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Perianal Paget's Disease-a Case Report and a Review of Current Diagnosis and Management.

ABSTRACT: Paget's disease is an intraepithelial adenocarcinoma arising from the apocrine gland component of the skin. Paget's disease is most common in the breast but extra mammary disease is also seen. Perianal Paget's disease is a rare form of extramammary disease with few cases reported in literature. It can be primary-arising from the skin or secondary-cutaneous metastases of anorectal or genitourinary malignancy. We hereby wish to report a case of perianal Paget's disease that presented as an eczematous lesion and was diagnosed incidentally on biopsy. After appropriate staging, the patient underwent wide local excision till negative margins were obtained. The resultant tissue defect was successfully covered by split-thickness skin grafting.

SUBMITTER: Godbole C

PROVIDER: S-EPMC5705495 | biostudies-other | 2017 Dec

REPOSITORIES: biostudies-other

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Perianal Paget's Disease-a Case Report and a Review of Current Diagnosis and Management.

Godbole Chintamani C Mehta Jyoti J Methil Bijoy B Palep Reshma R Bhuta Prajesh P

Indian journal of surgical oncology 20160901 4

Paget's disease is an intraepithelial adenocarcinoma arising from the apocrine gland component of the skin. Paget's disease is most common in the breast but extra mammary disease is also seen. Perianal Paget's disease is a rare form of extramammary disease with few cases reported in literature. It can be primary-arising from the skin or secondary-cutaneous metastases of anorectal or genitourinary malignancy. We hereby wish to report a case of perianal Paget's disease that presented as an eczemat ...[more]

PMID: 29203997

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Project description:An evidence-based clinical guideline for the diagnosis and management of Paget's disease of bone (PDB) was developed using GRADE methodology, by a Guideline Development Group (GDG) led by the Paget's Association (UK). A systematic review of diagnostic tests and pharmacological and nonpharmacological treatment options was conducted that sought to address several key questions of clinical relevance. Twelve recommendations and five conditional recommendations were made, but there was insufficient evidence to address eight of the questions posed. The following recommendations were identified as the most important: 1) Radionuclide bone scans, in addition to targeted radiographs, are recommended as a means of fully and accurately defining the extent of metabolically active disease in patients with PDB. 2) Serum total alkaline phosphatase (ALP) is recommended as a first-line biochemical screening test in combination with liver function tests in screening for the presence of metabolically active PDB. 3) Bisphosphonates are recommended for the treatment of bone pain associated with PDB. Zoledronic acid is recommended as the bisphosphonate most likely to give a favorable pain response. 4) Treatment aimed at improving symptoms is recommended over a treat-to-target strategy aimed at normalizing total ALP in PDB. 5) Total hip or knee replacements are recommended for patients with PDB who develop osteoarthritis in whom medical treatment is inadequate. There is insufficient information to recommend one type of surgical approach over another. The guideline was endorsed by the European Calcified Tissues Society, the International Osteoporosis Foundation, the American Society of Bone and Mineral Research, the Bone Research Society (UK), and the British Geriatric Society. The GDG noted that there had been a lack of research on patient-focused clinical outcomes in PDB and identified several areas where further research was needed. © 2019 The Authors. Journal of Bone and Mineral Research Published by Wiley Periodicals Inc.

Dataset Information

Perianal Paget's Disease-a Case Report and a Review of Current Diagnosis and Management.

Publications

Perianal Paget's Disease-a Case Report and a Review of Current Diagnosis and Management.

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