Published online Feb 28, 2021. doi: 10.13105/wjma.v9.i1.1
Peer-review started: December 23, 2020
First decision: December 30, 2020
Revised: January 2, 2021
Accepted: February 25, 2021
Article in press: February 25, 2021
Published online: February 28, 2021
Processing time: 68 Days and 12.9 Hours
Inflammatory pseudotumor (IPT)-like follicular dendritic cell (FDC) sarcoma is rare. The 2017 World Health Organization classification of tumors of hematopoietic and lymphoid tissues noted that data on its clinical outcome are limited, but that the tumor appears to be indolent. The aim of this study was to summarize the clinical characteristics, treatment outcomes, and prognostic factors for IPT-like FDC sarcoma. A literature review was conducted on retrospective analyses of clinical data and prognostic information on IPT-like FDC sarcoma reported between 2001 and 2020. A total of 67 cases of IPT-like FDC sarcoma were retrieved from the literature, documenting that it occurs predominantly in middle-aged adults, with a marked female predilection. Six patients had a separate malignancy and five had an autoimmune disease. Typically involving the spleen and/or liver, it may also selectively involve the abdomen, gastrointes-tinal tract, pancreas, retroperitoneum, and mesentery. Necrosis, hemorrhage, noncaseating epithelioid granulomas, and fibrinoid deposits in blood vessel walls are often present. The neoplastic cells are predominantly positive for follicular dendritic cell markers such as cluster of differentiation 21 (CD21), CD23, CD35 and CNA.42 and are consistently Epstein-Barr virus (EBV)-positive. Mitoses were very rare in most cases. Most patients were treated by surgery alone. Disease status at the time of last follow-up was known for 57 patients with follow-up time ranging from 2 to 144 mo. Local and/or distant recurrence after initial treatment was seen in 15.8% of the patients. The 1- and 5-year progression-free survival for the entire group was 91.5% and 56.1%, respectively. Kaplan-Meier and multivariate analyses showed that age, sex, tumor size, and pathological features were not risk factors for disease progression. IPT-like FDC sarcoma appears to be mildly aggressive and requires annual surveillance. Surgery is the most effective treatment modality, and the role of adjuvant chemotherapy for postoperative management is unclear. EBV is likely to play an important role in the etiology of IPT-like FDC sarcoma.
Core Tip: We show that inflammatory pseudotumor-like follicular dendritic cell (FDC) sarcoma appears to be mildly aggressive and does require annual surveillance. Surgery is the most effective treatment modality, and the role of adjuvant chemotherapy for postoperative management is unclear. Epstein-Barr virus is likely to play an important role in the etiology of inflammatory pseudotumor-like FDC sarcoma.