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Diffuse Mesenteric and Omental Infiltration by Amyloidosis with Omental Calcification Mimicking Abdominal Carcinomatosis
Clinical Radiology (2001) 00: 000±000 doi:10.1053/crad.2000.0654, available online at http://www.idealibrary.com on
Case Report Diuse Mesenteric and Omental In®ltration by Amyloidosis with Omental Calci®cation Mimicking Abdominal Carcinomatosis M A R C CO U M B A R A S *, JO C E LY N E C H O PI E R *, M A R I E - A N G E M A S S I A N I {, M A R T I N E A N TO I N E{, F R A N K B O U D G H EÁ N E *, M A R C B A Z OT * Departments of *Radiology, {Pathology and {Chest Department, HoÃpital Tenon, Paris, France
Amyloidosis is de®ned as extracellular deposition of ®brous protein in various anatomical structures. The peritoneum is rarely involved. We detected extensive and partly calci®ed amyloidosis of the omentum with diuse involvement of the retroperitoneum on computed tomography (CT). Despite the variety of radiological manifestations of amyloidosis described in the literature, to our knowledge this is the ®rst report of such omental calci®ed involvement.
omentum and the peritoneal surfaces were diusely thickened. Surgical ®ndings were suggestive of diuse intra-abdominal carcinomatosis, and multiple biopsies were taken of the mesentery and omentum. Because
CASE REPORT An 80-year-old woman was referred to our institution with weight loss, renal failure and proteinuria. She reported the recent development of a rectovaginal ®stula. Physical examination showed left jugular and supraclavicular lymphadenopathy. The serum monoglobulin A level was signi®cantly elevated. Urinary protein electrophoresis detected a monoclonal gammopathy, consisting of k light chains on immunoelectrophoresis. A bone marrow aspirate revealed 20% plasmocytosis compatible with stage I light chain myeloma. The patient had undergone hysterectomy and anexectomy for a haemorrhagic ®bromyoma when she was 50 years old. Abdominal ultrasound was performed to detect other disease sites. Diuse thickening and increased echogenicity of the mesentery was found. This soft tissue in®ltration appeared to engulf the pancreas. The omentum was thickened and homogenously hyperechoic. No pelvic abnormalities were found. Abdominal CT was performed with oral contrast medium for bowel opaci®cation without contrast injection because of the poor renal function. Increased soft tissue in®ltration of the retroperitoneal, mesenteric and peritoneal fat was observed. The omentum was clearly involved by this in®ltrative process, which was also encasing the pancreas and para-aortic structures (Fig. 1). The retroperitoneal and mesenteric lymph nodes were markedly enlarged. Calci®cation in the lymph nodes had an amorphous or annular pattern (Fig. 2). In addition to the diuse involvement of the mesentery and peritoneum, a widespread mantle of speckled calci®cation within the omentum was observed on CT (Fig. 3). Slight ascites and thickening of the bowel wall were present. The dierential diagnoses included: calci®ed peritoneal carcinomatosis, lymphoma or other desmoplastic neoplasms. At exploratory laparotomy, the small and large bowel mesenteries, the Author for correspondence and guarantor of study: Dr Jocelyne Chopier, Service de Radiologie, HoÃpital Tenon, 4 rue de Chine, 75020 Paris, France. Fax: 3315 6016402; E-mail: [email protected] 0009-9260/01/000000+00 $35.00/0
Fig. 1 ± Axial CT without intravenous contrast medium, at the level of the kidneys. Diuse in®ltration leading to encasement of the pancreas and para-aortic structures. Note the enlargement of calci®ed mesenteric lymph nodes (arrows).
Fig. 2 ± Abdominal CT demonstrating amorphous calci®cations in enlarged lymph nodes near the root of the mesentery (arrows). # 2001 The Royal College of Radiologists
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CLINICAL RADIOLOGY
Fig. 3 ± Abdominal CT showing a widespread mantle of speckled calci®cations of the omentum (arrows).
Fig. 4 ± Photomicrograph showing amyloid deposits (A) in the peritoneal ®broadipose tissue, associated with calci®cations (C) and lymphoid in®ltration (L). Hematoxylin±eosin±saron staining, magni®cation 100. of the rectovaginal ®stula, colostomy was also performed. Histological examination revealed characteristic depositions of amyloid. The diagnosis was con®rmed by Congo red staining viewed under polarized light (Fig. 4). Aggregations of plasma cells, calci®cation and foreignbody giant cells were associated with the amyloidosis.
serum amyloid A protein (SAA), an acute-phase protein produced in response to chronic in¯ammatory diseases such as rheumatoid arthritis, familial Mediterranean fever and chronic infection. The familial amyloidoses constitute a group of autosomal dominant diseases. The most common form is caused by mutant transthyretin (ATTR). This patient had amyloidosis AL associated with monoclonal gammopathy. The organs most commonly involved are the kidneys and the heart. Diuse mesenteric and retroperitoneal fat-in®ltration amyloidosis is uncommon [3±6]. In retroperitoneal and mesenteric amyloidosis the protein deposits usually show two distinct patterns on CT [5], namely nodal [3,6] or diuse [4,5]. The nodal form is due to enlargement of retroperitoneal lymph nodes. In our patient the in®ltration had a mixed nodular and diuse pattern. Amorphous or irregular calci®cations are occasionally identi®ed within amyloid deposits, in tracheobronchopathia osteoplastica, and in nodular pulmonary amyloidopsis [7]. Calci®ed retroperitoneal lymphadenopathies have previously been reported [6]. Calci®cations have also been described in primary amyloidosis in subcutaneous tissue [8], the kidneys [9] and the urethra [10]. The discovery of such calci®cation on CT is highly suggestive of amyloidosis [7]. The dierential diagnosis of diuse retroperitoneal, mesenteric and peritoneal soft tissue in®ltration includes primary or metastatic neoplasms that may provoke a desmoplastic reaction. The extensive calci®cations associated with soft tissue in®ltration restrict the dierential diagnosis. Calci®ed metastasis along peritoneal surfaces in frequent in ovarian carcinoma, and serous cystadenocarcinomas contain histologic calci®cations in approximately 30% of cases [11]. Peritoneal mesothelioma may present on CT with peritoneal nodules or masses with ascites [12]. Guest et al. showed that extensive omental and peritoneal in®ltration was possible [12] and diuse omental calci®cations were noted in two previous studies [12,13], possibly as a result of tumour necrosis during treatment [12]. Amyloid deposition simulates both in¯ammation and neoplastic conditions. The case we describe shows that nonneoplastic disease is another possible aetiology of extensive omental calci®cation. REFERENCES
DISCUSSION
Amyloidosis is a heterogeneous group of conditions caused by extracellular deposition of an insoluble ®brillar protein in organs and tissues. The modern classi®cation of amyloidosis is based on the nature of the precursor plasma proteins that form ®bril deposits [1]. This classi®cation mainly describes three common forms of amyloidosis, termed primary (AL), familial (ATTR) and secondary amyloid protein A (SAA). These forms dier entirely in their pathogenesis [2]. Amyloidoses AL are associated with monoclonal immunoglobulin light chains and are thus related to multiple myeloma. Amyloidoses AA (secondary amyloidoses) are the result of systemic accumulation of
1 Falk RH, Commenzo RL, Skinner M. The systemic amyloidoses. N Engl J Med 1997;337:898±909. 2 WHO-IUS Nomenclature Sub Committee. Nomenclature of amyloid and amyloidosis. Bull World Health Org 1993;71:105±112. 3 Takebayashi S, Ono Y, Sakai F, Tamura S, Unuyama S. Computed tomography of amyloidosis involving retroperitoneal lymph nodes mimicking lymphoma. J Comput Assist Tomogr 1984;8:1025±1027. 4 Glynn TP, Kreipke DL, Irons JM. Amyloidosis: diuse involvement of the retroperitoneum. Radiology 1989;170:726. 5 Posner RP, Ffrad SA, Saks AM, Leiman G. Diuse retroperitoneal amyloidosis: further radiological observations. Br J Radiol 1991;64: 469±471. 6 Borge MA, Parker LA, Mauro MA. Amyloidosis: CT, appearance of calci®ed, enlarged periaortic lymph nodes. J Comput Assist Tomogr 1991;15:855±857. 7 Urban BA, Fishman EK, Goldman SM, et al.. CT evaluation of amyloidosis: spectrum of disease. Radiographics 1993;10: 1295±1308.
CASE REPORT
8 Bottomley JP, Bradley J, Withehouse GH. Waldenstrom's macroglobulinaemia and amyloidosis with sub cutaneous calci®cation and lymphographic appearances. Br J Radiol 1974;47: 232±235. 9 Ekelund L. Radiologic ®ndings in renal amyloidosis. Am J Roentgenol 1977;129:851±853. 10 Noone TC, Clark RL. Primary isolated urethral amyloidosis. Abdom Imaging 1997;22:448±449.
3
11 Mitchell DG, Hill MC, Hill S, Zaloudek C. Serous carcinoma of the ovary: CT identi®cation of metastatic calci®ed implants. Radiology 1986;158:649±652. 12 Guest PJ, Reznek RH, Selleslag D, Geraghty R, Slevin M. Peritoneal mesothelioma: the role of computed tomography in diagnosis and follow-up. Clin Radiol 1992;45:79±84. 13 Stambaugh JE, Burrows S, Jacoby J, Shivers H. Peritoneal mesothelioma associated with diuse abdominal ossi®cation and unusual presentation. J Med Soc NJ 1977;74:689±693.
Case Report Diuse Mesenteric and Omental In®ltration by Amyloidosis with Omental Calci®cation Mimicking Abdominal Carcinomatosis M A R C CO U M B A R A S *, JO C E LY N E C H O PI E R *, M A R I E - A N G E M A S S I A N I {, M A R T I N E A N TO I N E{, F R A N K B O U D G H EÁ N E *, M A R C B A Z OT * Departments of *Radiology, {Pathology and {Chest Department, HoÃpital Tenon, Paris, France
Amyloidosis is de®ned as extracellular deposition of ®brous protein in various anatomical structures. The peritoneum is rarely involved. We detected extensive and partly calci®ed amyloidosis of the omentum with diuse involvement of the retroperitoneum on computed tomography (CT). Despite the variety of radiological manifestations of amyloidosis described in the literature, to our knowledge this is the ®rst report of such omental calci®ed involvement.
omentum and the peritoneal surfaces were diusely thickened. Surgical ®ndings were suggestive of diuse intra-abdominal carcinomatosis, and multiple biopsies were taken of the mesentery and omentum. Because
CASE REPORT An 80-year-old woman was referred to our institution with weight loss, renal failure and proteinuria. She reported the recent development of a rectovaginal ®stula. Physical examination showed left jugular and supraclavicular lymphadenopathy. The serum monoglobulin A level was signi®cantly elevated. Urinary protein electrophoresis detected a monoclonal gammopathy, consisting of k light chains on immunoelectrophoresis. A bone marrow aspirate revealed 20% plasmocytosis compatible with stage I light chain myeloma. The patient had undergone hysterectomy and anexectomy for a haemorrhagic ®bromyoma when she was 50 years old. Abdominal ultrasound was performed to detect other disease sites. Diuse thickening and increased echogenicity of the mesentery was found. This soft tissue in®ltration appeared to engulf the pancreas. The omentum was thickened and homogenously hyperechoic. No pelvic abnormalities were found. Abdominal CT was performed with oral contrast medium for bowel opaci®cation without contrast injection because of the poor renal function. Increased soft tissue in®ltration of the retroperitoneal, mesenteric and peritoneal fat was observed. The omentum was clearly involved by this in®ltrative process, which was also encasing the pancreas and para-aortic structures (Fig. 1). The retroperitoneal and mesenteric lymph nodes were markedly enlarged. Calci®cation in the lymph nodes had an amorphous or annular pattern (Fig. 2). In addition to the diuse involvement of the mesentery and peritoneum, a widespread mantle of speckled calci®cation within the omentum was observed on CT (Fig. 3). Slight ascites and thickening of the bowel wall were present. The dierential diagnoses included: calci®ed peritoneal carcinomatosis, lymphoma or other desmoplastic neoplasms. At exploratory laparotomy, the small and large bowel mesenteries, the Author for correspondence and guarantor of study: Dr Jocelyne Chopier, Service de Radiologie, HoÃpital Tenon, 4 rue de Chine, 75020 Paris, France. Fax: 3315 6016402; E-mail: [email protected] 0009-9260/01/000000+00 $35.00/0
Fig. 1 ± Axial CT without intravenous contrast medium, at the level of the kidneys. Diuse in®ltration leading to encasement of the pancreas and para-aortic structures. Note the enlargement of calci®ed mesenteric lymph nodes (arrows).
Fig. 2 ± Abdominal CT demonstrating amorphous calci®cations in enlarged lymph nodes near the root of the mesentery (arrows). # 2001 The Royal College of Radiologists
2
CLINICAL RADIOLOGY
Fig. 3 ± Abdominal CT showing a widespread mantle of speckled calci®cations of the omentum (arrows).
Fig. 4 ± Photomicrograph showing amyloid deposits (A) in the peritoneal ®broadipose tissue, associated with calci®cations (C) and lymphoid in®ltration (L). Hematoxylin±eosin±saron staining, magni®cation 100. of the rectovaginal ®stula, colostomy was also performed. Histological examination revealed characteristic depositions of amyloid. The diagnosis was con®rmed by Congo red staining viewed under polarized light (Fig. 4). Aggregations of plasma cells, calci®cation and foreignbody giant cells were associated with the amyloidosis.
serum amyloid A protein (SAA), an acute-phase protein produced in response to chronic in¯ammatory diseases such as rheumatoid arthritis, familial Mediterranean fever and chronic infection. The familial amyloidoses constitute a group of autosomal dominant diseases. The most common form is caused by mutant transthyretin (ATTR). This patient had amyloidosis AL associated with monoclonal gammopathy. The organs most commonly involved are the kidneys and the heart. Diuse mesenteric and retroperitoneal fat-in®ltration amyloidosis is uncommon [3±6]. In retroperitoneal and mesenteric amyloidosis the protein deposits usually show two distinct patterns on CT [5], namely nodal [3,6] or diuse [4,5]. The nodal form is due to enlargement of retroperitoneal lymph nodes. In our patient the in®ltration had a mixed nodular and diuse pattern. Amorphous or irregular calci®cations are occasionally identi®ed within amyloid deposits, in tracheobronchopathia osteoplastica, and in nodular pulmonary amyloidopsis [7]. Calci®ed retroperitoneal lymphadenopathies have previously been reported [6]. Calci®cations have also been described in primary amyloidosis in subcutaneous tissue [8], the kidneys [9] and the urethra [10]. The discovery of such calci®cation on CT is highly suggestive of amyloidosis [7]. The dierential diagnosis of diuse retroperitoneal, mesenteric and peritoneal soft tissue in®ltration includes primary or metastatic neoplasms that may provoke a desmoplastic reaction. The extensive calci®cations associated with soft tissue in®ltration restrict the dierential diagnosis. Calci®ed metastasis along peritoneal surfaces in frequent in ovarian carcinoma, and serous cystadenocarcinomas contain histologic calci®cations in approximately 30% of cases [11]. Peritoneal mesothelioma may present on CT with peritoneal nodules or masses with ascites [12]. Guest et al. showed that extensive omental and peritoneal in®ltration was possible [12] and diuse omental calci®cations were noted in two previous studies [12,13], possibly as a result of tumour necrosis during treatment [12]. Amyloid deposition simulates both in¯ammation and neoplastic conditions. The case we describe shows that nonneoplastic disease is another possible aetiology of extensive omental calci®cation. REFERENCES
DISCUSSION
Amyloidosis is a heterogeneous group of conditions caused by extracellular deposition of an insoluble ®brillar protein in organs and tissues. The modern classi®cation of amyloidosis is based on the nature of the precursor plasma proteins that form ®bril deposits [1]. This classi®cation mainly describes three common forms of amyloidosis, termed primary (AL), familial (ATTR) and secondary amyloid protein A (SAA). These forms dier entirely in their pathogenesis [2]. Amyloidoses AL are associated with monoclonal immunoglobulin light chains and are thus related to multiple myeloma. Amyloidoses AA (secondary amyloidoses) are the result of systemic accumulation of
1 Falk RH, Commenzo RL, Skinner M. The systemic amyloidoses. N Engl J Med 1997;337:898±909. 2 WHO-IUS Nomenclature Sub Committee. Nomenclature of amyloid and amyloidosis. Bull World Health Org 1993;71:105±112. 3 Takebayashi S, Ono Y, Sakai F, Tamura S, Unuyama S. Computed tomography of amyloidosis involving retroperitoneal lymph nodes mimicking lymphoma. J Comput Assist Tomogr 1984;8:1025±1027. 4 Glynn TP, Kreipke DL, Irons JM. Amyloidosis: diuse involvement of the retroperitoneum. Radiology 1989;170:726. 5 Posner RP, Ffrad SA, Saks AM, Leiman G. Diuse retroperitoneal amyloidosis: further radiological observations. Br J Radiol 1991;64: 469±471. 6 Borge MA, Parker LA, Mauro MA. Amyloidosis: CT, appearance of calci®ed, enlarged periaortic lymph nodes. J Comput Assist Tomogr 1991;15:855±857. 7 Urban BA, Fishman EK, Goldman SM, et al.. CT evaluation of amyloidosis: spectrum of disease. Radiographics 1993;10: 1295±1308.
CASE REPORT
8 Bottomley JP, Bradley J, Withehouse GH. Waldenstrom's macroglobulinaemia and amyloidosis with sub cutaneous calci®cation and lymphographic appearances. Br J Radiol 1974;47: 232±235. 9 Ekelund L. Radiologic ®ndings in renal amyloidosis. Am J Roentgenol 1977;129:851±853. 10 Noone TC, Clark RL. Primary isolated urethral amyloidosis. Abdom Imaging 1997;22:448±449.
3
11 Mitchell DG, Hill MC, Hill S, Zaloudek C. Serous carcinoma of the ovary: CT identi®cation of metastatic calci®ed implants. Radiology 1986;158:649±652. 12 Guest PJ, Reznek RH, Selleslag D, Geraghty R, Slevin M. Peritoneal mesothelioma: the role of computed tomography in diagnosis and follow-up. Clin Radiol 1992;45:79±84. 13 Stambaugh JE, Burrows S, Jacoby J, Shivers H. Peritoneal mesothelioma associated with diuse abdominal ossi®cation and unusual presentation. J Med Soc NJ 1977;74:689±693.