Malignant pheochromocytoma in childhood: Report of a case with familial neurofibromatosis

Quissel, Barbara; Mohammad, Akbar M.; Bauer, John H.; Hakami, Nasrollah

doi:10.1002/mpo.2950070407

Cited by 11 publications

(1 citation statement)

References 13 publications

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“…Phaeochromocytomas are uncommon in patients younger than 20 years of age (when extra-adrenal tumours mostly occur) and their incidence peaks in the fourth decade of life (Bravo & Tagle 2003). Malignant phaeochromocytoma is rare in childhood and most published reports refer to isolated case reports (Quissel et al 1979). The incidence of extra-adrenal disease is higher in children, reaching 50% of cases to one extensive review (Coutant et al 1999).…”

Section: Clinical Features Of Malignant Chromaffincell Tumoursmentioning

confidence: 99%

The diagnosis and management of malignant phaeochromocytoma and paraganglioma

Chrisoulidou

Kaltsas²,

Ilias³

et al. 2007

Endocr Relat Cancer

293

219

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Malignant phaeochromocytomas are rare tumours accounting for w10% of all phaeochromocytomas; the prevalence of malignancy among paragangliomas is higher, especially those associated with succinate dehydrogenase subunit B gene mutations. Although a subset of these tumours has metastatic disease at initial presentation, a significant number develops metastases during follow-up after excision of an apparently benign tumour. Clinical, biochemical and histological features cannot reliably distinguish malignant from benign tumours. Although a number of recently introduced molecular markers have been explored, their clinical significance remains to be elucidated from further studies. Several imaging modalities have been utilised for the diagnosis and staging of these tumours. Functional imaging using radiolabelled metaiodobenzylguanidine (MIBG) and more recently, 18 F-fluorodopamine and 18 F-fluorodopa positron emission tomography offer substantial sensitivity and specificity to correctly detect metastatic phaeochromocytoma and paraganglioma and helps identify patients suitable for treatment with radiopharmaceuticals. The 5-year mortality rate of patients with malignant phaeochromocytomas and paragangliomas greater than 50% indicates that there is considerable room for the improvement of currently available therapies. The main therapeutic target is tumour reduction and control of symptoms of excessive catecholamine secretion. Currently, the best adjunctive therapy to surgery is treatment with radiopharmaceuticals using 131 I-MIBG; however, this is very rarely curative. Chemotherapy has been used for metastatic disease with only a partial and mainly palliative effect. The role of other forms of radionuclide treatment either alone or in combination with chemotherapy is currently evolving. Ongoing microarray studies may provide novel intracellular pathways of importance for proliferation/cell cycle control, and lead to the development of novel pharmacological agents.

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Section: Clinical Features Of Malignant Chromaffincell Tumoursmentioning

confidence: 99%

The diagnosis and management of malignant phaeochromocytoma and paraganglioma

Chrisoulidou

Kaltsas²,

Ilias³

et al. 2007

Endocr Relat Cancer

293

219

View full text Add to dashboard Cite

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Malignant phaeochromocytoma: Report of a case and a critical review

et al. 1982

Journal of British Surgery

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A right adrenal phaeochromocytoma is described in a 64-year-old man. Metastases to bone and subsequent death following cord compression occurred within 2 years of diagnosis. Twenty-seven cases in the English literature have been reviewed since 1968. In three of these, the pathological proof was unsatisfactory for the diagnosis of malignancy, and one further case was inadequately documented. The variation in incidence of malignancy found in large retrospective series may relate to inconsistency in pathological criteria, as well as to selective reporting of cases.

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