Objective: Latin American reports on pheochromocytomas and paragangliomas (PPGL) are scarce. Recent studies have shown changes in both clinical presentation and management of these patients. We aimed to assess the main characteristics of PPGL patients in a single academic center over the last four decades. Experimental design: Cohort study. Patients and methods: Demographic, clinical, biochemical, genetic and perioperative data from 105 PPGL patients were retrospectively and prospectively collected over the 1980–2019 period. Patients were categorized into four groups (14 patients in the 1st, 25 patients in the 2nd, 27 patients in the 3th and 39 patients in the 4th decade) according to the date of diagnosis. Results: The mean age at diagnosis was 46±19 years, and the tumor size was 5.3±2.2 cm, female gender was 63%, bilateral tumor of 15%, paragangliomas 9% and metastatic disease in 15%. The aforementioned parameters remained stable across the four decades. During the study period we observed significant increases in doxazosin dosing (2.7±2.6 mg vs. 8.0±4.5 p<0.003) and laparoscopic procedures (28% vs. 84% p<0.001) along with a decrease in the length of hospital stay (10.0±8.9 vs. 3.8±1.7 days p=0.007). Among the 24 genetic tests performed, we identified 59% germline mutations. The most frequent mutations were RET (18%) and SDHX (18%), followed by VHL (14%), MAX (5%) and NF1 (4%). Notably, in the last decade we observed a dramatic increase in the proportion of incidental PPGL diagnosis (0% vs. 53% p<0.001) and genetic testing analyses (0 vs. 19 p<0.001). When comparing incidental diagnosis (n=25) versus clinically suspicious cases(n=50), incidentalomas had fewer adrenergic symptoms (38 vs. 62%; p<0.001), and lower rates of hypertension (64 vs. 80%; p=0.01), hypertension crises (28 vs. 44%; p=0.02), functionality (79 vs. 100%; p=0.01) and total catecholamines and/or metanephrine levels (8.4 vs. 12.5 fold above the upper normal limit; p=0.04). Conclusions: The implementation of a multidisciplinary program increased diagnosis and genetic testing and also optimized anesthesia and surgical procedure, translating into a notorious improvement in perioperative outcomes. In addition, we observed a change in the clinical presentation of PPGL in recent decades with a marked increase in incidental cases, which highlights the importance of early diagnosis and treatment.
Papillary thyroid cancer is the most common endocrine malignancy, and due to its favorable prognosis, the extent of surgery has been a matter of debate. About 10% of these tumors are located in the thyroid isthmus, with no specific management in current guidelines. In the last decades, isthmusectomy has been proposed as a therapeutic option for isthmic papillary thyroid carcinoma, although there is no consensus on its management. We present two cases of patients from our institution with solitary isthmic papillary thyroid carcinoma without clinical macroscopic extrathyroidal extension or clinical-radiological lymph node involvement who were treated with isthmusectomy without prophylactic lymph node dissection. Neither of them had any postoperative complications. Both had an intermediate risk of recurrence due to aggressive variants. None of them had signs of recurrence during follow-up. We intend to show that isthmusectomy seems to be an effective and safe surgical alternative in selected patients through these cases.
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