Table 1 Clinicopathological data of 19 cases with ovarian PNETs

Aguirre, 1982 (Aguirre and Scully 1982) [10]

Increased AC, dyspnea, AUB

R/20 × 15 cm, 1278 g

IIIB^a

RSO + OM + appendectomy; RAD

Progression

DOD, 2 M

Aguirre, 1982 (Aguirre and Scully 1982) [10]

Abdominal pain and distention after RSO+ appendectomy

8 × 5 cm

III^a

Suboptimal CRS (LSO+ pelvic mass resection); melphalan×1; (CTX + KSM) × 2

Progression

DOD, 20 M

Aguirre, 1982 (Aguirre and Scully 1982) [10]

Abdominal pain; family history of tumor

L/15 cm, 464 g

IA

LSO+ appendectomy; TAH + RSO 6 days later; no adjuvant therapy

Relapse, 36 M

DOD, 47 M

Aguirre, 1982 (Aguirre and Scully 1982) [10]

Pelvic mass

L/18 cm

IA

LSO + ROV biopsy; no adjuvant therapy

Complete remission

NED, 84 M

Kanbour-Shak, 1993 (Kanbour-Shakir et al. 1993) [11]

Abdominal distention and increased AC for 2 M

R/18 × 10 × 10 cm

IIIA^a

Complete surgical staging (TAH + RSO + LS (history of LOV resection) + OM + PLND+PALND); BEP × 6

Complete remission

NED, 42 M

Lawlor, 1997 (Lawlor et al. 1997) [12]

Intermittent fever, abdominal pain and diarrhea

Ovarian tumor 1448 g, omentum tumor 1855 g

IIIC^a

Suboptimal CRS (partial resection) (RSO + OM); (DDP + VP16 + doxorubicin+CTX) Q4W × 4, PBPC

Complete remission

NED, 18 M

Kawauchi, 1998 (Kawauchi et al. 1998) [13]

Pelvic mass

L/22 × 17 × 15 cm; R/7 × 7 × 5 cm

IIIC^a

Optimal CRS (without any residual disease) (TAH + BSO + OM + PALN biopsy); CHEM×12

Relapse

DOD, 11 M

PUMCH case 1, 2003^b

Backache and lower limb aching

12.0 × 8.4 cm

IV

CHEM×11; RAD×2; PEB × 2; PVB × 1; RAD× 1; palliative surgery

Progression

DOD, 24 M

Demirtas, 2004 (Demirtas et al. 2004) [14]

Abdominal pain and distention for 3 M

L/8 cm

IC1

Fertility-sparing complete surgical staging (LSO + OM + partial ROV + PLND+PALND); BEP × 4; VIP × 6 when recurrent

Relapse, 3 M

NED, 28 M, CS twice

Chow, 2004 (Chow et al. 2004) [15]

Abdominal pain and distention for 1 M

N/A

IIIC^a

Optimal CRS (without any residual disease) (BSO + OM + partial bladder); PEB × 2; RCRS and RAD

Progression

DOD, 17 M

Kim, 2004 (Kim et al. 2004) [16]

Pelvic mass

16 × 13 cm

IIIA^a

Optimal CRS (without any residual disease) (RSO + Partial LOV + OM + PLN/PALN biopsy); TC × 6; RAD when recurrent; OP when intestinal obstruction; VACA

Relapse, 4 M

Died of septic shock, 10 M

PUMCH case 2, 2005^b

Pelvic mass

N/A

III^a

Optimal CRS (with residual tumor less than 1 cm)

N/A

N/A

Fischer, 2006 (Fischer et al. 2006) [17]

Abdominal distention for 4 M

6 × 4 cm

III

Optimal CRS (TAH + BSO + OM + rectum and sigmoid colon resection+ partial bladder+ peritoneum+ PALND); CHEM

Complete remission

NED, 6 M

Ateser, 2007 (Ateser et al. 2007) [18]

Stomach ache, nausea and vomiting at 12 weeks of gestation

25 × 30 cm

N/A

RSO; doxorubicin+CTX + VCR; (at 37 weeks of gestation) CS + Optimal CRS (without any residual disease) (TAH + LSO + OM+ metastatic lesion); altered with VACA and PEI, RAD

Progression

DOD, 13 M

Anfinan, 2009 (Anfinan et al. 2008) [19]

Abdominal pain and increased AC

15 × 12 × 10 cm

IIIC^a

Optimal CRS (without any residual disease) (TAH + BSO + OM); Altered with VID and VIA; docetaxel+DDP

Progression

DOD, 15 M

Ostwal, 2012 (Ostwal et al. 2012) [20]

Pelvic mass for 2 M

12 × 11 × 10 cm

N/A

LOV resection; CHEM (Altered with VIE, VAC and VCD for 2 courses); CRS when recurrent

Relapse, 18 M

DOD, 18 M

Chu, 2014 (Chu et al. 2014) [21]

Pelvic mass

16.5 × 9.2 cm

IC

Fertility-sparing complete surgical staging (LSO + LPLND+OM); TC × 6

Complete remission

NED,13 M

PUMCH case 3, 2014^b

Abdominal distention for 4 M

N/A

III^a

Optimal CRS (without any residual disease) (history of TAH + LSO) (RSO + OM + appendectomy+partial small intestine and sigmoid colon resection); without adjuvant therapy

Progression

DOD, 6 M

PUMCH case 4, 2016^b

Abdominal distention for one week

30 cm

IIIC

Optimal CRS (without any residual disease) (RSO + LOV biopsy+ OM + appendectomy+metastasis); TC × 1

Progression

DOD, 5 M