Infantile Cranial Fasciitis: A Clinicopathologic Evaluation

Area of Science:

• Pathology
• Pediatric Oncology
• Skeletal System Neoplasms

Background:

• Cranial fasciitis (CF) is a rare, benign pseudosarcomatous lesion.
• It typically affects the skull of infants and young children.
• Distinguishing CF from other bone lesions can be diagnostically challenging.

Purpose of the Study:

• To comprehensively analyze the clinicopathologic, immunophenotypic, and molecular genetic features of cranial fasciitis.
• To evaluate the diagnostic utility of various methods, including imaging and histopathology.
• To provide insights into the differential diagnosis of cranial fasciitis.

Main Methods:

• Retrospective analysis of 19 cranial fasciitis cases.
• Inclusion of clinical data, imaging findings, surgical techniques, and histopathological characteristics.
• Utilized special staining, immunohistochemistry, and USP6 break-apart fluorescence in situ hybridization (FISH) assays.

Main Results:

• The study included 19 pediatric patients (11 boys, 8 girls) aged 5-144 months.
• Lesions were most common in the temporal and parietal bones, presenting as rapidly growing, painless masses that eroded the skull.
• Histopathology revealed spindle fibroblasts/myofibroblasts; immunohistochemistry was positive for SMA and Vimentin, negative for other markers. USP6 rearrangement was detected in 10.52% of cases. No recurrence or metastasis was observed post-operation.
• Ocin blue-PH2.5 staining indicated mucinous stromal features.

Conclusions:

• Cranial fasciitis is a benign pseudosarcomatous fasciitis occurring in the infant skull.
• Preoperative diagnosis is often difficult, highlighting the importance of computed tomography (CT) for imaging diagnosis.
• Pathological examination remains the most reliable method for diagnosing cranial fasciitis.