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Table 1 Reported cases of CNSET

From: Calcifying nested stromal epithelial tumor of the liver in a patient with Klinefelter syndrome: a case report and review of the literature

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
  1. ND not determined, ☓ not administered, OCP oral contraceptive pill use, BWS Beckwith-Wiedmann syndrome