One hundred two patients with pheochromocytoma treated at a single institution since the introduction of laparoscopic adrenalectomy. Arch Surg
University of California-San Francisco/Mount Zion Medical Center, 1600 Divisadero Street, San Francisco, CA 94115, USA. Archives of surgery (Chicago, Ill.: 1960)
(Impact Factor: 4.93).
09/2010; 145(9):893-7. DOI: 10.1001/archsurg.2010.159
Pheochromocytoma can be safely treated laparoscopically; "subclinical" pheochromocytoma is increasingly common.
Patients undergoing adrenalectomy for pheochromocytoma at our institution in 1994 to 2009.
Laparoscopic, hand-assisted, and open adrenalectomy.
Preoperative and postoperative clinical and biochemical data.
One hundred two patients (52 women, 50 men) with pheochromocytoma underwent 108 operations. Ninety-seven operations were laparoscopic; 7, open; and 4, converted from laparoscopic to hand assisted or open. Six operations were bilateral; 3 were subtotal cortex-sparing resections. Thirty-four patients (33%) presented with adrenal incidentaloma and minimal symptoms, 28 within the past decade. Ten patients had paragangliomas, 7 of whom underwent laparoscopic resection. The mean (SD) tumor size was 5.3 (2.8) cm. Seven patients had recurrence requiring reoperation; the mean length of time between initial operation and recurrence was 6 years (range, 6 months to 17 years). There were no perioperative deaths.
Laparoscopic adrenalectomy can be safely performed for pheochromocytoma in more than 90% of cases. More than one-third of patients presented with subclinical pheochromocytoma. Patients should be followed up closely because recurrence may develop several years after adrenalectomy.
Available from: Alexandra Smirnova
- "Cardiogenic shock can be the first major clinical manifestation of pheochromocytoma   . Extra-adrenal pheochromocytoma (paraganglioma) is extremely rare, but can also evoke a catecholamine crisis . "
Available from: PubMed Central
- "Incidental discovery of PPGLs on imaging is becoming an increasingly important mode of their diagnosis; for instance, the proportion of PCs detected incidentally is now 25–30% of all cases (Mannelli et al. 1999, Amar et al. 2005, Kopetschke et al. 2009, Shen et al. 2010). Specific imaging findings are discussed in more detail below. "
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ABSTRACT: The paraganglioma (PGL) syndromes types 1-5 are autosomal dominant disorders characterized by familial predisposition to PGLs, phaeochromocytomas (PCs), renal cell cancers, gastrointestinal stromal tumours and, rarely, pituitary adenomas. Each syndrome is associated with mutation in a gene encoding a particular subunit (or assembly factor) of succinate dehydrogenase (SDHx). The clinical manifestations of these syndromes are protean: patients may present with features of catecholamine excess (including the classic triad of headache, sweating and palpitations), or with symptoms from local tumour mass, or increasingly as an incidental finding on imaging performed for some other purpose. As genetic testing for these syndromes becomes more widespread, presymptomatic diagnosis is also possible, although penetrance of disease in these syndromes is highly variable and tumour development does not clearly follow a predetermined pattern. PGL1 syndrome (SDHD) and PGL2 syndrome (SDHAF2) are notable for high frequency of multifocal tumour development and for parent-of-origin inheritance: disease is almost only ever manifest in subjects inheriting the defective allele from their father. PGL4 syndrome (SDHB) is notable for an increased risk of malignant PGL or PC. PGL3 syndrome (SDHC) and PGL5 syndrome (SDHA) are less common and appear to be associated with lower penetrance of tumour development. Although these syndromes are all associated with SDH deficiency, few genotype-phenotype relationships have yet been established, and indeed it is remarkable that such divergent phenotypes can arise from disruption of a common molecular pathway. This article reviews the clinical presentations of these syndromes, including their component tumours and underlying genetic basis.
© 2015 The authors.
Available from: degruyter.com
- "It is a result of the overproduction of catecholamines , uncontrolled release of which may end up in a life-threatening rise of arterial blood pressure. The incidence of pheochromocytoma is estimated to be from 2 to 8 cases annually per million habitants . It is the cause of secondary hypertension in 0,1% to 0,6% of patients , being the top reason of paroxysmal hypertension cases  . "
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Pheochromocytomas may cause life-threatening episodes of arterial hypertension and surgical treatment is obligatory following proper general medical preparation.
Material and methods
There were 63 patients in years 2006–2011 operated in the department due to pheochromocytoma. The group comprised 38 women and 25 men of the age range 16–80, mean 44,7. All the specimen were analyzed in pathological examination. The regressive changes that were found were subsequently compared with the clinical course of the pheochromocytoma both in the preoperative period and at the time of the surgery.
There were 44 laparoscopic adrenalectomies performed, out of which 5 resulted in conversions to open surgery, while 19 patients were operated primarily via open access. The indications for the open procedures: extraadrenal tumors, fibrotic-infiltrative lesions suggestive of malignancy, vast intratumoral extravasation, and respiratory failure. In all the postoperative specimens pheochromocytomas were found. In 29 cases intratumoral haemorrhages were observed, in 17 — tumoral necrosis at different stages, and in 3 cases posthaemorrhagic cystis. In 6 cases the lesions were accompanied by major fibrosis and hyalinization.
There is a statistically significant relationship between regressive changes observed within phaeochromocytomas and a reduction of paroxysmal hypertension at the time of adrenalectomy (p=0,012).
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