The role of twinning in sustainable care for children with cancer: A TIPPing point? SIOP PODC Working Group on Twinning, Collaboration, and Support.

With the World Health Organization (WHO) Global Initiative for Childhood Cancer, there is renewed interest in sustainable interventions to improve childhood cancer care in low-/middle-income countries (LMICs). Practitioners in LMICs have traditionally practiced "twinning," i.e., targeted international pediatric oncology partnerships (TIPPs) between one or more institutions in a high-income country (HIC) and an LMIC, to improve care for children with cancer in the latter. The International Society of Paediatric Oncology Committee for Paediatric Oncology in Developing Countries Working Group on Twinning, Collaboration, and Support reviewed guidelines from https://cancerpointe.com and the current literature, gathered input from practitioners in LMICs, and in this article discuss the role of TIPPs in the WHO initiative.

Hematopoietic Stem Cell Regulation by Type I and II Interferons in the Pathogenesis of Acquired Aplastic Anemia.

Aplastic anemia (AA) occurs when the bone marrow fails to support production of all three lineages of blood cells, which are necessary for tissue oxygenation, infection control, and hemostasis. The etiology of acquired AA is elusive in the vast majority of cases but involves exhaustion of hematopoietic stem cells (HSC), which are usually present in the bone marrow in a dormant state, and are responsible for lifelong production of all cells within the hematopoietic system. This destruction is immune mediated and the role of interferons remains incompletely characterized. Interferon gamma (IFNγ) has been associated with AA and type I IFNs (alpha and beta) are well documented to cause bone marrow aplasia during viral infection. In models of infection and inflammation, IFNγ activates HSCs to differentiate and impairs their ability to self-renew, ultimately leading to HSC exhaustion. Recent evidence demonstrating that IFNγ also impacts the HSC microenvironment or niche, raises new questions regarding how IFNγ impairs HSC function in AA. Immune activation can also elicit type I interferons, which may exert effects both distinct from and overlapping with IFNγ on HSCs. IFNα/ß increase HSC proliferation in models of sterile inflammation induced by polyinosinic:polycytidylic acid and lead to BM aplasia during viral infection. Moreover, patients being treated with IFNα exhibit cytopenias, in part due to BM suppression. Herein, we review the current understanding of how interferons contribute to the pathogenesis of acquired AA, and we explore additional potential mechanisms by which interferons directly and indirectly impair HSCs. A comprehensive understanding of how interferons impact hematopoiesis is necessary in order to identify novel therapeutic approaches for treating AA patients.

A de novo desmoid tumor of the surgical site following foramen magnum meningioma resection in a patient with Gardner's Syndrome: a case report and review of the literature.

BACKGROUND: Gardner's Syndrome is a familial adenomatous polyposis (FAP) syndrome that presents with colorectal polyps, craniofacial osteomas, lipomas, epidermoid cysts, and ampullary cancers. These patients are at significant risk for developing desmoid tumors, which are locally infiltrating fibromatous lesions. These are most commonly found in the abdominal cavity, but may be extra-abdominal. Although FAP-associated desmoid tumors often occur at sites of surgical scarring, this is the first report following resection of an intracranial meningioma. CASE DESCRIPTION: We present a patient with Gardner's Syndrome who underwent resection of a foramen magnum meningioma and subsequently developed a de novo desmoid tumor at the surgical site. She had a complete surgical resection of the tumor but did not receive adjuvant therapy. At the time of her death 7 years after her desmoid tumor resection, she had no evidence of a recurrence at the skull base. CONCLUSION: This case report emphasizes the need for careful follow-up in patients with Gardner's Syndrome who undergo resection of a meningioma or other neurosurgical procedure. Adjuvant therapy may be of value in these patients.

Advanced small cell carcinoma of the ovary in a seventeen-year-old female, successfully treated with surgery and multi-agent chemotherapy.

Advanced small cell carcinoma of the ovary (FIGO stage III or IV) is a rare and usually lethal tumor seen in adolescents and young women. In pediatric patients with advanced disease, there have been only two case reports of successful therapy, we report a third patient, diagnosed at 17 years of age, with an abdominal mass and metastatic disease to regional and distant lymph nodes, who was successfully treated with surgery and intensive multi-agent chemotherapy. Imatinib, thalidomide, and celecoxib were also administered for up to 24 months following initial chemotherapy. She remains in remission 3 years from diagnosis.