RESUMEN

Pituitary adenomas are very common representing 18.1% of all brain tumors and are the second most common brain pathology. Transsphenoidal surgery is the mainstay of treatment for all pituitary adenomas except for prolactinomas which are primarily treated medically with dopamine agonists. A thorough endocrine evaluation of pituitary adenoma preoperatively is crucial to identify hormonal compromise caused by the large sellar mass, identifying prolactin-producing tumors and comorbidities associated with Cushing and acromegaly to improve patient care and outcome. Transsphenoidal surgery is relatively safe in the hands of experienced surgeons, but still carries a substantial risk of causing hypopituitarism that required close follow-up in the immediate postoperative period to decrease mortality. A multidisciplinary team approach with endocrinologists, ophthalmologists, and neurosurgeons is the cornerstone in the perioperative management of pituitary adenomas.

RESUMEN

CONTEXT: Prolactin, a hormone synthesized by the anterior pituitary gland demonstrates promise as a neuroprotective agent, however, its role in humans and in vivo during injury is not fully understood. OBJECTIVE: To investigate whether elevated levels of prolactin attenuate injury to the retinal nerve fiber layer (RNFL) following compression of the optic chiasm in patients with a prolactin secreting pituitary macroadenoma (i.e., prolactinoma). DESIGN SETTING AND PARTICIPANTS: A retrospective cross-sectional study of all pituitary macroadenoma patients treated at a single institution between 2009 and 2019. MAIN OUTCOME MEASURE(S): Primary outcome measures included RNFL thickness, mean deviation, and prolactin levels for both prolactin-secreting and non-secreting pituitary macroadenoma patients. RESULTS: Sixty-six patients met inclusion criteria for this study (14 prolactin-secreting and 52 non-secreting macroadenoma patients). Of 52 non-secreting macroadenoma patients, 12 had moderate elevation of prolactin secondary to stalk effect. Patients with moderate elevation in prolactin demonstrated increased RNFL thickness compared to patients with normal prolactin levels (p < 0.01). Additionally, a significant positive relation between increasing levels of prolactin and RNFL thickness was identified in patients with moderate prolactin elevation (R = 0.51, p-value = 0.035). No significant difference was identified between prolactinoma patients and those with normal prolactin levels. CONCLUSIONS: Moderately increased serum prolactin is associated with increased RNFL thickness when compared to controls. These associations are lost when serum prolactin is < 30 ng/ml or elevated in prolactinomas. This suggests a neuroprotective effect of prolactin at moderately increased levels in preserving retinal function during optic chiasm compression.

Asunto(s)

Adenoma , Fármacos Neuroprotectores , Neoplasias Hipofisarias , Prolactinoma , Adenoma/complicaciones , Estudios Transversales , Humanos , Fármacos Neuroprotectores/uso terapéutico , Prolactina , Prolactinoma/complicaciones , Estudios Retrospectivos

RESUMEN

Background: The goal of this study was to relate diffusion MR measures of white matter integrity of the retinofugal visual pathway with prolactin levels in a patient with downward herniation of the optic chiasm secondary to medical treatment of a prolactinoma. Methods: A 36-year-old woman with a prolactinoma presented with progressive bilateral visual field defects 9 years after initial diagnosis and medical treatment. She was diagnosed with empty-sella syndrome and instructed to stop cabergoline. Hormone testing was conducted in tandem with routine clinical evaluations over 1 year and the patient was followed with diffusion magnetic resonance imaging (dMRI), optical coherence tomography (OCT), and automated perimetry at three time points. Five healthy controls underwent a complementary battery of clinical and neuroimaging tests at a single time point. Results: Shortly after discontinuing cabergoline, diffusion metrics in the optic tracts were within the range of values observed in healthy controls. However, following a brief period where the patient resumed cabergoline (of her own volition), there was a decrease in serum prolactin with a corresponding decrease in visual ability and increase in radial diffusivity (p < 0.001). Those measures again returned to their baseline ranges after discontinuing cabergoline a second time. Conclusions: These results demonstrate the sensitivity of dMRI to detect rapid and functionally significant microstructural changes in white matter tracts secondary to alterations in serum prolactin levels. The inverse relations between prolactin and measures of white matter integrity and visual function are consistent with the hypothesis that prolactin can play a neuroprotective role in the injured nervous system.

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BACKGROUND: Rathke cleft cyst (RCC) has a recurrence rate of 10% to 22%, and preventing recurrence is challenging. For patients who experience persistent recurrence of RCC, placement of steroid-eluting bioabsorbable intrasellar stents has been rarely described. However, recurrences are often delayed, suggesting that dissolvable stents may not be successful long-term. The release of steroids in close proximity to the pituitary gland may also unintentionally influence the hypothalamic-adrenal-pituitary axis. OBSERVATIONS: The authors present a case of a 66-year-old woman with a persistently recurrent RCC who underwent drainage of her cyst with placement of a nonabsorbable intrasellar stent in the form of a tympanostomy tube. After repeat transsphenoidal drainage of her cyst, a tympanostomy T-tube was placed to stent open the dural aperture. Postoperatively, the patient's condition showed improvement clinically and radiographically. LESSONS: Placement of an intrasellar stent for recurrent RCC has rarely been described. Steroid-eluting bioabsorbable stents may dissolve before RCC recurrence and may have an unintentional effect on the hypothalamic-pituitary-adrenal axis. The authors present the first case of nonabsorbable stent placement in the form of a tympanostomy tube for recurrence of RCC. Additional studies and longer follow-up are necessary to evaluate the long-term efficacy of both absorbable and nonabsorbable stent placement.

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We report a case of a 21-year-old African American female with history of pre-diabetes, and a diagnosis of a rare leukemia, blastic-plasmacytoid dendritic neoplasm (BPDCN), who developed diabetic ketoacidosis (DKA) after the third dose of PEG-asparaginase infusion. She was successfully treated with insulin. Asparaginase is a vital part of treatment protocols for acute lymphoblastic leukemia (ALL) in combination with other chemotherapeutic drugs. Asparaginase therapy has been reported to cause hyperglycemia especially when used in conjunction with glucocorticoids for the treatment of ALL in the pediatric population. Multiple mechanisms for hyperglycemia have been hypothesized which include decreased insulin secretion, impaired insulin receptor function and excess glucagon formation. Hyperglycemia is usually self-limiting but can deteriorate to diabetic ketoacidosis. DKA is a rare adverse effect with asparaginase therapy with an incidence rate of about 0.8%. Learning points: •• DKA is a rare finding following asparaginase therapy. •• Hyperglycemia is most commonly seen with asparaginase treatment when used along with glucocorticoid. •• Frequent blood glucose monitoring and prompt initiation of insulin treatment with hyperglycemia can prevent severe complications. •• Patients and physician education on this complication can reduce morbidity due to DKA.

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OBJECTIVE: Cushing disease (CD) results from excessive exposure to glucocorticoids caused by an adrenocorticotropic hormone-secreting pituitary tumor. Inadequately treated CD is associated with significant morbidity and elevated mortality. Multicenter data on CD patients treated in routine clinical practice are needed to assess treatment outcomes in this rare disorder. The study purpose was to describe the burden of illness and treatment outcomes for CD patients. METHODS: Eight pituitary centers in four U.S. regions participated in this multicenter retrospective chart review study. Subjects were CD patients diagnosed at ≥18 years of age within the past 20 years. Descriptive statistical analyses were conducted to examine presenting signs, symptoms, comorbidities, and treatment outcomes. RESULTS: Of 230 patients, 79% were female (median age at diagnosis, 39 years; range, 18 to 78 years). Length of follow-up was 0 to 27.5 years (median, 1.9 years). Pituitary adenomas were 0 to 51 mm. The most common presenting comorbidities included hypertension (67.3%), polycystic ovary syndrome (43.5%), and hyperlipidemia (41.5%). Biochemical control was achieved with initial pituitary surgery in 41.4% patients (91 of 220), not achieved in 50.0% of patients (110 of 220), and undetermined in 8.6% of patients (19 of 220). At the end of follow-up, control had been achieved with a variety of treatment methods in 49.1% of patients (110 of 224), not achieved in 29.9% of patients (67 of 224), and undetermined in 21.0% of patients (47 of 224). CONCLUSION: Despite multiple treatments, at the end of follow-up, biochemical control was still not achieved in up to 30% of patients. These multicenter data demonstrate that in routine clinical practice, initial and long-term control is not achieved in a substantial number of patients with CD. ABBREVIATIONS: BLA = bilateral adrenalectomy CD = Cushing disease CS = Cushing syndrome eCRF = electronic case report form MRI = magnetic resonance imaging PCOS = polycystic ovary syndrome.

Asunto(s)

Adenoma Hipofisario Secretor de ACTH/terapia , Adenoma/terapia , Hipersecreción de la Hormona Adrenocorticotrópica Pituitaria (HACT)/terapia , Inhibidores de 14 alfa Desmetilasa/uso terapéutico , Adenoma Hipofisario Secretor de ACTH/complicaciones , Adenoma Hipofisario Secretor de ACTH/metabolismo , Adenoma Hipofisario Secretor de ACTH/patología , Adenoma/complicaciones , Adenoma/metabolismo , Adenoma/patología , Adolescente , Adrenalectomía , Adulto , Anciano , Antineoplásicos/uso terapéutico , Cabergolina , Comorbilidad , Inhibidores Enzimáticos/uso terapéutico , Ergolinas/uso terapéutico , Femenino , Estudios de Seguimiento , Hirsutismo/etiología , Antagonistas de Hormonas/uso terapéutico , Hormonas/uso terapéutico , Humanos , Hiperlipidemias/epidemiología , Hipertensión/epidemiología , Hipoglucemiantes/uso terapéutico , Cetoconazol/uso terapéutico , Masculino , Metirapona/uso terapéutico , Persona de Mediana Edad , Mifepristona/uso terapéutico , Debilidad Muscular/etiología , Atrofia Muscular/etiología , Procedimientos Neuroquirúrgicos , Obesidad Abdominal/etiología , Hipersecreción de la Hormona Adrenocorticotrópica Pituitaria (HACT)/complicaciones , Hipersecreción de la Hormona Adrenocorticotrópica Pituitaria (HACT)/epidemiología , Hipersecreción de la Hormona Adrenocorticotrópica Pituitaria (HACT)/metabolismo , Irradiación Hipofisaria , Síndrome del Ovario Poliquístico/epidemiología , Estudios Retrospectivos , Rosiglitazona , Somatostatina/análogos & derivados , Somatostatina/uso terapéutico , Estrías de Distensión/etiología , Tiazolidinedionas/uso terapéutico , Resultado del Tratamiento , Carga Tumoral , Adulto Joven

RESUMEN

PURPOSE: Follow-up guidelines are needed to assess quality of care and to ensure best long-term outcomes for patients with Cushing's disease (CD). The purpose of this study was to assess agreement by experts on recommended follow-up intervals for CD patients at different phases in their treatment course. METHODS: The RAND/UCLA modified Delphi process was used to assess expert consensus. Eleven clinicians who regularly manage CD patients rated 79 hypothetical patient scenarios before and after ("second round") an in-person panel discussion to clarify definitions. Scenarios described CD patients at various time points after treatment. For each scenario, panelists recommended follow-up intervals in weeks. Panel consensus was assigned as follows: "agreement" if no more than two responses were outside a 2 week window around the median response; "disagreement" if more than two responses were outside a 2 week window around the median response. Recommendations were developed based on second round results. RESULTS: Panel agreement was 65.9% before and 88.6% after the in-person discussion. The panel recommended follow-up within 8 weeks for patients in remission on glucocorticoid replacement and within 1 year of surgery; within 4 weeks for patients with uncontrolled persistent or recurrent disease; within 8-24 weeks in post-radiotherapy patients controlled on medical therapy; and within 24 weeks in asymptomatic patients with stable plasma ACTH concentrations after bilateral adrenalectomy. CONCLUSIONS: With a high level of consensus using the Delphi process, panelists recommended regular follow-up in most patient scenarios for this chronic condition. These recommendations may be useful for assessment of CD care both in research and clinical practice.

Asunto(s)

Hipersecreción de la Hormona Adrenocorticotrópica Pituitaria (HACT)/tratamiento farmacológico , Hipersecreción de la Hormona Adrenocorticotrópica Pituitaria (HACT)/cirugía , Adrenalectomía , Hormona Adrenocorticotrópica/sangre , Glucocorticoides/uso terapéutico , Humanos , Hipersecreción de la Hormona Adrenocorticotrópica Pituitaria (HACT)/sangre , Hipófisis/efectos de los fármacos , Hipófisis/cirugía

RESUMEN

Co-secretion of growth hormone (GH) and prolactin (PRL) from a single pituitary adenoma is common. In fact, up to 25% of patients with acromegaly may have PRL co-secretion. The prevalence of acromegaly among patients with a newly diagnosed prolactinoma is unknown. Given the possibility of mixed GH and PRL co-secretion, the current recommendation is to obtain an insulin-like growth factor-1 (IGF-1) in patients with prolactinoma at the initial diagnosis. Long-term follow-up of IGF-1 is not routinely done. Here, we report two cases of well-controlled prolactinoma on dopamine agonists with the development of acromegaly 10-20 years after the initial diagnoses. In both patients, a mixed PRL/GH-cosecreting adenoma was confirmed on the pathology examination after transsphenoidal surgery (TSS). Therefore, periodic routine measurements of IGF-1 should be considered regardless of the duration and biochemical control of prolactinoma. LEARNING POINTS: Acromegaly can develop in patients with well-controlled prolactinoma on dopamine agonists.The interval between prolactinoma and acromegaly diagnoses can be several decades.Periodic screening of patients with prolactinoma for growth hormone excess should be considered and can lead to an early diagnosis of acromegaly before the development of complications.

RESUMEN

PURPOSE: Geography is known to affect cost of care in surgical procedures. Understanding the relationship between geography and hospital costs is pertinent in the effort to reduce healthcare costs. We studied the geographic variation in cost for transsphenoidal pituitary surgery in hospitals across New York State. METHODS: Using the Healthcare Cost and Utilization Project State Inpatient Database for New York from 2008 to 2011, we analyzed records of patients who underwent elective transsphenoidal pituitary tumor surgery and were discharged to home or self-care. N.Y. State was divided into five geographic regions: Buffalo, Rochester, Syracuse, Albany, and Downstate. These five regions were compared according to median charge and cost per day. RESULTS: From 2008 to 2011, 1803 transsphenoidal pituitary tumor surgeries were performed in New York State. Mean patient age was 50.7 years (54 % were female). Adjusting prices for length of stay, there was substantial variation in prices. Median charges per day ranged from $8485 to $13,321 and median costs per day ranged from $2962 to $6837 between the highest and lowest regions from 2008 to 2011. CONCLUSION: Within New York State, significant geographic variation exists in the cost for transsphenoidal pituitary surgery. The significance of and contributors to such variation is an important question for patients, providers, and policy makers. Transparency of hospital charges, costs, and average length of stay for procedures to the public provides useful information for informed decision-making, especially for a highly portable disease entity like pituitary tumors.

Asunto(s)

Procedimientos Neuroquirúrgicos/economía , Neoplasias Hipofisarias/cirugía , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , New York

RESUMEN

OBJECTIVE: Perioperative glucocorticoid (GC) is rarely needed in patients undergoing transsphenoidal surgery (TSS). We instituted a steroid-sparing protocol in the settings of intraoperative dexamethasone use. We evaluated the safety of using a cut off cortisol level of 14 µg/dL on postoperative day (POD)-1 and -6 after dexamethasone use during the surgery. We also analyzed the efficacy of serial morning cortisol levels for weaning GC replacement. METHODS: The charts of 48 adult patients who received dexamethasone 4 mg intraoperatively were reviewed. Morning cortisol levels were measured on POD-1. Patients with cortisol ≥14 µg/dL were discharged without CG replacement. Morning cortisol level was checked routinely on POD-6, and GC replacement was initiated when the level was <14 µg/dL. Serial cortisol levels were measured in patients requiring GC after the first postoperative week. RESULTS: Overall, 67% patients had POD-1 cortisol ≥14 µg/dL and did not require GC on discharge. After POD-6, 83% of patients were not on GC replacement. A cosyntropin stimulation testing (CST) was only performed in 3 patients. There were no hospital admissions for adrenal crisis during the postoperative period. CONCLUSION: A steroid-sparing protocol with POD-1 and -6 morning cortisol levels can be safely and effectively used in the settings of intraoperative dexamethasone administration. It leads to avoidance of GC in more than two-thirds of patients on discharge and more than 80% of patients after the first postoperative week. We found that dynamic adrenal testing could be omitted in the majority of patients by using serial morning cortisol levels to assess the hypothalamic-pituitary-adrenal (HPA) axis.

Asunto(s)

Adenoma/sangre , Hidrocortisona/sangre , Sistema Hipotálamo-Hipofisario/metabolismo , Procedimientos Neuroquirúrgicos/métodos , Neoplasias Hipofisarias/sangre , Sistema Hipófiso-Suprarrenal/metabolismo , Cuidados Posoperatorios/métodos , Adenoma/cirugía , Adulto , Anciano , Dexametasona/administración & dosificación , Femenino , Glucocorticoides/administración & dosificación , Humanos , Masculino , Persona de Mediana Edad , Neoplasias Hipofisarias/cirugía , Seno Esfenoidal/cirugía , Resultado del Tratamiento