Myofibroblastoma of the male breast: A case report / Miofibroblastoma en la mama de un varón, descripción de un caso clínico

Myofibroblastoma (MFB) is a rare spindle stromal tumour of the breast that predominates in 60–80 years-old adult males. Its imaging features are nonspecific, leading to misdiagnosis. Thus, core biopsy is needed for definitive diagnosis. Macroscopically, MFB is usually a well-circumscribed, firm and rubbery, unencapsulated, pale white to grey round mass. Microscopically, it consists on spindle cells arranged in haphazardly intersecting fascicles or clusters, thick hyalinized collagen bundles and low mitotic activity with a lack of myoepithelial component and necrosis. Immunohistochemistry shows consistently positive immunoreactivity to vimentin and CD34, while expression of desmin, SMA, bcl-2 and CD99 varies. Oestrogen, progesterone and androgen receptors are usually expressed. They are constantly negative to cytokeratins, EMA, S100 protein, HMB-45 and c-kit (CD117). These differentiate them from fibroadenoma, phyllodes tumour, round pattern gynecomastia, carcinoma and sarcoma, since they present infiltrative growth and are negative to CD34. Wide local excision is curative, with no need of sentinel lymph node biopsy, since local recurrence is extremely low and has been reported to be less than 1.5%. No distant metastases have been described on the literature. We report a rare case MFB on a 73-year-old male attended at our institution presenting with a nodule on the right breast. (AU)

El miofibroblastoma (MFB) es un tumour estromal de células fusiformes que aparece en varones de 60-80 años. Las características radiológicas son inespecíficas, por lo que es necesaria la realización de biopsia para el diagnóstico definitivo. Macroscópicamente se trata de una lesión bien circunscrita, firme, no encapsulada. Microscópicamente consiste en células fusiformes organizadas en fascículos entremezclados con bandas de colágeno hialino, con baja actividad mitótica y ausencia de componente mioepitelial y necrosis. La inmunohistoquímica muestra la expresión constante de vimentina y CD34, con expresión variable de desmina, AML, bcl-2 y CD99. Los receptores de estrógenos, progesterona y andrógenos normalmente son positivos, mientras que la expresión de citoqueratinas, EMA, S100, HMB-45 y c-kit (CD117) es negativa. Estas características lo diferencian del fibroadenoma, tumour filodes, ginecomastia, carcinoma y sarcoma, ya que la mayoría de ellos se caracterizan por ser negativos para CD34 y presentar crecimiento infiltrativo. La tumorectomía es considerada curativa, sin necesidad de realizar biopsia selectiva de ganglio centinela, dado que la recurrencia local es baja (menos del 1,5%). No se ha descrito la presencia de metástasis a distancia en la literatura. Presentamos el caso de MFB en un varón de 73 años que debutó con un nódulo en la mama derecha. (AU)

Impacto de la resección no planificada y la reintervención de sarcomas de partes blandas en su pronóstico / Impact of unplanned resection and re-excision of a soft tissue sarcoma on prognosis

INTRODUCCIÓN: Hasta un 40% de los sarcomas de partes blandas (SPB) son resecados de forma no planificada, dejando tumor residual en más del 50% de los casos. La implicación pronóstica de estas resecciones no está claramente definida, dado que existen escasos estudios comparativos que demuestren cómo afecta a la tasa de recurrencia local, de metástasis y de supervivencia. MÉTODOS: Revisión retrospectiva de pacientes intervenidos de un SPB de enero de 2000 a enero de 2016 clasificándolos respecto a intervención planificada o no planificada. Se compararon las tasas de recurrencia y metástasis en global y por estadios. RESULTADOS: Ceintitrés pacientes con SPB fueron tratados de forma planificada y 16 de forma no planificada, con 13 reintervenciones. El 40% del grupo planificado presentó un estadio avanzado respecto al 20% del grupo no planificado. El 77% de los pacientes con resección no planificada reintervenidos presentaron tumor residual en la pieza. La tasa de recidiva local en el grupo de no planificados fue considerablemente más alta (73,5% frente al 43,8%). La tasa de metástasis en no planificados fue del 45,5%, frente al 56,3% en planificados (p > 0,05). En el grupo de no planificados el patrón de recidiva fue más errático con peores resultados en estadios precoces. Concusiones: La resección no planificada de los SPB asocia mayores tasas de recurrencia local y peores resultados funcionales a pesar del manejo oncológico posterior. En las lesiones de partes blandas es fundamental reconocer los signos de alarma que sugieren malignidad para llevar a cabo un estudio diagnóstico específico y evitar resecciones inadecuadas

INTRODUCTION: Up to 40% of all initial operations for soft tissue sarcoma (STS) are unplanned, which would leave residual macroscopic tumor in more than 50% of the cases. The effect this has on local recurrence rate, metastases rate and survival has never been fully established, due to the lack of randomized studies. METHODS: Retrospective review of patients with STS treated in our unit between January 2001-January 2016. We classified them whether they had been treated by initial planned or unplanned operation. Outcomes were compared in both groups globally and stage-matched. Endpoints were local recurrence and distant metastases. RESULTS: Twenty-three patients of STS underwent a planned excision and 16 an unplanned excision, 13 of them underwent further re-excision.40% of patients with planned excision had an advanced stage in regard to the unplanned excision group which presented earlier stages.77% of patients with unplanned excision had residual tumor identified after surgical re-excision. Local recurrence rate in the unplanned excision group was considerably higher 73,5% vs.43,8%. Metastases rate was lower in planned excision group, 45,5% vs 56,3% (P > .05). The recurrence pattern in the unplanned excision group was unstable, with worse outcomes in earlier stages. CONCLUSION: The unplanned excision of a soft tissue sarcoma may compromise disease local control, with higher rates of local recurrence and metastases, and worse functional out- comes, despite further oncological treatment. We need to recognize the clinical features for malignancy risk in soft tissue lumps for a safe diagnosis to avoid inadequate resections

Impact of unplanned resection and re-excision of a soft tissue sarcoma on prognosis. / Impacto de la resección no planificada y la reintervención de sarcomas de partes blandas en su pronóstico.

INTRODUCTION: Up to 40% of all initial operations for soft tissue sarcoma (STS) are unplanned, which would leave residual macroscopic tumor in more than 50% of the cases. The effect this has on local recurrence rate, metastases rate and survival has never been fully established, due to the lack of randomized studies. METHODS: Retrospective review of patients with STS treated in our unit between January 2001-January 2016. We classified them whether they had been treated by initial planned or unplanned operation. Outcomes were compared in both groups globally and stage-matched. Endpoints were local recurrence and distant metastases. RESULTS: Twenty-three patients of STS underwent a planned excision and 16 an unplanned excision, 13 of them underwent further re-excision. 40% of patients with planned excision had an advanced stage in regard to the unplanned excision group which presented earlier stages. 77% of patients with unplanned excision had residual tumor identified after surgical re-excision. Local recurrence rate in the unplanned excision group was considerably higher 73,5% vs. 43,8%. Metastases rate was lower in planned excision group, 45,5% vs 56,3% (P > .05). The recurrence pattern in the unplanned excision group was unstable, with worse outcomes in earlier stages. CONCLUSION: The unplanned excision of a soft tissue sarcoma may compromise disease local control, with higher rates of local recurrence and metastases, and worse functional out- comes, despite further oncological treatment. We need to recognize the clinical features for malignancy risk in soft tissue lumps for a safe diagnosis to avoid inadequate resections.