Table 5.
Reported cases of pediatric (<18 years) spinal ependymoma in the literature (to the best of our knowledge).
| No | Lead Author; year | Mean age/sex | No of Pts | Location | Prodrome (mns) | Treatment | Pathology and postoperative course | Mean of FU (mns) |
|---|---|---|---|---|---|---|---|---|
| 1 | Nisenson [24]; 1945# | 12/M | 1 | N.M | N.M | STR+RT | Ependymoma (NOS); No Rec or Dis | 42 |
| 2 | Dereymaeker [24]; 1962# | 14/F | 1 | N.M | N.M | GTR | Ependymoma (NOS); No Rec or Dis | 60; Dead |
| 3 | Hendren [25]; 1963*# | 16/M | 1 | SC | 108 | Resection*+RT + ChT | Ependymoma (NOS); Rec+Dis (Extraneural met: inguinal lymph nodes, pelvis) | 72 |
| 4 | Sloof [17]; 1964 | 14/M | 1 | L (CE) | N.M | STR+RT | MPE; 3 times Rec at 2, 4 and 8 years. Dis(+) | 150; Dead |
| 5 | Anderson [6]; 1966 | 9/1M: 2F | 3 | SC (3) | N.M | GTR (2); STR+RT (1) | MPE (3); Rec (1): in STR+RT; no PO details for 1 pt. | 45 |
| 6 | Probhaker [25]; 1969*# | 0.83/M | 1 | SC | Since birth | Resection* | Ependymoma (NOS); Rec (+) | 24 |
| 7 | Rubinstein [26]; 1970 | 17/F | 1 | L (FT) | N.M | STR+RT | Ependymoma (NOS); Rec afte 3 yrs, Dis after 6 yrs; Extraneural metastasis (Lungs, pleural and lymph nodes): DOD after 29 yrs | 348; Dead |
| 8 | Wolf [27]; 1972**# | 4/M | 1 | SC | N.M | Resection* | Ependymoma (NOS); Rec+Dis (extraneural metastas: Lungs and inguinal lymph nodes) | 228 |
| 9 | Payne [28]; 1973 | 11/F | 1 | L (CE) | Acute; Posttrauma | Resection*+RT | MPE; No Rec or Dis | 36 |
| 10 | Scharrer [25]; 1974*# | 7/F | 1 | SC | N.M | Resection* | Ependymoma (NOS); Rec/Dis (N.M) | N.M |
| 11 | Scott [29]; 1974 | 17/M | 1 | L | N.M | STR+RT | MPE; Rec (after 5 yrs); Dis (No) | 276 |
| 12 | Ammerman [24]; 1975# | 16/F | 1 | N.M | N.M | GTR | MPE; No Rec or Dis | 24 |
| 13 | Cameron [30]; 1976 | 13/F | 1 | Multiple (supra-, infratentorial and spinal) | N.M | Resection* (4 operations in 4 yrs) | MPE; Rec? and Dis? Or multiple at presentation | 78 |
| 14 | Fisher [24]; 1977# | (6–14)/4M: 4F | 8 | N.M | N.M | GTR (5); STR+RT (3) | Ependymoma grade I (5); Ependymoma grade II (2); Ependymoma NOS (1); Dead at PO 36 mn | 43.5 |
| 15 | Mavroudis [31]; 1977 | 7/M | 1 | L (CE) | N.M | GTR | MPE; Rexploration after 2yrs (No lesion); Rec (after 24 yrs); Extraneural metastasis to lungs after 29 yrs) | 348 |
| 16 | Mork [10]; 1977 | 13/2M: 3F | 5 | N.M | N.M | NOS | MPE and Classic ependymoma (NOS) | N.M |
| 17 | Bale [25]; 1980*# | 4/F | 1 | SC | 1 | Resection* | Ependymoma (NOS); Rec/Dis (No) | 168 |
| 18 | Mork [32]; 1980 | 12/M | 1 | TL | 6 | STR+RT | Anaplastic ependymoma; Rec (after 2 yrs), Dis (after 6 yrs) | 78 |
| 19 | Morris [33]; 1983 | 10/F | 1 | L (CE) | 60 | NTR+RT | Ependymoma grade I; Rec and Dis (Extraneural metastasis: lungs and hip) after 6 yrs | 144 |
| 20 | Chan [34]; 1984 | 10.6/5M: 2F | 14* | L (7) [4: CE+ 3: FT] | 10.6 | GTR (5); STR+RT (2) | MPE (7); Rec in 2 GTR;Dis in 2 pts | 105.6 |
| 21 | Helwig [35]; 1984 | 17/F | 1 | SC | 4 | Resection* | MPE; Rec+Dis after 6 mns (DOD) | 60; Dead |
| 22 | Matsuo [36]; 1985 | 11/F | 1 | SC | N.M | GTR | MPE; Rec/Dis (No) | 7 |
| 23 | Sonneland [18]; 1985 | N.M/10M: 5F | 15 | L (15) | N.M | NOS | MPE (15); Rec (5 pts); 3 pts DOD | 205.1 |
| 24 | West [37]; 1985 | 7.9/4M | 4 | T (2); L (CE) (1); Extrasacral (CS) (1) | N.M | STR+RT+ChT (1); STR+RT (1); Bx+RT (2) | Ependymoma (Low grade) (4); No Rec or Dis | 102.5 |
| 25 | Ciraldo [38]; 1986 | 0.83/2M: 3F | 5 | SC (5) | Since birth | GTR (5) | MPE (5); Rec/Dis (No) | 26 |
| 26 | Chou [27]; 1987**# | 12.5/1M: 1F | 2 | SC (2) | N.M | Resection* (2) | MPE (2); Rec or Dis (N.M) | N.M |
| 27 | Murphy [27]; 1987**# | 0.09/M | 1 | SC | Since birth | N.M | MPE; Rec/Dis (No) | N.M |
| 28 | Di Marco [39]; 1988 | 17/F | 1 | L | N.M | STR+RT | Low grade ependymoma; Rec after 169 months (treated) | 182 |
| 29 | Kramer [25]; 1988 | 15/M | 1 | SC | N.M | GTR | MPE; Rec (twice)+Dis (extraneural: inguinal, lymph nodes and skin) | 240 |
| 30 | Pulitzer [40]; 1988 | 0.6/3M: 1F | 4 | SC (4) | Since birth | Resection* | MPE (4); Rec/Dis (No) | 7–40 |
| 31 | Naidu [41]; 1989 | 14/M | 1 | L (CE) | 6 | Resection*+RT | Ependymoma (NOS); Dis after one yr, Rec after two yrs | 24 |
| 32 | Fujiyama [42]; 1990 | 6/F | 1 | TL | 6 | GTR+RT | Anaplastic Ependymoma; Dis and Rec after 3 yrs | 89 |
| 33 | Le Marc’hadour [43]; 1991 | 14/F | 1 | SC | 6 | Resection* | MPE; Rec/Dis (No) | 24 |
| 34 | Wen [44]; 1991 | 12/NOS | 2 | L (2) | 18 (Median) | STR+RT (2) | MPE (2); Both had Dis in spine at 32 and 48 mns; both DOD at 209 and 110 mns. | 159; Dead |
| 35 | Gupta [45]; 1992 | 1.5/M | 1 | SC | Since birth | NOS | MPE; since birth, Dis (metastasis to inguinal lymph nodes) PO aggressive tm | 18 |
| 36 | Serour [46]; 1992 | 11/M | 1 | SC | N.M | GTR | MPE; Rec/Dis (No) | 12 |
| 37 | Clover [47]; 1993 | 16/2M: 1F | 3 | Multiple [LS+CE] (2); LS (1) | 24 (Median) | STR+RT (2); STR (1) | MPE (3); Rec (1) after 3.2 yrs in STR+RT (F-U: 6 yrs -AWD) | 52 |
| 38 | Gagliardi [24]; 1993 | 13.5/3M: 1F | 4 | L [FT] (4) | 12 | GTR (2); STR+RT (1); STR (1) | MPE (4); 1 Rec in STR | 93.3 |
| 39 | Lunardi [48]; 1993 | 10–16/NOS | 9 | Int [NOS] (9) | NOS | GTR+RT (3); GTR (4); STR+RT (2) | Low grade ependymoma (NOS) (9); Rec (2) | 48 [Median] |
| 40 | Ross [16]; 1993 | 10.7/2M: 1F | 3 | N.M | N.M | GTR+RT (3) | MPE (3); Rec/Dis (No) | 93.3 |
| 41 | Waldron [23]; 1993 | 11.5/NOS | 4 | LS (4) | N.M | Resection*+RT (4) | MPE (1), Classic Ep (1), Anaplastic (1), NOS (1); Rec (1); Dis (3): 2 of Dis were DOD; 1 Reopere+RT (30 yrs: NED); 1 Reopere (5 yrs: NED) | 114.8 |
| 42 | Botti [49]; 1994 | 10.5/N.M | 1 | SC | 4 | GTR | MPE; Rec/Dis (No); but the pt was presented with Dis. | N.M |
| 43 | Do-Dai [50]; 1995 | 12/F | 1 | CT | 5 dys | Bx+ChT | MPE; Rec: (+), Dis (+); Dis at prersentation | 6 |
| 44 | Mottl [51]; 1997 | N.M/F | 1 | L (FT) | N.M | Partial resection+RT | WHO grade I Ependymoma; Rec: No, Dis: No; alive | N.M |
| 45 | Nagib [52]; 1997 | 9.3/1M: 2F | 3 | TL (FT) [1]; L (CE) [2] | 1.7 | GTR (3) | MPE (3); Rec: No, Dis (1) | 42.7 |
| 46 | Ilhan [53]; 1998 | 8/M | 1 | SC | 3 | GTR | MPE; Rec/Dis (No) | 20 |
| 47 | Lonjon [54]; 1998 | 14/11M: 9F## | 32## | CM (2); C (4); CT (5); T (8); TL (1); 12 MPEs were located in LS | 5–84 | GTR+RT (1); GTR (13); STR+RT (5); STR (1); No details about treatment of MPEs | MPE (12), low grade ependymoma (17), high grade ependymoma (3); Rec (2) after 2 and 3 yrs (both were treated with STR+RT; Dis (4): 2 of them were anaplastic (In 1 GTR+R, 1 GTR and 2 STR+RT). 11 presented with syrinx, 3 pts had NF Type 2. 3 operations for kyphoscoliosis, 1 operation for shunting hydrocephalus | 67 [Median](25–177) |
| 48 | Graf [55]; 1999 | 15/M | 1 | TL (CE) | 3 | GTR | MPE; Rec (+): after 32 yrs; Neuroaxial Dis to Lungs and Liver (+) after 37 yrs and dead | 480; Dead |
| 49 | Johnson [56]; 1999 | 7/M | 1 | SC | 18 | GTR | Grade II ependymoma; No Rec/Dis | 96 |
| 50 | Ohata [57]; 1999 | 16/2M | 2 | CM (1); CT (1) | N.M | GTR (1); STR+RT (1) | Grade II (1) and grade III (1); No Rec/Dis. | 86.2 |
| 51 | Aktug [27]; 2000 | 5/M | 1 | SC | 6 | GTR | MPE; Rec/Dis (No) | 36 |
| 52 | Chinn [13]; 2000 | 11/2M: 1F | 3 | Multiple (2); L (1) | 11.3 | GTR+RT (1); GTR (1); STR+RT (1) | MPE (3); Rec (1) in GTR; Dis in all pts (3): 2 at presentation, one after 7 mns of GTR | 24 |
| 53 | Constantini [9]; 2000 | <21/NOS | 26 | Int (NOS)** | 11.6** | NOS** | Ependymoma (19), MPE (7); 10-yrs survival rate is 86%; 4-yr PFS rate is 75%** | 85.1** |
| 54 | Merchant [58]; 2000 | 8.6/3M: 5F | 8 | CM (1); CT (1); TL (3); L (1); LS (2) | N.M | GTR+RT+ChT (1); STR+RT+ChT (4); STR+RT (3) | MPE (4), ependymoma (4); Rec (2): in STR+RT+ChT both were ependymomas; Dis (4): 3 were MPEs which were treatd with STR+RT+ChT (2) and STR+RT (1), 1 ependymom which was treated as GTR+RT+ChT | 85.9 |
| 55 | Nishio [59]; 2000 | 15/2M | 2 | C+T (1); L (1) | 12.5 | GTR | Ependymoma grade II/III (NOS); Rec/Dis (No) | 137 |
| 56 | Gelabert-Gonzalez [60]; 2001 | 15/F | 1 | FT (2 tms: L+S) | 2 | GTR | MPE (both lesion); Rec/Dis (No) | 6 |
| 57 | Helseth [61]; 2001 | 8.76/3M: 2F | 5 | L (CE): 5 | N.M | GTR (2); STR+RT (3) | MPE (3), grade II (2); Dis (No); Rec (2): 1 GTR, 1 STR+RT | 164 |
| 58 | Hirose [62]; 2001 | 14.1/7M: 2F | 9 | C (2); L (1); CE (6) | N.M | N.M | MPE (6), Ependymoma grade II (3); Rec and Dis: N.M (Genetic study) | N.M |
| 59 | Hanbali [63]; 2002 | 17/M | 1 | T+L (Multiple) | N.M | GTR (both tms) | Grade II ependymoma; Rec/Dis (No) | 31 |
| 60 | Goto[64]; 2003 | 12.5/2M | 2 | CM (1); CT (1) | 13 | GTR (1); STR+RT (1) | Ependymoma (NOS); Rec/Dis (No) | 206.5 |
| 61 | Hallacq [15]; 2003 | 13/M | 1 | L+S (Multiple) | 5 | GTR | MPE; Rec/Dis (No) | 60 |
| 62 | Wolf [65]; 2003 | 1.5/F | 1 | TL | 0.5 | GTR | MPE; Rec/Dis (No) | 33 |
| 63 | Peker [66]; 2004 | 9/F | 1 | C (Int) | 4 | GTR | Low grade ependymoma (NOS); Rec/Dis (No) | 108 |
| 64 | Sebire [67]; 2004 | 13/F | 1 | SC | N.M | Resection* | MPE; Rec/Dis (NOS). | N.M |
| 65 | Fassett [14]; 2005 | 11.2/4M: 1F | 5 | L (5) | 18.7 | GTR+RT (1); GTR (1); STR+RT (3) | MPE (5); Rec (1): STR+RT; Dis (4). | 58.2 |
| 66 | Lin [68]; 2005 | 12/M | 1 | C | N.M | GTR | Ependymoma grade II; Rec/Dis (No) | 48 |
| 67 | Tubbs [69]; 2005 | 2/F | 1 | S | 6 | GTR | MPE; Rec/Dis (N.M) | N.M |
| 68 | Akyurek [22]; 2006 | 15/NOS | 2 | TL (1); L (1) | N.M | GTR+RT (1); GTR (1) | MPE (2); Rec (2): GTR+RT at 15 mns and GTR at 20 mns | 131 |
| 69 | Bagley [7]; 2007 | 12.6/10M: 4F | 14 | T (1); TL (4); L (1); LS (5); S (1); Multiple (2) | 23.2 | GTR (7); STR+RT (2); STR (2); Resection* (1); Bx+RT (1); Bx (1) | MPE (14); Rec (11): in 4 GTR, 2 STR+RT, 2 STR, 1 Bx+RT, 1 Bx, 1 Resection*; Dis (6) | 63.7 |
| 70 | Mridha [70]; 2007 | 13/M | 1 | TL | 18 | GTR | MPE; Rec and Dis (1) (After 36 mns and 38 mns, repectively) | 38 |
| 71 | Jatana [71]; 2008 | 11/M | 1 | N/A | N/A | GTR | MPE; Rec and Dis (1); NF-2 pt | N/A |
| 72 | Kabler [72]; 2008 | 16/M | 1 | L | N.M | GTR | MPE; No Rec or Dis | 1.5 |
| 73 | Cho [73]; 2009 | 16/M | 1 | C (Int) | N.M | Bx+RT (No response) thenGTR | Grrade III Ependymoma; No Rec or Dis. (After 6 mns follow-up had lost) | 6 |
| 74 | Al-Halabi [12]; 2010 | 15.4/5M: 2F | 7 | TL (1); L (5); LS (1) | 12 | GTR+RT (3); GTR (1); STR+RT (2); Bx then STR+RT after progression(1) | MPE (7); Rec after STR+RT (1); Rec+Dis (2): after GTR+RT; presentation with cranial and spinal ependymomas (2) | 78 |
| 75 | Benesch [4]; 2010 | 13.6 [median]/12M: 17F | 29 | CM (1); C (3); T (2); LS (6); 2 regions (14); >2 regions (3) | 2 [Median] | GTR+RT+ChT (3); GTR+RT (5); GTR (9); STR+RT+ChT (2); STR+RT (2); STR+ChT (2); STR (3); Bx+RT+ChT (2); Bx+RT (1) | MPE (6), grade II (17), grade III (6); 1 pt grade III died PO65.mn; progressive disease or relapse: 2 GTR (both are MPEs) and 5 less than GTR (2: MPEs, 2: typical and 1: anaplastic ependymomas) | 50.4 [Median] |
| 76 | Dulai [74]; 2010 | 8/F | 1 | T (Int) | N.M | STR then GTR | MPE; Rec (PO 2 yrs)+Dis (PO 5 yrs) | 108 |
| 77 | Kaner [75]; 2010 | 17/F | 1 | CT (1) | 23.2 | GTR | Classic Ependymoma; No Rec or Dis | 14 |
| 78 | Moon [76]; 2010 | 11/M | 1 | Multiple (L+S) | 4 | GTR+RT | Classic Ependymoma; No Rec or Dis | 36 |
| 79 | Boström [77], 2011 | 12/M | 1 | TL | N.M | GTR+RT | Anaplastic ependymoma; No Rec or Dis | 30 |
| 80 | Chakraborti [78], 2012 | 0.92/F | 1 | SC | 1 | GTR | MPE with anaplastic component; Rec and Dis after 6 weeks | 13.5 |
| 81 | Choi [79]; 2012 | 13.7/1M: 2F | 3 | L (1); LS (1); T2-S1 (1) | 8.5 | GTR (1); STR+RT (1); Bx+RT (1) | MPE (3); No Rec; Dis (1): Bx+RT | 13 |
| 82 | Stephen [80]; 2012 | 13/11M: 6F | 17 | C (1); C+T (1); T (3); L (9): [FT (5)+CE (1), Int (3)]; LS (2); S (1) | 9.5 | GTR+RT (5); GTR+Proton (1); GTR (9); STR+RT (2) | MPE (7), ependyomoma (9), anaplastic (1); Rec (4): all are MPEs (Rec after an average of 37.8 mns). 1 pt had NF Type 2 (Rec in primary site). | 58.8 (4–203) |
| 83 | Agbahiwe [21]; 2013 | 14.3/9M | 9 | TL (2); L (4); LS (2); S (1) | N.M | GTR+RT (1); GTR (4); STR+RT (3); STR (1) | MPE (9); Dis in GTR+RT (1), and 2 Rec in GTR and STR | 87 [Median] (9–317) |
| 84 | Becco de Souza [81]; 2013 | 13/M | 1 | L | 3 | GTR | MPE; Rec/Dis (No) | 2 |
| 85 | Liu [5]; 2013 | 11.5/3M: 1F | 4 | C (3); CT(1) | 2.7 | GTR+RT+CT (2); GTR (1); STR (1) | Anaplastic (4); Rec in 1 STR (dead after 23 mns) | 50.7 for 3 pts, 4th (N/A) |
| 86 | Pedziwiatr [82]; 2013 | 13.3/15M: 13F | 28 | L (CE) (17); Other spinal (8); Multiple (3) | 3–60 | GTR+RT (18); GTR (2); STR+RT (2); Partial resection+RT (4); Bx+RT (2).Out of them 3 recieved ChT | MPE (13), Typical ependymoma (12), Anaplastic ependymoma (3); DOD (5): Rec (2), Rec+Dis (3): Out of them 2 anaplastic; DOC (1) after GTR | 104 [Median] 36–300 |
| 87 | Cimino[83]; 2014 | 10.3/6M: 5F | 11 | Extradural (7): [SC (5)+ Lower back (1)+ Pelvis (1)]; L (2); LS (2) | N.M | GTR (11) | MPE (11); Rec in 2 Lumbar tms followed-up for 5 and 10 years; No Dis | N.M |
| 88 | Khalatbari [84]; 2014 | 15/M | 1 | TL | Acute | GTR | MPE; Rec/Dis (No) | 48 |
| 89 | Lundar [85]; 2014 | 10.9/7M: 3F | 10 | TL (1); L (7); LS (2) | N.M | GTR (6); STR+RT (4) | MPE (6), Ependymoma grade II (2), grade III (2); Rec (1): MPE/GTR; Dis (1): MPE/STR+RT; Rec+Dis (3): 1 MPE/STR +RT, 1 Ep gr II/GTR and 1 Ep gr III/GTR | 268 |
| 90 | Pencovich [86]; 2014 | 15.5/5M: 1F | 6 | C (1); T+TL (1); T+L+LS (1); L+S (3) | 10 | GTR (2); STR+RT (2); STR (2) | MPE (5); Ependymoma grade II (1); All pts presented with regional metastasis (Multiple); No Rec or Dis | 55 |
| 91 | Lin [87]; 2015 | 11.9/30M: 34F | 64 | Spinal cord (62); CE (2) | N.M | GTR+RT (4); GTR (17); STR+RT (11); STR (5); No resection [only RT] (23); Unknown (4) | Grade II ependymopma (64); No details about Rec or Diss. [The patients were reported here thought to be duplicated in previous published studies] | 110.4 [Median] |
| 92 | Current study; 2018 | 13.3/2M: 1F | 3 | T (1); L (2) | 9.1 | GTR (2); NTR (1) | MPE (2) and Classic ependymoma (1); Dis (1): GTR (MPE) | 51.3 |
AWD – alive with disease; Bx – biopsy; C – cervical; CE – Cauda equine; ChT – chemotherapy; CM – cervicomedullary; CT – cervicothoracic; Dis – neuroaxis dissemination; DOC – dead of other causes; DOD – dead of disease; dys – days; F – Female; FT – filum terminale; GTR – gross total resection; L – lumbar; LS – lumbosacral; M – male; mns – months; N/A – not available; NED – no evidence of disease; NF – neurofibromatosis; N.M – not mentioned; No – number; NOS – non otherwised specifised; PFS – progression-free survival; PO – postoperative; pts – patients; Rec – recurrence; Resection* – surgical resection borders had not been defined; RT – radiotherapy; S – sacral; SC – sacrococcygeal; STR – subtotal resection; T – thoracic; TL – thoracolumbar; wks – weeks; yrs – years.
*
Chan et al.
[34] series contain 14 pediatric ependymoma patients, while only details of 7 patients of childhood MPE were reported.
**
Constantini et al.
[9] reported 164 patients 21 years of age and younger in whom intradural-intramedullary tumors were resected. Their study is not specific for ependymomas.
#
We could not reach to the original studies, the data included in this table about the studies, which were indicated with (#), were obtained from Gagliardi et al.’s study [24].
##
Lonjon et al.
[54] series contain 32 pediatric ependymoma patients, while only details of 20 patients of childhood ependymomas were reported, 12 MPEs were excluded.
*#
The data of these studies were obtained from Kramer et al. study (1988) [25].
**#
The data of these studies were obtained from Aktug et al. study (2000) [27].