Dermatologica Sinica

: 2020  |  Volume : 38  |  Issue : 4  |  Page : 250--251

Radiotherapy for invasive extramammary Paget's disease: A case report

Jia-Di Lin1, Dai-Wei Liu2, Ming-Hsun Li3, Chung-Hsing Chang4,  
1 Department of Dermatology, Skin Institute, Hualien Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Hualien, Taiwan
2 Department of Radiation Oncology, Hualien Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Hualien, Taiwan
3 Department of Pathology, Hualien Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, Hualien, Taiwan
4 Department of Dermatology, Skin Institute, Hualien Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation; Department of Dermatology, Institute of Medical Sciences, Tzu Chi University, Hualien, Taiwan

Correspondence Address:
Dr. Chung-Hsing Chang
No. 707, Sec. 3, Chung-Yang Rd., Hualien 970

How to cite this article:
Lin JD, Liu DW, Li MH, Chang CH. Radiotherapy for invasive extramammary Paget's disease: A case report.Dermatol Sin 2020;38:250-251

How to cite this URL:
Lin JD, Liu DW, Li MH, Chang CH. Radiotherapy for invasive extramammary Paget's disease: A case report. Dermatol Sin [serial online] 2020 [cited 2021 Aug 5 ];38:250-251
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Full Text

Dear Editor,

Extramammary Paget's disease (EMPD), is a rare intraepithelial adenocarcinoma that arises in apocrine gland-bearing areas of the skin such as the vulva, anus, scrotum, and penis, typically presents as a slow-progressing carcinoma in situ, with favorable prognosis, but possibly fatal with progress beyond the basement membrane. This invasive form of EMPD (iEMPD) occurs in 5%–25% of the cases.[1] Complete surgical removal of localized disease is the chosen treatment for EMPD. However, surgical excision carries the risk of anesthesia-related complications and a functional decline; accordingly, radiotherapy has been suggested as a less invasive and potentially curative treatment, with a lower risk of adverse effects.[2] Previous studies have reported significantly higher frequencies of p53 overexpression and high Ki-67 and cyclin D1 expression in invasive lesions than in in situ lesions.[3],[4],[5] Here, we report our experience with a case of scrotal iEMPD in an elderly man. The lesion was treated with radiotherapy, and a pathologic analysis revealed p53 overexpression and high cyclin D1 and Ki-67 expression.

A 79-year-old male presented at the dermatology clinic in December 2014 with skin lesions over the right scrotal area. A skin examination revealed a 3.5 cm × 3.5 cm, ill-defined, scaly, and erythematous patch of lesions over the right scrotal area, associated with mild itchiness for several months. A skin biopsy revealed clusters of large cells with clear cytoplasm in the epidermis, consistent with EMPD. Surgical treatment was suggested; however, he refused surgery and was lost to follow-up. He visited our clinic 4 years later, in May 2018. The skin lesions had progressed to a 7.5 cm × 5 cm area of irregular, ill-defined erythematous patches with central erosion, oozing and scales over the right scrotal area. A second biopsy revealed the invasion of Paget's cells into the dermis (thickness >4 mm). Periodic acid–Schiff staining (+) and carcinoembryonic antigen (+), cytokeratin 7 (+), cytokeratin 20 (−), and gross cystic disease fluid protein-15 (+) test results indicated iEMPD. The computed tomography revealed neither lymphadenopathy nor distant metastasis. TNM staging yielded a diagnosis of pT2N0M0, Stage II.[6]

A discussion with the patient and his family revealed their preference for a nonsurgical treatment strategy because of the patient's age. After consultation with the radiologist, the patient underwent intensity-modulated radiation therapy at a total dose of 50.4 Gy in 1.8-Gy daily fractions to the gross tumor over a total duration of 5 weeks. The disappearance of the erythematous plaque and healing of the ulcers were observed 4 weeks after radiotherapy [Figure 1]. A 6-month follow-up revealed that the disease was controlled without local recurrence. We evaluated Ki-67, cyclin D1, and p53 expression at the first and second biopsy and detected high expression of Ki-67 and cyclin D1 both in situ and in the iEMPD lesions (3+, >50% of Paget cells positive). Moreover, positive p53 expression was detected in both the in situ and invasive lesions [Figure 2].{Figure 1}{Figure 2}

Previous studies have observed significantly higher Ki-67 and cyclin D1 expression in iEMPD than in in situ EMPD, and positive p53 immunostaining in iEMPD cases.[3],[4],[5] However, no longitudinal studies have evaluated Ki-67, cyclin D1, and p53 expression during the progression of in situ EMPD to iEMPD. Our case had been diagnosed with EMPD 4 years before presentation at our hospital, during which time the lesion progressed to the invasive form. The pathology 4 years prior indicated p53 overexpression and high Ki-67 and cyclin D1 expression in the in situ EMPD lesions, suggestive of potential for invasiveness.

A previous study included 41 genital EMPD patients who underwent radiation therapy (total doses: 45–80.2 Gy) with curative intent. The local progression-free and disease-free rates were 88% and 55%, respectively, at 3 years. Tumor invasion into the dermis and regional lymph node metastasis were poor prognostic factors. Adverse effects were mild (Grade 1 or 2), included hematologic toxicities, dermatitis, colitis, and cystitis.[7] A large series study examined 1439 patients with iEMPD, of whom 2.7% (forty patients) received radiotherapy alone. Radiotherapy was associated with the male sex, old age, advanced stage, and anorectal disease, and patients receiving radiotherapy alone had a mean disease-specific survival of approximately 12 years (143.4 months).[8] In our case, the patient chose to receive radiotherapy after considering the age-related risks of anesthesia complications and a postoperative functional decline. No intolerable adverse effects were associated with radiotherapy, except for cutaneous candidiasis infection during radiotherapy [Figure 1]f, which was successfully treated with topical antifungal agents. Although the disease was controlled without local recurrence at the 6-month follow-up, the high risk of iEMPD recurrence and metastasis mandate long-term follow-up.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.


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