{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,7]],"date-time":"2026-09-07T17:08:24Z","timestamp":1788800904152,"version":"build-2803163510"},"reference-count":10,"publisher":"SAGE Publications","issue":"7","license":[{"start":{"date-parts":[[2026,5,14]],"date-time":"2026-05-14T00:00:00Z","timestamp":1778716800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Int J Surg Pathol"],"published-print":{"date-parts":[[2026,10]]},"abstract":"<jats:p>\n                    Atypical neurofibromatous neoplasm of uncertain biological potential (ANNUBP) lies between plexiform neurofibroma and malignant peripheral nerve sheath tumor (MPNST) in neurofibromatosis type 1 (NF1). Pediatric data are limited. We report two children with NF1 and rapidly enlarging peripheral nodules. Patient 1: a 13-year-old boy developed a lumbogluteal nodule despite prior selumetinib. Ultrasound showed a hypoechoic lesion with vascularity; magnetic resonance imaging confirmed interval growth. Excision revealed hypercellularity, architectural loss, cytologic atypia, and up to 2 mitotic figures\/10 high-power fields. Immunohistochemistry showed S100\/SOX10 positivity, focal CD34 loss, p16 loss, p53 wild-type pattern, and Ki-67 \u22481% overall, consistent with ANNUBP. Patient 2: a 15-year-old girl had a painful mass on the volar forearm. Resection disclosed a tumor embedded with a median-nerve fascicle. Histology showed hypercellularity, architectural loss, and 1 mitotic figure\/10 high-power fields without necrosis; Immunohistochemistry showed S100, focal CD34\/p16 reduction, and Ki-67\u2009&lt;\u20091%. Both patients remain recurrence-free under risk-adapted surveillance. ANNUBP is defined by \u22652 of atypia, hypercellularity, architectural loss, and low-level mitotic figures. Frameworks integrate morphology with\n                    <jats:italic toggle=\"yes\">CDKN2A\/B<\/jats:italic>\n                    and emphasize retained H3K27me3 to distinguish ANNUBP from MPNST. In children, ultrasound, magnetic resonance imaging, and positron emission tomograph support triage and biopsy. When feasible, complete excision is preferred; unresectable lesions need closer imaging. Early recognition widens cure opportunities and may refine risk stratification.\n                  <\/jats:p>","DOI":"10.1177\/10668969261438511","type":"journal-article","created":{"date-parts":[[2026,5,15]],"date-time":"2026-05-15T03:18:48Z","timestamp":1778815128000},"page":"1819-1824","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":0,"title":["Atypical Neurofibromatous Neoplasms of Uncertain Biological Potential in Children with Neurofibromatosis Type 1: Report of Two Patients and Review"],"prefix":"10.1177","volume":"34","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4463-6249","authenticated-orcid":false,"given":"Nelly Marlene","family":"Rom\u00e1n-Mendoza","sequence":"first","affiliation":[{"name":"Hospital Infantil Universitario Ni\u00f1o Jes\u00fas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eunate","family":"Mart\u00ed-Carrera","sequence":"additional","affiliation":[{"name":"Department of Plastic Surgery, Hospital Infantil Universitario Ni\u00f1o Jes\u00fas, Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7642-5599","authenticated-orcid":false,"given":"Daniel","family":"Azor\u00edn-Cuadrillero","sequence":"additional","affiliation":[{"name":"Hospital Infantil Universitario Ni\u00f1o Jes\u00fas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0907-3041","authenticated-orcid":false,"given":"Luis","family":"Blasco-Santana","sequence":"additional","affiliation":[{"name":"Hospital Infantil Universitario Ni\u00f1o Jes\u00fas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta","family":"Escribano-P\u00e9rez","sequence":"additional","affiliation":[{"name":"Hospital Infantil Universitario Ni\u00f1o Jes\u00fas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Felisa","family":"V\u00e1zquez-G\u00f3mez","sequence":"additional","affiliation":[{"name":"Hospital Infantil Universitario Ni\u00f1o Jes\u00fas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0907-1959","authenticated-orcid":false,"given":"Fernando","family":"Mart\u00edn-del-Valle","sequence":"additional","affiliation":[{"name":"Hospital Infantil Universitario Ni\u00f1o Jes\u00fas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1532-4123","authenticated-orcid":false,"given":"Anna","family":"Duat-Rodr\u00edguez","sequence":"additional","affiliation":[{"name":"Hospital Infantil Universitario Ni\u00f1o Jes\u00fas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-1633-9890","authenticated-orcid":false,"given":"\u00c1ngela","family":"Hern\u00e1ndez-Mart\u00edn","sequence":"additional","affiliation":[{"name":"Hospital Infantil Universitario Ni\u00f1o 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