Imaging findings of Castleman disease of the abdomen and pelvis

BACKGROUND: The purpose of this study was to analyze the characteristic features of Castleman disease in the abdomen and pelvis as suggested by imaging findings in order to deepen the recognition and understanding of this rare disease. METHODS: A group of ten patients with pathologically proven Castleman disease in the abdomen (n = 9) and pelvis (n = 1) were included in this study. Patients were 18 approximately 56-year-old (mean = 40); seven of them were men and three were women. Imaging findings (CT&MRI, n = 4; only CT, n = 4; only MRI, n = 2) were retrospectively reviewed and correlated with clinical and pathologic findings. RESULTS: The lesions were divided into those with localized Castleman (n = 9) and disseminated Castleman (n = 1). The pathologic subtype of all nine cases of localized disease was hyaline vascular with six patients showing a solitary mass and three having a single dominant mass surrounded by small satellite nodules. On nonenhanced CT images, the lesions were manifested as homogeneous masses of soft tissue attenuation, which was isoattenuated relative to normal muscle. On MRI, the lesions were isointense or slightly hypointense compared with that of normal muscle on T1-weighted images and hyperintense on T2-weighted images. After intravenous injection of contrast media, most of the masses (7/9) showed marked enhancement and slow washout with the degree of enhancement approaching that of the large arteries. And in the interior of four cases of larger masses (>5 cm) was observed fissured and radial patterns in both low-density area on CT and low-signal area on MRI. These patterns were pathologically proved to be fibrous. The pathological subtype of a sole disseminated case was plasma-cell type, where imaging findings showed a lining of well defined, sharply enhanced soft-tissue nodules in retroperitoneal zone. CONCLUSION: Imaging findings of Castleman disease in the abdomen and pelvis are closely related to pathological type diagnosed. The characteristic features of localized and hyaline vascular type of Castleman disease include a solitary mass or a dominant mass surrounded with small satellite nodules, and high enhancement and slow washout with the degree of enhancement approaches that of large arteries. The presence of central areas of fibrosis of the larger tumors is one of the characteristic features of this disease.     

Hyaline vascular-type Castleman disease: a rare cause of a hypervascular retroperitoneal mass

We present the cross-sectional imaging and angiographic findings of hyaline vascular-type Castleman disease located in the retroperitoneum. The diagnosis was made postoperatively. This entity can simulate a malignant neoplasm. The histologic subtypes and presentations of Castleman disease and the differential diagnosis of retroperitoneal masses are discussed. Received: 12 August 1999/Accepted: 8 September 1999     

Portal tumor thrombus due to gastrointestinal cancer

Methods: We studied the clinical data of seven patients with portal tumor thrombus (PTT) due to gastrointestinal (GI) cancer to determine the radiologic patterns and clinical implications of this rare complication. Results: (a) PTT was located along the entire splenic vein in three cases, at the splenomesenteric confluence in one case, and in the superior mesenteric vein in one case. Intrahepatic PTT occurred in two of four cases with liver metastasis. (b) One cirrhotic case was complicated by the occurrence of colon cancer associated with PTT in the splenic vein; the esophageal varices became rapidly enlarged and poorly controlled, and the patient died due to repeated variceal rupture. (c) In all patients, abdominal sonography (US) detected PTT and color Doppler sonography confirmed the US findings. Conclusions: The splenic vein should be meticulously observed by color Doppler sonography to check for PTT in patients with GI cancer to improve patient care. Received: 29 December 1998/Accepted: 24 February 1999     

Nodular regenerative hyperplasia of the liver in Budd–Chiari syndrome: CT and MR features

We report the imaging findings of spiral computed tomography (CT), magnetic resonance (MR) imaging, and MR angiography in a patient with nodular regenerative hyperplasia of the liver associated with Budd–Chiari syndrome. Spiral CT showed multiple enhancing nodules during the hepatic arterial and portal venous phases. MR images showed multiple hyperintense nodules on T1-weighted images and hypointense or isointense nodules on T2-weighted images. MR angiography showed thrombotic occlusion of three hepatic veins, suggesting Budd–Chiari syndrome. Received: 25 June 1999/Revision accepted: 22 September 1999     

Evaluation of Crohn disease activity with magnetic resonance imaging

Background: The purpose of this study was to assess the accuracy of magnetic resonance imaging (MRI) in evaluating Crohn disease (CD) activity. The intestinal inflammatory activity is usually present in patients under pharmacologic treatment, despite their clinical remission. Methods: Twenty patients with CD, all under pharmacologic treatment, were prospectively studied by MRI at 1.5 T as a periodic control. Positivity of three acute-phase reactants was considered an index of biologic activity (BA). T2-weighted, T2-weighted fat-suppressed turbo spin-echo, and breath-hold T1-weighted turbo field-echo sequences, before and after gadolinium intravenous injection, were obtained. A negative superparamagnetic contrast agent was orally administered. The following MRI parameters were qualitatively evaluated by three radiologists at the level of the affected bowel and compared with clinical data: wall thickness (WT), wall T2-weighted signal (T2W), wall contrast enhancement (WE), amount of fibrofatty proliferation (FP), and T2-weighted signal of fibrofatty proliferation on fat-suppressed images (T2FP). The κ coefficient of agreement was calculated. The Spearman rank correlation was used for the analysis of clinical and radiologic data. Results: Nineteen of 20 patients were in clinical remission (Crohn Disease Activity Index < 150). On the basis of laboratory tests, nine of 20 patients had biologically active disease. An excellent correlation was found between BA and WE, T2W, and T2FP (0.900, 0.927 and 0.961, respectively; p < 0.0001), and a lower correlation was found between BA and WT and between BA and FP (0.78 and 0.62). Excellent statistical correlation was also found between WE and T2W and between WE and T2FP (0.876 and 0.892). Conclusions: An excellent statistical correlation was found between biologically “active” disease and the following MRI parameters: wall gadolinium enhancement, wall hyperintensity on T2-weighted fat-suppressed images, and hyperintensity of fibrofatty proliferation on T2-weighted fat-suppressed images. Therefore, MRI can be valuable in assessing CD activity. Received: 22 January 1999/Revision accepted: 3 November 1999     

Intrahepatic splenosis: imaging features

We report a patient who presented with asymptomatic focal liver lesions and in whom a diagnosis of intrahepatic splenosis was made. This rare condition mostly occurs in patients who previously underwent splenic trauma or surgery. Magnetic resonance imaging (MRI) characteristics suggesting this diagnosis are described. The lesions were mainly hypointense on T1- and hyperintense on T2-weighted images. After administration of small iron oxide particles (SPIO-Endorem), the lesions remained slightly hyperintense relative to the hypointense liver parenchyma but showed a 50% loss in signal intensity. Knowledge of these MRI characteristics may avoid the use of surgical interventions to arrive at the correct diagnosis of these rare liver lesions. Received: 14 June 1999/Accepted: 14 July 1999     

Spiral CT virtual endoscopy of abdominal arteries: clinical applications

Virtual endoscopy enables the creation of endoluminal views of the aorta and its branches by processing spiral computed tomographic (CT) images, thereby allowing the preoperative and postoperative evaluations of abdominal aortic aneurysms, aneurysms of the splenic, celiac, and common iliac arteries, and renal artery stenoses. Moreover, it is helpful for verifying the position of stents and endoprostheses from within the aortic lumen. This method is a promising addition to spiral CT. Received: 3 May 1999/Accepted: 2 June 1999     

Magnetic resonance imaging of Crohn disease: early recognition of treatment response and relapse

A patient with active Crohn disease was evaluated by MRI at admission, clinical remission, and a new relapse. The MRI-estimated disease extension correlated with surgical findings, whereas ultrasonography underestimated and a small bowel series overestimated the extension. MRI disclosed the disappearance of intestinal edema at the time of clinical remission and, in contrast to ultrasonography, showed an abscess and a fistula, confirmed by surgery, at the new relapse. Received: 30 August 1995/Accepted: 20 September 1995     

Splenic lymphoma: differentiation from splenic cyst with ultrasonography

Background and Methods: Lymphoma can be nearly anechoic and mimic a cyst on ultrasonography (US). To investigate whether this phenomenon occurs at the level of the spleen, we analyzed the US findings of 38 cases of splenic lymphoma and 16 cases of splenic cyst. Results: (1) With regard to shape, echogenicity of the lesion, and mode of posterior echo, there was no difference between splenic lymphomas and splenic cysts. However, the boundaries of the lesions were indistinct in splenic lymphomas and distinct in splenic cysts. (2) Blood flow signals and vascular penetration were seen exclusively in splenic lymphomas. Conclusion: The mode of boundary echo (distinct or indistinct) distinguishes splenic lymphomas from splenic cysts. Color Doppler US increases the diagnostic confidence of US. Received: 25 September 2000/Revision accepted: 27 December 2000     

Crohn disease: CT findings after treatment

Within a period of 5 years, we followed by computed tomography (CT) three patients with Crohn disease who were undergoing treatment. From the spectrum of disease abnormalities, some subsided and others remained. Bowel wall thickening was the most common pretreatment CT finding but was somewhat altered after treatment. Received: 10 October 1995/Accepted after revision: 3 January 1996     

Urinary excretion of orally ingested gastrografin on CT

Renal excretion of orally ingested gastrografin has rarely been reported on computed tomography (CT). We studied the unenhanced scans of 82 patients with bowel disorders or perforation to assess the prevalence of urinary contrast material (CM) in various bowel diseases. We also assessed the clinical significance of this sign. In addition, we reviewed the unenhanced CT scans of 100 randomly selected patients without bowel diseases as a control group. Twenty-nine of the 58 patients with bowel diseases, six of nine with free perforation, and one of 15 with covered perforation had CM in the urinary tract. None of the 100 without bowel disease showed urinary CM. Statistical analysis was done by using the Fisher's exact test. The prevalence of urinary CM was highest in inflammatory bowel disease, radiation enteritis, and free perforation (p < 0.0001). This study shows that the CT finding of orally ingested gastrografin in the urinary tract differentiates patients with bowel disease from those without. Received: 2/13/97/Accepted: 3/19/97     

Splenic calcifications in heterozygote sickle cell patients

A small, fibrotic, and even calcified spleen is the hallmark of homozygote sickle cell disease in adults. Such a condition is very rare in sickle-thalassemia and, to our knowledge, not previously reported in a sickle cell trait. We report two heterozygote sickle cell siblings with splenic calcifications, one sickle cell trait and one sickle-beta+-thalassemia, without any history suggestive of splenic vasooclusive-infarctive crisis.     

Adult Crohn disease: can ileoscopy replace small bowel radiology?

Background: This study aimed to document the radiological features and distribution of small bowel Crohn disease (CD) in adults by using a barium follow-through (BaFT) technique and to determine whether disease would be missed or its distribution underestimated if only colonoscopy with ileoscopy were performed. Methods: The BaFT examinations of 121 adults with proven CD were reviewed retrospectively with respect to the stage and distribution of disease. Colonoscopy with attempted ileoscopy was performed in 37 of these subjects, and the results were compared with radiological findings. Results: A normal villous pattern was visualized in 89 studies (74%). BaFT showed small bowel CD in 71 (59%) of 121 patients studied. The terminal ileum (TI) was the most common site of disease, affecting 62 (87%) of patients with small bowel CD. Forty-six patients (65%) had more proximal small bowel disease, including nine (13%) with a normal TI. BaFT showed early mucosal changes of CD in 52 subjects (73%), which was the sole manifestation in 15 (21%). Ileoscopy was possible in the majority of patients colonoscoped but was not achieved in 14 (38%), nine of whom had CD on BaFT. Of the 23 patients in whom ileoscopy was performed, findings agreed with BaFT assessment of the TI in 22. Conclusion: BaFT adequately demonstrates the stage and extent of small bowel CD. The majority of patients with small bowel CD have disease proximal to the TI, which cannot be diagnosed by ileoscopy. Received: 27 August 1996/Accepted: 16 October 1996     

Unusual liver MR findings of Wilson's disease in an asymptomatic 2-year-old girl

We describe the unusual magnetic resonance (MR) findings of a case of Wilson's disease (WD) in an asymptomatic 2-year-old girl. Preenhanced computed tomography revealed multiple hyperdense areas in the liver. These lesions were hyperintense on T1-weighted and hypointense on T2-weighted MR images, results that might be ascribed to the paramagnetism of copper deposited in liver at a relatively early stage of the disease before severe liver cirrhosis had evolved. Received: 9/10/96/Accepted: 10/16/96     

Mesenteric Castleman's disease: case report

An asymptomatic case of hyaline vascular–type Castleman's disease localized to the mesentery and detected incidentally by ultrasound is presented. Computed tomographic, angiographic, and histologic findings are reported. This type of Castleman's disease predominates in the thorax and is very rare in the mesentery. Received: 5 January 2000/Accepted: 26 January 2000     

Helical CT of the small bowel with an alternative oral contrast material in patients with Crohn disease

Background: We assessed the usefulness of helical computed tomography (CT) with a negative oral contrast material for detecting Crohn disease. Methods: Thirty-eight patients with proven Crohn disease were examined. We administered a large volume of a new negative oral contrast material (Mucofalk suspended in water) and then proceeded with helical CT scanning. This technique is an alternative to CT and conventional enteroclyses that use a nasojejunal tube. Two radiologists interpreted the scans, and patients were interviewed about their tolerance of the procedure. We created multiplanar reformatted images in all cases. Potential of small bowel distention by Mucofalk was evaluated by two observers on a three-point scale, and interobserver agreement was calculated with κ statistics. Results: All patients who underwent enteroclysis stated that CT was the more comfortable method, the taste of the peroral contrast medium was considered good by 52.6% and acceptable by 47.4%. Small bowel distention was excellent in 55% of cases, moderate in 26%, and poor in 19%, with an interoberserver agreement of 78%. CT findings correlated with enteroclysis in 27 patients who underwent both methods. Analysis of CT versus enteroclysis showed a sensitivity of 89% for CT versus 78% for small bowel enteroclysis. Conclusion: Mucofalk CT is a simple, rapid, noninvasive, and accurate method of evaluating extramucosal manifestations of Crohn disease. The tubeless procedure improved patients' comfort and decreased time, cost, and radiation exposure.     

Enteroclysis findings of intestinal Behcet disease: a comparative study with Crohn disease

Background: The aim of this study was threefold; to define the enteroclysis (EC) findings of intestinal involvement in Behcet disease (BD), to compare these findings with those seen in Crohn disease (CD), and to determine the relation between the duration of BD and severity of the EC findings. Methods: From 1997 to 2000, 17 BD and 50 CD cases were examined by EC examination. EC was performed with a 13-F balloon catheter via transnasal entubation. Mucosal and mural changes were evaluated. Statistical analysis was performed with the Mann-Whitney U test to determine the relation between duration of BD and severity of the EC findings. P ≤ 0.05 was considered statistically significant. Results: In 12 (70.58%) of 17 BD cases, EC demonstrated intestinal pathologic findings such as ulcerations, fold thickening, granular pattern, pseudopolyp formation, and bowel wall thickening. No statistical correlation between the severity of EC findings and the duration of BD was found. Conclusion: EC should be the radiologic method to evaluate the intestinal pathology in symptomatic BD patients. The main EC finding in BD was ulceration mostly in the aphthous form. The EC findings in BD, even in longstanding cases, are usually mild when compared with those seen in CD.     

戈谢氏病伴血小板减少一例并文献复习

目的：探讨骨髓细胞形态学检查在戈谢氏病诊断中的价值。方法：采用瑞氏一吉姆萨染色法、糖原染色法对一例戈谢氏病伴血小板减少患者的骨髓片、血片进行化学染色,辅助骨髓基因及染色体检查,结合文献复习,提高对该病的认识。结果：瑞氏一吉姆萨染色发现骨髓片中的戈谢氏细胞,糖原染色该细胞呈阳性反应,确诊为戈谢氏病。结论：骨髓细胞形态学检查有助于戈谢氏病的早期诊断。     

Monitoring of Gaucher patients with a novel chitotriosidase assay

BACKGROUND: Chitotriosidase (CT) is a surrogate plasma marker for Gaucher disease. The enzyme is released by storage cells and is on average thousand fold elevated in serum of Gaucher patients. Plasma CT level is measured with the substrate 4-methylumbelliferyl (4MU)-chitotriose or 4MU-chitobiose. Given the limitations associated with the use of these substrates, a novel substrate, 4MU-deoxychitobiose, has recently been conceived. METHODS: Chitotriosidase activity was measured with all three substrates in serum samples from 91 type 1 Gaucher patients. Glucocerebrosidase and chitotriosidase genotypes were determined as well as disease parameters. RESULTS: Chitotriosidase activity when measured with 4MU-deoxychitobiose gave higher values and was proportional to enzyme concentration over a much larger range as compared to the other two substrates. Patients that were carrier for the common CT mutation showed on average half the activity of those with wild type CT genotype. Plasma CT levels correlated best with combined liver and spleen volume: r=0.53 (p<0.001). CONCLUSIONS: The use of 4MU-deoxychitobiose as substrate renders a substantial improved CT activity assay and may further facilitate accurate laboratory monitoring of Gaucher patients.     

Dysplastic nodules of the liver: imaging findings

Background: To verify characteristic features of hepatic dysplastic nodules at different imaging modalities. Methods: Twenty-eight patients with 37 dysplastic nodules of the liver (0.8–3.0 cm) underwent sonography (28 patients), computed tomography (CT; 24 patients), magnetic resonance (MR; 11 patients), and angiography (12 patients). Each nodule was analyzed for echogenicity, attenuation, signal intensity, and vascularity. Results: Echogenicity of nodules was high in 16 (43%), homogeneous in two (6%), and low in 19 (51%) of 37 nodules. Attenuation of nodules was high in one (7%), homogeneous in four (26%), and low in 10 (67%) of 15 nodules on the arterial-phase CT images; homogeneous in five (33%) and low in 10 (67%) of 15 nodules on the portal-phase CT images; and high in four (17%), homogeneous in six (26%), and low in 13 (57%) of 23 nodules on the delayed-phase CT images. Signal intensity of nodules was high in 15 (94%) and homogeneous in one (6%) of 16 nodules on T1-weighted MR images and was homogeneous in seven (44%) and low in nine (56%) of 16 nodules on T2-weighted MR images. Vascularity of nodules was avascular in 14 (88%) and slightly vascular in two (12%) of 16 nodules. Conclusions: Hepatic dysplastic nodules show diverse imaging characteristics with different imaging techniques; however, common imaging findings of hepatic dysplastic nodules are low echo, low attenuation, and high, low, or homogeneous intensity on T1- and T2-weighted MR, and avascularity. Received: 13 May 1998/Accepted: 1 July 1998