Apoplexia subclínica em tumores pituitários

Apoplexia pituitária aguda é fenômeno relativamente raro, mesmo em macroadenomas. No entanto, a presença de áreas necro-hemorrágicas intratumorais nestes tumores que não se relacionam a qualquer sintomatologia aguda parece ser bem mais frequente do que se imaginava na era da tomografia. Com o advento da ressonância magnética estas áreas podem ser facilmente diagnosticadas pré-operatoriamente. Dentre os 40 últimos macroadenomas operados e examinados com RMN, 10 possuíam áreas apopléticas subclínicas em seu interior. Sete destes tumores eram não secretores, 2 secretores de GH e um de prolactina. O quadro clínico incluiu, além daqueles correspondentes à eventual secreção endócrina, perda visual progressiva (sem história de piora abrupta ou rápida) (n=8) e cefaléia (n=3). Após a remoção cirúrgica destes tumores e descompressão do aparato óptico, obtivemos melhora visual em 6 pacientes e em 2 a visão permaneceu inalterada. A cefaléia desapareceu em 2 casos. A presença de áreas apopléticas nestes macrotumores bem como sua ausência em séries de microtumores relatadas na literatura sugere que se relacionam mais ao tamanho do tumor do que ao seu caráter secretor ou não, o que é compatível com a provável natureza (insuficiência vascular) da apoplexia subclínica nesses casos

Cavernous sinus invasion by pituitary macroadenomas: neuroradiological, clinical and surgical correlation

The classical imaging gold-standard for this diagnosis is the presence of tumor lateral to the carotid artery. Seventeen patients with pituitary macroadenomas with intraoperative confirmation of cavernous sinus invasion were studied with MRI. Only 8 patients had tumor lateral to the carotid artery; 13 had tumor within the carotid syphon and all lacked the ring enhancement of the medial wall of the cavernous sinus. In 10 patients, widening of the posterior double leaflets of the cavernous sinus could be. All patients were operated by the transesphenoidal route. Only one patient was cured by surgery alone. Only 3 patients disclosing the above mentioned MRI features were identified in a series of 250 patients and did not have cavernous sinus invasion. The present criteria proved to be useful in the pre-operative diagnosis of cavenous sinus invasion and patients' counselling. Pre-operative diagnosis of cavernous sinus invasion of pituitary tumors has a great impact in the management of such patients

EVALUATION OF THE GONADOTROPHIC RESPONSIVENESS OF THE PITUITARY TO ACUTE AND PROLONGED ADMINISTRATION OF LH/FSH-RELEASING HORMONE (LH-RH) IN NORMAL FEMALES AND MALES

ABSTRACT In normal females, the injection of 25 μg of LH-RH (acute test) induced a greater LH and FSH release from the pituitary in the mid-luteal than in the mid-follicular phase of the menstrual cycle. In normal males, the responsiveness to 25 μg LH-RH was greater than that in females at mid-follicular but not at the luteal stage. The pituitary response to the prolonged LH-RH infusion (0.21 μg/min/8 h) was similar in both phases of the cycle of the females with a decline in serum gonadotrophins after the 4th hour and was paralleled by a significant increase of plasma oestradiol levels. In males the LH, but not the FSH secretion was lower as compared to female subjects, and gonadotrophin levels did not show a fall during the infusion. The acute injection of 25 μg LH-RH at the end of a prolonged infusion induced the same response in the female subjects in both phases of the cycle. In males, the acute test following prolonged infusion produced a similar LH secretion, but a lower FSH response than in females. The comparison of the acute test alone and that preceded by a prolonged LH-RH infusion, demonstrated that, in females, the only significant differences consisted of a greater LH secretion in the former test in the mid-luteal phase. In males there was greater FSH secretion in the acute isolated test than when this test was given after the prolonged infusion

Gêmeas idênticas discordantes para a doença de Cushing: relato de caso Identical twins discordant for Cushing's disease: case report

A doença de Cushing é rara em crianças e sua ocorrência em gêmeos é ainda mais rara. O presente estudo relata a ocorrência de gêmeas idênticas discordantes quanto à doença de Cushing. Uma das gêmeas, desenvolveu síndrome de Cushing aos 10 anos de idade. Sua investigação demonstrou cortisol urinário elevado, ACTH sérico elevado e testes de supressão com dexametasona compatíveis com doença de Cushing. A RMN mostrou macroadenoma hipofisário que foi operado por via transesfenoidal. O estudo imuno-histoquímico confirmou a presença de células produtoras de ACTH. A paciente entrou em remissão clínica e laboratorial desde a cirurgia. Após o desaparecimento dos estigmas do Cushing, a paciente reiniciou ganho estatural, mas permanece menor que sua irmã gêmea. Esta última permanece saudável 4 anos após o início da doença de sua irmã. Este é o terceiro caso do gênero relatado na literatura. Estes achados sugerem que fatores não genéticos estão envolvidos na gênese da doença de Cushing.<br>Cushing's disease is rare in children and its ocurrence in identical twins is extremely rare. This paper reports on identical twins discordant for Cushing's disease. One of them first presented with a cushingoid phenotype by the age of 10. Her evaluation showed an increased urinary free-cortisol and serum ACTH. Her pattern in the dexametazone supression tests was compatible with Cushing's disease. MRI disclosed a pituitary macroadenoma which was removed by the transesphenoidal approach. Immunohistochemical studies of the tumor showed the presence of ACTH-producing cells. The patient went into clinical and laboratorial remission after surgery. She re-started to grow after the disappearance of the Cushing's phenotype but she is still shorter than her healthy sister. The latter remains disease-free 4 years after her sister's diagnosis. This represents the third such case reported in the literature. Our findings suggest that acquired factors may be responsible for the genesis of Cushing's disease

Apoplexia pituitária seguida de remissão endócrina: relato de dois casos Pituitary apoplexy followed by endocrine remission: report of two cases

A apoplexia pituitária é evento raro e a ocorrência de remissão endócrina em pacientes portadores de tumores secretores é ainda mais incomum. O presente estudo relata os casos de dois pacientes portadores de macroadenomas (um com doença de Cushing e outro com acromegalia) nos quais houve remissão endócrina após apoplexia tumoral. A primeira paciente era portadora de doença de Cushing e teve episódio ictal espontâneo de cefaléia e vômitos, após o qual iniciou remissão endócrina. Como houvesse persistência de imagem de macroadenoma à ressonância magnética, a paciente foi submetida a cirurgia transesfenoidal, sendo encontrado apenas cisto hemorrágico hipertensivo, sem sinais de tumor. O segundo paciente apresentava acromegalia e enquanto realizava um teste de LHRH teve evento agudo de cefaléia e vômitos, sem perda visual e instalação de diabetes insipidus. A tomografia computadorizada de sela túrcica mostrou sinais de sangue. Como não houve quadro visual agudo, o paciente foi seguido com exames de imagens seriadas, que demonstraram o desaparecimento completo da lesão e o aparecimento de sela vazia. A avaliação endócrina mostrou remissão da acromegalia. Tendo em vista a tendência à recidiva já documentada na literatura, esses pacientes devem continuar em seguimento a longo prazo.<br>Pituitary apoplexy is rare and endocrine remission in patients with apopletic secreting pituitary adenomas is even rarer. This study reports on two patients with pituitary macroadenomas (one with Cushing's disease and the other with acromegaly) in whom endocrine remission occurred after apoplexy. The first patient had Cushing's disease and had an ictus of headache and vomiting after which she started a progressive remission of hypercortisolism. A post-apoplexy MRI disclosed persistence of a sellar and supra-sellar mass. She was submitted to transesphenoidal surgery. An hypertensive hemorhagic cyst was found with no tumor. The second patient had acromegaly. While performing a LHRH-stimulation test he had an ictus of headache, vomiting, no visual loss and appearance of diabetes insipidus. A CT scan disclosed an intrasellar hematoma. Despite the size of the tumor and since there was no visual impairment, this patient was followed up without surgery. Imaging follow-up showed a progressive shrinkage and disappearance of the mass, which was corroborated by endocrine remission. A high rate of recurrence is reported in such patients in the literature. Both patients are being currently followed-up on a long-term basis