Pigmentación asintomática en la mama / No disponible

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Clinically silent Paget disease of male nipple.

Male breast cancer constitutes less than 1% of all breast cancer cases. Special types, such as Paget disease of the breast, are extremely rare. To our knowledge there are less than 50 cases of Paget disease of male breast described in world medical literature. Authors present pathologically proven but clinically silent case of Paget disease of the nipple in an 86-year old male with breast cancer. Literature data suggest that the prognosis of male Paget cancer is worse than in "ordinary" male breast cancer. Therefore, the histologic diagnosis of Paget disease, even in an asymptomatic male patient, may have negative prognostic value.

Breast-conserving therapy for Paget disease of the nipple: a prospective European Organization for Research and Treatment of Cancer study of 61 patients.

BACKGROUND: The purpose of the current study was to assess the outcome of breast-conserving therapy by means of a cone excision and radiotherapy in patients with Paget disease of the nipple without associated invasive breast carcinoma. METHODS: Between 1987 and 1998, 61 eligible patients were registered in the European Organization for Research and Treatment of Cancer Study 10873. The majority of patients (97%) presented without an associated palpable mass. At histologic examination, the majority (93%%) of patients had an underlying ductal carcinoma in situ (DCIS); in the remaining 7%, only Paget disease was found. Treatment was comprised of a complete excision of the nipple-areolar complex including the underlying breast tissue with tumor free margins, followed by external irradiation to the whole breast (50 gray in 25 fractions). The primary endpoint was local recurrence. RESULTS: At a median follow-up of 6.4 years, 4 of the 61 patients developed a recurrence in the treated breast (1 patient with DCIS and 3 patients with invasive disease). One patient with an invasive local recurrence died of disseminated breast carcinoma. The 5-year local recurrence rate was 5.2% (95% confidence interval, 1.8-14.1%). CONCLUSIONS: Breast-conserving therapy is a feasible alternative for patients with Paget disease and a limited extent of underlying DCIS. To achieve good local control, treatment should be comprised of a complete excision of the nipple-areolar complex including the underlying disease, followed by irradiation to the whole breast.

Enfermedad de Paget extramamaria y mamaria, anaplásica y acantolíca: presentación de 7 casos y revisión de la literatura / Paget disease, extrammamary and mammary, anaplastic, acantholytic: a report of 7 cases and review of the literature

La enfermedad de Paget extramamaria anaplásica y acantolítica (EPeMAA) y la enfermedad de paget mamaria anaplásica y acantolítica (EPMAA), son formas raras de la EP; no se sabe en el momento actual su frecuencia real. Se presentaron por arriba de la cuarta decada, en 4 mujeres y 3 hombres. Su evolución fué crónica, clínicamente fueron placas verrugosas, ulceradas, facilmente sangrantes, bien limitadas. Clínica e histológicamente se confundieron con enfermedad de Bowen y con el pénfigo vegetante. Microscópicamente se observó en todo el espesor de la epidermis células anaplásicas pequeñas, con núcleo ovoide, cromatina fina sin disqueratosis, escaso citoplasma; la acantólisis fué acentuada con formaciones vegetantes. Se observaron células clásicas de Peget en forma alterna; todos exhibieron ulceración y en la periferia la imagen típica de EP. En la base de la neoplásia hubo infiltrado linfocitario en banda "patrón liquenoide". En 4 de los casos se identificó neoplásia maligna sincrónica en la profundidad (1 caso Ca de conductos mamarios, 2 casos Ca de conductos de glándulas apócrina y 1 caso de Ca. de conductos de glándulas ecrina de tipo esclerosante) fueron positivos al antígeno carcinoembrionario (ACE) 5/5, al antígeno de membrana epitelial (AMA) 5/5, y a la citoqueratina de bajo peso molecular (Cqbpm) 5/5. Hay que hacer el diagnóstico diferencial con la enfermedad de Bowen (EB), melanoma de diseminación superficial (MMDS) y con el pénfigo vegete. No hay diferencias en pronóstico entre la EP mamaria y extramamaria clásica y la EPeMAA u EPMAA

Enfermedad de Paget: presentación de 8 casos / Paget's disease: report of 8 cases

Se comunican 8 casos de enfermedad de Paget estudiados en la consulta dermatológica del Hospital General "Dr. Manuel Gea González", de 1980 a 1993. Cinco casos se presentaron en mama y el tiempo de evolución varió de 6 meses a un año. Tres fueron extramamarios de 1 a 6 años de evolución. Clínicamente presentaron placas eritemato-escamosas con eccema y en genitales eritema y descamación con aspecto de candidosis. Cinco pacientes fueron tratados con mastectomia radical y quimioterapia. Un caso extramamario falleció por asociación con otro carcinoma y metástasis del mismo

[Intramammary spread of breast cancer]. / Les disséminations intramammaires des carcinomes du sein

At the conclusion of this study based on a careful histological analysis of 454 mastectomy specimens carried out at the Institut Gustave-Roussy between 1966 and 1971 it appears that 39 per cent of tumours which are neither central nor deep to the nipple have microscopic foci outside the main focus of tumour of which 24 per cent are at the level of the single nipple. The importance of these extensions is bound up with the size and histological type of the adenocarcinoma: pure intraduct and infiltrating lobular forms are accompanied frequently by microscopic intramammary foci at a distance (89% and 72%) the opposite of findings in the case of well differentiated and medullary forms. On the other hand no connection between the Scarff and Bloom grading is apparent except where the nipple only is involved. Finally there is a strong relationship between intramammary dissemination and regional lymph node metastases.