Phaeochromocytomas in 72 patients: Clinical and diagnostic features, treatment and long term results

Modlin, I M; Farndon, John; Shepherd, Annemarie F.; Johnston, I. D. A.; Kennedy, T. L.; Montgomery, D. A. D.; Welbourn, Richard

doi:10.1002/bjs.1800660704

Cited by 169 publications

(79 citation statements)

References 27 publications

(22 reference statements)

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“…We found that the incidence of malignant pheochromocytomas among 152 patients with pheochromocytomas was 11.2%, in good agreement with the 8% to 12.5% previously reported [7][8][9][10][11]. We also found that the 5-year survival rate of our patients with malignant pheochromocytoma was 11.8%, which was thus somewhat lower than the 20% previously reported [12].…”

Section: Discussionsupporting

confidence: 89%

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: Discussionsupporting

confidence: 89%

Predictive Characteristics of Malignant Pheochromocytoma

Park¹,

Park²,

Song³

et al. 2009

Journal of Urology

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“…These include rhabdomyosarcoma and phaeochromocytoma (Modlin et al, 1979;Hartley et al, 1988). We encountered one case of rhabdomyosarcoma and one case of paraganglioma.…”

Section: Discussionmentioning

confidence: 99%

A prospective study of neurofibromatosis type 1 cancer incidence in the UK

et al. 2006

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Neurofibromatosis type 1 (NF1) is an autosomal dominant condition affecting around one in 3000 live births. The manifestations of this condition are extremely variable, even within families, and genetic counselling is consequently difficult with regard to prognosis. Individuals with NF1 are acknowledged to be at increased risk of malignancy. Several studies have previously attempted to quantify this risk, but have involved relatively small study populations. We present prospective data from 448 individuals with NF1 with a total of 5705 years of patient follow-up. These data have been collected via the UK NF1 association for patients. Demographic information on the affected individuals was cross-referenced with UK cancer registry data by the UK Office of National Statistics. The overall risk of cancer was 2.7 times higher in this cohort of NF1 patients than in the general population (95% confidence interval (CI) 1.9 -3.7). The cumulative risk of a malignancy by age 50 years was 20% (95% CI 14 -29%); beyond this age, the risk of cancer was not significantly elevated (P ¼ 0.27). The most frequent types of cancer were connective tissue (14% risk by age 70, 95% CI 7.8 -24%) and brain tumours (7.9, 95% CI 3.9 -16%). There was no statistically significant excess of cancers at other sites (P ¼ 0.22).

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“…The most rigorous definition of malignancy is that which requires that metastases should be present at a site where chromaffin tissue is not otherwise found, thus excluding the possibility of misclassifying multifocal primary lesions as metastases. The prognosis from a variety of reports of malignant phaeochromocytomas appears highly variable (Palmieri e f d., James et al, 1972;Mahoney & Harrison, 1977;Modlin et al, 1979).…”

Section: B Sliapiro Etmentioning

confidence: 99%

Malignant Phaeochromocytoma: Clinical, Biochemical and Scintigraphic Characterization

Shapiro

Sisson

Lloyd

et al. 1984

Clinical Endocrinology

128

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SUMMARYWe have evaluated thirty patients with malignant metastatic phaeochromocytoma with regard to clinical features, indices of catecholamine secretion, histology of lesions and a number of imaging procedures including scintigraphy with the recently developed sympathetic tissue-seeking radiopharmaceutical '3'I-metaiodobenzylguanidine (I3'I-MIBG). The primary tumour was extraadrenal in 13 cases. The commonest site of metastases was the axial skeleton (20 cases), followed by liver (four cases), lymph nodes (four cases), peritoneum (two cases) and lung (three cases). The malignancies were indolent, the mean time following the initial diagnosis was 9.18 years (range 0 to 33 years) and the mean duration of known metastases 3.7 1 years (range 0 to 18 years). There was a wide range of abnormalities in plasma and urinary catecholamines which did not correlate with the extent of tumour spread, histological pattern (mitotic index, Zellballen pattern, capsular or vascular invasion pleomorphism or necrosis) or I3II-MIBG uptake by tumour deposits. I3'I-MIBG scintigraphy was found to be a useful technique for determining the extent of metastatic disease in most cases (26 of 30) and in some patients (1 6 of 30) was more sensitive than other radiological procedures. No false positive scans were encountered.

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Phaeochromocytomas in 72 patients: Clinical and diagnostic features, treatment and long term results

Abstract: SUMMARY Phaeochromocytomas were diagnosed in 72 patients in Hammersmith, Beljiast and Newcastle between 1955 and 1976. Fourteen

Cited by 169 publications

References 27 publications

Predictive Characteristics of Malignant Pheochromocytoma

Predictive Characteristics of Malignant Pheochromocytoma

A prospective study of neurofibromatosis type 1 cancer incidence in the UK

Malignant Phaeochromocytoma: Clinical, Biochemical and Scintigraphic Characterization

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