Sarcoma de mama. Experiencia en el Instituto Oncológico Ángel H. Roffo (ioar) / Breast Sarcoma. Experience at the Angel H. Roffo Institute (ioar)

Introducción Los sarcomas de mama son una entidad oncológica poco frecuente y heterogénea, por lo cual la literatura se limita a pequeñas revisiones retrospectivas e informes de casos. Por tal motivo, no hay consenso sobre su diagnóstico, tratamiento y seguimiento. Objetivo Sobre la base de un trabajo retrospectivo de la experiencia en el Instituto Ángel H. Roffo en el período comprendido entre 2000 y 2018, el estudio se propuso: a) analizar las propiedades de la enfermedad en cada paciente; b) evaluar el seguimiento, la sobrevida y las características de la población en estudio, acrecentando la interpretación de una entidad poco frecuente. Material y método Se trata de un trabajo retrospectivo, descriptivo, observacional que abarca los últimos 18 años de experiencia en esta patología en el Instituto Oncológico Ángel H. Roffo. Resultados Fueron diagnosticados y tratados 20 casos: 19 mujeres y 1 hombre. La incidencia fue de 0,36% sobre un total de 5.442 pacientes ingresados. La media de edad al diagnóstico fue de 50,5 años y la mediana de 49,3 años. Al separar los casos en intervalos de cada 2 décadas, se encontró que 6 pacientes tenían entre 20-30 años, 9 entre 40-59 años y 5 más de 60 años. Según el subtipo histológico, el orden de frecuencia fue el siguiente: 10 Sarcomas Fusocelulares; 2 Angiosarcomas; 1 Mixofibrosarcoma; 1 Cistosarcoma; 1 Liposarcoma Pleomorfo; 1 Dermatofibrosarcoma Protuberans; 1 Osteosarcoma. En tres casos no se obtuvieron datos por pérdida de historia clínica. La sobrevida libre de enfermedad y la sobrevida global se analizaron utilizando las curvas de Kaplan-Meier, siendo respectivamente de 152 y 260 meses. Conclusiones En conclusión, determinamos que el manejo multidisciplinario de la enfermedad en estadios iniciales ha demostrado una buena sobrevida global en la población evaluada

Introduction Breast sarcomas are a rare and heterogeneous oncological entity, so the literature is limited to small retrospective reviews and case reports. For this reason there is no consensus on its diagnosis, treatment and follow-up. Objective Based on a retrospective work of the experience at the Angel H. Roffo Institute in the period between 2000 and 2018, the study proposed: a) analyze the properties of the patient's disease; b) evaluate the monitoring, survival and characteristics of the study population, increasing the interpretation of a rare entity. Materials and method It is a retrospective, descriptive, observational work that covers the last 18 years of experience on this pathology at the Angel H. Roffo Institute. Results A total of 20 cases were diagnosed and treated: 19 women and 1 man. The incidence was 0,36% out of a total of 5,442 patients admitted. The mean age at diagnosis was 50.5 years and the median was 49.3 years. When we separate the cases every 2 decades, it was found that 6 patients are between 20-30 years old, 9 between 40-59 years old and 5 were more than 60 years old. According to the histological subtype, the frequency order was the follow: 10 Fusocelullar Sarcomas; 2 Angiosarcomas; 1 Mixofibrosarcoma; 1 Cystosarcoma; 1 Liposarcoma Pleomorfo; 1 Dermatofibrosarcoma Protuberans; 1 Osteosarcoma. In 3 cases no data were obtained due to loss of clinical history. Disease-free survival and overall survival were analyzed using the Kaplan-Meier curves, respectively 152 and 260 months. Conclusions In conclusion, we determined that the multidisciplinary management of the disease, in initial stages, has demonstrated a good overall survival in the evaluated population

Osteosarcoma primario de mama en una paciente con antecedentes de tratamiento radioterápico loco-regional por carcinoma in situ mamario / Primary breast osteosarcoma in a patient with a history of locoregional radiotherapy treatment for breast carcinoma in situ

RESUMEN El cáncer de mama es el tumor más prevalente a nivel mundial entre las mujeres. A pesar de que la supervivencia global alcanza cifras cercanas al 90%, sigue suponiendo un importante problema sanitario y económico para la población. Los sarcomas primarios de mama suponen menos del 1% de estas neoplasias, pero sus tasas de recidiva y mortalidad son elevadas. Dado que no existen pruebas de imagen específicas para el diagnóstico, la confirmación de dicha entidad supone un reto a nivel histopatológico. El tratamiento del osteosarcoma de mama es principalmente quirúrgico, con la extirpación de la tumoración con márgenes adecuados, que será trascendental para el pronóstico de las pacientes. No se ha demostrado que sea necesaria una disección axilar, ni un tratamiento adyuvante estándar de quimioterapia ni radioterapia, salvo casos indicados. Presentamos el caso de una paciente postmenopaúsica que tras haber desarrollado un carcinoma ductal in situ de mama tratado de forma satisfactoria, desarrolla dos años posterior a la finalización del tratamiento radioterápico, y en la misma localización del tumor inicial, un osteosarcoma primario de mama de rápido crecimiento atribuible al efecto de la radioterapia administrada.

ABSTRACT Breast cancer is the most prevalent tumor worldwide among women. Despite the fact that overall survival reaches figures close to 90%, it continues to be an important health and economic problem for the population. Primary breast sarcomas account for less than 1% of these neoplasms but their rates of recurrence and mortality are high. Given that there are no specific imaging tests for diagnosis, confirmation of this entity is a challenge at the histopathological level. The treatment of the OSM is mainly surgical with the removal of the tumor with adequate margins, which will be transcendental for the prognosis of the patients. It has not been demonstrated that an axillary dissection is required, nor a standard adjuvant treatment of chemotherapy or radiotherapy, unless indicated. We present the case of a postmenopausal patient who, after having developed a ductal carcinoma in situ of the breast (DCIS) successfully treated, developed two years after the end of the radiotherapy treatment, and in the same location of the initial tumor, an osteosarcoma primary breast of rapid growth attributable to the effect of radiotherapy administered.

Fibrohistiocitoma maligno mamario: descripción de un caso

El fibrohistiocitoma maligno de mama es una entidad muy poco frecuente que debuta como un nódulo mamario de reciente aparición y de crecimiento relativamente rápido. El diagnóstico debe establecerse mediante una muestra histológica, debido a que las pruebas de imagen suelen ser inespecíficas. El tratamiento habitual es la mastectomía con o sin vaciamiento axilar y la terapia adyuvante (quimioterapia y radioterapia) suele tener poca efectividad en esta patología.

Malignant fibrous histiocytoma of the breast is arare entity that debuts as a breast lump of recent onset and relatively fast growth. Diagnosis is by histology because imaging tests are often nonspecific. Usual treatment is mastectomywith orwithout axillary dissection; acjuvant therapy (chemotherapy and radiotherapy) often have limited effectiveness in this pathology.

Primary osteogenic sarcoma of the breast.

Primary osteogenic sarcomas of the breast are extremely rare with less than 100 cases reported in literature. They generally present in middle aged and older women and are highly aggressive. These tumors need to be differentiated from osteoid/bone- producing metaplastic carcinomas as the two differ in their biologic behavior and treatment protocols. A 60-year-old lady presented with a short history of a rapidly enlarging breast lump. Histopathogical examination revealed a richly cellular, pleomorphic tumor with abundant osteoid. The tumor cells were immunoreactive for vimentin and negative for cytokeratin and estrogen and progesterone receptors.

Sarcoma primário de mama gigante em adolescente de 13 anos / Giant primary breast sarcoma in a 13-year-old adolescent

Os sarcomas primários da mama (SPM) são entidades raras. A sua incidência anual pode ser estimada em 44,8 novos casos por 10 milhões de mulheres, sendo mais comum entre a quarta e a quinta década de vida e incomum em pacientes jovens. O prognóstico depende do tamanho da tumoração, tipo histológico e grau de atividade mitótica. Os autores apresentam e discutem o caso de uma adolescente de 13 anos com história de surgimento de nódulo endurecido e doloroso em mama direita, associado a eritema local há quatro meses da admissão. Ao exame físico admissional, apresentava volumosa tumoração em mama direita de aproximadamente 25 cm em seu maior diâmetro. A investigação diagnóstica pré-operatória incluiu raio X de tórax, ultra-sonografia de mama e biópsia incisional cujo resultado foi tumor malígno de origem mesenquimal com muitas áreas de necrose. A paciente submeteu-se a mastectomia toral com inclusão do músculo peitoral maior e reconstrução com rotação de retalho de pele mais enxertia. A evolução pós-operatória foi satisfatória e o exame anatomopatológico evidenciou diagnóstico de sarcoma de mama. A imunoistoquímica mostrou ser um fibrossarcoma de alto grau. A mastectomia total sem dissecção axilar é o tratamento padrão-ouro para SPM, podendo se realizar ressecção local alargada em casos selecionados. A radioterapia e a quimioterapia adjuvante têm se mostrado útil em sarcomas volumosos e de alto grau.

The primary breast sarcomas are an unusual pathology. Its annual incidence may be estimated in 44.8 new cases per 10 million women, been more common between 40-50 years old and very uncommon in young patients. The prognosis is based on tumor size, histopathology and mitotic count.The authors present and discuss the case of a 13th years old adolescent with a hard and painful nodule in the right breast, associated with erythema four months before the entrance. The physical entrance examination pointered a giant tumor in the right breast measuring about 25 cm in its major diameter. The preoperative diagnostic investigation included chest x-ray, ultrasonography of breast and biopsy of lesion witch pointed a malignant tumor full of necrosis coming from mesenquina. The patient was submitted to a total mastectomy including major peitoral muscle muscle regarding a skin reconstruction with flap and skin graft. Post operative evolution was satisfactory and histopathology diagnosed breast sarcoma. Imunohistochemical was classified in a higher-grade fibrosarcoma. The total mastectomy without axillary lymph node dissection is the first line treatment to primary breast sarcoma, but local resection may to be use selective cases. Radiation therapy and adjuvant chemotherapy have showed to be useful in large tumours with high mitotic rate.