Citations

SummaryPatient‐specific primers from 10 children/adolescents with Burkitt leukaemia (BL) ± central nervous system disease who were treated with French‐British‐American/Lymphome Malins de Burkitt 96 C1 plus rituximab were developed from diagnostic blood/bone marrow. Minimal residual disease (MRD) was assessed by real‐time polymerase chain reaction at the end of induction (EOI) and consolidation (EOC). Seventy per cent (7/10) and 71% (5/7) were MRD‐positive at EOI and EOC, respectively, with no disease recurrences. MRD after induction and consolidation did not predict relapse and subsequent therapy appeared to eliminate MRD. Thus, assessing MRD at a later time point is warranted in future trials to determine its clinical significance.

AimsEpithelioid haemangioendothelioma (EHE) is a malignant vascular neoplasm. Subsets have been characterized previously by translocations resulting in either WWTR1–CAMTA1 or YAP1–TFE3 fusion. We sought to develop molecular and immunohistochemical (IHC) assays to aid in the diagnosis and characterization of EHE.Methods and resultsFifty‐two formalin‐fixed, paraffin‐embedded (FFPE) cases diagnosed between 2002 and 2014 were retrieved from the pathology files of our institutions. Reverse transcription–polymerase chain reaction (RT–PCR) assays were optimized to detect WWTR1–CAMTA1 and YAP1–TFE3 fusion transcripts in FFPE tissue and transcription factor E3 (TFE3) protein accumulation was examined by immunohistochemistry (IHC). RNA was extracted from 33 adequate samples, with more recent cases providing a greater yield of high quality RNA. Fourteen of 18 informative cases were positive for WWTR1–CAMTA1 fusion transcripts, four of which showed higher‐grade cytological features termed by some as ‘malignant EHE’. Novel in‐frame fusion transcripts were identified in four cases by direct sequencing. IHC revealed variable nuclear TFE3 staining in six of 17 cases; three with patchy staining showed WWTR1–CAMTA1 fusion. One of 18 informative cases was positive for YAP1–TFE3 fusion and showed strong nuclear TFE3 staining by IHC.ConclusionsThis study confirms the high incidence of WWTR1–CAMTA1 and YAP1–TFE3 rearrangements in EHE and indicates that the staining pattern for TFE3 IHC is critical for specificity.

Exercise studies were performed on six intact and seven pancreatectomized, unanesthetized dogs to examine the conditions under which exercise decreases the concentration of glucose in blood. No major change in blood glucose concentration was demonstrated during exercise experiments on normoglycemic intact dogs. In the pancreatectomized dogs, the blood glucose concentration curves during exercise followed either one of two patterns: Some rose with exercise and some fell. In dogs with baseline blood glucose concentrations less than 400 mg. per 100 ml., a sustained decline in blood glucose occurred with exercise, while in those dogs with an initial level greater than 400, a rise occurred. No effect of exercise was evident in a pancreatectomized dog with blood glucose concentration near the normal range during continuous infusion of insulin. Exercise produced a decrease in blood glucose concentration in an intact dog with hyperglycemia induced by glucose infusion, but this dog's blood glucose level increased during rest periods following exercise.

Identifying patients who will benefit from a palliative care approach is the first critical step in integrating palliative with curative therapy. Criteria are established that identify hospitalized medical patients who are near end of life, yet there are no criteria with respect to hospitalized patients with psychiatric disorders. The records of 276 consecutive patients admitted to a dedicated inpatient psychiatric unit were reviewed to identify prognostic criteria predictive of mortality. Mortality predictors were 2 or more admissions in the past year ( P = .0114) and older age ( P = .0006). Twenty-two percent of patients met National Hospice and Palliative Care Organization noncancer criteria for dementia. Palliative care intervention should be considered when treating inpatients with psychiatric disorders, especially older patients who have a previous hospitalization or history of dementia.

BACKGROUNDMunchausen syndrome by proxy (MSBP), more formally known as factitious disorder imposed on another, is a form of abuse in which a caregiver deliberately produces or feigns illness in a person under his or her care so that the proxy will receive medical care that gratifies the caregiver. Although well documented in the pediatric literature, few cases of MSBP with adult proxies (MSB‐AP) have been reported. This study reviews existing literature on MSB‐AP to provide a framework for clinicians to recognize this disorder.METHODSWe searched Ovid MEDLINE, Ovid EMBASE, PubMed, Web of Knowledge, and PsychINFO, supplemented by bibliographic examination.RESULTSWe identified 13 cases of MSB‐AP. Perpetrators were caregivers, most (62%) were women, and many worked in healthcare. The age range of the victims was 21 to 82 years. Most were unaware of the abuse, although in 2 cases the victim may have colluded with the perpetrator. Disease fabrication most often resulted from poisoning.CONCLUSIONSMSB‐AP should be included in the differential diagnosis of patients presenting with a complex constellation of symptoms without a unifying etiology and an overly involved caregiver with suspected psychological gain. Early identification is necessary so that healthcare providers do not unknowingly perpetuate harm through treatments that satisfy the perpetrator's psychological needs at the proxy's expense. Journal of Hospital Medicine 2015;10:32–35. © 2014 Society of Hospital Medicine

SummaryChildren and adolescents with Burkitt Lymphoma (BL) and combined central nervous system (CNS) and bone marrow involvement still have a poor prognosis with chemotherapy alone. We therefore investigated in children and adolescents with bone marrow (≥25% blasts) and/or CNS‐positive Burkitt lymphoma the chemoimmunotherapy combination of rituximab (375 mg/m2) and the standard chemotherapy arm of our previously reported French‐American‐British (FAB) Lymphome Malins de Burkitt (LMB) 96 trial. Central pathological and cytogenetic characterization was also performed. There were 40 evaluable patients with Burkitt histology (25 with leukaemia and 15 with CNS disease ± leukaemia). The chemoimmunotherapy regimen was well tolerated. The incidence of grade III/IV mucositis during induction cycles with combined chemotherapy and rituximab was 31% and 26%, respectively. The 3‐year event‐free survival (EFS)/overall survival (OS) was 90% (95% confidence interval [CI], 76–96%) in the entire cohort and 93% (95% CI, 61–99%) in patients with CNS disease. Based on the results of this trial, an international randomized study of FAB/LMB 96 chemotherapy ± rituximab for high‐risk patients is currently under investigation.

Object Traditionally, hemisacrectomy and internal hemipelvectomy procedures have required both an anterior and a posterior approach. A posterior-only approach has the potential to complete an en bloc tumor resection and spinopelvic reconstruction while reducing surgical morbidity. Methods The authors describe 3 cases in which en bloc resection of the hemisacrum and ilium and subsequent lumbopelvic and pelvic ring reconstruction were performed from a posterior-only approach. Two more traditional anterior and posterior staged procedures are also included for comparison. Results In all 3 cases, an oncologically appropriate surgery and spinopelvic reconstruction were performed through a posterior-only approach. Conclusions The advantage of a midline posterior approach is the ability to perform a lumbosacral reconstruction, necessary in cases in which the S-1 body is iatrogenically disrupted during tumor resection.