-2 :: Case - 9844 :: - Malignant Rhabdomyosarcoma
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Tx and Followup:
After the discovery of many metastases within the abdomen on CT, the patient underwent surgery for biopsy and resection of the tumor. Tissue was removed from the abdominal mass as well as a vaginal nodule, a uterine nodule, a rectosigmoid nodule, a vena cava lymph node and the appendix. The abdominal mass tissue was partially encapsulated, lobulated and yellow-tan in appearance with a size of 20cm X 11cm x 11cm. The mass weighed 1355g. The tumor was compatible with rhabdomyosarcoma on frozen section. The patient had metastatic disease (group IV) and was subsequently treated with systemic chemotherapy and radiation. At 6 month follow up, the patient had no evidence of new metastasis and was tolerating chemotherapy.

Additional Discussion:
Rhabdomyosarcoma is a frequent cause of death due to malignant disease in children and is the most common soft tissue sarcoma in pediatrics. Roughly 10% of all solid tumors in childhood are Rhabdomyosarcomas. These tumors are extremely malignant and tend to invade locally and metastases by hematogenous and lymphatic routes. Without treatment, 90% of patients die within one year of diagnosis. Rhabdomyosarcomas are equal between genders and are bimodal in age distribution with peaks at 4-6 years and 16-18 years of age. These tumors are thought to arise from totipotent mesenchymal cells and the histologic diagnosis can be difficult. Histologic types that can be seen in a rhadomyosarcoma include alveolar, embryonal, embryonal-botryoid, pleomorphic, and undifferentiated. Among these types, the alveolar variety has the worst prognosis. The botyroides type has the classic grape-like appearance. Rhabdomyosarcomas most commonly originate from the pelvis and GU tract with nearly 40% in these areas.

Discussion Author: Matthew Kinzie

Ovarian Tumors occur in roughly 2% per 1,000,000 of the US population. Most tumors are functional but some are nonfunctioning in children and present as a pelvic or abdominal mass. Ovarian tumors are classified according to the origin of their cellular components including germ cell, surface epithelial cell, and sex cord stromal tumors. Germ cell types are the most common in children and among these, the most is the teratoma. These tumors have a potential for malignancy and this is found more commonly in solid teratomas, though the solid type is less common than the cystic variety. Teratomas commonly present in adolescent females and are usually not found until they have grown to a palpable size or are large enough to twist and produce abdominal pain. The tumors may be picked up on plain film due to the presence of calcification in two thirds of teratomas.

Dysgerminoma is the second most common ovarian tumor in pediatrics after the teratoma and is the most common malignant ovarian tumor in children and adolescents. The tumor is typically low-grade and on imaging is solid, smooth and well encapsulated. 20% of dysgerminomas are bilateral and the tumors are usually large at diagnosis. Dysgerminomas are usually non-functional in nature. These tumors can spread locally and are known to be very radiosensitive. They have a good prognosis with greater than 90% survival.

Simple ovarian cysts are the most common ovarian mass in adolescents. These cysts can be functional when a mature follicle fails to involute. In an asymptomatic patient, if the cyst is <5cm in diameter it is treated conservatively. Complications of cysts include a predisposition to torsion and hemorrhaging into the cyst. Functional ovarian cysts can be subject to hormonal control and fluctuate in size according to hormone levels.

Polycystic Ovarian Syndrome is a disorder resulting from an abnormal feedback loop between the Hypothalamic-Pituitary Axis and the ovary. The disease may cause primary amenorrhea and hirsutism. PCOS is diagnosed by detecting increased LH to FSH levels. Transvaginal ultrasound is used to demonstrate the enlarged ovaries.

Rhabdomyosarcoma is a frequent cause of death due to malignant disease in children and is the most common soft tissue sarcoma in pediatrics. Roughly 10% of all solid tumors in childhood are Rhabdomyosarcomas. These tumors are extremely malignant and tend to invade locally and metastases by hematogenous and lymphatic routes. Without treatment, 90% of patients die within one year of diagnosis. Rhabdomyosarcomas are equal between genders and are bimodal in age distribution with peaks at 4-6 years and 16-18 years of age. These tumors are thought to arise from totipotent mesenchymal cells and the histologic diagnosis can be difficult. Histologic types that can be seen in a rhadomyosarcoma include alveolar, embryonal, embryonal-botryoid, pleomorphic, and undifferentiated. Among these types, the alveolar variety has the worst prognosis. The botyroides type has the classic grape-like appearance. Rhabdomyosarcomas most commonly originate from the pelvis and GU tract with nearly 40% in these areas.

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