An unusual pituitary pathology

peer reviewe

Multivariable Prediction Model for Biochemical Response to First-Generation Somatostatin Receptor Ligands in Acromegaly

CONTEXT: First-generation somatostatin receptor ligands (fg-SRLs) represent the mainstay of medical therapy for acromegaly, but they provide biochemical control of disease in only a subset of patients. Various pretreatment biomarkers might affect biochemical response to fg-SRLs. OBJECTIVE: To identify clinical predictors of the biochemical response to fg-SRLs monotherapy defined as biochemical response (insulin-like growth factor (IGF)-1 ≤ 1.3 × ULN (upper limit of normal)), partial response (>20% relative IGF-1 reduction without normalization), and nonresponse (≤20% relative IGF-1 reduction), and IGF-1 reduction. DESIGN: Retrospective multicenter study. SETTING: Eight participating European centers. METHODS: We performed a meta-analysis of participant data from 2 cohorts (Rotterdam and Liège acromegaly survey, 622 out of 3520 patients). Multivariable regression models were used to identify predictors of biochemical response to fg-SRL monotherapy. RESULTS: Lower IGF-1 concentration at baseline (odds ratio (OR) = 0.82, 95% confidence interval (CI) 0.72-0.95 IGF-1 ULN, P = .0073) and lower bodyweight (OR = 0.99, 95% CI 0.98-0.99 kg, P = .038) were associated with biochemical response. Higher IGF-1 concentration at baseline (OR = 1.40, (1.19-1.65) IGF-1 ULN, P ≤ .0001), the presence of type 2 diabetes (oral medication OR = 2.48, (1.43-4.29), P = .0013; insulin therapy OR = 2.65, (1.02-6.70), P = .045), and higher bodyweight (OR = 1.02, (1.01-1.04) kg, P = .0023) were associated with achieving partial response. Younger patients at diagnosis are more likely to achieve nonresponse (OR = 0.96, (0.94-0.99) year, P = .0070). Baseline IGF-1 and growth hormo

Acromegaly at diagnosis in 3173 patients from the Liège Acromegaly Survey (LAS) Database

Acromegaly is a rare disorder caused by chronic growth hormone (GH) hypersecretion. While diagnostic and therapeutic methods have advanced, little information exists on trends in acromegaly characteristics over time. The Liège Acromegaly Survey (LAS) Database, a relational database, is designed to assess the profile of acromegaly patients at diagnosis and during long-term follow-up at multiple treatment centers. The following results were obtained at diagnosis. The study population consisted of 3173 acromegaly patients from ten countries; 54.5% were female. Males were significantly younger at diagnosis than females (43.5 vs 46.4 years; P 3100 patients is the largest international acromegaly database and shows clinically relevant trends in the characteristics of acromegaly at diagnosis

Extensive inflammatory pseudotumor of the pituitary.

A 40-yr-old female presented with an extensive lesion of the sellar area and the sphenoid sinus, spreading to the optic nerves and associated with pachymeningitis. Histological findings were consistent with an inflammatory pseudotumor, and steroid treatment allowed the disappearance of all the lesions. Inflammatory pseudotumors of the pituitary are very rare. This case appears unique with regard to the extension of the lesions and the dramatic response to medical treatment. The differential diagnosis of inflammatory lesions of the pituitary is difficult. It relies mainly on histological analysis and includes sarcoidosis, Wegener's granulomatosis, histiocytosis (Langerhans, Rosai-Dorfman, and Erdheim-Chester diseases) and lymphocytic hypophysitis

Primary hyperparathyroidism: etiology, diagnosis and treatment

peer reviewedPrimary hyperparathyroidism is a not so infrequent disease that is often overlooked by physicians. Its incidence is estimated to be about 28 in 100.000 subjects, mainly women over seventy years old. Three to four percents of women over seventy are affected. Hyperparathyroidism can be isolated or be a component of a more complex syndrome like multiple endocrine neoplasms (MEN). Patients can be asymptomatic or present with symptoms like asthenia, hypertension or nephrolithiasis. The biological investigation requires the combined measurements of plasmatic ionized calcium and parathormone. In some cases, more specific explorations like calcium loading tests have to be performed in order to confirm the diagnosis.L'hyperparathyroïdie primaire est une maladie trop souvent méconnue. Son incidence est estimée à 28/100.000, soit environ 2.800 nouveaux cas par an en Belgique. La fréquence de l'hyperparathyroïdie augmente avec l'âge. En effet, 50 % des cas sont rencontrés après l'âge de 70 ans et 3 à 4 % des femmes au-delà de 70 ans sont touchées par l'affection. Cette affection peut être sporadique ou survenir dans un cadre étiologique plus complexe tel que les polyendocrinopathies ou la neurofibromatose. L'attention vers ce diagnostic doit être attirée par une symptomatologie, souvent bâtarde, de fatigue (parfois isolée), de polyurie, de polydipsie, d'hypertension artérielle ou la présence de lithiases rénales. Lorsque le diagnostic d'hyperparathyroïdie est renteu, il convient de localiser la ou les glande(s) pathologique(s) et de définir le contexte étiologique qui pourra déterminer le choix thérapeutique le plus approprié. Une étude biologique simple sera réalisée devant toute suspicion d'hyperparathyroïdie. Dans certains cas, le diagnostic sera aidé par la réalisation d'un test de surcharge calcique qui peut permettre de confirmer l'hyperparathyroïdie et d'apprécier son degré d'autonomisation

Treatment of macroprolactinomas with the long-acting and repeatable form of bromocriptine (Parlodel LAR): report on 29 cases.

Twenty-nine patients with macroprolactinomas were treated by monthly intramuscular injections of the long-acting and repeatable form of bromocriptine (Parlodel-LAR) in doses ranging from 50-150 mg. They were divided into two groups: group I consisted of 22 patients who received Parlodel LAR before transsphenoidal adenomectomy; group II was composed of 7 patients with earlier neurosurgery and of 2 patients from group I not cured by transsphenoidal adenomectomy. Duration of therapy varied from 1-12 months, and a total of 104 injections was given. At nadir day, serum PRL levels were situated between less than 1% and 43% of pretreatment values. At day 28 after the first injection, serum PRL levels varied between less than 1% to 139% of initial values. No difference could be detected between the two groups regarding the percent of PRL inhibition. Long-term treatment with Parlodel-LAR resulted in a sustained inhibition of PRL secretion, except for 1 case. Resumption of menstrual cycles occurred in 4 out of 15 women and correction of hypogonadism in 4 out of 14 men. Amelioration of disturbed visual fields was recorded in 3 out of 8 patients. Diminution of the adenoma volume was radiologically documented in 14 out of 22 cases. Only few and mild side effects were recorded. One patient with partial adrenal deficiency suffered from a syncope, but this was prevented by hydrocortisone supplementation during the subsequent Parlodel-LAR administration. In conclusion, Parlodel-LAR proved effective in the treatment of macroprolactinomas, achieving rapid inhibition of PRL secretion, and in some patients amelioration of hypopituitarism, reduction in tumor size, and improvement in visual fields, and caused no serious side effects. It is a valuable preparation to surgery and can also be used in long-term medical therapy