Table 2 SFTL cases with malignant features, local recurrence or metastatic disease

1

Fuksbrumer et al. (2000) [18]

71/F

Nil

n/a

R

14–17

n/a

Resection (UM)

Dense cellularity, increased nuclear atypia, mitoses 8/10 HPF

n/a

CD34+, bcl-2+, V+

n/a

2

Yilmaz et al. (2000) [30]

25/F

Met

Weakness, fatigue, anorexia, vomiting and progressive jaundice

L + R

32 × 30

4500

Resection (UM)

Cellularity ranged from 20–60%, necrosis, hypervascularity

NAD

V+

Bone metastasis 1 month postsurgery managed with 6 months of chemo (cyclophosphamide, adriamycin)

3

Chan et al. (2007) [35]

70/M

Rec + Met

Hypoglycaemia and progressive jaundice

R

27 × 24 × 12

4400

Failed TACE 6 weeks preoperatively followed by successful resection (UM)

Mildly atypical spindle cells, highly cellular, plemorphia, necrosis, mitoses > 20 HPF

CA-125 145U/ml (normal < 35 U/ml)

CD34+, CD99+, bcl-2+, V+

Asymptomatic widespread bilateral lung metastasis and bi-lobar recurrence at 9 months review

4

Brochard et al. (2010) [51]

54/M

Rec + Met

RUQ pain and weight loss

R

17

n/a

Resection (FM)

Moderately cellular, polymorphic cells, mitoses < 5/10 HPF

GGT 438 IU/ml (normal < 45 U/ml)

CD34+, V+, desmin+, actin+

Local recurrence 6 years postsurgery managed with resection (findings: necrotic, haemorrhagic, highly cellular, moderately atypical nuclei, mitoses >20/10 HPF, CD34, bcl-2. Negative for desmin and actin).

Cranial base metastasis managed by embolization and resection. Retroperitoneal and iliac bone metastasis weeks later, patient died 1 month after

5

Peng et al. (2011) [52]

24/F

Met

RUQ discomfort and distention

R

30 × 17 × 15

3750

TACE few days prior to resection (FM).

Highly cellular, pleomorphic, necrosis, mitoses > 10/HPF

CA-125 abnormal

CD34+, bcl-2+, V+

Craniotomy 13 days postsurgery for skull base metastases with large residual lesion. Vertebral metastasis 1 month later managed with 4× PEI and 4 rounds of chemo (adriamycin, ifosfamide, mesna). Tumour relapsed and rapidly enlarged with paralysis on 7 months review, patient died 16 months after initial surgery

6

Belga et al. (2012) [70]

66/F

Nil

Increase in abdominal girth

R

14

n/a

Resection (UM)

Mitoses > 4/10 HPF, necrosis, mild nuclear atypia

NAD

CD34+

30 months

7

Jakob et al. (2013) [72]

62/F

Nil

Upper abdominal pain and weight loss

L

n/a

n/a

Resection (UM)

High cellularity, cytological atypia, necrosis, mitoses 6/10 HPF

NAD

CD34+, CD99+, bcl-2+

n/a

8

Vythianathan and Yong (2013) [74]

78/M

Nil

Epigastric pain

L

17 × 13

n/a

Resection (UM)

Cellular pleomorphism, necrosis, mitoses > 4/10 HPF

n/a

CD34+, CD99+, bcl-2+, V+

n/a

9

Song et al. (2014) [75]

49/M

Nil

Abdominal pain

L + R

7.6 × 5 × 4.8

n/a

Resection (UM)

NAD

n/a

CD34+, bcl-2+, V+

n/a

10

Du et al. (2015) [56]

55/F

Rec

Hypoglycaemia and weight loss

L

15.3 × 15.5 × 15.4

n/a

Resection (UM)

n/a

NAD

CD34+, bcl-2+

Local recurrence 5 years postsurgery, resected

11

Feng et al. (2015) [20]

52/F

Rec

n/a

R

12

n/a

Resection (UM)

Haemorrhage, necrosis

NAD

CD34+

Local recurrence 2 years postsurgery on L lobe managed with PEI. New lesion 6 months after PEI

12

Silvanto et al. (2015) [24]

65/M

Nil

Incidental finding

L

18

n/a

Resection (lesion 1–2 mm from margins)

Myxoid changes, infarction, necrosis mitoses 5–7/10 HPF

NAD

CD34+, CD99+, bcl-2+

16 months

13

Maccio et al. (2015) [46]

74/F

Met

Right abdominal pain and distension

R

24 × 16

n/a

Resection (FM)

Nuclear pleomorphism, cytological atypia, necrosis, haemorrhage, mitoses 9/10 HPF

n/a

CD34+, bcl-2+, V+, STAT6+

Lung, omentum, mesentery and abdominal wall metastasis at 9 months review managed with chemotherapy—patient died 4 months later

14

Maccio et al. (2015) [46]

80/F

Met

Dyspnoea, cough, asthenia, abdominal pain

R

19 × 15

n/a

Palliative chemotherapy

Highly cellular, pleomorphism, necrosis, haemorrhage, mitoses 7/10 HPF

n/a

CD34+, bcl-2+, V+, STAT6+

R lung metastasis managed with palliative chemotherapy—patient died 5 months later

15

Maccio et al. (2015) [46]

65/M

Met

Abdominal discomfort, vomiting and pain

R

3 × 2

n/a

Chemotherapy

Cytological atypia, necrosis, mitoses > 6/10 HPF

n/a

CD34+, bcl-2+, V+, STAT6+

Bilateral lung metastasis managed with chemotherapy, patient died 5 months later

16

Present case (2016)

61/M

Rec + Met

Diarrhoea

R

15 × 11.5 × 7.5

n/a

Resection (FM)

Myxoid changes, mitoses > 9/10 HPF

NAD

CD34+, CD99+, bcl-2+

Extensive local recurrence and pleural metastases 6 years later—palliatively managed. Remains alive 1 month after discharge