Management of penoscrotal extramammary Paget disease: case series and review of the literature.

Extramammary Paget disease (empd) is a rare, slow-growing neoplasm, considered to be an adenocarcinoma of the apocrine glands. In men, the penoscrotal region is the most commonly affected area. The disease can present as carcinoma in situ or as invasive disease that can subsequently metastasize to lymph nodes and distant sites. Because of the rarity of empd, the medical literature available to guide management of the disease is limited, particularly in patients with metastases. In addition, metastatic disease may pose a diagnostic challenge, because invasive cancer of the genitourinary or gastrointestinal tract can occur in association with empd. In the present case series, we describe our experience in treating penoscrotal empd with multimodality therapy, and we review the existing literature concerning its diagnosis and management.

Primary retroperitoneal teratomas in children: a single institution experience.

OBJECTIVE: This study aims to highlight the clinical features, investigations and treatment outcome of retroperitoneal teratomas (RPT) in children. MATERIALS AND METHODS: A total of eight patients (six males and two females, age range between 6 months-10 years) of RPT admitted in the department of Paediatric Surgery, PGIMS, Rohtak, between 1996-2008, were studied. The patients were investigated with hematology, x-ray, ultrasound, and computerised tomography (CT) of abdomen and serum alpha-fetoprotein levels in pre and postoperative period. All patients underwent complete surgical resection. In one patient, the tumour had malignant component (yolk sac) and was given postoperative chemotherapy. Postoperative follow-up included serum alpha-fetoprotein in addition to clinical examination and radiological assessment to detect recurrences. RESULTS: The tumours were located on both sides in almost equal proportion (four on right, three on left, and one bilateral]. All tumours could be excised completely preserving the kidneys in all patients. But in one patient injury to inferior vena cava (IVC) occurred which was repaired successfully. Majority (7 out of 8) were histological benign, and in one yolk sac tumour was malignant component which needed chemotherapy. All children were on follow-up and one patient with malignancy lost to follow-up after three cycles of chemotherapy. In rest there was no tumour recurrence. CONCLUSION: RPT are rare paediatric neoplasms. As majority are benign, a complete excision preserving the kidneys, is usually curative. Serum alpha-fetoprotein is a reliable method of assessing recurrence. Malignancy in the tumour may warrant further chemotherapy.