Neoplasia papilar de polaridad inversa: un nuevo subtipo de tumor renal de buen pronóstico / Papillary renal cell neoplasm with reverse polarity: A new subtype of renal tumour with favorable prognosis

Introducción: La neoplasia papilar renal de polaridad inversa (NPRPI) ha sido recientemente reconocida como una entidad separada de la clasificación tradicional de los carcinomas papilares de células renales por sus peculiares características histopatológicas, inmunofenotípicas y moleculares, y un comportamiento indolente. Material y métodos: En este trabajo aportamos 6 nuevos casos y realizamos una revisión de la literatura publicada al respecto hasta el momento actual, recopilándose un total de 104 casos. Resultados: Nuestros casos de NPRPI corresponden a 5 hombres y una mujer, con edades comprendidas entre los 47 y los 91 años. En 5 casos la NPRPI resultó un hallazgo incidental en piezas de nefrectomía indicada por la presencia de otro tumor renal y en uno la NPRPI fue el motivo de la intervención quirúrgica. Nuestros casos presentan tamaños entre los 2 y los 13mm, y una histología papilar con revestimiento en monocapa de células eosinófilas con núcleos de bajo grado en localización apical. Inmunohistoquímicamente muestran una constante positividad para GATA3 y negatividad para vimentina. Se identificaron mutaciones en KRAS en el 50% de ellos. Tras un seguimiento comprendido entre uno y 60 meses, 5 de los pacientes seguían vivos sin recurrencia o metástasis y uno falleció a causa de un carcinoma urotelial. Conclusiones: Nuestros casos concuerdan con las características clínicas y patológicas descritas en los publicados hasta el momento. Aportamos la primera serie nacional y corroboramos la existencia de unos criterios diagnósticos definidos y constantes que permiten considerar la NPRPI como una entidad propia distintiva. (AU)

Introduction: Papillary renal cell neoplasm with reverse polarity (PRNRP) has recently been recognized as an entity separate from the traditional classification of papillary renal cell carcinomas, due to its specific histopathological, immunophenotypic and molecular characteristics, as well as its indolent behavior . Material and methods: We provide 6 new cases and a review of the literature published until the present time, which comprises a total number of 104 cases. Results: Our PRNRP cases correspond to 5 men and one woman aged between 47 and 91 years. In 5 of the 6 cases, the PRNRP was an incidental finding in nephrectomy specimens. Nephrectomy had been indicated due to the presence of another renal tumor, except for one case, in which surgical intervention was indicated due to PRNRP. Our cases present mass sizes between 2 and 13mm, as well as papillary histology with a monolayered lining of eosinophilic cells with low-grade nuclei in apical location. Immunohistochemically, they show a constant positivity for GATA3 and negativity for vimentin. KRAS mutations were identified in 50% of our cases. After a follow-up ranging between one and 60 months, 5 of the cases were still alive without recurrences or metastases, and one died from urothelial carcinoma. Conclusions: Our cases agree with the clinical and pathological characteristics described in the PRNRP cases published to date. With the present study, we provide the first series of national cases corroborating the existence of well-defined and constant diagnostic criteria that allow PRNRP to be considered as a distinctive entity. (AU)

Papillary renal cell neoplasm with reverse polarity: A new subtype of renal tumour with favorable prognosis.

INTRODUCTION: Papillary renal cell neoplasm with reverse polarity (PRNRP) has recently been recognized as an entity separate from the traditional classification of papillary renal cell carcinomas, due to its specific histopathological, immunophenotypic and molecular characteristics, as well as its indolent behavior. MATERIAL AND METHODS: We provide 6 new cases and a review of the literature published until the present time, which comprises a total number of 104 cases. RESULTS: Our PRNRP cases correspond to 5 men and one woman aged between 47 and 91 years. In 5 of the 6 cases, the PRNRP was an incidental finding in nephrectomy specimens. Nephrectomy had been indicated due to the presence of another renal tumor, except for one case, in which surgical intervention was indicated due to PRNRP. Our cases present mass sizes between 2 and 13 mm, as well as papillary histology with a monolayered lining of eosinophilic cells with low-grade nuclei in apical location. Immunohistochemically, they show a constant positivity for GATA3 and negativity for vimentin. KRAS mutations were identified in 50% of our cases. After a follow-up ranging between one and 60 months, 5 of the cases were still alive without recurrences or metastases, and one died from urothelial carcinoma. CONCLUSIONS: Our cases agree with the clinical and pathological characteristics described in the PRNRP cases published to date. With the present study, we provide the first series of national cases corroborating the existence of well-defined and constant diagnostic criteria that allow PRNRP to be considered as a distinctive entity.

Management of ampullary tumours in children: still a challenge.

This study describes the case of the youngest patient ever reported with ampullary adenoma. The incidence of ampullary adenomas in childhood is unknown. Endoscopic retrograde cholangiopancreatography (ERCP) and endoscopic ultrasound are used in adults to assess and treat these lesions, although there are no instruments designed specifically for use in young children. A six-year-old girl was admitted for abdominal pain, vomiting, pruritus and weight loss. Abdominal ultrasound showed biliary tree (8mm) and pancreatic duct dilatation (4mm). Magnetic resonance cholangiopancreatography and computed tomography confirmed these findings, and also showed displacement of the ampulla to the left upper quadrant. An upper endoscopy confirmed a large ampullary adenoma. A laparotomy was performed and a 5cm villous tumour arising from the ampulla was excised. The postoperative course was uneventful. The histology demonstrated adenoma of the ampulla (intestinal type) without low-grade dysplasia. all clinical and radiological parameters are normal at 20 months follow-up. We describe the case of the youngest patient ever reported with ampullary adenoma. Pancreaticoduodenectomy carries high morbidity and mortality rates, and therefore it should be avoided in absence of histologically proven malignancy. We believe that surgical ampullectomy is a safe and oncologically correct procedure until better endoscopic instruments for peadiatric use will be designed.

Whole-exome sequencing in splenic marginal zone lymphoma reveals mutations in genes involved in marginal zone differentiation.

Splenic marginal zone lymphoma (SMZL) is a B-cell neoplasm whose molecular pathogenesis remains fundamentally unexplained, requiring more precise diagnostic markers. Previous molecular studies have revealed 7q loss and mutations of nuclear factor κB (NF-κB), B-cell receptor (BCR) and Notch signalling genes. We performed whole-exome sequencing in a series of SMZL cases. Results confirmed that SMZL is an entity distinct from other low-grade B-cell lymphomas, and identified mutations in multiple genes involved in marginal zone development, and others involved in NF-κB, BCR, chromatin remodelling and the cytoskeleton.

Reproducibilidad interobservador de un sistema de grado para el carcinoma de células renales tipo cromófobo / Interobserver Reproducibility of a Grading System for Chromophobe Renal Cell Carcinoma

Objetivos: Valorar la reproducibilidad interobservador y evaluar el sistema de gradación propuesto por Paner et al. para el carcinoma de células renales cromófobo. Material y métodos: Tras seleccionar 23 casos de carcinoma renal de tipo cromófobo de los hospitales Xeral-Cíes, Meixoeiro y POVISA de Vigo de los últimos 15 años se ha realizado una sesión informativa de los criterios del sistema de gradación de Paner et al. Posteriormente los patólogos observadores han aplicado dicho sistema a cada caso, valorando una laminilla seleccionada. Se ha calculado el índice Kappa de reproducibilidad interobservador, ponderado según la escala de Landis y Koch. Resultados: La distribución de grados en la mayoría de los 6 observadores participantes es similar, con predominio del grado 1 en 4 de los mismos. Los 2 observadores restantes consideraron una mayoría relativa de casos como grado 2. Los valores de Kappa oscilan entre 0,136 y 0,674, observándose un predominio de valores indicadores de reproducibilidad discreta-moderada (0,21-0,60). El mayor valor de Kappa (0,674) se ha dado entre un observador novel y el patólogo más experto. Entre los 2 observadores más veteranos se ha obtenido el índice más bajo (0,136). Conclusiones: La reproducibilidad interobservador en nuestros centros para el grado propuesto por Paner et al. es discreta-moderada. La asignación de los grados 1 y 2 no es homogénea entre los 6 observadores participantes. En espera de la existencia de una gradación consensuada por las sociedades científicas, creemos prudente no utilizar ningún sistema de gradación en los carcinomas de células renales de tipo cromófobo (AU)

Objectives: To evaluate interobserver reproducibility of a grading system proposed by Paner et al. for chromophobe renal cell carcinoma. Material and methods: After selecting 23 cases of chromophobe renal cell carcinoma from the Xeral-Cíes Hospital, Meixoeiro Hospital and POVISA Hospital from the last 15 years, an informative meeting on the Paner et al. grading system criteria was held. After, the participating pathologists applied the system to each case, evaluating one slide selected. Kappa index for interobserver reproducibility was calculated, and it was classified according to the Landis and Koch scale. Results: The grading distribution was similar for most of the 6 participating observers, with grade 1 predominance. The remaining 2 observers considered a relatively higher proportion of grade 2. Kappa index values ranged from 0.136 to 0.674, with a discrete-moderate reproducibility index predominance (0.21-0.60). Highest Kappa value (0.674) was obtained between the most novel and the most expert interobservers. The lowest Kappa value was obtained among the most veteran pathologists (0.136). Conclusions: Interobserver reproducibility for chromophobe renal cell carcinoma is discrete-moderate in our institutions when the novel grade proposed by Paner et al. is used. Labeling of grades 1 and 2 is not homogeneous among 6 participating observers. While awaiting a grading consensus on a new classification by the scientific societies, we consider that the routine use of a grading system for chromophobe renal cell carcinoma should not be used (AU)

Interobserver reproducibility of a grading system for chromophobe renal cell carcinoma.

OBJECTIVES: To evaluate interobserver reproducibility of a grading system proposed by Paner et al. for chromophobe renal cell carcinoma. MATERIAL AND METHODS: After selecting 23 cases of chromophobe renal cell carcinoma from the Xeral-Cíes Hospital, Meixoeiro Hospital and POVISA Hospital from the last 15 years, an informative meeting on the Paner et al. grading system criteria was held. After, the participating pathologists applied the system to each case, evaluating one slide selected. Kappa index for interobserver reproducibility was calculated, and it was classified according to the Landis and Koch scale. RESULTS: The grading distribution was similar for most of the 6 participating observers, with grade 1 predominance. The remaining 2 observers considered a relatively higher proportion of grade 2. Kappa index values ranged from 0.136 to 0.674, with a discrete-moderate reproducibility index predominance (0.21-0.60). Highest Kappa value (0.674) was obtained between the most novel and the most expert interobservers. The lowest Kappa value was obtained among the most veteran pathologists (0.136). CONCLUSIONS: Interobserver reproducibility for chromophobe renal cell carcinoma is discrete-moderate in our institutions when the novel grade proposed by Paner et al. is used. Labeling of grades 1 and 2 is not homogeneous among 6 participating observers. While awaiting a grading consensus on a new classification by the scientific societies, we consider that the routine use of a grading system for chromophobe renal cell carcinoma should not be used.

[Multivariate analysis of recurrence and progression in stage T1 transitional-cell carcinoma of the bladder. Prognostic value of p53 and Ki67]. / Análisis multivariado de recidiva y progresión en el carcinoma de células transicionales de vejiga en estadio T1. Valor pronóstico de p53 y Ki67.

OBJECTIVE: To establish prognostic factors of recurrence and progression in stage T1 transitional cell bladder carcinoma, paying special attention to prognostic value of p53 and ki67. MATERIALS AND METHODS: 175 patients with incident bladder tumour at T1 category. The immunohistochemical study was performed using the monoclonal antibodies DO-7, for p53 and MIB-1, for ki67. Kaplan-Meier methodology was used, and a multivariate analysis using Cox's proportional hazards model was carried out in order to determine the variables associated with recurrence and progression. ROC curves were also drawn up. RESULTS: The average follow up period was 8.55 years. 5 and 12-year recurrence-free survival rates were 57.98% and 48.47%. The independent variables for recurrence were histological grade, tumour multifocality, tumour size > 3 cm, presence of carcinoma in situ and ki67 expression. 5 and 12-year progression-free survival rates were 83.12% and 75.63%. The independent variables for progression were age, tumour multifocality, solid microscopic morphology, p53 expression and ki67 expression. CONCLUSIONS: Solid microscopic pattern and p53 expression are the variables which best predict progression. A positive relationship was observed between p53 and progression: the greater the expression of p53, the greater the progression. Tumour multifocality and ki67 expression > 27% are the main prognostic factors for recurrence.

Análisis multivariado de recidiva y progresión en el carcinoma de células transicionales de vejigaen estadio t1. Valor pronóstico de p53 y ki67 / Multivariate analysis of recurrence and progression in stage t1 transitional cell bladder carcinoma. Prognostic value of p53 and ki67

OBJETIVO: Establecer los factores pronósticos de recidiva y progresión en el carcinoma de células transicionales de vejiga en estadio T1, prestando especial atención al valor pronóstico de p53 y ki67. MATERIAL Y MÉTODOS: 175 pacientes con tumor vesical incidente de la categoría T1. El estudio inmunohistoquímico fue realizado con los anticuerpos monoclonales DO7, para p53 y MIB-1, para ki67. Se utilizó la metodología de Kaplan-Meier y se realizó un análisis multivariado de regresión de Cox, para determinar variables asociadas a recidiva y progresión. Se realizaron asimismo curvas ROC para p53 y ki67.RESULTADOS: El seguimiento medio fue de 8,55 años. La supervivencia libre de recidiva a los 5 y 12 años fue 57,98 por ciento y 48,47 por ciento. Las variables independientes de recidiva fueron: grado histológico, multifocalidad tumoral, tamaño tumoral >3 cm, presencia de carcinoma in situ y expresión de ki67. La supervivencia libre de progresión a los 5 y 12 años fue 83,12 por ciento y 75,63 por ciento. Las variables independientes de progresión fueron: edad, multifocalidad tumoral, morfología microscópica sólida, expresión de p53 y expresión de ki67. CONCLUSIONES: La morfología microscópica sólida y la expresión de p53 son las variables con mayor capacidad para predecir progresión tumoral. Se observa una correlación positiva entre la expresión de p53 y la progresión, de forma que cuanto mayor es la expresión de p53, mayor es la probabilidad de progresión. La multifocalidad tumoral y la expresión de ki67 >27 por ciento son los principales factores pronósticos de recidiva. (AU)

[Pulmonary capillary hemangiomatosis: a rare cause of pulmonary hypertension]. / Hemangiomatosis capilar pulmonar: una causa poco frecuente de hipertensión pulmonar.

Pulmonary capillary hemangiomatosis (PCH) is a rare cause of pulmonary hypertension characterized by capillary proliferation infiltrating the structures of the pulmonary parenchyma. Although veins are particularly involved, proliferation also affects bronchiolar, interstitial and other structures. We report a case of PCH in a 70-year-old man. Pulmonary artery hypertension was demonstrated by echocardiogram and angiography. Severe emphysema could be seen in a computed tomographic scan of the thorax, even though spirometric values indicated that airflow obstruction was mild. Dyspnea and respiratory insufficiency progressed with marked shunting until death. Tissue inspection at the autopsy revealed capillary proliferation in the alveolar walls with occasional oviform protrusions into air spaces or around small vessels and bronchioles. Endothelial cells in newly formed vessels were not atypical and mitosis was scarce; p53 expression was negative and Ki67 proliferation slight, indicating that PCH is not a neoplastic process as has sometimes been suggested.

Hemangiomatosis capilar pulmonar: una causa poco frecuente de hipertensión pulmonar / Pulmonary capillary hemangiomatosis: a rare cause of pulmonary hypertension

La hemangiomatosis capilar pulmonar (HCP) es una causa rara de hipertensión pulmonar, caracterizada por la proliferación de capilares que infiltran las estructuras del parénquima pulmonar, particularmente las vénulas, pero también los bronquiolos, el intersticio y otras. Presentamos un caso de hemangiomatosis capilar pulmonar en un varón de 70 años. Se demostró la existencia de una hipertensión arterial pulmonar mediante ecocardiografía y cateterismo.La tomografía axial computarizada (TAC) torácica evidenció que el enfermo presentaba un enfisema grave, a pesar de los valores de la espirometría que reflejaban una obstrucción al flujo aéreo de grado leve. Desarrolló una disnea progresiva y una insuficiencia respiratoria con efecto shunt marcado hasta el fallecimiento. El diagnóstico patológico se realizó en la autopsia. Se observó una proliferación capilar en las paredes alveolares, que formaba en ocasiones ovillos que protruían en los espacios aéreos o rodeaban pequeños vasos y bronquiolos. Las células endoteliales de los vasos neoformados no evidenciaban atipias y las mitosis eran escasas. La negatividad de la técnica inmunohistoquímica del antígeno p53 y la baja tasa de proliferación celular evaluada por Ki67 no apoyaban la naturaleza neoplásica de la HCP, como se ha sugerido en algunos trabajos. (AU)

Nosocomial Kikuchi's disease--a search for herpesvirus sequences in lymph node tissues using PCR.

BACKGROUND: Histiocytic necrotizing lymphadenitis, also known as Kikuchi's disease (KD), is a rare disease. Fever and lymphadenopathies with characteristic pathologic features are present. The etiology of this disease remains undetermined. Since the disorder is self-limiting, different viruses have been implicated as the causative agent. PATIENTS AND METHODS: Seven cases of KD were studied. Three patients acquired the disease nosocomially, three had community-acquired KD and one case was associated with systemic lupus erythematosus. PCR was performed on DNA extracted from lymph node tissues in order to detect herpesvirus-specific DNA sequences: herpes simpLex virus type 1 and 2 (HSV1-2), varicella zoster virus (VZV), human cytomegalovirus (HCMV), human herpesvirus 6 (HHV6), Epstein-Barr virus (EBV) and human herpesvirus 8 (HHV8). RESULTS: Viral DNA was not detected in any of the lymph node tissues from the seven cases of KD. CONCLUSION: We conclude that these herpesviruses were not involved in the etiology of the three cases of nosocomial KD, nor in the other four cases of KD investigated in this study.

Aparición paradójicade tuberculoma y formaciónde absceso cerebelosoen un paciente con el virusde la inmunodeficiencia humanatratado con triple terapiaantirretrovírica / Parodoxical appearance of tuberculoma and formation of a cerebellar abscess in a patient with human immunodeficiency virus treated with triple antiretroviral therapy

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[Acute scrotum as clinical presentation form of testicular tumor]. / Escroto agudo como forma de presentación clínica de un tumor de testículo.

Testis tumours usually emerge in young patients as a painless increase in the testis size accidentally noticed by the patient. Contribution of one case of non-seminomatous testis tumour which presented clinically as an acute scrotum. An exhaustive appraisal of the clinical data allowed to choose the most appropriate approach for the patient in an emergency situation.

[Pulmonary lymphangioleiomyomatosis. Presentation of a new case]. / Linfangioleiomiomatosis pulmonar. Presentación de un nuevo caso.

A 48-year-old woman hospitalized because of spontaneous pneumothorax. She suffered dyspnea since three years before. Diffuse reticulonodular interstitial pattern was observed in the radiography. The transbronchial biopsy suggested lymphangioleiomyomatosis, the diagnosis being supported by open pulmonary biopsy. Usefulness of transbronchial biopsy is discussed and several treatment modalities are described.

[Tumor of the vitelline sac of the testicle (yolk sac carcinoma)]. / Tumor del saco vitelino de testiculo (yolk sac carcinoma).

The tumour of the yolk sac is a germinal cell neoplasia arising in the prepubertal testicles which was included in the WHO's histological classification of neoplasia of the testicles in 1977. We contribute a case of this histological variety in Stage I, according to the Royal Marsden criteria. The patient was treated with inguinal orchidectomy only and 3 years later remains disease-free.

[The incidence of gastric carcinoma after gastrectomy for peptic ulcer in the Vigo region. A case-control study]. / Frecuencia del carcinoma gástrico postgastrectomía para úlcera péptica en la comarca de Vigo. Estudio caso-control.

After reviewing 10,000 upper gastrointestinal endoscopies performed at the endoscopy unit of the city of Vigo over a 38 month period, we have found 485 partial gastric resections for peptic ulcer, 357 gastric carcinomas were found, of which 26 occurred after partial gastric resection for peptic ulcer. Therefore the incidence of gastric cancer in this area was 22-23/100,000. The frequency of gastric cancer after partial resective surgery was lower than expected during the first 20 years after surgery. However, thereafter a significant increase of gastric cancer occurred in those patients in which a Billroth-II but not Billroth-I procedure was used.

[Carcinosarcoma and papillary transitional cell carcinoma coexisting in the same bladder]. / Carcinosarcoma y carcinoma papilar de células transicionales coexistentes en la misma vejiga.

A case of synchronically coexistent carcinosarcoma and transitional cell carcinoma, with separate neoplastic processes in the same urinary bladder is presented. This tumour association is as far as we know, the second case reported in the literature. Carcinosarcoma was formed by an epithelial component represented by a high level transitional cell carcinoma and a mesenchymal component characterized by an unspecific fusocellular sarcoma with chondrosarcomatous differentiation. An immunohistochemical analysis was performed, confirming the dual nature of the carcinosarcoma.

Aspergillosis of the sphenoid sinus simulating a pituitary tumor.

Sphenoidal aspergillosis is an unusual cause of sella turcica enlargement. Pituitary abscess secondary to Aspergillus had been reported. In the present case, a woman with sphenoid sinus aspergillosis mimicked a pituitary tumor. This patient survived her infection with intact pituitary function following a transsphenoidal approach. No postoperative amphotericine-B and 5-fluorocytosine were necessary. CT scan revealed a mass occupying the sphenoid sinus extending to the sella turcica. Factors that should alert the clinican to the presence of a sphenoidal and pituitary abscess in a patient with sella turcica enlargement are prior episodes of sinusitis, meningitis and immunosuppression and, as in the present case, hyperglycemia.