Clinical and pathologic considerations in a case of inflammatory myofibroblastic tumor of the spleen.

Inflammatory myofibroblastic tumors, also called inflammatory pseudotumors, are rare pseudosarcomatous proliferations. Their behavior is generally not aggressive, but in most instances they can simulate malignant neoplasms, from which they are impossible to distinguish before excision. One case of myofibroblastic tumor of the spleen in a 5-year-old boy is described: the patient was treated with a partial splenectomy, which was found to be the best approach for diagnosis and treatment. The purpose of this report is to call attention to this entity with peculiar histologic and immunohistochemical characteristics, which has to be included in the differential diagnosis of splenic lesions and may be excised by a nonaggressive procedure.

Primary transcrotal excision for paratesticular rhabdomyosarcoma: is hemiscrotectomy really mandatory?

BACKGROUND: To evaluate the role of primary reexcision (PRE) with scrotal resection in patients with paratesticular rhabdomyosarcoma enrolled in the German-Italian Cooperative Studies. The authors compared patients who underwent this procedure, according to the protocol guidelines, with those who did not. METHODS: In 32 of 198 patients with localized disease, the primary surgery was performed through a noncorrect scrotal approach. Twenty-four patients underwent PRE as recommended by the protocol guidelines (Group A) and 8 did not receive this treatment (Group B). The Group B patients were treated with the same chemotherapeutic regimens as the Group A patients and no radiotherapy was given to either group. RESULTS: After PRE, residual tumor was not detected in 21 of the 24 Group A patients. Twenty patients are alive in first complete remission 26-250 months after diagnosis (median, 40 months), 2 are alive in second complete remission at 3 and 9 months from diagnosis of lymph node and lung recurrence, and 2 died of disease after lymph node and distant metastases at 16 and 13 months from diagnosis. Three-fourths of these patients were older than 10 years old and the tumor was larger than 5 cm. The eight Group B patients are all alive in first complete remission 24-250 months since diagnosis. CONCLUSIONS: The data on the eight patients who obtained local control without PRE or radiotherapy warrant further investigation. Because of the supposed high risk of contamination with subsequent microscopic residual tumor after a transcrotal approach, we emphasize the utility of PRE with hemiscrotectomy.

Teratoid Wilms' tumor: report of a unilateral case.

Teratoid Wilms' tumor is a rare histologic variant of the classical Wilms' tumor, containing predominantly heterologous tissues (adipose, glial, muscle, cartilage, or bone). This report presents the case of a teratoid Wilms' tumor in a 4-year-old girl. The mass, which originated from the right kidney, was very large and encased the inferior vena cava and renal vessels. The child did not respond to chemo- or radiotherapy and underwent operation. Despite the enormous dimensions of the tumor and the involvement of the inferior vena cava, a radical excision was obtained, and now the child is well 32 months after surgery. The behavior of this kind of tumor usually is not aggressive, and the outcome is good. Surgery should be the treatment of choice, because the efficacy of chemotherapy and radiotherapy is probably reduced by the high amount of differentiated and mature tissue which characterizes this neoplasm.