Rare Case of Unicentric Castleman Disease in a Boy

Wolfgang Paik

Key Takeaways

A 13-year-old boy was admitted to the hospital after noticing a palpable enlarged mass in the left inguinal region. His medical history was unremarkable, except for a surgical intervention on his spine 6 years ago due to an accident, which resulted in the loss of motor function and sensation in both lower extremities.

This case report by paediatrician Dr Omran Khaled Janoud and colleagues from Damascus University in Damascus, Syria, documented a rare lymphoproliferative disease.

The Patient and His History

The patient presented with a newly developed space-occupying lesion in the left groin. His medical history was unremarkable except for an accident 6 years prior. Since the accident, he had been paraplegic. Family, medication, drug, social, and travel histories were unremarkable.

Findings and Diagnosis

The patient’s vital signs were measured and showed normal body temperature, respiratory and heart rates, and blood pressure. Upon inspection, decubitus sores were noted on the patient’s left foot.

A deep-seated, movable, round mass was observed in the left inguinal region. The mass was firm to the touch, and there were no indications of a local inflammatory reaction in its vicinity. The remainder of the examination, including auscultation, palpation, and percussion, was unremarkable.

Laboratory tests revealed an elevated erythrocyte sedimentation rate (119 mm/h in the first hour). Other basic laboratory tests, including complete blood cell count, liver function tests, electrolytes, urea, creatinine, and lactate dehydrogenase concentrations in the patient’s blood, were within normal ranges. Overall, there were no indications of systemic disease; therefore, additional diagnostics, such as an HIV test or direct antiglobulin determination, were not performed.

CT of the abdomen, chest, and pelvis revealed enlarged lymph nodes inferior to the inguinal ligament, with the largest measuring approximately (3.5 × 2.4 cm). The other organs and nodes were normal.

Considering the overall findings, lymphoma and leukaemia were deemed unlikely. Consequently, a decision was made to surgically resect the space-occupying lesion.

A biopsy of the mass was performed and pathologically examined. Biopsy revealed a hyperplastic nodal architecture with proliferation of histiocytes and plasma cells with vascular proliferation, consistent with the plasma cell subtype of Castleman disease.

The patient was discharged from the hospital after a 14-day observation period with oral prednisolone. The follow-up period was every 3 months and included a whole-body CT scan and laboratory tests.

After a 12-month follow-up period, the patient reported no new symptoms or lymph node enlargement. Additionally, no abnormalities were observed during the clinical examination or laboratory tests.

Discussion

Castleman disease is an idiopathic, rare lymphoproliferative disorder. It is classified as multicentric Castleman disease, which affects multiple lymph nodes and manifests systemically, or unicentric Castleman disease, which involves only one lymph node. The plasma cell subtype is considered one of the rarest forms of Castleman disease.

“To our knowledge, this case is the first documented instance of unicentric Castleman disease occurring in the inguinal lymph nodes,” the authors wrote.

The fact that it is a plasma cell subtype makes this case report even more valuable. It further illustrates to practising physicians the importance of following a clearly defined diagnostic approach and prioritising the exclusion of malignant or systemic differential diagnoses.

This article was translated from Univadis Germany using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.

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