Angelos Kalovidouris

Bone resorption is increased in young adults with thalassaemia major

Bone disease in patients with thalassaemia major is a multifactorial and still poorly understood process. The present study evaluated 45 thalassaemic patients using dual X‐ray absorptiometry at three sites (lumbar spine, head of femur and forearm) to assess bone mineral density, in parallel with a series of biochemical markers to measure bone formation and bone resorption. To identify possible interfering factors, our patients were grouped according to whether or not they needed transfusion therapy; the presence of hypogonadism was also considered. Our results showed that patients on regular transfusions had a markedly low bone mineral density in contrast to those not requiring blood support and that this finding was more pronounced in the hypogonadic group, irrespectively of sex. The decrease of bone mineral density values was more prominent in the forearm, thus making this site particularly interesting for such studies. Bone formation, as evidenced by the levels of serum alkaline phosphatase and osteocalcin, did not appear to be impaired, while bone resorption was grossly increased in all patient groups. The latter process was clearly evident using the recently introduced measurement of the urinary N‐terminal peptides of collagen type I, the sensitivity of which has already been established in other groups of osteoporotic patients. Our conclusion is that, in spite of the severe bone destruction that occurs in thalassaemia major, the fact that bone formation remains intact calls for a more intensive treatment comprising hormonal replacement, bisphosphonates and other agents.

Assessment of liver iron overload by T2-Quantitative magnetic resonance imaging: Correlation of T2-QMRI measurements with serum ferritin concentration and histologic grading of siderosis

PURPOSE To correlate hepatic 1/T2 values obtained by means of a T2-Quantitative MRI (T2-QMRI) technique with three widely applied methods for the evaluation of hemosiderosis, i.e., (a) liver iron concentrations (LFeC) (b) serum ferritin (SF), and (c) histologic grading of siderosis. The impact of coexisting hepatitis was also considered. T2-QMRI measurements were compared with signal intensity (SI) ratio measurements on conventional SE images. MATERIALS AND METHODS Liver T2 relaxation times were calculated in 40 thalassemic patients, on a 0.5 T magnetic resonance imaging system using a multiple spin-echo sequence with parameters: TR = 2500 ms, TE = 12 ms in 20 symmetrically repeatable echoes. RESULTS (a) 1/T2 values were well correlated (r = 0.97) with liver iron concentrations, which ranged from 2.32 to 18.0 mg/g dry weight (normal < 1.6 mg/g). (b) 1/T2 values were also correlated with serum ferritin levels (r = 0.84). At various 1/T2 values, serum ferritin levels were higher for the anti-HCV(+) patients than the anti-HCV(-) ones. (c) T2 values corresponding to successive grades of siderosis presented statistically significant differences. (d) SI ratio measurement assigned less statistically significant results, as compared to T2 values. CONCLUSION T2-QMRI measurement of T2 relaxation time is more accurate than SI ratios in evaluating liver iron overload. It is particularly useful for hemosiderotic patients with coexisting hepatitis since, in this case, serum ferritin is not considered a reliable index of hemosiderosis.

Primary Non-Hodgkin's Lymphoma of the Gall Bladder

Primary non-Hodgkin lymphoma of the gallbladder is a very rare location of extranodal non-Hodgkin lymphomas. A patient with a primary non-Hodgkin lymphoma of the gallbladder is reported and in addition, the English literature is reviewed. Clinical presentation, diagnostic evaluation, histopathologic findings, treatment modalities and prognosis of primary gallbladder lymphomas reported up to date are reviewed and discussed. Our patient was diagnosed as a T-cell lymphoblastic lymphoma, after cholecystectomy, and had no evidence of disease elsewhere. She was treated with combination chemotherapy and complete remission was achieved. She remains free of disease 9 years later. Review of the literature over a 30-year period revealed only 12 cases of well-documented primary non-Hodgkin lymphoma involvement of the gallbladder, including the present case. Patients present clinically with symptoms and signs indicating either biliary tract pathology or a gastrointestinal tumor. Diagnostic investigation included ultrasound of the upper abdomen, computed tomography of the abdomen and pelvis, oral cholecystography, percutaneous cholangiography and endoscopic retrograde cholangiopangreatography. Preoperative diagnosis was established in none of the patients. Treatment modalities included surgery and postoperative chemotherapy and irradiation. The prognosis is overall poor and only 2 patients are alive after 1 and 9 years respectively, the latter being our case.Here we document the first reported case of a patient with primary T-cell lymphoblastic non-Hodgkin lymphoma of the gallbladder. Review of the literature shows the existence of non-Hodgkin lymphoma of the gallbladder, its rarity and its general dismal prognosis.

Treatment of hydatid liver disease.

BACKGROUND Echinococcal disease is still a serious problem in certain parts of the world. The liver is the organ affected most frequently. Over recent decades, various reports have been published comparing standard surgical and more conservative modalities for the treatment of the disease. Proper selection among the strategies reported so far could be the most appropriate part of successful patient management. METHODS A thorough review of the most recent reports on the treatment of liver Echinococcus infection, including percutaneous drainage, medical management, and laparoscopic, endoscopic and open surgical approaches has been conducted, focusing on patient outcomes. RESULTS The standard surgical approach, namely partial capsectomy, drainage, and epiploplasty, remains the most frequent operative method. Previous problems such as disease recurrence and a residual cavity seem to be managed optimally with more radical operations such as total pericystectomy. Laparoscopic and endoscopic approaches (endoscopic retrograde cholangiopancreatography and sphincterotomy) appear to have a crucial role, especially when the location of the cyst or the patients status does not permit more radical approaches. Percutaneous computed tomography- or ultrasound-guided drainage appears to be of great value in certain cases. Chemotherapeutic agents remain useful as an adjuvant treatment. CONCLUSIONS Appropriate patient selection and proper use of the various available treatments are of great importance. A tailor-made approach should be employed for each patient. The surgeons experience constitutes a principal criterion. To achieve the best possible results, an interdisciplinary approach should be used in the majority of patients.

Primary Lung Involvement in Waldenström’s Macroglobulinaemia

Pulmonary involvement in Waldenström’s macroglobulinaemia (WM) occurs in 3–5% of cases, but lung involvement without bone marrow infiltration is extremely rare. We report 2 patients who presented with bilateral consolidations on chest X-ray and non-specific symptoms and were treated for a long period of time for pulmonary infections until the diagnosis was made by open lung biopsy. Both patients presented high monoclonal IgM in the serum and one also had blood lymphoplasmacytosis. Trephine bone biopsy and bone marrow smears were normal and there was no other site of involvement. Along with the presentation of our patients, we review the literature, discuss some of the possible underlying mechanisms and raise the attention of clinicians to this rare manifestation of the disease.

Spinal neurilemmomas and neurofibromas : central dot sign in postgadolinium MRI

The MR studies of three histologically proven spinal neurilemmomas and neurofibromas were reviewed retrospectively. There were two benign neurilemmomas (schwannomas) and one neurofibroma. The common characteristic of these cases was a central low intensity focus (“dot”) seen on postcontrast T1-weighted imaging. The low intensity foci corresponded histologically to a congeries of changes including edema, microcysts, foam cells, hyalinization of blood vessels, old hemorrhage, and dystrophic calcification.

The Real Incidence of Extracapsular (Satellite)Cysts of Liver Echinococcus

Background: The presence of extracapsular (Satellite) cysts in liver echinococcus granulosus is known for many years. In one of our previous studies of radiological (CT) material they were found to be present in 16% of cases. Methods: In the present study the operative findings, in cases of total capsectomy (cystopericystectomy) or partial lobectomy are presented. Results: The real incidence of these cysts in operative specimens was as high as 29,5%. They were present in 15 out of 51 totally excised cysts. Conclusions: We conclude that satellite cysts are present more often than they are radiologically detected. As they can be incriminated as a cause of recurrence of the disease they must be excised en block with the main parasitic cysts, by means of more radical procedures such as cystopericystectomy or partial hepatectomy, whenever it is feasible.

A randomized phase II trial of aminoglutethimide and hydrocortisone versus combined aminoglutethimide, hydrocortisone and fluoxymesterone in advanced breast cancer

Fifty postmenopausal women with advanced breast cancer were included in the following randomized phase II trial: 25 patients received aminoglutethimide 1000 mg and hydrocortisone 40 mg daily. Twenty-five patients received aminoglutethimide 1000 mg, hydrocortisone 40 mg and fluoxymesterone 20 mg daily. The two groups of patients were comparable in respect to the most important pretreatment characteristics. The majority of patients in both groups had bone lesions. There was a history of response to tamoxifen in all the cases and 17 patients had positive estrogen and progesterone receptors. The evaluation of response was based on the system adopted by the UICC. In the aminoglutethimide-hydrocortisone group, 16 (64%) patients obtained a partial remission, 3 (12%) remained stable and 6 (24%) had progressive disease. In the combination treatment group, 17 (68%) patients obtained a partial remission, 3 (12%) remained stable and 5 (20%) developed progressive disease. The median duration of partial remission and stabilization of the disease was 9 and 7 months respectively in both groups.

CT and MR of pineal region tumors

Magnetic Resonance (MR) imaging features of pineal region tumors were analyzed in 14 oncologic cases. The tumors were classified as germ-cell tumors, glial tumors, pineal parenchymal tumors, meningiomas, and cysts. They demonstrated different MR signal characteristics on precontrast scans and nodular or ring type enhancement with occasional central lucencies, except for benign cysts, which have not shown enhancement. MR images were useful in defining the relationship of the tumor to the posterior third ventricle, sylvian aqueduct, vein of Galen, and tentorium. Although CT can demonstrate in more evident fashion displacement of the original pineal calcification as well as tumor calcifications, MR imaging demonstrates different signal characteristics in germinomas and pineoblastomas which can be a useful adjunct in the evaluation and differential diagnosis of these tumors.

Characterization of pheochromocytomas using quantitati analysis of the parameter T2 of the mass (T2-QMRI)

The usefullness of MR imaging in the characterization of pheochromocytomas was evaluated in 9 patients with 13 pheochromocytomas and 1 paraganglioma. In two patients the tumors were multiple and in one, extra-adrenal. Two patients conformed to MEN2 syndrome. Adrenal masses were characterized using several parameters: (a) visual inspection of the signal intensities in T1-, T2-, and Gd-DTPA-enhanced T1-weighted images; (b) observed signal intensity and observed signal intensity ratios (adrenal mass/liver, adrenal mass/retroperitoneal fat and adrenal mass/ subcutaneous fat) in the T2-weighted images; (c) calculated T2 relaxation times and calculated T2 relaxation time ratios (adrenal mass/liver, adrenal mass/retroperitoneal fat and adrenal mass/subcutaneous fat) of the adrenal masses.All pheochromocytomas had a T2 relaxation time greater than 82 ms with a maximum value of 134.3 ms. These values were calculated using T2 quantitative analysis methods based on in-house designed mathematical routines (T2-QMRI). The signal intensities of the tumor on T2-weighted spin-echo images were extremely high.As a conclusion of this preliminary report it is postulated that T2 quantitative MRI examination (T2-QMRI) is an accurate method for the diagnosis and characterization of pheochromocytomas because it gives positive diagnostic results whether the pheochromocytoma is secreting or nonfunctioning. It can also detect extra-adrenal pheochromocytomas (paragangliomas). Comparative studies with other adrenal tumors is essential to assess accuracy of this method for characterizing these tumors.