Malignant transformation of phosphaturic mesenchymal tumor: a case report and literature review.
FGF23
malignant phosphaturic mesenchymal tumor-mixed connective tissue variant (PMT-MCT)
tumor-induced osteomalacia
Journal
Clinical pediatric endocrinology : case reports and clinical investigations : official journal of the Japanese Society for Pediatric Endocrinology
ISSN: 0918-5739
Titre abrégé: Clin Pediatr Endocrinol
Pays: Japan
ID NLM: 9433330
Informations de publication
Date de publication:
2020
2020
Historique:
received:
14
10
2019
accepted:
26
12
2019
entrez:
22
4
2020
pubmed:
22
4
2020
medline:
22
4
2020
Statut:
ppublish
Résumé
Phosphaturic mesenchymal tumor, mixed connective tissue variant (PMT-MCT) causes tumor-induced osteomalacia (TIO). Most cases follow a benign clinical course, with rare occurrences of malignant transformation. We report a case of malignant PMT-MCT and review previous malignant cases to identify predictive factors for transformation. A 13-yr-old female, who presented with hypophosphatemic rickets, elevated serum intact fibroblast growth factor 23 (FGF23) levels, and a nodule in the back, received a diagnosis of TIO because of the benign PMT histopathology. After resection of the primary tumor, regular imaging analyses did not indicate any relapse. At 17 years of age, a tumor developed in the left leg and increased in size. The resected tumor showed a histopathology of pleomorphic sarcoma positive for the
Identifiants
pubmed: 32313375
doi: 10.1297/cpe.29.69
pii: 2019-0026
pmc: PMC7160461
doi:
Types de publication
Case Reports
Langues
eng
Pagination
69-75Informations de copyright
2020©The Japanese Society for Pediatric Endocrinology.
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