Highlights • TNM staging system for iEMPD has not yet been established. • To establish TNM staging system, we retrospectively collected 301 patients with invasive extramammary Paget’s disease. • 51 ...had distant metastasis and 112 had LN metastasis at diagnosis. • Distant metastasis, 2 or more lymph node metastases, lymphovascular invasion and tumor thickness over 4 mm correlated with worse survival. • We propose a TNM staging system for EMPD using simple factors for classification (distant metastasis, LN metastasis and local tumor status). • Each stage had a statistically distinct survival curve, which could provide important prognostic information in managing EMPD.
Highlights • We collected 151 patients with invasive extramammary Paget's disease. • The rate of LN metastasis in patients without lymphadenopathy was 15%. • On the other hand, patients with ...lymphadenopathy had LN metastasis in 80%. • No difference in survival between patients with or without SLN metastasis. • Patients with lymphadenopathy showed worse survival than those with positive SLN.
In invasive extramammary Paget's disease (EMPD), distant metastases may develop and the condition may become fatal; however, no standardized treatment has been established. Although based on only a ...few cases, several chemotherapy regimens were reported to be promising. We conducted a multicenter, retrospective study to evaluate the efficacy of docetaxel for metastatic EMPD. We retrospectively collected data on 18 metastatic EMPD patients treated using docetaxel from 1998 to 2012 in 12 institutes in Japan. The following clinical data were collected: tumor response, time to progression, overall survival and adverse effects. Of those, three patients treated combined with S-1, one patient treated with weekly schedule and one patient treated combined with radiotherapy were excluded from the further analysis. All 13 patients received monthly docetaxel as the first-line treatment. The average number of treatment cycles was 9.1. Among the 12 patients with a confirmed response, seven (58%) showed a partial response, three (25%) stable disease and two (17%) progressive disease. The disease control rate (partial response + stable disease) was as high as 83%. The time to progression and median overall survival were 7.1 and 16.6 months, respectively. The 1-year overall survival rate determined by the Kaplan-Meier method was 75.0%. All adverse effects were manageable and no treatment-related deaths were observed. The high disease control rate and overall survival shown by this study suggest that first-line use of docetaxel may be a promising treatment for metastatic EMPD. A prospective clinical trial is required to confirm our results.
Subungual melanomas (SUM) are rare, and amputation is often required. Non-amputative wide local excision (WLE) of the nail unit with the periosteum of the distal phalanx, followed by skin graft, has ...been accepted for in situ or SUM of 0.5 mm or less thickness. However, previous reports have included a limited number of cases, and not all more than 0.5-mm thick SUM exhibit invasion or attachment to the distal phalanx. The aim of the present study was to investigate the local recurrence and prognosis for in situ, minimally invasive and invasive SUM that were treated using WLE. We retrospectively reviewed 50 patients with in situ (n = 48) or minimally invasive SUM (n = 2) (in situ or minimally invasive group) and 12 patients with more than 0.5-mm thick invasive SUM (invasive group) who were treated using WLE. All patients survived the follow-up period (24-207 months), although four patients with in situ SUM experienced local recurrence at the lateral margin and re-excision was required. In the invasive group, no patients experienced local recurrence, although one patient (8.3%) developed nodal metastasis at 86 months and regional lymph node dissection was required. WLE may provide acceptable local control for in situ and intermediate thickness SUM, without compromising the vital prognosis. However, a larger randomized prospective study with long-term follow up is required to evaluate adequately the risks associated with a non-amputative WLE for in situ and invasive SUM.
Historically, angioleiomyoma is a subtype of leiomyoma that comprises about 4%-5% of all benign soft tissue tumors, and calcifications occur in 2%-3% of them. Because of the very low incidence of ...calcifications, their mechanism of formation is still unknown. In this article, we present the five cases of angioleiomyomas with calcification and discuss the ultrasonographic features in three of those cases. In general, from the aspect of ultrasonography, angioleiomyomas are recognized as hypoechogenic homogenous structures with peritumoral or intratumoral vascularity. However, our cases revealed heterogeneity or hyperechogenicity in inclusions, which has never been reported before. Histologically, calcifications were found not only under the dystrophic pathway which were considered in the past literature but also have the possibility under the metastatic pathway. According to our findings, we also classified the ultrasonographic patterns into three patterns according to the progression and distribution of calcifications. This is the first report to identify the location and possible pathways of calcifications and to classify the ultrasonographic features of calcified angioleiomyomas.
Immune checkpoint inhibitors have drastically changed in the treatment of many kinds of malignancies, especially malignant melanoma. The focus of the recent experiments has not only been on their ...efficacy but also immune-related adverse events (irAEs). We report a case of fulminant hepatitis due to nivolumab. In this case, the patient had undergone long-term nivolumab therapy. He did not complain of any symptoms but his liver enzyme levels were extremely elevated (grade 4). We promptly decided to start oral corticosteroids in the patient. His liver function rapidly improved. The dose of corticosteroids was gradually reduced. Our case demonstrates that sudden onset fulminant hepatitis can occur despite the safe use of long-term nivolumab therapy. The irAE can improve rapidly with proper corticosteroid treatment. This report will be useful for the physicians who always use immune checkpoint inhibitors.
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