Extramammary Paget's disease (EMPD) is an intraepithelial neoplastic disorder which is included as a rare malignant condition. However, it sometimes shows aggressive behavior of local recurrence and coexisting malignancy. We had experienced nine cases of EMPD involving the scrotum for seven years. Two cases of them presented metastasis. The first case presented extensive inguinal lymph node metastasis with underlying adnexal adenocarcinoma one year after wide local excision. The second case initially presented multiple metastasis to the liver and in the lymph node. The latter, showing fulminant progression with liver metastasis, may be only the second case reported in English literature. EMPD is considered as a malignant neoplasm with aggressive behavior from initial presentation. Because wide local excision of the lesion alone may be occasionally insufficient, a careful follow-up must be done to detect recurrence or internal malignancy.
Aged ; Case Report ; Fatal Outcome ; Female ; Human ; Liver Neoplasms/secondary* ; Magnetic Resonance Imaging ; Male ; Paget's Disease, Extramammary/surgery ; Paget's Disease, Extramammary/pathology* ; Testicular Neoplasms/surgery ; Testicular Neoplasms/pathology*

OBJECTIVETo investigate the clinical pathological characteristics, diagnosis and differential diagnosis of Paget's disease of the scrotum and penis.

METHODSThirteen cases of Paget's disease of the scrotum and penis were analyzed by light microscopy, alcian-blue (AB)/periodic-acid-Schiff (PAS) and immunohistochemical staining.

RESULTSPaget's disease of the scrotum and penis mainly affected old individuals aged 55-84 (mean 71) years. Macroscopically, typical presentations of Paget's disease of the scrotum and penis were eczematoid lesions. Microscopically, Paget cells were distributed singly or in groups (as strands, nests or glandular patterns) within the epidermis. Paget cells were typically stained for AB/PAS, positive for CK7, CEA and EMA, and negative for CK5/6, S-100 and P63. The positive rates of GCDFP-15 and CK20 expressions were 76.92% (10/13)and 53.85% (7/13) respectively.

CONCLUSIONPaget's disease of the scrotum and penis is a low-malignancy cutaneous tumor with typical clinical and pathological features. Pathologic diagnosis is based on immunohistochemical findings.

Aged ; Aged, 80 and over ; Humans ; Immunohistochemistry ; Male ; Middle Aged ; Paget Disease, Extramammary ; pathology ; Penile Neoplasms ; pathology ; Penis ; pathology ; Scrotum ; pathology

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*

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*

OBJECTIVETo study the clinicopathologic features and treatment of Paget's disease of the scrotum.

METHODSFifteen cases of Paget's disease of the scrotum, pathologically diagnosed and successfully treated by surgery from 1987 to 2003, were studied retrospectively.

RESULTSTypical pathological features were observed in all the patients. Thirteen cases were followed up for 0. 5 approximately 13 years, of whom 1 case relapsed within 3 years, 2 died of metastasis after 2 years, and 3 died of cardiovascular diseases, with no recurrence in the others.

CONCLUSIONEarly biopsy and extensive excision of the scrotal lesion is the first choice of treatment for Paget's disease, with good operative effect and satisfactory prognosis. A 2nd operation could be considered for patients of local relapse.

Aged ; Aged, 80 and over ; Genital Neoplasms, Male ; pathology ; surgery ; Humans ; Male ; Middle Aged ; Paget Disease, Extramammary ; pathology ; surgery ; Retrospective Studies ; Scrotum ; pathology

OBJECTIVETo explore the clinical manifestation, management, recurrence factors and prognosis of scrotal Paget's disease.

METHODSWe retrospectively analyzed the clinical and pathological data of 23 cases of scrotal Paget's disease diagnosed and treated in our hospital from 1996 to 2008.

RESULTSThe disease was confined to one side of the scrotum in 15, and involved the whole scrotum and penis in 8 of the cases. Three patients showed enlarged inguinal lymph nodes in the same side, and 2 in both sides. All the cases were confirmed by biopsy and treated by surgery. Post-operative follow-up was conducted for 2-68 months, which revealed 5 cases of local recurrence and 1 case of death for systemic metastasis.

CONCLUSIONBiopsy is proved to be important for the early diagnosis of scrotal Paget's disease, and extended excision of local lesion is a preferred management.

Aged ; Genital Neoplasms, Male ; diagnosis ; pathology ; surgery ; Humans ; Male ; Middle Aged ; Paget Disease, Extramammary ; diagnosis ; pathology ; surgery ; Retrospective Studies ; Scrotum ; pathology

OBJECTIVETo evaluate the clinical manifestation, management and prognostic characteristics of scrotal extramammary Pagets disease (EMPD).

METHODSTwenty-three cases of EMPD were identified and retrospectively reviewed, and the clinical findings, surgical treatment, pathologic features and prognostic characteristics were evaluated. The patients ranged in age from 49 to 72 years (mean 62. 4 years). The diagnoses were delayed by 12 to 132 months (67. 6 on average) in 9 cases at Stage A1 , 12 at A2, 1 at B and 1 at D. The rate of initial misdiagnosis was 91.3% (21/23). Twenty of the patients underwent operations, 2 received radiotherapy and 1 chemotherapy.

RESULTSTwenty-two patients of the total number were followed up for 7 to 223 months, averaging at 119, therapy and 1 chemotherapy. of whom 12 remained cancer free, 1 died of tumor, 3 died of intercurrent disease, 4 experienced local recurrence, 1 relapsed with inguinal lymph node metastasis and 1 developed inguinal lymph node metastasis exclusively. Those with relapses received the second surgery, and 5 of them survived without cancer and 1 died of intercurrent disease.

CONCLUSIONThe primary treatment of choice for scrotal EMPD is radical excision. Precise, histological examination before and during the operation is of crucial importance. The positive excisal margins are one of the key causes of local recurrence, and the involvement of dermis by Pagets cells may suggest possible metastasis. Scrotal EMPD tends to occur as a slowly growing lesion, mainly in the elderly, and has a good prognosis in cases of non-invasion. Otherwise the prognosis is poor.

Aged ; Follow-Up Studies ; Genital Neoplasms, Male ; pathology ; therapy ; Humans ; Male ; Middle Aged ; Neoplasm Staging ; Paget Disease, Extramammary ; pathology ; therapy ; Prognosis ; Retrospective Studies ; Scrotum

BACKGROUND: Extramammary Paget's disease (EMPD) is a relatively rare cutaneous disorder. There are a few studies regarding the clinical features and prognostic factors of EMPD in Korean patients. OBJECTIVE: The aim of this study was to identify in detail the clinical findings and prognostic factors of EMPD in Korean patients. METHODS: A total of 19 patients with confirmed EMPD at our institution were included from January 2005 to December 2014. Clinical records and pathology slides of the patients were reviewed retrospectively. RESULTS: Patients' ages ranged from 50 to 87 years (median, 64.5 years), and incidence of EMPD was higher in men than in women (17:2, male:female). The most common predilection site was the penoscrotal area in men and the vulva in women. All patients with EMPD had no underlying malignancy. Four of 19 patients treated with wide excision had local recurrence during the follow-up period. Two patients with deep dermal invasion died due to multiple metastases. CONCLUSION: EMPD in Korea is characterized by its male predominance and penoscrotal location. Disease recurrence is common regardless of the surgical margin. Its prognosis could be associated with depth of invasion and lymph node metastasis.
Female ; Follow-Up Studies ; Humans ; Incidence ; Korea ; Lymph Nodes ; Male ; Neoplasm Metastasis ; Paget Disease, Extramammary* ; Pathology ; Prognosis ; Recurrence ; Retrospective Studies ; Vulva

PURPOSE: There are only a few reports of extramammary Paget disease (EMPD) of the external genitalia because it is a rare malignancy. We investigated patients with EMPD of the penis and scrotum and report the outcome of surgical management. MATERIALS AND METHODS: From 2000 to 2012, a total of 19 patients diagnosed as having penile and scrotal EMPD underwent wide local excision with or without intraoperative frozen biopsy or preoperative mapping biopsy. The medical charts of these patients were reviewed and analyzed retrospectively. Mean follow-up was 22.5 months (range, 1 to 60 months). RESULTS: The mean age of the patients was 68 years (range, 57 to 82 years). In some patients, the lesions were misdiagnosed as either eczema or some other benign skin lesion at outside institutions, with a mean delay in diagnosis of 43.5 months (range, 1 to 198 months). Intraoperative frozen biopsy or preoperative mapping biopsy was performed in 18 patients. The resection margin was negative in 9 patients (47.4%) and positive in 10 patients (57.6%). Intraepithelial EMPD without dermis invasion was observed in 5 patients (26.3%), whereas diseases with dermis invasion were noted in 14 patients (73.7%). During the follow-up period, recurrences occurred in four patients, and two patients with dermis invasion and recurrence died from the disease. CONCLUSIONS: Diagnosis of EMPD should not be delayed to allow for prompt management. Our findings suggest that intraoperative frozen biopsy or preoperative mapping biopsy cannot guarantee negative margins on final pathology. However, preoperative mapping biopsy and wide local excision with intraoperative frozen biopsy demonstrates good prognosis of EMPD, especially in those cases without dermal invasion.
Biopsy ; Dermis ; Diagnosis ; Eczema ; Follow-Up Studies* ; Genitalia* ; Humans ; Male ; Paget Disease, Extramammary* ; Pathology ; Penis ; Prognosis ; Recurrence ; Retrospective Studies ; Scrotum ; Skin

Carcinoma in Situ ; pathology ; virology ; Carcinoma, Squamous Cell ; pathology ; virology ; Diagnosis, Differential ; Female ; Humans ; Paget Disease, Extramammary ; pathology ; virology ; Papillomavirus Infections ; Precancerous Conditions ; pathology ; virology ; Vulvar Neoplasms ; classification ; pathology ; virology ; Warts ; pathology ; virology