BackgroundAlthough many patients with follicular lymphoma (FL) undergo routine radiographic surveillance during their first remission, no consensus exists on the modality, duration, frequency, or need for routine imaging studies. The authors retrospectively examined the effect of surveillance imaging on relapse detection and overall survival (OS) in patients with FL.MethodsPatients with newly diagnosed FL who had a response to induction therapy were identified from the Lymphoid Malignancies Enterprise Architecture Database (LEAD) at Emory University and from the Molecular Epidemiology Resource (MER) of the University of Iowa/Mayo Clinic. Patients were evaluated for both relapse and method of relapse detection (ie, clinical concerns vs radiologic detection through surveillance imaging in an asymptomatic patient).ResultsOf 148 patients in the LEAD cohort, 55 (37%) relapsed, and the majority (n = 35; 64%) of relapses were detected clinically. In the MER cohort, 63 of 177 relapses (54%) were detected clinically. There was no significant difference in OS from the date of diagnosis between the 2 methods of relapse detection in the LEAD (hazard ratio [HR], 0.61; 95% CI, 0.13‐2.94; P = .54) and MER (HR, 1.02; 95% CI, 0.47‐2.21; P = .96) cohorts. Similarly, there was no significant difference in OS from the date of relapse between the 2 methods of relapse detection in the LEAD (HR, 0.47; 95% CI, 0.10‐2.27; P = .35) and MER (HR, 1.02; 95% CI, 0.47‐2.21; P = .96) cohorts.ConclusionsThese findings suggest a limited role for routine surveillance imaging in patients with FL who complete front‐line therapy. Future studies should evaluate which patients may benefit from a more aggressive surveillance approach and should explore novel methods of relapse detection.
Concomitant Pheochromocytoma and Primary Aldosteronism: A Case Series and Literature Review
Abstract Context The detection and management of concomitant pheochromocytoma (PHEO) and primary aldosteronism (PA) is not well understood. Objective To investigate varying presentations and outcomes of cases with coexisting PHEO and PA to provide an approach to its diagnosis and management. Methods We conducted a retrospective case series of adult patients with concomitant PHEO and PA at Mayo Clinic from 2000-2020 and an additional review of cases before 2000 and from the medical literature. Clinical, biochemical, radiologic, and histologic parameters were measured. Results Fifteen patients (53% men, median age 53 years) were diagnosed with concomitant PHEO and PA. The majority presented with hypertension (13, 87%) and hypokalemia (13, 87%), and 6 (40%) presented with symptoms suggestive of catecholamine excess. All patients who underwent preoperative workup for catecholamine excess (14, 93%) were found to have biochemical levels above the upper limits of normal. Adrenal vein sampling (AVS) was performed in 9 patients (60%), where 5 (56%) were diagnosed with bilateral PA, and 4 (44%) with unilateral PA. Patients underwent either unilateral (12, 80%) or bilateral (3, 20%) adrenalectomy. Biochemical improvement or resolution of catecholamine excess was confirmed in all cases with documented measurements. Recurrence of PHEO was not observed. Six patients (40%) displayed persistent PA postoperatively. Conclusion Concomitant PHEO and PA is a rare but likely underreported condition. Hypertension with or without hypokalemia should prompt evaluation for PA, while any indeterminate adrenal mass should be assessed for PHEO. Coexisting disease warrants consideration of AVS to determine the laterality of PA to ensure appropriate management.
Concomitant Pheochromocytoma and Primary Aldosteronism: A Case Series and Literature Review
Abstract Objective: The detection and management of concomitant pheochromocytoma (PHEO) and primary aldosteronism (PA) is not well understood. Our objectives were to investigate varying presentations and outcomes of cases with coexisting PHEO and PA to provide an approach to its diagnosis and management. Design: Retrospective case series from 2000–2020 at a single institution tertiary center; additional review of previously known cases before 2000 and from the medical literature. Patients and Measurements: Adult patients with concomitant PHEO and PA. Clinical, biochemical, radiologic, and histologic parameters were reviewed. Results: Fifteen patients (53% men, median age 53 years) were diagnosed with concomitant PHEO and PA. The majority presented with hypertension (13, 87%) and hypokalemia (13, 87%), but only 6 (40%) presented with symptoms suggestive of catecholamine excess. All patients with preoperative work-up for catecholamine excess (14, 93%) were found to have elevated plasma or urinary metanephrines/catecholamines above the upper limit of normal. Adrenal vein sampling (AVS) was performed in 9 (60%) patients, where 5 (56%) were diagnosed with bilateral PA, and 4 (44%) with unilateral PA. All patients underwent either unilateral (12, 80%) or bilateral (3, 20%) adrenalectomy to treat their PHEO and/or PA. Postoperative catecholamines and/or catecholamine breakdown products normalized or improved in 13 (87%) patients and were not measured in 2. Recurrence of PHEO was not observed. Six (40%) displayed persistent PA postoperatively, where 4 required long-term mineralocorticoid blockade. Conclusions: Concomitant PHEO and PA is a rare but likely under-reported condition. Hypertension with or without hypokalemia should prompt evaluation for PA, while any indeterminate adrenal mass should be worked up for PHEO. Coexisting disease warrants consideration of AVS to determine the laterality of PA to ensure appropriate management.
Presentation, disease progression and outcomes of adrenal gland metastases
AbstractObjectiveData on the presentations and outcomes of patients with adrenal gland metastases are limited. Our objectives were to characterize the prevalence of adrenal metastases subtypes and investigate how varying clinical presentations affect disease progression, development of primary adrenal insufficiency (PAI) and mortality.DesignSingle institution tertiary centre, retrospective cohort study from 1997 to 2020.Patients and MeasurementsAdult patients with adrenal metastases. Clinical, radiologic and biochemical presentations and outcomes were reviewed.ResultsOf 579 patients (62.3% men, median age 67 years [range 25‐92]) with adrenal metastases (median tumour size of 30 mm [range 5‐200]), 339 (58.5%) were discovered during cancer staging, 210 (36.3%) were found incidentally, and 29 (5.0%) based on symptoms. Tumours originated from the lung (226, 39.0%), genitourinary (GU) (160, 27.6%), gastrointestinal (GI) (79, 13.6%) and other (114, 19.7%) organ systems. Bilateral metastases were found in 140 (24.2%) patients at the time of initial diagnosis, and 249 (43.0%) had bilateral disease throughout the study course. PAI developed in 12.4% of patients with bilateral disease and was associated with larger tumour size. Median follow‐up time was 14 months (range 0‐232), and 442 (76.3%) patients died. Higher mortality was independently associated with older age, adrenal metastases originating from the lung, bilateral disease, and the absence of adrenalectomy.ConclusionsAdrenal gland metastases originated most commonly from lung, GU and GI malignancies. Bilateral adrenal metastases occurred in 43% of patients, and PAI occurred in 12.4% of those with bilateral disease, warranting further case detection strategies.
SAT-154 Clinical Presentations and Outcomes of Adrenal Metastases Vary Based on Etiology
Abstract Background: Adrenal metastases occur in 1–8% of patients with an adrenal mass. Recognizing patterns in the presentation of adrenal metastases is critical in dictating management. Objective: To describe the presentation of patients with adrenal metastases and identify baseline characteristics predicting the etiology. Methods: A retrospective analysis of adult patients diagnosed with adrenal metastases between 2000–2019 at a single institution tertiary center was performed. Partial cohort analysis is presented. Results: In 327 patients (127 (39%) women, median age at diagnosis of 67 years (range 25–92)), median tumor size was 2.7 cm (range 0.5–15), and 99 (30%) had bilateral tumors. While most patients (188, 57%) were found to have an adrenal mass during cancer staging, 117 (36%) were found incidentally and 22 (7%) based on symptoms. Adrenal metastases originated from the lung (118, 36%), genitourinary (GU) (100, 31%), gastrointestinal (GI) (47, 14%), and other (43, 13%) organ systems. Unknown primary malignancies were diagnosed in 19 (5.8%) patients. Male predominance was observed in GU (72%) and lung (62%) metastases, but equal gender distributions were noted for all other metastases, p=0.01. Patients with GI and lung metastases were diagnosed with smaller tumors (median 2.1 and 2.6 cm, respectively) compared to those with GU (median 3.5 cm) and other (median 4 cm) metastases, p=0.0008. Bilateral metastases were more frequently found in patients with lung (37%) and other (38%) metastases compared to those with GI (17%) and GU (24%) metastases, p=0.01. Of 99 (30%) patients with bilateral metastases, 23% developed primary adrenal insufficiency (PAI), most commonly in those with lung (36%) and GU (30%) malignancies. Only 123 (38%) patients were evaluated by an endocrinologist. Pheochromocytoma work-up was more often pursued if seen by an endocrinologist (71% vs. 15%, p<0.0001) in 118 (36%) patients. Adrenalectomy was performed in 94 (29%) patients, most frequently in those with GU metastases (57%), compared to only 10% of those with lung metastases. Patients were followed for a median time of 14 months (range 0.1–181), and 222 (68%) died. GU metastases carried the best prognosis with a mortality rate (MR) of 43%, as opposed to a MR of >70% in all other metastases, with lung metastases carrying the worst prognosis (MR of 85%). Multivariate analysis revealed that mortality was associated with increasing age (OR 1.3 (95% CI 1.04–1.6) for each decade) and metastasis subgroup (lung vs. GU: OR 7.2 (95% CI 3.7–14)). Conclusion: Adrenal metastases most commonly originated from lung, GU and GI malignancies, with a third of patients discovered incidentally. Bilateral metastases occurred in 30% of patients, where 1 in 4 developed PAI. Only a minority were evaluated by an endocrinologist or had work up for pheochromocytoma. Mortality was highest in those with adrenal metastases originating from the lung.
Continuous infusion of recombinant porcine factor VIII for neurosurgical management of intracranial haemorrhage in a patient with severe haemophilia A with factor VIII inhibitor
7536 Background: While most patients (pts) with follicular lymphoma (FL) achieve an initial response to treatment, the majority relapse. Recently, Goldman et al. reported that surveillance imaging (SI) in FL is not associated with improved post-relapse outcomes and is frequently associated with false positive scans (ASH 2017). The goal of this study was to validate these findings. Methods: Pts were enrolled in the University of Iowa/Mayo Clinic SPORE Molecular Epidemiology Resource (MER), a prospective cohort of newly diagnosed lymphoma pts. All pts were followed for events including relapse, re-treatment, and death, with events verified by medical records. Pts eligible for this study had biopsy proven FL grade 1, 2, or 3a who had achieved a response after induction therapy and later relapsed. Initial and post-treatment management was per treating physician. Medical records were re-reviewed in pts with events for clinical details at relapse in relationship to planned follow-up visits and SI. Relapse detection was categorized either by clinical suspicion (CS) via history, exam, and/or labs; or by SI in an asymptomatic pt. Univariate survival analysis was conducted for each variable. The hazard ratio with 95% confidence interval based on Cox Proportional Hazard models was presented along with the log rank test p-value. Kaplan-Meier plots were produced to evaluate the difference in overall survival (OS) between groups. Results: 1121 FL pts were enrolled in MER from 2002-2015, of which 117 were eligible. Median age at diagnosis was 60 years (range 35-83), and 61% were men. Median time to relapse of the 117 pts was 26 months (range 9-125). At a median follow-up from relapse of 69 months (range 0.23-179), 26 pts died. Pts completed a median of 3 imaging studies (range 0-15) during post-induction surveillance. 63 relapses (56%) were detected based on CS; 50 (44%) were detected by SI; and 4 were unknown. There was no difference in OS from relapse for those with relapse detected via CS vs. SI (HR = 0.98 [0.45,2.12], p = 0.96). Conclusions: Routine SI does not appear to improve survival outcomes in FL after achieving first remission, and its common practice should be questioned. Further investigation of prognostic markers to identify high-risk FL pts who may benefit from SI is needed.