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Cranial Fasciitis in Children: Expanding the Spectrum of USP6-Associated Clonal Transient Neoplasms
Faizan Malik1, Anas Bernieh2, Siraj M El Jamal3
1Department of Pathology and Laboratory Medicine, 12326University of Tennessee Health Science Center, Memphis, TN, USA.
Insights
Cranial fasciitis (CF) is a benign pediatric tumor that often shows USP6 gene rearrangements. This condition may spontaneously regress, highlighting its nature as a transient neoplasia.
Area of Science:
- Pediatric Pathology
- Oncology
- Genetics
Background:
- Cranial fasciitis (CF) is a benign fibroblastic tumor typically affecting infants.
- It presents as a rapidly growing cranial mass, often in the temporal or parietal regions.
- CF exhibits a unique growth pattern of rapid proliferation followed by arrest, characteristic of transient neoplasia.
Purpose of the Study:
- To analyze the clinicopathological, immunohistochemical, and molecular features of pediatric cranial fasciitis.
- To investigate the role of USP6 gene rearrangements in CF.
- To evaluate the clinical behavior and treatment outcomes for CF.
Main Methods:
- Retrospective analysis of 18 pediatric CF cases.
- Histological and immunohistochemical examination.
- Molecular analysis for USP6 gene rearrangement.
Main Results:
- Sixty-six percent of CF cases showed USP6 gene rearrangement.
- Tumors typically presented as painless, rapidly enlarging temporal masses.
- Surgical outcomes varied, with some recurrences after gross total resection and spontaneous regression observed in others.
Conclusions:
- This study represents the largest series of pediatric cranial fasciitis to date.
- USP6 gene rearrangement is a common finding in CF, supporting its classification as transient neoplasia.
- The variable clinical course, including spontaneous regression, suggests conservative management may be appropriate for some cases.
Abstract:
Background: Cranial fasciitis (CF) is a benign (myo)fibroblastic proliferation of children. Typical presentation consists of a rapidly growing solitary mass on the temporal or parietal cranium in the first 2 years of age. CF is characterized by a rapid growth followed by a relative slowdown and even growth arrest. The finding of somatic USP6 gene rearrangements demonstrating clonality in CF together with its clinical behavior places it in the category of diseases recently termed "transient neoplasia."Methods: Histological, immunohistochemical, and molecular findings of 18 patients with CF were retrospectively studied.Results: The tumor typically presented as a painless rapidly enlarging mass in the temporal region. Sixty-six percent of the cases harbored USP6 gene rearrangement. Nine patients were treated with gross total resection (GTR) and 9 with subtotal tumor resection (STR). Two patients treated with GTR had recurrence. Five patients treated with STR had progression-free disease for at least 10 months after surgery and in four patients the tumor regressed spontaneously a median 16 months after surgery.Conclusions: In this largest series to date, we reported the clinicopathological, immunohistochemical, and molecular findings of 18 pediatric cases of CF with emphasis on the clinical growth pattern of these tumors.
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