[Fibromatosis infantile. Report of an aggressive disease]
Francisco Javier Alvarez-Rodríguez1, Carlos Baeza-Herrera, Luis Manuel García-Cabello
1Departamento de Oncología, Hospital Pediátrico Moctezuma, Oriente 158-189, Col. Moctezuma Segunda Sección, Deleg. Venustiano Carranza, 15500 México, D.F., México.
Insights
This rare condition involves multiple nodular lesions in infants, affecting various tissues. A case study highlights a familial form with extensive surgical treatment and a 10-year survival.
Area of Science:
- Pathology
- Pediatric Oncology
- Surgical Oncology
Background:
- Describes a rare entity characterized by collagen-forming spindle cell nodular lesions.
- Lesions involve subcutis, skeletal muscle, bone, and viscera in newborns and infants.
- Presents in solitary or multicentric forms.
Observation:
- A 2-year-old male presented with a large, firm, subcutaneous, immobile, painless cervical mass (4.5x6 cm).
- The patient underwent 13 complete surgical resections.
- This represents the eleventh reported familial case.
Findings:
- The rare entity is defined by specific histological features of collagen-forming spindle cells.
- Multicentric involvement and familial occurrence are key characteristics.
- Aggressive surgical management was required for this pediatric case.
Implications:
- Highlights the aggressive nature and challenges in managing this rare pediatric condition.
- Emphasizes the importance of recognizing familial patterns in diagnosis and management.
- Suggests a need for further research into underlying mechanisms and improved therapeutic strategies.
Abstract:
This rare entity is characterized by multiple nodular lesions largely composed of collagen-forming spindle cells and involving the subcutis, skeletal muscle, bone and viscera of newborns and infants that occurs in either a solitary or multicentric form. The clinical course of a case is presented. The patient is a 2-year-old male who was brought to our hospital because of a large cervical mass. On physical examination a firm, subcutaneous, immobile painless mass measuring 4.5x6 cm was palpated at right side of neck. After the first admission, 13 complete surgical resections were performed. This patient is the eleventh familial case in the literature. The patient died 10 years after the first surgery.
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