RESUMEN
Sarcomas comprise a heterogeneous group of mesenchymal neoplasms. They can be grouped into 3 general categories, soft tissue sarcoma, visceral and primary bone sarcoma, which have different staging and treatment approaches. Soft tissue sarcomas are typically classified on the basis of genetic alterations and light-microscopic examination of hematoxylin-eosin-stained tissue, in which recognizable morphological characteristics of normal tissues are identified. Sarcomas are further characterized by histologic grade. The 3 most important prognostic variables are grade, size, and location of the primary tumor. This review includes a discussion of both soft tissue sarcomas (unclassified sarcoma, liposarcoma, leiomyosarcoma, synovial sarcoma, dermatofibrosarcoma protuberans, angiosarcoma, Kaposi sarcoma, gastrointestinal stromal tumor, rhabdomyosarcoma, ...) and primary bone sarcomas (osteosarcoma, Ewing sarcoma and chondrosarcoma). The approach to a patient with a sarcoma begins with a biopsy that obtains adequate tissue for diagnosis without interfering with subsequent optimal definitive surgery. Subsequent treatment depends on the specific type of sarcoma. Due to the absence of clear knowledge for incidence rate, we conducted in 2005 and 2006 an exhaustive analysis of all diagnosed cases in the Rhône-Alpes region. Because sarcomas are relatively uncommon yet comprise a wide variety of different entities, second opinion was systematically performed for all included cases.
Asunto(s)
Neoplasias Óseas/epidemiología , Sarcoma/epidemiología , Neoplasias de los Tejidos Blandos/epidemiología , Adulto , Neoplasias Óseas/genética , Neoplasias Óseas/patología , Niño , Femenino , Francia/epidemiología , Humanos , Incidencia , Masculino , Factores de Riesgo , Sarcoma/genética , Sarcoma/patología , Neoplasias de los Tejidos Blandos/genética , Neoplasias de los Tejidos Blandos/patologíaRESUMEN
AIMS: Collagenous spherulosis (CS) is a rare lesion which is an incidental finding in breast and salivary glands. It is characterized by fibrillar spherules exhibiting an intrinsic radiating or concentric pattern which are surrounded by myoepithelial cells. This entity can be misdiagnosed as adenoid cystic carcinoma and in-situ ductal carcinoma. METHODS AND RESULTS: We report here the first case of CS arising in a borderline endometrioid tumour of the ovary where it merged with squamous metaplasia. CONCLUSION: This observation illustrates another pitfall of CS which can be misidentified as keratin pearls. The pathogenesis remains unclear but it has been claimed that the accumulation of basement membrane material may be due to the proliferation of pre-existing myoepithelial cells that secrete matrix components. Since ovarian tumours do not contain myoepithelial cells, one should assume that the epithelial cells differentiate towards myoepithelial cells as it has been shown in vitro and ex vivo.
Asunto(s)
Carcinoma Endometrioide/patología , Colágeno/metabolismo , Queratinas/metabolismo , Neoplasias Ováricas/patología , Ovario/patología , Adulto , Carcinoma Endometrioide/metabolismo , Diagnóstico Diferencial , Errores Diagnósticos , Matriz Extracelular/metabolismo , Femenino , Humanos , Inmunohistoquímica , Metaplasia/metabolismo , Metaplasia/patología , Quistes Ováricos/metabolismo , Quistes Ováricos/patología , Neoplasias Ováricas/metabolismo , Ovario/metabolismoRESUMEN
The Peutz-Jeghers syndrome includes periorificial melanin spots and digestive adenoma, transmitted according to an autosomal dominant mode. We report on a case discovered in a 13 year-old girl presenting with anaemia.