Inflammatory pseudotumor-like follicular dendritic cell sarcoma: Literature review of 67 cases

Table 1 Clinical characteristics of patients with inflammatory pseudotumor-like follicular dendritic cell sarcoma

Case No.	Ref.	Sex/age	Site	Maximum diameter in cm	Symptom	Treatment	Time to recurrence in mo	Last follow-up in mo	Outcome
1	Rao et al[22]	39/M	Spleen	7.2	Asymptomatic	Surgery	NA	NA	NA
2	Zhao[23]	28/F	Abdomen	9	Skin rash, cough	Surgery	NA	NA	NA
3	Kazemimood et al[12]	53/F	Colon	3	Abdominal discomfort	Surgery	NA	NA	NA
4	Hommel et al[24]	77/F	Spleen	12	Left upper quadrant abdominal pain	Surgery	-	4	NED
5	Hang et al[25]	57/M	Spleen	2.7	Asymptomatic	Surgery	-	9	NED
6	Granados et al[26]	57/F	Liver	13	Abdominal pain, vomiting	Surgery	-	24	NED
7	Kiryu et al[27]	56/F	Spleen	4	Asymptomatic	Surgery	-	24	NED
8	Li et al[28]	32/F	Liver	3	-	Surgery	-	8	NED
9	Pan et al[29]	78/F	Colon	3.9	Abdominal discomfort, bloody stool	Surgery	-	5	NED
10	Ang et al[30]	63/F	Liver	13.4	Fever and lethargy	Surgery	-	48	NED
11	Wu et al[31]	45/M	Liver	8	Abdominal pain and weight loss	Surgery	-	9	NED
12	Zhang et al[32]	19/F	Liver	6	Abdominal discomfort	Surgery	-	12	NED
13	Deng et al[33]	67/F	Liver	4	Cough	Surgery	NA	NA	NA
14	Horiguchi et al[34]	77/F	Spleen	8.5	Epigastralgia	Surgery	-	36	NED
15	Kitamura et al[35]	74/F	Spleen	3.6	Asymptomatic	Surgery	-	24	NED
16	Kim et al[36]	76/M	Spleen	3.2	Asymptomatic	Surgery	-	7	NED
17	Kwon[37]	58/F	Spleen	5	Asymptomatic	Surgery	-	24	NED
18	Nishiyama et al[38]	73/F	Spleen	8	Asymptomatic	Surgery	-	144	NED
19	Bui et al[39]	50/F	Spleen	8.5	Abdominal pain	Surgery	NA	NA	NA
20	Mograbi et al[40]	70/F	Spleen, Pancreas	Spleen: 3, Pancreas: 7	Asymptomatic	Surgery	NA	NA	NA
21	Hu et al[41]	49/F	Retroperitoneum	5	Asymptomatic	Surgery	Tail of pancreas (60)	60	NED
22	Vardas et al[42]	61/M	Spleen	10	Abdominal pain	Surgery	-	12	NED
23	Agaimy et al[43]	52/M	Ileum and mesentery	6	Abdominal pain	Surgery	NA	NA	NA
24	Ge et al[44]	54/F	Spleen	3.5	Left upper quadrant abdominal pain	Surgery	-	10	NED
25	Ge et al[44]	79/M	Spleen	6	Asymptomatic	Surgery	-	18	NED
26	Zhang et al[45]	31/F	Liver	2 masses: 3.5, 2.5	Anorexia	Surgery	-	10	NED
27	Zhang et al[45]	48/M	Liver and hepatoduodenal ligament lymph node	Liver: 10, Lymph: 3.5	Asymptomatic	Surgery	-	2	NED
28	Ke et al[46]	53/M	Colon	1	Chest and back pain	ESD	-	11	NED
29	Ke et al[46]	48/M	Colon	4.5	Left lower quadrant pain	Surgery	-	7	NED
30	Li et al[47]	64/F	Spleen	7.2	Upper abdominal pain	Surgery	-	8	NED
31	Li et al[47]	61/M	Spleen	6.2	Asymptomatic	Surgery	-	16	NED
32	Li et al[47]	42/F	Spleen	4	Left-sided flank pain	Surgery	-	9	NED
33	Li et al[47]	57/F	Spleen	13.3	Upper abdominal pain	Surgery	Pulmonary (4)	4	LWD
34	Li et al[47]	52/M	Spleen	2.9, 3.7	Back pain	Surgery, chemotherapy	Multiple bone (5)	5	LWD
35	Li et al[48]	49/F	Spleen	4.7	Asymptomatic	Surgery	-	NA	NA
36	Li et al[48]	56/F	Spleen	8	Abdominal pain	Surgery	-	17	NED
37	Li et al[48]	38/M	Liver	8.5	Fatigue, anorexia	Surgery	-	11	NED
38	Li et al[48]	42/F	Liver	2, 1.7	Abdominal pain	Surgery	-	36	NED
39	Li et al[48]	50/M	Spleen and liver	Spleen: 10, Liver: 3, 1.5, 1	Abdominal bloating, fatigue	Surgery	-	17	NED
40	Li et al[48]	39/F	Liver	9	Asymptomatic	Surgery, chemotherapy	Liver (12)	84	NED
41	Choe et al[19]	64/F	Spleen	5.5	Asymptomatic	Surgery	-	78	NED
42	Choe et al[19]	72/F	Spleen	7.2	Asymptomatic	Surgery	-	18	NED
43	Choe et al[19]	53/F	Spleen	3.2	Asymptomatic	Surgery	-	13	NED
44	Choe et al[19]	76/M	Spleen	3.2	Asymptomatic	Surgery	-	8	NED
45	Choe et al[19]	72/M	Spleen	6	Asymptomatic	Surgery	-	18	NED
46	Choe et al[19]	75/M	Spleen	3.5	Abdominal pain	Surgery	-	30	NED
47	Chen et al[49]	28/F	Liver	6	Abdominal pain, fatigue, anorexia	Surgery	Liver (48)	48	LWD
48	Chen et al[49]	39/M	Spleen	7.4	Asymptomatic	Surgery	-	40	NED
49	Chen et al[49]	48/M	Liver	23.3	Abdominal pain, fever, fatigue	Surgery	-	23	NED
50	Chen et al[49]	65/M	Spleen and liver	Spleen: 22.3, Liver: Multiple masses (largest: 5.8)	Abdominal pain, fever, fatigue, anorexia, weight loss	Surgery	-	2	DOD
51	Chen et al[49]	51/M	Spleen	8.5	Weight loss	Surgery	-	19	NED
52	Chen et al[49]	68/M	Spleen	2.3	Asymptomatic	Surgery	-	6	NED
53	Chen et al[49]	51/F	Spleen	5.3	Epigastric discomfort	Surgery	-	5	NED
54	Chen et al[49]	67/M	Spleen	7.5	Asymptomatic	Surgery	-	5	NED
55	Chen et al[49]	60/M	Liver	3	Asymptomatic	Surgery	-	3	NED
56	Chen et al[49]	52/F	Spleen	0.9	Asymptomatic	Surgery	-	12	NED
57	Cheuk et al[4]	19/F	Liver	12	Right upper quadrant pain, weight loss	Surgery	-	40	NED
58	Cheuk et al[4]	56/F	Liver	15	Abdominal discomfort	Surgery	Liver (15, 27 ,35)	56	LWD
59	Cheuk et al[4]	40/F	Liver	12.5	Abdominal pain, weight loss	Surgery	Intraabdominal (108)	108	LWD
60	Cheuk et al[4]	49/F	Liver	4.2	Asymptomatic	Surgery	-	9	NED
61	Cheuk et al[4]	37/M	Liver	15	Weight loss	Surgery	-	42	NED
62	Cheuk et al[4]	35/F	Liver	20	Abdominal discomfort, fever, weight loss	Surgery	Liver (30, 40), peritoneum and ascending colon (60)	95	DOD
63	Cheuk et al[4]	31/F	Liver	15	Abdominal distension, weight loss	Surgery	-	60	NED
64	Cheuk et al[4]	58/F	Spleen	22	Abdominal distension	Surgery	-	4	NED
65	Cheuk et al[4]	39/F	Spleen	7.5	Weight loss, fever	Surgery	-	2	NED
66	Cheuk et al[4]	61/F	Spleen	3.5	Asymptomatic	Surgery	NA	NA	NA
67	Cheuk et al[4]	49/F	Peri-pancreas	9.5	Abdominal distension	Surgery	NA	NA	NA

DOD: Dead of disease; ESD: Endoscopic submucosal dissection; LWD: Live with disease; NA: Not available; NED: No evidence of disease.

Table 2 Pathological features of inflammatory pseudotumor-like follicular dendritic cell sarcoma

Markers	Number of cases positive/number of cases tested	Cases positive, %
Necrosis	40/67	59.7
Hemorrhage	22/67	32.8
Granuloma	23/67	34.3
Fibrinoid deposits in blood vessel wall	22/67	32.8
CD1a	0/6	0
CD3	0/7	0
CD20	1/9	11.1
CD21	54/65	83.1
CD23	34/47	72.3
CD30	1/27	3.7
CD31	1/8	12.5
CD34	5/15	33.3
CD35	50/55	90.9
CD68	8/16	50
CD117	0/9	0
LMP1	28/34	82.4
EBER	60/63	95.2
D2-40	6/10	60
CNA.42	13/13	100
Clusterin	8/11	72.7
EGFR	3/8	37.5
Vimentin	6/6	100
CK-pan	0/9	0
Caldesmon	0/7	0
ALK1	0/42	0
Desmin	0/30	0
Fascin	9/9	100
SMA	32/39	82.1
MSA	5/9	55.5
EMA	2/8	25
S100	6/23	26.1
p53	1/6	16.7
HHV-8	0/7	0
TUBB3	3/6	50
γ-synuclein	2/6	33.3

EMA: Epithelial membrane antigen; HHV-8: Human herpes virus 8; LMP1: Latent membrane protein 1; MSA: Muscle-specific actin; SMA: Smooth muscle actin; TUBB3: Tubulin beta 3 class III.

Table 3 Factors evaluated by univariate and multivariate for association with progression-free survival

Variable	Univariate analysis		Multivariate analysis
	P value	HR (95%CI)	P value	HR (95%CI)
Age ≤ 40 yr	0.50	1.55 (0.39-6.16)	0.48	1.78 (0.36-8.77)
Gender, male/female	0.97	0.97 (0.21-4.64)	0.67	1.51 (0.23-10.04)
Tumor size < 5 cm	0.59	0.59 (0.11-3.32)	0.43	0.38 (0.04-4.10)
Necrosis	0.31	0.51 (0.10-2.59)	0.24	0.25 (0.03-2.49)
Hemorrhage	0.99	0.99 (0.27-3.70)	0.84	1.25 (0.13-11.91)
Granuloma	0.58	0.66 (0.16-2.71)	0.85	0.84 (0.14-5.15)

HR: Hazard ratio; CI: Confidence interval.