An unusual case of spontaneous rupture of a clinically "silent" phaeochromocytoma.
Sapienza P,Tedesco M,Graziano P,Moretti M,Mingazzini P L,Cavallaro A
A case of an adrenal mass which presented as spontaneous rupture contained by its own capsule is described. Clinical symptoms were aspecific and endocrinological examinations were within normal range. CT scan missed the presence of the adrenal tumor and interpreted the mass as a large haematoma arising from the left kidney. The pathological diagnosis was phaeochromocytoma. The management of "silent" phaeochromocytoma and its atypical presentation are discussed. A review of the international literature was also made collecting 29 cases of spontaneous rupture of an adrenal phaeochromocytoma.
Mannelli Massimo,Lenders Jacques W M,Pacak Karel,Parenti Gabriele,Eisenhofer Graeme
Best practice & research. Clinical endocrinology & metabolism
Phaeochromocytomas and paragangliomas are neural crest-derived tumours. Autopsy studies indicate that relatively large numbers of these tumours remain undiagnosed during life. This may reflect non-specific signs and symptoms and low medical alertness in evaluating the clinical picture or it may reflect a silent clinical presentation - the subclinical phaeochromocytoma. The associated clinical picture depends on the capacity of the tumours to release catecholamines and sometimes biologically active peptides. Hypertension is the hallmark of catecholamine release, but the amount, type and pattern of catecholamine secretion is extremely variable. Some tumours have low or intermittent secretory activity, some produce mainly or solely dopamine, while others very rarely do not synthesize or release any catecholamines (non-secretory or non-functional tumours). Such tumours may present with mild or even absent signs and symptoms of catecholamine excess. Low secretory activity may reflect small tumour size or differences in secretory phenotypes associated with the biochemical and genetic background of the tumours. Tumours due to succinate dehydrogenase subunit B mutations are often subclinical, poorly differentiated, contain low amounts of catecholamines, and are usually malignant at diagnosis. Adrenoceptor desensitization can result in a subclinical presentation, even when catecholamine levels are high. Subclinical phaeochromocytomas are often discovered as incidentalomas during radiological procedures or during routine screening for phaeochromocytoma in carriers of mutations in one of the ten currently identified tumour susceptibility genes. Undiagnosed phaeochromocytomas, whether or not subclinical and even if biologically benign, may cause extremely deleterious consequences or even death, following abrupt release of catecholamines.
Preoperative alpha-blockade in phaeochromocytoma and paraganglioma: is it always necessary?
Isaacs Michelle,Lee Paul
Resection of phaeochromocytoma and paraganglioma (PPGL) is traditionally preceded by alpha-blockade to prevent complications of haemodynamic instability intraoperatively. While there is general agreement on preoperative alpha-blockade for classic PPGLs presenting with hypertension, it is less clear whether alpha-blockade is necessary in predominantly dopamine-secreting tumours, normotensive PPGLs, as well as tumours that appear to be biochemically 'silent'. Preoperative management of these 'atypical' PPGLs is challenging and the treatment approach must be individualized, carefully weighing the risk of intraoperative hypertension against the possibility of orthostatic and prolonged postoperative hypotension. Consideration of antihypertensive medication pharmacology in the light of catecholamine physiology and PPGL secretory profile will facilitate the formulation of individualized preoperative preparatory strategies.