SummaryPheochromocytoma is a rare tumor of the adrenal gland. It often presents with the classic triad of headache, palpitations and generalized sweating. Although not described as a typical symptom of pheochromocytoma, anxiety is the fourth most common symptom reported by patients suffering of pheochromocytoma. We report the case of a 64 year old man who had severe anxiety and panic disorder as presenting symptoms of pheochromocytoma. After 13 years of psychiatric follow-up, the patient was diagnosed with malignant pheochromocytoma. After surgical resection of his pheochromocytoma and his hepatic metastases, the major panic attacks completely disappeared, the anxiety symptoms improved significantly and the psychiatric medications were stopped except for a very low maintenance dose of venlafaxine. We found in our cohort of 160 patients with pheochromocytoma 2 others cases of apparently benign tumors with severe anxiety that resolved after pheochromocytoma resection. These cases highlight that pheochromocytoma should be included in the differential diagnosis of refractory anxiety disorder.Learning points:Anxiety and panic disorder may be the main presenting symptoms of pheochromocytoma.The diagnosis of pheochromocytoma should be excluded in cases of long-term panic disorder refractory to medications since the anxiety may be secondary to a catecholamine-secreting tumor.Surgical treatment of pheochromocytoma leads to significant improvement of anxiety disorders.
Context: Adrenocortical carcinoma (ACC) is a rare tumor with an incidence of 0.7-2 per million. Based on the ENSAT staging classification, tumor stage is the most important prognostic factor; the presence of lymph nodes involvement and metastases is an indicator of poor prognosis. Absence of any local or distant tumor invasion represents an early stage disease and is classified based on tumor size of <5 cm (stage I) or >5 cm (stage II). Advanced disease is confirmed if there is tumoral invasion, either locally in the surrounding tissues/nodes (stage III) or in other organs/vascular structures (stage IV). Objective: To describe patient characteristics, staging and modes of presentation at initial diagnosis in our cohort of ACC patients. Methods: We retrospectively reviewed paper and electronic charts of patients with pathology-confirmed ACCs who were treated at our referral center from 1995 to May 2019. Results: One hundred four patients were diagnosed with ACC: 28 were men (26.9%) and 76 (73.1%) were women and median age was 51 years. The overall modes of presentation were hormonal hypersecretion (40.4%), mass-related symptoms (36.5%), incidentalomas (17.3%) and unknown (1.9%). Hormonal profile was available for 71 tumors: 67,6 % were secreting [androgen and cortisol co-secretion (39.4%), cortisol only (28.2%)] and 18,3% were non-secreting. At initial diagnosis, sixty-four patients (61.5%) had tumors >10 cm including 32.7% between 10-14.9 cm (n:34), 19.2% were 15-20 cm (n:20) and 9.6% were >20cm (n:10). Initial ENSAT stages were I (6.7%), II (17.3%), III (28.8%) and IV (44.2%) and unknown (2,9%). The age repartition was similar for most patients (median ~50 yo) regardless of disease stage or tumor size except in the subgroup of very large tumors (>20 cm) for which the median age was 40 yo. The mode of presentation at initial diagnosis varied at various stages. Incidentaloma was a frequent mode of presentation of earlier ACC stages; Stage 1: 3/7 (42,9%), stage II: 7/18 (38,9%), stage III: 4/30 (13,3%) and stage IV: 4/46 (8,7%). Hormonal excess symptoms led to ACC diagnosis less frequently in early stages (stages I and II) (24%) than in later stages (stage III and IV) (47,3%), while the hormonal work up showed high prevalence of secreting tumors in both groups (58,8% and 88,7%). Mass-related initial symptoms were similar in both groups 36% vs 39%. Conclusions: In our cohort, 61.5% of ACC tumors were larger than 10 cm at initial diagnosis. Seventy-three percent of ACC patients had an advanced ENSAT stage III or IV disease which is associated with a 5 years survival of less than 50%. Incidentalomas is a frequent mode of presentation in stages I and II, while clinical hormonal excess symptoms were more frequent in later stages III and IV. Early stage diagnosis presents a difficult challenge in ACC and new biomarkers are needed to improve the odds against this deadly cancer.
Background: This study examined the magnitude of changes and the time required to observe maximal changes in LDL cholesterol (LDL-c), HDL cholesterol (HDL-c), triglycerides (Tg) and non-HDL-c after the introduction of mitotane. Methods: Retrospective study of 45 patients with ACC who were treated at the Centre hospitalier de l’Université de Montréal (CHUM). Clinical and biochemical data were collected, including lipid profiles before and during the first year of treatment with mitotane. Results: Among the 45 studied patients, 26 (58%) had a complete lipid profile before the introduction of mitotane, and at least 1 lipid profile during the first year of treatment, and 19 patients (42%) had a lipid profile following initiation of the treatment. Among the 26 patients who had lipid profiles before and after the introduction of mitotane, the increase of LDL-c was 2.19 mmol/L (76%) (P<0.0001), HDL-c was 0.54 mmol/L (35%) (P=0.0002), Tg was 1.80 mmol/L (129%) (P<0.0001) and non-HDL-c was 2.73 mmol/L (79%) (P<0.0001). Between the first and the sixth month of mitotane treatment, peak values (n=45) of LDL-c and non-HDL-c were reached in 42 patients (93%) and 37 patients (82%), respectively, whereas peak values of HDL-c were reached after 6 months of mitotane treatment in 29 patients (66%). The peak value of Tg was almost equal throughout the first year. The mean peak values of HDL-c, Tg and non-HDL-c showed significant associations with their respective mitotane concentrations (β=0.352, P=0.03; β=0.406, P=0.02; and β=0.339, P=0.05). Conclusion: The introduction of mitotane produces a clinically significant elevation of lipid parameters (LDL-c, HDL-c, Tg and non-HDL-c) during the first year of treatment.
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