Miocardiopatía dilatada y feocromocitoma / Dilated myocardiopathy and pheochromocytoma

El feocromocitoma, un tumor de baja incidencia con un comportamiento benigno en la mayoría de los casos, puede ser el responsable de extensas manifestaciones cardiovasculares. Las manifestaciones sobre el miocardio pueden ser de profundas consecuencias, pues llegan a producir una miocardiopatía dilatada con insuficiencia cardiaca severa. La exéresis del tumor suele ser curativa en el 90 por ciento de los casos y el manejo perioperatorio muy complejo. Se presenta un paciente joven portador de un feocromocitoma, que sometido a la larga acción de las catecolaminas llegó a desarrollar una miocardiopatía dilatada con criterio de trasplante cardiaco. La cirugía adrenal fue curativa en nuestro paciente(AU)

Pheochromocytoma, a low incidence tumor of benign behavior in most of cases, can cause extensive cardiovascular manifestations. These manifestations on the myocardium can have deep consequences since they may lead to dilated myocardiopathy with severe heart failure. The excision of tumor may be the curative solution for 90 percent of cases but the perioperative management is very complex. This is the case of a young patient who carries pheochromocytoma and after a long-acting catecholemine-based treatment, he developed dilated myocardiopathy, and the medical criterion was that he required heart transplantation. The adrenal surgery was successfully healing in our patient(AU)

Pheochromocytomas

Introduction: Pheochromocytomas are rare neuroendocrine tumors, producing catecholamines, which usually affect the adrenal medulla region of the adrenal gland. These tumors may clinically manifest in several ways, presenting themselves in most patients with persistent hypertension or paroxysmal. Ten percent of cases are considered malignant, confirmed by the presence of metastases and approximately 24% of cases are associated with inherited syndromes. Diagnostic confirmation of these syndromes implies preparatory workup, treatment and stringent follow-up, preferably with a multidisciplinary team. Objective: This study is a survey of recent studies to clarify issues related to clinical, diagnosis, genetic and treatment aspects of these patients. Conclusion: It is widely accepted that a significant percentage of patients with sporadic pheochromocytoma may have germline mutations leading to more widespread disease development and/or malignancy, and that surgical treatment in these cases must be complemented by careful clinical surveillance for early diagnosis of recurrences. This study prioritized the importance of conducting a proper pretreatment workup in cases of pheochromocytoma, which provides the additional information required for a rational course of treatment for patients.

Pheochromocytoma in children and adolescents.

Eleven subjects aged <20 yr with histologically proven pheochromocytoma between 1987 and 2006 were analyzed. Family history was present in 18%. In 2 patients, pheochromocytoma was part of VHL and in one it was associated with MEN 2. Twenty four hour urine VMA level was elevated in 100% and metanephrine level in 73%. CT/ MRI were showing the tumor in all. Prazosin extended release tablets (maximum 30 mg/day) were used in 73% and doxazosin (maximum 12 mg/ day) in 27%. Intraoperative BP fluctuations were seen in 27%. All were biochemically cured after surgery. Preoperative á blockade with extended release prazosin and doxazosin were effective in controlling perioperative BP fluctuations. Hence these drugs can be used in children and adolescents without fear of postoperative hypotension.

G691S/S904S polymorphism in the RET protooncogene of a 25-year-old medical student with bilateral pheochromocytoma.

The case of a 25-year-old medical student with bilateral pheochromocytoma is described. Following diagnostic testing, tumors were surgically removed. Genetic analysis revealed that the patient is a heterozygote with the following mutations on opposite homologs: G691S (exon 11) and S904S (TCC-TCG, exon 15), suggesting the diagnosis of multiple endocrine neoplasia 2A (MEN2A). A diagnosis of MEN2 would be an indication of thyroidectomy in this patient. Although this mutation is described in the literature, it has no known connection to pheochromocytomas. Therefore, it is unknown whether there is a causal connection between the G691S genotype and the pheochromocytomas in this patient. If so, G691S is to be added to the list of genotypes causing MEN2A. Here, the procedure of sequencing the RET protooncogene is described and a possible association between the G691S genotype and MEN2A is discussed.

Feocromocitoma: diagnóstico e manejo / Feocromocitoma: diagnosis and management

Este artigo de revisão tem como objetivo esclarecer conceitos e orientar conduta diagnóstica e manejo clínico deste tumor, que apesar de raro, trata-se de uma causa curável de hipertensão.

Feocromocitoma em bovinos de abate: relatos de casos / Pheochromocytoma in slaughtered bovine: cases reports

Relatou-se a ocorrência de dois casos de tumor de medular da adrenal em bovino. O caso 1 foi representado por uma neoplasia de 6,0×4,5cm de tamanho e cor amarelo-acastanhada. O caso 2 constituiu-se de neoplasia maligna, com a lesão primária medindo 33,0×18,0cm e peso de 6,5kg. Metástases estavam presentes nos pulmões e nos linfonodos tráqueobronquiais, mediastínicos e esofagianos. A análise histológica das neoplasias permitiu o diagnóstico de feocromocitoma.

Two cases of adrenal medular tumor in bovine are described. In case 1, yellow-brownish neoplasia of 6.0×4.5cm size was observed. Case 2, was constituted of a malignant neoplasia with the primary lesion measuring 33.0×18.0cm and weighing 6.5kg. Metastases were found in the lungs and in the tracheobronchial, mediastinic and oesophageal lymph nodes. Histopathological analysis confirmed the diagnosis of pheochromocytoma.

Endocrine cancer in Iran: based on cancer registry system.

BACKGROUND: A population-based registry of endocrine cancer cases in four Iranian provinces, was performed for the years 1996-2000. MATERIALS AND METHODS: Patients in each province were grouped according to age, gender and tumor specifics (site, morphology, behavior) and the data was coded according to the international classification of diseases for oncology. STATISTICAL ANALYSIS USED: Person-years of population at risk were calculated and the results were presented as incidence rates by sex, age, age specific rates and age standard rate (ASR) per 100,000 person-years, using direct method of standardization to the world population. RESULTS: A total of 319 cases of primary endocrine cancer were found and registered, including 313 cases of thyroid carcinoma and 6 cases of adrenal cancer. The thyroid carcinoma group cases consisted of papillary (82.7%), follicular (8.6%), medullary (7.0%) and anaplastic (1.6%) carcinomas. The ASR for thyroid carcinoma was 1.289 (0.627 for men, 1.59 for women), with the highest incidence rate in Kerman (ASR 1.643) and the lowest incidence rate in Golestan (ASR 0.735). For the 6 cases of adrenal cancer, 4 were neuroblastoma and 2 were pheochromocytoma. CONCLUSIONS: Iran was considered as an endemic, iodine-deficient area, until fairly recently. Iodinization of salt has been started about 12 years ago, in the nation. Considering the effect of improvement in the iodine intake in previously deficient communities, which is associated with an increase in the incidence of papillary carcinoma compared to other histologic types, the frequency and distribution of histologic types of thyroid carcinoma was closer to what can be seen in iodine-rich areas.

Pheochromocytoma - an institutional experience

The present study envisages evaluating the clinical presentation, diagnosis, management, with particular reference to pharmacological and anesthetic considerations, and outcome in patients with pheochromocytoma. A retrospective study. The study was conducted at the Department of Surgery, Kasturba Medical College Hospital, Manipal, India from January 1, 1996 to December 31, 2003. Seventeen patients diagnosed to have pheochromocytoma were included for this study. Our assessment of age at presentation, sex, presenting complaints with details of hypertension [HT], diagnostic tests, surgical procedure and follow-up was based on patient records. Peak incidence was observed in 40-50 years age group, with male preponderance. All the patients had diastolic HT of more than 100 mm of Hg at presentation. Systolic blood pressures [SBP] were between 150 mm Hg to 230 mm Hg, with mean of 170 mm Hg; nine patients presented with persistent HT and seven had paroxysmal HT. One patient infact presented with hypotension due to septic shock and was incidentally detected to have an adrenal tumor. Computed tomography [CT] scan and urinary levels of vanillylmandelic acid [VMA] were mainstay of diagnosis. These patients had adequate preoperative control of HT. All patients underwent exploration of the tumor by an extraperitoneal approach. 70% tumors were on the right and 30% were on the left side. Histopathologically, all the tumors were benign except for one, which was malignant. An average of three to five units of blood transfusion was required and mean operating time was 4.5 hours. Six patients had extensive intra-operative blood pressure [BP] fluctuation, but were adequately managed by the anesthetic team. One patient with post-operative pulmonary edema died in our series; rest all the patients are on regular follow-up, till date and doing well. Pheochromocytoma is an important cause of endocrine HT. Elevated urinary VMA is diagnostic of this tumor with imaging studies used to localize the tumor. Adrenalectomy results in complete cure of HT with an excellent long-term outcome

Feocromocitoma del órgano de Zuckercandl

Se presenta una mujer de 23 años con hipertensión arterial, cefalea, visión borrosa, palpitaciones y masa hipogástrica, a quien se le hizo diagnóstico de feocromocitoma del órgano de Zuckercandl. Durante el acto operatorio desarrolla arritmia cardíaca y colapso circulatorio. Se revisa el tema de feocromacitoma haciendo énfasis en el originado en el órgano de Zuckercandl.

Tratamiento quirúrgico del feocromocitoma / Surgical treatment of the pheochromocytoma

Se presentan 13 pacientes operados por feocromocitoma. El diagnóstico se hizo en todos los casos con la determinación de ácido vainillilmandélico en orina. Inicialmente, los tumores se localizaron con urograma excretor y retroneumoperitoneo. A partir de 1979 se utilizó la tomografía axial computada en 11 casos con buenos resultados. También fue de utilidad el centellograma con MIBG I 131. Se obtuvo un adecuado bloqueo adrenérgico preoperatorio tanto con dibencilina como con prazosin. Doce pacientes presentaron feocromocitomas benignos, 3 de ellos bilaterales, y todos fueron resecados. El único caso de tumor maligno fue irresecable por invasión de la vena cava inferior y fue tratado con 2 dosis terapéuticas de MIBG I 131, manteniéndose asintomático a 15 meses de la última aplicación.