A term infant with Crouzon syndrome underwent a computed tomography (CT) study to rule out the presence of hydrocephalus. Computed tomography revealed hydrocephalus, a large cerebrospinal fluid cyst in the posterior fossa and hypoplastic cerebellar hemispheres with a winged appearance, consisting of Dandy-Walker malformation (Figure 1). The patient underwent a cystoperitoneal shunt insertion to alleviate pressure, and a craniofacial reconstructive procedure was performed at a later time.Crouzon syndrome is a genetic disorder characterized by cranial synostosis, exophthalmos, and midface hypoplasia. The birth prevalence has been calculated to be 16.5 cases per 1 000 000 live births and constitutes approximately 4.8% of all cases of craniosynostosis. 1 Patients with Crouzon syndrome often develop hydrocephalus that requires treatment. Dandy-Walker malformation is a congenital disorder that is characterized by cerebellar dysgenesis, cystic dilation of the fourth ventricle, and large posterior fossa with elevation of the dural sinuses and tentorium. 2 Although Dandy-Walker malformation is usually associated with several other disorders, to our best knowledge this is the first reported case of a patient with Crouzon syndrome. Physicians should be aware of this potential coexistence, given that both disorders have been associated with mental retardation and may act synergistically.

Ewing's sarcoma is a highly malignant neoplasm of bones which accounts for the 10% of primary bone malignancies. Primary Ewing's sarcoma of skull vault is very rare and constitutes 1-6% of all Ewing's sarcomas. We present a case of a primary and a radiation-induced skull Ewing's sarcoma. The symptoms, neuroimaging findings and the treatment for these cases are reviewed. Both children were operated with favorable outcome.

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Background/Aim: Undifferentiated round cell sarcomas are a heterogeneous group of sarcomas. Identification of BCOR alterations, such as BCOR/CCNB3 and BCOR/MAML3 fusion genes and BCOR ITD has recently contributed in the precise diagnosis of these neoplasms, defining a new entity of the current classification of soft tissue and bone sarcomas. BCOR sarcomas share both morphological and genetic characteristics distinct from Ewing sarcomas. The scope of our study was to retrospectively identify BCOR sarcomas and find the correlations with the clinical outcome of these patients. Patients and Methods: Histopathology and immunohistochemistry of pediatric tumor samples were combined with molecular testing (PCR) and fluorescent in situ hybridization to find BCOR sarcomas. Results: We, herein, present our experience with BCOR sarcomas in a referral center of Greece. Moreover, we report in one case the detection of a variant BCOR/CCNB3 fusion not previously described. Conclusion: We are the first to report a splice variant of BCOR/CCNB3 which reveals the central position of BCOR in the oncogenesis of these tumors, furthermore we highlight the importance of molecular diagnostics in Ewing-like sarcomas and discuss the current treatment options for this rare entity.