Retroperitoneal dendritic cell sarcoma: A case report

RATIOANLE: Interdigitating dendritic cell sarcoma (IDCS) is a rare sarcoma that originates from interdigitating dendritic cells in lymphoid tissue, the imaging characteristics of which are poorly defined. Pathological examination can identify the tumor, but reports on the imaging characteristics of IDCS are limited. PATIENT CONCERNS: Here, we report a case of IDCS in a 48-year-old female involving the retroperitoneal area. The patient had a lumbar mass on her right lower back for 4 years, and which started increasing in size 1 year before. DIAGNOSES: An irregular soft tissue mass (10.1cm × 8.5 cm in size) in the right lower back of retroperitoneum was detected by CT examination with unclear borders, uneven density, and necrosis. The solid components of the mass were significantly enhanced on postcontrast imaging. The soft tissue was irregular and uneven. Cystic solid masses were observed on MRI examination in the right retroperitoneum, lateral abdominal wall, waist, and back. Necrosis, hemorrhage, and cystic transformation were observed inside the lesion. The cyst wall, separation, and wall nodules were significantly enhanced on the postcontrast image. No distant metastasis was observed. Postoperative pathology confirmed the diagnosis of IDCS. INTERVENTIONS: The patient underwent surgical resection. The resected margin was positive, and the patient received adjuvant radiotherapy 2 months after the surgery. OUTCOMES: Twelve months after radiotherapy, the patient's chest CT showed multiple metastases in both lungs. The patient was started on combination chemotherapy of doxorubicin and ifosfamide, and the follow-up is still ongoing. LESSONS: Imaging provides a unique advantage to determine the extent of the IDCS, the invasion of adjacent tissues, and the presence or absence of distant metastases.