RESUMO

Cushing's disease (CD) is usually caused by secretion of ACTH by a pituitary corticotroph microadenoma. Nevertheless, 7%-20% of patients present with ACTH-secreting macroadenomas. Our aim is to report a 36-year-old female patient with CD due to solid-cystic ACTH-macroadenoma followed up during 34 months. The patient presented spontaneous remission due to presumed asymptomatic tumor apoplexy. She showed typical signs and symptoms of Cushing's syndrome (CS). Initial tests were consistent with ACTH-dependent CS: elevated urinary free cortisol, abnormal serum cortisol after low dose dexamethasone suppression test, and elevated midnight salivary cortisol, associated with high plasma ACTH levels. Pituitary magnetic resonance imaging (MRI) showed a sellar mass of 1.2 x 0.8 x 0.8 cm of diameter with supra-sellar extension leading to slight chiasmatic impingement, and showing hyperintensity on T2-weighted imaging, suggesting a cystic component. She had no visual impairment. After two months, while waiting for pituitary surgery, she presented spontaneous resolution of CS. Tests were consistent with remission of hypercortisolism: normal 24-h total urinary cortisol and normal midnight salivary cortisol. Pituitary MRI showed shrinkage of the tumor with disappearance of the chiasmatic compression. She has been free from the disease for 28 months (without hypercortisolism or hypopituitarism). The hormonal and imaging data suggested that silent apoplexy of pituitary tumor led to spontaneous remission of CS. However, recurrence of CS was described in cases following pituitary apoplexy. Therefore, careful long-term follow-up is required.

Assuntos

RESUMO

Cushing's disease (CD) is usually caused by secretion of ACTH by a pituitary corticotroph microadenoma. Nevertheless, 7%-20% of patients present with ACTH-secreting macroadenomas. Our aim is to report a 36-year-old female patient with CD due to solid-cystic ACTH-macroadenoma followed up during 34 months. The patient presented spontaneous remission due to presumed asymptomatic tumor apoplexy. She showed typical signs and symptoms of Cushing's syndrome (CS). Initial tests were consistent with ACTH-dependent CS: elevated urinary free cortisol, abnormal serum cortisol after low dose dexamethasone suppression test, and elevated midnight salivary cortisol, associated with high plasma ACTH levels. Pituitary magnetic resonance imaging (MRI) showed a sellar mass of 1.2 x 0.8 x 0.8 cm of diameter with supra-sellar extension leading to slight chiasmatic impingement, and showing hyperintensity on T2-weighted imaging, suggesting a cystic component. She had no visual impairment. After two months, while waiting for pituitary surgery, she presented spontaneous resolution of CS. Tests were consistent with remission of hypercortisolism: normal 24-h total urinary cortisol and normal midnight salivary cortisol. Pituitary MRI showed shrinkage of the tumor with disappearance of the chiasmatic compression. She has been free from the disease for 28 months (without hypercortisolism or hypopituitarism). The hormonal and imaging data suggested that silent apoplexy of pituitary tumor led to spontaneous remission of CS. However, recurrence of CS was described in cases following pituitary apoplexy. Therefore, careful long-term follow-up is required.

A doença de Cushing (DC) é usualmente causada por um microadenoma produtor de ACTH. Entretanto, 7%-20% dos pacientes apresentam um macroadenoma. O objetivo deste trabalho é reportar uma paciente de 36 anos, feminina, com diagnóstico de DC devido a macroadenoma hipofisário sólido-cístico com seguimento de 34 meses que apresentou remissão espontânea presumidamente em decorrência de uma apoplexia tumoral assintomática. Inicialmente, ela apresentava sinais e sintomas típicos da síndrome de Cushing (SC). Na admissão, os testes foram consistentes com o diagnóstico de SC ACTH-dependente: cortisol urinário livre de 24h elevado, não supressão do cortisol sérico após dose baixa de dexametasona e cortisol salivar noturno elevado, associado a concentrações elevadas do ACTH plasmático. Ressonância magnética (RM) de hipófise revelou uma massa selar de 1.2 x 0.8 x 0.8 cm com extensão suprasselar levando a uma discreta compressão do quiasma óptico e mostrando região de hipersinal na imagem ponderada em T2 sugerindo um componente cístico. A paciente não apresentava queixas visuais. Após dois meses, enquanto aguardava o tratamento cirúrgico, a paciente apresentou remissão espontânea da SC. A repetição dos exames indicou remissão do hipercortisolismo: normalização do cortisol urinário livre de 24h e normalização do cortisol salivar noturno. Nova RM de hipófise revelou redução do volume tumoral com desaparecimento da compressão quiasmática. A paciente permanece livre da doença por 28 meses (sem hipercortisolismo ou hipopituitarismo). Os dados hormonais e de imagem sugerem que tenha ocorrido uma apoplexia tumoral assintomática, levando à remissão espontânea da SC. Entretanto, como há relatos de recorrência após apoplexia hipofisária, cuidadoso seguimento a longo prazo faz-se necessário.

Assuntos

RESUMO

Pituitary tumor apoplexy is a medical emergency due to acute infarction or hemorrhage in the pituitary gland. In this review, the authors discuss the sellar anatomy, the pituitary gland and adenomas' vascularization and the general aspects of the syndrome such as its ethiopatogenesis, predisposing factors, clinical features, treatment and prognosis.

Assuntos

RESUMO

Pituitary tumor apoplexy is a medical emergency due to acute infarction or hemorrhage in the pituitary gland. In this review, the authors discuss the sellar anatomy, the pituitary gland and adenomas' vascularization and the general aspects of the syndrome such as its ethiopatogenesis, predisposing factors, clinical features, treatment and prognosis.

A apoplexia em tumor hipofisário é uma emergência médica decorrente do infarto agudo ou hemorrágico na glândula hipófise. Nesta revisão os autores discutem a anatomia da região selar, a vascularização da hipófise e adenomas hipofisários, e demais aspectos da síndrome como etiopatogenia, fatores predisponentes, quadro clínico, tratamento e prognóstico.

Assuntos

RESUMO

Pituitary apoplexy is a rare and life-threatening clinical condition caused by hemorrhage and/or infarction of the pituitary gland or adenoma. Although pituitary apoplexy is usually spontaneous, it has been associated with numerous precipitating factors, such as bromocriptine use. However, reports of pituitary apoplexy during cabergoline therapy are scarce. We report three patients with cystic macroprolactinomas who developed pituitary apoplexy during cabergoline treatment.

Assuntos

RESUMO

We report a man in whom a 15 cm. renal tumor was excised at the age of 49. The pathological examination showed a clear cell carcinoma. Five years later, he presented with headache, vomiting and unilateral palpebral ptosis. Imaging studies showed a sellar tumor with pituitary apoplexy. The tumor was excised and the pathological study disclosed a clear cell tumor, positive for vimentin, cytokeratins AE1 and AE3 and immunohistochemically negative for LH, TSH, ACTH and GH. Considering the similar histopathological features, it was considered as a metastasis of the renal tumor. The patient was supplemented with thyroid, adrenal and gonadal hormones. Seven years later, he presented a new tumor in the remaining kidney, that corresponded to a cystic papillary renal cell carcinoma. Afterwards, he presented a transitional urinary bladder tumor. Mortality associated to renal cell tumors is 90% at 5 years, and pituitary metastases are extraordinarily uncommon.

Assuntos

RESUMO

Hypophyseal apoplexy is not always easy to confirm morphologically or analytically. We present a patient with Cushing's syndrome due to hypophyseal apoplexy and optochiasmic pachyarachnoiditis which produced serious visual alterations. The adenoma necrotized behind an area of hypophyseal apoplexy which cured the patient's Cushing's disease. The second case was admitted with hypophyseal apoplexy, presenting hypophyseal tumoration with signs of hemorrhage and subsequent panhypopituitarism.

Assuntos