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

2016 ◽  
Vol 9 (1) ◽  
pp. 6-10 ◽  
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.

scholarly journals Extramammary Paget’s Disease of the Vulva: Report of Two Cases

Medicina ◽  
2021 ◽  
Vol 57 (10) ◽  
pp. 1029
Author(s):  
Christoforos S. Kosmidis ◽  
Christina Sevva ◽  
Panagiota Roulia ◽  
Charilaos Koulouris ◽  
Nikolaos Varsamis ◽  
...  
Keyword(s):  
Histopathological Examination ◽  
Paget’S Disease ◽  
Rare Condition ◽  
Surgical Excision ◽  
Paget's Disease ◽  
Extramammary Paget’S Disease ◽  
Incisional Biopsy ◽  
Efficient Treatment ◽  
Diagnostic Difficulties ◽  
Extramammary Paget's Disease

Extramammary Paget’s disease is a rare condition, affecting 6.5% of all patients with Paget’s disease. The most common extramammary site is the vulvar area. Although diagnosis in some patients is difficult to set, early diagnosis is of high importance in order to detect the irreversible progression of the lesion early and prevent distant metastasis. An 89-year-old female and a 69-year-old female presented within three months with an eczematous lesion with leukoplakia in the vulva. The incisional biopsy of the skin revealed extramammary Paget’s disease. Both patients underwent a surgical wide local excision of the lesion and the specimens were sent for histopathological examination. Extramammary Paget’s disease has a high potential for distant malignancies and local recurrence, dictating that surgical excision is the most efficient treatment. The rareness of the condition and the diagnostic difficulties underline the need for early skin biopsy, which is the most efficient diagnostic tool.

scholarly journals Trop2 Expression in Extramammary Paget’s Disease and Normal Skin

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.

Weekly docetaxel monotherapy for metastatic extramammary Paget’s disease: Retrospective single‐institute analysis

2020 ◽  
Vol 47 (4) ◽  
pp. 418-422 ◽  
Author(s):  
Yoshio Nakamura ◽  
Keiji Tanese ◽  
Ikuko Hirai ◽  
Keitaro Fukuda ◽  
Yutaka Kawakami ◽  
...  
Keyword(s):  
Paget’S Disease ◽  
Paget's Disease ◽  
Extramammary Paget’S Disease ◽  
Weekly Docetaxel ◽  
Extramammary Paget's Disease

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

2014 ◽  
Vol 8 ◽  
pp. CMO.S13107 ◽  
Author(s):  
Derek Isrow ◽  
Karlos Z. Oregel ◽  
Javier Cortes ◽  
Henry Gomez ◽  
Clara Milikowski ◽  
...  
Keyword(s):  
Locally Advanced ◽  
Paget’S Disease ◽  
Treatment Strategies ◽  
Excisional Biopsy ◽  
Paget's Disease ◽  
Extramammary Paget’S Disease ◽  
Apocrine Glands ◽  
Surgical Interventions ◽  
Early Surgical Intervention ◽  
Extramammary Paget's Disease

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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