Int J Radiat Oncol Biol Phys 1992;24(1):73-8

Extramammary Paget's disease of the perineal skin: role of radiotherapy.

Besa P, Rich TA, Delclos L, Edwards CL, Ota DM, Wharton JT

Department of Clinical Radiotherapy, University of Texas M.D. Anderson Cancer Center, Houston 77030.

We have reviewed our treatment results in 65 patients with extramammary Paget's disease arising in the vulva, perianal area, or scrotum. In 30 patients with primary disease, positive surgical margins were found in 53%, and there was an actuarial local recurrence rate of 40% within 5 years. The median follow-up period for primary extramammary Paget's disease patients treated with surgery alone was 198 months, and none died of this disease. Three patients treated with definitive radiotherapy were without recurrence at 12, 21, and 60 months after 56 Gy of supervoltage x-rays. In 22 patients with extramammary Paget's disease and associated adnexal or rectal adenocarcinoma, nine treated with surgery alone had a 75% local control rate. Three patients treated with surgery and adjuvant radiotherapy all had local control; of two patients treated with radiotherapy alone, one had persistent adenocarcinoma. The median survival for all patients with extramammary Paget's disease and adenocarcinoma was 22 months. We conclude that patients with extramammary Paget's disease have excellent survival but that local recurrence and morbidity from surgery, especially in the elderly, can be high. Radiotherapy greater than 50 Gy as primary treatment for extramammary Paget's disease in those medically unfit for surgery, or as an alternative to further surgery for recurrence after surgery and for anyone wishing to avoid mutilating surgery, is indicated. For those with adenocarcinoma and extramammary Paget's disease, the use of adjuvant postoperative radiotherapy in doses greater than 55 Gy is indicated because of the high risk of local recurrence after surgery alone.

Clin Oncol (R Coll Radiol) 1991 Jan;3(1):3-5

Radiotherapy: an effective treatment for extramammary Paget's disease.

Brierley JD, Stockdale AD

Regional Centre for Radiotherapy and Oncology, St Luke's Hospital, Guildford, Surrey.

Extramammary Paget's disease is a rare condition. Simple excision may result in local relapse, wide local excision may not be appropriate in elderly patients with extensive disease. The case records of six patients with extramammary Paget's disease treated by radiotherapy were reviewed retrospectively. Radical radiotherapy to the skin controlled the disease in four patients.

Br J Dermatol 1995 Jun;132(6):970-2

Treatment of extramammary Paget's disease by radiotherapy.

Burrows NP, Jones DH, Hudson PM, Pye RJ

Department of Dermatology, Addenbrooke's Hospital, Cambridge, UK.

Extramammary Paget's disease (EMPD) is a rare cutaneous malignancy, which usually occurs in the elderly. Wide local excision is the recommended treatment, although this may not always be feasible. We report our experience of EMPD treated by radiotherapy in five patients. The radiotherapy was well tolerated in each case, and there were no signs of recurrence during follow-up (6 months-8 years). This study shows that radiotherapy is a useful alternative therapy for EMPD, and should be considered particularly in elderly patients who may not tolerate surgery.

Clin Oncol (R Coll Radiol) 1995;7(6):400-1

Perineal Paget's disease: effective treatment with fractionated high dose rate brachytherapy.

Kwan WH, Teo PM, Ngar YK, Yu KH, Choi PH

Clinical Oncology Department, Prince of Wales Hospital, Shatin, NT, Hong Kong.

A patient with perineal extramammary Paget's disease is described. He was treated successfully with fractionated high dose rate brachytherapy.

J Am Acad Dermatol 1985 Dec;13(6):1009-14

Extramammary Paget's disease: prognosis and relationship to internal malignancy.

Chanda JJ

Extramammary Paget's disease is a rare cutaneous adenocarcinoma, usually of epidermal origin and glandular differentiation and frequently associated with an underlying adnexal carcinoma and perhaps with underlying internal malignancy. One hundred ninety-seven cases of extramammary Paget's disease (196 cases reported in the English literature from 1962 to 1982 and one case of my own) are reviewed. It remains a rare cutaneous malignancy that occurs primarily in elderly people. It is seen more frequently in women than in men and occurs predominantly in vulvar and perianal locations. Twenty-six percent of patients with this disease will ultimately die of it or an associated internal malignancy. Twenty-four percent of patients with the disease have an associated underlying cutaneous adnexal adenocarcinoma. These patients have a higher mortality rate--46%--than patients with extramammary Paget's disease without underlying cutaneous adnexal adenocarcinoma. Twelve percent of patients with extramammary Paget's disease have an associated concurrent underlying internal malignancy. The location of the underlying internal malignancy appears to be closely related to the location of the extramammary Paget's disease--i.e., a perianal location is associated with adenocarcinoma of the digestive system, a penile-scrotal-groin location with genitourinary malignancy, etc. A directed internal malignancy search may be of benefit in patients who are diagnosed as having extramammary Paget's disease.

Cancer Invest 1988;6(3):293-303

Extramammary Paget's disease: an annotated review.

Balducci L, Crawford ED, Smith GF, Lambuth B, McGehee R, Hardy C

Division of Hematology Oncology, Jackson VA Medical Center, Mississippi.

The incidence, clinical features, histogenesis, and treatment of extramammary Paget's disease (EMPD) are reviewed. This unusual skin lesion is associated with an underlying adnexal neoplasm in about 50% of cases. Also, the incidence of distant organ malignancies of EMPD is higher than expected by chance. Even in the absence of a recognizable underlying cancer, EMPD may occasionally produce distant metastases, indicating the malignant potential of this condition. Histochemical, immunohistological, and lectin binding studies demonstrate that the cell of origin of EMPD is the exocrine cell of sweat glands. Although EMPD may arise from eccrine cells, derivation from apocrine cells appears more common. The treatment of the primary lesion, by wide margin excision, is fraught by a high recurrence rate. Chemosurgery may reduce local relapse of EMPD. The value of adjuvant radiation therapy is unestablished. Chemotherapy has induced remission in 2 cases of advanced EMPD and needs testing in clinical trials.

Ann Acad Med Singapore 1995 Jul;24(4):636-9

Extramammary Paget's disease: a report of three cases and review of the literature.

Png JC, Tung KH, Wong YE, Mokal N, Rajagopalan R, Chong PY

Department of Surgery, National University Hospital, Singapore.

Extramammary Paget's disease (FMPD) is a rare skin disease with a tendency to recur even in the face of adequate excision. It was first suggested by Weiner in 1937 to be a carcinoma of the apocrine sweat gland with intraepithelial excision. Thus, most cases of EMPD occur in the perianal, perigenital and axilla regions where sweat glands abound, with scrotal EMPD being the commonest. EMPD can occur in both benign or malignant form and is often confused with chronic dermatitis. Diagnosis can only be confirmed by an excision biopsy. The long-term survival in patients without an underlying carcinoma is good. However, EMPD associated with an underlying adnexal carcinoma tends to be more aggressive and the prognosis is poor in such cases. EMPD is also associated with other malignancies such as prostatic, rectal, cervical, breast, bladder and skin carcinomas. Incidences of up to 40% have been reported. Thus, patients with EMPD should be thoroughly screened for associated primary growths. The treatment of EMPD is essentially by surgical excision with clear margins. The options available for a clear margin include intraoperative frozen section,Moh's surgery and paraffin section with delayed re-excision. In this report, we present three cases of EMPD with markedly different outcomes and a review of the literature.

Australas J Dermatol 1991;32(1):45-50

Extramammary Paget's disease and prostatic carcinoma.

Gudmundsen KJ, Smith JM, Dervan PA, Powell FC

Department of Dermatology, Mater Misericordiae Hospital, Dublin, Ireland.

A patient with perianal extramammary Paget's disease (EMPD) occurring simultaneously with prostatic adenocarcinoma is presented. Four previously reported cases of genital EMPD associated with prostatic carcinoma are reviewed. The use of immunohistologic techniques to help establish the diagnosis in some cases, and the relationship of EMPD to underlying adenocarcinoma of sweat glands and of the lower urinary and gastrointestinal tracts is discussed. The importance of a directed search for internal malignancy in the individual patient with EMPD is emphasised.

Gynecol Oncol 2000 Apr;77(1):183-9

Paget's disease of the vulva: pathology, pattern of involvement, and prognosis.

Parker LP, Parker JR, Bodurka-Bevers D, Deavers M, Bevers MW, Shen-Gunther J, Gershenson DM

Department of Gynecologic Oncology, The University of Texas M.D. Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, Texas 77030, USA.

OBJECTIVE: The aim of this study was to determine prognostic factors and risk factors for recurrence in patients with Paget's disease of the vulva. METHODS: The medical records of 76 patients with a diagnosis of Paget's disease of the vulva were retrospectively reviewed. The diagnosis in each case was confirmed by reviewing the pathology. Patients were then divided into four groups by diagnosis: intraepithelial Paget's disease (IEP) (n = 46), invasive Paget's disease (IP) (n = 9), intraepithelial Paget's disease with underlying adenocarcinoma (IEPUA) (n = 13), and intraepithelial Paget's disease with a coexisting cancer (CCA) (n = 8). Comorbid conditions, location of disease, pathologic diagnosis, method of treatment, margin status, and current status of the patient were evaluated. Descriptive statistical data and univariate analysis were generated using the Statview statistical package. RESULTS: A diagnosis of IEPUA, IP, or CCA predicted a poor survival (P = 0. 0017). Patients who had received chemotherapy or radiation as treatment had a poor survival (P < 0.0001 and 0.0002). Patients with clitoral Paget's disease had a higher incidence of death from disease (P = 0.026). When death from all causes was considered, patients treated with wide local excision (WLE) had a significantly longer survival than patients treated with other more radical treatments (P = 0.02). Risk factors for recurrence included treatment with WLE (P = 0.004). CONCLUSIONS: Patients with IP, IEPUA, or CCA have a poorer prognosis than patients with IEP. Location of Paget's disease is important for prognosis; and patients with clitoral Paget's disease may require more aggressive treatment. WLE is associated with a higher risk of recurrence, but overall patients with WLE tend to survive longer than patients treated more radically.

Br J Dermatol 2000 Jan;142(1):59-65

The Roswell Park Cancer Institute experience with extramammary Paget's disease.

Zollo JD, Zeitouni NC

Roswell Park Cancer Institute, Department of Dermatology, Elm and Carlton Streets, Buffalo, NY 14263, USA.

Extramammary Paget's disease (EMPD) is a rare intraepithelial neoplasm. Common sites of occurrence include the vulva, perianal region, perineum and scrotum. Despite frequent recurrences, surgery is the standard treatment. This study examines the recurrence rate for EMPD treated by conventional surgical management. Alternative and multimodal therapeutic approaches are reviewed. This retrospective analysis included all 30 patients treated for EMPD at Roswell Park Cancer Institute (RPCI) between 1970 and 1998. Following conventional surgical treatment, 44% of our patients developed recurrence. Vulvectomy provided the lowest recurrence rate, but involved extensive tissue loss and functional debility. Multimodal treatment using Mohs' micrographic surgery and photodynamic therapy has been used at RPCI to manage EMPD with minimal tissue loss and no functional impairment. Surgical treatment offers a moderate chance of EMPD cure. Long-term multimodal approaches require close follow-up, but may conserve both tissue and function.

Plast Reconstr Surg 1997 Aug;100(2):336-9

Paget's disease of the groin: report of seven cases.

Yugueros P, Keeney GL, Bite U

Division of Plastic and Reconstructive Surgery and Pathology, Mayo Graduate School of Medicine, Rochester, Minn., USA.

Only 27 cases of Paget's disease of the groin have been reported to the present. Our aim was to describe the clinical behavior and treatment of this disease. A retrospective analysis of patients seen at the Mayo Clinic over a period of 25 years (January of 1970 to December of 1995) was undertaken. We included patients with lesions of the groin (isolated or associated with penile/scrotal locations) and with histologic confirmation of the diagnosis. We found seven patients, all male; three patients had isolated lesions. Their mean (SD) age was 73 (8.3) years. Special stains and immunohistochemistry confirmed the diagnosis in all patients. A wide local excision was performed in every patient. Three patients had recurrence; one of them died with multiple pulmonary metastases 4 years later. Two patients presented with history of associated malignancy (prostatic and renal cell carcinoma). Paget's disease of the groin is extremely infrequent. Its origin seems not to be a simple extension from the genital area. Most diagnoses can be made by light microscopy. Wide local excision with free margins is the treatment of choice. Local recurrence occurs in half of patients with tumor-free margins by frozen section; long-term follow-up is warranted. Distant metastases occur rarely, although they can be fatal.

Cancer 1992 Aug 1;70(3):704-8

Cytoreductive combination chemotherapy for regionally advanced unresectable extramammary Paget carcinoma.

Voigt H, Bassermann R, Nathrath W

Department of Dermatological Oncology, St. Bernhard-Hospital, Kamp-Lintfort, Germany.

Extramammary Paget carcinoma (EPC) is considered to originate from the eccrine and apocrine glands of the adnexal skin of the axilla, vulva or penis, scrotum, or perineum. If immunohistochemical techniques with the use of cytoskeletal markers are applied in addition to histologic examination, EPC lesions are found to be multifocal adenocarcinoma of the adnexal sweat glands. So far, therapeutic approaches have included primary surgery, followed optionally by radiation therapy. It is possible to achieve a cure only when manifestations are detected early and confined regionally. The role of palliative chemotherapy has not yet been assessed in this disease, particularly because of the lack of compliance and follow-up in patients with EPC.