Sarcoma neurogénico de la mama masculina. Presentación de un caso / Male breast neurogenic sarcoma. A case

RESUMEN El cáncer de mama en el sexo masculino es una entidad clínica poco frecuente, tiene una presentación unimodal a los 71 años de edad, generalmente se presenta de manera similar a la forma en que se presenta en el sexo femenino. Su causa es poco conocida. Los sarcomas son tumores de componentes mesenquimatoso que constituyen del 0,2-1 % de todos los tumores de mama, y menos del 5 % del total. El sarcoma neurogénico, a su vez, es un tumor extremadamente raro. Representa del 1-2 % aproximadamente, de los tumores de los nervios periféricos con transformación maligna. Debido a la rareza geográfica e histopatológica de este tipo y mucho más en pacientes masculinos se presentó este caso. Paciente masculino de 57 años de edad, con el diagnóstico de un sarcoma de la mama derecha. Se le realizó una mastectomía radical más quimioterapia y radioterapia adyuvante. Los estudios de inmunohistoquímicos permitieron llegar al diagnóstico de sarcoma neurogénico.

ABSTRACT Breast cancer in men (BCM) is a rare clinical entity that has a unimodal presentation at the age of 71 years, and generally presents in a similar way it presents in the female sex. Its etiology remains almost unknown. Sarcomas are tumors of mesenchymal components representing from 0.2 to 1 % of all the breast tumors and less than 5 % of the total. The neurogenic sarcoma is also an extremely rare tumor. It represents around 1-2 % of the peripheral nerves tumors with malignant transformations. Due to location and histopathological rarity of this kind of tumors, much more in male patients, the authors presented the case of a male patient, aged 57 years, with the diagnosis of a left breast sarcoma. He undergone a radical mastectomy plus adjuvant chemotherapy and radiotherapy. The immunohystochemical studies allowed arriving to the diagnosis of neurogenic sarcoma.

Características del cáncer de mama masculino en una población uruguaya / Characteristics of male breast cancer in a Uruguayan population / Características do câncer de mama masculino em uma população uruguaia

El cáncer de mama (CM) en el hombre (CMM) es una entidad poco frecuente. Si bien tiene algunas semejanzas con el CM femenino, es una patología con un perfil propio. El objetivo del estudio consiste en conocer las características del CMM y su manejo en nuestro medio. Materiales y métodos: estudio retrospectivo que incluyó pacientes diagnosticados de CMM en tres centros del Uruguay en un período de 15 años. Resultados: se incluyeron 12 pacientes; la mediana de edad fue 68 años; un tercio de ellos tenían antecedentes familiares (AF). Características clínico patológicas: todos los tumores fueron carcinomas ductales con grado histológico (GH) 2-3, » se hallaban en estadio (E) I, la mitad (6) en EII, 6 tuvieron metástasis axilares, 2/3 fueron receptores de estrógeno (RE) / receptores de progesterona (RP) +. Se definieron 3 subtipos biológicos: I) HER2- RE/RP+: 2/3 de los pacientes; II) HER2+: 1/6; y III) triple negativo: 1/6. Todos los pacientes con enfermedad localizada fueron sometidos a mastectomía y la mayoría recibió tratamiento con quimioterapia (QT). La totalidad de quienes presentaron enfermedad localizada RE/RP+ recibieron hormonoterapia adyuvante con buena adherencia y tolerancia. Dos de los once pacientes tratados con criterio radical recayeron en la evolución; el resto permanece en controles o tratamiento sin evidencia de recaída. Conclusión: presentamos una serie de pacientes con CMM, con un perfil similar al reportado en la literatura. La edad de presentación fue superior a la del CM femenino, y la mayoría fueron RE/RP +, HER 2-; sin embargo, en esta serie los pacientes se presentaron en estadio localizado y con tumores de alto grado en una proporción mayor a lo descrito en la literatura.

Breast cancer (BC) in men (MBC) is an uncommon clinical entity. Even though it shares some similarities with female BC, it has a distinctive profile. The objective of this study was to identify the characteristics of MBC and learn how it is managed in our setting. Materials and methods: retrospective study including patients diagnosed with MBC in three centers in Uruguay for a period of 15 years. Results: 12 patients were enrolled; the median age was 68 years; a third of whom had a family history (FH). Clinical and pathological characteristics: all of the tumors were ductal carcinomas of histological grade (HG) 2-3, 1/4 were stage (S) I, half (6) were SII, 6 had axillary metastases, 2/3 were estrogen receptor (ER) / progesterone receptor (PR) +. Three biological subtypes were defined: I) HER2- ER/PR+: 2/3 of patients; II) HER2+: 1/6; and III) triple-negative: 1/6. All patients with localized cancer underwent a mastectomy and most were treated with chemotherapy (CHT). All of those who presented with localized, ER/PR+ cancer received adjuvant hormone therapy, with good adherence and tolerance. Two of the eleven patients treated with radical surgery recurred during evolution; the rest remain in follow-up or treatment without evidence of recurrence. Conclusion: we presented a series of patients with MBC, with a profile similar to the one reported in the literature. Age at presentation was higher than that of female BC, and most were ER/PR +, HER 2-. However, patients in this series presented with breast cancer in the localized stage and high-grade tumors in a higher proportion than is described in the literature.

O câncer de mama (CM) em homens (CMM) é uma entidade clínica incomum. Embora tenha algumas semelhanças com o CM feminino, é uma patologia com o seu próprio perfil. O objetivo deste estudo foi conhecer as características do CMM e seu manejo no nosso meio. Materiais e métodos: estudo retrospectivo com pacientes com diagnóstico de CMM em três centros do Uruguai em um período de 15 anos. Resultados: foram incluídos 12 pacientes; a mediana da idade foi 68 anos; um terço deles tinha antecedentes familiares (AF). Características clínico-patológicas: todos os tumores foram carcinomas ductais de grau histológico (GH) 2-3, 1/4 estavam no estágio (E) I, a metade (6) no EII, 6 apresentaram metástases axilares, 2/3 foram receptores de estrogênio (RE) / receptores de progesterona (RP) +. Foram definidos 3 subtipos biológicos: I) HER2- RE/RP+: 2/3 dos pacientes; II) HER2+: 1/6; e III) triplo-negativo: 1/6. Todos os pacientes com doença localizada foram submetidos a mastectomia e a maioria receberam tratamento com quimioterapia (QT). A totalidade dos que apresentaram doença localizada RE/RP+ recebeu hormonioterapia adjuvante com boa adesão e tolerância. Dois dos onze pacientes tratados com critério radical recaíram na evolução; o resto permanece sob monitoramento ou tratamento sem evidência de recaída. Conclusão: apresentamos uma série de pacientes com CMM, com um perfil semelhante ao descrito na literatura. A idade de apresentação foi maior do que para o CM feminino, e a maioria foram RE/RP +, HER 2-; entretanto, os pacientes desta série apresentaram-se com estágio localizado e tumores de alto grau em uma proporção maior do que a descrita na literatura.

Male breast cancer in Indian patients: Is it the same.

Background: Cancer of the male breast accounts for about 1% of all malignancies in men and 1% of all breast cancers. Poor level of awareness often results in late presentation and delayed diagnosis in our environment. This study was done to analyse the demographic data, management and survival of male breast cancers in Indian subset of patients and compare it with that of western literature. Materials and Methods: A 10 year (2001-2010) retrospective study of all male breast cancers was done. Data regarding the incidence, presentation, histopathology, stage and grade of tumor, management and outcome of patients were analysed. Results: 26 cases of male breast cancer were encountered. This comprised 0.4% of all breast cancers seen in our department during the 10 year period. The ages of patients ranged from 45-75 years with a mean age of 57 years. Family history was present in 4 patients. Clinically, symptoms included self-detected lump in 23 (88.5%) patients, nipple retraction in 12 (46.1%) and pain in 12 (46.1%). All cases were unilateral (16 on right, 10 on left). Disease most commonly involved central quadrant (9 patients). Grade 3 disease was found in 13 patients and Stage 3 disease was most commonly encountered (13 patients). None of our patients received neo adjuvant chemotherapy. 20 (76.9%) patients had modified radical mastectomy and 6 (23.1%) had radical mastectomy. Most of our patients were hormone receptor positive (21 patients). Bilateral orchidectomy, Adjuvant chemotherapy, Adjuvant radiotherapy and Tamoxifen were offered in 3 (11.5%), 16 (61.5%), 17 (65.4%) and 15 (57.7%) patients respectively. Follow up ranged from 1-59 months. Conclusion: Male breast cancer is rare in our centre. Late presentation with advanced disease is a common feature in our environment. Further multiinstitutional, prospective studies are needed for better understanding of management of male breast cancers in Indian subset of patients.

Carcinoma de mama en el hombre: revisión de casos / Breast carcinoma in men: report of cases

El objetivo del presente trabajo es presentar el comportamiento clínico patológico de 8 pacientes del sexo masculino con carcinoma de la mama, atendidos en el Instituto Oncológico "Dr. Luis Razetti" durante los años 2004-2005. El cáncer de mama en hombres representó el 2,33 por ciento, 37,5 por ciento fueron adenocarcinoma ductal infiltrante, 37,5 por ciento estadio IIIA, 87,5 por ciento se trató con mastectomías radicales modificadas, 57,25 por ciento expresan receptores de estrógenos, 28,57 por ciento sobre expresan Her2neu, 37,5 por ciento recibió adyuvancia, 62,5 por ciento con tamoxifeno, el seguimiento promedio fue de 44 meses, 37,5 por ciento de recaídas local y 62,5 por ciento están libres de enfermedad a los 5 años. El cáncer de mama en hombres es muy poco frecuente. El tratamiento primario es la cirugía, siguiendo los mismos lineamientos para la adyuvancia que en las mujeres.

The objective of the present work is analyzing the clinic pathologic status of 8 patients of sex male, attending in the Oncological Institute "Dr. Luis Razetti" during the 2004-2005 years. The breast cancer in male represented the 2.33 percent, in 37.5 percent of them were ductal infiltrante adenocarcinoma, 37.5 percent were state IIIA,87.5 percent were treated with surgery: Modified radical mastectomy, 57.25 percent express estrogen receptors, 28,57 percent over express Her2neu. 37.5 percent received adjuvant, 62.5 percent with tamoxifen, the average of periodic control was 44 month, and 37.5 percent of local recurrence and 62.5 percent of the patients are free of disease to 5 years. The breast cancer in male is less frequent. The primary treatment is surgery, with the same form to the adjuvant as in women.

Systemic chemotherapy and tamoxifen induced regression of choroidal metastasis from a breast carcinoma in a male.

We report a case of a 55-year-old male patient with breast carcinoma, who developed choroidal metastasis. The patient had undergone mastectomy for carcinoma of right breast, five years ago. The patient was advised close follow-up for the left eye, as he was already on tamoxifen therapy (started a month ago) for spinal metastasis. On last follow-up, a year later, the choroidal lesion had completely scarred, with no recurrences. Systemic hormonal therapy like tamoxifen given for the breast primary and other systemic metastases may cause regression of the choroidal metastasis, thereby avoiding ocular radiotherapy. Medline search revealed only one published case of regression of choroidal metastasis from a male breast primary, on tamoxifen therapy.