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.