ABSTRACT
Background/Objective: Extramammary Paget’s Disease (EMPD) seems to be more common in Caucasians than Chinese. We report the clinical manifestations, management, and prognostic characteristics in 17 Chinese patients. Methods: Medical records and biopsies of 17 patients who had been treated at a large university hospital in China between March 2005 and January 2012 were reviewed. Results: Of the 17 patients, 14 were men. They had lesions on the scrotum and the penis. Of the three women, two had vulvar and one had inguinal lesions. All patients underwent Mohs micrographic surgery (MMS). Three men had metastasis to the inguinal lymph nodes and underwent an extensive local excision with inguinal lymphadenectomy. Eight patients who had positive excision margins received additional radiation therapy. The mean follow-up duration was 54 months (4-85 months). One patient had two recurrences. Three had metastasis to the inguinal lymph node. One had metastasis to the bone and concomitant prostate cancer. Two patients died of the disease. Conclusion: A striking difference in presentation of EMPD in Chinese compared with Caucasians is the male predominance and location on the penis and scrotum. Mohs micrographic surgery followed by radiotherapy is an effective treatment. Long-term follow-up suggests that the disease has a good prognosis when it does not metastasise.
Subject(s)
Adult , Aged , Asian People , Combined Modality Therapy , Female , Follow-Up Studies , Genital Neoplasms, Female/pathology , Genital Neoplasms, Female/radiotherapy , Genital Neoplasms, Female/surgery , Genital Neoplasms, Male/pathology , Genital Neoplasms, Male/radiotherapy , Genital Neoplasms, Male/surgery , Humans , Lymph Node Excision , Male , Middle Aged , Mohs Surgery , Paget Disease, Extramammary/pathology , Paget Disease, Extramammary/radiotherapy , Paget Disease, Extramammary/surgery , Penis/pathology , Penis/surgery , Prognosis , Scrotum/pathology , Scrotum/surgery , Treatment Outcome , Vulva/pathology , Vulva/surgerySubject(s)
Adult , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Aminoquinolines/therapeutic use , Antineoplastic Agents/therapeutic use , Paget Disease, Extramammary/therapy , Paget's Disease, Mammary/therapy , Prostatic Neoplasms/therapy , Vulvar Neoplasms/therapy , Paget Disease, Extramammary/radiotherapy , Paget's Disease, Mammary/radiotherapy , Prostatic Neoplasms/radiotherapy , Vulvar Neoplasms/radiotherapyABSTRACT
Se presenta dos pacientes con enfermedad de Paget de la vulva con múltiples recurrencias. La evaluación histológica confirmó el diagnóstico y descartó la posibilidad de adenocarcinoma subyacente. Después de varias resecciones quirúrgicas, ambas pacientes fueron tratadas con radioterapia. Los resultados estéticos así como también la tolerancia y toxicidad durante y después del tratamiento, fueron aceptables. Ambos casos han completado al menos 1 año de seguimiento sin recurrencia clínica demostrable por vulvoscopia
Subject(s)
Humans , Female , Aged , Middle Aged , Paget Disease, Extramammary/pathology , Vulvar Neoplasms/pathology , Paget Disease, Extramammary/radiotherapy , RecurrenceABSTRACT
Presentamos un caso de Paget perianal como contribución a la casuística, actualización de la patología y discusión de los controvertidos tratamientos. Se trató un paciente de sexo masculino y de 60 años, con lesión de 10 años de evolución, eritematosa, con erosiones, muy pruriginosa, de 12 cm de diámetro, que involucra circunferencialmente la piel perianal. No se halló tumor maligno asociado, cuya frecuencia oscila entre el 50 y el 86 por ciento. Se realizó, por negativa del paciente a la cirugía, terapia radiante con Co 60,3500 rads. Se obtuvo mejoría sintomática y disminución del tamaño lesional en un 60 por ciento. Se comprobó por biopsia la persistencia de enfermedad microscópica en la piel aparentemente recuperada. Se enfatiza que el tratamiento de la lesión es el quirúrgico, con adecuados márgenes de resección en superficie y profundidad, utilizando biopsias pre e intraoperatorias, para evaluar correctamente la verdadera extensión lesional y evitar las recidivas que se calculan en más de 35 por ciento cuando no se han tomado estos recaudos.