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Jason T Little, Vance T Lehman, Jonathan M Morris, Julia S Lehman, Felix E Diehn
Extramammary Paget disease (EMPD) is an uncommon malignancy. It manifests either in the primary form in the skin as an intraepithelial neoplasm, or in secondary form as pagetoid (intraepithelial) spread of an underlying internal carcinoma to the skin. Although local invasion and recurrence of primary extramammary Paget disease are relatively frequent, widespread metastases are rare. As such, there are very few reports and little characterization of the radiologic features of widespread spinal metastases. To our knowledge, there are no prior reports of a metastatic extramammary Paget disease presenting as a painful pathologic vertebral body compression fracture...
May 2016: Journal of Radiology Case Reports
Yuzo Nagai, Sinsuke Kazama, Daisuke Yamada, Takuya Miyagawa, Koji Murono, Koji Yasuda, Takeshi Nishikawa, Toshiaki Tanaka, Tomomichi Kiyomatsu, Keisuke Hata, Kazushige Kawai, Yuri Masui, Hiroaki Nozawa, Hironori Yamaguchi, Soichiro Ishihara, Takafumi Kadono, Toshiaki Watanabe
Treatment of perianal and vulvar extramammary Paget disease (EMPD), rare intraepithelial malignancies, is often challenging because of its potential to spread into the anal canal. However, there is still no consensus regarding the optimal resection margin within the anal canal. Between 2004 and 2014, six patients (three with perianal EMPD and three with vulvar EMPD) in which the spread of Paget cells into the anal canal was highly suspected were referred to our department. To evaluate the disease extent within the anal canal, preoperative mapping biopsy of the anal canal was performed in five out of six patients...
October 2016: Annals of Dermatology
Yao Zhu, Ding-Wei Ye
Occupational exposure has been causally linked to scrotal cancer. Primary preventative care and avoiding carcinogenic substances have decreased the incidence and changed the treatment of scrotal cancer. The current incidence of scrotal malignancy is approximately 1 per 1,000,000 male persons/year. The rarity of cases and of research impedes our understanding of the changing nature of scrotal cancer. This article summarizes the current knowledge, focusing mainly on pathogenesis and diagnostic evaluation, which may influence prevention and early recognition of the disease...
November 2016: Urologic Clinics of North America
Seung Woo Cho, Hak Chang, Sung Tack Kwon
Extramammary Paget's disease (EMPD) is a rare, slow-growing intraepithelial malignancy that mainly involves the genital region, including the vulva, penis, scrotum, perianal, and periurethral areas. Although several treatment options exist, wide local excision with a safe margin is considered the treatment of choice. After resection of the lesion, it is often challenging to reconstruct the defect because defects in the perineal region require adequate volume for protection and are susceptible to infections, which is a particularly significant risk for large defects...
September 2016: Archives of Plastic Surgery
Jillian W Millsop, Victoria R Sharon, Tatyana Petukhova, Maxwell A Fung, Maija Kiuru
The histopathologic characteristics of reactions caused by the many novel anticancer agents are under-recognized. We report a case of a 67-year-old female with locally advanced metastatic breast cancer, who initially presented with an extensive reticulated erythematous patch on the trunk caused by intravascular metastases confirmed by a skin biopsy. Due to disease progression, she was started on ixabepilone, a mitotic inhibitor. While receiving ixabepilone, another skin biopsy was obtained and initially interpreted as extramammary Paget's disease...
September 30, 2016: Journal of Cutaneous Pathology
Lingling Fan, Jin Zhu, Xiang Tao, Congjian Xu
OBJECTIVE: This study aimed to analyze the clinicopathologic characteristics, management, and outcome of patients with intraepithelial extramammary Paget's disease of the vulva (EMPDV). MATERIALS AND METHODS: This retrospective study included medical records and pathological slides of 18 female patients with EMPDV between January 2001 and December 2012. Diagnosis was confirmed using conventional hematoxylin and eosin staining. RESULTS: The median age was 65 years...
October 2016: Dermatologic Surgery: Official Publication for American Society for Dermatologic Surgery [et Al.]
Diogo Morbeck, Aline Caldart Tregnago, Glauco Baiocchi Netto, Carlos Sacomani, Patricia Maria Peresi, Cynthia Toledo Osório, Luciana Schutz, Stephania Martins Bezerra, Louise de Brot, Isabela Werneck Cunha
AIMS: GATA3 has been reported as a specific urothelial marker among organs in the pelvic region and has been classified as highly sensitive and specific for urothelial and breast carcinomas. Our aim was to verify GATA3 expression in extramammary Paget disease (EMPD) and check if it can be use to differentiate primary vulvar Paget disease (PVPD) from pagetoid urothelial intraepithelial neoplasia (PUIN). Also we analyzed Her2 protein expression and HER2 gene amplification and its role as prognostic factors in EMPD METHODS AND RESULTS: We analyzed GATA3 and Her2 expression in 11 primary vulvar Paget disease and also 2 PUIN cases...
September 16, 2016: Histopathology
Ryoko Kimura, Yuichi Yoshida, Tatsushi Shiomi, Shigeto Yanagihara, Reiko Tsutsumi, Kazunari Sugita, Osamu Yamamoto
No abstract text is available yet for this article.
September 14, 2016: European Journal of Dermatology: EJD
Hana Kennedy, Jayanth Kunjur, Kavita Amarasinghe, Graham Smith
Extramammary Paget's disease (EMPD) is a rare, cutaneous adenocarcinoma in situ characterized by a chronic eczema-like rash of the anogenital regions. It is usually slow growing, locally invasive and presents mainly with pruritis. Extremely rare in the oral tissues, here we present a case of EMPD of the hard palate and a review of the existing literature base.
2016: Journal of Surgical Case Reports
Aya Banno, Keijiro Chiba, Hiroko Kasai, Keiichi Nagami
Pulmonary tumour thrombotic microangiopathy (PTTM) resulting in fatal pulmonary hypertension is a rare complication of malignancy. Patients with PTTM generally suffer rapid deterioration of hypoxaemia, and a diagnosis is often only made at autopsy. We report a case of extramammary Paget's disease associated with PTTM. An ante-mortem diagnosis was made based on cytology of blood aspirated from a pulmonary artery catheter in a wedged position. Together with a review of the literature, this case highlights the potential diagnostic value of blood cytology in patients with cancer with rapidly progressing pulmonary hypertension...
2016: BMJ Case Reports
Anant Shukla, Matthew Stringer, Pansy Uberoi, Steven Hudak, George Kallingal
Extramammary Paget's disease (EMPD) is a rare intraepithelial adenocarcinoma arising from apocrine glands. We describe an innovative surgical technique to manage extensive cutaneous malignancies in a notoriously challenging location. The patient is a 78-year-old male who presented with 'jock itch' on his left groin refractory to topical treatment. A shave biopsy of the lesion demonstrated non-invasive EMPD which yielded a urology consult. Rather than the standard wide local excision (WLE), which can lead to positive margins, progression, and recurrence, we used a modified the Mohs technique and staged the procedure, providing excellent oncologic and cosmetic outcomes...
August 2016: Canadian Journal of Urology
MeiQi May Liau, Shiyao Sam Yang, Kong Bing Tan, Chen Wee Derrick Aw
Extramammary Paget's disease (EMPD) is a rare intraepithelial adenocarcinoma usually found in apocrine-rich areas. Although surgery remains standard treatment, topical imiquimod has emerged as a promising drug for the treatment of EMPD in recent years. We present our experience in treating EMPD in Asian skin successfully with topical imiquimod 5% cream, over the past 10 years in our tertiary institution.
August 16, 2016: Dermatologic Therapy
Stephen R Knight, Charlotte Proby, Dorin Ziyaie, Frank Carey, Sacha Koch
Extramammary Paget disease (EMPD) is a rare perineal neoplasia associated with a high rate of local recurrence. Surgical excision is the standard treatment; however, this has high rates of post-operative morbidity in combination with potentially mutilating results. Previous literature has demonstrated good response with imiquimod 5% cream in patients with vulval EMPD, yet its effectiveness in primary perianal disease is unknown.We describe the case of a 40-year-old woman presenting with EMPD of the perianal region, providing detailed histological and pictoral evidence of its response to topical imiquimod 5% cream over a 16-week period, which initially resulted in remission prior to metastatic lymph node recurrence...
2016: Journal of Surgical Case Reports
Eun Jeong Jang, Young Kyung Bae, Dong Hoon Shin, Doo Jin Lee
No abstract text is available yet for this article.
August 2016: Annals of Dermatology
Zhihua Kang, Feng Xu, Yingfeng Zhu, Pan Fu, Qiao-An Zhang, Tingting Hu, Xiangyu Li, Qunfeng Zhang, Zhiyuan Wu, Xinju Zhang, Hua Wang, Jinhua Xu, Zujun Fang, Ming Guan
Extramammary Paget disease (EMPD) is a rare cutaneous malignant neoplasm. The familial occurrence of EMPD and the high risk of concomitant secondary tumors in EMPD patients have gained much attention. These findings highlight the importance of genetic alterations in the tumorigenesis of this skin cancer. Genetic tests and functional analysis of mismatch repair (MMR) genes were performed in EMPD. The results showed that 8 of 20 cases with germline MMR genes mutations and 5 of them exhibited microsatellite instability (MSI)...
November 2016: American Journal of Surgical Pathology
Gabriel Djedovic, Barbara Del Frari, Johannes Matiasek, David Schiltz, Timm O Engelhardt, Gerhard Pierer, Ulrich M Rieger
Soft tissue defects in the genito-perineal region are predominantly because of trauma, infections, neoplasms or iatrogenic causes. As a result of the region's urological, reproductive and gastrointestinal function, defects in this area may cause devastating physical and psychological consequences as well as diminished sexual functioning. The purpose of this study was to examine the efficacy of implementing a medial thigh lift for defect coverage in the perineal region. A retrospective analysis of all medial thigh lift procedures for defect coverage in the genito-perineal region between November 2010 and March 2015 was conducted at three institutions...
August 1, 2016: International Wound Journal
Maria Tolia, Nikolaos Tsoukalas, Chrisostomos Sofoudis, Constantinos Giaginis, Despoina Spyropoulou, Dimitrios Kardamakis, Vasileios Kouloulias, George Kyrgias
BACKGROUND: Primary invasive Extramammary Paget's vulvar disease is a rare tumor that is challenging to control. Wide surgical excision represents the standard treatment approach for Primary invasive Extramammary Paget's vulvar disease. The goal of the current study was to analyze the appropriate indications of radiotherapy in Primary invasive Extramammary Paget's vulvar disease because they are still controversial. DISCUSSION: We searched the Cochrane Gynecological Cancer Group Trials Register, Cochrane Register of Controlled Trials (CENTRAL), MEDLINE and EMBASE database up to September 2015...
2016: BMC Cancer
Akiko Hagiwara-Takita, Taku Fujimura, Aya Kakizaki, Setsuya Aiba
Ectopic extramammary Paget's disease (EMPD) is a rare variant of EMPD that develops in nonapocrine regions. Since reports about ectopic EMPD are limited, little is known about the biological and immunological background of ectopic EMPD. In this report, we present a case of ectopic EMPD on the lower abdomen that expressed RANKL but lacked the expression of MMP7. As we previously reported, Paget's cells express RANKL and MMP7, release soluble RANKL in the tumor microenvironment, and stimulate tumor-associated macrophages to produce tumor-loading factors in conventional EMPD...
May 2016: Case Reports in Dermatology
H Bontinck, J Bontinck, T Rondou, P Pattyn, F Lockefeer
A peri-anal skin lesion, often eczema-like and with symptoms of pruritus, that does not resolve after classical local therapy should be biopsied. We present a case of peri-anal extramammary Paget's disease (EMDP) and associated anal adenocarcinoma. Reviewing the literature, more than 30% of patients with EMDP present a second primary tumour in their past, present or future history. In Europe, the risk of developing a new primary tumour in patients with this condition is increased compared with the standard population...
May 26, 2016: Acta Chirurgica Belgica
T Fujimura, Y Kambayashi, S Furudate, A Kakizaki, T Hidaka, S Aiba
BACKGROUND: Extramammary Paget's disease (EMPD) is a skin adenocarcinoma of apocrine gland origin, in which Paget cells express receptor activator of nuclear factor kappa-B ligand (RANKL) and matrix metalloproteinase (MMP)7, and release soluble (s)RANKL into the tumor microenvironment. We previously reported that about 60% of the RANK(+) cells among the stromal cells are M2 macrophages, but the identity of the remaining population of RANK(+) cells is still unknown. OBJECTIVE: In this report, we investigated this unknown subpopulation of RANK-expressing cells in EMPD...
July 14, 2016: British Journal of Dermatology
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