High incidence of malignant pheochromocytoma in a surgical unit. 26 cases out of 100 patients operated from 1971 to 1991

Proye, C; Vix, Michel; Goropoulos, Apostolos; Kerlo, P.; Lecomte-Houcke, M

doi:10.1007/bf03345810

Cited by 71 publications

(50 citation statements)

References 16 publications

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“…Although none of our patients with malignant pheochromocytomas were alive after 10 years, others have reported somewhat better results [2]. The poor prognosis of patients with malignancy is thought to be attributable to dissemination to other organs, along with the heart failure associated with hypercatecholaminemia [6,[13][14][15][16][17][18]. Thus, long-term prognosis will not improve unless other features that can predict malignant behavior can be identified.…”

Section: Discussionmentioning

confidence: 98%

Predictive Characteristics of Malignant Pheochromocytoma

Park¹,

Park²,

Song³

et al. 2009

Journal of Urology

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Purpose:The prognosis of patients with malignant pheochromocytoma is poor, but the predictive factors are not well understood. We aimed to identify the clinical characteristics predictive of malignancy after initial surgical removal in patients with pheochromocytoma. Materials and Methods:We retrospectively reviewed the records of 152 patients diagnosed with pheochromocytoma, including 5 (3.3%) with metastasis at the time of the initial surgical excision and 12 (7.9%) who developed metastasis during follow-up. To determine the factors predictive of malignancy, we compared clinical, radiographical, and urinary chemical findings between patients with benign and malignant disease. Mean follow-up was 41.5 months (range, 0.9-298 months) after surgery. Results: Malignant tumors were significantly larger than benign tumors (11.1±4.0 cm vs. 6.2±3.4 cm, p＜0.001), and postoperative persistence of arterial hypertension was more frequent after removal of malignant than benign tumors (p=0.001). Among the 147 patients without metastatic disease at diagnosis, those who developed metastasis had significantly lower concentrations of urinary catecholamine metabolites per unit of tumor, including vanillylmandelic acid (1.2 vs. 3.7 mg/day/cm, p=0.049), epinephrine (4.5 vs. 168.9 μg/day/cm, p=0.008), and norepinephrine (13.1 vs. 121.8 mg/day/cm, p ＜0.001). The overall 5-year metastasis-free survival rate was 84.4% and was significantly higher in patients with smaller tumors (≤5.5 vs. ＞5.5 cm; 90.6% vs. 81.2%, p=0.025) and higher 24-hour secretion of vanillylmandelic acid (＞2.1 vs. ≤2.1 mg/ day/cm; 94.9% vs. 70.9%, p=0.019). Conclusions: Large tumor size (＞5.5 cm) and minimally elevated 24-hour urinary vanillylmandelic acid (≤2.1 mg/day/cm) were significantly associated with a higher probability of a malignant pheochromocytoma portending a lower metastasis-free survival and mandating more rigorous follow-up after surgery.

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Section: Discussionmentioning

confidence: 98%

Predictive Characteristics of Malignant Pheochromocytoma

Park¹,

Park²,

Song³

et al. 2009

Journal of Urology

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“…The prevalence of malignancy in PC ranges from 2.4 to 26%, depending on the definition of malignancy (Melicow 1977, Proye et al 1992. Although the study of Proye et al apparently overestimated the rate of malignancy by defining microscopic venous thrombi and regional invasion as true signs of malignancy.…”

Section: Introductionmentioning

confidence: 99%

Microarray analysis reveals differential expression of benign and malignant pheochromocytoma

Waldmann

Fendrich²,

Holler³

et al. 2010

Endocrine-Related Cancer

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The diagnosis of a malignant pheochromocytoma (PC) can only be established by the presence of distant metastases, but a subset of apparently benign PCs develop metastases. We have employed a microarray analysis to identify a typical gene expression profile which distinguishes malignant from benign PC. Total RNA was isolated from fresh-frozen tissue of five benign and five malignant PCs. The reference consisted of laser microdissected tissue from normal adrenal medulla. After generating Cy3-and Cy5-fluorescently labeled cDNAs, F-chips containing 11 540 spots were hybridized. Data were analyzed with the IMAGENE 3.0 software. Gene expression levels were validated by real-time (RT)-PCR and immunohistochemistry (IHC). The analysis revealed a more than twofold difference in expression between benign and malignant PCs in 132 genes: 19 were up-regulated and 113 were down-regulated. Expression differences of six genes (calsequestrin, NNAT, neurogranin, secreted protein acidic and rich in cysteine (SPARC), EGR2, and MAOB) were confirmed by RT-PCR in 25 PCs. IHC for calsequestrin revealed an overexpression in malignant PCs (7/10 vs 1/10, PZ0.03). Comparative analysis by microarray of all ten PCs (benign/malignant) versus normal adrenal medulla revealed a more than twofold expression difference in 455/539 and 491/671 genes respectively. Several of these genes are known to participate on adrenal tumorigenesis, potential tumor suppressor genes, and oncogenes. Comprehensive gene expression analysis of malignant and benign PCs revealed different gene profiles, which could be used to discriminate between malignant and benign PCs. Based on these findings, the strategy for further follow-up and treatment could be modified accordingly.

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“…However, even local phaeochromocytomas may be symptomatic due to high catecholamine secretion, leading to high blood pressure, pallor, flushing and constipation. From 2.4 to 26% of all, phaeochromocytomas are metastatic ( Melicow 1977, Proye et al 1992). …”

Section: Introductionmentioning

confidence: 99%

High frequency of SNAIL-expressing cells confirms and predicts metastatic potential of phaeochromocytoma

Häyry¹,

Salmenkivi²,

Arola³

et al. 2009

Endocrine-Related Cancer

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Phaeochromocytomas are uncommon tumours of adrenal or extra-adrenal chromaffin tissue. About 2-26% of these have been reported to metastasize, but, on histological criteria, it is virtually impossible to predict malignant behaviour of the tumour. Using immunohistochemistry, we analysed the protein expression of SNAIL, a zinc-finger transcription factor, in a series of 50 phaeochromocytoma specimens from 42 patients. We found that SNAIL-expressing cells are frequent in metastatic primary tumours and their metastases, whereas in tumours without metastases, SNAIL expression is commonly absent. We conclude that the expression of SNAIL may be of use in predicting the metastatic potential of phaeochromocytoma.

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High incidence of malignant pheochromocytoma in a surgical unit. 26 cases out of 100 patients operated from 1971 to 1991

Cited by 71 publications

References 16 publications

Predictive Characteristics of Malignant Pheochromocytoma

Predictive Characteristics of Malignant Pheochromocytoma

Microarray analysis reveals differential expression of benign and malignant pheochromocytoma

High frequency of SNAIL-expressing cells confirms and predicts metastatic potential of phaeochromocytoma

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