Benign and malignant vascular tumors of the liver in adults

Vascular tumors of the liver in adult patients include cavernous hemangioma, a common benign tumor; epithelioid hemangioendothelioma, a rare, usually low-grade malignant tumor; and angiosarcoma, a rare and very aggressive tumor. All these primary mesenchymal tumors develop on a normal liver and may also affect other organs. Their pathogenesis remains largely unknown. Hepatic tumors are increasingly detected incidentally due to widespread use of modern abdominal imaging techniques. Therefore, reliable noninvasive characterization and differentiation of such liver tumors is of major importance for clinical practice. Hemangioma follows a benign course, and a nonoperative approach for the majority of these lesions is recommended. A definitive diagnosis of epithelioid hemangioendothelioma and angiosarcoma requires histopathologic examination. Liver transplantation at an early stage has greatly improved the prognosis of epithelioid hemangioendothelioma. The prognosis of angiosarcoma remains dismal. Designing a worldwide database that contains all data about patients with these rare diseases is recommended.     

The value of serologic markers in indeterminate colitis: a prospective follow-up study

BACKGROUND & AIMS: In the absence of pathognomonic markers for Crohn's disease (CD) and ulcerative colitis (UC), the diagnosis of inflammatory bowel disease depends on a compendium of clinical, radiographic, endoscopic, and histologic criteria that bears imperfect specificity to the individual disorders. In 10% of cases of colitis, no differentiation can be made between CD and UC; these patients are diagnosed with indeterminate colitis (IC). We evaluated the value of anti-Saccharomyces cerevisiae antibodies (ASCA) and perinuclear antineutrophil cytoplasmic antibodies (pANCA) to increase diagnostic accuracy in categorizing IC. METHODS: Since 1996, 97 patients with IC from 3 centers (Leuven, Lille, and Vienna) were enrolled, analyzed for pANCA and ASCA, and followed up prospectively. RESULTS: A definitive diagnosis has been reached for 31 of 97 patients (32%). In these patients, ASCA+/pANCA- correlated with CD in 8 of 10 patients, whereas ASCA-/pANCA+ correlated with UC in 7 of 11 patients. The remaining 4 cases became CD, clinically behaving as UC-like CD. Almost half of the patients (47 of 97 [48.5%]) were negative for ASCA and pANCA, and 40 remain diagnosed with IC to date. Only 7 seronegative cases (14.9%) became CD or UC compared with 48% (24 of 50) of seropositive patients (P < 0.001). CONCLUSIONS: Results so far show that ASCA+/pANCA- predicts CD in 80% of patients with IC and ASCA-/pANCA+ predicts UC in 63.6%. Interestingly, 48.5% of patients do not show antibodies against ASCA or pANCA. Most of these patients remain diagnosed with IC during their further clinical course, perhaps reflecting a distinct clinicoserological entity.     

t(3;17)(q21;q25) in Epstein-Barr virus associated peripheral T-cell lymphoma: a paediatric case

We describe an immunocompetent 8-year-old boy with a serological profile indicating chronic infection by Epstein-Barr virus (EBV) who developed subcutaneous and pulmonary lesions related to a peripheral T-cell proliferation. No clonal rearrangement of T-cell receptor or immunoglobulin genes was seen. However, the finding of a t(3;17)(q21;q25) in 44 metaphases from one skin lesion demonstrated a clonal origin. We also showed that the proliferative T cells contained EBV genome leading to the diagnosis of EBV-associated peripheral T-cell lymphoma. Further cytogenetic and molecular studies are needed to identify genes implicated in the pathogenesis of this haematological malignancy.     

Home unattended vs hospital telemonitored polysomnography in suspected obstructive sleep apnea syndrome: a randomized crossover trial

STUDY OBJECTIVE: To compare home unattended polysomnography (H-PSG) with polysomnography performed in a local hospital and telemonitored by a sleep laboratory (T-PSG) in the diagnosis of obstructive sleep apnea syndrome (OSAS). DESIGN: Randomized crossover trial. PATIENTS: Ninety-nine patients with suspected OSAS who underwent H-PSG and T-PSG on 2 consecutive nights, according to a randomized order. MEASUREMENTS: H-PSG and T-PSG were compared in terms of (1) effectiveness, only recordings providing interpretable signals from at least one EEG, the electro-oculograph, the electromyograph, air flow, thoracic or abdominal movements, and arterial oxygen saturation for 180 min of sleep were considered to be effective; (2) patient preference assessed by a questionnaire; and (3) polysomnographic indexes and final interpretative results in patients for whom both recordings were legible. RESULTS: Recordings were considered to be ineffective in 11.2% of T-PSG (95% confidence interval [CI], 4.9 to 17.4%) and in 23.4% of H-PSG (95% CI, 19.12 to 27.68%). Thermistor problems were the main cause of failure of H-PSG. Forty-one percent of patients preferred H-PSG, and 55% preferred T-PSG. H-PSG and T-PSG did not differ in terms of sleep and respiratory indexes in the 65 patients in whom both recordings were legible. H-PSG and T-PSG were concordant in 58 of 65 patients using a 10-event-per-hour apnea-hypopnea index cutoff value for the diagnosis of OSAS. CONCLUSIONS: T-PSG is clearly superior to H-PSG from a technical point of view and tends to be preferred by patients. The site of recording (home vs hospital) has no influence on polysomnographic indexes.     

Post-Traumatic Pseudoaneurysm of the Left Sinus of Valsalva Revealed by an Acute Coronary Compression: An Echocardiographic Case Report

A 69-year-old woman presented with chest pain and electrocardiographic evidence of subacute anterior ischaemia. Transthoracic and transesophageal echocardiography allowed correct diagnosis of a posttraumatic pseudoaneurysm of the left sinus of Valsalva. The large pseudoaneurysm caused compression of the left main coronary artery and was successfully treated by aortic root replacement, left main coronary artery ligation, and arterial bypass.     

Lymphocytic hypophysitis with lachrymal, salivary and thyroid gland involvement

We report on hypophysitis associated with a prominent lymphoid infiltration of salivary and lachrymal glands in a 35-year-old woman with a dramatic response to steroids. Four years later, overt Graves' disease developed. To our knowledge, pseudotumoral lymphocytic infiltration of both lachrymal and salivary glands has never been described in association with hypophysitis. Benign lymphocytic hypophysitis may belong to a spectrum that extends from low-grade lymphoid proliferation to autoimmune disease. Such a process may follow a regional tissue distribution including pituitary, thyroid, lachrymal and salivary glands.