A 35-year-old African-American man was referred to our urology clinic by his primary care physician for consultation about a large left scrotal mass. The patient reported a 3-month history of left scrotal swelling that had progressively increased in size and was associated with mild left scrotal pain. He also had complaints of mild constipation, with hard stools every other day. He denied any urinary complaints. On physical examination, a hard paratesticular mass could be palpated in the left hemiscrotum extending into the left groin, separate from the left testicle, and measuring approximately 10 × 7 cm in size. A hard, lower abdominal mass in the suprapubic region could also be palpated in the midline. The patient was admitted urgently to the hospital for further evaluation with cross-sectional imaging and blood work.
Laboratory results, including results of a complete blood cell count with differential, liver function tests, coagulation panel, and basic chemistry panel, were unremarkable except for a serum creatinine level of 2.6 mg/dL. Typical markers for a testicular germ cell tumor were within normal limits: the beta–human chorionic gonadotropin level was less than 1 mIU/mL and the alpha fetoprotein level was less than 2.8 ng/mL. A CT scan of the chest, abdomen, and pelvis with intravenous contrast was obtained, and it showed large multifocal intra-abdominal, retroperitoneal, and pelvic masses (Figure 1). On cross-sectional imaging, a 7.8-cm para-aortic mass was visualized compressing the proximal portion of the left ureter, creating moderate left hydroureteronephrosis. Additionally, three separate pelvic masses were present in the retrovesical space, each measuring approximately 5 to 10 cm at their largest diameter; these displaced the bladder anteriorly and the rectum posteriorly.
The patient underwent ultrasound-guided needle biopsy of one of the pelvic masses on hospital day 3 for definitive diagnosis. Microscopic examination of the tissue by our pathologist revealed cellular islands with oval to elongated, irregular, and hyperchromatic nuclei; scant cytoplasm; and invading fibrous tissue—as well as three mitoses per high-powered field (Figure 2). Immunohistochemical staining demonstrated strong positivity for cytokeratin AE1/AE3, vimentin, and desmin. Further mutational analysis of the cells detected the presence of an EWS-WT1 fusion transcript consistent with a diagnosis of desmoplastic small round cell tumor.
Which of the following treatment strategies has demonstrated the best chance for both optimal progression-free survival and optimal overall survival?
A. Surgical resection alone
B. Surgical resection with chemotherapy and radiation
C. Chemotherapy alone
D. Chemotherapy and radiation alone
E. Radiation alone
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