is vaginal haemorrhages [2]. Scintigraphy studies the renal impact. The cystography shows an upward and forward displacement of the bladder in the extravesical forms and, in the bladder forms, polycyclic gaps. A cystoscopy assesses the lesions and allows biopsies to be taken. An extension assessment is performed locally and at a distance to detect possible metastases [3].
Histologically, rhabdomyosarcomas have multiple aspects which vary according to the degree of cellular differentiation [4]. The majority of these tumours can be classifi ed in four histological categories: embryonic, botryoid, alveolar or pleomorphic: Embryonal rhabdomyosarcoma is the most common. It accounts for 50-60% of all rhabdomyosarcomas. It mainly affects children under 15 years of age. It is located mainly on the head and neck. It is also found in the genitourinary tract and retroperitoneum. Microscopically, it is a microscopically undifferentiated cell. Immunohistochemical examinations are most often necessary to formally identify the lesion.