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Congenital Infantile Fibrosarcoma Associated With a Lipofibromatosis-Like Component: One Train May Be Hiding Another
Romain Swiadkiewicz1, Louise Galmiche, Kahina Belhous
1Departments of *Maxillofacial and Plastic Surgery, and †Pathology, Necker Children Hospital, APHP, Paris, France; ‡Paris Descartes University, Paris, France; Departments of §Pediatric Radiology, and ¶Dermatology, Necker Children Hospital, APHP, Paris, France; ‖Laboratory of Solid Tumors Genetics and Central Laboratory of Pathology, Nice University Hospital, Nice, France; **Microenvironment, Signaling and Cancer, INSERM, Université Nice Sophia-Antipolis, Nice, France; ††Molecular Oral Pathology, INSERM, Centre de Recherche de Cordeliers, Paris, France; and ‡‡Adolescent and Pediatric Oncology Department, Institut Curie, Paris, France.
Insights
Congenital infantile fibrosarcoma (CIFS), a rare infant cancer, can present with benign lipofibromatosis-like areas. This unusual combination can delay CIFS diagnosis in children.
Area of Science:
- Pediatric oncology
- Soft tissue pathology
- Developmental biology
Background:
- Congenital infantile fibrosarcoma (CIFS) is a malignant soft tissue tumor typically affecting infants, primarily in the lower extremities.
- Lipofibromatosis is a rare, benign infiltrative soft tissue tumor found in children, distinct from CIFS.
- Accurate diagnosis of pediatric soft tissue tumors is crucial for appropriate management and outcomes.
Observation:
- This study describes a unique case of CIFS exhibiting surrounding areas resembling lipofibromatosis.
- The patient presented with a lesion that initially suggested a benign condition due to these lipofibromatous features.
Findings:
- Histopathological examination revealed a CIFS diagnosis despite the presence of benign-appearing lipofibromatous tissue.
- The co-occurrence of these distinct entities in a single lesion is rare and poses diagnostic challenges.
Implications:
- The findings highlight the importance of comprehensive histopathological evaluation for pediatric soft tissue lesions.
- Recognizing this unusual presentation can help clinicians avoid diagnostic delays and ensure timely treatment for CIFS.
- Further research may elucidate the biological mechanisms underlying this combined tumor presentation.
Abstract:
Congenital infantile fibrosarcoma (CIFS) is a soft tissue sarcoma of infants mainly involving lower extremities and usually developing during the first year of life. At another end of the spectrum of pediatric fibroblastic lesions, lipofibromatosis is a rare benign infiltrative soft tissue tumor that affects children. The authors report in this study a particular presentation with a CIFS surrounded by lipofibromatosis-like areas. The presence of a surrounding benign tumor confused and delayed CIFS diagnosis.
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