Perianal Paget's disease

Williams, Scott; Rogers, Lowell W.; Quan, Stuart H. Q.

doi:10.1007/bf02590848

Cited by 69 publications

(9 citation statements)

References 6 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Generally, the prognosis of PD confined to the epidermis is good, however, it is poorer in patients with underlying anorectal carcinoma [19, 20]. pPD associated with malignancy is reported to have a high risk of local recurrence [21].…”

Section: Discussionmentioning

confidence: 99%

Recurrence with pagetoid spread arising 17 years after surgery for intramucosal rectal cancer: a case report

et al. 2017

View full text Add to dashboard Cite

BackgroundPerianal Paget’s disease (pPD) is uncommon, with only about 180 cases documented in the literature. Anorectal carcinoma with pagetoid spread is even rarer.Case presentationAn 81-year-old woman underwent rectal cancer extirpation with a transanal approach 17 years prior. She has since undergone two reoperations for local rectal cancer recurrence. Then, warts frequently appeared on the vulva on several occasions. Warts appeared on the vulva 1 year ago, which were diagnosed as pPD by biopsy. She underwent perineal tumor resection, and the final histological diagnosis was rectal cancer recurrence with pagetoid spread. The resected stump was positive for cancer cells, and tumor progression was rapid. She underwent additional abdominoperineal resection (Miles’ operation) with lymph node dissection. However, disease progression was rapid and she died 7 months after the Miles’ operation.ConclusionsThere are some case reports describing anorectal carcinoma with pagetoid spread, however, almost of all those cases were synchronous primary anorectal cancer. Here, we report the first case of metachronous recurrence rectal cancer with pagetoid spread arising 17 years after surgery.

show abstract

Section: Discussionmentioning

confidence: 99%

Recurrence with pagetoid spread arising 17 years after surgery for intramucosal rectal cancer: a case report

et al. 2017

View full text Add to dashboard Cite

show abstract

“…9, 10 In this series, we liberally used mapping with 4-mm dermatologic punch biopsies taken at 1 cm intervals beyond the visible margin of the lesion to define the extent of local excision. However, in light of the high local recurrence rate of Paget’s disease noted in this and other studies, 11 the value of such extensive mapping procedures is uncertain.…”

Section: Discussionmentioning

confidence: 72%

“…Sphincter preservation to optimize quality of life is paramount in patients with non-invasive disease. 11 Patients with invasive disease should be considered for more aggressive surgery, including APR if necessary. The role of neoadjuvant radiation to assist in complete resection or to avoid APR may deserve investigation but was not used in our cohort.…”

Section: Discussionmentioning

confidence: 99%

Management and Outcome of Perianal Paget’s Disease

Perez

Trakarnsanga

Shia

et al. 2014

Diseases of the Colon &Amp; Rectum

View full text Add to dashboard Cite

Background Perianal Paget’s disease (intraepithelial adenocarcinoma) is rare, and sometimes difficult to diagnose because symptoms are nonspecific. It is often non-invasive but frequently recurs locally. Invasive disease can metastasize to distant sites. Objective Review diagnosis, management, outcomes of patients with perianal Paget’s disease. Design Institutional databases were queried for all cases of perianal Paget’s disease at Memorial Sloan-Kettering Cancer Center 1950-2011. Clinicopathologic factors were investigated for association with recurrence and survival. Setting Tertiary care center. Patients 65 patients with perianal Paget’s disease: 35(54%) female; median age at diagnosis 66(60–72 years);41 had invasive disease/24 non-invasive; 56% with invasive disease were male. Main Outcome Measures Median follow-up; disease status; local and distant recurrence; distant recurrence; sites of recurrence; disease-specific survival; overall survival; treatment modality. Results 95% with invasive, 87% with non-invasive disease were symptomatic at presentation; the most common symptoms were pruritus and perianal bleeding. Duration of symptoms was longer in patients with invasive(12 months, 4-18 months) versus non-invasive (3.5 months, 1-10 months)(p = 0.02)) disease. Synchronous malignancies unrelated to the primary were noted in 5 with invasive/3 with non-invasive disease. Non-invasive disease was treated with wide local excision; invasive disease with wide local excision(32 (78%)) or abdominoperineal resection(9 (22%)). Forty-one patients(27 invasive/14 non-invasive) required multiple operations for tumor clearance. In those with invasive disease, median time to recurrence was 5 years, median tumor-specific survival 10 years. Limitations Retrospective study, limited by selection bias. Conclusions Perianal Paget’s disease is associated with nonspecific symptoms, frequently delaying diagnosis. Wide local excision is the treatment of choice if negative margins can be obtained. Abdominoperineal resection should be considered for invasive disease. Local recurrence is common; follow-up includes periodic proctoscopy and digital exam. Invasive disease can metastasize to distant sites; follow-up should include examination of inguinal lymph nodes and imaging of liver and lungs.

show abstract

“…The prognosis of simple PPD is relatively favorable, both overall and disease-free survival are approximately 60% at 5-year [4] . However, one subtype of this malignancy, PPD with synchronous or metachronous carcinoma of the anorectum, has a poor prognosis, based on limited reports on this entity [4] , [5] . The natural course of the disease and its optional management is still unknown.…”

Section: Introductionmentioning

confidence: 99%

Perineal Paget's disease: A rare disorder and review of literature

Carbotta

Sallustio

Prestera

et al. 2016

Annals of Medicine &Amp; Surgery

View full text Add to dashboard Cite

IntroductionExtramammary Paget's disease (EMPD) occurs commonly in perineum, vulva and perineal region and is considered as a complex disorder due to different clinical and histological features.Presentation of caseA 61 years old woman had a dermatologic evaluation for anal itch and underwent a skin biopsy with diagnostic of Paget disease in perianal region. Pelvic magnetic resonance showed a huge tumor which involved skin, derma and gluteus and she firstly refused any surgical treatment. One year later, because of increasing of the tumor and bleeding, she underwent the surgical procedure with a complete excision, resection of the skin of the anus, inguinal lymphadenectomy and left colostomy. Because of lymph nodes metastasis, a VLS Miles was sequentially performed. She started oncological follow up that showed liver, lung and pelvic metastasis. She survived for 24 months after surgery.DiscussionIn literature, less than 200 cases of perineal Paget's disease have been reported. EMPD in some cases has an associated adenocarcinoma, which has been associated with a worse prognosis and high mortality. Wrong diagnosis and a superficial consideration of a benign evolution should be considered as the first mistake in clinical practice.ConclusionOur surgical approach is considered in literature as the best one for those cases, followed by the oncological treatment. Those patients need to be better studied and more attention should be paid to the clinical presentation.

show abstract

Perianal Paget's disease

Cited by 69 publications

References 6 publications

Recurrence with pagetoid spread arising 17 years after surgery for intramucosal rectal cancer: a case report

Recurrence with pagetoid spread arising 17 years after surgery for intramucosal rectal cancer: a case report

Management and Outcome of Perianal Paget’s Disease

Perineal Paget's disease: A rare disorder and review of literature

Contact Info

Product

Resources

About