Undifferentiated sarcoma of the liver: a rare pediatric tumor

Undifferentiated embryonal sarcoma of the liver [UESL] is an uncommon hepatic tumor of mesenchymal origin recognized as a unique clinicopathologic entity since 1978. UESL has historically been considered an aggressive neoplasm with an unfavorable prognosis. Survival has improved using recent multimodal approaches, designed for patients with soft tissue sarcomas at other sites. Several small series have reported survival of up to 70% of children. We report a case of a 12-year-old boy from the United Arab Emirates who relapsed after complete surgical resection and was then successfully treated with re-resection followed by chemotherapy and radiotherapy. With a follow-up of 5 years, he is well and asymptomatic, and is leading a healthy life. This case emphasizes the fact that these poorly prognostic tumors may benefit from post-surgery chemotherapy. This case illustrates the improved survival of UESL patients following the multimodality therapy with a relatively long follow-up. This is the first case of UESL reported in this region of the world

Immunization following stem cell transplantation - A need for reappraisal

Haematopoietic stem cell transplantation [HSCT] is being used with increasing frequency as treatment option for various high risk malignant and non-malignant disorders. Myeloablative conditioning regimens given to eradicate malignant cell and to suppress the host immune system to avoid rejection results in dysfunctional immunity that persists for up to one year or longer. During this period, patients are at increased risk of infections representing major source of morbidity and mortality. The immune manipulation often required to manage graft rejection and graft versus host disease [GVHD] further delays immune reconstitution. This vulnerable period is also characterized by loss of immune memory to infectious agents and vaccines that the patients were exposed previously. Guidelines for immunization in post stem cell transplant patients is not clearly defined and there is no standard protocol exist for them moreover developments of new vaccines that utilize different immune mechanisms suggest that reappraisal of the immunization practices after HSCT is needed. So we think this appraisal will help making unified protocol for immunization in transplant patients

Familial haemophagocytic lymphohistiocytosis

Familial haemophagocytic lymphohistiocytosis [FHL] is a rare genetic disease, typically occurs during infancy and early childhood and is fatal in 1-2 months from presentation if not diagnosed and appropriately treated. The dramatic clinical presentation with multisystem involvement is described in our typical case. Early diagnosis and treatment offers this child a good chance of prolonged remission and possibly cure. As this condition is frequently not diagnosed, with dire consequences except we describe our case to increase awareness and improve the outcome for others

Congenital duodenal diaphragm in eight children

Congenital duodenal obstruction [CDO] is a common and usually easy to diagnose cause of intestinal obstruction in the newborn, except when the cause of the obstruction is a duodenal diaphragm. We describe our experience with eight children who had intrinsic duodenal obstruction secondary to a duodenal diaphragm. The medical records of 22 children with the diagnosis of congenital intrinsic duodenal obstruction were reviewed for age at diagnosis, sex, gestation, birth weight, clinical features, associated anomalies, method of diagnosis, treatment and outcome. Operative findings and procedures were obtained from the operative notes. Eight of the 22 children [36.4%] had congenital duodenal diaphragm [CDD]. In all children, the diagnosis was made from plain abdominal X-ray, which showed the classic double-bubble appearance, and barium meal, which showed duodenal obstruction. Four patients had associated anomalies, including two with Down's syndrome. Intraoperatively, five patients were found to have duodenal diaphragm with a central hole, while the other three had complete duodenal diaphragms. Postoperatively, all patients did well. Six required total parenteral nutrition. Conclusions: The 100% survival rate among these children is comparable to that in Western countries, and can be attributed to the lack of major associated abnormalities, good perioperative management, and the availability of total parenteral nutrition

Seroprevalence of hepatitis B, hepatitis C and HIV among multiply transfused thalassemic children treated at Tawam Hospital

The management of children with beta-thalassaemia major is based upon regular blood transfusions and prevention of iron overload. Blood transfusions, however, carry a definite risk of transmitting blood-borne viruses such as hepatitis B [HBV], hepatitis C [HCV] and human immunodeficiency virus [HIV]. This study was undertaken to determine the prevalence of HBV, HCV and HIV as well as the success rate of the immune response to HBV vaccine in 16 multiply-transfused children with beta-thalassemia major. None of our patients were hepatitis B surface antigen positive, and none were anti-HIV positive. Only 3 [18.75%] patients were anti-HCV positive. In spite of the administration of a full course of HBV vaccine to all patients, only 2 out of 16 patients responded. These patients, however, will require more booster doses of vaccine or a higher dose of vaccine

Neuroblastoma - the Tawam Hospital experience

Over the past two decades, there have been significant improvements in the survival of children with different types of cancer. Neuroblastoma is the exception, as in spite of many different kinds of chemotherapeutic regimens, the prognosis of advanced neuroblastoma remains unsatisfactory. This study is an analysis of our experience in the management of 53 children [25 males and 28 females] with neuroblastoma in this part of the world. Their ages at presentation ranged from birth to 10 years [mean 2.9 years]. The majority of our patients [83%] had intra-abdominal neuroblastoma. Five had intrathoracic neuroblastoma, 2 had intracranial neuroblastoma, 1 had oropharyngeal neuroblastoma, and one had metastatic neuroblastoma without a known primary. The distribution of our patients according to stage was as follows: 3 stage I, 5 stage II, 9 stage III, 33 stage IV and 3 stage IV-S. All our patients with stage I, II, III, and IV-S survived. A large number of our patients [62.3%] presented with advanced stage IV neuroblastoma, and this contributed to the high mortality rate [34%] in our series. The exact incidence of neuroblastoma in the United Arab Emirates [UAE] is not known, but it accounted for 7.5% of the total number of malignancies in the paediatric age group. The recently established cancer registry in the UAE should prove useful regarding the incidence and prevalence of cancer in the future

Cryptosporidium as a cause of diarrhoea

We herein describe 8 children [4 immunocompetent and 4 undergoing chemotherapy for acute lymphoblastic leukaemia] with severe diarrhoea and dehydration which was identified using modified Ziehl-Neelsen stain caused by Crytosporidium. The four immunocompetent children were treated with intravenous hydration only without specific antimicrobial therapy and they responded well. The other four immunocompromized children received intravenous hydration and antimicrobial chemotherapy. Two of them received paromomycin and responded well. One patient was started on paromomycin for 10 days, and although there was clinical improvement, his stool examination continued to be positive for Cryptosporidium. He then received azithromycin for 14 days to which he responded well and his stools became negative for Cryptosooridium. The fourth patient received azithromycin from the start and responded well. Cryptosporidium should be considered as a causative organism in children, especially those who are immunocompromized, and who present with severe or prolonged nonbloody diarrhoea. The organism is not seen in a routine 'ova and parasite' examination and the lab should be notified of its possibility in the differential diagnosis for diagnostic confirmation using modified Ziehl-Neelsen stain. Immunocompetent children with cryptosporidiosis will respond to intravenous hydration without specific antimicrobial therapy while immunocompromized children may benefit from paromomycin or azithromycin therapy. We wish to increase the awareness of this condition and its management in clinicians and microbiologists

Malignant hypertension with seizures as the initial presentation of a pelvic neuroblastoma

Malignant hypertension is an uncommon but well described cause of seizures in children. Immediate treatment is needed to prevent morbidity and mortality. We report a child with seizures as the initial presentation of a pelvic neuroblastoma

Outcome of children treated for cancer in Tawam Hospital, Al Ain, United Arab Emirates

The Paediatric Oncology Unit in Tawam Hospital was established in 1983 as the United Arab Emirates [UAE] referral center for childhood cancer. The activity, scope and success of the unit have increased over the years. To review the types of cancer seen and relative frequency, patient demographics, and the success rate for children treated here compared to other centres. A survey was undertaken of all cases of paediatric cancer during the 7-year period from January 1995 to December 2001