[Abdominal manifestations in von Hippel Lindau disease in a group of 7 patients and literature review.]. / Manifestaciones abdominales en la enfermedad de von Hippel Lindau en una serie de 7 pacientes y revisión de la bibliografía.

OBJECTIVE: To evaluate the radiologic findings (ultrasound, computed tomography and magnetic resonance) of the abdominal manifestations in von Hippel-Lindau disease (VHL). MATERIAL AND METHODS: We retrospectively reviewed the imaging studies performed to seven patients diagnosed of VHL in our hospital. RESULTS: In all exmined patients, abdominal involvement was found. All of them showed renal affectation: cysts or renal cell carcinoma. Pancreatic involvement was found in the majority of patients: simple cysts, serous cystadenomas and one neuroendocrine tumor. One patient had a papillary cystadenoma of the broad ligament.

Clinical and Pathological Analysis of Two Cases of Cutaneous Malignant Melanoma.

The purpose of this study was the clinical and histo-immunohistochemical analysis of two cases: a cutaneous pigmented facial malignant melanoma and a lumbar congenital nevus with malignant transformation. A series of clinical elements raised the suspicion of some malignant melanocytic lesions and the histopathological analysis through the paraffin embedding technique confirmed the clinical suspicion. The immunohistochemical analysis using the streptavidin-biotin-peroxydase method of the facial malignant melanoma showed: S100 protein intense and diffuse positive, Tyrosinase diffuse positive, HMB45 strong and focal positive, Cyclin D1 positive in approximately 40% and Ki-67 positive in almost 70% of the tumor cells. The malignant melanoma developed on the nevocellular nevus displayed: S100 protein intense and diffuse positive, both in the nevus cells and in the malignant melanocytes as well, Tyrosinase intense and diffuse positive in the malignant melanocytes, poor and focal positive in the nevus cells and HMB45 intense and focal positive in the malignant cells and positive in the isolated nevus cells. Cyclin D1 was positive in about 70% of the malignant cells, but negative in the nevus area and Ki-67 was found positive in relatively 30% of the malignant melanocytes, also in less than 1% of the nevus cells. The pattern and the intensity of the Tyrosinase and HMB45 immunoexpression are important in the differentiation of the nevus cells from the malignant melanocytic cells. The immunoexpression of Cyclin D1 does not correlate directly with the proliferating activity of the malignant melanocytic cells in all types of malignant melanomas.

Manifestaciones abdominales en la enfermedad de von Hippel Lindau en una serie de 7 pacientes y revisión de la bibliografía / Abdominal manifestations in von Hippel Lindau disease in a group of 7 patients and literature review

Objetivo Valorar los hallazgos radiológicos (ecografía, tomografía computarizada y resonancia magnética) de las diferentes manifestaciones abdominales de la enfermedad de von Hippel Lindau (EVHL). Material y métodosSe revisaron de forma retrospectiva los estudios de imagen de 7 pacientes diagnosticados de EVHL en nuestro hospital. Resultados Se detectó afectación abdominal en todos los pacientes estudiados. Todos mostraron algún tipo de afectación renal: quistes y carcinomas renales. También se detectó afectación pancreática en la práctica totalidad de los casos: quistes pancreáticos simples, varios cistoadenomas serosos y un tumor neuroendocrino. Una paciente presentó un cistoadenoma papilar del ligamento ancho. Conclusiones Las técnicas de imagen desempeñan un papel esencial, tanto en el diagnóstico inicial de las distintas manifestaciones abdominales como en el control y seguimiento de estos pacientes (AU)

Objective To evaluate the radiologic findings (ultrasound, computed tomography and magnetic resonance) of the abdominal manifestations in von Hippel-Lindau disease (VHL). Material and methods We retrospectively reviewed the imaging studies performed to seven patients diagnosed of VHL in our hospital. Results In all exmined patients, abdominal involvement was found. All of them showed renal affectation: cysts or renal cell carcinoma. Pancreatic involvement was found in the majority of patients: simple cysts, serous cystadenomas and one neuroendocrine tumor. One patient had a papillary cystadenoma of the broad ligament (AU)