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[Fibromatoses and related disorders in childhood]
R Kodet1, J Stejskal, V Smelhaus
1Ustav patologie 2. lékarské fakulty UK, Praha.
Insights
This study on childhood fibromatoses in 34 children found aggressive forms and common types like infantile myofibromatosis. Extra-abdominal fibromatoses frequently recurred locally, unlike adult forms.
Area of Science:
- Pediatric Pathology
- Soft Tissue Tumors
- Oncology
Context:
- Fibromatoses are a group of fibrous tissue neoplasms with varying clinical behaviors.
- Childhood fibromatoses present unique diagnostic and management challenges compared to adult counterparts.
- Understanding the spectrum of these conditions in children is crucial for accurate diagnosis and prognosis.
Purpose:
- To retrospectively analyze a series of 34 pediatric cases of fibromatoses and related proliferative lesions.
- To characterize the clinicopathologic features and immunohistochemical profiles of childhood fibromatoses.
- To compare the presentation and behavior of fibromatoses in children versus adults.
Summary:
- The study reviewed 34 children with fibromatoses, including aggressive and typical forms (e.g., sternocleidomastoid tumor, infantile myofibromatosis, digital fibromatosis, fibrous hamartoma).
- Immunohistochemistry showed muscle-specific actin in most cases, with actin/desmin coexpression in infantile myofibromatosis and variable actin expression in aggressive infantile fibromatosis, suggesting poor differentiation.
- Extra-abdominal fibromatoses demonstrated a high propensity for local recurrence, and abdominal fibromatosis occurred in both sexes, contrasting with adult forms.
Impact:
- Provides insights into the diverse spectrum and immunohistochemical findings of fibromatoses in pediatric populations.
- Highlights the aggressive local recurrence potential of extra-abdominal fibromatoses in children.
- Contributes to differentiating pediatric fibromatoses from adult forms, aiding in diagnosis and treatment strategies.
Abstract:
A retrospective study of fibromatoses and related diseases was performed on a series of 34 children. Aggressive forms of fibromatoses similar to those in adults as well as typical forms of childhood fibromatoses and fibrous proliferations, such as sternocleidomastoid tumor, infantile myofibromatosis, digital fibromatosis and fibrous hamartoma were observed. Immunohistochemistry revealed muscle specific actin in eleven out of 13 cases, including hyaline cytoplasmic inclusions in digital fibromatosis. In two patients with infantile myofibromatosis a coexpression of actin and desmin was found. One of two cases of infantile type of aggressive fibromatosis was weakly actin positive whereas the other was negative. This result suggests poorly differentiated character of cells in infantile fibromatosis. Clinicopathologic correlation showed that extraabdominal fibromatoses had a strong propensity for local recurrence. Multiple lesions affecting different muscle groups were diagnosed in two boys. Abdominal fibromatosis affected two girls and two boys, in contrast to adult forms which occur exclusively in women.