Fifteen-minute consultation: A structured approach to the child with palpable cervical lymph nodes.

Palpable cervical lymph nodes are common in children and are a frequent reason for presentation to both primary and secondary care. Enlarged lymph nodes are most commonly the result of self-limiting infection, and in children, are rarely the first indicator of a malignant process. This article presents an evidenced-based approach to evaluating these patients.

Fifteen-minute consultation: Initial management of suspected acute leukaemia by non-specialists.

Leukaemia is the most common cancer of childhood. Most children with a new diagnosis of leukaemia are clinically stable at initial presentation. However, there are a number of life-threatening complications that have to be considered and monitored for. These complications include sepsis, tumour lysis syndrome, mediastinal masses, bleeding and pain. The aim of this article is to equip the general paediatrician with a framework for managing children with suspected leukaemia, prior to transfer to the primary treatment centre. The presentation, diagnosis and definitive treatment of acute leukaemia is not in the remit of this article.

Lingual Alveolar Soft Part Sarcoma in a 1-Year-Old Infant: Youngest Reported Case With Characteristic ASPSCR1-TFE3 Fusion.

Alveolar soft part sarcoma (ASPS) is an exceptionally rare non-rhabdomyosarcomatous soft tissue sarcoma (NRSTS), characterized by the translocation t(X;17) p(11.2;q25). This translocation results in the chimeric ASPSCR1-TFE3 transcription factor which drives tumorigenesis. Complete surgical resection is crucial in allowing a successful outcome in these cases. Here, we describe an 11-month-old female infant who presented with a well-circumscribed lesion of the tongue, with the clinical and radiologic appearances of an infantile hemangioma. This led to an initial plan for surveillance management. However, the mass continued to enlarge and the lesion was therefore biopsied when the infant was 17 months old. Histology showed plump epithelioid tumor cells, in many places lining pseudoalveolar spaces. Occasional Pas-D inclusions were present in the cytoplasm. Immunostaining showed nuclear positivity for TFE-3. Real-time quantitative polymerase chain reaction testing confirmed the presence of ASPSCR1-TFE3 fusion transcripts, characteristic of the translocation t(X;17) p(11.2;q25) observed in ASPS. This represents the youngest reported ASPS case with a confirmed molecular diagnosis. Complete surgical resection was undertaken and a surveillance imaging schedule implemented. This case highlights the need for regular review of the initial diagnosis and the importance of multidisciplinary discussion and early biopsy where the clinical course does not follow that expected for the putative (nonhistologically confirmed) diagnosis.

Delphi method to identify expert opinion to support children's cancer referral guidelines.

BACKGROUND: The National Institute for Health and Care Excellence (NICE) guidance for referral of children with suspected cancer was first published in 2005 and updated in 2015. The updated version relied on sparse primary care evidence and published without input from key stakeholders, for example, acute general paediatricians and paediatric haematologists/oncologists. This led to a document that fell short as a practical guide for referring physicians managing children with potentially life-threatening conditions. Following discussions between the Children's Cancer and Leukaemia Group (CCLG, the UK multidisciplinary professional body for healthcare professionals caring for children with cancer) and NICE, it was agreed that a practical supplement should be produced for the 2015 guidance. A prerequisite was evidence gathering from tertiary care to balance the existing primary care evidence, and a Delphi consensus method was therefore convened. METHODS: A CCLG NICE Guidance Committee formulated 25 draft statements for review. The CCLG emailed its paediatric haematologist/oncologist membership (n=179) and 88 responded (49%). To achieve consensus, statements required ≥70% agreement from ≥60% of actual respondents, from the denominator (n=88). RESULTS: Fifteen of 25 original statements were accepted at the first round of voting. Three of 25 statements where >50% did not support were rejected outright. One statement could not be revised without replicating a previously accepted statement. The six remaining statements were revised and a second round of voting undertaken; all six revised statements were accepted. Overall, 21 of 25 statements (84%) met consensus criteria. CONCLUSIONS: This expert opinion should help streamline suspected cancer referral in children and help optimise subsequent outcomes.