✓Spontaneous intracerebral hemorrhage in children is usually related to cerebrovascular conditions. Brain tumors presenting with spontaneous bleeding account for approximately 10% of intracranial hemorrhages in children. The occurrence of primitive central nervous system lesions in the Ewing sarcoma family of tumors (ESFT) not related to bone or metastatic disease is a rare condition. The authors report on a child who presented with intracranial bleeding secondary to a nonmetastatic tentorial ESFT confirmed by detection of the fusion gene EWS-ERG. A detailed review of the literature reveals that most primary intracranial ESFT had a meningeal attachment, and that almost half of them presented at diagnosis with hemorrhage. Distinguishing between ESFT and other intracranial neoplasms is essential because the treatment and prognosis differ remarkably from that of other tumors, namely central primitive neuroectodermal tumors (PNETs). Whereas adjuvant treatment for ESFT consists of local or regional radiotherapy and chemotherapy containing alkylating agents, central PNETs are generally treated with whole neuraxis radiation and platinum-based chemotherapy. Additionally, the prognosis for intracranial ESFT might be better than the one for nonpineal central PNETs.
Case report and review of the literature
Ramon Navarro, Astrid Laguna, Carmen de Torres, Juan Cruz Cigudosa, Mariona Suñol, Ofelia Cruz and Jaume Mora
Maria-Jesus Lobon-Iglesias, Vicente Santa-Maria Lopez, Patricia Puerta Roldan, Santiago Candela-Cantó, Monica Ramos-Albiac, Marta Gomez-Chiari, Stephanie Puget, Stephanie Bolle, Liliana Goumnerova, Mark W. Kieran, Ofelia Cruz, Jacques Grill and Andres Morales La Madrid
Diffuse intrinsic pontine glioma (DIPG) is a highly aggressive and lethal brainstem tumor in children. In the 1980s, routine biopsy at presentation was abandoned since it was claimed “unnecessary” for diagnosis. In the last decade, however, several groups have reincorporated this procedure as standard of care or in the context of clinical trials. Expert neurosurgical teams report no mortality and acceptable morbidity, and no relevant complications have been previously described. The aim of this study was to review needle tract dissemination as a potential complication in DIPG.
The authors retrospectively analyzed the incidence of dissemination through surgical tracts in DIPG patients who underwent biopsy procedures at diagnosis in 3 dedicated centers. Clinical records and images as well as radiation dosimetry from diagnosis to relapse were reviewed.
Four patients (2 boys and 2 girls, age range 6–12 years) had surgical tract dissemination: in 3 cases in the needle tract and in 1 case in the Ommaya catheter tract. The median time from biopsy to identification of dissemination was 5 months (range 4–6 months). The median overall survival was 11 months (range 7–12 months). Disseminated lesions were in the marginal radiotherapy field (n = 2), out of the field (n = 1), and in the radiotherapy field (n = 1).
Although surgical tract dissemination in DIPG is a rare complication (associated with 2.4% of procedures in this study), it should be mentioned to patients and family when procedures involving a surgical tract are proposed. The inclusion of the needle tract in the radiotherapy field may have only limited benefit. Future studies are warranted to explore the benefit of larger radiotherapy fields in patients with DIPG.