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doi: 10.1007/s10787-025-01840-9 236 Gmez-JimnezVBurggraaf-Snchez De Las MatasROrtegaL

The generation of these NF1-pLGG mouse models paved the way for numerous investigations into multiple aspects of NF1-associated OPG including the pathways affected by loss of NF1 (79, 84, 85), the role of microenvironment in regulating these tumors (83, 8690), the remarkable specificity of timing, region, and cell-of-origin of NF1-OPG (70, 8082, 9193)

The majority of eligible studies were classified as Class C (non-randomized trials, case-control studies, or time series) or D (cross-sectional, trend, case report/series, or before-and-after studies)

As a result, the increased presence of M1 macrophages not only contributes to immunotherapy, but also sustains a proinflammatory tumor microenvironment that supports tumor growth at early stage [137]

Diagnostic methods The diagnosis is made by quantitative amino acid analysis of urine