An Exceedingly Rare Case of Nodular Fasciitis of Tongue in an Infant

Abstract

Abstract Introduction/Objective Nodular fasciitis is a rapidly growing but benign self-limited myofibroblastic/fibroblastic tumor of unknown etiology. It was first described in the literature by Konwaler in 1955 and can occur in any location. Nodular fasciitis of the tongue is a rare entity in infancy with only one case reported so far. Accurate diagnosis of this entity is crucial to avoid a possible misdiagnosis of malignancy leading to radical resections. Methods/Case Report A 6-month old, previously heathy full term male presented to pediatric otolaryngology clinic with a rapidly growing painless mass on the right tongue. The mass was associated with feeding difficulty. There was no history of trauma to the area, no personal and family history of cancer was noted. Computed tomography scan demonstrated a 1.5 x 1.4 x 1.5 cm mass confined to the right anterior undersurface of tongue. Biopsy of the mass showed ulcerated mucosa with an underlying spindle cell lesion composed of reactive-appearing myofibroblasts arranged in a variably fascicular to "tissue-culture" pattern, intermixed with inflammatory cells and scattered extravasated erythrocytes. These morphologic features in conjunction with immunohistochemistry and demonstration of fusion of SRSF3 [serine and arginine rich splicing factor 3] and USP6 [ubiquitin specific peptidase 6] by Targeted next-generation RNA sequencing confirmed the diagnosis of nodular fasciitis. On follow up, spontaneous regression of the mass occurred in 3 months. Results (if a Case Study enter NA) NA. Conclusion Nodular fasciitis of tongue in infancy is an exceedingly rare entity and this case demonstrates the importance of including this unique entity in the differential diagnosis of a rapidly-growing tongue lesion. Because it can simulate malignancy both clinically and histologically, awareness and familiarity with this benign proliferative lesion is crucial in effective management.