EXTRAMAMMARY PAGET´S DISEASE: A CASE REPORT OF VULVAR RECONSTRUCTION WITH GRACILIS MYOCUTANEOUS FLAP AFTER TOTAL VULVECTOMY

INTRODUCTION

Extramammary Paget’s disease is a rare tumor, often associated with multiple recurrences after extensive excisions.¹ It usually affects elderly patients, developing commonly in the anogenital region (vulva, penis, scrotum, perineum and perianal region) and less commonly in the axillary region.^1.2

Paget’s disease was first described by Sir James Paget in 1874, who described 15 patients with eczematous periareolar lesions, who later developed breast cancer.³ The first description of extramammary Paget’s disease was made by Crocker in 1889, who identified skin lesions histologically similar to Paget’s disease, on the skin of the penis and scrotum.⁴

Vulvar reconstruction in Paget’s disease is usually a challenge for the plastic surgeon, depending on the size and stage of the lesion, which can compromise the vulva both unilaterally and bilaterally, and can also compromise the vaginal or anorectal mucosa. Local or regional flaps are generally used for vulvar reconstruction, ranging from random to axial, both fasciocutaneous and myocutaneous.⁵

The ideal flap should (1) bring a good amount of well vascularized tissue with similar thickness to the defect, (2) with minimal morbidity to the donor areas, (3) without tension on the edges, (4) that is able to reestablish the functionally, (5) minimizing damage in gait and in the sitting position, (6) providing a good aesthetic result, (7) with sensitivity if possible, and (8) requiring only one surgical time.⁶

CASE REPORT

65-year-old female patient, hysterectomized for 38 years, with a family history of breast cancer and gastric neoplasia, complaining of 2-year history of pruritus in the vulvar region, without improvement with topical treatments, presenting to the physical examination with an erythematous macula, slightly scaly, without erosion (Figure 1). After biopsy at the site, the presence of Paget cells was confirmed. It was initially treated with topical imiquimod, with a 20% reduction in the size of the lesion. No radiation therapy was performed at the site. Subsequently, she underwent total vulvectomy without lymphadenectomy by the gynecology team, according to the preoperative marking (Figures 2 and 3). For reconstruction, the fasciocutaneous V-Y flap of the medial region of the bilateral thigh was chosen, but after trying to advance the flap towards the defect, it was noted that it would arrive with difficulty, under tension, and its vascularization could be compromised (Figure 4). It was then decided to reconstruct the defect with a fascio-myocutaneous flap of the bilateral gracilis muscle, with perfusion being guaranteed through the branches of the medial circumflex arteries of the thigh (Figure 5). Then, the branches of the obturator nerve to the gracilis muscle were separated. At the end of the procedure, two continuous suction drains were placed below the flaps, bilaterally exiting through the inguinal crease. It was recommended, after the surgery, to use low molecular weight heparin (LMWH) subcutaneously, 40 mg daily for 10 days, in addition to antibiotic prophylaxis with ciprofloxacin 500 mg twice daily for 7 days. Gait was restricted for 1 week, but the patient could sit normally. After 1 week, the drains were removed and the patient was able to walk a few steps.

The patient returned again to the outpatient clinic, within 30 days, walking, without any functional deficit and a good aesthetic result was achieved, with adequate defect coverage, as seen both in the immediate postoperative period and 30 days after the surgery (Figure 6). She continued her follow-up with the team of gynecologists.

DISCUSSION

The lesions of extramammary Paget’s disease usually appear as erythema plaques or spots that can later become erosive and infiltrative.^2,7 These tumors can present clinically in two ways, differing in prognosis. A primary form, as in the presented case, represented by an in situ epithelial carcinoma, usually originated from the proliferation of cells of the apocrine glands, such as the cells called Paget cells, and which has an excellent prognosis after extensive excision. The secondary form is usually represented by a primary, invasive cutaneous adenocarcinoma, underlying an injury, occurring synchronously, leading to a worse prognosis and having a greater variety of immunological markers.^8-10 There is an association with extravulvar adenocarcinoma in 30% of cases .¹⁰ Cytokeratin 7 (CK7) is a Paget cell marker that has high sensitivity in detecting extramammary Paget’s disease.¹¹ Other immunohistochemical marker numbers, such as Her2, p53, Ki-67, cyclin D1, CK20 and p-FAK, are recommended for disease detection and differentiation from primary and secondary forms.^7,12-15 The identification of the combined expression of high levels of Ki-67 and cyclin D1 is significantly associated with invasive lesions.⁷ The patient in question had high levels of CK7 and p53; other markers were negative. Three biomarkers have been shown to be promissing, including serum cell-free (cf)DNA and a combination of serum cytokeratin 19 fragment 21-1 (CYFRA) and serum carcinoembryonic antigen (CEA), because they present a significant increase in patients with evidence of distant metastases, suggesting that they are useful as prognostic markers for therapeutic response.^16-17

The most common differential diagnoses of extramammary Paget’s disease include contact eczema, seborrheic dermatitis, psoriasis, ringworm and intertrigo.⁸ It should also be differentiated from squamous cell carcinoma in situ, Pagetoid variant, which would be the vulvar equivalent of Bowen’s disease.¹⁸

Surgical excision is the treatment of choice, being generally a complex procedure, involving extensive excisions and requiring major reconstructions. Excision generally requires safety margins of at least 2 cm¹⁹, with a recurrence rate that can vary from 8-50% .^8,19 Other treatments have been recommended such as radiotherapy, CO2 laser and topical treatment with 5-fluoracil or imiquimod, however with results inferior to surgery.⁸ Topical treatment with imiquimod 5% cream monotherapy or combined with 5-fluorouracil, can be useful for non-invasive extramammary Paget’s disease, but these chemotherapeutic agents can induce cytological abnormalities in benign epithelial cells around the lesion that simulate a recurrent malignancy.

CONCLUSION

Extramammary Paget’s disease is a disease with a high rate of recurrence when not resected with wide margins, which generally leads to great mutilation, making the reconstruction process difficult. Fortunately, this disease comprise only 1% of neoplasms of the vulva. Vulvar reconstruction with a myocutaneous flap of the gracilis muscle is a safe procedure, and although fasciocutaneous flaps are preferred in this type of reconstruction because they are easier, have good versatility and result in less morbidity, myocutaneous flaps may be necessary in the presence of major defects or to ensure better vascularization.

Role of authors: The author performed all stages of the manuscript. TSS was involved in the care of the patient. TSS summarised the clinical history, obtained clinical images, drafted the manuscript, reviewed and edited. Written informed consent to publication was obtained.

Conflict of interest: None

Role of the funding source: There was no external funding for this study.

Ethical compliance: The study conforms to the World Medical Association Declaration of Helsinki (June 1964) and subsequent amendments. The patient signed the informed consent for the procedure and for use of clinical data for scientific purposes and publication. Patient anonymity was ensured.

Corresponding author

Tiago Sarmento Simão, MD

Rua Capitão Macedo 171, Vila Clementino

CEP 04021-020 São Paulo, SP Brazil

E-mail: tiagossimao@yahoo.com.br