Abstract
Sri Lanka Journal of Medicine is the official journal of the Kandy Society of Medicine. It is a peer-reviewed journal currently published biannually. Original articles, reviews, brief reports, case reports, points of view, and letters to the editor, in all fields of medical science and practice are accepted for publication.The Sri Lanka Journal of Medicine is now indexed in DOAJ
Highlights
Phaeochromocytomas are rare neuroendocrine tumours derived from chromaffin cells of the adrenal medulla or sympathetic ganglia that secrete catecholamines, with 0.1% prevalence and 10% malignant potential[1]
We report a patient with a clinically silent giant malignant phaeochromocytoma, to the best of our knowledge, the sixth largest published in the current literature and the largest reported in Asia
Complete surgical excision is the gold standard of treatment, which has a 5-year survival rates of 95% for benign and less than 50% for malignant phaeochromocytomas[1,3]
Summary
Phaeochromocytomas are rare neuroendocrine tumours derived from chromaffin cells of the adrenal medulla or sympathetic ganglia that secrete catecholamines, with 0.1% prevalence and 10% malignant potential[1]. A 65-year-old previously healthy female presented with a large abdominal mass, multiple neck lumps and loss of appetite and weight of one-year duration. She did not complain of chest pain, headache or palpitations. Biopsy of the cervical lymph nodes showed metastatic tumour deposits. Immunohistochemistry demonstrated positivity for Vimentin (Figure 5), focal positivity for Chromogranin (Figure 6) and negativity for PCK, LCA, Synaptophysin, EMA, CEA, CK7, CK20, CD 117, CD 10, Melan A, HMB 45 and S 100 These appearances were those of a phaeochromocytoma. The cervical lymph nodes showed metastatic deposits of a malignancy with similar immunohistochemical features as the primary tumour, enabling a conclusion of a malignant phaeochromocytoma (Figure 7). Reevaluation of the patient excluded the possibilities of associated hereditary forms of phaeochromocytoma
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