[Two cases of malignant and benign systemic mastocytosis]. / Malignus és benignus systemás mastocytosis egy-egy esete.

The authors give a short account about the clinical histories of two patients: one with malignant systemic mastocytosis resulting in acute myeloid leukaemia, the other with indolent systemic mastocytosis. A brief review is reported about the physiological and pathophysiological role of mastocyte system. Benign and malignant types, classification of mastocyte proliferation are detailed, several distinct characteristics of clinical appearance, main aspects of diagnosis, therapy and prognosis in patients with different forms of systemic mastocytosis are briefly discussed.

[Ganglioneuroma of the adrenal gland]. / Mellékvese ganglioneuromák.

149 patients with adrenal incidentalomas were examined. Sixty-eight cases were histologically confirmed, five of them had ganglioneuromas. On the basis of these patients history current knowledge of this benign tumour was summarized. Histological and pathological characteristics of one tumour suggest that ganglioneuromas may develop by maturing of malignant neuroblastic tumours. The clinical symptoms (abdominal pain, meteorism) were local. In 2 of 5 cases mildly elevated levels of urinary vanillylmandelic acid and catecholamine could be measured. One patient had persisting hypertension after surgery. In an other patient previous diarrhoea stopped after the removal of tumour. On the basis of ultrasound and computertomographic features, the size and origin of a tumour and its relation to the surrounding organs can be well characterized. One patient was inoperable because of an infiltratively spreading tumour, but during five years of follow-up no tumour progression could be observed with computertomography. After surgery we could follow only 2 of 4 patients. Until now no recurrence of tumour were detected.

Low dehydroepiandrosterone sulfate (DHEA-S) level is not a good predictor of hormonal activity in nonselected patients with incidentally detected adrenal tumors.

To assess its differential diagnostic value, dehydroepiandrosterone sulfate (DHEA-S) was measured in a nonselected cohort of 84 patients with incidentally detected adrenal tumors (incidentaloma). Of the 38 histologically confirmed cases, 6 of 12 patients with primary or metastatic malignant tumor of the adrenals and 7 of 14 patients with benign cortical adenoma had low DHEA-S levels. Thus, the sensitivity, specificity, and predictive value of a low DHEA-S level to indicate a benign adrenal tumor were 0.35, 0.50, and 0.60, and the values to indicate a cortical adenoma were 0.50, 0.67, and 0.47, respectively. Of the 14 cases of histologically confirmed benign cortical adenoma, 10 had signs of hormonal activity, but DHEA-S was suppressed in only 7 cases. Thus, the sensitivity, specificity, and predictive value of a low DHEA-S level to indicate clinically significant hormonal activity of a benign cortical adenoma were 0.60, 0.75, and 0.86, respectively. For comparison, 5 of 5 males and 2 of 5 females with metastatic carcinomatosis, but without involvement of the adrenals, also had low DHEA-S levels. The data clearly show that in nonselected cases of incidentaloma a suppressed DHEA-S level is not a good predictor of hormonal activity and that DHEA-S measurement may be valuable only after having ascertained the cortical origin and benign feature of the tumor.

Incidentally detected adrenal tumours (incidentalomas): histological heterogeneity and differentiated therapeutic approach.

OBJECTIVES: To find a differentiated therapeutical approach to incidentalomas. DESIGN: Prospective study of incidentalomas: their histology, hormonal activity, and growth if primarily non-operated. SETTING: Admissions to an academic tertiary care centre because of incidentaloma. PATIENTS: Thirty-eight female, 25 male, 27-85 years old. INTERVENTIONS: Ultrasound, computed tomography and adrenal scintigraphy when appropriate; investigations to exclude malignancy giving metastasis into the adrenal gland; ACTH, cortisol, aldosterone, renin activity, androgene, catecholamine measurements; surgery or follow-up. MAIN OUTCOME MEASURES: Rate of malignancy; linkage of tumour size to probability of malignancy; prevalence of tumours with subtle hormone excess. RESULTS: Cortical adenomas occurred in 13/31, metastases in 7/31 of the histologically confirmed cases. In 10/31 cases, the computed tomography indicated a size that was smaller than the pathological size (mean = 20 mm). 20.6% of all incidentalomas and 61.5% of the 13 operated corticol adenomas showed subtle hormonal activity. Twenty-seven incidentalomas < 30 mm were followed-up (3-41 months; mean 18 months) and showed growth in only one case. Sensitivity, specificity, and predictive value (PV) of a tumour > or = 30 mm to indicate malignancy were: 1.0, 0.56 and 0.27, respectively. The PV of a < 30 mm tumour to exclude malignancy was 1.0. CONCLUSIONS: Oncological screening tests are necessary in all incidentalomas. Tumours > or = 30 mm should be operated but smaller ones can be followed-up, because they are usually benign and rarely show progressive growth. Cortical adenomas with subtle hormonal overproduction and hypertension, diabetes or osteoporosis should be operated, irrespective of their size, but in the absence of relevant clinical symptoms they can be followed-up.