RESUMEN

Renal cell carcinoma is a term that represents multiple different disease processes, each driven by different genetic alterations, with distinct histology, and biological potential which necessitates divergent management strategies. This review discusses the genetic alterations seen in several forms of hereditary kidney cancer and how that knowledge can dictate when and how to intervene with a focus on the surgical management of these tumors.

Asunto(s)

Carcinoma de Células Renales/genética , Neoplasias Renales/genética , Leiomiomatosis/genética , Síndromes Neoplásicos Hereditarios/genética , Neoplasias Cutáneas/genética , Neoplasias Uterinas/genética , Carcinoma de Células Renales/clasificación , Carcinoma de Células Renales/patología , Carcinoma de Células Renales/cirugía , Humanos , Neoplasias Renales/clasificación , Neoplasias Renales/patología , Neoplasias Renales/cirugía , Leiomiomatosis/clasificación , Leiomiomatosis/patología , Leiomiomatosis/cirugía , Mutación/genética , Síndromes Neoplásicos Hereditarios/clasificación , Síndromes Neoplásicos Hereditarios/patología , Síndromes Neoplásicos Hereditarios/cirugía , Neoplasias Cutáneas/clasificación , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/cirugía , Succinato Deshidrogenasa/genética , Proteínas Supresoras de Tumor/genética , Ubiquitina Tiolesterasa/genética , Neoplasias Uterinas/clasificación , Neoplasias Uterinas/patología , Neoplasias Uterinas/cirugía , Proteína Supresora de Tumores del Síndrome de Von Hippel-Lindau/genética

RESUMEN

UNLABELLED: The fourth edition of the World Health Organization (WHO) classification of urogenital tumours (WHO "blue book"), published in 2016, contains significant revisions. These revisions were performed after consideration by a large international group of pathologists with special expertise in this area. A subgroup of these persons met at the WHO Consensus Conference in Zurich, Switzerland, in 2015 to finalize the revisions. This review summarizes the most significant differences between the newly published classification and the prior version for renal, penile, and testicular tumours. Newly recognized epithelial renal tumours are hereditary leiomyomatosis and renal cell carcinoma (RCC) syndrome-associated RCC, succinate dehydrogenase-deficient RCC, tubulocystic RCC, acquired cystic disease-associated RCC, and clear cell papillary RCC. The WHO/International Society of Urological Pathology renal tumour grading system was recommended, and the definition of renal papillary adenoma was modified. The new WHO classification of penile squamous cell carcinomas is based on the presence of human papillomavirus and defines histologic subtypes accordingly. Germ cell neoplasia in situ (GCNIS) of the testis is the WHO-recommended term for precursor lesions of invasive germ cell tumours, and testicular germ cell tumours are now separated into two fundamentally different groups: those derived from GCNIS and those unrelated to GCNIS. Spermatocytic seminoma has been designated as a spermatocytic tumour and placed within the group of non-GCNIS-related tumours in the 2016 WHO classification. PATIENT SUMMARY: The 2016 World Health Organization (WHO) classification contains new renal tumour entities. The classification of penile squamous cell carcinomas is based on the presence of human papillomavirus. Germ cell neoplasia in situ of the testis is the WHO-recommended term for precursor lesions of invasive germ cell tumours.

Asunto(s)

Carcinoma de Células Renales/clasificación , Carcinoma de Células Escamosas/clasificación , Neoplasias Renales/clasificación , Neoplasias de Células Germinales y Embrionarias/clasificación , Neoplasias del Pene/clasificación , Neoplasias Testiculares/clasificación , Carcinoma de Células Renales/enzimología , Carcinoma de Células Renales/patología , Carcinoma de Células Escamosas/virología , Humanos , Neoplasias Renales/patología , Leiomiomatosis/clasificación , Leiomiomatosis/patología , Masculino , Clasificación del Tumor , Invasividad Neoplásica , Neoplasias de Células Germinales y Embrionarias/patología , Síndromes Neoplásicos Hereditarios/clasificación , Síndromes Neoplásicos Hereditarios/patología , Infecciones por Papillomavirus/complicaciones , Neoplasias del Pene/virología , Neoplasias Cutáneas/clasificación , Neoplasias Cutáneas/patología , Succinato Deshidrogenasa/deficiencia , Neoplasias Testiculares/patología , Neoplasias Uterinas/clasificación , Neoplasias Uterinas/patología , Organización Mundial de la Salud

RESUMEN

Intravascular leiomyomatosis (IVL) is characterized by the presence of smooth muscle in venous and lymphatic spaces within the myometrium. Although the intravascular component usually consists solely of typical smooth muscle or variants of smooth muscle differentiation, we report 5 cases in which the intravascular component also included endometrioid glandular and stromal elements. We propose the term "intravenous adenomyomatosis" to describe this unusual variant of IVL. The mean age of the patients in this series was 50.2 years, slightly older than that of patients with conventional IVL. In addition to intravenous adenomyomatosis, both adenomyosis and leiomyomas were identified in all of our cases, supporting the hypothesis that the intravascular smooth muscle component in IVL is derived from associated myometrial pathology rather than from vessel walls. In our series, intravenous adenomyomatosis had a similar benign clinical behavior to most cases of IVL with no metastatic or recurrent disease identified at follow-up in 4 cases for which follow-up information was available. The main differential diagnoses are adenomyosis with vascular involvement, low-grade endometrial stromal sarcoma (ESS), including ESS with smooth muscle and glandular differentiation, and adenosarcoma with lymphovascular invasion. The possibility of intravenous adenomyomatosis should be borne in mind when considering these diagnoses, particularly ESS and adenosarcoma, which have different implications for patient management and prognosis.

Asunto(s)

Adenomiosis/patología , Adenosarcoma/patología , Leiomiomatosis/patología , Miometrio/patología , Sarcoma Estromático Endometrial/patología , Neoplasias Uterinas/patología , Venas/patología , Adenomiosis/clasificación , Adenomiosis/metabolismo , Adenomiosis/cirugía , Adenosarcoma/química , Adenosarcoma/clasificación , Adenosarcoma/cirugía , Adulto , Anciano , Biomarcadores de Tumor/análisis , Biopsia , Diagnóstico Diferencial , Femenino , Humanos , Inmunohistoquímica , Leiomiomatosis/química , Leiomiomatosis/clasificación , Leiomiomatosis/cirugía , Persona de Mediana Edad , Miometrio/química , Clasificación del Tumor , Invasividad Neoplásica , Valor Predictivo de las Pruebas , Pronóstico , Sarcoma Estromático Endometrial/química , Sarcoma Estromático Endometrial/clasificación , Sarcoma Estromático Endometrial/cirugía , Terminología como Asunto , Neoplasias Uterinas/química , Neoplasias Uterinas/clasificación , Neoplasias Uterinas/cirugía , Venas/química

RESUMEN

Smooth muscle tumors rather benign or malignant can arise wherever the muscular tissue presents but cutaneous leiomyoma is one of the rare benign tumors of the which even the diagnostic criteria from the malignant type of the tumor is still in doubt. This study was aimed to compare the subtypes of cutaneous leiomyoma from different histologic aspects in order to find unique criteria for better classification and diagnosis. The six year data base of our center was reviewed and 25 patients with cutaneous leiomyoma were included in this study. Of 25 patients, 5 were female and 20 were male. 5 patients had angioleiomyoma (ALM) and 20 had pilar leiomyoma (PLM). ALM had following characteristics: dilated vascular canals intermingled with compact smooth muscle bundles; well circumscribe counter and myxoid and hyaline changes through the tumor. In contrast, PLMs had following histologic features: poor defined outline, entrapped hair follicles and eccrine glands, acanthosis and elongated rete ridges with hyperpigmentation and smooth muscle bundles which are interdigitated with elongated rete ridges. Here we introduced some distinct histological features for each subtype of the cutaneous leiomyoma which can lead to create novel criteria for classification and diagnosis of the lesion.

Asunto(s)

Leiomioma/patología , Neoplasias Cutáneas/patología , Adulto , Anciano , Angiomioma/clasificación , Angiomioma/patología , Biopsia , Glándulas Ecrinas/patología , Femenino , Folículo Piloso/patología , Humanos , Hialina/metabolismo , Hiperpigmentación , Leiomioma/clasificación , Leiomioma/metabolismo , Leiomiomatosis/clasificación , Leiomiomatosis/patología , Masculino , Persona de Mediana Edad , Miocitos del Músculo Liso/patología , Valor Predictivo de las Pruebas , Neoplasias Cutáneas/clasificación , Neoplasias Cutáneas/metabolismo

RESUMEN

BACKGROUND: There is a great deal of heterogeneity in the surgical strategy to treat intracardiac leiomyomatosis (ICL), leading to a need to create a theoretical tool to clarify this situation. METHODS: The data of 14 cases of ICL surgically treated in Anzhen Hospital from February 1995 to February 2009 were retrospectively reviewed. A system for classifying ICL was proposed based on four features of the lesion: size of intracardiac component; extent of inferior vena cava (IVC) involvement; venous pathway from uterus to IVC; and laterality of the lesion in the pelvis. The 14 cases of ICL were treated through multiple surgical strategies. RESULTS: There were no operative deaths. The follow-up was 73.1 +/- 59.2 months and one patient died from recurrence due to incomplete excision 5 months after the primary procedure. The 5-year survival rate calculated through Kaplan-Meier survival curve was 93.16 +/- 4.98%. Of the surviving patients, 13 had ICL, 10 were in the New York Heart Association (NYHA) class I, and three were in NYHA class II. CONCLUSION: The surgical treatment of ICL can obtain a good mid- to long-term survival rate and satisfactory heart function, and the proposed classification system for ICL may be helpful to guide the selection of the surgical strategy for ICL, and may serve as the future basis for standardising the reporting of ICL management.

Asunto(s)

Neoplasias Cardíacas/cirugía , Leiomiomatosis/cirugía , Adulto , Estudios de Cohortes , Femenino , Neoplasias Cardíacas/clasificación , Neoplasias Cardíacas/patología , Humanos , Leiomiomatosis/clasificación , Leiomiomatosis/patología , Persona de Mediana Edad , Estudios Retrospectivos , Adulto Joven

RESUMEN

OBJECTIVE: To approach an anatomic pathological classification scheme for the intracardiac leiomyomatosis (ICL), and to guide the choice of the surgical strategy for ICL treatment. METHODS: Retrospectively reviews the data of 13 cases of ICL from February 1995 to March 2007 in Anzhen Hospital. They were surgically treated under the CPB with different surgical strategy, and a quaternary classification scheme was used to classify them according to their anatomic pathological features of ICL. RESULTS: There was no operative death. One patient with YBOR type of ICL died from recurrence due to the incomplete excision 5 months after the primary operation. The 5 yrs survival rate calculated by the Kaplan-Meier survival curve was 93% +/- 5%. Of the surviving 12 patients, 9 were in NYHA functional class I, and 3 in class II. CONCLUSION: The surgical treatment of ICL can get a good mid-long term survival rate and living quality, and a quaternary classification scheme for ICL can be used to guide the choosing of surgical strategy and to understand the symptoms of ICL.

Asunto(s)

Neoplasias Cardíacas/patología , Leiomiomatosis/patología , Adulto , Femenino , Estudios de Seguimiento , Neoplasias Cardíacas/clasificación , Neoplasias Cardíacas/cirugía , Humanos , Leiomiomatosis/clasificación , Leiomiomatosis/cirugía , Persona de Mediana Edad , Estudios Retrospectivos

Asunto(s)

Leiomioma/patología , Leiomiomatosis/patología , Neoplasias Cutáneas/patología , Neoplasias Uterinas/patología , Adulto , Femenino , Humanos , Leiomiomatosis/clasificación , Persona de Mediana Edad , Neoplasias Cutáneas/clasificación , Síndrome

RESUMEN

El angioleiomioma es un tumor benigno originado en el músculo liso de la pared de una vena. Es relativamente raro, se observa con mayor frecuencia en mujeres durante la cuarta década de la vida, y se localiza con preferencia en los miembros inferiores. Presentamos 50 casos de esta entidad con las siguientes ubicaciones: 39 (78 por ciento) en las extremidades inferiores, 3 en la cabeza, 2 en el abdomen, 2 en los miembros superiores y 1 en la mama; en 3 casos la localización no se informó. En el estudio histopatológico de la pieza quirúrgica se hallaron lesiones bien delimitadas que contienen gran cantidad de venas de diferente calibre, con pared de espesor variable. Se subdividieron en capilares o sólidos (33 casos), venosos (16 casos) y cavernosos (1 caso). Entre los diagnósticos histológicos debe considerarse el resto de los leiomiomas. Para algunos autores se trata de una malformación hamartomatosa debida a la presencia de células adiposas maduras en el interior del angioleiomioma, un hallazgo que nosotros observamos en sólo uno de nuestros pacientes (AU)

Asunto(s)

Humanos , Masculino , Preescolar , Adolescente , Adulto , Persona de Mediana Edad , Femenino , Lactante , Niño , Anciano , Angiomioma/diagnóstico , Angiomioma/patología , Leiomioma/clasificación , Leiomioma/diagnóstico , Leiomiomatosis/clasificación , Leiomiomatosis/diagnóstico

RESUMEN

La Leiomiomatosis peritoneal diseminada (LPD) es una entidad muy rara caracterizada por múltiples nódulos benignos del músculo liso que aparecen en mujeres en edad reproductiva. Su degeneración maligna sólo ha sido reportada en la literatutra mundial en 5 casos (1). el presente reporte, constituye el sexto caso con degeneración maligna histológicamente comprobada. nuestro caso representa el único con trasformación maligna en una paciente menopáusica que recibía tratamiento hormonal sustitutivo continuo y con todos los estudios preoperatorios

Asunto(s)

Humanos , Femenino , Persona de Mediana Edad , Histerectomía , Leiomiomatosis/clasificación , Miometrio , Ovario/anomalías , Útero/anomalías