Comparison of mohs micrographic surgery

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Comparison of Mohs Micrographic Surgery and Wide Excision for Extramammary Paget’s Disease W ILLIAM J O’C ONNOR , MD, n K ATHERINE K L IM , MD, n M ARK J Z ALLA , MD, n M AUREEN G AGNOT, HT, n C LARK C O TLEY, MD, w T RI H N GUYEN , MD, w AND R ANDALL K R OENIGK , MD w Department of Dermatology, Mayo Clinic, Scottsdale, Arizona, and wMayo Clinic, Rochester, Minnesota n Extramammary Paget’s disease is a rare cutaneous adenocarcinoma that occurs in an apocrine gland distribution mainly in the anogenital region OBJECTIVE To formulate treatment recommendations for this rare disease, we examined clinical and follow-up data of patients with it METHODS A retrospective review is given about the treatment and outcome for 95 patients at Mayo Clinic, Rochester, Minnesota, and Scottsdale, Arizona, between 1976 and 2001 The literature regarding diagnosis and treatment of this disease is also reviewed BACKGROUND RESULTS Of the 95 patients, 86 had primary disease and had recurrent disease At mean follow-up (wide excision, 65 months; Mohs surgery, 24 months), disease had recurred in 18 of 83 (22%) who underwent standard wide excision, compared with recurrence in of 12 (8%) who had the Mohs micrographic excision CONCLUSION Mohs micrographic surgery compares favorably with wide excision Intraoperative immunostaining with cytokeratin is helpful in delineating disease, as are preoperative scouting biopsies and photodynamic diagnosis W J O’CONNOR, MD, K K LIM, MD, M J ZALLA, MD, M M GAGNOT, HT, C C OTLEY, MD, T H NGUYEN, MD, AND R K ROENIGK, MD HAVE INDICATED NO SIGNIFICANT INTEREST WITH COMMERCIAL SUPPORTERS EXTRAMAMMARY PAGET’S disease is a rare cutaneous adenocarcinoma that occurs in an apocrine gland distribution The most commonly affected sites are the vulva, penis, scrotum, anal and perianal area, axilla, and umbilicus Rarer sites include the eyelid, external auditory canal, truncal skin, and cheek.1 Extramammary Paget’s disease may occur as a primary process or as epidermotropic metastases from an underlying contiguous gastrointestinal or genitourinary carcinoma or from a noncontiguous carcinoma.2 Thus, extensive evaluation of patients at presentation is mandatory Clinically, extramammary Paget’s disease is characterized by a red, moist, eroded plaque, typically in the anogenital region (Figure 1) Itch and discomfort are common symptoms A delay in diagnosis of to 10 years before a biopsy is performed is not unusual.3 Often, the disease is misdiagnosed and treated as ‘‘jock itch.’’ Occasionally, it occurs multicentrically, and triple extramammary Paget’s disease that involves both axillae as well as the perineum has been described.4 Evaluation of the axilla is recommended at initial presentation Address correspondence to: William J O’Connor, MD, Department of Dermatology, Mayo Clinic, 13400 East Shea Boulevard, Scottsdale, AZ 85259 The tumor behaves as a slow-growing intraepithelial adenocarcinoma However, it may become invasive, and dermal tumor nests may metastasize through dermal lymphatics As many as 10% of patients with extramammary Paget’s disease may have lymph node involvement at presentation, but whether sentinel lymph node biopsy is useful is not known A typical pattern of erythema (‘‘underpants pattern’’) has been described as indicating dermal metastases.5 Patients with metastatic extramammary Paget’s disease are poor surgical candidates, and they have a mean survival of only 13 months Histopathology shows epidermal acanthosis or hyperkeratosis Paget’s cells are large round cells with abundant pale staining cytoplasm and a large central reticulated nucleus Frequent mitotic figures may be found Paget’s cells may appear singly or scattered in clusters throughout the epidermis (Figure 2) Immunohistochemistry is important to confirm the diagnosis of extramammary Paget’s disease Cytokeratin is a structural component for cytoskeleton that is expressed in poorly differentiated neoplasms of epithelial origin Paget’s celare disease Results Ninety-five patients (45 men and 50 women) with biopsy-proved disease were studied The mean age was 70.4 years for the men and 69.8 years for the women (overall range, 53 to 87) Contiguous carcinoma was found in 19 of the 95 patients (20%) The most common primary disease sites were the vulva, anal and perianal region, inguinal fold, scrotum, penis, and axilla The patient with axillary disease also had simultaneous disease in the perineum Eighty-six patients had primary disease, and nine had recurrent disease Of the 83 treated with standard wide excision, with intraoperative vertical frozen section control, recurrent disease had occurred in 18 (22%) at followup (mean of 65 months) Urethral disease was noted frequently, and some patients received CO2 laser or radiation therapy after their surgical procedure Twelve patients were treated with Mohs surgery, and in four, immunostains were used at the time of Mohs (Table 1) Cytokeratin (Figure 3) was used in four patients, and carcinoembryonic antigen also was used in two of these four patients Preoperative scouting biopsies were performed in five patients Photodynamic diagnosis was used in two patients (Figure 4) Photodynamic therapy with daminolevulinic acid (Levulan Kerastick) and Wood’s Figure Positive staining of extramammary Paget’s disease with intraoperative cytokeratin at time of Mohs light 16 to 18 hours later also was attempted in one patient The length of follow-up was determined by clinical examinations or biopsy specimens recorded in the patients’ medical record At follow-up (mean of 24 months), local disease had recurred in one Mohs patient (8%) A second patient died of metastatic adenocarcinoma of an unknown primary cause, which raises the question of whether sentinel lymph node biopsy should be performed in advanced cases Of those patients treated with Mohs surgery, one patient was allowed to heal by second intention, and eight had defects that were closed primarily, which emphasizes that extensive laxity in the scrotum often allows primary repair despite large defects Two patients underwent repair with a flap, and one patient had a combined flap full-thickness and split-thickness graft Five patients required more than two layers Two 726 Dermatol Surg 29:7:July 2003 O’CONNOR ET AL.: MOHS FOR PAGET’S DISEASE (a) (b) (c) (e) (d) Figure (a) Recurrent extramammary Paget’s disease after surgery and radiation therapy (b) Topical 20% application of d-aminolevulinic acid (Levulan Kerastick) to perineum (c) Bright red fluorescence of extramammary Paget’s disease with Wood’s light examination 18 hours later, which is helpful in guiding the first Mohs layer (d) (e) Primary closure after clearance Dermatol Surg 29:7:July 2003 patients had Mohs lasting more than day This reinforces the importance of preoperative tumor visualization Discussion The initial diagnosis of extramammary Paget’s disease is often delayed, and early biopsy should be considered in patients with tinea cruris or erythrasma who are unresponsive to appropriate therapy All patients should have a thorough work-up to exclude underlying gastrointestinal, genitourinary, or internal malignancy Immunophenotypes other than cytokeratin 71/ cytokeratin 20– in Paget’s cells suggest underlying regional internal malignancy Preoperative scouting biopsies may be helpful in planning Mohs operations for patients with this disease Shave biopsies are appropriate at the periphery of the tumor, but punch biopsy may be more helpful at its center to evaluate the degree of adnexal and deeper dermal involvement In advanced cases with dermal involvement, sentinel lymph node biopsy may be a consideration Photodynamic diagnosis using topical d-aminolevulinic acid and Wood’s light 16 to 18 hours later also may help delineate the extent of disease, as may preoperative topical 5-fluorouracil Cytokeratin is the immunostain of choice for intraoperative immunostaining It is important to have a histotechnician who is experienced with the processing of large tissue sections Histologic discrimination between eccrine coil and dermal Paget’s disease may also be difficult on frozen sections, and a debulking layer for permanent sections with Mohs for the peripheral margin may be a useful option Treating patients with this type of tumor requires a multidisciplinary approach that involves a colorectal surgeon and a urologist in the work-up, because their expertise may be required in following any urethral and anal extensions of the disease and in repairing defects in these areas Preoperative 5-fluorouracil or photodynamic diagnosis may be used to assess whether extension to these structures has occurred Photodynamic diagnosis may also be considered in following these patients because recurrences often lack symptoms and are not visible clinically A biopsy should be performed if in doubt We found that Mohs micrographic surgery compared favorably with wide excision and had a local recurrence rate of 8% (at mean follow-up of 24 months) versus 22% for patients who underwent wide excision (at mean follow-up of 65 months) Longer follow-up of these patients and a prospective study of a larger series of patients may provide additional O’CONNOR ET AL.: MOHS FOR PAGET’S DISEASE 727 support for Mohs as an excellent treatment for this disease Acknowledgments Dr O’Connor is the recipient of the 2001 Theodore Tromovitch award for this article, which was presented at the Mohs Meeting, Dallas, Texas, October 2001 As a recipient of this award, Dr O’Connor submitted his article to Dermatologic Surgery for publication References Chilukuri S, Page R, Reed JA, Friedman J, Orengo I Ectopic extramammary Paget’s disease arising on the cheek Dermatol Surg 2002;28:430–3 Chanda JJ Extramammary Paget’s disease: prognosis and relationship to internal malignancy J Am Acad Dermatol 1985;13:1009– 14 Coldiron BM, Goldsmith BA, Robinson JK Surgical treatment of extramammary Paget’s disease: a report of six cases and a reexamination of Mohs micrographic surgery compared with conventional surgical excision Cancer 1991;67:933–8 Kitajima S, Yamamoto K, Tsuji T, Schwartz RA Triple extramammary Paget’s disease Dermatol Surg 1997;23:1035–8 Murata Y, Kumano K, Tani M Underpants-pattern erythema: a previously unrecognized cutaneous manifestation of extramammary Paget’s disease of the genitalia with advanced metastatic spread J Am Acad Dermatol 1999;40:949–56 Smith KJ, Tuur S, Corvette D, Lupton GP, Skelton HG Cytokeratin staining in mammary and extramammary Paget’s disease Mod Pathol 1997;10:1069–74 Ohnishi T, Watanabe S The use of cytokeratins and 20 in the diagnosis of primary and secondary extramammary Paget’s disease Br J Dermatol 2000;142:243–7 Harris DW, Kist DA, Bloom K, Zachary CB Rapid staining with carcinoembryonic antigen aids limited excision of extramammary Paget’s disease treated by Mohs surgery J Dermatol Surg Oncol 1994;20:260–4 Del Castillo LF, Garcia C, Schoendorgg C, et al Spontaneous apparent clinical resolution with histologic persistence of a case of extramammary Paget’s disease: response to topical 5-fluorouracil Cutis 2000;65:331–3 10 Moreno-Arias GA, Conill C, Castells-Mas A, Arenas M, Grimalt R Radiotherapy for genital extramammary Paget’s disease in situ Dermatol Surg 2001;27:587–90 11 Zollo JD, Zeitouni NC The Roswell Park Cancer Institute experience with extramammary Paget’s disease Br J Dermatol 2000;142:59–65 12 Henta T, Itoh Y, Kobayashi M, Ninomiya Y, Ishibashi A Photodynamic therapy for inoperable vulval Paget’s disease using d-aminolaevulinic acid:successful management of a large skin lesion Br J Dermatol 1999;141:347–9 13 Shieh S, Dee AS, Cheney RT, et al Photodynamic therapy for the treatment of extramammary Paget’s disease Br J Dermatol 2002;146:1000–5 14 Zampogna JC, Flowers FP, Roth WI, Hassenein AM Treatment of primary limited cutaneous extramammary Paget’s disease with topical imiquimod monotherapy: two case reports J Am Acad Dermatol 2002;47(Suppl):229–35 15 Bamford J, Seidelmann S Clinical and immunologic response of extramammary Paget’s disease to imiquimod [abstract] J Invest Dermatol 2001;117:537 16 Mohs FE, Blanchard L Microscopically controlled surgery for extramammary Paget’s disease Arch Dermatol 1979;115:706–8 17 Wagner RF Jr, Cottel WI Treatment of extensive extramammary Paget disease of male genitalia with Mohs micrographic surgery Urology 1988;31:415–8 18 Eliezri YD, Silvers DN, Horan DB Role of preoperative topical 5fluorouracil in preparation for Mohs micrographic surgery of extramammary Paget’s disease J Am Acad Dermatol 1987;17: 497–505 ... BM, Goldsmith BA, Robinson JK Surgical treatment of extramammary Paget’s disease: a report of six cases and a reexamination of Mohs micrographic surgery compared with conventional surgical excision... Cottel WI Treatment of extensive extramammary Paget disease of male genitalia with Mohs micrographic surgery Urology 1988;31:415–8 18 Eliezri YD, Silvers DN, Horan DB Role of preoperative topical... rate of 8% (at mean follow-up of 24 months) versus 22% for patients who underwent wide excision (at mean follow-up of 65 months) Longer follow-up of these patients and a prospective study of a
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