Neha Patel

Outcome of young children with high-grade glioma treated with irradiation-avoiding intensive chemotherapy regimens: Final report of the Head Start II and III trials.

To report the final analysis of survival outcomes for children with newly diagnosed high‐grade glioma (HGG) treated on the “Head Start” (HS) II and III protocols with chemotherapy and intent to avoid irradiation in children <6 years old.

Extensive sinovenous thrombosis and hemorrhagic infarction during therapy for T-cell acute lymphoblastic leukemia.

Abstract Sinovenous thrombosis (SVT) is a well-recognized and serious complication in children treated for acute leukemia. This frequently occurs during or immediately upon completion of induction therapy and is commonly attributed to asparaginase therapy. Headache is the first and most common clinical symptom to occur during the early development of SVT. With advancement of the thrombosis, the clinical symptoms can progress to increased sleepiness, focal neurological deficit, seizures, and altered consciousness. We report the case of a 4-year-old girl who presented after several days of headaches and anorexia, which then progressed to seizures, left-sided weakness, and altered consciousness. She was later found to have a widespread and occlusive SVT with right cerebral hemorrhagic infarction. This case is notable for the extensive nature of the cerebral SVT and the child’s complete clinical recovery from the neurological event. The report discusses the relation of the thrombosis and leukemia and also emphasizes the importance of early recognition and prompt management, while incorporating a collaborative multidisciplinary approach to prevent long-term consequences.

Neck Rhabdoid Tumors: Clinical Features and Consideration of Autologous Stem Cell Transplant

Extrarenal malignant rhabdoid tumors (MRT) have a poor prognosis despite aggressive therapy. Adding high-dose chemotherapy with autologous stem cell rescue (HDC-ASCR) as consolidative therapy for MRT is controversial. We describe 2 patients, age 13 years and 19 months, with unresectable neck MRT. After chemotherapy and radiotherapy, both underwent HDC-ASCR and remain in remission over 4 years later. We reviewed all published cases of neck MRT, and found poorer outcomes and more variable age of presentation and time to progression than MRT at other sites. Neck MRT may represent a higher-risk subset of MRT, and addition of HDC-ASCR merits consideration.

Synchronous Presentation of Renal Cell Carcinoma and Hodgkin Lymphoma in an Adolescent

Background: Coincidence of renal cell carcinoma (RCC) and hematologic malignancies has been reported in adults but not in children. Observation: We report a case of a 16-year-old girl in whom RCC was incidentally discovered on the computed tomography scan that was performed to stage her underlying Hodgkin lymphoma. Analysis of constitutional cytogenetics for common genetic aberrations that predispose to RCC did not reveal any mutations or genetic variations. However, cytogenetics on the RCC tumor demonstrated a rare reciprocal translocation between chromosomes 6 and 11, t(6;11)(p21;q12). After undergoing partial nephrectomy with regional lymphadenectomy and treatment with multiagent chemotherapy, patient is cancer-free, now 33 months from end of therapy. Conclusions: This case highlights the importance for histologic confirmation of a renal mass when concurrently discovered during the diagnostic evaluation of other malignancies.

An 11-Year-Old Boy with A Sacral Spinal Mass

An 11 year old boy presents with a 1-year history of sharp sacral pain radiating to the buttock and legs, left greater than right. His past medical history is unremarkable. No focal neurologic deficits are noted on examination. However, he has a pronounced antalgic gait with a crouched, widened stance. MRI of the lumbosacral spine (Figure 1a) respectively reveals a sagittal T1 weighted image with a slightly hypointense lesion extending from S1 to S3. The lesion is hyperintense on T2, and in the T1 weighted sagittal image the mass exhibits homogeneous contrast enhancement after Gadolinium injection. The axial imaging post contrast redemonstrates the lesion within the spinal canal. The inferior aspect is located asymmetric to the left and on lower cuts there is likely extension along an exiting nerve root due to thickening and enhancement of the left S3 nerve root (not shown). No other lesions are identified within the cervical, thoracic, or lumbar spine. The patient underwent S1 and S2 laminectomies for tumor resection with use of intraoperative microscope and intraoperative monitoring (SSEP, EMG, and bladder sphincter monitoring). Intraoperatively, the mass was dumbbell shaped with a small intradural and a large extradural component. The extradural portion had a well-defined capsule. Once de-vascularized, the extradural component was amputated from the intradural segment. It was easily separated from the bone. The calcified intradural component was easily separated from the dura. Several rootlets appeared to terminate within the mass and were sacrificed to allow for complete resection. GROSS AND MICROSCOPIC PATHOLOGY