Multimodal Therapy Including Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy Can Result in Long-term Disease-free Survival in Pediatric Desmoplastic Small Round Cell Tumor With Extraperitoneal Disease.

Desmoplastic small round cell tumor is a rare sarcoma with 5-year overall survival of 15%. An 8-year-old female presented with diffuse abdominal/pelvic desmoplastic small round cell tumor including numerous liver metastasis. She underwent neoadjuvant chemotherapy followed by cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). Residual disease was found shortly after CRS/HIPEC which was resected, followed by whole abdomen/pelvic radiation and autologous hematopoietic cell transplant. Previous papers have reported dismal survival in patients with liver metastasis and residual disease arguing against CRS/HIPEC. Our patient remains disease-free over 6 years after completing therapy indicating long-term survival is achievable with aggressive multimodal therapy.

Clinical utilization of whole-body PET/MRI in childhood sarcoma.

Hybrid positron emission tomography (PET)/magnetic resonance imaging (MRI) has emerged as a useful tool that combines the superior tissue contrast of MRI with the targeted functional imaging of PET. In the assessment of sarcomas in children, PET/MRI has the potential to serve as a single point of service, allowing superior anatomical imaging and evaluation of metabolic uptake during one imaging session. In this pictorial essay, we review our preliminary experience with PET/MRI in the evaluation of pediatric sarcoma. The limitations and contraindications of PET/MRI are also discussed.

Patient-reported neuropathic pain in adolescent and young adult cancer patients.

BACKGROUND: Neuropathic pain, a known complication of cancer and its treatments, negatively impacts quality of life. There are limited data using screening tools to aid in the diagnosis of neuropathic pain in cancer patients. Our primary objective was to determine the proportion of adolescent and young adult cancer patients reporting neuropathic pain on a patient-completed, neuropathic pain screening tool. PROCEDURES: This prospective, cohort study enrolled patients 14-39 years of age who were receiving therapy for primary cancer diagnosis, cancer relapse, or had recently completed treatment. The painDETECT, a patient-completed, neuropathic pain screening tool used down to age 14, was administered a maximum of three times in on-therapy patients and once in off-therapy patients. Provider documentation of neuropathic pain at the corresponding visit was abstracted from the medical record. RESULTS: Seventy-eight patients participated. Median (interquartile range) age at study enrollment was 18.1 (16-19.4) years and 47% were female. Cancer diagnoses included 41% leukemia, 26% solid tumor, 23% lymphoma, and 10% central nervous system tumor. The proportion of patients reporting neuropathic pain was 26% (95% confidence interval [CI] 16-40%) in on-therapy patients and 11% (95% CI 3-27%) in off-therapy patients. In patients reporting neuropathic pain, only 26% had a clinical diagnosis of neuropathic pain documented in the medical record at the corresponding visit. CONCLUSIONS: Neuropathic pain occurs in one in four adolescents and young adults receiving cancer therapy. Use of screening tools may increase the detection of neuropathic pain in adolescents and young adults receiving cancer therapy and could ultimately improve pain treatment.

En bloc resection of a C-1 lateral mass osteosarcoma: technical note.

Osteosarcoma is an aggressive primary bone tumor. It is currently treated with multimodality therapy including en bloc resection, which has been demonstrated to confer a survival benefit over intralesional resection. The authors present the case of an 8-year-old girl with a C-1 lateral mass osteosarcoma, which was treated with a 4-stage en bloc resection and spinal reconstruction. While technically complex, the feasibility of en bloc resection for spinal osteosarcoma should be explored in the pediatric population.

Expanding the phenotype of multifocal lymphangioendotheliomatosis with thrombocytopenia.

Multifocal lymphangioendotheliomatosis with thrombocytopenia is characterized by vascular skin and gastrointestinal (GI) tract lesions, thrombocytopenia, and GI bleeding. The first patient had scattered red macules and subcutaneous nodules on the skin with involvement of the lungs, liver, omentum, and right kidney. At 10 months of age he continues to have severe GI bleeding. The second patient had innumerable vascular plaques on the skin plus muscle, bone, lung, liver, and brain involvement. She died from respiratory failure at 8 months of age due to brainstem involvement. Both patients required aggressive management of GI bleeding, but had quite different skin findings and long-term outcomes.

The T cell activation marker CD150 can be used to identify alloantigen-activated CD4(+)25+ regulatory T cells.

We have been investigating whether alloantigen-specific CD4(+)25+ regulatory T cells can be identified for use in treating graft-versus-host disease. CD150, which is upregulated on the surface of all activated T lymphocytes, was identified as a candidate marker for alloantigen-activated CD4(+)25+ regulatory T cells by gene chip analysis. Freshly isolated CD4(+)25+ cells had only low cell-surface expression of CD150, comparable to that of CD4(+)25- T cells. Increased CD150 expression was observed on all T cells after coculture with allogeneic stimulator cells. When purified CD4(+)25+ cells were precultured with allogeneic stimulator cells, then sorted into CD150+ and CD150- subsets, allosuppressive activity was contained primarily in the CD150+ fraction. These cells also suppressed the proliferation of alloantigen-activated autologous T cells, and they could be expanded in vitro without loss of their suppressive capacity. These results suggest that CD150 can be used as a marker for the identification of purified alloantigen-activated CD4(+)25+ regulatory T cells.