[Carney triad: Report of one case]. / Tríada de Carney. Una rara asociación de tumores infrecuentes: Caso clínico.

Carney described a disorder characterized by the presence of several uncommon tumors which were pulmonary chondromas, gastric sarcomas and extra-adrenal paragangliomas. We report a 14 year-old girl in whom multiple gastric tumors were discovered during a study of an iron deficiency anemia and was subjected to a partial gastrectomy. At 25 years of age, she developed several pulmonary chondromas and at 33 years, a mediastinal tumor with features of an extra-adrenal paraganglioma was found. At 35 years of age, a total gastrectomy was performed to remove a gastrointestinal stromal tumor with excision of peritoneal and lymph node metastasis. One year later, the patient died due to liver failure secondary to liver metastases.

Tríada de Carney. Una rara asociación de tumores infrecuentes: caso clínico / Carney triad: report of one case

Carney described a disorder characterized by the presence of several uncommon tumors which were pulmonary chondromas, gastric sarcomas and extra-adrenal paragangliomas. We report a 14 year-old girl in whom multiple gastric tumors were discovered during a study of an iron deficiency anemia and was subjected to a partial gastrectomy. At 25 years of age, she developed several pulmonary chondromas and at 33 years, a mediastinal tumor with features of an extra-adrenal paraganglioma was found. At 35 years of age, a total gastrectomy was performed to remove a gastrointestinal stromal tumor with excision of peritoneal and lymph node metastasis. One year later, the patient died due to liver failure secondary to liver metastases.

Management of Carotid Bifurcation Tumors: 30-Year Experience.

BACKGROUND: The carotid bifurcation can host a variety of tumors requiring complex surgical management. Treatment requires resection and, in some cases, vascular reconstruction that may compromise the cerebral circulation. The most frequent lesion at this location is the carotid body tumor (CBT). CBT are classified according to Shamblin in 3 types depending on the degree of carotid vessels encasement. Our main objective was to report our clinical experience managing carotid bifurcation tumors throughout the last 30 years. METHODS: Between 1984 and 2014, we treated 30 patients with 32 carotid bifurcation tumors. There were 21 women and 9 men (2.3:1), with a mean age of 45.5 years (18-75). The most frequent presentation was an asymptomatic neck swelling or palpable mass localized at the carotid triangle (86.7%). RESULTS: Thirty of 32 tumors were resected. Since 1994, computed tomography scan has been the most frequently used diagnostic imaging tool (80%), followed by magnetic resonance imaging. Angiography was used mainly during the first 10 years of the study period. Mean size of the tumor was 44.6 mm (20-73 mm). Nineteen (63%) were classified as Shamblin II and 6 (20%) as Shamblin's III. All specimens were analyzed by a pathologist; 28 tumors (93%) were confirmed as paragangliomas, 2 (7%) were diagnosed as schwannomas. Two patients underwent preoperative embolization of the CBT; 5 patients (17%) required simultaneous carotid revascularization, all of them Shamblin III. Mean hospitalization time was 4.5 days (1-35 days). Transient extracranial nerve deficit was observed in 7 patients (23.3%). Three patients (Shamblin III) required red blood cells transfusion. One patient (Shamblin III) underwent a planned en bloc excision of the vagus nerve. There was no perioperative mortality or procedure-related stroke. No malignancy or tumor recurrence were observed during follow-up. CONCLUSIONS: CBTs can be diagnosed on clinical grounds requiring vascular imaging confirmation. These infrequent lesions are generally benign. Early surgical removal by surgeons with vascular expertise avoids permanent neurologic and or vascular complications.

[A comparative study of 9 cases of adrenal pheochromocytoma and 11 cases of extra-adrenal pheochromocytoma]. / Estudio comparativo de nueve casos con feocromocitoma adrenal y once casos con feocromocitoma extraadrenal.

Twenty patients with the diagnosis of pheochromocytoma were studied from January 1990 to January 1998. Nineteen patients had a pathologic investigation performed. The mean age of patients was 49 +/- 16 years (range: 24-71 years), 8 males and 12 females. Nine cases corresponded to adrenal and eleven to extra-adrenal (paragangliomas) pheochromocytomas. The anatomic location was similar in both adrenal glands and for paragangliomas the cervical location predominated. The most consistent clinical finding in our patients was maintained arterial hypertension, followed by headache, palpitations and flushing. Biochemically, an increase in urine catecholamine levels or their metabolites was found in 82.2%. The clonidine test was very useful, particularly for cases where biochemical results were discrepant. CT, NMR and gammagraphy with 123I-MIBG have a high sensitivity for locating pheochromocytomas. NMR and gammagraphy with 111In-pentetreotide located cases in which CT or 123I-MIBG were negative. Preoperative treatment with adrenergic alpha-blockers allowed to surgery with no arterial tension complications. Only one patient with multiple abdominal paragangliomas relapsed. No differences regarding clinical manifestations, biochemical parameters or imaging studies were found between pheochromocytomas and paragangliomas, except their location.

Diagnóstico y terapéutica de los tumores glómicos timpanoyugulares / Diagnosis and therapeutic of the tympanoyugular glomus tumors

El objetivo del diagnóstico y embolización de los tumores glómicos es facilitar la erradicación quirúrgica mediante la desarterialización y consecuente disminución de la vascularización tumoral previo al acto operatorio. Entre mayo de 1989 y mayo de 1991 se estudiaron 1.480 pacientes de este servicio, ocho de ellos eran portadores de tumor glómico diagnosticados por datos clínicos y estudios radiológicos convencionales, TC contrastada de alta resolución, RNM y angiografía por sustracción digital. La embolización fue evaluada y programada por un equipo compuesto por angiorradiólogos y otoneurocirujanos. El presente trabajo analiza las características angiográficas y fija las pautas para el tratamiento endovascular prequirúrgico

Chemodectoma (non-chromaffin paraganglioma) of the face

A case of chemodectoma of the face is reported and its origin is discussed (AU)

Tumours of the chemoreceptor system

Since the opening of the University Hospital in 1952, not more than a total of ten patients were seen in whom a diagnosis of chemodectoma was made. There were six patients with a glomus jugulare tumour, three with a carotid body tumour while one patient had 3 tumours (glomus jugulare, carotid body and glomus intra vagale). The literature is reviewed with regards to site, histology, frequency, clinical presentation, behaviour and radiological diagnosis of these tumours. The case histories of our patients demonstrate: i) there is a definite pattern of presentation allowing a clinical diagnosis to be made with some degree of confidence. ii) biopsy is hazardous due to the vascularity of the growths and angiography appears much to be preferred. iii) Glomus jugulare tumours may erode bone, while functioning tumours may produce phaeochromocytoma-like pictures; such would require adequate surgical excision. iv) otherwise, the chemodectomas behave in a non-malignant way whatever their histological picture. This often justifies non-surgical treatment (AU)