Multimodal Management With Immunotherapy, Radiation, and Surgery of Histiocytic Sarcoma Following Acute Lymphoblastic Leukemia: An Unusual Presentation of a Rare Disease.

Histiocytic sarcoma (HS) is a rare neoplasm with no known cause. This sarcoma is characterized by morphology similar to that demonstrated by mature tissue histiocytes and mostly afflicts adults. HSs typically have a poor prognosis due to a rapidly progressive clinical course. Our patient's case was unique due to its presentation four years after completion of treatment for B-cell acute lymphoblastic leukemia. The patient experienced progression with initial therapy for HS. With dual immunotherapy and radiation, however, the patient has remained clinically stable without detectable disease. Immunotherapy may be a successful and tolerable therapeutic option for histiocytic disease.

Giant Multilocular Prostatic Cystadenoma in a 14-Year-Old Male: A Case Report of a Pediatric Pelvic Mass.

Giant multilocular prostatic cystadenoma (GMC) is an extremely rare, benign tumor seen in both adult and pediatric males. The neoplasm originates from prostatic tissue and is typically found within the rectovesical pouch, varying in both size and morphology. Microscopically, GMC contains both glandular and cystic prostatic tissue lined by cuboidal and columnar epithelium. Symptoms often arise once the pelvic mass begins to obstruct the surrounding structures and organs, although invasion into surrounding tissue is unlikely. Common symptoms include abdominal pain, urinary retention, and dysuria. The standard treatment for GMC is surgical removal of the mass with good outcomes and only 1 known case of recurrence. Here we present the case of a 14-year-old male with GMC-the youngest patient reported to date-who presented with abdominal pain, difficulty voiding, and hydroureteronephrosis.

An exploratory study of culture in treatment for Aboriginal Australian men in residential drug and alcohol rehabilitation services.

The objectives of the research are to describe the cultural activities offered in residential drug and alcohol rehabilitation for Aboriginal Australian men, along with client perceptions of the benefits associated with these cultural activities. Participants were 101 Australian Aboriginal male clients attending five residential drug and alcohol rehabilitation services in New South Wales. Participants completed a semi-structured interview and questionnaires that included the Growth and Empowerment Measure, the Aboriginal Cultural Engagement Survey, Clinical Global Impressions, and two questionnaires regarding cultural engagement while in treatment. Service users indicated that the most beneficial cultural activities offered within services were traditional art/craft, culturally-focused talks/meetings, and being on the land. Hierarchical multiple regression analysis indicated that cultural engagement in everyday life significantly predicted empowerment but not other measures of mental ill-health. The opinions of service users are presented, and recommendations are made regarding ways to enhance the effectiveness of cultural activities within drug and alcohol rehabilitation programs.

Preliminary development and content validity of a measure of Australian Aboriginal cultural engagement.

OBJECTIVES: Aboriginal people form one of the populations most in need of mental health and substance abuse services within Australia, although many services are not adequately sensitive to, or inclusive of, relevant aspects of Aboriginal culture in their programmes. The Aboriginal Cultural Engagement Survey (ACES) was developed with the objective of assessing the level of cultural engagement of Aboriginal clients. A measure of cultural engagement is an important step in establishing an association between culture and health benefits, so that future interventions may be designed which better meet the cultural needs of Aboriginal Australians within health services. DESIGN: The process of development of the ACES involved four stages of scale development utilising a series of group discussions and reviews with Aboriginal consultants. Assessment of content validity is conducted using the Content Validity Index (CVI). RESULTS: The ACES was found to have excellent content validity with CVIs over 0.80 for all items in the final version. CONCLUSION: The ACES shows promise for being a useful tool in assessing the cultural engagement of Australian Aboriginal clients. There is a need for further psychometric assessment and field trials to assess its utility.

Recurrent EGFR alterations in NTRK3 fusion negative congenital mesoblastic nephroma.

OBJECTIVES: To identify oncogenic driver mutations in congenital mesoblastic nephroma (CMN) cases lacking ETV6-NTRK3 fusion and discuss their diagnostic value. DESIGN: The institutional pathology database was queried for cases with a morphologic diagnosis of CMN. Cases positive for ETV6 rearrangement or with unavailable blocks were excluded. Four cases met the inclusion criteria and were sequenced by next-generation sequencing. Three additional cases were contributed by our collaborators. RESULTS: Three of four internal cases harbor an EGFR kinase domain duplication (KDD), which is known to be oncogenic yet exceedingly rare in other histologies. All three outside cases are positive for EGFR alterations, including KDD in two and a splicing site mutation in one. The splicing site mutation is predicted to be EGFR activating. One of the outside cases was a retroperitoneal mass without a clear site of origin. A diagnosis of CMN is suggested based on exclusion of differential diagnoses by expert consultation and detection of EGFR KDD. CONCLUSIONS: EGFR activation, predominantly via EGFR KDD, is a common recurrent genetic alteration in CMN lacking NTRK3 fusions. CMN can be molecularly classified into NTRK3 fusion type, EGFR activation type and others.

Polymerase chain reaction for pathogen identification in persistent pediatric cervical lymphadenitis.

OBJECTIVE: To study routine culture-negative persistent cervical lymphadenitis in children treated surgically during a 10-year period (December 26, 1997, to October 1, 2007) at a single institution. DESIGN: Retrospective case series. SETTING: Tertiary university-based pediatric referral center. PATIENTS: Patients 18 years or younger with cervical lymphadenitis managed surgically (incision and drainage, curettage, and/or excisional lymphadenectomy) and medically (antibiotic therapy), culture-negative after 48 hours, and subsequently diagnosed using the polymerase chain reaction, extended culture incubation, and/or histopathologic evaluation. MAIN OUTCOME MEASURES: Number of surgical interventions, causative organisms, histopathologic features, and resolution of lymphadenitis. RESULTS: Ninety surgical procedures were performed in 60 patients. The cure rate was 23% (approximately 14 patients) with incision and drainage, 58% (approximately 35 patients) with curettage, and 95% (57 patients) with excisional lymphadenectomy. Nontuberculous mycobacteria were the most prevalent causative organisms, followed by Bartonella and Legionella organisms. Four of 6 patients with Bartonella infection had a history of cat exposure, and 4 of 6 patients with Legionella infection had a history of hot tub exposure. CONCLUSIONS: Excisional lymphadenectomy is the preferred treatment of mycobacterial persistent cervical lymphadenitis in children. Sufficient data are lacking for similar recommendations in patients with disease caused by Bartonella organisms, whereas for neck disease caused by Legionella organisms, excisional lymphadenectomy may be superior to incision and drainage. The polymerase chain reaction is useful for pathogen identification in pediatric cervical lymphadenitis, although it is less sensitive in identification of mycobacteria. To our knowledge, our study is the first to report multiple cases of legionellosis in otherwise healthy children. Legionella seems to be a previously unrecognized but relatively common pathogen in culture-negative persistent cervical lymphadenitis in children.