A propósito de un caso; paciente joven con tumor desmoide de mama diagnosticado tras tumorectomía / Desmoid breast tumor diagnosed after lumpectomy in a young patient a case report

Presentamos el caso de una paciente de 16 años, sin antecedentes ginecológicos de interés, con previo diagnóstico de tumor filoides en línea intercuadrántica de mama derecha. Tras ser tratada con tumorectomía, y tras el estudio anatomopatológico de la pieza, resultó estar afecta de tumor desmoide en mama. Dada la escasa prevalencia de esta afección, y la afectación de los bordes quirúrgicos tras el tratamiento quirúrgico, consideramos necesario elaborar una revisión bibliográfica sobre el tumor desmoide en mama; etiología, clínica y tratamiento (AU)

We report the case of a 16-year-old patient with no relevant gynecologic history, who was diagnosed with a phyllodes tumor in the border between the lower and upper outer quadrants of the right breast. The patient was treated with a tumorectomy/lumpectomy, and after pathological study of the specimen, was diagnosed with a desmoid breast tumor. Given the rarity of this entity, and the positive microscopic surgical margins, we consider that a literature review of desmoid breast tumor (etiology, clinical presentation and treatment) is timely (AU)

Reporte de un caso de tumor desmoplásico de células pequeñas redondas / Case report: patient with round cell desmoplastic tumor

Introducción: El tumor de células pequeñas redondas desmoplásico (TCPRD), es una neoplasia agresiva maligna poco frecuente que afecta a adolescentes y adultos jóvenes. Usualmente son intraabdominales asociados con un mal pronóstico. Existen algunas publicaciones donde incluyen manifestaciones paratesticulares. Caso: Reportamos un caso de un joven de 29 años que consulta por aumento de volumen del hemiescroto izquierdo de 3 meses de evolución. Al examen destacaba 2 nódulos duros uno en relación al polo inferior y el otro al epidídimo de aproximadamente 2 cm. Alfafetoproteína y beta HCG eran normales. Se decide exploración quirúrgica donde biopsia rápida confirma tumor que se origina en las envolturas y que infiltra hacia el testículo. La biopsia definitiva informó tumor desmoplásico de células pequeñas redondas. El estudio de extensión con tomografía axial computarizada y radiografía de tórax no mostró metástasis. El paciente recibió 2 ciclos de QMT con ciclofosfamida, etoposido, adriamicina y cisplatino, con buena tolerancia, evolucionando con depresión medular moderada que se recupera. Actualmente 6 años después del diagnóstico el paciente se encuentra en remisión completa. Discusión: Al parecer y según los últimos reportes de la literatura, la ubicación paratesticular ha mostrado mejor pronóstico en comparación con los tumores abdominales. Se debe incluir este diagnóstico diferencial al enfrentarse con tumores paratesticulares.

Introduction: Small round cell desmoplastic tumor (SRCDT) is an infrequent malignant tumor that affects adolescents and young adults. Usually they occur in the abdomen. Paratesticular manifestations have been reported. Case report: A 29 year old male presented with a 3 month history of a mass in the left scrotum. Physical exam showed 2 hard nodules in the scrotum. Serum levels of alpha-fetoprotein and beta-HCG were normal. The patient was submitted to surgery. Frozen section confirmed a tumor arising in the paratesticular area with involvement of the testis. Permanent sections showed a SRCDT. CT scans and chest x-rays showed no metastases. The patient received two courses of ciclophosphamide, etoposide, adryamicin and cisplatinum. Treatment was well tolerated. The patient is in complete remission at 6 years following the diagnosis. Discussion: Paratesticular location seems to have a better prognosis compared to intraabdominal tumors. SRCDT should be included in the differential diagnosis of paratesticular tumors.

[Desmoplastic small round-cell tumor of the paratesticular region: a case report and review of the literature].

OBJECTIVE: To investigate the clinical and pathological features of paratesticular desmoplastic small round cell tumor (DSRCT), and to improve the diagnosis and treatment of the disease. METHODS: One case of paratesticular DSRCT was studied retrospectively and a considerable amount of related literature from Medline and Chinese journals reviewed. The patient was a 27-year-old man presenting with a painless testicular mass in the left hemiscrotum. On physical examination, a cystic mass was palpable while the testis was not in the left hemiscrotum. RESULTS: During the operation the paratesticular area was found full of multiple nodular tumor masses of various sizes ranging from 0.5 cm to 1.5 cm in diameter. Pathological examination showed the characteristic histological pattern of nests of small undifferentiated cells embedded in a dense fibrous stroma. The tumor presented an immunohistochemical feature of epithelial, mesenchymal as well as neural multidirectional differentiation. Following testicular tumor orchiectomy, chemotherapy was performed with DDP, VP16, ifosfamide and EPI. Three years follow-up found no tumor recurrence. CONCLUSION: Desmoplastic small round cell tumor has a specific clinicopathologic stigmata, usually occurring in young males, for which surgical resection with chemotherapy is the treatment of choice. DSRCT located in the paratesticular region may have a better prognosis than its more frequently abdominal counterpart.

Tumor desmoplásico de células redondas pequeñas: presentación de dos casos y revisión de la bibliografía / Desmoplastic small round cell tumor: a report of two cases and a review of the literature

El tumor desmoplásico de células redondas pequeñas (TDCRP) es una neoplasia maligna que se presenta típicamente como una masa abdominopélvica en un paciente varón durante la segunda o tercera década de la vida. Presentamos dos casos de esta rara patología, describiendo los hallazgos en la tomografía computarizada (TC) y en la anatomía patológica. Realizamos una breve revisión de la bibliografía, haciendo hincapié en las manifestaciones radiológicas de dicha neoplasia (AU)