Doege-Potter syndrome by malignant solitary fibrous tumor of the liver: A case report and review of literature

Table 1 Solitary fibrous tumor liver cases with malignant features

No	Author (yr)	Age/Sex	Presenta-tion	Lobe	Size	Treatment	Histopatho-logy	Tumor-markers	IHC	Follow up
1	Fuksbrumera et al[28] (2000)	71/F	NA	R	14 × 17	Resection (UM)	Increased nuclear atypia, mitoses 8/10 HPF	NA	CD34+, Bcl2+, V+	NA
2	Yilmaz et al[18] (2000)	25/F	Weakness,fatigue, anorexia, vomiting and progressive jaundice	L + R	32 × 30	Resection (UM)	Cellularity ranged from 20%-60%, necrosis, hypervascularity	NAD	V+	Bone metastasis 1 mo postsurgery managed with 6 mo of chemo (cyclophosphamide, adriamycin)
3	Terkivatan et al[29] (2006)	74/M	Gastric fullness, postprandial nausea, and weight loss	L	24 × 21 × 15	Resection (FM)	A few highly cellular areas mitoses 10 - 13/10 HPF	NAD	CD34+, CD99+, Bcl2+, V+	12 mo no signs of local recurrence or distant metastases
4	Chan et al[16] (2007)	70/M	Hypoglycaemia, progressive jaundice	R	27 × 24 × 12	Failed TACE 6 wk preoperatively followed by successful resection (UM)	Mildly atypical spindle cells, highly cellular, plemorphia, necrosis, mitoses > 20 HPF	CA-125: 145 U/mL (normal< 35 U/mL)	CD34+, CD99+, bcl2+, V+	bilateral lung metastasis and bi-lobar recurrence at 9 mo
5	Brochard et al[30] (2010)	54/M	Abdominal pain, weight loss	R	17	Resection (FM)	Moderately cellular, polymorphic cells, mitoses < 5/10 HPF	NAD	CD34+, V+, desmin+, actin+	Patient died 1 mo after for Local recurrence 6 yr postsurgery, cranial base metastasis, Retroperitoneal and iliac bone metastasis
6	Fama et al[21] (2008)	68/M	Hypoglycaemic coma	R	15	Resection (FM)	Hypercellular, moderately atypical nuclei, mitoses 20/10 HPF	NAD	CD34+, Bcl2+	25 mo no signs of local recurrence or distant metastases
7	Peng et al[17] (2011)	24/F	Abdominal discomfort and distention	R	30 × 17 × 15	TACE few days prior to resection (FM)	Highly cellular, pleomorphic, necrosis, mitoses > 10/HPF	CA-125 augmented	CD34+, bcl2+, V+	Patient died 16 mo after initial surgery for skull base metastases, Vertebral metastasis
8	Belga et al[31] (2012)	66/F	Increase in abdominal girth	R	14	Resection (UM)	Mitoses > 4/10 HPF, necrosis, mild nuclear atypia	NAD	CD34+	30 mo no signs of local recurrence or distant metastases
9	Jakob et al[32] (2013)	62/F	Upper abdominal pain, weight loss	L	NA	Resection (UM)	High cellularity, cytological atypia, necrosis, mitoses 6/10 HPF	NAD	CD34+, CD99+, bcl2+	NA
10	Vythianathan and Yong[33] (2013)	78/M	Epigastric pain	L	17 × 13	Resection (UM)	Cellular pleomorphism, necrosis, mitoses > 4/10 HPF	NA	CD34+, CD99+, bcl2+, V+	NA
11	Song et al[34] (2014)	49/M	Abdominal pain	L+R	7.6 × 5 × 4.8	Resection (UM)	NAD	NA	CD34+, bcl2+, V+	NA
12	Du et al[4] (2015)	55/F	Hypoglycaemia, weight loss	L	15.3 × 15.5 × 15.4	Resection (UM)	NA	NAD	CD34+, bcl-2+	Local recurrence 5 yr postsurgery, resected
13	Feng et al[8] (2015)	52/F	NA	R	12	Resection (UM)	Haemorrhage, necrosis	NAD	CD34+	Local recurrence 2 yr postsurgery on L lobe managed with PEI. New lesion 6 mo after PEI
14	Silvanto et al[35] (2015)	65/M	Incidental finding	L	18	Resection (FM)	Myxoid changes, infarction, necrosis mitoses 5-7/10 HPF	NAD	CD34+, CD99+, Bcl2+	16 mo no signs of local recurrence or distant metastases
15	Maccio et al[26] (2015)	74/F	Right abdominal pain, distension	R	24 × 16	Resection (UM)	Nuclear pleomorphism, cytological atypia, necrosis, haemorrhage, mitoses 9/10 HPF	NA	CD34+, Bcl2+, V+, STAT6+	Lung, omentum, mesentery and abdominal wall metastasis at 9 mo. Patient died 4 mo later
16		80/F	Dyspnoea, cough, asthenia, abdominal pain	R	19 × 15	Palliative Chemotherapy	Highly cellular, pleomorphism, necrosis, haemorrhage, mitoses 7/10 HPF	NA	CD34+, Bcl-2+, V+, STAT6+	R lung metastasis. Patient died 5 mo later
17		65/M	Abdominal discomfort, vomiting and pain	R	3 × 2	Chemotherapy	Cytological atypia, necrosis, mitoses > 6/10 HPF	NA	CD34+, Bcl2+, V+, STAT6+	Bilateral lung metastasis. Patient died 5 mo later
18	Nelson et al[1] (2016)	61/M	Diarrhoea	R	15 × 11.5 × 7.5	Resection (FM)	Myxoid changes, mitoses > 9/10 HPF	NAD	CD34+, CD99+, Bcl2+	Extensive local recurrence and pleural metastases. Patient died 6 yr post-surgery
19	Esteves et al[7] (2018)	68/F	Incidental finding	R	13.3 × 11.6 × 13.5	Resection (FM)	Focal high-grade cytologic atypia, mitoses 25-27/10 HPF	NAD	CD34+, STAT6+	20-mo follow-up multiple bilateral pulmonary
20	De Los Santos-Aguilar et al[36] (2019)	61/M	hypoglycemia disorientation, incoherent speech	R	16 × 13 × 11	Right portal embolization. Six weeks after resection (FM)	High proliferation rate of 8/10 HPF Ki-67 15%	NAD	CD34+, Bcl2+, CD99+	No evidence of metastases
21	Yugawa et al[10] (2019)	49/F	Malaise abdominal bloating	R	14	Resection (FM)	Foci of hemorrhage and necrosis Mitosis 1/20 HPF)	NAD	STAT6+ V+	12 mo no signs of local recurrence or distant metastases
22	Present case	74/M	Hypoglycemia	L	15 × 13	Resection (FM)	Moderately cellular, pleomorphism, necrosis, mitoses 4/10 HPF Ki-67 35%		CD34+, Bcl2+, CD99+	At the moment, 2 mo no signs of local recurrence or distant metastases

IHC: Immunohistochemistry; M: Male; F: Female; L: Left; R: Right; NA: Not available; UM: Unknown margins; FM: Free margins; HPF: High-power fields; TACE: Transarterial chemoembolization; NAD: No abnormality detected; PEI: Percutaneous ethanol injection; V: Viment.