Table 3.
Thirty-seven reported cases of primitive neuroectodermal tumor in the vulva
| Ref. | Age | Size (cm) | Immunohistochemistry | Molecular/ cytogenetic analysis | MATOD | Therapy | Follow-up | Relapse | Outcome |
| Present case, 2019 | 40 mo | 3.3 × 5 × 2.5 | Vimentin, CD99 (+) | NA | No | S + Ch (VAC/IE) | 14 mo | YES | Death |
| Pei et al[28], 2018 | 33 yr | 0.5 × 0.5 | PAS, CD99, vimentin (+) | EWSR1 gene (+) | Yes | S + RT + Ch | 15 mo | NO | Survival |
| Chiang et al[29], 2017 | 65 yr | NA | CD99, NSE, SYN, CD56, S100, FLI-1 (+) | FISH (+) | NA | NA | NA | NA | NA |
| Kakoti et al[30], 2017 | 16 yr | 15 × 10 | CK, vimentin, CD99, FLI-1 (+) | NA | No | Ch (VDC/IE) | NA | NA | Death |
| Tunitsky-Bitton et al[31], 2015 | 15 yr | 5 | CD99 (+) | RT-PCR (+) | No | S + Ch (VDC/IE 14 cycles) | 29 | NO | Survival |
| Huang et al[32], 2015 | 20 yr | 8 × 10 × 10 | CD99, vimentin, NSE (+) | NA | Yes | S | 2 wk | NA | Death |
| Rekhi et al[33], 2015 | 10 yr | 12 × 8 | MIC2/CD99, FLI-1(+) | FISH (+) | NA | S + Ch (VIME 5 cycles) | 18 mo | YES | Survival |
| Narayanan et al[34], 2014 | 17 yr | 3 × 2 × 2 | MIC2 (+) | NA | NA | S + RT + Ch (VAC/IE) | 22 mo | YES | Death |
| Matsuda et al[35], 2014 | 60 yr | NA | MIC-2, synaptophysin, NSE, neurofilament antibodies (+) | NA | No | S + RT + Ch (VACI) | 48 mo | YES | Survival |
| Xiao et al[36], 2014 | 20 yr | NA | CD99, NSE (+) | NA | NA | Not done | NA | NA | Death |
| Xiao et al[36], 2014 | 36 yr | NA | CD99, Syn, NSE (+) | NA | NA | S + Ch (PEI, 4 cycles; PAC, 2 cycles) | 13 mo | YES | Death |
| Che et al[37], 2013 | 37 yr | 5 × 3.5 × 3; 3 × 2 × 1.2 | CD99, vimentin, FLI-1 (+) | NA | NA | S + Ch (VAC) | 12 mo | YES | Survival |
| Tang et al[38], 2012 | 17 yr | 5.5 × 5 × 5 | CD99 and FLI-1 (+) | NA | NA | S | NA | NA | LS |
| Tang et al[38], 2012 | 25 yr | 2 × 2 × 2 | CD99 and FLI-1 (+) | NA | NA | S | NA | NA | LS |
| Yang et al[39], 2012 | 20 yr | 20 × 10 × 7 | CD99 and NSE (+) | RT-PCR (+) | Yes | Ch | NA | NA | Death |
| Kelling et al[40], 2012 | 18 yr | 1.7 × 9 × 1.5 | CD99 and vimentin (+) | RT-PCR (+) | Yes | S + RT + Ch | 3 mo | NA | Survival |
| Anastasiades et al[41], 2012 | 28 yr | 3 | CD99 (+) | NA | No | S + RT + Ch | 12 mo | YES | Death |
| Dong et al[42], 2012 | 20 yr | 11 × 7.7 × 6.5 | CD99, NSE, CK (AE1/AE3) and Syn (+) | NA | Yes | S | 3 mo | NA | Death |
| Dong et al[42], 2012 | 12 yr | 3.1 | CD99, NSE, CK (AE1/AE3) and Syn (+) | NA | Yes | NA | 13 mo | NA | Survival |
| Dong et al[42], 2012 | 35 yr | NA | CD99 and NSE (+) | NA | Yes | S + Ch | 20m | YES | Death |
| Halil et al[43], 2011 | 14 yr | NA | CD99 (+) | NA | NA | S + RT + Ch | 9 mo | YES | Death |
| Boldorini et al[44], 2010 | 52 yr | NA | CD99, CK(AE1/AE3) and vimentin (+) | FISH (+) | No | S + RT + Ch(VAI/IE) | 12 mo | NO | Survival |
| Dadhwal et al[45], 2010 | 20 yr | 20 × 15 × 10 | CD99 (+) | NA | Yes | S | 20 d | YES | Death |
| Cetiner et al[46], 2009 | 23 yr | 4 × 4 | CD99 and vimentin (+) | RT-PCR (+) | Yes | S + R + Ch (VDC/IE) | 7 yr | NO | Survival |
| Cetiner et al[46], 2009 | 29 yr | NA | CD99 and vimentin (+) | RT-PCR (-) | NA | S + Ch | 51 mo | NO | Survival |
| Fong et al[47], 2008 | 17 yr | 0.7 × 0.6 × 0.2; 2.1 × 1.7 × 1.5 | CD99 and Fli-1 (+) | RT-PCR (+) | No | S + Ch (VDC) | 48 mo | NO | Survival |
| McCluggage et al[48], 2007 | 19 yr | 4 | CD99 and FLI-1 (+) | RT-PCR and FISH (-) | NA | S + Ch | NA | NA | NA |
| McCluggage et al[48], 2007 | 20 yr | 6.5 | CD99 and FLI-1 (+) | FISH (+) | NA | S | NA | NA | Death |
| McCluggage et al[48], 2007 | 40 yr | 3 | CD99, FLI-1 (+) | FISH (+) | NA | S + Ch | 12 mo | NA | Survival |
| Moodley et al[49], 2005 | 26 yr | 4 × 5 | NA | NA | No | Ch + RT | NA | YES | Death |
| Takeshima et al[50], 2001 | 45 yr | 4 (at recurrence) | Neuron specific enolase, vimentin, HBA 71 (+) | NA | No | S | 1 yr (at recurrence) | YES | Survival |
| Lazure et al[51], 2001 | 15 yr | 20 | CD99 (+) | RT-PCR (+) | NA | S + Ch | 7 mo | NO | Survival |
| Vang et al[52], 2000 | 28 yr | 0.9 | CD99 (+) | RT-PCR (+) | NA | S + Ch | 18 mo | NA | Survival |
| Paredes et al[53], 1995 | 29 yr | 5 | Vimentin (+) | NA | NA | S + RT + Ch (6 cycles of VAC) | 8 mo | NO | Survival |
| Nirenberg et al[54], 1995 | 20 yr | 12 | PAS (+) | NA | NA | S + RT + Ch (VA) | 10 mo | YES | Death |
| Scherr et al[55], 1994 | 10 yr | 6.5 × 5.5 × 2.0 | HBA-71 (+) | NA | No | S | NA | NA | NA |
| Habib et al[56], 1992 | 23 yr | 1.5 | CK, EMA (+) | NA | NA | NA | NA | NA | NA |
+: Positive; MATOD: Metastatic at the time of diagnosis; S: Surgery; Ch: Chemotherapy; RT: Radiotherapy; NA: Not available; FISH: Fluorescence in situ hybridization; RT-PCR: Reverse transcription-PCR; PEI: Cisplatin, ifosfamide, and etoposide; PAC: Cisplatin, cyclophosphamide, and actinomycin D; VAC: Vincristine, actinomycin D, and cyclophosphamide; IE: Ifosfamide and etoposide; VDC: Vincristine, doxorubicin, and cyclophosphamide; VIDE: Vincristine, ifosfamide, doxorubicin, and etoposide; VIME: Vincristine, ifosfamide, mesna, and etoposide; LS: Loss to follow-up.