Mural Calcifications Research Articles

Vascular anomaly, lipoma, and polymicrogyria associated with schizencephaly: developmental and diagnostic insights. Illustrative case.

Schizencephaly is an uncommon central nervous system malformation. Intracranial lipomas are also rare, accounting for approximately 0.1% of brain "tumors." They are believed to be derived from a persistent meninx primitiva, a neural crest-derived mesenchyme that develops into the dura and leptomeninges. The authors present a case of heterotopic adipose tissue and a nonshunting arterial vascular malformation arising within a schizencephalic cleft in a 22-year-old male. Imaging showed right frontal gray matter abnormality and an associated suspected arteriovenous malformation with evidence of hemorrhage. Brain magnetic resonance imaging revealed right frontal polymicrogyria lining an open-lip schizencephaly, periventricular heterotopic gray matter, fat within the schizencephalic cleft, and gradient echo hypointensity concerning for prior hemorrhage. Histological assessment demonstrated mature adipose tissue with large-bore, thick-walled, irregular arteries. Mural calcifications and subendothelial cushions suggesting nonlaminar blood flow were observed. There were no arterialized veins or direct transitions from the arteries to veins. Hemosiderin deposition was scant, and hemorrhage was not present. The final diagnosis was consistent with ectopic mature adipose tissue and arteries with meningocerebral cicatrix. This example of a complex maldevelopment of derivatives of the meninx primitiva in association with cortical maldevelopment highlights the unique challenges from both a radiological and histological perspective during diagnostic workup.

An Incidental Pancreatic Simple Mucinous Cyst Reveals an Incidental Well-Differentiated Neuroendocrine Tumor.

A 61-year-old woman with tobacco abuse presented after screening computed tomography scans demonstrated an incidental large pancreatic body cyst with mural calcifications (Figure A). FNA showed macrophages and debris with an elevated CEA level. A mucinous cystic neoplasm was therefore presumed. A distal pancreatectomy and splenectomy specimen showed an 11.2 × 8.0 × 7.8-cm unilocular cyst with yellow viscous fluid, a tan yellow lining, and a 0.3- to 0.7-cm-thick rubbery wall (Figure B). Histologically, the cyst lining was mostly denuded with residual benign simple cuboidal gastric-type epithelium and underlying dense hyalinization and calcification without ovarian-type stroma (Figures C, D). An incidental well-differentiated (WHO grade 1) neuroendocrine tumor (NET) infiltrated the cyst wall diffusely (Figure D). Pancreatic simple mucinous cysts (SMCs) measure greater than 1.0 cm and occur mostly in asymptomatic postmenopausal women. They lack features of intraductal papillary mucinous neoplasms and mucinous cystic neoplasms. SMCs exhibit a rather benign behavior, but their neoplastic potential has been suspected with KRAS mutations demonstrated in 13%–55% of cases. As a result, the original designation of simple nonneoplastic cyst was changed to SMC. NETs may rarely present as cystic lesions resulting from central necrosis of solid tumors. They show a cavity surrounded by neoplastic neuroendocrine cells unlike the SMCs' thick hyalinized wall and gastric-type epithelial monolayer lining seen in the current lesion. Whether the NET is arising from the cyst is uncertain. This concurrence of SMC and NET has not been previously reported.

Retrospective case-control study to predict a potential underlying appendiceal tumor in an acute appendicitis context based on a CT-scoring system

ObjectivesTo assess CT signs to discriminate an appendiceal tumor versus a non-tumoral appendix in an acute appendicitis context. MethodsA 10-year bicentric retrospective case-control study was performed in adults. Patients with a histopathological appendiceal tumor and appendicitis were paired for age and sex with patients with non-tumorous appendicitis (1/3 ratio, respectively). Two senior radiologists blindly analyzed numerous CT findings with final consensus to perform univariate and multivariate statistical analyses. A diagnostic CT scan score was calculated with a bootstrap internal validation. Reproducibility was assessed based on the kappa statistic. ResultsA total of 208 patients (51 +/- 21 years; 114 males) were included (52 patients in the tumor group and 156 in the non-tumor group). In the multivariate analysis, an appendicolith and fat stranding were protective factors with OR = 0.2 (p = 0.01) and OR = 0.3 (p = 0.02), respectively, while mural calcifications (OR = 47, p = 0.0001), an appendix mass (OR = 7.1, p = 0.008), a focal asymmetric wall abnormality (OR = 4.9, p = 0, 001), or a ≥ 15 mm diameter (OR = 3.5, p = 0.009) were positive predictive factors of an underlying tumor. Using a ≥1 cut-off, our diagnostic score had an AUC = 0.87 (95 % CI, 0.82−0.93) and a positive likelihood ratio = 13.5 (95 % CI, 6.7–27.1). ConclusionWe developed a reliable scoring system based on CT findings, which is highly predictive of an underlying appendiceal neoplasm in an appendicitis context using a ≥1 cut-off.

A rare case of a completely thrombosed bilobed giant intracranial aneurysm of the anterior cerebral artery with spontaneous parent vessel thrombosis: case report

BackgroundA huge spherical intracranial mass can sometimes be misdiagnosed, due to the lack of typical radiographic features. Thrombosed giant intracranial aneurysms (GIAs) are an uncommon but still a possible differential diagnosis that must be kept in mind to guarantee the best surgical approach and resection of the lesion. We describe an extremely rare case of a huge bifrontal mass mimicking a cystic echinococcosis, in which the surgery unveiled a completely thrombosed GIA of the left anterior cerebral artery (ACA).Case presentationA 61-year-old patient complained about intermittent weakness of the right leg, mild holocephalic headache, beginning cognitive deficits and lethargy. Magnetic resonance imaging (MRI) showed a huge partially calcified and bilobed frontal mass with peripheral edema. Based on a time-resolved angiography with interleaved Stochastic trajectories MRI (TWIST-MRI), a vascular origin of the lesion was considered unlikely. Therefore, the surgery was performed under the suspicion of a cystic echinococcosis but revealed a bilobed GIA of the left ACA with a parent vessel thrombosis. Although only a limited left frontal craniotomy was performed, a proximal control of the parent vessel could be ensured, and the aneurysm was successfully clipped. The patient showed postoperatively no new neurological deficits.ConclusionsCompletely thrombosed GIAs with parent vessel thrombosis are rare lesions that might be misdiagnosed if typical radiographic features are missing. Thus, in case of an intracranial spherical mass with signs of intralesional hemorrhage and mural calcifications, presence of a completely thrombosed GIA should be considered as a possible differential diagnosis.

1544 Klippel-Trenaunay-Weber: Rare Congenital Syndrome Causing Persistent Rectal Bleeding

INTRODUCTION: Klippel-Trenaunay Weber syndrome (KTS) is a rare congenital disorder characterized by a triad of cutaneous capillary malformation (port-wine stain), venous malformations and limb hypertrophy. Diffuse vascular malformations of the gut in KTS can lead to gastrointestinal (GI) bleeding ranging from minor to life threatening hemorrhage necessitating surgical intervention. We describe a case of persistent rectal bleeding and iron dependent anemia in a patient with KTS. CASE DESCRIPTION/METHODS: A 32-year-old female with previous diagnosis of KTS was seen for recurrent hematochezia and iron deficiency anemia. A recent colonoscopy demonstrated non-bleeding arteriovenous malformations (Figure 1), presumed to be the source for the recurrent rectal bleeding episodes. On exam, she appeared pale, had a right hip deep purple telangiectasia, right posterior thigh palpable mass without visible varicosities and no limb hypertrophy. Her laboratory tests revealed: Hb: 9 g/dl, mean corpuscular volume: 75.0 fl, ferritin: 3 ng/ml and serum iron: 22 mcg/dl. CT abdomen/pelvis showed diffuse thickening of the rectum and sigmoid colon with scattered mural calcifications. A repeat colonoscopy demonstrated non-bleeding large circumferential vascular malformation extending from the dentate line to about 20 cm in the rectum (Figure 2) and multiple soft, pale lymphatic blebs in the colon and rectum (Figure 3). Her iron deficiency anemia from chronic blood loss was treated with oral and IV iron therapy. Despite conservative therapy, patient continues to bleed intermittently but has decided against surgical intervention at this time. DISCUSSION: GI hemorrhage occurring in association with KTS is an uncommon cause of rectal bleeding but is the most common symptom and potentially life-threatening emergency of KTS involving the gut. Investigation should begin with visualization of the entire GI tract for localization and extension of the disease. Angiography remains the gold standard and carries the advantage of allowing therapeutic interventions to control bleeding. The management of GI vascular malformations in KTS depends on the frequency and severity of blood loss requiring transfusions. Conservative management and iron supplements may be sufficient in the patient who presents with occasional non-debilitating bleeding. Transfusion dependency and life-threatening bleeding episodes necessitate definitive surgical therapy which involves resection of the diseased bowel.

Chylous Ascites in a Patient With Cirrhosis and Acute Restrictive Cardiomyopathy Due to a Pericardial Cyst

Chylous ascites is the accumulation of triglyceride-rich peritoneal fluid from the disruption of the lymphatic system due to damage of the thoracic duct due to injury, surgery, and infection. It can also occur from right heart failure, pericarditis, and cirrhosis. Chylous ascites is rare affecting 1 in 20,000 hospital admissions. Here we report a unique cause of chylous ascites in a patient with both cardiac and hepatic diseases. A 53 year-old gentleman with alcoholic cirrhosis decompensated by ascites and hepatic encephalopathy presented in undifferentiated shock requiring vasopressors. Initially he was presumed to be in septic shock from spontaneous bacterial peritonitis with paracentesis demonstrating 410 granulated cells and no triglycerides. The patient was prescribed Ceftriaxone and his clinical condition stabilized. Repeat paracentesis demonstrated decreased granulated cells and a triglyceride count greater than 200 mg/dL. A chest x-ray revealed a calcified pericardium and a subsequent cardiac MRI demonstrated a 13 x 7.5 cm extrinsic mass compressing the right ventricle and right atrium. Unfortunately, the patient's white blood cell continued to rise and vasopressor support escalated, although blood cultures remained negative. The patient underwent a coronary angiogram and a CTA of the chest for pre-operative evaluation for excision of this mass. He consequently developed worsening renal function and was placed on continuous veno-venous hemofiltration (CVVH). Despite aggressive vasopressor support and broad spectrum antibiotics, the patient continued to clinically deteriorate, and passed away. An autopsy was performed that demonstrated a 14 cm pericardial cystic mass with associated mural calcifications and acute inflammation consistent with active infection. This case highlights a rare, multifactorial cause of chylous ascites. This patient had a large calcified pericardial cyst compressing his right ventricle and right atrium leading to the development of chylous ascites due to increased hepatic venous pressure and hepatic lymph production. The large mass also caused right-sided heart failure with constrictive physiology, thus impairing hepatic lymph drainage. He had hepatic ascites chronically but never developed chylous ascites prior to the heart failure. This case highlights some of the many causes of chylous ascites and demonstrates that patient can have multiple coincident etiologies of a single manifestation of a disease.

Mucinous cystic neoplasms of the pancreas: high-resolution cross-sectional imaging features with clinico-pathologic correlation.

To describe the high-resolution cross-sectional (MDCT/MRI) features of mucinous cystic neoplasms (MCN) of the pancreas with clinico-pathologic correlation; to identify imaging predictors of high-grade dysplasia/carcinoma; and to estimate MCN growth rate. Thirty-two women (mean age: 46; range, 25-79years) with resected MCN who underwent preoperative MDCT (n=20) or MRI (n=12) examinations over a 14-year period were included. Two radiologists examined retrospectively in consensus the following MDCT/MRI features: MCN location, size/volume, presence of capsule and thickness of the capsule, and presence of mural nodules, enhancing septations, calcifications, chronic pancreatitis, and main pancreatic duct dilation. Imaging features were correlated with clinical symptoms, biochemistry results, and histopathologic features. A univariate model was analyzed for the prediction of high-grade dysplasia/carcinoma. Preoperative MCN growth rate was assessed using a subset of patients with more than one imaging study available (n=6). Twenty-five (78%) patients presented with symptoms and 8 (25%) patients had abnormal serum biochemical values. Mean MCN maximum dimensions were 48×45×45mm with a mean volume of 169mL. MCN were located in the tail (n=18), body (n=10), neck (n=2), and (head=2); 30 (93.5%) MCN were encapsulated, 3 (9%) had calcifications, 4 (12%) showed enhancing nodules, 9 (28%) had enhancing septations, and 5 (15%) had main pancreatic duct dilation. Associated chronic pancreatitis was observed in 4 (12%) patients. The only predictors for high-grade dysplasia/carcinoma were MCN size and volume. Using a cut-off size greater than 8.5cm, the specificity and sensitivity for high-grade dysplasia/carcinoma were 97 and 60%, respectively (p=0.003; OR 81, 95% CI 3.9-1655.8). Mean MCN growth rate was estimated at 4.2mm/year with a doubling time of 8.23years. MCN size (>8.5cm) and volume are the only features on MDCT/MR imaging that correlate with high-grade dysplasia/carcinoma. The average growth rate for MCNs is slow at approximately 4mm per year.

Utility of Appendiceal Calcifications Detected on Computed Tomography as a Predictor for an Underlying Appendiceal Epithelial Neoplasm.

Mucinous appendiceal neoplasms can contain radiopaque calcifications. Whether appendiceal radiographic calcifications indicate the presence of an appendiceal epithelial neoplasm is unknown. This study aimed to determine whether appendiceal calcifications detected by computed tomography (CT) correlate with the presence of appendiceal epithelial neoplasms. From prospective appendiceal and pathology databases, 332 cases of appendiceal neoplasm and 136 cases of control appendectomy were identified, respectively. Only cases with preoperative CT scans available for review were included in the study. Images were reviewed by two abdominal radiologists. Sensitivity, specificity, negative predictive value (NPV), and positive predictive value (PPV) were calculated, and the kappa statistic was used to determine agreement between the radiologists' interpretations. Interobserver agreement between the radiologists was substantial, with a kappa of 0.74. Appendiceal mural calcifications were identified on CT scans in 106 appendiceal neoplasm cases (32%) and in 1 control case (1%) (P=0.0001). In the appendiceal neoplasm subgroup, the presence of radiographic calcifications was associated with mucinous histology (35% vs 17%; P=0.006; odds ratio [OR], 0.38; 95% confidence interval [CI], 0.18-0.78) and with well-differentiated histologic grade (40% vs 24%; P=0.002; OR, 0.47; 95% CI, 0.29-0.76). The findings showed a sensitivity of 31.9% (95% CI, 26.9-37.2%), a specificity of 99.3% (95% CI, 96-100%), a PPV of 99.1% (95% CI, 94.9-100%), and an NPV of 37.4% (95% CI, 32.4-42.6%). This case-control study showed that appendiceal mural calcifications detected on CT are associated with underlying appendiceal epithelial neoplasms and that the identification of incidental mural appendiceal calcifications may have an impact on decisions regarding surgical intervention.

Mural calcifications in keratocystic odontogenic tumor: Report of a case with a brief review

Hard tissue deposits or mineralization in the wall of the keratocystic odontogenic tumor (KCOT) are infrequent and unusual. They are usually of dystrophic calcification or cartilage type, and dentinoid formation, although reported, is a rare phenomenon. Here, a rare case of extensive mural calcifications in the wall of the KCOT involving maxillary premolar-molar area, in a 14-year-old boy is described. These calcifications were found to be dentinoid, confirmed with the use of special stains.

Anatomical features and clinical importance of left atrial diverticula

To assess the anatomical features and clinical importance of left atrial diverticula and atrial accessory appendages in patients undergoing cardiac computed tomography with multidetector computed tomography. A total of 1305 consecutive patients (385 female, 29.5%; 920 male, 70.5%) were assessed using electrocardiogram-gated computed tomography between May 2010 and June 2013. The anatomical features and the prevalences of left atrial diverticula and left atrial accessory appendages were retrospectively assessed by four radiologists. The relationships between the prevalence and size of the diverticula and the age and gender of the patients were assessed. Among the 1305 patients, 610 (46.7%) exhibited 708 left atrial diverticula, and 62 (4.8%) exhibited left atrial accessory appendages. The most common locations of the left atrial diverticula were the right anterior superior wall (n = 328, 46.3%) and the lateral superior wall (n = 96, 13.5%). In addition to classical cystic and tubular diverticula, 49 (3.7%) of the patients exhibited mixed (cystic-tubular), conical, or hook-shaped diverticula and diverticular forms containing mural calcifications. There was no significant relationship between the prevalence of diverticula and the age and gender of the patients (P > 0.05). In addition to tubular and cystic diverticula, the left atrial wall can host different diverticular forms (such as mixed, conical, calcific, and hook shaped). It could be beneficial to assess the left atrium using MDCT to determine the source of emboli in cryptogenic embolism and to reduce complications associated with interventional procedures performed for left atrial arrhythmias.

Pseudomyxoma peritonei due to mucinous adenocarcinoma of the appendix

A 60-year-old man was referred for evaluation of long-standing abdominal pain. CT scan of the abdomen showed a fluid-filled dilated appendix with mural calcifications (Fig. A, asterisk) and intraperitoneal low-attenuation mass like nodular formations (Fig. B, arrows) causing scalloping of liver contour (Fig. A, C, arrowheads). The patient underwent appendectomy and peritonectomy followed by uneventful postoperative recovery. Histopathological examination confirmed the radiological suspicion of pseudomyxoma peritonei (PMP) due to appendiceal mucinous adenocarcinoma.

Testicular Epidermoid Cysts

The purpose of this series was to describe the sonographic features of intratesticular epidermoid cysts with clinicopathologic correlation. A retrospective review of the sonographic features of all intratesticular epidermoid cysts within the radiologic pathology archive of the Armed Forces Institute of Pathology with correlation with clinical, surgical, and pathologic data was performed. On sonograms, intratesticular epidermoid cysts were evaluated for shape, borders, and the presence of characteristic features, including alternating hypoechoic and hyperechoic rings, mural calcifications, and echogenic central core. Color Doppler imaging was evaluated for the presence of internal flow. Of 21 cysts seen in 20 male patients (mean age, 24 years; range, 11-52 years), the most common finding was mural calcifications, which were seen in 18 lesions (86%). Concentric rings of alternating echogenicity were shown in 13 lesions (62%), and 7 cysts (33%) had an echogenic central core. No cysts showed internal vascularity. Testicular epidermoid cysts are uncommon solid-appearing testicular masses that usually present as palpable masses and are benign. Knowledge of the sonographic and pathologic features may help differentiate these lesions from malignancy and possibly prevent unnecessary orchiectomy.

Case Report

Case Report

Unusual imaging appearances of pancreatic serous cystadenoma: correlation with surgery and pathologic analysis

We describe imaging and pathologic features of serous cystadenoma of the pancreas on multislice helical computed tomography CT (MS-CT) and surgical resection. Radiologic and pathologic features were analyzed in five patients. All patients underwent MS-CT and digital subtraction angiography (DSA), and four patients underwent magnetic resonance (MR) imaging. Preoperatively, three cases showed radiologic evidence of mainly solid appearance on MS-CT, and the suspected diagnoses were solid pancreatic tumors (patients 1-3). The other two cases showed radiologic evidence of macrocystic tumor of the pancreas, and the suspected diagnoses were mucinous cystic tumors (cases 4 and 5). All patients underwent surgery, and the diagnosis of serous cystadenoma was confirmed on pathologic examination. In three cases that showed a solid appearance on MS-CT, a microcystic appearance was identified on microscopic examination, and the tumors were found to be hypervascular lesions on multiphasic contrast-enhanced CT and DSA. In cases 1 and 2, the lesions showed high intensity with internal septation on T2-weighted MR images. In two cases, the tumors were classified as a macrocystic variant of serous cystadenoma, and no mural nodules, papillary projections, or calcifications were seen in the tumors. Imaging appearance of serous cystadenoma on MS-CT is various and sometimes indistinguishable from that of solid tumor or mucinous cystic tumors of the pancreas. Imaging findings of hypervascularity and a well-marginated high-intensity lesion with internal septation on T2-weighted MR imaging may be crucial to identify serous cystadenoma that contains no visible cystic compartments on MS-CT.

Calciphylaxis Involving Both the Upper and Lower Extremities

Calciphylaxis is an uncommon complication of end stage renal disease (ESRD) and secondary hyperparathyroidism. It characterized by cutaneous necrosis with mural calcifications and thrombosis in the small vessels of dermis. It is important to diagnose and treat, because of mortality rate from calciphylaxis is very high. We present the case of a patient with ESRD and type II diabetes mellitus developing calciphylaxis of the both upper and lower extremities had normal corrected calcium-phosphate product level. After amputation, necrosis was showed rapid progression resulting in death in one month.

Semitransparent Volume-rendering CT Angiography for Lesion Display in Aortoiliac Arteriosclerotic Disease

The potential of semitransparent volume-rendering (STVR) computed tomographic (CT) angiography was evaluated for the assessment of hemodynamically significant stenoses in aortoiliac arteriosclerotic disease. In a prospective study, 76 patients (57 men, 19 women; mean age, 70 years) underwent single-detector (n = 26) or multidetector (n = 50) CT angiography of the aortoiliac region. Intraarterial digital subtraction angiography (DSA) was performed in each patient. STVR images with semitransparent display of arterial lumen (opacity, 50%) and vascular calcifications (opacity, 20%), as well as maximum-intensity projection (MIP), frontal/sagittal curved planar reformation (CPR), and MIP/axial studies were independently reviewed for hemodynamically significant lesions (> or =70% cross-sectional area reduction). DSA combined with invasive pressure measurement was used as the standard of reference. Vessel wall calcifications were classified according to a four-point scale (0, not calcified; 1, mildly calcified; 2, moderately calcified; 3, severely calcified). Of the 380 reviewed vessel sections, 28 represented 70%-99% stenoses and 14 represented occlusions. For detecting >/==" BORDER="0">70% lesions, STVR as well as CPR and MIP/axial studies revealed significantly higher specificity (91%-94%), positive predictive value (PPV; 0.62-0.72), and accuracy (90%-94%) than MIP (specificity, 59%; PPV, 0.27; accuracy, 64%; P <.001) in moderately or severely calcified vessel sections. In noncalcified or mildly calcified sections, the specificity of MIP was comparable with the other rendering techniques (96%-98%; P values ranging from.34 to.77). No significant sensitivity differences were noticed among the CT angiography modalities: STVR, 79%; MIP, 88%; CPR, 83%; and MIP/axial, 93% (P values ranging from.12 to.78). Negative predictive values ranged from 0.97 to 0.99 (P values ranging from.14 to 1). Median calcification scores in sections with overestimated lumen narrowings were significantly higher (3, severely calcified) than in sections with correctly graded lumen width (1, mildly calcified) with all CT angiography modalities (P <.05). With use of STVR, visualization of vascular lumen can be improved by rendering highly transparent mural calcifications. Hence, for three-dimensional presentation of aortoiliac arteriosclerotic disease, STVR studies should be preferred to MIPs as supplements to review of axial-source images.

Macrocystic serous adenoma of the pancreas: radiologic-pathologic correlation.

Macrocystic serous adenoma is a rare benign pancreatic neoplasm, recently described in the pathology literature. We describe the CT and MR imaging features in a series of five consecutive pathologically proven cases. Of seven cases fulfilling the pathology criteria for macrocystic serous adenoma over an 11-year period, five patients underwent preoperative CT and MR imaging at our institution. In addition to the clinical presentation and pathologic features of the tumor, the following CT and MR imaging features were reviewed: size and location; wall thickness; internal septations; and presence of mural nodules, papillary projections, or calcifications. All patients but one were women (age range, 36-78 years; mean age, 48.6 years). The sizes of the tumors ranged from 1.5 to 5.0 cm (mean, 3.1 cm). Three (60%) of five tumors were located in the pancreatic head. The wall measured less than 2 mm in four lesions and 4 mm in one. No mural nodules, papillary projections, or calcifications were present. Lesions were unilocular (n = 3) or bilocular (n = 2). Excellent correlation of imaging features with gross pathology was observed. On CT and MR imaging, the macrocystic variant of serous adenoma typically appears as a small (< 5 cm), uni- or bilocular cyst with a thin (< 2 mm) wall that lacks mural nodules or calcifications. The imaging appearance of macrocystic serous adenoma is distinctly different from that of microcystic serous cystadenoma, but the imaging appearance of macrocystic serous adenoma is indistinguishable from mucinous cystadenoma and cystadenocarcinoma of the pancreas.

Adult Presentation of Metanephric Stromal Tumor

A 53-year-old woman presented with right upper quadrant pain and biliary colic. Ultrasound showed multiple gallstones and a solid cystic mass in the right kidney. Computerized tomography of the abdomen confirmed the presence of a central contrast enhancing mixed solid cystic mass with mural calcifications (fig. 1). No adenopathy was noted. The patient denied a history of flank pain, nephrolithiasis, gross hematuria, voiding symptoms, bone pain, fever, chills or weight loss. Medical history was negative for hypertension, and family history was negative for renal disease. Bone scan, chest x-ray, urine cytology and urinalysis were negative. Excretory urography demonstrated no filling defects in the collecting system. Given the solid nature of the tumor, its central location and the presence of a normal contralateral kidney, right laparoscopic radical nephrectomy and concomitant laparoscopic

Tracheobronchial amyloidosis. The Boston University experience from 1984 to 1999.

Tracheobronchial amyloidosis (TBA), an idiopathic disorder characterized by deposition of fibrillar proteins in the tracheobronchial tree, occurred in 10 patients referred to the Amyloid Program at Boston University over the past 15 years. Fewer than 100 cases of TBA have been described; only 1 series encompassed more than 3 patients. We analyzed our experience with biopsy-proven TBA to define better its natural history. Follow-up averaged approximately 8 years and was obtained in all cases, making this outcome reporting the largest and most complete to date. Three of these patients were prospectively studied for up to 24 months to examine the utility of bronchoscopy, computerized tomography (CT) imaging, and pulmonary function tests (PFTs) in monitoring disease progression. No patient with TBA developed signs or symptoms of systemic amyloidosis during the period reviewed. Conversely, tracheobronchial disease was not diagnosed in 685 patients with primary systemic (AL) amyloidosis during the 15-year study period at Boston University. Bronchoscopy proved most useful in establishing the diagnosis by biopsy. Narrowing of major airways limited its inspection of the tracheobronchial tree, however. In contrast, CT imaging provided quantitative assessment of airway narrowing and mural thickening--2 major consequences of amyloid infiltration. These CT features, in the presence of mural calcifications sparing the posterior tracheal membrane, have been reported in few disorders other than TBA. The ability of CT to map airway involvement and identify extraluminal manifestations of TBA made it the study of choice for establishing disease extent. Three patterns of disease were evident by CT imaging and bronchoscopic examination: proximal, mid, and distal airways involvement. Those with severe proximal disease had significantly decreased air flows, air trapping, and fixed upper airway obstruction on PFTs. Patients with distal disease had normal airflows. PFTs could not clearly distinguish proximal from severe mid airways disease. Thirty percent of patients died within 7-12 years after diagnosis, all having proximal or severe mid airways disease. Repeated rigid bronchoscopic debridement and laser treatments did not prevent progressive airways narrowing in patients dying from TBA. Most patients with mid airways involvement, and all distal airway cases, had either stagnant disease or slowly increasing amyloid deposits when followed for up to 14 years. In a small subset of patients followed prospectively, serial PFTs were most sensitive to disease progression. CT-derived measures of airway lumen diameter and wall thickness did not change significantly despite marked improvements in airflow after rigid bronchoscopy. Our experience suggests that serial PFTs and CT imaging together offer the best assessment of airway involvement and disease progression in patients with TBA. In the future, radiation therapy may provide more definitive treatment of TBA than debulking procedure have to date.

Takayasu arteritis: evaluation of mural changes in the aorta and pulmonary artery with CT angiography.

To evaluate mural changes in the aorta and pulmonary artery in Takayasu arteritis by using computed tomographic (CT) angiography. CT angiography was prospectively performed in 12 women with Takayasu arteritis and in 10 healthy adults. Scanning included three distinct phases: precontrast (before administration of contrast material), arterial, and delayed. Precontrast transverse images revealed a high-attenuation aortic wall (10 patients) and mural calcifications in the aorta (nine patients). Arterial-phase images revealed circumferential wall thickening of 1-4 mm in all patients and enhancement in five patients with active disease. Delayed-phase CT revealed circumferential enhancement in eight patients. A concentric, low-attenuation ring was also identified inside the aorta on the arterial-phase (two patients) and delayed-phase (three patients) images. In the pulmonary artery, wall thickening was revealed with early and delayed enhancement in two patients. CT angiography may demonstrate changes in the vascular wall in patients with Takayasu arteritis.