Related Experiment Videos
Pigmented dermatofibrosarcoma protuberans (Bednár tumor) occurring in a Japanese infant
Y Kaburagi1, N Hatta, S Kawara
1Department of Dermatology, Kanazawa University School of Medicine, Japan. hattanao@med.kanazawa-u.ac.jp
Insights
A rare pigmented dermatofibrosarcoma protuberans (Bednár tumor) was found in a Japanese infant. This pediatric skin cancer case highlights diagnostic considerations for similar pigmented lesions in infants.
Area of Science:
- Dermatology
- Pediatric Oncology
- Surgical Pathology
Background:
- Pigmented dermatofibrosarcoma protuberans, also known as Bednár tumor, is a rare soft tissue sarcoma.
- This tumor typically presents as a slow-growing lesion, often on the extremities.
Observation:
- A 6-month-old Japanese infant presented with an erythematous back tumor that progressively enlarged.
- Initial biopsy revealed spindle-shaped cells; a second biopsy showed increased cellularity, nuclear atypia, storiform pattern, and pigmented dendritic cells.
Findings:
- Immunohistochemistry confirmed spindle-shaped tumor cells positive for vimentin and CD34, but negative for factor XIIIa.
- CD34 expression increased in the second biopsy, suggesting tumor progression.
Implications:
- This case underscores the importance of considering Bednár tumor in the differential diagnosis of pediatric pigmented skin lesions.
- Early and accurate diagnosis is crucial for appropriate management and improved outcomes in pediatric sarcoma cases.
Abstract:
A case of pigmented dermatofibrosarcoma protuberans (Bednár tumor) occurring in a Japanese infant is reported. A 6-month-old girl developed a 16 x 10 mm erythematous tumor with a slight elevation on the lower back at 1 month of age. Histological examination revealed proliferation of spindle-shaped, fibroblast-like cells in the myxoid interstitium. Subsequently the tumor grew gradually to a red-purplish tumor measuring 45 x 36 mm. The second biopsy specimen presented hypercellular proliferation composed of spindle-shaped cells showing slight nuclear atypia and a characteristic storiform pattern, with scattered slender dendritic cells containing a large amount of brown pigment. Immunohistochemical studies of the second biopsy specimen showed that the spindle-shaped tumor cells were positive for vimentin and CD34 and negative for factor XIIIa. The number of CD34-reactive spindle-shaped tumor cells increased in the second biopsy specimen compared with the first biopsy.