ECE2022 Poster Presentations Pituitary and Neuroendocrinology (127 abstracts)
Pheochromocytoma/paraganglioma metastatic potential prediction
Ewelina Rzepka 1 , Anna Kurzyńska 1 , Anna Grochowska 2 , Magdalena Ulatowska-Białas 3 , Anna Skalniak 4 , Martyna Lech 3 , Marta Opalinska 5 , Anna Bogusławska 1 , Elwira Przybylik-Mazurek 1 , Anna Sowa-Staszczak 6 , Aleksandra Gilis-Januszewska 1 & Alicja Hubalewska-Dydejczyk 1
1Jagiellonian University, University Hospital, Endocrinology, Kraków, Poland; 2Jagiellonian University, University Hospital, Radiology, Kraków, Poland; 3Jagiellonian University, University Hospital, Pathomorphology, Kraków, Poland; 4Jagiellonian University, University Hospital, Genetics, Kraków, Poland; 5University Hospital in Krakow, Department of Endocrinology, Oncological Endocrinology and Nuclear Medicine, Kraków, Poland; 6Jagiellonian University, University Hospital, Nuclear Medicine, Kraków, Poland
Pheochromocytoma and paraganglioma (PPGL) are rare neuroendocrine tumours, which derive from the chromaffin cells of the adrenal medulla or extra-adrenal sympathetic and parasympathetic ganglia. About 15-20% of those neoplasms could present malignant course. Prediction of PPGL metastatic potential still remains a great clinical challenge, since the sensitivity and specificity of proposed prediction systems are not satisfactory. We performed a retrospective database search for pheochromocytoma and paraganglioma patients, diagnosed and treated in Endocrinology Department, University Hospital in Cracow from 2005 to 2021. 206 patients with pheochromocytoma and 27 patients with paraganglioma were included to the analysis. The mean follow-up period was 59 months (range: 2-396 months). In the whole group, 5 metastatic pheochromocytomas and 2 metastatic paragangliomas have been found. Our objective was to investigate clinicopathological characteristics of the patients with malignant PPGLs in the light of current metastatic potential predictors. The group of patients with disseminated disease comprise 2 men and 5 women. Median patient age was 51 years (range 19-72). In two patients metastatic disease was observed at the moment of diagnosis, in remaining cases metastases developed 5 to 84 months after the diagnosis (median time 32 months). The most common localizations of metastases were lymph nodes and bones (71.4% for each). The leading symptom of metastatic disease was fatigue (86%). The median size of the primary tumour was 7.7 cm. Hormonal assessment revealed significantly elevated 24-h urinary fractionated metanephrines in 6 patients. The dominant catecholamine profile was adrenergic (57%). In all patients concentration of 3- metoxytyramine was elevated. In one patient with paraganglioma it was the only elevated metabolite in 24-h urine collection. Moreover, chromogranin A level was substantially increased in all cases (minimum 4.8- fold the normal upper limit) and it was positively correlated with the progression of the disease. PASS score was known in three patients with pheochromocytoma, in all cases it was higher than 6. In patients with PPGL, detailed analysis of histopathological, clinical, hormonal and imaging results is essential to properly predict the possible course of the disease. In our patients, the most commonly observed metastatic potential predictors were PASS score more than 6, tumour size more than 6 cm, elevation of dopamine metabolite level. A repetitive assessment of chromogranin A concentration during follow-up may have additional value in monitoring of the disease. Due to rarity of the PPGL, the establishment of a new predictive system is difficult and requires multicentre, long-term studies.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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