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The hyper-inflammatory response, also known as multisystem inflammatory syndrome in children (MIS-C), represents a major concern in children with SARS-CoV-2 infection. We report bone marrow features of three patients with MIS-C who were diagnosed during the first wave of the SARS-CoV-2 pandemic. A bone marrow evaluation was performed at onset of the inflammatory condition in order to exclude secondary hemophagocytic lymphohistiocytosis (sHLH). The bone marrows of the patients presented common features: the erythroid and megakaryocytic lineages were prominently affected and hemophagocytosis was moderately increased, differently than observed in sHLH. Megakaryocytopoiesis was increased, representing a peculiar feature of MIS-C differing from sHLH. SARS-CoV-2 RT-PCR and viral panel were studied in bone marrow aspiration samples. MIS-C is a rare complication of SARS-CoV-2 infections in children. An immuno-dysregulation considering both innate and adaptive immunity together with vascular inflammation and endothelial dysfunction play a major role. Our observations, although limited due to the small sample size, suggest that there are unique features in the bone marrow of patients with MIS-C that are likely secondary to immuno-dysregulation, and there are notable differences in bone marrow features compared to those reported in sHLH., (© 2022 The Authors. Pediatric Blood & Cancer published by Wiley Periodicals LLC.)

Aim: Activity and toxiciy of gefitinib in combination with topotecan and cyclophosphamide (CPA) were evaluated in a case-series of relapsed neuroblastoma (NB) patients. The in vitro activity of the combination was also assessed., Procedure: Gefitinib (250 mg/day), topotecan (0.8 mg/m(2)/day), and CPA (50 mg/m(2)/day) (GTC) were administered orally for 14 consecutive days out of 28 days. Antitumor activity of gefitinib as single agent and in combination with either topotecan or CPA was assessed in a panel of NB cell lines., Results: Ninety-two courses were given in 10 patients. Grade 4 neutropenia was observed in 7/92 courses (8%) and grade 4 thrombocytopenia in 8/92 (9%). Two patients had a grade 2 liver toxicity, four a grade 1/2 skin toxicity, and two a grade 1/2 diarrhea. Dose reduction of topotecan and/or CPA was required in eight patients. After four courses, three patients were in partial response (PR) and four with a stable disease (SD), while three experienced a progressive disease (PD). Time to progression (TTP) was 9 months (range, 1-27). After a median follow-up of 16 months (range 5-54), seven patients are died of disease (DOD) and three alive with disease (AWD). All but one patient discontinued oral chemotherapy because of a PD, whilst one patient stopped chemotherapy after 27 months with a SD. In vitro, gefitinib was synergistic with topotecan and additive with CPA., Conclusion: The GTC combination was well tolerated and the TTP was encouraging. These promising results, also supported by in vitro evidence, should be further confirmed in a phase II study., (Copyright 2009 Wiley-Liss, Inc.)

The most common etiologies of acute pancreatitis in children are trauma, multi-system disease, drugs, infections, idiopathic and congenital anomalies of the pancreaticobiliary system. Acute pancreatitis is rarely associated with underlying childhood malignancies. We report a 12-year-old male with acute pancreatitis as the presenting symptom of an alveolar metastatic rhabdomyosarcoma., ((c) 2009 Wiley-Liss, Inc.)

Epithelioid osteosarcoma (OS) is a rare sub-type of OS with an aggressive behavior. An epithelioid OS was diagnosed in an 8-year-old female with painful swelling of the left jaw. After two courses of chemotherapy (cisplatin/methotrexate/doxorubicin), the patient presented a progressive disease. After hemimandibulectomy, 13 courses of post-operative chemotherapy (cisplatin/methotrexate/doxorubicin/ifosfamide) were performed. Histological and ultra-structural examination showed a high grade neoplasm consisting of sheets of epithelioid cells with focal osteoid formation. The patient is alive and in complete remission 42 months from diagnosis., ((c) 2009 Wiley-Liss, Inc.)