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Introduction: Germline pathogenic variants in succinate dehydrogenase subunit B (SDHB) cause paraganglioma/pheochromocytoma syndrome type 4 (PGL-4). SDHB-associated pheochromocytomas (PCC) are thought to be rare and little data exist about their clinical behavior., Patients and Methods: Retrospective review of patients treated (1993-2023) at a tertiary cancer center for SDHB-associated PCC. Clinical and demographic variables were retrieved to characterize disease-free survival, disease progression, and overall survival., Results: In total, 90 SDHB-carriers were identified, 18% had PCC (n = 16). Median age at diagnosis of was 40 (19-76) years, 50% (n = 8) of patients were male, 25% (n = 4) had distant metastasis (DM) at diagnosis, and 13% (n = 2) had synchronous PGL. No patients had bilateral disease, and 94% of patients underwent surgery as initial treatment with a curative intent in 75%. Overall, 64% of patients underwent open resection. Recurrence occurred in 77% of patients (n = 10), 75% in minimally invasive surgery (MIS) versus 77% open, p = 0.63. Bone was the most common site of DM (100%, n = 13). Metaidobenzyleguanidine (MIBG) imaging was performed in 69% of patients, 91% of which were positive. Median time from surgery until recurrence was 36 months (1-295 months). Radiation therapy was the most common adjuvant treatment (44%) followed by Iobenguane I-131 (31%) and systemic therapy (31%). Median follow-up time was 56 months (1-408 months). Overall, 33% of patients were alive, 19% of patients were disease-free, and 50% of the patients with DM had stable disease at last follow-up., Conclusions: Overall, 18% of germline SDHB mutation-carriers were diagnosed with PCC, all of which were unilateral. SDHB-associated PCC was associated with advanced and recalcitrant disease and was often MIBG positive. More studies are needed to better understand the clinical behavior of PCC in PGL-4., (© 2024. Society of Surgical Oncology.)

Background: Oncocytic adrenocortical neoplasms (OANs) are rare endocrine tumors that present as a spectrum from benign to malignant. The outcomes after surgical resection of the oncocytic variant of adrenocortical carcinoma remain poorly understood. We sought to characterize the clinicopathologic features of OAN and compare oncocytic adrenocortical carcinoma (OAC) with conventional adrenocortical carcinoma (ACC)., Patients and Methods: Adult patients who underwent adrenalectomy for OAN or ACC between January 1990 and September 2020 were identified. Demographics, clinicopathologic factors, American Joint Committee on Cancer stage, and cancer-related outcomes were reviewed. A matched cohort analysis of disease-free survival (DFS) and overall survival (OS) was performed between patients with OACs and those with ACCs., Results: Forty-one patients with OAN and 214 patients with ACC were included. The OAN cohort median age was 45.2 years [interquartile ratio (IQR) 38.5-54.0 years], and 61.0% were female. OANs were benign (n = 11), of uncertain malignant potential (UMP, n = 9), or OAC (n = 21). Disease recurrence occurred in 12 (57.1%) patients with OAC compared with 1 (11.1%) and 0 patients with UMP or benign OAN, respectively (p < 0.001). Seven (33.3%) patients with OAC died during follow-up compared with 0 patients with UMP or benign OAN (p = 0.020). Kaplan-Meier survival analysis found no difference in DFS between ACC and OAC groups before (p = 0.218) and after 2:1 matching (p = 0.417). Overall survival was shorter for patients who had ACC compared with those who had OAC (p = 0.031), but the difference was not evident with matched analysis (p = 0.200)., Conclusions: OAN presents as a spectrum from benign indolent tumors to aggressive carcinomas. OACs demonstrate similar clinicopathologic behavior and recurrence-free and overall survival when matched to conventional ACCs., (© 2022. Society of Surgical Oncology.)

Background: Development of distant metastases (DM) is associated with markedly decreased survival in parathyroid carcinoma (PC). We sought to identify factors associated with development of DM and to quantify the effect that development of DM had on overall survival (OS)., Methods: Patients with surgically resected local/regional PC treated or surveilled at a tertiary-referral cancer hospital from 1980 to 2017 were included. We assessed the association between biochemical and clinicopathologic factors (preoperative parathyroid hormone (PTH) levels, sex, race, age, preoperative serum calcium levels, serum calcium levels at 6 months postop, tumor size, and extent of resection) with the development of DM. We also assessed the effect of development of DM on OS., Results: Seventy-five patients with PC were assessed; 17 (22.7%) developed DM at a median follow-up of 77 months. The cumulative incidence of DM in the cohort was 20, 30, and 38% at 5, 10, and 20 years respectively. Tumor size > 3.2 cm based on recursive partitioning analysis was the only significant predictor for development of DM (hazard ratio (HR) = 3.51; 95% confidence interval [CI] 1.04-11.91; p = 0.04). Median OS for the entire cohort was 17 years compared with 40 months for the cohort who developed DM. The HR for death after distant metastasis was 9.6 (95% CI 4.2-22.3; p < 0.0001)., Conclusions: Development of distant metastasis during surveillance is associated with decreased OS, including late recurrences. Primary tumor size should be considered in future interval surveillance and development of treatment algorithms.