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Table 1 Clinicopathological data of 19 cases with ovarian PNETs

From: Ovarian primary primitive neuroectodermal tumor: a review of cases at PUMCH and in the published literature

Author Age (Y) Clinical manifestation Tumor size Stage Treatment Outcome OS
Aguirre, 1982 (Aguirre and Scully 1982) [10] 14 Increased AC, dyspnea, AUB R/20 × 15 cm, 1278 g IIIBa RSO + OM + appendectomy; RAD Progression DOD, 2 M
Aguirre, 1982 (Aguirre and Scully 1982) [10] 13 Abdominal pain and distention after RSO+ appendectomy 8 × 5 cm IIIa Suboptimal CRS (LSO+ pelvic mass resection); melphalan×1; (CTX + KSM) × 2 Progression DOD, 20 M
Aguirre, 1982 (Aguirre and Scully 1982) [10] 16 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] 17 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] 35 Abdominal distention and increased AC for 2 M R/18 × 10 × 10 cm IIIAa 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] 13 Intermittent fever, abdominal pain and diarrhea Ovarian tumor 1448 g, omentum tumor 1855 g IIICa 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] 29 Pelvic mass L/22 × 17 × 15 cm; R/7 × 7 × 5 cm IIICa Optimal CRS (without any residual disease) (TAH + BSO + OM + PALN biopsy); CHEM×12 Relapse DOD, 11 M
PUMCH case 1, 2003b 33 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] 25 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] 13 Abdominal pain and distention for 1 M N/A IIICa 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] 18 Pelvic mass 16 × 13 cm IIIAa 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, 2005b 59 Pelvic mass N/A IIIa Optimal CRS (with residual tumor less than 1 cm) N/A N/A
Fischer, 2006 (Fischer et al. 2006) [17] 79 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] 28 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] 31 Abdominal pain and increased AC 15 × 12 × 10 cm IIICa 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] 28 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] 16 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, 2014b 67 Abdominal distention for 4 M N/A IIIa 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, 2016b 14 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
  1. Abbreviations: AC Abdominal circumference, AUB Atypical uterine bleeding, BSO Bilateral salpingo-oophorectomy, CHEM Chemotherapy, CRS Cytoreductive surgery, CS Cesarean section, CTX cyclophosphamide, DDP cisplatin, DOD died of disease, EP + THP Etoposide, cisplatin, pirarubicin, EP + TPT etoposide, cisplatin, topotecan, EWS Ewing’s sarcoma, IFO Ifosfamide, KSM actinomycin, L Left side, LOV Left ovary, LPLND Left pelvic lymph node dissection, LS Left salpingectomy, LSO Left salpingo-oophorectomy, M Months, N/A Not available, NED No evidence of disease, OM Omentectomy, OP Operation, OS Overall survival, PAC Cisplatin, actinomycin, cyclophosphamide, PALND Paraaortic lymph node dissection, PBPC Peripheral Blood Progenitor Cell, PEB Cisplatin, etoposide, bleomycin, PEI Cisplatin, etoposide, ifosfamide, PLND Pelvic lymph node dissection, PUMCH Peking Union Medical College Hospital, PVB Cisplatin, vincristine, bleomycin, R Right side, RAD Radiotherapy, RCRS Recytoreductive surgery, ROV Right ovary, RSO Right salpingo-oophorectomy, TAH total abdominal hysterectomy, TC Paclitaxel, carboplatin, VAC Vincristine, actinomycin, cyclophosphamide, VACA Vincristine, actinomycin, cyclophosphamide, doxorubicin, VCD Vincristine, cyclophosphamide, doxorubicin, VCR Vincristine, VIA Vincristine, ifosfamide and actinomycin, VID Vincristine, ifosfamide and doxorubicin, VIE Vincristine, ifosfamide, etoposide, VIP Vincristine, ifosfamide, cisplatin, VP16 etoposide, Y years
  2. aat least this FIGO stage
  3. bThese cases are reported in this study