ABSTRACT
INTRODUCTION AND IMPORTANCE: Juvenile ossifying fibromas (JOF) are rare benign tumors affecting the craniofacial area, and they present more in younger age groups. JOFs are aggressive lesions and have a high tendency for recurring after surgical resection. CASE PRESENTATION: A 9-years-old female who was a known case of major histocompatibility complex class II deficiency since birth and post hematopoietic stem cell transplant was diagnosed with B-cell acute lymphoblastic leukemia (ALL). Right anterior ethmoidal sinus opacification with mucosal thickening found on sinus imaging was initially thought to be infectious in origin due to the patient's immunodeficiency. However, further investigation and endoscopic sinus surgery with lesion debridement and biopsy revealed psammomatoid JOF. Follow up and imaging results urged the need for a second surgery, but no recurrence was detected. As the patient's ALL was being treated, the case was followed up for two months with imaging showing opacification in multiple sinuses which was suspected to be fungal infection due to immunodeficiency. The complicated character of the case may have hindered any additional invasive management. Opacification persisted despite conservative management. Unfortunately, the patient suffered from disease complication, infections, and died from multi-organ failure. CLINICAL DISCUSSION: This case highlights the importance of a thorough diagnostic workup and management strategy for immunodeficient patients with JOF. JOF's recurrent nature along with the pre-existing immunodeficiency made this case difficult to manage as the patient had other life-threatening condition. CONCLUSION: Specific management considerations should take place in immunocompromised patients. Post-operative long-term follow-ups are needed for early detection of recurrence.