Tags

Type your tag names separated by a space and hit enter

Myofibromatosis.
Fetal Pediatr Pathol. 2019 Nov 18 [Online ahead of print]FP

Abstract

Introduction: Myofibromatosis is a distinctive mesenchymal disorder occurring predominantly in childhood, which on microscopy shows peripheral light areas of spindle cells and central cellular areas of primitive oval to spindle cells arranged around hemagiopercytomatous vessels. PDFGRB mutations in the familial and multifocal sporadic forms and SRF-RELA fusions in the cellular variants have been identified. The index case is being presented to discuss the clinico-pathological features, differential diagnosis, and management of the lesion.Case presentation: An 11-year-old male presented with an infraorbital mass of 3 months duration. The mass was excised and microscopy revealed the morphological features of myofibroma with tram-track SMA immunopositivity. Nodular fasciitis and fibromatosis were the differentials considered.

Conclusion:

The SRF-RELA gene fusion may represent a subset that in the future may be used to differentiate these myofibromas/myopericytomas from the ACTB-GLI fusion myopericytomas, and PDGFRB may be used to perhaps separate out familial myofibromas from other myofibromas.

Authors+Show Affiliations

Pathology, Chacha Nehru Bal Chikitsalaya, Geeta Colony, Delhi, India.Department of Pathology, Chacha Nehru Bal Chikitsalaya, Delhi, India.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

31738635

Citation

Rastogi, Kanika, and Lavleen Singh. "Myofibromatosis." Fetal and Pediatric Pathology, 2019, pp. 1-8.
Rastogi K, Singh L. Myofibromatosis. Fetal Pediatr Pathol. 2019.
Rastogi, K., & Singh, L. (2019). Myofibromatosis. Fetal and Pediatric Pathology, 1-8. https://doi.org/10.1080/15513815.2019.1686785
Rastogi K, Singh L. Myofibromatosis. Fetal Pediatr Pathol. 2019 Nov 18;1-8. PubMed PMID: 31738635.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Myofibromatosis. AU - Rastogi,Kanika, AU - Singh,Lavleen, Y1 - 2019/11/18/ PY - 2019/11/19/entrez PY - 2019/11/19/pubmed PY - 2019/11/19/medline KW - Myofibromatosis KW - childhood KW - myofibroblastic SP - 1 EP - 8 JF - Fetal and pediatric pathology JO - Fetal Pediatr Pathol N2 - Introduction: Myofibromatosis is a distinctive mesenchymal disorder occurring predominantly in childhood, which on microscopy shows peripheral light areas of spindle cells and central cellular areas of primitive oval to spindle cells arranged around hemagiopercytomatous vessels. PDFGRB mutations in the familial and multifocal sporadic forms and SRF-RELA fusions in the cellular variants have been identified. The index case is being presented to discuss the clinico-pathological features, differential diagnosis, and management of the lesion.Case presentation: An 11-year-old male presented with an infraorbital mass of 3 months duration. The mass was excised and microscopy revealed the morphological features of myofibroma with tram-track SMA immunopositivity. Nodular fasciitis and fibromatosis were the differentials considered.Conclusion: The SRF-RELA gene fusion may represent a subset that in the future may be used to differentiate these myofibromas/myopericytomas from the ACTB-GLI fusion myopericytomas, and PDGFRB may be used to perhaps separate out familial myofibromas from other myofibromas. SN - 1551-3823 UR - https://www.unboundmedicine.com/medline/citation/31738635/Myofibromatosis L2 - http://www.tandfonline.com/doi/full/10.1080/15513815.2019.1686785 DB - PRIME DP - Unbound Medicine ER -
Try the Free App:
Prime PubMed app for iOS iPhone iPad
Prime PubMed app for Android
Prime PubMed is provided
free to individuals by:
Unbound Medicine.