OC-07 - Decoding risk for thromboembolic events in lymphoma patients.

INTRODUCTION: There are few prediction tools for estimating the risk of thrombosis but they are based on studies performed on hospitalized medical patients without cancer or on hospitalized neutropenic cancer patients without special consideration to lymphoma patients. AIM: Aim of our study was to determine incidence of thromboembolic (TE) events in patients with non Hodgkin lymphoma (NHL), Hodgkin lymphoma (HL) and chronic lymphocytic leukemia (CLL)/ small lymphocytic lymphoma (SLL) who were hospitalized to the lymphoma department in the Clinic of hematology, Clinical Center Serbia, Belgrade and Clinic of hematology, Clinical Center Kragujevac. Also, we assessed 2 predictive models (Padua and Khorana score) and create new model for the identification of lymphoma patients at risk for thromboembolism. MATERIALS AND METHODS: We reviewed all medical records of patients with with NHL, HL and CLL/SLL diagnosed and treated at two previously mentioned institution between January 2006 and December 2014. RESULTS: The study population included 1820 eligible lymphoma patients. Of all the patients included in the study, 99 (5.4%) developed at least one TE during a follow-up period of 3 months from the end of therapy. In the final multivariate analysis, the following variables were independently associated with risk of TE: previous VTE and/or arterial events, reduced mobility (ECOG 2-4), obesity (BMI >30 kg/m(2)), extranodal localization, mediastinum involvement, development of neutropenia during therapy and hemoglobin level less than 100g/L. Subsequently, we assigned points for the risk model based on the regression coefficients obtained from the final model and developed Thrombosis Lymphoma (ThroLy) score consisting of all significant variables from the multivariate analysis. The Throly score was arrived at by assigning 2 points for all parameters with an OR >5 in multivariate regression analyses (e.g., previous VTE and arterial events, mediastinum involvement, and BMI) and 1 point for rest all other significant variables. Finally, population were divided into 3 risk categories for TE based on the score from the risk model: low (score 0-1), intermediate (score 2-3) and high (score >3). High risk score had a positive predictive value (probability of TE in those designated high risk) of 65.2%. CONCLUSIONS: Significance of our investigation is development of score that help phisicians to recruit lymphoma patients at risk for development of thromboembolic complications. Also, we can say that our score is dynamic allowing us to change approach during different phase of therapy and is not limited to outpatient settings or with some complicated laboratory analysis.

[Cystic mesenchimal hamartoma of the liver in two-year-old patient--case report].

Mesenchymal hamartoma is an uncommon benign hepatic tumor arising from the mesenchyme of the portal triad. This lesion is relatively uncommon, representing 5% of all primary hepatic pediatric tumors. This form of hamartoma usually presents before the age of 2 years, typically with abdominal swelling as the initial symptom. The classic management of these lesions has been excision either by hepatic lobectomy or wedge resection. We present a case of 2-year-old girl with a right hepatic lobe tumor, 66 x 57 x 71 in diameter that was completely removed by right hepatic lobectomy.

Intravascular large B-cell lymphoma of central nervous system - a report of two cases and literature review.

Intravascular large B-cell lymphoma (IVL) is a rare form of diffuse large B cell lymphoma (DBCL) frequently presenting with skin and/or central nervous system (CNS) involvement. IVL involves CNS in 75 - 85% of patients and neurological symptoms include sensory and motor deficits or neuropathies, meningoradiculitis, paresthesia, hypostenia, aphasia, dysarthria, hemiparesis, seizures, transient visual loss, vertigo and impaired cognitive function. Neuroimaging discloses CNS involvement only in half of patients with neurological symptoms because there are no pathognomonic neuroradiological findings for IVL; ischemic foci are the most common presentation pattern and therefore vasculitis is the most common differential diagnosis. According to all mentioned data, diagnosis of CNS IVL requires a histopathological confirmation. Brain biopsy is absolutely indicated in patients with progressive neurological deterioration with unclear abnormalities in cerebral MR imaging. A general policy is that patients with IVL should be considered to have disseminated disease and should be treated with systemic chemotherapy. In younger patients with unfavorable features the high-dose chemotherapy with autologous stem cell transplantation should be used. Nevertheless, the course of IVL is rapidly progressive and ultimately fatal.

[Successful treatment of second hepatoblastoma relapse--case report].

INTRODUCTION: The prognosis of hepatoblastoma has changed since effective adjuvant chemotherapy had been introduced in 1980's. There is a general agreement that complete resection is the cornerstone of treatment for children with hepapatoblastoma and the only way for eventual cure. CASE REPORT: We describe a boy with relapsed hepatoblastoma presenting with elevated -fetoprotein (AFP) and no visible tumor by ultrasound and computed tomography (CT). The relapse was treated with chemotherapy. Second relapse occurred shortly after therapy was completed, but this time we waited for tumor mass to appear. Combined surgery and chemotherapy resulted in remission status with 48 months of follow up. CONCLUSION: Hepatoblastoma relapse without evidence of tumor is not unusual but its treatment remains controversial. Radiological investigations should be repeated until site of relapse is identified. Based on our experience it seems of no benefit to treat isolated elevation of AFP.

Gastroesophageal reflux in infants and children.

Gastroesophageal reflux disease (GERD) is the most common esophageal disorder and the most frequent reason why infants are referred to the pediatric gastroenterologist, affecting as much as 30% of the pediatric population. Presenting features of GERD in infants and children are quite variable and follow patterns of gastrointestinal and extra-esophageal manifestations that vary between individual patients and may change according to age. Patients may be minimally symptomatic, or may exhibit severe esophagitis, bleeding, nutritional failure, or severe respiratory problems. GERD is also complex for the diagnostic techniques required to assess its repercussions or explain its origin. Although different abnormalities in motility variables, such as lower eso-phageal sphincter (LES) function, esophageal peristalsis and gastric motor activity can contribute to the development of GERD, the degree of esophageal acid exposure represents the key factor in its pathogenesis. Esophageal pH monitoring, based on both the detection of acid reflux episodes and the measurement of their frequency and duration, has been regarded as the most sensitive and specific diagnostic tool for diagnosing reflux disease. The aim of this paper is to give a concise review for the clinicians encountering this specific disease in infants and children.

Surgical treatment of the Müllerian duct remnants.

BACKGROUND: Persistent Müllerian duct tissue in male individuals may result in an enlarged prostatic utricle (utricular cysts and utricle) or a Müllerian duct cysts, either distinctively or synonymously. In intersex patients Müllerian duct remnants (MDR) are an usual occurrence. Surgical excision is the definitive treatment of symptomatic remnants, as well as during the reconstruction of intersexual genitalia. Many approaches have been described. The authors review their experience in intersex patients. METHODS: From 1986 to 1999, the authors treated 111 patients with intersex disorders. The records of 47 patients raised as boys with MDR were reviewed. Based on the symptoms and the size of the remnants, in 32 patients the structures were removed. In 13 patients extirpation was done by perineal approach, in 9 by transperitoneal approach, and in the remaining 9 patients the combined abdominal and perineal approach were undertaken. In one patient the large prostatic utricle was extirpated by a posterior sagittal pararectal approach. Perineal approach was mainly used in younger asymptomatic children, with the prostatic utricle disclosed incidentally during genitography because of intersex disorders. Operation was performed only in cases in which the prostatic utricle was observed by cystoscopy or identified by Fogarty balloon catheter introduction into the prostatic utricle. In older patients these structures were discovered frequently after failed urethroplasty, or after symptoms of urinary infection, urinary retention, or epididymitis. We elected to use the transperitoneal approach based on the extension of these structures into the pelvis. The average age of patients at the time of surgery was 8.6 years, with a range of 1 to 30 years. RESULTS: There were no rectal or bladder injuries during surgery. An older patient had temporary impotence after abdomino-perineal extirpation. The lack of ejaculation, seen in 5 patients, was related to frequent intra-utricular termination of the vas deferens. Posterior sagittal pararectal approach certainly enabled complete exposure and exact visualization of all structures, with considerably decreased bleeding. If gonadal biopsy or gonadectomy were necessary, the transperitoneal approach could not be avoided. CONCLUSIONS: Surgical treatment of MDR in intersex patients varies according to the size of the utricle, and a double approach is often necessary. A high degree of success may be achieved with minimal morbidity. J Pediatr Surg 36:870-876.

[Pancreatoblastoma in a child (case report)]. / Pankreatoblastoma u dece (prikaz slucaja).

This paper presents a rare form of pancreatic malignancy in childhood, pancreatoblastoma, tumor rarely encountered in children and adolescents. This tumor is also very interesting because of differential diagnosis toward other retroperitoneal tumors in children. They are most frequently diagnosed accidentally during clinical or ultrasonographic examination. We are presenting a case of 14 year old girl in whom a tumor was found during the investigation for the dull pain below the left rib margin. Tumor was successfully removed surgically. A 26 months follow-up was without any signs of recurrence of the primary tumor.