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Interdigitating dendritic cell sarcoma successfully treated with ABVD therapy in which serum CEA levels correlated
Reona Sakemura1,2, Junji Hiraga1,3, Satoshi Kitagawa4
1Department of Hematology, Toyota Memorial Hospital.
Insights
Interdigitating dendritic cell sarcoma (IDCS) is rare, with no standard therapy. This case shows ABVD chemotherapy achieved significant clinical improvement in advanced IDCS, suggesting its potential efficacy.
Area of Science:
- Oncology
- Hematology
- Pathology
Background:
- Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare neoplasm.
- No established standard therapy exists for advanced IDCS.
- CHOP-like regimens are typically used as first-line therapy.
Observation:
- A 76-year-old male presented with fatigue, pancytopenia, and elevated CEA.
- Diagnostic workup revealed colonic polyps and bone marrow infiltration by IDCS.
- Initial CHOP therapy yielded limited clinical improvement.
Findings:
- Six cycles of ABVD chemotherapy led to remarkable regression of colonic polyps and bone marrow IDCS.
- The patient became transfusion-independent post-ABVD treatment.
- Serial Carcinoembryonic Antigen (CEA) levels normalized, correlating with disease status.
Implications:
- This case highlights the potential efficacy of the ABVD regimen in treating advanced IDCS.
- Serial CEA measurements may serve as a useful biomarker for monitoring treatment response in IDCS.
- Further investigation into ABVD as a therapeutic option for IDCS is warranted.
Abstract:
Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare neoplasm of spindle to ovoid cells with phenotypic features similar to those of interdigitating dendritic cells. No standard therapy for advanced IDCS has yet been established. According to past reports, CHOP-like regimens are often chosen as primary therapy. Herein, we report a case with advanced IDCS, for which ABVD achieved remarkable clinical improvement and serial CEA levels correlated with disease status. A 76-year-old man presented with general fatigue and pancytopenia with CEA elevation. Colonoscopy showed erosion and polyps in the colon. FDG-PET showed marked abnormal accumulation throughout the bone marrow. Pathologically, polyps of the colon and IDCS of the bone marrow were diagnosed. Although the patient received CHOP as initial therapy, clinical improvement was not significant. Subsequently, six cycles of ABVD resulted in remarkable regression of both the colonic polyps and the bone marrow infiltration. Moreover, the patient was transfusion-free after ABVD. In addition to these clinical responses, serial CEA levels normalized. Our case highlights the efficacy of ABVD for IDCS and serial CEA measurements might reflect treatment efficacy.
