[Lhermitte-Duclos's disease associated to Cowden s disease: a case report]. / Enfermedad de Lhermitte-Duclos asociada a enfermedad de Cowden: a propósito de un caso.

Cowden's disease is a rare genodermatosis that is characterized for multiple cutaneous and visceral hamartoma . Lhermitte-Duclos's disease is a cerebelous lesion that consists in the displasic enlargement of the cerebelous circumvolution. It's incluyed in phacomatosis and usually presents associated to Cowden's disease, tuberous sclerosis and overlap syndromes.A 56 years old man, diagnosed in Dermatology with Cowden's disease ten years ago. In the extension study, he had hamartoma intestinal polip, esophagic glucogenic acanthosis and two solid thyroid nodules. The craneal TC didn't show significant alteration. Ten years after diagnosis a cerebral magnetis resonance was performed for intense cephalea, and it was found a bad-defined mass in right cerebelous hemisphere without contrast captation, compatible with cerebelous glangliocytoma.

Enfermedad de Lhermitte-Duclos asociada a enfermedad de Cowden: a propósito de un caso / Lhermitte-Duclo´s disease associated to Cowden´s disease: a case report

La enfermedad de Cowden es una rara genodermatosis que se caracteriza por la aparición de multiples hamartomas tanto a nivel cutaneomucoso como visceral. Es una lesión cerebelosa que consiste en el engrosamiento displásico de las circunvoluciones cerebelosas. Se incluye dentro de las facomatosis y suele presentarse junto a la enfermedad de Cowden, la esclerosis tuberosa o síndromes de solapamiento. Presentamos un paciente de 56 años diagnosticado en la consulta de Dermatología de enfermedad de Cowden hace 10 años. En el estudio de extensión se le diagnostica de poliposis intestinal hamartomatosa, acantosis glucogénica esofágica y se encuentran dos nódulos sólidos tiroideos. A los 10 años del diagnóstico se realiza resonancia magnética nuclear cerebral por aparición de cefalea encontrando una masa mal definida en hemisferio cerebeloso derecho compatible con gangliocitoma displásico cerebeloso

Cowden’s disease is a rare genodermatosis that is characterized for multiple cutaneus and visceral hamartoma . Lhermitte-Duclos´s disease is a cerebelous lesion that consists in the displasic enlargement of the cerebelous circumvolution. It´s incluyed in phacomatosis and ussually presents associated to Cowden’s disease, tuberous sclerosis and overlap syndromes. A 56 years old man, diagnosed in Dermatology with Cowden’s disease ten years ago. In the extension study, he had hamartoma intestinal polip, esophagic glucogenic acanthosis and two solid thyroid nodules. The craneal TC didn´t show significant alteration. Ten years after diagnosis a cerebral magnetis resonance was performanced for intense cephalea, and it was found a bad-defined mass in right cerebelous hemisphere without contrast captation, compatible with cerebelous glangliocytoma

[Endometriosis: an infrequent cause of colonic obstruction]. / Endometriosis: una causa infrecuente de obstrucción colónica.

Endometriosis localized in the intestinal wall is not an infrequent finding. Diagnosis is difficult given the diverse symptomatology presented with unspecific abdominal pain being the most common. Implantation of endometrial tissue in the intestinal wall may involve the mucosa and present as rectorhagia, with colonscopic exploration providing diagnosis by biopsy of the affected area. In other cases this may only involve the intestinal wall producing very varied symptomatology. Presentation as a picture of colon obstruction is little reported. The main problem is its difficult differential diagnosis with neoplasm which, in most cases, leads to surgery. A case of colon obstruction provoked by implantation of endometrial tissue in the wall of the sigma which was surgically resolved is herein presented.

[Abdominal pain and ascites as manifestations of hereditary angioneurotic edema]. / Dolor abdominal y ascitis como manifestaciones del edema angioneurótico hereditario.

Hereditary angioneurotic edema (HAE) is an infrequent autosomal dominant disorder characterized by a decrease in the levels or a dysfunction of the complement C1 inhibitor factor (C1 inh). The clinical presentation varies widely and involves any area of the organism. Gastrointestinal involvement is usually as abdominal pain and may be accompanied by ascites. De novo diagnosis of HAE with abdominal pain and ascites as a form of presentation is difficult with differential diagnosis with abdominal pain of unknown origin. The appearance of ascites is rare with few cases reported in the literature. Both abdominal pain and ascites disappear a few days after initiation of medical treatment. Occasionally exploratory laparotomy has been required. A new case of abdominal pain and ascites as manifestations of HAE is herein reported.

[A malignant extra-adrenal pheochromocytoma. Apropos 2 cases]. / Feocromocitoma extraadrenal maligno. A propósito de dos casos.

We present the case of two patients with extra-adrenal pheochromocytoma (left and right para-aorta abdominal) in which the pre-surgery search with metaiodobenzylguanidine-I-123 (MIBG-I-123) showed the existence of bone metastasis in skullcap, not previously suspected. In both patients a total resection of tumor was performed together with the bone lesions. These two cases confirm the great usefulness of MIBG-I-123 in the localization of metastasis in patients with malignant pheochromocytoma. At the same time we think that the resection of whole tumoral tissue through surgery is an adequate treatment when dealing with pheochromocytoma with isolated metastasis.

[Gammagraphic findings in hepatic focal nodular hyperplasia]. / Hallazgos gammagráficos en la hiperplasia nodular focal hepática.

Two cases, of focal nodular hyperplasia (FNH) are presented which were detected by chance and in which the gammagraphic study was the only parameter which indicated the changes that occurred in the hyperplasia revealing a hypervascular mass, colloid capture and 99mTc-DISIDA retention in the later image. These liver masses are now seen more frequently given the wide use of echography and the gammagraphic studies could be useful in the orientation of their nature in a non aggressive manner.

Diagnosis by ultrasonography and isotopic phlebography of a malformation of the inferior vena cava: proximal transposition with anterior crossing over the aorta.

A case of a previously undescribed malformation of the inferior vena cava is reported: there was distal normoposition and proximal transposition, with anterior crossing over the aorta; the right renal vein flowed into the inferior vena cava located on the left, after crossing over the anterior face of the aorta. This anomaly was shown by echography and isotopic phlebography.