SECONDARY EXTRAMAMMARY PAGET'S DISEASE ASSOCIATED WITH RECTAL TUBULOVILLOUS ADENOMA WITH FOCAL HIGH GRADE DYSPLASIA
Juliane Y. Cruz*1, Sassan Soltani5, Christopher Tiner4, Xuedong Wang3, Annie Yessaian6, Howard S. Kaufman1,2
1General Surgery, Huntington Hospital, Pasadena, CA; 2Colorectal Research, Huntington Medical Research Institute, Pasadena, CA; 3Pathology, Huntington Hospital, Pasadena, CA; 4Surgery, Huntington Hospital, Pasadena, CA; 5Gastroenterology, Huntington Hospital, Pasadena, CA; 6Obstetrics/Gynecology, Keck School of Medicine of USC, Los Angeles, CA
Background
Perianal Paget's disease is a rare intraepithelial adenocarcinoma that can be associated with visceral malignancy.
Case Presentation
A 67 year-old female presented in July, 2016 with perianal pain, burning, pruritis, and sloughing of her perianal skin. She was first evaluated by a dermatologist and referred for colonoscopy, which showed no colorectal neoplasia. She underwent a course of antifungal therapy for a possible yeast infection; however, she continued to have thickened skin and pruritus at the posterior aspect of the perianal region and erythema to the perineum. She was taken to the operating room for exam under anesthesia and noted to have white thickened lesions circumferentially from the perianal skin into the anal canal to the dentate line. Biopsies revealed Paget's disease without any invasive component. Urologic and GYN/ONC evaluations as well as PET/CT were performed without evidence of malignancy. After presentation at multidisciplinary tumor board, she was treated with several courses of topical imiquimod with observation for progression of disease, including repeat biopsies. At 14 months from initial presentation she was noted to have a significant response with improvement in symptoms and decreased evidence of Paget's disease clinically and pathologically in all areas except the left posterolateral quadrant of the perianal skin and anal canal. Pathology of these biopsies revealed Paget's disease and a 4mm tubulovillous adenoma with high grade dysplasia at the posterior dentate line. Additional perianal skin biopsies were positive for high risk HPV infection on immunostains. She underwent additional courses of imiquimod, observation, and repeat perianal biopsies and colonoscopy. Her disease persisted in the left posterolateral quadrant. Due to these findings she underwent transanal re-excision of the posterior dentate line of the previous adenoma, high resolution anoscopy, and mapping to determine the ability to locally resect her disease. She most recently underwent full thickness wide local excision of her perianal Paget's with anorectal mucosectomy and reconstruction with a gluteal myocutaneous V-Y flap. The final pathology of the Paget lesion was positive for CK20 and CDX-2 and negative for GCDFP-15, which suggests secondary extramammary Paget's disease associated with a colorectal primary. All margins were widely clear. At 3 months postoperatively, she has complete continence and no evidence of recurrence.
Conclusions
This case is a rare example of secondary extramammary Paget's disease associated with a rectal tubulovillous adenoma with focal high grade dysplasia in the absence of invasive adenocarcinoma. Perianal Paget's disease should always lead to a thorough investigation to differentiate between a primary lesion versus secondary extramammary Paget's disease arising in association with a visceral malignancy.
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