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

Congenital epulis

Epulis is a rare tumour, with female preponderance that is only seen in the newborns. It arises from the mucosa of the gingiva and protrudes out of the infant's mouth. It can potentially obstruct the airways and may require an EXIT [ex-utero intrapartum treatment] procedure which involves establishing an airway before the feto-maternal circulation is interrupted. We present a female newborn with such a mass, which was diagnosed antenatally. A multidisciplinary team including the neonatologist, anaesthesiologist and ENT specialist should be present in the delivery room to establish the airways, which may require an EXIT procedure. Recommended treatment is early surgical resection. Recurrences of the tumour and damage to future dentition have not been reported, suggesting that radical excision is not warranted

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