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Infantile fibrosarcoma associated with urticaria pigmentosa
Sameer Bakhshi1, Süreyya Savaşan, Esteban Abella
1Department of Pediatrics, Children's Hospital of Michigan, Wayne State University School of Medicine, Detroit 48201, USA.
Insights
This study details the first reported case of infantile fibrosarcoma in an infant with urticaria pigmentosa. While the tumor was removed, the associated skin condition, mastocytosis, remains.
Area of Science:
- Pediatric Oncology
- Dermatology
- Hematology
Background:
- Urticaria pigmentosa is a rare condition characterized by mast cell accumulation in the skin.
- Mastocytosis can be associated with systemic involvement and, rarely, hematologic malignancies.
- The association between mastocytosis and solid tumors is not well-established.
Observation:
- A case report of an infant diagnosed with urticaria pigmentosa is presented.
- The infant subsequently developed an infantile fibrosarcoma, a rare soft tissue tumor.
- The fibrosarcoma was surgically removed with successful outcomes.
Findings:
- This case represents the first documented instance of infantile fibrosarcoma occurring in conjunction with urticaria pigmentosa.
- Post-resection, the infantile fibrosarcoma did not recur, but the urticaria pigmentosa lesions persisted.
- A review of existing literature on mastocytosis and solid tumor associations was conducted.
Implications:
- This finding suggests a potential, albeit rare, link between mastocytosis and the development of specific pediatric solid tumors like fibrosarcoma.
- Further research is warranted to explore the potential underlying mechanisms connecting mast cell disorders and tumorigenesis.
- Clinicians should maintain awareness of potential oncologic associations in patients with extensive or systemic mastocytosis.
Abstract:
The authors report the case of an infant with urticaria pigmentosa who developed infantile fibrosarcoma. The tumor was successfully resected but the skin lesions have persisted. This is the first report of a fibrosarcoma in association with urticaria pigmentosa. A brief review is presented of the available medical literature on associations of solid tumors with mastocytosis.