Alberto Berni

Premature Subclinical Atherosclerosis in Pediatric Inflammatory Bowel Disease

OBJECTIVES To investigate the risk for developing an early endothelial dysfunction based on increased intima media thickness (IMT) and reduced flow-mediated dilation (FMD) in children with inflammatory bowel disease (IBD), and to evaluate the role of traditional and nontraditional risk factors in determining premature atherosclerosis. STUDY DESIGN We studied 27 patients with Crohns disease (CD) and 25 patients with ulcerative colitis (UC) (mean age, 15.2 years; mean duration of disease, 48.05 months); 31 subjects served as controls. Demographic data (age, sex, family history of diabetes, cardiovascular disease, hypertension, hypercholesterolemia), traditional risk factors for atherosclerosis (blood pressure, body mass index, active and passive smoking, dyslipidemia), and UC and CD activity indexes (Pediatric Ulcerative Colitis Activity Index and Pediatric Crohns Disease Activity Index, respectively) were collected. The IMT of the carotid arteries was measured by high-resolution B-mode ultrasound, and endothelial function was evaluated by FMD in the brachial artery in response to reactive hyperemia. RESULTS Compared with controls, patients with CD had significantly greater exposure to passive smoking and had lower body mass index and high-density lipoprotein cholesterol values. IMT was significantly higher in patients than controls (P < .0001), and the percentage of FMD was significantly lower in both patients with CD (P < .0001) and patients with UC (P < .01) versus controls. In multivariate analysis, diagnosis of IBD was an independent risk factor for atherosclerosis. CONCLUSION Premature endothelial dysfunction occurs in pediatric IBD. This represents a new challenge in the management of pediatric IBD, leading to prevention strategies of cardiovascular disease.

Sclerosing Papillary Carcinoma Arising in a Lingual Thyroid: Report of a Case

Ectopic lingual thyroid tissue is an uncommon congenital anomaly. Tumors with identical pathological characteristics to those arising in thyroid tissue may be present in ectopic locations, but there are very few cases of malignant ectopic thyroid tumors reported in the literature. We present a review of this phenomenon and report a case of papillary carcinoma of the base of the tongue, located in ectopic lingual thyroid tissue, in a 30-year-old woman. The patient’s presenting symptoms were dysphagia and oral bleeding, and we performed radical resection of the neoformation at the base of the tongue with part of the muscles of the floor of the mouth and the body of the hyoid bone, as well as total thyroidectomy. Histological examination revealed a “sclerosing” papillary carcinoma. The patient was treated with 131I and substitutive thyroid hormonal therapy. An ultrasonogram done 5 years later showed bilateral laterocervical lymph node recurrence, which was effectively treated with bilateral laterocervical lymphectomy.

Retroperitoneal liposarcoma associated with small plaque parapsoriasis

BackgroundExtremely rare cases of paraneoplastic syndromes or ectopic production of proteins associated with liposarcoma are reported in literature. Production of Granulocyte-Colony Stimulating Factor, alpha-fetoprotein, paraneoplastic pemphigus and leucocytosis, Acrokeratosis paraneoplastica (Bazexs syndrome) are reported.The present report describes a case of retroperitoneal liposarcoma associated with small plaque parapsoriasis. Our search in the English literature of such a kind of association did not reveal any case reported.Case presentationA 74 year male patient was admitted to our hospital because of the presence of an abdominal mass in right iliac fossa. He also complained of a two-year history of psoriasiform eruptions. The CT scan showed a retroperitoneal pelvic mass. Therefore surgical resection of the tumor was performed. After surgery, the skin eruptions disappeared completely in seven days and so a diagnosis of parapsoriasis syndrome was done.ConclusionParallel disappearing of skin eruptions after surgery, typical clinical picture and not specific histology of the cutaneous lesions suggest the diagnosis of small plaque parapsoriasis. Therefore we propose to add Small Plaque Parapsoriasis to the list of paraneoplastic syndromes associated to liposarcoma.

Selective Embolization of Thyroid Arteries for Preresection or Palliative Treatment of Large Cervicomediastinal Goiters

Background: The authors have applied the selective embolization of thyroid arteries in the treatment of voluminous cervicomediastinal goiters, especially in patients at high surgical risk or reluctant to undergo surgical intervention and radioiodine therapy. Method: Selective arteriography was used to embolize the thyroid arteries in 2 patients with voluminous hyperfunctioning cervicomediastinal goiters and mediastinal compressive symptoms. The first patient had already undergone unsuccessful radioiodine metabolic therapy and had severe left ventricular insufficiency contraindicating surgery. The second patient, despite having no contraindications, declined surgery and radioiodine metabolic therapy. Results: Radiological embolization markedly reduced the goiters in volume, resolved the compressive symptoms, and also normalized thyroid hyperfunction. The second patient needed a second embolization procedure because the embolized arterial branches had partly recanalized. Conclusion: Selective embolization of thyroid arteries can be successfully used to treat selected patients as a preoperative procedure and as an alternative to thyroid resection. Embolization can be repeated to achieve the required therapeutic aims.

Peripheral primitive neuroectodermal tumor (PPNET) of pelvic origin: report of a case arising from an unusual location.

We report a rare case of a peripheral primitive neuroectodermal tumor (PPNET) originating from the left ileopsoas muscle in an adult patient with neoplastic thrombosis of the left external iliac vein, the common femoral vein and the left popliteal vein. We performed a median laparotomy with an oblique left inguinal incision to remove the neoplasm, which consisted of a large mass surrounding the iliac-psoas muscles, extending from the transverse apophysis of the spinal column to Scarpas triangle, and passing through the lacuna musculorum. Histopathological examination revealed a primitive neuroectodermal tumor (PNET) with focal areas of necrosis, hemorrhage and vascular invasion. Immunophenotyping was positive for CD99, NSE, and focally for CK. Ultrastructural examination of the neoplastic cells showed often multiple nuclei with dense chromatin and very large nucleoli. The patient was discharged ten days after the operation. Adjuvant treatment consisted of radiotherapy at a dose of 2000 cGy in five fractions followed by six cycles of chemotherapy. The venous thrombosis was treated by anticoagulant therapy and recanalization of the occluded veins was obtained after two months of therapy. An MRI scan, carried out 12 months later, showed a local relapse, which was treated with chemotherapy and arterial chemoembolization.A peripheral primitive neuroectodermal tumor arising from the and prostate gland) and urinary and fecal diversion. Adjuvant abdominopelvic cavity is reported in a 24-year-old young male chemotherapy (VAIA) was delivered once the histology was without any previous remarkable pathology. He was admitted confirmed. We reviewed the available literature focusing on to our hospital with complaints of urinary symptoms (acute the varied nomenclature of this tumor (peripheral neuroepitheurinary retention) and mild intestinal occlusion that had been lioma, Askins tumor, Ewings extraosseous tumor, peripheral present for three months. Physical examination and CT scan adult neuroblastoma, peripheral primitive extracranial neurevealed a pelvic mass occupying the entire pelvic cavity. The roectodermal tumor (PPNET), the clinical features, the role of diagnostic workup included a CT-guided biopsy which defined diagnostic imaging techniques, pathologic assessment and the tumor as a sarcomatous type. Radical surgery was perforcontroversial therapeutic management. In addition, the promed including tumor resection, pelvic exenteration (bladder gnosis and survival of this rare condition were analyzed.