Discussion
The infrequency with which CD is diagnosed in pediatric patients has hampered comprehensive clinical studies, leading to an incomplete understanding of the disease, its subtypes, and its prognosis.
Unicentric CD is more common than multifocal CD in children. It usually presents clinically with compressive symptoms, which are investigated with imaging, rather than abnormal labs or physical findings. Our patient’s presentation was unique because microcytic anemia is more often seen in multifocal CD and in the absence of other significant symptoms. His presentation delayed diagnosis for several months. The patient’s anemia likely resulted from IL-6–mediated hepcidin secretion, given his IL-6 level was elevated until removal of the mass6. Evaluation for short stature, abdominal pain with anemia in this case prompted the abdominal ultrasound that identified the mass. Although this mass was identified with abdominal ultrasonography, it is important to note that abdominal CT is more sensitive for the HVV due to the rich vascularization of these masses. Unicentric HVV CD is generally more common in the fourth decade of life2, however, our patient was 12 years old at diagnosis.
As seen in this case, surgical excision is preferable to needle biopsy to properly analyze the architecture of the entire germinal center and interfollicular zone2. Removal of the mass was curative in our patient, leading to normalization of the patient’s laboratory values. However, unicentric CD is sometimes unresectable due to location or size and may require systemic therapy. While there is no standardized therapy, options include immunotherapy, corticosteroids, chemotherapy, or radiotherapy.3
Following excision, surveillance can be gradually spaced out as laboratory findings normalize; radiologic imaging may be considered 6 to 12 months later to verify cure and no recurrence2. Patients with systemic involvement must be monitored closely postoperatively, with regular laboratory tests and imaging studies2. However, since little data and few reported cases have described systemic symptoms in unicentric CD, the long-term monitoring plan for this patient was based on the normalization of his laboratory values and reassuring clinical examination.