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Pathology - Phaeochromocytoma
A neoplasm originating from the chromaffin cells of the adrenal medulla.
Epidemiology: Rare, having a yearly incidence of 8 per million. Aetiology • The majority are irregular. Approximately 10% are linked to hereditary illnesses, including MEN 2, neurofibromatosis type 1, and von Hippel–Lindau disease. Hereditary tumors are more prone to manifest at a younger age and exhibit bilateral occurrence. Genetics • Sporadic tumors exhibit a significant prevalence of 1p loss of heterozygosity.
Presentation • Patients experience sudden episodes of pulsating headaches, diaphoresis, palpitations, thoracic pain, and abdominal discomfort resulting from elevated circulating catecholamines produced by the tumor. • Hypertension is frequently observed
Elevated urine catecholamines and their metabolites serve as a significant diagnostic tool.
Macroscopy • A well-defined tumor exhibiting a firm grey cut surface. • Average dimensions of 6 cm and a mass of 200 g.
Histopathology • Tumor cells exhibit distinctive clusters ('zellballen') interspersed with a fine vascular network. • The cells are polygonal, possessing granular basophilic cytoplasm. • Nuclei display a peculiar stippled chromatin pattern. • Scattered pleomorphic nuclei may be observed.
Prognosis • 90% exhibit a benign behavior. • Regrettably, histological analysis is not dependable in forecasting the 10% that may be malignant; however, concerning indicators include invasive development, necrosis, and elevated mitotic activity.
A neoplasm originating from the chromaffin cells of the adrenal medulla.
Epidemiology: Rare, having a yearly incidence of 8 per million. Aetiology • The majority are irregular. Approximately 10% are linked to hereditary illnesses, including MEN 2, neurofibromatosis type 1, and von Hippel–Lindau disease. Hereditary tumors are more prone to manifest at a younger age and exhibit bilateral occurrence. Genetics • Sporadic tumors exhibit a significant prevalence of 1p loss of heterozygosity.
Presentation • Patients experience sudden episodes of pulsating headaches, diaphoresis, palpitations, thoracic pain, and abdominal discomfort resulting from elevated circulating catecholamines produced by the tumor. • Hypertension is frequently observed
Elevated urine catecholamines and their metabolites serve as a significant diagnostic tool.
Macroscopy • A well-defined tumor exhibiting a firm grey cut surface. • Average dimensions of 6 cm and a mass of 200 g.
Histopathology • Tumor cells exhibit distinctive clusters ('zellballen') interspersed with a fine vascular network. • The cells are polygonal, possessing granular basophilic cytoplasm. • Nuclei display a peculiar stippled chromatin pattern. • Scattered pleomorphic nuclei may be observed.
Prognosis • 90% exhibit a benign behavior. • Regrettably, histological analysis is not dependable in forecasting the 10% that may be malignant; however, concerning indicators include invasive development, necrosis, and elevated mitotic activity.
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