1. Clinical decision making in small non-functioning VHL-related incidentalomas
- Author
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Hartmut P. H. Neumann, Christoph Bode, Tobias Krauss, Peggy Sekula, Roland Därr, Birke Bausch, Jonas Kater, Stefan Zschiedrich, and Gerd Walz
- Subjects
0301 basic medicine ,medicine.medical_specialty ,Endocrinology, Diabetes and Metabolism ,030209 endocrinology & metabolism ,rare diseases/syndromes ,030105 genetics & heredity ,Gastroenterology ,lcsh:Diseases of the endocrine glands. Clinical endocrinology ,Pheochromocytoma ,03 medical and health sciences ,0302 clinical medicine ,Endocrinology ,endocrine cancers ,Clinical decision making ,Paraganglioma ,Internal medicine ,Internal Medicine ,Medicine ,lcsh:RC648-665 ,business.industry ,Research ,Optimal treatment ,Mean age ,Metanephrines ,medicine.disease ,adrenal ,neuroendocrinology ,business ,Symptom score ,Tumour diameter - Abstract
The optimal treatment strategy for patients with small non-functioning VHL-related incidentalomas is unclear. We searched the Freiburg VHL registry for patients with radiologic evidence of pheochromocytoma/paraganglioma (PHEO/PGL). In total, 176 patients with single, multiple, and recurrent tumours were identified (1.84 tumours/patient, range 1–8). Mean age at diagnosis was 32 ± 16 years. Seventy-four percent of tumours were localised to the adrenals. Mean tumour diameter was 2.42 ± 2.27 cm, 46% were P < 0.025) and before surgery (4.89 ± 3.47 cm vs 1.36 ± 0.43 cm; P < 0.02). Disease was stable in 67% of 21 patients with evaluable tumours ≤1.5 cm according to RECIST and progressed in 7. Time till surgery in these patients was 29.5 ± 20.0 months. A total of 155 patients underwent surgery. PHEO/PGL was histologically excluded in 4 and proven in 151. Of these, one had additional metastatic disease, one harboured another tumour of a different type, and in 2 a second surgery for suspected disease recurrence did not confirm PHEO/PGL. Logistic regression analysis revealed 50% probability for a positive/negative biochemical test result at 1.8 cm tumour diameter. Values of a novel symptom score were positively correlated with tumour size (Rs = 0.46, P < 0.0001) and together with a positive biochemistry a linear size predictor (P < 0.01). Results support standardised clinical assessment and measurement of tumour size and metanephrines in VHL patients with non-functioning incidentalomas
- Published
- 2020