Jessica Brzana

Factors Associated with Biochemical Remission after Microscopic Transsphenoidal Surgery for Acromegaly

Objectives To analyze surgical outcomes and predictive factors of disease remission in acromegaly patients who underwent microscopic transsphenoidal surgery (TSS) for a growth hormone (GH)-secreting adenoma. Design A 6-year retrospective review of 86 consecutive acromegaly surgeries. Setting Procedures performed at a single institution by a single surgeon. Participants Seventy acromegaly patients. Main Outcome Measures Demographic information, preoperative laboratory values, tumor imaging data, and morphological and immunohistochemical data were collected. Predictive values using the latest and most stringent biochemical remission criteria were determined using univariate and multivariate statistical analyses. Results Remission rate for 59 (18 males) acromegaly patients meeting the study inclusion criteria was 52.5%. Remission rates for micro- and macroadenomas were 81.8% and 45.8%, respectively. Patients of older age, with a smaller tumor, lower Knosp grade, lower preoperative GH, and insulinlike growth factor 1 levels were more likely to achieve remission. Remission rate decreased significantly with repeat surgeries. Those patients with adenomas that stained positive for somatostatin receptor subtype 2A were less likely to experience tumor recurrence and more likely to respond to medical treatment with persistent or elevated GH hypersecretion. Conclusions Microscopic TSS continues to be a viable means for treating acromegaly patients. Patients should be followed long term.

FRAX score in acromegaly: does it tell the whole story?

being treated with pentoxifylline (600 mg bd), but again with no improvement. One year ago, the patient reported nonspecific abdominal pain without any associated symptoms and with no evidence of connective tissue disease. An abdominal ultrasound showed bilateral hydronephrosis. Computerized tomography and magnetic resonance imaging revealed bilateral periureteric masses reaching the pelvis, with a large aqueous component (Fig. 1). A biopsy showed ureteric myxoedema situated between the adventitia and muscle layer of both ureters. The sample, from the periureteric sheath, was composed of fibroadipose tissue infiltrated by a basophilic granular material with a lax aspect that corresponded to mucin deposits separating the individual collagen bundles. Also, visible were several foci of an accompanying lymphoplasmacytic inflammatory infiltrate (Fig. 1). The patient had no history of any prior ureteric process such as nephrolithiasis or stenting, and renal function was normal at that time. A double-J catheter was placed to reduce the hydronephrosis. Extrathyroid manifestations of Graves’ disease are rare, with the exception of exophthalmos. Pretibial myxoedema or thyroid dermopathy can also occasionally be found in Hashimoto’s thyroiditis. These lesions are usually asymptomatic and take the form of benign plaques or nodules, remitting in 50% of cases, and which mainly pose an aesthetic problem. Their appearance is usually preceded, sometimes by several years, by severe ophthalmopathy. The reason for their preferentially pretibial location is unknown, and factors such as local trauma, surgical scars or venous stasis have all been suggested. Severe forms of elephantiasic dermopathy have also been reported. Other very unusual sites include the toes and feet, shoulders, back, neck and nose. Exophthalmos, pretibial myxoedema and osteoarthropathy comprise the classic triad of the EMO syndrome. Myxoedema is characterized by the accumulation of acid mucopolysaccharides (glycosaminoglycans) in the dermis and subcutaneous tissue. The pathogenesis is not well known. All patients with myxoedema have high concentrations of TSH receptor antibodies, and receptors for these antibodies have been found in fibroblasts from the affected tissue. An initial immunological explanation could be that stimulation of these fibroblasts would result in greater production of hyaluronic acid. A second cellular theory is that antigen-sensitized T cells could infiltrate the dermis, releasing cytokines that stimulate the production of acid mucopolysaccharides. Various different therapeutic strategies have been suggested for patients with severe myxoedema, including topical glucocorticoids in an occlusive dressing or their subcutaneous injection, intralesional injection of octeotride, or pentoxifylline combined with steroids, as well as compression bandages or decongestive physiotherapy. None of the treatments used in our patient proved effective. Concerning the bilateral ureteric lesions, the differential diagnosis is retroperitoneal fibrosis, a process that can be associated with autoimmune thyroid disorders. The histology showed positive staining for Alcian Blue/PAS and colloidal iron, as well as IgG4. However, the clinical course and the radiological images during the follow-up rule out the presence of retroperitoneal fibrosis. The extent was just periureteric bilaterally with no progression. There was no retroperitoneal perivascular involvement or ureteral medialization, which is the classic sign of retroperitoneal fibrosis. MRI T2-weighted sequences always showed hyperintensity, whereas in retoperitopeal fibrosis, there are phases of hyperintensity followed by hypointensity, and there was no involvement of other organs (pancreas, biliary tract, mediastinum, etc.). Thus, our patient is the first to be reported with Graves’ disease and bilateral, ureteric myxoedema.

Serum IGF-1 in the diagnosis of acromegaly and the profile of patients with elevated IGF-1 but normal glucose-suppressed growth hormone.

OBJECTIVE To report the utility of insulin-like growth factor-1 (IGF-1) as a single biomarker for establishing the diagnosis of acromegaly and to examine the clinical and biochemical profile of patients with an elevated IGF-1 in whom a diagnosis of acromegaly could not be confirmed by means of the oral glucose tolerance test (OGTT). METHODS Between the years 1999 and 2010, we identified 101 patients who underwent pituitary surgery and had histologically proven somatotroph adenomas (Group 1, Gr 1). We selected 149 patients with non-growth hormone (GH) secreting pituitary macroadenomas (Gr 2, n = 97) and microadenomas (Gr 3, n = 52) to serve as control subjects. In addition, we identified 34 patients with elevated IGF-1values in whom acromegaly could not subsequently be proven by the OGTT (Gr 4). RESULTS IGF-1 was elevated in all patients with acromegaly prior to therapy with a median (range) standard deviation score (SDS) of +9.52 (+2.34 to +9.2), compared to SDS -1.46 (-2.91 to +2.17) and -1.22 (-2.8 to +1.58) in Gr 2 and 3, respectively (P<0.001). IGF-1 SDS values were +3.28 (+2.05 to +6.1), and IGF-1 was less than twice the upper limit of normal in all patients in Gr 4. OGTT was performed in 51 of the 101 acromegalic patients. The nadir GH in these patients was 4.01 (0.2 to 46.7) in comparison with 0.2 (<0.05 to 0.6) in Gr 4 (P<0.001). CONCLUSION Elevated IGF-1 levels, alone, are sufficient to establish a diagnosis of acromegaly in the majority of clinically suspected cases. The OGTT may be useful to obtain corroborative evidence when there is modest elevation of IGF-1 with absent or equivocal clinical features.

Monitoring Patient Improvement Parameters following Pasireotide Treatment in Cushing’s Disease

Cushings disease (CD) is a disorder in which chronic excess adrenocorticotropic hormone production is associated with multiple comorbidities and diminished quality of life. Postsurgical monitoring is important, and newer therapies are available for the management of surgical failure or disease recurrence. In this clinical case, we illustrate the importance of the nursing role in long-term management of CD, particularly as nurses may be the first point of contact for patients with CD. Alertness to disease signs and symptoms is crucial for timely diagnosis and improved outcomes. Successful therapy for CD requires careful monitoring of hormonal control, metabolic parameters, and therapy complications. Ongoing management requires lifelong monitoring of metabolic parameters, of side effects of treatment, and of signs of disease recurrence. Appropriate referrals may be required to facilitate overall outcomes and patient wellbeing. This patient was enrolled in a Phase III trial that was registered in the USA with clinicaltrial.gov.

Polycystic ovarian syndrome and Cushing's syndrome: a persistent diagnostic quandary

OBJECTIVE To retrospectively review institutional records of female patients of reproductive age with Cushings disease (CD) and determine if and how many had been previously diagnosed as having solely polycystic ovarian syndrome (PCOS). To determine whether clinical patterns might be useful in identifying appropriate candidates for hypercortisolism screening in women suspected of PCOS. STUDY DESIGN The study included 50 patients with pathologically proven CD at Oregon Health & Science University, Northwest Pituitary Center between 2006 and 2011. Physical, clinical, and biochemical features for hypercortisolism were compared. RESULTS Of 50 patients with pathologically proven CD, 26 were women of reproductive age. Of these, half had previously been diagnosed with and treated initially solely for PCOS. Hirsutism and menstrual abnormalities were more common in the group with an initial PCOS diagnosis than in the group with an initial CD diagnosis. CONCLUSIONS Prolonged exposure to hypercortisolism has been linked with increased mortality and morbidity. Tests for hypercortisolism in all the PCOS cases we report led to an appropriate CD diagnosis. Future research should focus on when and which (if not all) women with suspected PCOS should be tested for hypercortisolism.

Acromegaly, Awareness Is Paramount for Early Diagnosis: Highlights of Diagnosis and Treatment Challenges

To highlight clinical features of acromegaly at presentation, to facilitate early diagnosis. To review the multi-modal approach in the management of acromegaly: surgical indications and medical treatment.

A Nontraumatic Hip Fracture in a Young Woman: Cushing’s Disease—Consequences of a Late Diagnosis and Treatment Highlights

To describe the late diagnosis of hypercortisolemia and associated severe clinical complications. To review the multimodal approach in the management of Cushing’s disease after failed transsphenoidal surgery: surgical re-exploration and the role of medical treatment.