Establishing a Pediatric Hematology-Oncology Program in Botswana.

PURPOSE: Annually, 300,000 children are diagnosed with cancer, and the majority of these children live in low- and middle-income countries (LMICs). Currently, there is incomplete information on pediatric cancer incidence, diagnosis distribution, and treatment outcomes in Africa. Since 2007, a pediatric hematology-oncology program has been operating in Botswana through a partnership between the Botswana government, Baylor College of Medicine, and Texas Children's Hospital. METHODS: To better understand patient characteristics and outcomes at Botswana's only pediatric cancer program, a hospital-based data base-the Botswana Pediatric Oncology Database-was established in 2014. Children younger than 18 years of age at the time of diagnosis who presented between 2008 and 2015 were included. Data for this study were extracted in February 2016. RESULTS: Of the 240 potential enrollees, 185 (77%) children met eligibility for this study. The median age was 6.4 years, and 50.8% were male. Leukemia was the most common malignancy representing 18.9% of the cohort and 88.1% of the total cohort had a histopathologic diagnosis. HIV seropositivity was confirmed in 13.5%. The 2-year overall survival of all pediatric cancer diagnoses was 52.4%. Abandonment of treatment occurred in 3.8% of patients. CONCLUSION: In the first 9 years of the program, capacity has been developed through a longstanding partnership between Botswana and Baylor College of Medicine/Texas Children's Hospital that has led to children receiving care for cancer and blood disorders. Although continued improvements are necessary, outcomes to date indicate that children with cancer in Botswana can be successfully diagnosed and treated.

Purkinje cell cytoplasmic antibody type 1 (anti-Yo) autoimmunity in a child with Down syndrome.

IMPORTANCE: Purkinje cell cytoplasmic antibody type 1 (PCA-1)-IgG (or anti-Yo) is characteristically detected in women with gynecological or breast adenocarcinoma. We describe 2 unique scenarios occurring in 1 patient: PCA-1 paraneoplastic autoimmunity in a child, and a paraneoplastic neurological disorder in the context of Down syndrome. OBSERVATIONS: A child with Down syndrome and a history of adrenocortical carcinoma resected at age 1 year presented at age 7 years with cerebellar ataxia of subacute onset. Paraneoplastic serological and cerebrospinal fluid evaluations revealed PCA-1. Serological and biochemical studies also supported a diagnosis of subclinical autoimmune hypothyroidism. Extensive serum, urine, and radiological testing did not reveal a new or recurrent neoplasm. Neurological improvements after standard immunotherapy were lacking. CONCLUSIONS AND RELEVANCE: Solid organ neoplasms are uncommon among patients with Down syndrome, but organ-specific autoimmune diseases are common. In our patient, Down syndrome-related impaired T regulatory lymphocyte function (previously reported) may have resulted in both enhanced immunity against an undetected solid neoplasm and paraneoplastic neurological (PCA-1) autoimmunity.

Melanotic xp11.2 neoplasm of the ovary: report of a unique case.

We describe a primary ovarian neoplasm, occurring in a 15-year-old female patient, with morphologic, immunohistochemical, and molecular genetic features identical to those of the very rare tumors of the kidney previously described as "melanotic Xp11 translocation renal cancer." This represents, to the best of our knowledge, the first report of a melanotic Xp11 translocation-associated neoplasm arising outside of the kidney. We discuss the relationship of these rare tumors to neoplasms showing perivascular epithelioid cell differentiation, in particular those showing TFE3 rearrangements.

Spirituality and pediatric cancer.

Spiritual beliefs are an important source of comfort and support to many cancer patients and their families, but they may play a particularly large role when the patient is a child. Parents facing a child's serious illness and possible death, and children themselves trying to make sense of illness, suffering, and death, often look beyond the material world for comfort and explanations.