A Case of Abdominal Aortic Retroperitoneal and Mesenteric Amyloid Light Chain Amyloidoma

Abstract

We report the case of a Japanese woman with amyloid light chain (AL) amyloidoma in the abdominal aortic retroperitoneum and mesentery. Irregular soft tissue mass lesions with calcification in the abdominal aortic retroperitoneum and mesentery were initially detected by computed tomography at another hospital. The lesions gradually compressed the duodenum, causing symptoms of bowel obstruction. The patient was clinically diagnosed with retroperitoneal fibrosis, and prednisolone was administered at a dose of 40 mg/day. However, the lesions did not change in size and her symptoms continued. She was transferred to our hospital and underwent mesenteric biopsy for histopathology using abdominal laparotomy. The histopathological and immunohistological findings of the mesenteric specimen demonstrated lambda light chain deposition. Accordingly, the patient was finally diagnosed with AL amyloidoma with no evidence of systemic amyloidosis. After laparotomy, her general condition worsened because of complications of pneumonia and deep vein thrombosis. She died suddenly from acute myocardial infarction. We have concluded that abdominal aortic retroperitoneal and mesenteric AL amyloidoma may have very poor prognoses in accordance with previous reports. In addition, the size and location of AL amyloidoma directly influence the prognosis. We suggest that early histopathology is important for improving prognosis.

A case of primary retroperitoneal amyloidoma resected laparoscopically

“…Nine cases of retroperitoneal amyloidosis have been previously reported. 3 , 4 The data available have shown that these cases were secondary amyloidosis associated with malignancies such as multiple myeloma, renal cancer, and plasmacytoma. Therefore, many cases were treated by chemotherapy or radiation therapy and were diagnosed by biopsy specimens.…”

Mesenteric Amyloidosis: Radiologic Imaging with Pathologic Correlation

“…[3,[9][10][11] Amyloid deposition along the mesentery is very rare. [12] Similarly to pulmonary amyloidosis, variable forms of the gastrointestinal amyloidosis can be recognized. [13] On CT amyloidosis has been radiologically described as softtissue attenuation lesions isodense to the skeletal muscle that may contain calcifications or focal areas of necrosis.…”

“…[26,29] In our case, amyloid deposits were predominately extending along the perivascular sheaths of the mesenteric root with a few areas of amyloid deposits extending to the very periphery where they manifested as serosal deposits along the bowel loops [ Figure 1a In the literature, there have been only eight cases of mesenteric amyloidoma reported. [8,12,30,31] All eight cases were associated with AL amyloidosis. [12] e mean age was 66 (with a range of 48-29), with equal gender occurrence.…”

“…[8,12,30,31] All eight cases were associated with AL amyloidosis. [12] e mean age was 66 (with a range of 48-29), with equal gender occurrence. ree of the patients had an underlying lymphoproliferative disorder, two had multiple myeloma, and two patients had plasmacytoma.…”