Unicentric Castleman Disease With Systemic Symptoms: A Rare Case From Family Practice

Palpable lymphadenopathy is very common in children. The vast majority of cases are due to benign conditions and self-limiting diseases. Careful clinical evaluation, with thorough history taking and make a comprehensive physical examination, is essential to avoid unnecessary invasive procedures and not to misdiagnose possible serious underlying conditions. We report the case of a 9-year-old child with a lump in the right axilla that was first noticed with a swelling two months ago. The lump was not painful but its size has been gradually increasing. The symptom was associated with night sweats. However, there was no history of cough, fever, or weight loss. The child had no history of animal contact or insect bites. No recent travel or history of contact with any sick person was reported. His vaccination schedule was up-to-date. Upon examination, the patient had a smooth non-tender swelling in the right axilla. It measured around 4 x 4 cm. The overlying skin was normal with no erythema or ulceration. The swelling was mobile and was not adherent to the overlying skin. Laboratory investigation showed mild anemia, thrombocytosis, and elevated C-reactive protein level. An ultrasound examination demonstrated a well-circumscribed enlarged lymph node, measuring 3.4 cm in short axis, with an increased blood flow on color doppler. Biopsy findings showed proliferation of the follicular lymphoid tissues that were centered around penetrative vessels giving the appearance of “onion skin” in keeping with Castleman disease. Complete surgical resection of the lymph node was performed and resulted in the resolution of the systemic symptoms. Castleman disease is a rare lymphoproliferative disorder with shared histopathological features. Unicentric Castleman disease usually presents with isolated asymptomatic lymphadenopathy. However, the present case demonstrated that patients with unicentric Castleman disease may exhibit systemic constitutional symptoms similar to that of the multicentric subtype.