Extramammary Paget Disease: a Therapeutic Challenge, for a Rare Entity.

PURPOSE OF REVIEW: Extramammary Paget disease (EMPD) is a rare entity which is more frequently localized at the vulva, though it only accounts for 1-2% of vulvar neoplasms. It is a primary cutaneous adenocarcinoma whose cell of origin is still a matter of controversy: it can either arise from apocrine/eccrine glands or from stem cells. The diagnosis demands a biopsy and entails a histopathological analysis by which cells show similar characteristics as breast Paget disease. RECENT FINDINGS: Treatment approach can entail surgery, radiotherapy, photodynamic therapy, systemic chemotherapy, and topical chemotherapy. For metastatic disease, many different chemotherapy regimens have been explored and even targeted therapy can play an important role in this disease. Since almost 30-40% of patients overexpress HER-2, trastuzumab and anti-HER-2 therapies can be employed in this setting. Due to its low incidence, there is almost no specific evidence on therapeutic interventions for this disease. Thus, there is a neat unmet need for molecular characterization of EMPD and diagnostic tools that allow clinicians to guide treatment both in the early and in the advanced disease settings. In this review, we aim to summarize available evidence about diagnosis and treatment of EMPD, both localized and metastatic, and to provide a comprehensive analysis that may help clinicians for therapeutic decisions.

Intra-cavitary brachytherapy in endometrial carcinoma: experience with cobalt-60 source.

Purpose: To report vaginal cuff brachytherapy (VCB) dosimetry parameters and clinical outcomes of patients with localized endometrial cancer treated with adjuvant high-dose-rate (HDR) brachytherapy using a cobalt-60 (60Co) source. Material and methods: Between 2011 and 2017, we identified patients with endometrial cancer treated with surgery and adjuvant VCB. Dosimetry variables analyzed included D2cc, D1cc, and D0.1cc for organs at risk (OARs) and distance from cylinder surface to 150% and 200% isodose line in vaginal mucosa. Local relapse (LR), regional relapse (RR), distant metastasis (DM), progression-free survival (PFS), and overall survival (OS) were analyzed using Kaplan-Meier, and log-rank test was applied to assess differences between groups. Toxicity evaluation was tested for possible cross-correlation within dosimetric parameters using Pearson r test and stepwise multivariate linear regression. Results: We identified 93 suitable patients. Mean age at diagnosis was 66 years (range, 45-85 years). Most patients had endometrioid adenocarcinoma (61.3%), followed by papillary-serous carcinoma (11.8%). 71% of patients presented with FIGO stage I (35.5% IA and 35.5% IB), 11.8% were stage II, and 17.2% were stage III. Adjuvant external beam radiotherapy (EBRT) (range, 46-50.4 Gy) was used in 53.8% of patients, and adjuvant chemotherapy in 38.7%. Median follow-up was 39 months (range, 5-84 months). Three-year OS and PFS were 87.5% and 85.5%, respectively. LR was seen in 2.2% of cases, RR in 7.5%, and DM in 12.9%. Mean rectum D2cc/D0.1cc were 88.1% and 116%, and mean bladder D2cc/D0.1cc were 79.2% and 103.2%, respectively. The most common acute toxicity was vaginal mucositis (8.9% ≥ G2), and the most frequent chronic toxicity was vaginal stenosis (25.3% ≥ G1). Conclusions: Adjuvant high-dose-rate VCB with 60Co source for patients with endometrial cancer is well tolerated, with clinical and toxicity outcomes comparable to those reported with iridium-192 (192Ir) source.