To Transplant or Not to Transplant? Late-Onset Primary HLH in a Patient: A Case Report and Review of Literature.

Primary, or familial, hemophagocytic lymphohistiocytosis (P-HLH) is a rare inherited autosomal-recessive immune deficiency which generally manifests during infancy or early childhood. Recent literature suggests an increased number of reports of late-onset P-HLH, especially in association with infection and underlying malignancy. The authors describe a case of subcutaneous T-cell lymphoma in a 8-year-old child that was complicated by primary, perforin-deficient HLH.

Effective Use of Sirolimus and Zoledronic Acid for Multiosteotic Pseudomyogenic Hemangioendothelioma of the Bone in a Child: Case Report and Review of Literature.

Pseudomyogenic hemangioendothelioma (PMH) is a rare neoplasm with vascular and sarcomatous elements, unpredictable course, and uncommon metastatic or fatal potential. Although systemic chemotherapy has been reported with variable success, generally accepted treatment is aggressive surgery with wide margins. Evidence-based treatment options are lacking, and lack of clear prognostic features poses a risk of undertreatment or overtreatment with associated morbidity and mortality.

Docetaxel, bevacizumab, and gemcitabine for very high risk sarcomas in adolescents and young adults: A single-center experience.

Background: Adolescent and young adult (AYA) patients with very high risk sarcomas have poor outcomes and are in need of novel therapies.

Procedure: From January 2005 to February 2016, we retrospectively identified all AYA patients with relapsed or metastatic high-grade sarcomas, who were treated with at least one cycle of docetaxel (T), bevacizumab (A), and gemcitabine (G) (TAG ; T = 100 mg/m Day 8, A = 15 mg/kg Day 1, G = 1,000 mg/m Days 1 and 8).

Results: Fourteen patients, median age of 20 (15-30), received a total of 80 cycles of TAG, and were followed for a median of 83 months.