RESUMEN

Superior mediastinal syndrome secondary to an anterior mediastinal mass can be seen in acute lymphoblastic leukaemia (ALL) of T-cell lineage. We report a 3-year-old child with B-cell ALL, who presented with the superior mediastinal syndrome. The CT scan chest showed a huge anterior mediastinal mass and the peripheral blood immunophenotyping showed B-cell ALL. High-risk remission induction chemotherapy was given and he achieved remission by the end of induction therapy, both in terms of medullary and extramedullary disease, and is on maintenance chemotherapy now. This is the first reported case of a paediatric B-cell ALL presenting with superior mediastinal syndrome secondary to an anterior mediastinal mass.

RESUMEN

Superior mediastinal syndrome (SMS) is a relatively common emergency in the practice of Pediatric Oncology. It typically results from the compression of large airways and superior vena cava by a swiftly growing mass. T-lineage acute lymphoblastic leukemia or lymphoma, neuroblastoma, and germ cell tumor are the common etiologies of SMS in children. Occasionally, SMS can be an unexpected presentation of less common childhood cancers and a surprise for the diagnostic and treating teams. The present paper reports the diagnostic and therapeutic challenge of managing a 9-y-old boy with SMS resulting from mediastinal myeloid sarcoma. The presence of a sizeable intracardiac thrombus, in addition, contributed to the SMS. The initial pleural fluid cytology and image-guided fine-needle aspiration cytology of the mediastinal mass were nondiagnostic. A thoracotomy was subsequently performed to debulk the tumor for symptomatic relief and obtain tissue for diagnosis.

Asunto(s)

Neoplasias del Mediastino , Sarcoma Mieloide , Síndrome de la Vena Cava Superior , Trombosis , Niño , Humanos , Masculino , Neoplasias del Mediastino/complicaciones , Neoplasias del Mediastino/diagnóstico , Neoplasias del Mediastino/cirugía , Sarcoma Mieloide/complicaciones , Sarcoma Mieloide/diagnóstico , Sarcoma Mieloide/cirugía , Síndrome de la Vena Cava Superior/diagnóstico , Síndrome de la Vena Cava Superior/etiología , Síndrome de la Vena Cava Superior/patología , Trombosis/diagnóstico por imagen , Trombosis/etiología , Trombosis/cirugía , Vena Cava Superior

RESUMEN

An oncological emergency may be the initial presentation of a cancer, a sign of cancer progression, or a complication of cancer treatment. The most frequently encountered paediatric oncological emergencies include neutropenic sepsis, hyperleukocytosis, brain tumours presenting with raised intracranial pressure, tumour lysis syndrome and superior mediastinal syndrome. These are all life-threatening conditions that require urgent recognition and management. Health professionals working in an emergency department (ED) are likely to be involved in managing these children. This article brings together the current guidance and recommendations for these specific emergencies. It also includes two case studies that demonstrate the challenges health professionals can face while managing these situations. It is important that health professionals have an acute awareness of oncological emergencies. Confidence in recognising the presentations, diagnoses and initial management are essential because these conditions may be life-threatening and time critical.

Asunto(s)

Neoplasias , Atención de Enfermería , Sepsis , Niño , Urgencias Médicas , Humanos , Oncología Médica , Neoplasias/complicaciones , Neoplasias/terapia

RESUMEN

BACKGROUND: With advancements in the field of oncology, cancer survival rates have improved dramatically but modern cancer treatments also come with an increasing number of disease and treatment-associated complications. This article provides an updated narrative review on the pathophysiology, clinical presentations and latest management strategies for common paediatric oncological emergencies. METHODS: An extensive PubMed® search of all human studies in the English literature was performed in Clinical Queries for different oncology syndromes and conditions using the following Medical Subject Headings: "tumour lysis syndrome", "hyperleukocytosis", "disseminated intravascular coagulation", "superior mediastinal syndrome", "superior vena cava syndrome", "sepsis", "severe inflammatory response syndrome", "acute respiratory distress syndrome", "posterior reversible encephalopathy syndrome" and "reversible posterior leukoencephalopathy syndrome". Categories were limited to clinical trials and reviews for ages from birth to 18 years. RESULTS: The general description, presentation and management of these oncologic emergencies are systematically described. Early recognition along with prompt and proactive treatment can reduce the chances of potential complications and improve the clinical outcomes, thereby improving not only survival rates in oncology patients but also their clinical outcomes and quality of life. CONCLUSIONS: Oncologic emergencies are associated with significant mortality and morbidity. Healthcare professionals involved with the care of oncology patients must be vigilant of these emergencies.

RESUMEN

Superior vena cava syndrome (SVCS) is rare in childhood. 18 cases of SVCS were seen in children ranging from 3-14 years with a mean age of 8.8 years. There were 15 males and 3 female children. Diagnosis could be confirmed in 17 cases as one child succumbed to severe respiratory distress without a definitive diagnosis. The commonest cause of SVCS was lymphoma. Non-Hodgkin's lymphoma (NHL) was more common than Hodgkin's disease. In two cases the final diagnosis was tuberculosis of mediastinal lymph nodes. The diagnosis was confirmed by cervical lymph node biopsy in 6 cases, mediastinal biopsy in 6 cases and bone marrow aspiration in the remaining 5 cases. Intravenous Dexamethasone provided relief of symptoms in 13 patients. None of the children received emergency radiotherapy. Anti-tubercular treatment produced complete cure in the two patients with tubercular mediastinal lymphadenopathy.