Scrotal pathology in pediatrics with sonographic imaging.

Scrotal pathology in pediatrics ranges from the more benign hydrocele and varicocele to acute testicular torsion requiring emergent surgery. Malignant testicular tumors can be insidious in onset or may present acutely when trauma brings a swollen scrotum to the patient's or physician's attention. Three common conditions can present as an acute scrotum, all of which can suggest testicular torsion clinically. Epididymitis often has a less acute onset than testicular torsion, although it does not always present with a straightforward diagnosis. Although it is generally an inflammatory process affecting males from 9 to 14 years of age, it can be seen in younger males with Henoch-Schonlein purpura and Kawasaki disease. Torsion of the appendix of the testis and epididymis can present acutely and mimic acute testicular torsion and generally occurs from 6 to 12 years of age. Testicular torsion itself usually occurs from 12 to 18 years of age and usually results from the anatomical "bell-and-clapper" deformity. Infarction of the testis can occur within as early as 4 to 6 hours after torsion, depending on the duration of symptoms and degree of twist of the spermatic cord. Advances in ultrasound technology in recent years have made ultrasound the examination of choice for imaging scrotal pathology, whether acute or chronic in nature. Doppler technology has tremendously increased the radiologist's ability to assess flow within the prepubertal testicle, which allows assessment of viability in the undescended testis as well as in neonatal torsion. The ability of ultrasound to diagnose the pathogenesis of the acute scrotum is unsurpassed by any other imaging modality. Ultrasound is a readily available, noninvasive examination without radiation that provides excellent anatomic detail and serves as an important and tremendously helpful imaging modality in all types of pediatric scrotal pathology.

Clear cell sarcoma of the kidney: an unusual presentation and review of the literature.

PURPOSE: To describe a child with clear cell sarcoma of the kidney (CCSK) with an unusual presentation, including a primary tumor of the left kidney with metastases to the right kidney and soft tissues of the lower extremities, and to review the literature. PATIENT AND METHODS: An 8-month-old infant presented with hypertension, an abdominal mass, and soft tissue masses in the left thigh and right foot. Imaging studies revealed a large left-sided renal tumor, left paravertebral soft tissue masses, and left thigh mass. At laparotomy, a lesion was noted in the lower pole of the contralateral kidney. CCSK with metastases to the contralateral kidney and to the soft tissues of left thigh, right foot, and left paravertebral region was diagnosed on histopathologic examination. RESULTS: Multimodal oncologic treatment included surgery, chemotherapy, and radiotherapy. Three months after completion of therapy, a soft tissue lesion in the left arm and, later, soft tissue lesions involving multiple parts of the body developed. The patient died 18 months after diagnosis without clinical or radiographic evidence of bone involvement. CONCLUSIONS: In a review of the literature, CCSK is most commonly associated with bone and lung metastases. Soft tissue involvement is uncommon. Metastasis to the contralateral kidney at initial diagnosis has not previously been reported. This case represents an unusual metastatic pattern of CCSK.

Hepatic angiosarcoma in a child: successful therapy with surgery and adjuvant chemotherapy.

We report a 3-year and 11-month-old Caucasian female, who initially presented with an unresectable hepatic angiosarcoma. After three courses of chemotherapy with adriamycin/cisplatin, the tumor decreased in size considerably, allowing complete surgical resection. She also received postoperative chemotherapy with alternating cycles of ifosfamide/etoposide, cisplatinum/adriamycin, and vincristine/actinomycin D/cyclophosphamide for 18 months. She remains disease-free for greater than 44 months from the initial diagnosis. Our experience suggests that total excision of the tumor, together with an aggressive chemotherapy regimen, can improve the disease-free survival for children with this highly malignant vascular tumor of the liver.

Intraabdominal desmoplastic small round cell tumor presenting as a gastric mural mass with hepatic metastases.

PURPOSE: An atypical case of childhood intraabdominal desmoplastic small round cell tumor (DSRCT) is presented. PATIENT AND METHODS: An 11-year-old boy presented with progressive nausea and vomiting, abdominal pain, hepatomegaly, and an epigastric mass. Computed tomographic scanning as well as findings at gastroscopy and laparotomy revealed a large gastric mural tumor accompanied by multiple large intrahepatic masses. Histopathologic examination of biopsy samples revealed evidence of a polyphenotypic neoplasm diagnostic of DSRCT. RESULTS: Unlike most reported cases of DSRCT, no evidence of peritoneal involvement or ascites was detected. Despite an excellent initial response to multiagent chemotherapy, the patient eventually died of progressive tumor. CONCLUSIONS: Though the radiographic and surgical findings in DSRCT usually suggest a mesenteric, peritoneal, or retroperitoneal site of tumor origin, this case demonstrates that intraabdominal DSRCT can present with a primary visceral lesion without evidence of peritoneal or mesenteric involvement or ascites. Combination chemotherapy using vincristine, doxorubicin, cyclophosphamide, cisplatin, and 5-fluorouracil may be of some benefit to patients with this rare tumor.