Malignancies Occurring Several Years After Onset Of The Vasculitis

Rinaldi et al. [20] studied 41 patients with cirrhosis alone, and 41 patients with cirrhosis and hepatocellular carcinoma. They showed that patients with cirrhosis are at high risk of developing hepatocellular carcinoma, especially when they have hepatitis

B or C virus infection. They also demonstrated a strong association between hepatitis C virus infection and cryoglobulinemia in cirrhosis patients with and without hepatocellular carcinoma. Cryoglobulins were detected in 88% of patients with hepatocellular carcinoma and in only 58% of patients without hepatocellular carcinoma. Ferri et al. [21] reported on the development of hepatocellular carcinoma in 3 of 250 patients with cryoglobulinemic vasculitis (mixed cryoglobulinemia) after a period of 8 (2 patients) and 16 years (1 patient). Two of the patients had hepatitis C virus infection and the remaining patient had chronic hepatitis B virus infection. Thus, not only is the hepatitis C virus associated with cryoglobulinemic vasculitis, but also with the occurrence of hepatocellular carcinoma several years after the onset of the cryoglobulinemic vasculitis [22],

La Civita et al. [4] reported the occurrence of non-Hodgkin's lymphoma in 14 out of 200 patients with cryoglobulinemic vasculitis, suggesting that cryoglobulinemia is a preneoplastic disorder. The non-Hodgkin's lymphoma developed one to 11 years (mean ±SD, 5.6 ± 3.8 years) after diagnosis of cryoglobulinemic vasculitis. In all cases, the non-Hodgkin's lymphoma was confirmed by histology. All 14 patients had anti-hepatitis C virus antibodies, and in 12 of the patients hepatitis C virus RNA was detected in the serum. These findings suggest that hepatitis C virus may influence the transition of cryoglobulinemic vasculitis from a benign to a malignant disorder. In another study, Zignego et al. [23] investigated the pathogenetic relevance of hepatitis C virus infection in cryoglobulinemic vasculitis with or without complicating B-cell non-Hodgkin's lymphoma in comparison with other immunological and lymphoproliferative disorders. They found that hepatitis C virus infection is involved in the pathogenesis of cryoglobulinemic vasculitis, both by direct participation in the immune complex- related vasculitis and by triggering the lymphoproliferative disorder underlying the disease. They concluded that non-Hodgkin's lymphoma seems to be related to hepatitis C virus lym-photropism, which could also be responsible for the evolution of cryoglobulinemic vasculitis to malignant lymphoma.


Intravascular proliferation of cells in angiocentric lymphomas of B- or T-cell origin can mimic systemic necrotizing vasculitis and lead to misdiagnosis [24— 26],

Cardiac myxomas can produce a clinical picture resembling polyarteritis nodosa. These intracardiac tumors occur predominantly in middle-aged women and are associated with fever, weight loss, arthralgias, Raynaud's phenomenon and ischemic extremities [27]. Multiple cerebral, visceral or renal aneurysms, have been detected by angiography in patients with cardiac myxomas [28], Two recent case-reports described mimicking of systemic vasculitis by the tumor: A young girl had a stroke that was attributed to a vasculitic process until the atrial myxoma was found [29], Kawasaki disease was suspected in a 13-year-old girl after detection of coronary artery occlusions and aneurysms. Echocardiography revealed myxoma and a tricuspid valve lesion that led to embolism by myxomatous material [30]. Microemboli of small arteries can cause vascular changes through seeding of the vascular wall [31]. Destruction of the internal elastic lamina and media can lead to aneurysm formation and mimic vasculitis.

A case of primary amyloidosis limited to the vascular system and masquerading as giant cell arteritis was described by Rao [32], A biopsy revealed the amyloidosis and did not show signs of vasculitis. McColl and Fraser [33] described a patient with pheochromocytoma mimicking systemic vasculitis. This patient developed ischemic colitis and died of respiratory renal failure. An autopsy revealed a unilateral pheochromocytoma and there was no evidence of vasculitis on macro- or microscopic examination.


A few reports of Wegener's granulomatosis masquerading as neoplasm have been published. Two patients presented with a pulmonary mass initially thought to be bronchial carcinoma. A biopsy revealed granulomas and led to a diagnosis of Wegener's granulomatosis [34, 35]. In 2 other patients, Wegener's granulomatosis presented with tumor-like lesions in the kidney. Histology showed granuloma formation and demonstrated necrotizing vasculitis within these lesions but disclosed no evidence of malignant disease [36, 37], Wegener's granulomatosis masqueraded as breast cancer in a woman who had had a previous diagnosis of necrotizing ophthalmitis requiring enucleation. The patient underwent mastectomy and the final pathology specimen revealed the vasculitic disease [38]. Another patient presented with unilateral ocular symptoms suspicious of a subretinal tumor. Positive titres of antineutrophil cytoplasmic antibodies led to further diagnostic procedures, including an open lung biopsy that revealed Wegener's granulomatosis [39],

In polyarteritis nodosa case-reports have documented the occurrence of a vasculitis limited to the testes which mimicked the tumor [40, 41]. Testicular cancer was excluded when pathology revealed the surprising diagnosis of the vasculitis.

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