British Journal of Radiology (2008) 81, e1-e3
© 2008 British Institute of Radiology
doi: 10.1259/bjr/13509947
Amyloidosis of the mesentery and small intestine presenting as a mesenteric haematoma
M S Kim, MD1,
J-A Ryu1,
C S Park1,
E J Lee1,
N H Park1,
H E Oh3 and
J Jeong2
Departments of 1 Diagnostic Radiology, 2 Surgery and 3 Pathology, Myongji Hospital, Kwandong University College of Medicine, 697-24 Hwajung-dong, Dukyang-ku, Koyang, Kyunggi, 412-270, Korea
Correspondence: Professor Jinho Jeong, Surgery Department, Myongji Hospital, Kwandong University, 697-24 Hwajung-dong, Dukyang-ku, Koyang, Kyunggi 412-270, Republic of Korea. E-mail: jeongjinho{at}kwandong.ac.kr; nk1631{at}freechal.com
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Abstract
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Amyloidosis involving the mesentery is very rare, and its radiological appearance has been poorly documented. To our knowledge, this is the first case of amyloidosis involving the mesentery being presented as a mesenteric haematoma.
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Introduction
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Amyloidosis is a pathological process encompassing a spectrum of diseases that results from the extracellular deposition of fibrillar amyloid proteins. Although it is usually seen in systemic form, 10–20% of cases can be localized [1]. Systemic amyloidosis is subclassified into an idiopathic primary form and a secondary or reactive form. Patients with primary amyloidosis have no underlying condition or disease. The progressive deposition of amyloid compresses and replaces normal tissue, which leads to organ dysfunction and a wide variety of clinical presentations. Gastrointestinal tract amyloidosis occurs in 98% of patients with systemic amyloidosis, with the small intestine being the most common site [2, 3]. Amyloidosis involving the mesentery is very rare, and the radiological appearance of this condition has been poorly documented [4–6]. We report a case of amyloidosis involving the mesentery and small intestine that presented as a mesenteric haematoma.
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Case report
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A 47-year-old man was admitted to hospital with acute onset of sharp, constant left-upper abdominal pain accompanied by nausea and vomiting. Until these symptoms began, he had an unremarkable medical history except for a history of meningitis 14 years earlier, and took no medication. His family history was non-contributory. On admission, he looked pale and was in acute distress. He was afebrile with stable vital signs. On physical examination, bowel sounds were reduced, and direct tenderness in the left-upper abdomen with a palpable mass was observed. Laboratory data collected on admission included a white blood cell count of 13 100 µl–1 (normal: 4 000–10 000 µl–1) and a Hb level of 13 g dl–1 (normal: 13–17 g dl–1). The following day, the Hb level fell to 12.1 g dl–1. The platelet count, coagulation studies and other routine biochemical tests were within normal ranges.
An abdominal ultrasound was performed on admission, which showed a multilocular cystic mass with mixed echogenicity at the centre of abdomen (Figure 1a
). Subsequently, CT of the abdomen and pelvis was performed. Non-contrast CT revealed aggregates of multiple nodular masses that showed predominantly high attenuation, indicating recent bleeding along the root of the small bowel mesentery (Figure 1b). Contrast-enhanced CT revealed central enhancing dots in an area of low density, increased soft tissue infiltration, and thickening of the jejunal wall with a small amount of ascites (Figure 1c). The differential diagnoses included mesenteric haematoma with small bowel ischaemia, lymphoma or other primary or metastatic neoplasms with a desmoplastic reaction. The next day, abdominal angiography was performed to evaluate the active bleeding; however, no evidence of active bleeding or tumour staining on superior mesenteric arteriography was detected. Follow-up CT obtained 20 days after the initial scan demonstrated that the internal haemorrhage in the mass had liquefied and overall tumour size had decreased (Figure 1d).
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Figure 1. 47-year-old man with acute onset of left-upper abdominal pain. (a) Longitudinal ultrasonogram shows a multinodular mass with mixed echogenicity (arrows) at the centre of abdomen. (b) Non-contrast CT scan reveals nodular mass with high attenuation, indicating acute haemorrhage (arrows). (c) Coronal reformatted contrast-enhanced CT scan shows a multinodular mass (arrows), including an area of central enhancement (arrowheads) along the root of the small bowel mesentery. Note the increased mesenteric infiltrations and thickening of the jejunal wall (*). (d) Coronal reformatted follow-up CT scan obtained 20 days after the initial CT scan shows that internal haemorrhage in the mass has liquefied and overall tumour size (arrows) has decreased. (e) Photomicrograph from the specimen of mesenteric mass shows that the yellow-green birefringence of the amyloid deposits have strong affinity for the Congo red stain (arrows). Note the amyloid deposits in the wall of blood vessels (B).
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The patient underwent exploratory laparotomy to rule out an underlying intra-abdominal neoplasm. The mesenteric mass could not be dissected from the mesenteric vessels and small bowel loops because of severe adhesions. The proximal third of the jejunum revealed ischaemic changes. Consequently, a 120 cm segment of jejunum, 30 cm distal to the ligament of Treitz, and the mesenteric mass were resected. Gross pathological examination revealed that the mesenteric mass consisted of brownish-yellow soft tissue and pus-like fluid accumulation. It was slightly firm, with a thick wall. No bacterial growth was detected by fluid culture. The resected jejunum showed slight thickening of the bowel wall; its mucosa was friable with diffuse haemorrhagic necrosis.
Microscopically, extensive diffuse amyloid deposits were found, especially in the blood vessels that included haemorrhage in the mesentery (Figure 1e
). Amyloid deposits were also noted in the small intestine with ischaemic infarction. The amyloid nature of the deposits was confirmed using crystal violet and Congo red stains.
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Discussion
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Amyloidosis is a rare systemic disease, which affects the abdominal viscera in a variety of ways. Although the cause of amyloidosis is unknown, some postulate that it represents a derangement of immunoregulation after a protracted antigenic challenge [7]. Amyloid is deposited in a twisted β-pleated sheet configuration, which results in its characteristic appearance when stained with Congo red. This configuration also accounts for the relative resistance of amyloid to normal proteolytic digestion, which promotes its accumulation and eventually compromises organ function.
According to the World Health Organization's classification, which is based on the structure of the variable fibrillar protein constituent, amyloid light chain is found in primary amyloidosis, whereas amyloid A protein is found mainly in secondary amyloidosis [8]. Secondary amyloidosis is seen in 10–15% of patients with multiple myeloma, and approximately 20–25% of patients with rheumatoid arthritis. Other causes include bronchiectasis, familial Mediterranean fever and chronic infections such as tuberculosis, syphilis, pyelonephritis, osteomyelitis and parasitic infection. We assume that this case is primary amyloidosis, although immunohistochemistry was not performed, according to the clinical setting.
Amyloid protein deposition can be seen in a variety of organs, although it is more frequent in the gastrointestinal tract, kidney and heart. In amyloidosis of the gastrointestinal tract, amyloid deposition is seen mainly around the arterioles and in muscles, causing ischaemia and muscle atrophy and eventually leading to mucosal erosion, ulceration and dysmotility. The small bowel is the most common site of involvement in the gastrointestinal tract [9–11]. CT findings include wall thickening, which can be either focal or diffuse and may be impossible to differentiate from ischaemic changes, and dilatation, depending in part on the degree of hypomotility. Amyloidosis involving the mesentery is uncommon. Two distinct patterns on CT have been reported: nodular and diffuse forms [4, 12–17]. In our case, multiple nodular mesenteric masses predominated, accompanied by mesenteric infiltrates and thickening of the bowel wall by the amyloid deposits. Although we could not explain the exact pathogenesis of the internal haemorrhage within the mass, two possible explanations do exist. One is that amyloid angiopathy, resulting from deposits of amyloid protein in the small vessel walls, might lead to haemorrhage. The microscopic findings support this hypothesis. The other hypothesis is that the bulky mass itself compresses the mesenteric vessels, leading to ischaemia and haemorrhage. Amorphous or irregular calcifications can occasionally be identified within amyloid deposits involving the mesentery and omentum [14–16]. The differential diagnosis of a multinodular mesenteric mass with mesenteric infiltration includes lymphoma and primary or metastatic neoplasms that might provoke a desmoplastic reaction, e.g. carcinoid tumour or metastatic lymphadenopathy.
On CT, lymphoma appears as either multiple, rounded, mildly enhancing, homogeneous masses that often encase the mesenteric vessels, or a large lobulated heterogeneous mass with low-attenuation areas of necrosis that often encase the mesenteric vessels [18, 19]. However, internal haemorrhage within a mass is rare. The CT findings of carcinoid tumour involve an enhancing soft-tissue mass with linear bands radiating in the mesenteric fat, resulting from the intense fibrotic proliferation and desmoplastic reaction in the mesenteric fat and adjacent mesenteric vessels [20]. The thickening of adjacent small bowel loops can result from tumour infiltration or ischaemia caused by the sclerosis of mesenteric vessels. Clinically, a patient with a carcinoid tumour may present with carcinoid syndrome caused by the release of vasoactive substances such as serotonin and 5-hydroxytryptophan. Metastatic disease, presenting as enlarged mesenteric lymph nodes, should be included in the differential diagnosis. However, the degree of nodal enlargement is less pronounced, and the involved nodes are comparatively more localized, in metastatic disease than in mesenteric amyloidosis. Although amyloidosis involving the mesentery is rare, in the right clinical setting, we believe that amyloidosis should be considered in the differential diagnosis of a multinodular mesenteric mass with or without intratumoral haemorrhage.
Received for publication July 25, 2006.
Revision received September 19, 2006.
Accepted for publication October 30, 2006.
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Abstract
Introduction
