A rare presentation of unicentric Castleman's disease in the thigh: A case report and review of literature

Abstract

BACKGROUND

Castleman's disease (CD) is a rare lymphoproliferative, emulating both benign and malignant diseases. The diagnosis of CD is formulated upon the combination of clinical and laboratory criteria and ultimately confirmed by histopathological assessment. Due to its rarity, CD presents a challenge in treatment selection, with available options encompassing surgery, chemotherapy, and autologous stem cell transplantation. However, studies suggest that surgical resection of the lesion is the most effective treatment modality, especially for unicentric CD (UCD).

CASE SUMMARY

Here, we describe the case of a 25-year-old woman who presented with painless left thigh swelling for 10 wk. She had been following a low-fat diet to lose weight and had normal laboratory results. Magnetic resonance imaging revealed a well-circumscribed, demarcated cystic lesion located in the left inguinal region with eccentrically positioned signal void vascular structures, measuring 4.3 cm × 3 cm × 3.2 cm, likely of lymphoid origin. The patient underwent surgical resection, and the final histopathology showed a vascular proliferation and hyalinization of the vessel walls, along with atretic germinal centers traversed by penetrating vessels, consistent with CD. The patient was discharged home one day after the procedure in good condition, with a follow-up appointment scheduled in our outpatient clinic.

CONCLUSION

Although surgical resection is the mainstay for UCD, a multidisciplinary approach is needed due the lack of specific diagnostic features and treatments.

Keywords: Castleman’s disease; Lymph nodes; Surgical resection; Lymphoproliferative disorder; Case report

Core Tip: Castleman's disease (CD) is a rare disorder primarily affecting lymph nodes and associated tissues. Clinical characteristics and survival vary significantly among the three histological subtypes of CD. Diagnosis of CD primarily relies on histopathological examination, supported by imaging modalities such as computed tomography scan, magnetic resonance imaging, and ultrasound. Histopathological examination is crucial for diagnosing both unicentric-CD (UCD) and multicentric-CD, especially after ruling out other disorders, including infections, malignancies, and autoimmune conditions. Despite its rarity, CD presents a range of treatment options. However, studies consistently highlight surgical resection as the optimal treatment modality, particularly for UCD.