Advanced systemic amyloidosis secondary to metastatic renal cell carcinoma

Abstract

Secondary amyloidosis is a rare complex complication related to chronic inflammatory disease. This complication is sparsely associated to malignant neoplasms. Renal cell carcinoma (RCC) is the most common solid organ malignancy related with this paraneoplastic syndrome. Some case reports have described stabilisation or even remission of amyloidosis with cytoreductive nephrectomy. Majority of those reports were based on locally advanced RCC. We report the first case of early aggressive systemic secondary amyloidosis in high-volume metastatic RCC. The subject was diagnosed with metastatic RCC within 6 months of secondary amyloidosis; on month 5 of initiation of targeted therapy (pazopanib) developed nephrotic syndrome with a heavy proteinuria (>18 g/day), severe hypoalbuminaemia (1.53 g/dL), intense and progressive oedema, severe pancolitis and mild dyspnoea with hypotension. A colon biopsy and the immunohistochemistry confirmed the histological diagnosis of a secondary amyloidosis. The multidisciplinary tumour board decided to perform cytoreductive nephrectomy in order to reduce the pro-inflammatory status. Pathology report showed a complete resection of clear cell RCC plus renal amyloid deposits. The patient died within 4 days of surgery due to multiorgan failure.

Renal Amyloidosis: Epidemiological, Clinical, and Laboratory Profile in Adults from One Nephrology Center

“…As described previously, AA amyloidosis has also been associated with Behcet's disease, inflammatory bowel disease, chronic inflammatory diseases, and malignancies [ 7 , 14 , 22 , 29 , 30 ].…”

Systemic AA Amyloidosis Secondary to Metastatic Renal Cell Carcinoma in a Hemodialysis Patient with Intractable Diarrhea

“…Some case reports about systemic AA amyloidosis with abdominal symptoms secondary to primary or metastatic renal cell carcinoma were reported [4,[8][9][10][11][12]. These symptoms were reported to improve after nephrectomy or chemotherapy in almost all cases.…”

Concurrent Presentation of Light Chain Amyloidosis and Renal Cell Carcinoma in an Elderly Male : A Case Report and Review of the Literature

“…7 RCC accounts for 3% of adult malignancies and is often referred to in the medical literature as "the great masquerader. 9,10 This is because RCC is frequently associated with multi-systemic paraneoplastic syndromes (10-40% of patients). The triad of (hematuria, flank pain, and palpable abdominal mass) is discovered in only 10-15% of patients at presentation and, when present, usually signifies advanced disease.…”

“…9 IL-6 produced by RCC is a potential para-neoplastic inflammatory link to MM. [9][10][11] In a case series of 119 patients with metastatic RCC, IL-6 levels were significantly higher in those with paraneoplastic fever and weight loss. High levels of IL-6 in RCC patients are correlated with worse survival and drug resistance.…”

“…Furthermore, resection of the malignancy may ameliorate or even potentially reverse the biochemical processes which involve IL-6 that drive the amyloidosis. [9][10][11] CyBorD has been the standard of care for managing AL amyloidosis, which has shown high rates of hematologic response and prolonged progression-free survival when used in newly diagnosed patients and at relapse. 12,13 Novel plasma cell-directed therapies for AL amyloidosis are emerging due to observed success in MM, which has been show to achieve hematologic control and suppress pathologic light chain precursor protein production.…”