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Managing a child with acute lymphoblastic leukemia (ALL) after relapse is arduous in low- and middle-income countries. A file review of children aged ≤15 years diagnosed with relapsed ALL from 2010 to 2019 was performed. Classification of relapse followed the Berlin-Frankfurt-Münster (BFM) scheme. The majority of patients were treated with a modified ALL-REZ-BFM protocol. Of 764 children treated for ALL in the study period, 163 (21.3%) relapsed. The median age at relapse was 101 months (range: 8–297). The immunophenotype was B-ALL and T-ALL in 140 (86%) and 23 (14%) patients. The site of relapse was extramedullary, combined, and medullary in 46 (28%), 45 (28%), and 72 (44%) patients. Very early, early, and late relapses were observed in 57 (35%), 66 (40%), and 40 (25%) patients. The proportions of extramedullary and medullary sites were greater among patients with early and late relapses, respectively (p = 0.039). Eighty-four (52%) patients were treated with palliative intent. The 2-year event-free survival (EFS) of patients treated with curative intent was 36.3 ± 6.3%. The 2-year EFS for very early/early and late relapses were 18.2 ± 6.2% and 67.6 ± 10.4% (p < 0.001). The 2-year EFS did not differ between extramedullary, combined, and medullary relapses. Treatment-related mortality occurred in 14 (20%) patients. More than 50% of the patients with relapse were treated with the intent of palliation. Extramedullary relapses were more likely to be early and did not have a better outcome than medullary relapses. Children with late relapse had a fair chance of survival with chemotherapy. [ABSTRACT FROM AUTHOR]

Survivors of childhood acute lymphoblastic leukemia (ALL) are vulnerable to late adverse events such as obesity and an associated metabolic syndrome. Children treated for ALL from 2002 to 2012 were included. BMI was calculated at diagnosis, end of treatment, and 5, 8, and 10-years from diagnosis. BMI-centiles were used to categorize the patients: underweight (<5th-percentile), normal (5th–85th percentile), overweight (85th–95th percentile), and obese (≥95th centile). The study included 179 children with ALL (median age: 59-months). The proportions of patients who were underweight, normal, overweight/obese, were 37%, 56% and 7%, respectively, at diagnosis; and 15%, 51% and 34%, respectively, at 5-years from diagnosis. The median (IQR) BMI Z-score at diagnosis was −1.12(−2.40, −0.26). The median (IQR) BMI z-score of the cohort was higher after 5 [0.22(−0.83,1.24), P < 0.001] and 10-years of diagnosis [0.30(−0.69,0.99), P < 0.001], respectively. The proportion of overweight/obese individuals was higher after 5 (34%, P < 0.001) and 10 (26%, P = 0.001) years. There was a significant correlation between the baseline BMI Z-score and that observed after 5-years (ρ = 0.49, P < 0.001), and 10-years (ρ = 0.55, P < 0.001). At 10-years of follow-up, >25% of children with ALL were overweight/obese. The BMI Z-score at the time of diagnosis continued to correlate with the Z-score after 10-years. [ABSTRACT FROM AUTHOR]

High-dose methotrexate (HDMTX) is an important component of treatment in pediatric acute lymphoblastic leukemia (ALL) and lymphoblastic lymphoma (LL). Optimal rescue therapy is essential for the safe administration of HDMTX. A cost-effective strategy that does not compromise safety is necessary for low- and middle-income countries. Consecutive admissions for HDMTX in children with ALL and LL over 12 months were analyzed. The dose of HDMTX was 3 g/m2 in B-ALL and B-LL and 5 g/m2 in T-ALL and T-LL. A methotrexate level was measured at 42 hours of starting HDMTX infusion (T42-MTX). Three doses of folinic acid at T42, T48, and T54 and alkalinized hydration till T54 were administered if T42-MTX <1 µM. A total of 282 cycles of HDMTX that were administered in 71 patients were analyzed. T42-MTX was <1 µM in 266 (94.3%) cycles. T42-MTX was ≥1 µM in 12% and 3% of cycles of HDMTX administered at a dose of 5 g/m2 and 3 g/m2, respectively (p =.074). The median duration of hospitalization for HDM was three days and did not differ with the dose of HDMTX administered (p =.427). Mucositis, delayed recovery of blood counts, and hospitalization for reversible toxicity occurred after 21 (7.4%), 28 (9.9%), and 19 (6.7%) cycles of HDMTX, respectively. Mucositis was greater following the administration of 5 g/m2 of HDMTX. A single T42-MTX measurement permits the safe administration of HDMTX and an expedited discharge from the hospital within three days in more than 90% of children with ALL/LL. [ABSTRACT FROM AUTHOR]

Multiple lytic bone lesions in a child can be a manifestation of various diseases like Langerhans cell histiocytosis, metastatic neuroblastoma, leukemia, hyperparathyroidism, multifocal osteomyelitis and histoplasmosis. Disseminated histoplasmosis caused by Histoplasma capsulatum var. duboisii is well known to present with multiple osteolytic lesions in immunocompromised adults and is mostly restricted to the African subcontinent. Histoplasmosis seen in American and Asian countries is caused by Histoplasma capsulatum var. capsulatum, which presents with pulmonary and systemic manifestations and rarely bone involvement. We report a case of histoplasmosis, caused by H. capsulatum var. capsulatum with extensive lytic bone lesions in a 13 year old immunocompetent boy who presented with prolonged fever, weight loss and multiple boggy swellings. He responded to amphotericin and is currently on Itraconazole. This case is unique for extensive osteolytic lesions with H. capsulatum var. capsulatum infection in an immunocompetent child. [ABSTRACT FROM AUTHOR]

Systemic dissemination of intracranial germ cell tumors (GCTs) occur only in 3% of cases and the common sites are bone, lungs, and lymph nodes. Metastasis to the liver is rare. As far as we could find, only six cases of liver metastasis of intracranial GCTs have been reported so far. We report an adolescent girl who presented with hepatic relapse 2½ years after successful completion of treatment of intracranial GCT. She was treated with chemotherapy and right hepatectomy and is doing well 30 months after treatment for the metastatic disease. [ABSTRACT FROM AUTHOR]

Background: The diagnosis of osteosarcoma (OSA) depends on clinicopathological and radiological correlation. A biopsy is considered the gold standard for OSA diagnosis. However, since OSA is a great histological mimicker, diagnostic challenges exist. Immunohistochemistry (IHC) can serve as an adjunct for the histological diagnosis of OSA. Special AT-rich sequence-binding protein 2 (SATB2) was recently described as a reliable adjunct immunohistochemical marker for the diagnosis of OSA., Methods: We investigated the IHC expression of SATB2 in 95 OSA and 100 non-osteogenic bone and soft tissue tumors using a monoclonal antibody (clone EPNCIR30A). The diagnostic utility of SATB2 and correlation with clinicopathological parameters were analyzed., Results: SATB2 IHC was positive in 88 out of 95 cases (92.6%) of OSA and 50 out of 100 cases (50.0%) of primary non-osteogenic bone and soft tissue tumors. Of the 59 bone tumors, 37 cases (62.7%) were positive for SATB2, and of the 41 soft tissue tumors, 13 cases (31.7%) were positive for SATB2. The sensitivity of SATB2 as a diagnostic test was 92.6%, specificity 50%, positive predictive value 63.8%, and negative predictive value 87.7%., Conclusions: Although SATB2 is a useful diagnostic marker for OSA, other clinical, histological and immunohistochemical features should be considered for the interpretation of SATB2.

Background: Primary intracranial germ cell tumors (ICGCT) are often diagnosed with tumor markers and imaging, which may avoid the need for a biopsy. An intracranial germ cell tumor with mild elevation of markers is seldom stratified as a distinct entity. Methods: Fifty-nine patients were stratified into three groups: pure germinoma (PG), secreting germinoma (SG) and non-germinomatous germ cell tumors (NGGCTs). Results: At 5 years, progression-free survival and overall survival of the three groups (PG vs SG vs NGGCT) were 91% versus 81% versus 59%, and 100% versus 82% versus 68%, respectively. There was no statistically significant difference in outcome among histologically and clinically diagnosed germinomas. Conclusion: A criterion for clinical diagnosis when a biopsy is not feasible is elucidated, and comparable outcomes were demonstrated with histologically diagnosed germinomas.