ACTH-producing remnants following apoplexy of an ACTH-secreting pituitary macroadenoma

Department of Endocrinology, Mount Kisco Medical Group, 90 South Bedford Road, Mount Kisco, NY, 10549, USA, .
Pituitary (Impact Factor: 3.2). 08/2010; 15(S1). DOI: 10.1007/s11102-010-0247-2
Source: PubMed


Describe a case of apoplexy of an ACTH-producing pituitary adenoma which resulted not only in an empty sella with concurrent hypothyroidism, hypoprolactinemia, and hypogonadism but persistent hypercortisolemia from two distinct extrasellar remnants of the original adenoma. Review the literature to identify other similar cases. The patient's medical history, physical exam, lab data, imaging exams and histopathological results were analyzed and compiled into a case report, and an extensive review of the literature was performed. Endocrinological data revealed hypercortisolism and an elevated ACTH with an otherwise suppressed pituitary axis. A pituitary MRI showed a macroadenoma in the left cavernous sinus in addition to an empty sella. An octreotide scan revealed lesions in the left sella turcica and the right sphenoid sinus. Tissue samples of both lesions stained positive for ACTH and negative for GH, prolactin, FSH, LH, and TSH. The lesions were surgically removed, and the patient treated with radiation and ketoconazole. This resulted in a significant decrease in ACTH and cortisol as well as a marked improvement in blood glucose control. The review of literature revealed the absence of any similar cases in the past. The patient presented with apoplexy of an ACTH-secreting pituitary macroadenoma with two hormonally active extrasellar remnants. Several cases in the literature describe recurrence of Cushing's disease following infarction of ACTH-secreting adenomas. This is the first documented case of infarction of an ACTH-producing adenoma resulting in two distinct ACTH-producing remnants without recurrence of the original adenoma.

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    ABSTRACT: 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.
    Arquivos brasileiros de endocrinologia e metabologia 08/2013; 57(6):486-9. DOI:10.1590/S0004-27302013000600012 · 0.84 Impact Factor