Cushing's disease: Pituitary imaging

Fourteen patients with adrenocorticotropic hormone (ACTH)-dependent hypercortisolism underwent pituitary scanning with computed axial tomography (CT) and magnetic resonance imaging (MRI). Computed tomography revealed pituitary macroadenomas in two patients, pituitary hyperplasia in one and a suspicion of pituitary microadenoma in one. Thirteen patients underwent MRI. One with a macroadenoma diagnosed on CT did not undergo MRI. The MRI revealed a pituitary macroadenoma in one, microadenoma in three and hyperplasia in two cases. Magnetic resonance imaging following gadolinium diethylene triamine penta acetic acid (gd-DTPA) enhancement revealed four more pituitary microadenomas. All patients who had pituitary adenomas (micro and macro) and hyperplasia underwent trans-sphenoidal pituitary surgery. One of the two patients, who had an enlarged pituitary on imaging but no demonstrable adenoma, was found to have a microadenoma at surgery. Patients with ACTH-dependent hypercortisolism should undergo MRI of the pituitary gland to identify/localize corticotroph pituitary adenomas. The study should include gd-DTPA enhancement in cases where the scan is normal.     

Castleman's Disease of the Chest Wall

Primary Castleman's disease of the chest wall is unusual. Furthermore, such tumors arising from a surgical wound are extremely rare. We report a 33-year-old female with a history of a thoracic surgery at 5 years of age. A round, homogenous 4 × 3.5-cm mass protruded into the thoracic cavity on the posterior portion of the previous posterolateral incision. The tumor was completely removed, with combined rib resection. The resected specimen showed Angiofollicular Lymph Node Hyperplasia (Castleman's disease), hyaline-vascular type. No recurrence has been found for 10 years. This is the first report of primary chest wall Castleman's disease arising from the surgical wound.     

Castleman's tumor with retroperitoneal location: a malignant-appearing benign tumor

A case of a large retroperitoneal tumor in the pararenal region is presented. The tumor had an initial appearance of a malignant neoplasm both radiographically and at operation but histopathologically it turned out to be a rare benign tumor, Castleman's disease. Based on the benign nature of this lymphoid tumor extensive surgical procedures should be avoided as limited excision of the tumor appears to have been curative in all the patients reported in the literature.     

Aggressive angiofollicular lymph node hyperplasia (Castleman's disease) treated with high dose melphalan and autologous bone marrow transplantation

A case of aggressive widespread angiofollicular lymph node hyperplasia in a 42-year-old male, treated with high dose melphalan is presented. The disease had failed to enter a durable remission after chemotherapy. High dose melphalan with autologous bone marrow transplantation achieved a complete remission which has lasted for 15 months to date. This approach can be considered when other measures fail.     

Castleman's Disease: Systematic Analysis of 416 Patients from the Literature

Background.

Castleman''s disease is a rare primary disease of the lymph nodes with limited available clinical information.

Methods.

A systematic literature search identified 416 cases amenable to detailed analysis.

Results.

In HIV⁻ patients, centricity, pathology type, the presence of symptoms, gender, and age all predict outcome in univariate analyses. The 3-year disease-free survival (DFS) rate for patients with unicentric hyaline vascular disease (49.5% of cases, class I) was 92.5%, versus 45.7% for those with multicentric plasma cell disease (20.2% of cases, class III) and 78.0% for those with any other combination (22.6% of cases, class II) (p < .0001). HIV⁺ patients (class IV) exclusively presented with multicentric plasma cell disease and had a 3-year DFS rate of only 27.8%. Kaposi''s sarcoma and lymphoma were observed in 59.3% and 9.4% of HIV⁺ patients and in 2.6% and 3.6% of HIV⁻ patients (p < .0001). Paraneoplastic pemphigus and the syndrome of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes were observed exclusively in HIV⁻ patients at a rate of 1.3% and 1.8%, respectively.

Conclusion.

Clinical, pathological, and viral markers allow for the classification of Castleman''s disease into groups with markedly different outcomes and disease associations.     

Castleman's disease: from basic mechanisms to molecular therapeutics

Castleman's disease is a rare lymphoproliferative disorder in which there has been recent progress in elucidating underlying mechanisms with potential therapeutic implications. Unicentric Castleman's disease is an indolent condition that is often treated with local approaches. In contrast, patients with multicentric Castleman's disease (MCD) have a less favorable prognosis and require systemic treatment. Cytotoxic chemotherapy, with its attendant risk for toxicity, has been widely used to treat MCD, with variable efficacy. The discovery of putative etiologic factors and targets in MCD, particularly human herpes virus 8, CD20, and interleukin (IL)-6, has been translated into the use of rituximab and anti-IL-6-based therapy, as well as antiviral agents. In this article, we review the current state of the art of our understanding of Castleman's disease and its treatment and we provide insight into future treatment strategies based on disease biology.     

Performance of magnetic resonance imaging in pulmonary fungal disease compared to high‐resolution computed tomography

To evaluate the performance of magnetic resonance imaging (MRI) compared to computed tomography (CT) in patients diagnosed with pulmonary mycosis. We prospectively included 21 patients diagnosed with pulmonary mycosis between January 2013 and October 2014. Inclusion criteria were presence of respiratory symptoms, histopathological diagnosis of mycosis and absence of mycosis treatment. Reviewers identified one predominant imaging pattern per patient: nodular, reticular or airspace pattern. Afterwards, all CT findings were analysed separately per lobe and compared to MRI. Nodular pattern was the most common found (CT: 76.20%; MRI: 80.96%), followed by airspace pattern (CT and MRI: 9.52%) and reticular (CT: 9.52%; MRI: 4.76%). Compared to CT, MRI performance varied according to radiological finding and pulmonary region. For nodules, MRI presented high sensitivity (100% [95% CI: 93.52‐100]) and specificity (100% [95% CI: 92.00‐100]). For bronchiectasis and septal thickening, there were poorer positive predictive values (33.33% [95% CI: 1.77‐87.47]; and 83.33% [95% CI: 50.88‐97.06] respectively). As specificity and negative predictive value had superior results than sensitivity and positive predictive value, rather than for diagnosis of this condition, MRI might be more considered for the follow‐up of patients with pulmonary mycosis, an alternative to multiple radiation exposures with CT follow‐up.     

Diagnostic Accuracy of MRI Compared to CCT in Patients with Brain Metastases

Objectives. In patients with extracranial neoplasms, the occurrence and number of brain metastases (BM) are critical for further diagnostic approaches and therapeutic strategies and the patient's prognosis. Although widely accepted, there is surprisingly little evidence in the literature that MRI is superior to CCT. Therefore, in patients with solitary BM according to diagnostic contrast-enhanced computed tomography (CCT), we investigated, what additional information could be gained by contrast-enhanced magnetic resonance imaging (MRI). Methods/Results. Among 55 patients with solitary BM according to CCT, 17 had multiple BM on MRI (31%) and 38 had solitary BM in both. Based on a presumed binomial distribution of our data, we calculated a rate of at least 19% of patients with solitary BM on CCT, in which MRI would show multiple lesions (p=0.05). The two main characteristics for BM missed by CCT were the smaller diameter, which averages 2cm less than in BM identified with both modalities, and a preferential frontotemporal location. Conclusion. MRI is indeed superior to CCT in the diagnosis of BM the essential reasons besides detection of smaller lesions being a better soft tissue contrast, significantly stronger enhancement with paramagnetic contrast agents, the lack of bone artifacts, fewer partial volume effects, and direct imaging in three different planes. Therefore, MRI is indispensable in the diagnostic workup of patients with BM for choosing the optimum therapeutic approach, especially with regard to the decision whether to operate or to primarily irradiate the patient's metastases.     

Relative efficacy of different MRI signs in diagnosing active Crohn's disease, compared against a histological gold standard

Introduction: There has been growing interest in the use of MRI in assessing Crohn's disease because of its lack of ionising radiation. Many MRI signs have been described in the literature, but their relative utility is unknown. Methods: The MRIs of the bowel performed at 1.5 Tesla were blindly reviewed on 26 patients with recent histology (surgery and/or colonoscopy and their associated reports) according to a dedicated pro forma. Each patient's bowel was divided into nine segments. Each segment was assessed as to the presence or absence of 15 MRI signs described in the literature: abnormal gadolinium enhancement (both subjective and objective), wall oedema, fat oedema, fat proliferation, nodal enlargement, free fluid, wall nodularity, serosal blurring, mural thickening >4 mm, stricture, multi‐segmental disease, fistula, abscess and layered contrast enhancement. The results were compared against a histological gold standard with a six‐point scale of disease severity. Results and conclusions: MRI correctly identified all 15 patients with at least established mucosal disease, and three of eight with only mild mucosal disease. Combining these results, a positive MRI correlated highly with at least early mucosal disease (positive predictive value 95%), while the presence of established mucosal disease was unlikely if MRI was negative (negative predictive value 100%). The MRI signs found to be the most sensitive for detecting active Crohn's disease were those related to the bowel wall, namely, wall thickening, nodularity, contrast enhancement and oedema. The most specific signs were the presence of multi‐segmental disease, layered contrast enhancement and complications (fistula and abscess).     

Lymphangioma: Imaging diagnosis

Lymphangiomas are congenital malformations of the lymphatics that are curable by extirpation. Accurate delineation of lesion extension is important for pre-operative diagnosis, surgical planning, and assessing recurrence. The radiologic findings were retrospectively evaluated to determine the imaging appearance of these benign tumours. The plain radiographs, barium meal, ultrasound, CT, and MR images of 18 patients with one or more pathologically proved lymphangiomas were reviewed. Plain radiography and barium study showed masses displacing adjacent organs. Ultrasound examination showed uni- or multilocular cystic masses with smooth, thin or irregular, thick walls. Enhancement of the cyst wall was variable on CT and MR studies. The CT density of the fluid ranged from ?4 to 34 HU depending on the lipid content and the presence of haemorrhage. The cysts were isointense to muscle on T1 -weighted and hyperintense to fat on T2-weighted MR images. The MR imaging delineated the tumour lesion extension more clearly than ultrasound and CT scans. Ultrasound, CT, and MR imaging are valuable for evaluating lymphangiomas. Magnetic resonance imaging allows accurate determination of lesion extension.     

Central neurocytoma occurring in the thalamus: CT and MRI findings

A case of central neurocytoma occurring in the thalamus, confirmed by electron microscopy and immunohistochemistry, is reported. Computed tomography demonstrated a hypodense mass with an irregular margin arising from the right thalamus, extending into the lateral ventricle and crossing the midline. It contained areas of calcification and showed slight enhancement after contrast injection. On magnetic resonance imaging (MRI) scan, the tumour was hypointense to the brain in T1-weighted images and was heterogeneously hyperintense to the brain in T2-weighted images.     

Tuberous sclerosis: Ultrasound,CT and MRI features of two cases with multiple organ involvement

The cases of two patients with tuberous sclerosis with multiple sites of involvement are presented. Both patients had characteristic cerebral lesions of tuberous sclerosis associated with bilateral renal angiomyolipomas and hepatic hamartomas. Additionally there were diffuse pulmonary cystic changes in one patient and cardiac rhabdomyoma in the other.     

Follow-up of patients with Hodgkin's disease following curative treatment: the routine CT scan is of little value

A total of 10-40% of patients with Hodgkin's disease relapse following initial curative therapy. Intensive follow-up is resource intensive and may identify false relapses. We performed a retrospective review of all patients with Hodgkin's disease treated at our centre between 1990 and 1999 to evaluate the utility of the components of follow-up. A total of 107 patients met the inclusion and exclusion criteria. The median age was 33 years and the median duration of follow-up 38 months. The total number of follow-up visits was 1209 and total number of CT scans 283. There were 109 suspected relapses of which 22 proved to be true relapses. Of the latter, 14 were identified clinically, six radiologically and two via lab testing. The routine CT scan detected only two relapses (9%), yet accounted for 29% of the total follow-up costs. Based on data from our centre, the cost per true relapse was $6000 US, 49% incurred by radiological tests. The majority of the cost of follow-up was incurred by routine follow-up (84%) as opposed to the investigation of suspected relapses (16%). We conclude that most true relapses are clinically symptomatic and that the routine CT is an expensive and inefficient mode of routine follow-up.     

Imaging features of primary pulmonary liposarcoma

Primary liposarcoma of the lung is extremely rare. We report a 28‐year‐old pregnant woman who complained of dyspnoea during the third trimester. Chest radiography, thoracic ultrasound, CT and MRI showed a huge heterogeneous tumour involving all the left lung and the mediastinum. The tumour was composed of soft tissue, and fatty and cystic components with calcifications. Diagnosis was made on core biopsy under CT guidance. Surgical excision was performed but unfortunately the patient died during the operation.     

TAFRO syndrome with fatigue and abdominal distension as the first symptom: A case report

TAFRO syndrome is a peculiar and rare type of multi-centric Castleman's disease which contained a series of symptoms such as thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O). Here we provide a case of TAFRO syndrome with the manifestation of fatigue, abdominal distension, and low fever at primary diagnosis, characterized by multiple lymphadenopathy of superficial mediastinal and retroperitoneal lymph nodes, and it was finally confirmed by lymph node biopsy. The patient recovered speedy after receiving CHOP chemotherapy. In this case report, the patient has a history of dust-exposure and hepatitis B virus infection, which may be potentially related to the disease. In addition, this case suggests the importance of pathological biopsy of complete lymph nodes in diagnostic process.     

Detection of malignant bone marrow involvement with dynamic contrast-enhanced magnetic resonance imaging.

BACKGROUND: The purpose of this study was to evaluate the role of dynamic contrast-enhanced magnetic resonance imaging (dMRI) in detecting bone marrow involvement in cancer patients. PATIENTS AND METHODS: We studied 50 consecutive patients with histologically confirmed malignant dissemination to the bone marrow, using dMRI of the lumbosacral spine. Time-signal intensity curves were generated from regions of interest (ROIs) obtained from areas of obvious bone marrow disease (group B). In 16 patients from group B with focal disease, ROIs were also placed on areas with apparently normal bone marrow on static magnetic resonance images (group C). Twenty-two patients with no history of malignancy were used as a control group (group A). Wash-in (WIN) and wash-out (WOUT) rates, time to peak (TTPK), time to maximum slope (TMSP) values and WIN/TMSP ratios were calculated for each patient. Mean values for the three groups were compared statistically. Six patients from group B had follow-up dMRI after chemotherapy: four patients achieved a clinical partial response and two had resistant disease. RESULTS: A significant difference was found between groups A and B for all values. Between groups A and C, in spite of the similar static MRI appearance, all values were significantly different. Between groups B and C, a significant difference was found for WIN, WOUT rates and WIN/TMSP ratio. Follow-up dMRI data analysis correlated well with clinical staging. CONCLUSIONS: dMRI can distinguish normal from malignant bone marrow. It may identify malignant bone marrow infiltration in patients with negative static MRI and serve as both a diagnostic and prognostic tool for patients with bone marrow malignancies.     

A Review of Imaging Practice in Bone and Soft Tissue Lesions

Thirty six consectutive bone and soft tissue lesions which were referred to the Bone Transplantation Service between January 1987 and June 1989 were reviewed with respect to the information provided by pre-operative CT, MR and plain X-ray and the final histopathologic diagnosis. The ability of CT and MR to demonstrate cortical destruction, periosteal reaction, a soft tissue mass and soft tissue calcification was scored on a scale of one (low suspicion of abnormality) to four (definite abnormality) in each case. Of the thirty six cases reviewed there were twenty-two histologically confirmed tumours with all of the above three imaging modalities available for comparison. In each of these tumours MR was found to provide an equal or more accurate assessment of soft tissue extent. On a scale of 1 to 4 the average point scores were 3.6 and 2.6 for MR and CT respectively. Little difference was shown between CT and MR with respect to medullary involvement (4.0 points vs. 3.9) and cortical destruction (2.5 points vs. 2.7). CT (3.7 points) was more sensitive than MR (1.2 points) in detecting fine soft tissue calcification. In only five cases did the radiology report correctly nominate the histologic diagnosis and thus the role of imaging was more valuable in staging than diagnosis. For this reason a management oriented report should include comment regarding cortical destruction, the intramedullary extent of tumour and the extent of soft tissue involvement. MR is recommended in the investigation of all suspected malignant soft tissue or bone tumours.     

Computed tomography and magnetic resonance imaging of unusual causes of ankle pain

Computed tomography and MRI are frequently utilized to evaluate ankle pain that remains unexplained by radiography. The most common causes of ankle pain are related to trauma and the imaging appearances of these entities are well established in the radiologic and orthopedic literature. A smaller percentage is comprised of non‐traumatic disorders. Our goal is to emphasize the value of CT and MRI in recognition of these less common and unusual causes of ankle pain.