[Rare mesenchymal lesions in siblings. Two case reports]
M Guschmann1, J Frege, E Lübbert
1Abteilung Paidopathologie und Placentologie, Institut für Pathologie, Charité, Campus Virchow-Klinikum, Medizinische Fakultät der Humboldt-Universität, Berlin. Michael.Guschmann@charite.de
Insights
Juvenile active ossifying fibroma and mesenchymal hamartoma present at birth with similar tissue composition and obstructive effects. These benign, tumor-like lesions highlight shared developmental pathways in pediatric growths.
Area of Science:
- Pediatric Pathology
- Developmental Biology
- Oncology
Background:
- Ossifying fibromas and mesenchymal hamartomas are rare pediatric tumors.
- Understanding their similarities can aid in diagnosis and treatment.
Observation:
- A 15-month-old boy had a juvenile active ossifying fibroma in his nasal cavity.
- His 9-month-old sibling had a mesenchymal hamartoma of the chest wall.
Findings:
- Both lesions presented at birth with obstructive symptoms.
- Histological analysis revealed a mixture of mature and immature mesenchymal tissue with ossification.
- Neither lesion showed atypical cytologic features, consistent with hamartomas.
Implications:
- These findings suggest a potential shared etiology or developmental pathway for these distinct pediatric lesions.
- Recognizing these similarities can improve diagnostic accuracy for rare pediatric tumors.
- Further research may elucidate common genetic or environmental factors influencing their development.
Abstract:
We report on a 15-month-old boy presenting a juvenile active ossifying fibroma in the right nasal cavity and the sibling, a 9-month-old girl with a mesenchymal hamartoma of the chest wall. The two lesions showed many similarities. Both lesions are present at the time of birth or in early life with local obstructive or compressive effects. The lesions have a similar mixture of mature and immature mesenchymal tissue with areas of ossification. The entities present a tumor-like development with an abnormal mixture of tissue indigenous to the specific area of the body without notable atypical cytologic features. These features are typical criteria for hamartoma lesions.
Related Concept Videos
01:43Sex-linked Disorders
02:41Lethal Alleles
Lucien Cuénot discovered lethal alleles in 1905 while studying the inheritance of coat color in mice. The agouti gene is responsible for the color of the coat in mice. This gene codes for an agouti-signaling protein, which is responsible for melanin distribution in mammals. The wild-type allele gives rise to gray-brown coat color in mice, while the mutant allele gives rise to yellow coat color. In addition to coat color, the agouti gene is associated with the yellow...
01:20The Retinoblastoma Gene
The first-ever tumor suppressor gene called Rb was identified in retinoblastoma - a rare eye tumor in children. In inherited forms of the disease, a child inherits one defective copy of the Rb gene, which predisposes them to retinoblastoma. However,...