A 67-year-old man noted a 4×6cm sized, erythematous, erosive, oozing patch on the penile shaft, which resisted conservative therapy for three years. Histopathologic examination revealed typical findings of extramammary Paget's disease. Interestingly, nests of Paget's cells in some hair follicular structures were shown in the middermis. The lesion was excised by microscopically controlled excision, using the fresh-tissue technique. There were histologically involved areas extending to quite a distance beyond the clinically normal appearing skin. Mohs micrographic surgery might offer the unique advantage of effective surgical treatment with microscopically controlled tumor free borders in the extramammary Paget's disease.
Aged ; Hair ; Humans ; Mohs Surgery ; Paget Disease, Extramammary* ; Skin

Extramammary Paget's disease (EMPD) is an intraepithelial neoplastic disorder which is included as a rare malignant condition. However, it sometimes shows aggressive behavior of local recurrence and coexisting malignancy. We had experienced nine cases of EMPD involving the scrotum for seven years. Two cases of them presented metastasis. The first case presented extensive inguinal lymph node metastasis with underlying adnexal adenocarcinoma one year after wide local excision. The second case initially presented multiple metastasis to the liver and in the lymph node. The latter, showing fulminant progression with liver metastasis, may be only the second case reported in English literature. EMPD is considered as a malignant neoplasm with aggressive behavior from initial presentation. Because wide local excision of the lesion alone may be occasionally insufficient, a careful follow-up must be done to detect recurrence or internal malignancy.
Aged ; Case Report ; Fatal Outcome ; Female ; Human ; Liver Neoplasms/secondary* ; Magnetic Resonance Imaging ; Male ; Paget's Disease, Extramammary/surgery ; Paget's Disease, Extramammary/pathology* ; Testicular Neoplasms/surgery ; Testicular Neoplasms/pathology*

Breast Neoplasms ; Female ; Humans ; Paget Disease, Extramammary/surgery* ; Sweat Gland Neoplasms ; Vulvar Neoplasms/surgery*

BACKGROUND: Extramammary Paget disease (EMPD) is an uncommon malignant neoplasm affecting apocrine gland-bearing skin which usually occurs in the anogenital area of patients older than 50 years. Although Mohs micrographic surgery (MMS) is recommended for the treatment of EMPD, wide local excision has also been performed by many other surgeons including dermatosurgeons. However, the extent of an adequate resection margin is still under debate. OBJECTIVE: The efficacy of minimal surgical therapy consisting of a wide excision combined with preoperative multiple scouting biopsies and postoperative topical imiquimod was investigated for the treatment of EMPD in Korean patients. METHODS: Between 2006 and 2012, 10 patients with primary EMPD were treated with wide surgical excision, with a surgical margin of less than 2.5 cm. Multiple preoperative scouting biopsies and postoperative topical imiquimod were also performed to delineate the lesional boundaries and to reduce the recurrence rate. RESULTS: During the 6-year follow-up period, complications and recurrences were not observed. CONCLUSION: Minimal surgical therapy may be an effective alternative when MMS is unavailable.
Aminoquinolines ; Biopsy ; Follow-Up Studies ; Humans ; Mohs Surgery ; Paget Disease, Extramammary ; Recurrence ; Skin

OBJECTIVETo investigate the clinical characteristics and surgical treatment of penile Paget's disease.

METHODSWe retrospectively analyzed the treatment and follow-up data of 10 cases of penile Paget's disease surgically treated in Jiangsu Provincial Government Hospital and Jiangsu Provincial People's Hospital from 2008 to 2012.

RESULTSAll the 10 patients received expanded local resection of the lesion with reconstruction of the defects with scrotal skin flaps or free skin flaps from the thigh. All surgeries were successful and the postoperative course was uneventful with complete graft survival and no lymph node metastasis. IIEF scores obtained before and 1 -2 months after surgery showed no statistically significant differences in the penile erectile function (P = 0.229), sexual orgasm (P = 0.761), and sexual satisfaction (P = 0.801) of the patients.

CONCLUSIONWhen penile skin lesions suggest the possibility of Paget's disease, biopsy should be performed and surgery should follow as soon as possible. The ideal surgical option is expanded local resection of the lesion with reconstruction of the defects with scrotal skin flaps or free flaps according to the patient's specific conditions.

Adult ; Aged ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Paget Disease, Extramammary ; surgery ; Penile Neoplasms ; surgery ; Retrospective Studies ; Treatment Outcome

Extramammary Paget's disease (EMPD) is a rare intraepidermal adenocarcinoma. Mohs micrographic surgery is considered the standard treatment. Other treatment modalities such as surgical excision, radiotherapy, topical chemotherapy, and photodynamic therapy are associated with varying degrees of effectiveness, and possible loss of tissue function and disease recurrence are seen. The search for an effective, safe treatment with minimal side effects proves to be challenging. Imiquimod is the first representative of a new class of immunomodulating drugs demonstrating potent antiviral and antitumor activity, and has been approved by the FDA for treating anogenital warts, actinic keratosis and basal cell carcinoma. Recent research has demonstrated its effective usage as a 5% cream in the management of EMPD. We report a case of EMPD of the scrotum successfully treated with topical 5% imiquimod cream. This has never been published in the Korea dermatologic literature.
Adenocarcinoma ; Carcinoma, Basal Cell ; Drug Therapy ; Keratosis, Actinic ; Korea ; Mohs Surgery ; Paget Disease, Extramammary* ; Photochemotherapy ; Radiotherapy ; Recurrence ; Scrotum ; Warts

Extramammary Paget's disease (EMPD) is a rare intraepidermal adenocarcinoma. Mohs micrographic surgery is considered the standard treatment. Other treatment modalities such as surgical excision, radiotherapy, topical chemotherapy, and photodynamic therapy are associated with varying degrees of effectiveness, and possible loss of tissue function and disease recurrence are seen. The search for an effective, safe treatment with minimal side effects proves to be challenging. Imiquimod is the first representative of a new class of immunomodulating drugs demonstrating potent antiviral and antitumor activity, and has been approved by the FDA for treating anogenital warts, actinic keratosis and basal cell carcinoma. Recent research has demonstrated its effective usage as a 5% cream in the management of EMPD. We report a case of EMPD of the scrotum successfully treated with topical 5% imiquimod cream. This has never been published in the Korea dermatologic literature.
Adenocarcinoma ; Carcinoma, Basal Cell ; Drug Therapy ; Keratosis, Actinic ; Korea ; Mohs Surgery ; Paget Disease, Extramammary* ; Photochemotherapy ; Radiotherapy ; Recurrence ; Scrotum ; Warts

Extramammary Paget's disease (EMPD) is an uncommon malignancy involving the epidermis, which sometimes extends into the dermis. Current treatments for EMPD are surgical excision, Mohs micrographic surgery, or laser ablation. We report a case of a 50-year-old man who presented with EMPD. The patient refused to undergo surgery and, as an alternative, he applied 5% imiquimod cream, an immune response modifier, on a daily basis for 8 months. During the initial weeks of therapy, he experienced moderate erythema and flu-like symptoms. However, imiquimod treatment resulted in clinical and histological eradication of EMPD after 8 months with no recurrence during the 18 month follow-up period.
Dermis ; Epidermis ; Erythema ; Follow-Up Studies ; Humans ; Laser Therapy ; Middle Aged ; Mohs Surgery ; Paget Disease, Extramammary* ; Recurrence

OBJECTIVETo investigate the causes, therapeutic methods and prognosis of recurrent extramammary Paget's disease of the penis and scrotum.

METHODSWe retrospectively studied the clinical presentations, therapeutic methods and prognosis of 18 cases of recurrent penile and scrotal Paget's disease, all diagnosed by biopsy and treated by surgical resection of the diseased sites. The incision reached the deep fascia. Frozen and olefin sections of the samples were pathologically examined to identify the involvement of the incisal edge. Seven cases had inguinal lymph node metastasis and underwent unilateral or bilateral inguinal lymph node resection.

RESULTSAll the patients were followed up for 6 months to 8 years (mean 34 months). Four died of distant metastasis and the other 14 experienced no recurrence.

CONCLUSIONFor the cases without metastasis of recurrent penile and scrotal Paget's disease, extended lesion-resection is a preferred option and the prognosis is good. And those with local lymph node metastasis need to undergo local lymph node resection.

Aged ; Aged, 80 and over ; Genital Neoplasms, Male ; surgery ; Humans ; Lymph Node Excision ; Male ; Middle Aged ; Paget Disease, Extramammary ; surgery ; Penile Neoplasms ; surgery ; Prognosis ; Scrotum

OBJECTIVETo explore the clinical manifestation, management, recurrence factors and prognosis of scrotal Paget's disease.

METHODSWe retrospectively analyzed the clinical and pathological data of 23 cases of scrotal Paget's disease diagnosed and treated in our hospital from 1996 to 2008.

RESULTSThe disease was confined to one side of the scrotum in 15, and involved the whole scrotum and penis in 8 of the cases. Three patients showed enlarged inguinal lymph nodes in the same side, and 2 in both sides. All the cases were confirmed by biopsy and treated by surgery. Post-operative follow-up was conducted for 2-68 months, which revealed 5 cases of local recurrence and 1 case of death for systemic metastasis.

CONCLUSIONBiopsy is proved to be important for the early diagnosis of scrotal Paget's disease, and extended excision of local lesion is a preferred management.

Aged ; Genital Neoplasms, Male ; diagnosis ; pathology ; surgery ; Humans ; Male ; Middle Aged ; Paget Disease, Extramammary ; diagnosis ; pathology ; surgery ; Retrospective Studies ; Scrotum ; pathology