Clinical Differences between Benign and Malignant Pheochromocytomas.

Abstract

Abstract. Most pheochromocytomas can be cured by resection. In view of the unfavourable prognosis for surgical therapy in cases of late tumour detection and malignant tumours, the aim of the present study is to differentiate between typical signs and symptoms of malignant versus benign pheochromocytomas. We investigated the records of 133 patients retrospectively . In cases of benign tumours (104 of 133, mean age 42± 15.8 years) tumour size was 5.9±3.4 cm, and history was 47.4±75.4 months. 7.7% of the tumours were extraadrenal, and 77% had paroxysmal manifestations. The other 29 patients (mean age: 39.2±21.9 years) had malignant lesions (tumour size: 9.4±5.9 cm (p=0.0022); history: 7.4±5.6 months (p=0.0137); extraadrenal: 24.1% (p=0.0219); paroxysmal: 37.9% (p=0.0012)). Symptoms of patients with benign tumours were hypertension (80%), headaches (42.3%), sweating (30.8%), tachycardia (26%) and pallor (24% (Malignant: Hypertension 46%, p=0.0873; headaches 11%, p=0.0008; sweating 11 %, p=0.0196; tachycardia 14%, p = 0.1961 and pallor 0%, p=0.0010). Abdominal pain and dorsalgia occurred more frequently in malignant pheochromocytomas (26% versus 7%, p = 0.0014). Unusually short histories and extraadrenal localization appear to be suspicious for malignancy. The "typical" clinical signs and symptoms occur more frequently in patients with benign tumours and can therefore be regarded as typical signs of benign pheochromocytomas. Malignant pheochromocytomas are more difficult to treat owing to local inoperability arising from either organ invasion or metastasis. A remission rate of up to 30% has been reported in response to radiotherapy with [1311]-meta-iodobenzylguanidine

Giant Malignant Cystic Pheochromocytoma: A Case Report

“…As with other neuroendocrine tumors, the diagnosis of malignancy is not primarily based on cytological characteristics, but is defined by the presence of local invasion or metastatic disease. The presence of metastases of chromaffin tissue at sites where no chromaffin tissue should be expected provides the only currently widely accepted means of defining malignancy [3]. In our case, the discovery of small liver metastasis confirms the diagnosis of malignancy [3].…”

“…The presence of metastases of chromaffin tissue at sites where no chromaffin tissue should be expected provides the only currently widely accepted means of defining malignancy [3]. In our case, the discovery of small liver metastasis confirms the diagnosis of malignancy [3]. The incidence of metastatic pheochromocytoma is between 13 % and 26 % [4].…”

Pheochromocytoma and Paraganglioma

“…Pheochromocytoma metastasizes via haematogenous or lymphatic pathways, and the most common metastatic sites are lymph nodes, bone, lung and liver (Bravo, 1994;Glodny et al, 2001;Goldstein et al, 1999;Kopf et al, 2001;Schlumberger et al, 1992). Among all pheochromocytomas, the frequency of malignant pheochromocytomas ranges from 1 to 90% (Amar et al, 2005a;Benn et al, 2006;Brouwers et al, 2006;Glodny et al, 2001;Timmers et al, 2007c;Timmers et al, 2009).…”

“…We have recently found an unexpected high frequency of SDHB mutation in paediatric malignant pheochromocytomas and paragangliomas (Pacak et al, unpublished observation). Recent advances in biochemical testing and nuclear imaging techniques, as discussed previously, have greatly improved our ability to diagnose and localize malignant pheochromocytoma at much earlier stages.Clinical manifestations of malignant pheochromocytoma are similar to that of its benign counterpart, and patients may have minimal symptoms or symptoms caused by local invasion despite marked hypercatecholaminaemia (Bravo and Gifford, 1984;Glodny et al, 2001;Goldstein et al, 1999;Kopf et al, 2001;Mornex et al, 1992;Schlumberger et al, 1992;Timmers et al, 2007c). Similar to benign pheochromocytomas, malignant pheochromocytomas predominantly secrete norepinephrine Schlumberger et al, 1992;Stumvoll et al, 1997).…”

Hypertension and the eye: changing perspectives

“…31 No difference, however, is seen in the prevalence of malignant hypertension, between malignant and nonmalignant pheochromocytomas. 32 In primary hyperaldosteronism hypertensive retinopathy is usually less severe. 26 Refractory hypertension: Refractory hypertension is associated with a high prevalence of target-organ damage at cardiac, macro-and microvascular level with advanced retinal involvement (grades II and III retinopathy).…”