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Plexiform fibrohistiocytic tumor in three children
Fatemeh Jafarian1, Catherine McCuaig, Victor Kokta
1Department of Pediatric Dermatology, Ste-Justine Hospital, University of Montreal, Montreal, Quebec, Canada.
Insights
Plexiform fibrohistiocytic tumor, a rare intermediate malignancy, typically affects children and young adults. This study details three pediatric cases, highlighting clinicopathologic findings and reviewing existing literature.
Area of Science:
- Dermatopathology
- Pediatric Oncology
- Surgical Pathology
Background:
- Plexiform fibrohistiocytic tumor (PFT) is an uncommon neoplasm characterized by intermediate malignancy.
- While PFT can occur at any age, it shows a predilection for pediatric and young adult populations.
Observation:
- This report presents three pediatric cases of PFT in girls aged 8 months, 14 months, and 7 years.
- Each patient presented with a solitary, asymptomatic, subcutaneous nodule or plaque.
- Clinical and histopathological examination, including immunohistochemistry, confirmed the diagnosis of PFT.
Findings:
- The clinicopathologic features observed in these three cases align with the established characteristics of plexiform fibrohistiocytic tumor.
- Microscopic examination revealed typical PFT morphology, supported by immunohistochemical staining patterns.
- A review of the English-language literature on PFT was conducted to contextualize these findings.
Implications:
- This case series contributes to the understanding of PFT in pediatric patients.
- Accurate diagnosis and characterization of PFT are crucial for appropriate patient management.
- Further research into the pathogenesis and optimal treatment strategies for PFT is warranted.
Abstract:
Plexiform fibrohistiocytic tumor is a very rare fibrohistiocytic tumor of intermediate malignancy. It can occur at any age but is more prevalent in children and in young adults. Here we present the clinicopathologic findings of three girls with this tumor. The patients were 8 months, 14 months, and 7 years of age. They each presented with a solitary, nontender, subcutaneous nodule or plaque. Light microscopy and immunohistochemical study findings were compatible with plexiform fibrohistiocytic tumor. We also review the previously published cases in the English-language literature.