Extramammary Paget Disease (EMPD) is a rare intraepithelial cancer often mistaken for inflammatory and infectious skin conditions, leading to diagnostic delays and increased morbidity. It constitutes 6.5% of cutaneous Paget Disease, predominantly affecting older individuals aged 50 to 80, with a higher prevalence in women. Notably, 10% to 30% of EMPD cases are linked to concurrent visceral malignancies, highlighting the importance of cancer screening.

This is a case of a 77-year-old Filipino male with a four-year history of a solitary, well-defined, irregularly-shaped, erythematous plaque with erosions on the perianal area. Despite two years of self-administered hydrocortisone cream, the lesion progressively expanded into an 8x4x2 cm plaque, accompanied by intermittent pruritus and stinging pain. On consultation, dermoscopy showed milky-red areas, telangiectasias, and hyperpigmentation. Histopathology revealed polygonal neoplastic cells with pleomorphic vesicular nuclei, conspicuous nucleoli, and pale eosinophilic cytoplasm above the basal epidermis. Immunohistochemistry was positive for CK7, GCDFP15, CEA, and EMA, and negative for CK20. Further imaging showed no evidence of concurrent malignancies, reinforcing the diagnosis of primary EMPD. While awaiting further evaluation by other specialties, he was managed with an eight-week course of topical Imiquimod 5% cream, resulting in a reduction in both erythema and lesion thickness.

Overall, this case highlights the importance of recognizing cutaneous cancers that mimic benign skin conditions. The application of diagnostic tools in dermatology, such as dermoscopy, histopathology, and immunohistochemical staining, is crucial for identifying key disease features and establishing an accurate diagnosis, enabling timely and appropriate intervention.

Perianal Paget's disease (PPD) is a rare malignant cutaneous tumor. This paper reported a case of PPD complicated by lung adenocarcinoma and anal canal cancer. The patient, a 76-year-old female, had been experiencing recurrent lower abdominal pain and perianal pruritus for the past 5 years. Upon physical examination, a cauliflower-like neoplasm in size of 5 cm×6 cm was observed on the right perianal skin, with local skin ulceration and a small amount of fluid discharge. The left perianal skin was also involved. In thoracoknee position, a hard mass was palpable in the rectal submucosa at 5-6 points 2 cm from the anal verge. Chest CT revealed multiple lesions in both lungs, indication of metastatic tumors. Further evaluation with fluorodeoxyglucose positron emission tomography and computed tomography (FDG-PET/CT) indicated multiple hypermetabolic nodules in the lungs, hypermetabolic lymph nodes throughout the body, early FDG uptake in a small patch of skin on the left hip, and increased FDG uptake in the anorectal region. Histopathological examination confirmed the diagnosis of lung adenocarcinoma. This resulted in the patient being diagnosed with PPD, lung adenocarcinoma, anal canal cancer, and systemic multiple lymph node metastasis. The combination of PPD with gastrointestinal tumors and other metachronous malignant tumors is highly prevalent. Colonoscopy, FDG-PET/CT, histopathology, and immunohistochemistry play crucial roles in early identification of local lymph node and distant involvement, facilitating the evaluation of potential malignant tumors and differential diagnosis. Treating methods for PPD are currently diverse, including postoperative combined or single chemotherapy, radiotherapy, targeted therapy, and photodynamic therapy. As trerapeutical options continue to develop, the extent and efficacy of surgery need to be reassessed.
Female ; Humans ; Aged ; Paget Disease, Extramammary/pathology* ; Fluorodeoxyglucose F18 ; Positron Emission Tomography Computed Tomography ; Adenocarcinoma of Lung/complications* ; Lung Neoplasms/complications*

Breast Neoplasms ; Female ; Humans ; Paget Disease, Extramammary/surgery* ; Sweat Gland Neoplasms ; Vulvar Neoplasms/surgery*

Extramammary Paget’s disease (EMPD) of the vulva is a rare vulvar neoplasm but commonly arises during the postmenopausal period. Intraepithelial Paget’s disease may persist for prolonged periods without demonstrating invasion but with high rates of recurrence. Appearance of Paget’s disease in a split-thickness skin graft, is associated with an occurrence outside the grafted area. It demonstrates retrodissemination as the pathologic process hypothesized in the spread of the disease within the skin via lymphatics and vessels creating tissue bridges between sites of involvement. We present a case of an 81-year-old female, the patient came in for complaints of vulvar pruritus beginning at the left inguinal area three years prior to her diagnosis. She consulted with a dermatologist and was initially treated with steroids and emollients. Persistence of symptoms and enlargement of the lesion prompted a vulvar punch biopsy which showed Paget’s disease and referral to the Gynecologic Oncology service. Wide local excision with split-thickness skin grafting was performed. However, one year after her surgery, patient noted vulvar pain and palpable vulvar lesions. Biopsy was done which showed Extramammary Paget’s Disease recurrence. Patient underwent repeat wide local excision with frozen section, and split-thickness skin grafting. With the aid of frozen section, the intraepithelial involvement was noted to spread beyond the grossly apparent lesion. After 6 months post re-excision, patient noted vulvar pruritus and palpable vulvar lesions. Biopsy was done which showed Extramammary Paget’s Disease recurrence. Due to the proximity of the lesion to the sphincter and need for a colostomy, the patient did not consent for re-excision. Imiquimod 5% was chosen as the mode of treatment. The challenges of interventions are to remove or treat disease that may not be visible, without overtreatment and to minimize morbidity from radical surgery. Surgery remains the primary management for EMPD of the vulva. Imiquimod 5% can be used in recurrences. Despite the advances in the knowledge and management of vulvar Paget’s disease the high rate of recurrent disease remains a challenge for optimal management and would require frequent and long-term follow-up.
Paget Disease, Extramammary ; Female ; vulvar neoplasms ;

No abstract available.
Carcinoma, Basal Cell ; Paget Disease, Extramammary

Primary cutaneous mucinous carcinoma (PCMC) is an uncommon tumor of the sweat gland origin. The occurrence of PCMC is mostly in middle-aged and older patients, with a slight male predominance. Most cases of PCMC arise on the head, with a preference for eyelids. The histogenesis of PCMC, whether eccrine or apocrine, remains controversial. We report a rare case of PCMC with secondary extramammary Paget’s disease in the groin of a 75-year-old man, which favored an apocrine origin. Furthermore, based on a review of the literature, we provide several histologic clues that can be used to differentiate PCMC from metastatic mucinous carcinoma.
Adenocarcinoma, Mucinous ; Aged ; Eyelids ; Groin ; Head ; Humans ; Male ; Mucins ; Paget Disease, Extramammary ; Sweat Glands

No abstract available.
Anal Canal* ; Paget Disease, Extramammary* ; Radiotherapy* ; Vulva*

No abstract available.
Hyperpigmentation ; Paget Disease, Extramammary* ; Scrotum*

Pigmented extramammary Paget's disease (PEMPD) is an uncommon intraepithelial adenocarcinoma and a rare variant of Paget's disease, characterized as a superficial pigmented scaly macule clinically and an increased number of melanocytes scattered between the Paget's cells histologically. So it may be confused clinically and histologically with melanocytic tumors, dermatitis and other dermatoses. Different therapeutic attitudes are required in this case of adenocarcinoma in situ as opposed to melanoma and dermatitis. Condyloma acuminatum (CA) is a common sexually transmitted disease caused by human papilloma virus infection, which is also called as genital warts. In this article, we first reported a case of a 65-year-old Chinese man who had pigmented extramammary Paget's disease complicated with CA. This patient presented with verrucous papules on the scrotum for 3.5 years, infiltrative erythema with itch on the mons pubis for 3 years, and scrotum and penis involved gradually for 4 months. Physical examination showed a 8 cm×10 cm dark red patch on the upper part of the scrotum, penis and mons pubis, as well as few maculopapules and nodules. Histopathologic examination of the lesion on the scrotum revealed a focus of Paget's disease, characterized by the presence of large round cells with abundant pale or granular/dusty cytoplasm, pleomorphic vesicular nuclei and prominent nucleoli (Paget's cells), while the histology of the verrucous lesion was consistent with CA. Immunohistochemistry was performed, which showed diffuse positive staining with CK, CEA, PAS, CK20, EMA, CK7, and Ki-67 (40%), HER2 in Paget's cells and negative with P53, P16, CK5/6, S100, MelanA, HMB45, estrogen receptor, progesterone receptor, and gross cystic disease flid protein 15 (GCDFP15). Human papillomavirus-11 (HPV-11) was positive by genotyping using gene amplification in the lesion of scrotum. According to clinical features and laboratory findings, a diagnosis of PEMPD complicated with CA was made. Local excision of the lesion was performed and sent for histological examination, with all margins clear of tumor. Both aforementioned diseases often occur in the vulva. Even so, it has been rarely reported coexisting of the above two diseases, of which the clinical significance and association are also unclear. In this article, we also reviewed the literature relating to PEMPD, and on this basis, the profile of this disease is discussed including its pathogenesis, clinical manifestation, diagnosis, treatment and advances. Due to PEMPD occasionally accompanied with an underlying carcinoma, it's essential to make an accurate diagnosis. Besides, review of the literature reveals that pigmented variant of Paget's disease could be initially misdiagnosed as melanocytic tumors and other dermatoses unless the entity is considered in the differential diagnosis and additional confirmatory studies are performed.
Aged ; Condylomata Acuminata/complications* ; Diagnosis, Differential ; Humans ; Immunohistochemistry ; Male ; Melanoma ; Paget Disease, Extramammary/pathology* ; Penis ; Scrotum/pathology*

Pagetoid Bowen disease is a histological variant of Bowen disease which demonstrates large pale staining cells (pagetoid cells). It requires differential diagnosis from other cutaneous malignancies with similar patterns, such as extramammary Paget's disease (EMPD) and Pagetoid melanoma in situ. Herein, we report a case of Pagetoid Bowen disease which was initially misdiagnosed as ectopic EMPD.
Bowen's Disease* ; Diagnosis, Differential ; Immunohistochemistry ; Melanoma ; Paget Disease, Extramammary*