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Extramammary Paget disease of the vulva: minimal excision with adjuvant radiation treatment for optimal aesthetic results

Abstract

Aims and background

Extramammary Paget disease (EMPD) is an uncommon malignant neoplasm that occurs in areas containing apocrine glands, and the vulva is the most commonly involved site. Wide surgical excision with subsequent defect reconstruction is widely accepted as the first-line treatment for EMPD. More recently, radiation treatment has been considered an appropriate primary or adjuvant treatment. In an effort to replace wide excision and avoid unfavorable aesthetic results and sexual dysfunction, we decided to excise the tumor minimally and to add adjuvant radiation treatment.

Methods

A 71-year-old woman had EMPD at the right labium majus. The patient was treated by minimal excision with a 1-cm safety margin and by adjuvant radiation treatment weekly from 1 month to 3 months after surgery to a total dose of 70.2 Gy. Four months after completing the radiation treatment, a 10-point surgical biopsy around the site of previous resection was performed. Radiation treatment caused hypertrophy of the left labium minus, which was treated by additional labiaplasty (labium minus reduction) to obtain optimal aesthetic results.

Results

Surgical biopsy revealed no definite evidence of recurrence. Currently, the patient had no EMPD symptoms, is satisfied with a symmetrical vagina, and experiences no discomfort during ordinary activities or intercourse.

Conclusions

Minimal tumor excision (with a 1-cm safety margin), adjuvant radiotherapy, and additional labiaplasty were performed to treat EMPD. Complete tumor removal without recurrence, an aesthetically satisfactory result, and normal sexual function were achieved in this patient.

Tumori 2016; 102(Suppl. 2): e84 - e86

Article Type: CASE REPORT

DOI:10.5301/tj.5000394

Authors

Myeong Su Jeon, Gyu Yong Jung, Joon Ho Lee, Kyung Won Kang, Kwang Won No

Article History

Disclosures

Financial support: None.
Conflict of interest: None.
Meeting presentation: Presented as a poster at the 5th Research & Reconstructive Forum of the Korean Society of Plastic and Reconstructive Surgeons, May 14-15, 2015, Gangwondo, Korea.

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Introduction

Extramammary Paget disease (EMPD) is an uncommon malignant neoplasm that occurs in areas containing apocrine glands, such as the perianal region, penis, scrotum, vulva, perineum, and axilla (1). The vulva is the most common site of involvement, and accounts for up to 60% of primary EMPD cases (2), which usually affects postmenopausal women (3). Extramammary Paget disease typically appears clinically as erythematous, moist, eczematous patches with irregular borders. Although an optimal surgical resection margin is difficult to establish, wide surgical excision with subsequent defect reconstruction is widely accepted as the first-line treatment for EMPD (4). More recently, radiation has been considered an appropriate primary or adjuvant treatment with curative intent, and may be considered in patients with dermal invasion, lymph node metastasis, or a positive surgical margin (5).

In an effort to replace wide excision and avoid unfavorable aesthetic results and sexual dysfunction, we decided to excise the tumor minimally and to add adjuvant radiation treatment.

Methods

A 71-year-old woman presented to our department of plastic and reconstructive surgery on August 4, 2014. The patient complained of pruritus and pain with erythematous plaque on both labia majora of 10 years’ duration; symptoms had worsened on the right labium majus during the previous 3 months. Preoperative punch biopsy showed several scattered Paget cells in epidermis on the side of the right labium majus and herpes simplex virus infection on the left labium majus. No regional adenopathies were detected. Staining revealed that Paget cells were positive for periodic acid-Schiff, alcian blue, carcinoembryonic antigen, and cytokeratin 7. The size of the EMPD lesion on the right labium majus was 5.3 × 2 cm (Fig. 1).

Preoperative photograph shows extramammary Paget disease at the right labium majus, 5.3 × 2 cm, and herpes simplex viral infection at the left labium majus.

Gastrointestinal and genitourinary neoplasms were excluded by computed tomography.

The operation was performed under regional anesthesia in the lithotomy position. After administering 2% lidocaine/ 1:80,000 epinephrine, minimal excision with an additional 1-cm safety margin was performed. Meticulous hemostasis and sufficient undermining were carried out, and the surgical defect was closed directly using 5-0 Monocryl and 5-0 nylon. Resected tissue was sent to pathology for histopathologic examination. Wound dressing was performed every other day, and oral antibiotics and antiviral agents (for the herpes simplex viral infection) were administered. Skin sutures were removed on the seventh postoperative day.

Postoperative histopathologic examination showed positive Paget cells at all resection margins but none at the deep resection margin. The tumor was confined within the epithelium of surface epidermis, and there was no dermal invasion.

One month after the operation, the patient consulted the department of radiation oncology at Sunlin Medical Center for adjuvant radiation treatment, which was administered using an electron beam of 6 MeV, at 1.8 Gy per day, 5 times per week, up to 50.4 Gy over 6 weeks (from September 18 to October 31, 2014) to perineum, both inguinal lymph nodes, followed by 19.8 Gy to perineum for 2 weeks (from November 3 to 18, 2014) by intensity modulated radiation therapy. The total dose prescribed to the skin surface was 70.2 Gy.

Four months after completing the radiation treatment, we decided to perform a surgical biopsy. The patient complained of hypertrophy at the left labium minus. The length of the right and left labia minora were 6.4 and 7.3 cm, respectively, and their widths were 1.6 and 2.7 cm, respectively. The most protuberant part of the left labium minus was at the middle and lower region, and thus, we planned additional labiaplasty (labium minus reduction) for optimal aesthetic results.

With the patient in a lithotomy position, regional anesthesia was performed, and a 10-point surgical biopsy was designed around the previous resection site and surgical marking of the left labium minus was performed for wedge excision (Fig. 2). A V-shape wedge excision was performed from the lower 2/3 portion of the left labium minus. The medial side of the wedge excision was extended internally to terminate distal to the hymeneal ring. Its lateral side was not extended to the labium majus. After excision and meticulous hemostasis, we reapproximated edges of mucosa and submucosa without tension.

Photograph shows hypertrophy of the left labium minus 4 months after radiation treatment and design of the surgical biopsy and labiaplasty (reduction of left labium minus by V-shape wedge excision).

Results

Postoperative surgical biopsy revealed no evidence of recurrent disease in the treated area. Currently, the patient has no EMPD symptoms, is satisfied with a symmetrical vagina, and experiences no discomfort during ordinary activities or intercourse. A postoperative photograph is provided in Figure 3.

Postoperative photograph taken 2 weeks after surgical biopsy and labiaplasty.

Discussion

James Paget first described mammary Paget disease in 1874, and EMPD was first described by Crocker in 1889 (6). Extramammary Paget disease is a rare disorder that is more common in women, especially in those aged between 50 and 80 years (5). The vulva is the most commonly affected site and this involvement accounts for up to 60% of primary EMPD cases (2). Primary EMPD of the vulva, first described by Dubreuilh in 1901 (7), can invade the dermis and metastasize via the lymphatic system. Patients commonly report pruritus, irritation, or burning in the vulvar area, which has an erythematous and/or eczematoid appearance (2).

According to Wilkinson and Brown (8), primary EMPD may be classified as intraepithelial Paget disease (type 1A), intraepithelial Paget disease with invasion (type 1B), or intraepithelial Paget disease with underlying adenocarcinoma of a skin appendage (type 1C). According to this classification, EMPD in our patient was of type 1A. Of these 3 subtypes, type 1A is the predominant type of primary EMPD of the vulva. Cai et al (2) analyzed 43 cases of primary EMPD of the vulva from 1996 to 2009, and reported that 33 (76.7%) of 43 cases were type 1A, followed by 7 (16.3%) type 1B and 3 (7.0%) type 1C.

Treatment options include wide surgical excision, topical 5-fluorouracil and topical bleomycin sulfate, radiation therapy, cryotherapy, chemotherapy, CO2 laser therapy, and photodynamic therapy. Surgical excision is widely considered the standard treatment for EMPD, but extent of resection has not been standardized. Traditionally, the recommended surgical margin for wide excision is 5 cm. In a retrospective study conducted by Hendi et al (9), margins of 5 cm were required to result in clear margins in 97% of EMPD patients. However, such a large margin is likely to cause serious functional and aesthetic impairments, including disfigurement of the vulva, sexual discomfort, and loss of self-confidence. Our patient was a 71-year-old woman with EMPD of the right labium majus, and the size of the tumor was 5.3 × 2 cm. To preserve aesthetic and functional aspects of the vulva, we decided to excise the tumor minimally (1 cm safety margin).

Optimal radiation doses have not been standardized. Dilmé-Carreras et al (10) treated EMPD of the vulva using a total dose of 60 Gy, whereas Park et al (4) treated EMPD of the scrotum to a total dose of 67 Gy. In our patient, a total dose of 70.2 Gy was administrated over 8 weeks after minimal excision. Four months after completing radiation treatment (7 months postoperatively), a 10-point surgical biopsy around the resection site revealed no definite evidence of EMPD.

Most women want straight, thin, light-colored, symmetrical labia minora. The majority of patients with a hypertrophic labia minora dislike its protrusion or dangling beyond the labia majora, and patients complain of pinching while walking or sitting and of invagination during intercourse. After radiation treatment, our patient complained of hypertrophy of the left labium minus, which protruded most at its middle and lower portion. Additional reduction of the labium minus was performed to resolve this problem and a cosmetically pleasing result was obtained.

In conclusion, we performed minimal tumor excision (with a 1-cm safety margin), adjuvant radiotherapy to avoid aesthetic disfigurement and sexual discomfort, and additional labiaplasty in a patient with EMPD of the vulva. These procedures achieved complete tumor removal without recurrence, aesthetically satisfactory results, and normal sexual function.

Disclosures

Financial support: None.
Conflict of interest: None.
Meeting presentation: Presented as a poster at the 5th Research & Reconstructive Forum of the Korean Society of Plastic and Reconstructive Surgeons, May 14-15, 2015, Gangwondo, Korea.
References
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Authors

Affiliations

  • Department of Plastic and Reconstructive Surgery, Dongguk University College of Medicine, Gyeongju - Republic of Korea
  • Department of Radiation Oncology, Sunlin Medical Center, Pohang - Republic of Korea

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