Table 1 Reported cases of CNSET
No | Authors | Age | Sex | Symptoms | Surgical treatment | Chemotherapy | Outcome/follow-up | Associated history or disease |
|---|---|---|---|---|---|---|---|---|
1 | Heywood et al. [2] 2002 | 28 | F | Incidental | Trisegmentectomy IVB, V, and VI wedge resection VII | ☓ | Recurrence/72 months | Persistent fetal hemoglobin, OCP |
2 | Hill et al. [4] 2005 | 2 | M | Abdominal mass | Partial hepatectomy | Post | Alive/84 months | |
3 | 6 | F | Incidental | Gross excision | ☓ | Alive/58 months | ||
4 | 6 | F | Incidental | Gross excision | ☓ | Alive/8 months | ||
5 | 14 | F | Abdominal mass | Left lobectomy | Post | Recurrence/11 years | ||
6 | Heerema-McKenney et al. [3] 2005 | 2 | M | Incidental | Gross excision | ☓ | Alive/8 months | Nephroblastomatosis and Wilms tumor |
7 | 3 | F | Incidental | Gross excision | Pre/post | Alive/6 months | ||
8 | 4 | M | Incidental | Enucleation | Post | Alive/36 months | ||
9 | 11 | F | Cushingoid features, abdominal mass | Left lobectomy | ☓ | Alive/24 months | Cushing syndrome | |
10 | 12 | F | Cushingoid feature, abdominal mass | Right hepatectomy | ☓ | Alive/168 months | Cushing syndrome | |
11 | 14 | F | Ileus | Gross excision | Post | Recurrence/12 months | BWS, hypoplastic kidney, omphalocele | |
12 | Brodsky et al. [5] 2008 | 17.5 | F | Cushingoid feature, abdominal pain | Left lobectomy + partial hepatectomy | ☓ | Recurrence/12 months | Cushing syndrome |
13 | Wirojanan et al. [6] 2008 | 2 | M | ND | Resection | Post | Alive/84 months | Fragile X syndrome |
14 | Meir et al. [8] 2009 | 2.5 | F | Incidental | Right lobectomy | ☓ | Alive/8 months | Asymptomatic hydronephrosis |
15 | Makhlouf et al. [7] 2009 | 2 | F | Incidental | Partial hepatectomy | ☓ | Alive/6 months | |
16 | 14 | F | Incidental | Partial hepatectomy | Post | Alive/264 months | ||
17 | 15 | F | Incidental | Partial hepatectomy | ☓ | Alive/151 months | ||
18 | 16 | M | Cushingoid feature | Partial hepatectomy | ☓ | Alive/56 months | Cushing syndrome | |
19 | 18 | F | Incidental | Transplant | ☓ | Death/40 months (no recurrence) | ||
20 | 19 | M | Incidental | No (needle biopsy) | ☓ | Lost to follow-up | ||
21 | 32 | F | Incidental | Partial hepatectomy | ☓ | Alive/13 months | ||
22 | 33 | F | Incidental | Partial hepatectomy | ☓ | Alive/14 months | ||
23 | Rod et al. [9] 2009 | 17 | F | Cushingoid feature, palpable right upper abdominal mass | Resection | ☓ | Alive/30 months | Cushing syndrome |
24 | Grazi et al. [10] 2010 | 25 | F | Diarrhea and recurrent abdominal pain | Right lobectomy extending the caudate lobe segment IV | ☓ | Alive/6 months | OCP |
25 | Ramirez et al. [11] 2010 | 33 | M | Unspecific abdominal pain and dyspepsia | Left lobectomy | ☓ | Alive/15 months | HBV(+) |
26 | Wang et al. [13] 2011 | 34 | F | Incidental | Left lobectomy | ☓ | Alive/42 months | OCP |
27 | Hommann et al. [12] 2011 | 14 | F | Incidental | Resection, transplantation | Post | Recurrence/28 months | Moderate hypoxic brain injury, omphalocele |
28 | Assmann et al. [14] 2012 | 16 | M | Palpable abdominal mass | Transplantation | Pre | Alive/24 months | Cushing-like habitus |
29 | 3 | F | Unclear obstipation | Partial hepatectomy | Post | Alive/5 years | ||
30 | Geramizadeh et al. [15] 2012 | 8 | M | Cushingoid feature | Right extended hepatectomy | ☓ | Death 10 days (no recurrence) | Cushing syndrome |
31 | Ghodke et al. [16] 2012 | 9 | M | Abdominal pain, fever, jaundice, weight loss, anorexia | Segmental hepatectomy | ND | Alive/12 months | |
32 | Malowany et al. [17] 2013 | 2 | F | Incidental | Resection | ND | No recurrence | BWS |
33 | Procopio et al. [18] 2014 | 23 | F | Abdominal distension and dyspepsia | Extended left hepatectomy | ☓ | Alive/21 months | OCP |
34 | Samarghandi et al. [19] 2015 | 11 | F | Weight gain, increased appetite, abdominal pain | Unknown | ND | ND | |
35 | Schaffer et al. [20] 2016 | 14 | F | Abdominal distention and swelling of cheeks | Transplant | ☓ | Alive/10 months | BWS, Cushing syndrome |
36 | Weeda et al. [21] 2016 | 16 | M | Cushingoid feature, weight gain, distended abdomen | Trisegmentectomy | ☓ | Alive/13 years | Cushing syndrome |
37 | Khoshnam et al. [22] 2017 | 14 | F | Cushingoid feature, abdominal swelling and pain | Transplantation | Pre | Alive/ND | BWS, Cushing syndrome |
38 | Tehseen et al. [23] 2017 | 13 | F | Abdominal pain and distention, Cushingoid features | Transplantation | Pre | Alive/28 months | Developmental delay, Cushing syndrome |
39 | Our case 2017 | 20 | M | Incidental | Right hepatectomy | Post | Death recurrence/2 months | Klinefelter syndrome |