Advanced Extramammary Paget's Disease of the Groin, Penis, and Scrotum

Extramammary Paget's disease (EMPD) is a rare intraepithelial malignancy arising in areas rich in apocrine glands, such as the perineum, vulva, axilla, scrotum, and penis. We describe the case of a man in his 50s who initially presented with a small eczematous lesion on his right groin, treated with topical ointments for eczema, until excisional biopsy of lesion unequivocally revealed invasive EMPD. Despite aggressive surgical interventions, his disease progressed to involve the scrotum and penis. Deemed unresectable, the patient was treated with systemic chemotherapy with minimal response. The rarity of EMPD, especially of the penis and scrotum, warrants an educated eye and heightened index of suspicion when dealing with eczematous lesions in the groin in any person. Early biopsy and histological examination is crucial for early surgical intervention of the lesions. There are no guidelines available to treat locally advanced unresectable disease. In addition, further studies are needed to identify genetic defects underlying the pathogenesis of this rare disease, to help improve treatment strategies and decrease morbidity.

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Trop2 Expression in Extramammary Paget’s Disease and Normal Skin

International Journal of Molecular Sciences ◽

10.3390/ijms22147706 ◽

2021 ◽

Vol 22 (14) ◽

pp. 7706

Author(s):

Takamichi Ito ◽

Keiko Tanegashima ◽

Yuka Tanaka ◽

Hiroki Hashimoto ◽

Maho Murata ◽

...

Keyword(s):

Normal Skin ◽

Paget’S Disease ◽

Disease Free Survival ◽

Paget's Disease ◽

Hair Follicles ◽

Surface Glycoprotein ◽

Epidermal Keratinocytes ◽

Extramammary Paget’S Disease ◽

Apocrine Glands ◽

Extramammary Paget's Disease

Extramammary Paget’s disease (EMPD) is a rare skin cancer arising in the apocrine gland-rich areas. Most EMPD tumors are dormant, but metastatic lesions are associated with poor outcomes owing to the lack of effective systemic therapies. Trophoblast cell surface antigen 2 (Trop2), a surface glycoprotein, has drawn attention as a potential therapeutic target for solid tumors. Sacituzumab govitecan, an antibody–drug conjugate of Trop2, has recently entered clinical use for the treatment of various solid cancers. However, little is known about the role of Trop2 in EMPD. In this study, we immunohistochemically examined Trop2 expression in 116 EMPD tissue samples and 10 normal skin tissues. In normal skin, Trop2 was expressed in the epidermal keratinocytes, inner root sheaths, and infundibulum/isthmus epithelium of hair follicles, eccrine/apocrine glands, and sebaceous glands. Most EMPD tissues exhibited homogeneous and strong Trop2 expression, and high Trop2 expression was significantly associated with worse disease-free survival (p = 0.0343). These results suggest the potential use of Trop2-targeted therapy for EMPD and improve our understanding of the skin-related adverse effects of current Trop2-targeted therapies such as sacituzumab govitecan.

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A Case of Advanced Extramammary Paget's Disease Successfully Controlled by Monthly but Not Weekly Docetaxel Chemotherapy

Case Reports in Oncology ◽

10.1159/000443234 ◽

2016 ◽

Vol 9 (1) ◽

pp. 6-10 ◽

Cited By ~ 1

Author(s):

Yuki Yoshihara ◽

Munenari Itoh ◽

Yoshimasa Nobeyama ◽

Hidemi Nakagawa

Keyword(s):

Paget’S Disease ◽

Optimal Schedule ◽

Surgical Excision ◽

Paget's Disease ◽

Japanese Woman ◽

Extramammary Paget’S Disease ◽

Apocrine Glands ◽

Weekly Docetaxel ◽

Docetaxel Chemotherapy ◽

Extramammary Paget's Disease

Extramammary Paget's disease (EMPD) is an uncommon cutaneous adenocarcinoma arising from the apocrine glands within the epidermis or underlying skin appendages in the anogenital and axillary regions. Surgical excision is basically performed as a treatment for EMPD. However, therapeutic options for EMPD in an advanced stage are limited. Herein, we report the case of a Japanese woman with advanced EMPD successfully controlled by monthly but not weekly docetaxel therapy. We also demonstrate the possibility that a monthly regimen of docetaxel is a more effective and optimal schedule than a weekly one through this case report.

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Extramammary Paget’s Disease: Diagnosis, Pathogenesis, and Treatment with Focus on Recent Developments

Current Oncology ◽

10.3390/curroncol28040260 ◽

2021 ◽

Vol 28 (4) ◽

pp. 2969-2986

Author(s):

Shoichiro Ishizuki ◽

Yoshiyuki Nakamura

Keyword(s):

Paget’S Disease ◽

Treatment Strategies ◽

Distant Metastases ◽

Disease Diagnosis ◽

Paget's Disease ◽

Extramammary Paget’S Disease ◽

High Incidence ◽

Recent Developments ◽

Extramammary Paget's Disease

Extramammary Paget’s disease (EMPD) is a rare neoplasm that usually develops in apocrine gland-bearing areas, such as the vulva, scrotum, and penis. EMPD may present with a focal, multifocal, or an ectopic lesion. Clinically, EMPD lesions often exhibit infiltrative erythema, which is sometimes similar to other skin disorders such as eczema. While primary EMPD arises as intraepithelial neoplasm of the epidermis, EMPD-like lesions may occur from epidermotropic spread of malignant cells or direct extension from an underlying internal neoplasm, known as secondary EMPD. Because treatment strategies differ for primary EMPD and secondary EMPD, accurate diagnosis based on detailed histopathological evaluation is required. In the early stages, EMPD usually shows indolent growth, and most cases are diagnosed as carcinoma in situ. However, invasive lesions may result in metastases, and deep invasion is associated with high incidence of metastases. Conventional chemotherapies have been used for EMPD treatment in patients with distant metastases, but the efficacy is not satisfactory, and the prognosis for such patients remains poor. Recent studies have provided various insights into the molecular pathogenesis of the development and advancement of EMPD, which may lead to novel treatment approaches for metastatic EMPD. This review addresses the diagnosis, pathogenesis, and treatment of EMPD with focus on recent progress in understanding this disease.

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Definitive Radiotherapy for Penoscrotal Extramammary Paget’s Disease: A Case Report with Long-Term Follow-Up

Clinical Medicine Insights Case Reports ◽

10.1177/11795476211009251 ◽

2021 ◽

Vol 14 ◽

pp. 117954762110092

Author(s):

Gen Suzuki ◽

Kazutaka Machida ◽

Akito Asato ◽

Sho Seri ◽

Chika Ikemoto ◽

...

Keyword(s):

Therapeutic Option ◽

Multidisciplinary Treatment ◽

Paget’S Disease ◽

Treatment Strategies ◽

Paget's Disease ◽

Severe Toxicity ◽

Extramammary Paget’S Disease ◽

Treatment Team ◽

Extramammary Paget's Disease

Penoscrotal extramammary Paget’s disease (EMPD) is a rare intraepithelial adenocarcinoma in older male patients, and no effective nonsurgical treatment strategies are currently available. The aim of this study was to report the usefulness of external radiotherapy (RT) for penoscrotal EMPD in an inoperable elderly patient. This report presents the treatment of an 89-year-old man with widespread penoscrotal EMPD. A multidisciplinary treatment team decided on radical RT. The patient received a radiation dose of 61.8 Gy in 30 fractions through electron and photon beams. His treatment tolerance was good, and no severe toxicity had been observed up the last follow-up. At 6.5 years after the RT, the patient showed no evidence of recurrence. Definitive RT resulted in excellent disease control and minimal toxicity; thus, it could be a promising nonsurgical therapeutic option for penoscrotal EMPD, even in extremely elderly individuals.

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