Extrapulmonary DICER1‐associated sarcomas (DS) can harbor morphological features overlapping with pleuropulmonary blastoma. We report three children with intracranial and genital tract sarcomas, suspected to have DS based on a heterogeneous yet defining combination of spindle‐cell sarcomatous and blastemal morphology, with rhabdomyomatous differentiation. Foci of immature cartilage at diagnosis (n = 2/3) and increased neuroepithelial differentiation at recurrence (n = 1) were noted. Morphological suspicion prompted somatic testing at reference centers, confirming likely biallelic, loss‐of‐function, and “hotspot” missense DICER1 variants in all three tumors. This can serve as a model for this diagnosis in resource‐limited settings and has implications for germline testing, surveillance, and tumor management.

Paediatric psycho-oncology is an evolving speciality and is increasingly being recognised as an essential component in children’s cancer care. Modern paediatric oncology services aspire to integrate physical care with psycho-social care and build capacity within clinical teams to address the emotional needs of parents and children side by side with other aspects of medical care. This article discusses the unique challenges of paediatric psycho-oncology and common situations where psychological assessment and management of children and young people with cancer become especially important. The authors propose a tiered structure of training. Providing empathic evidence-based psycho-social care is ‘everyone’s business’ in paediatric oncology and not merely that of mental health professionals. However, there are times when a more specialist intervention by a paediatric liaison psychiatrist and/or a clinical psychologist is needed for optimum outcome. Learning interviewing techniques suitable for children and adolescents should be a core part of the training in paediatric psycho-oncology. Professionals should be encouraged to reflect on their own emotional wellbeing, which in turn will provide a stable foundation of emotionally matured care to children, young people and their families.

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SMARCB1/integrase interactor (INI)-1 is one of the core subunit proteins of the ATP-dependent SWI/SNF chromatin remodeling complex and acts as a tumor suppressor. INI-1 loss can be easily assessed using immunohistochemistry and is an important diagnostic clue for a histopathologist. Chordoma is a malignant tumor commonly occurring in the sacrococcygeal spine of adults and is characterized by nuclear expression of brachyury. Poorly differentiated chordoma, a morphologically and molecularly distinct entity, also shows nuclear brachyury positivity along with INI-1 loss. It usually occurs in children and has a predilection to involve the base of the skull. We describe a case of a poorly differentiated chordoma in a 5-year-old girl and discuss its unusual histomorphologic and immunohistochemical features.

Metachronous primary distinct tumors are frequently and increasingly encountered in oncology clinical practice of recent times, but synchronous tumours are still a rarity. We report an unusual case of a 2 year old male child who had synchronous occurrence of rhabdomyosarcoma of pelvis and acute myeloid leukemia.Our search of literature suggests that this may be the first reported case of simultaneous occurrence of these two malignancies.