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Table 1 Reported cases of Primary Histiocytic Sarcoma of the CNS and their characteristics

From: Primary histiocytic sarcoma of the central nervous system: a case report with platelet derived growth factor receptor mutation and PD-L1/PD-L2 expression and literature review

Author & Year Age, Gender Site(s) Number of Lesions Size (cm) Primary Treatment Outcome
aGill-Samra et al. 2012 [14] 38 yr./F Temporal Multiple 5 Surgery+ WBRT: 45Gy in 25 fractions+ Chemotherapy DOD 3 weeks after presentation
aToshkezi et al. 2010 [29] 71 yr./F Spine and leptomeninges Solitary 2.5x1x 1.1 Surgery+ RT: 44 Gy in 22 fractions DOD 5 months after presentation
aBell et al. 2012 [3] 62 yr./F Cerebellum Solitary ND Surgery AWPD 24 months
aBell et al. 2012 [12] 34 yr./M Frontal Solitary 2 Surgery AWPD 10 months
a Devic et al., 2012 [10] 43 yr./F Corpus callosum, Cerebellum, and Spine Multiple ND Chemotherapy DOD 10 months after presentation
aWang et al., 2012 [31] 55 yr./F Corpus callosum Multiple ND Surgery+3D CRT: 16 Gy DOD 4 months after presentation
aTorres et al., 1996 [28] 20 mo/M Leptomeninges Solitary ND Chemotherapy DOD 3 months after presentation
a Cheuk et al., 2001 [9] 69 yr./F Parietal Solitary 1.5 Surgery+ WBRT+ Chemotherapy DOD 8 months after presentation
a Cheuk et al., 2001 [9] 43 yr./M Spine Solitary 1.7 Surgery+ WBRT+ Chemotherapy AWPD at 5 months
a Cheuk et al., 2001 [21] 11 yr./M Cerebellum, occipital, and frontal Multiple .7–1 Surgery DOD 4 months after presentation
a Sun et al., 2003 [26] 13 yr./M Occipital and leptomeninges ND ND 1.1 Surgery DOD 7 months after presentation
aCao et al., 2007 [6] 53 yr./F Cavernous sinus, relapsed to mediastinum Solitary 3.1 × 2.9 × 2.2 Surgery+ RT 54 Gy DOD 4 years after presentation
aAlmefty et al., 2013 [1] 16 yr./M Parietal Solitary 3.5 × 4.4 × 4.0 Surgery+ IMRT: 60 Gy in 30 fractions DOD 4 months after presentation
aWu et al., 2013 [32] 50 yr./M Parieto-occipital Solitary 1.7 Surgery+ SRS: 30 Gy in 5 fractions AWPD at 18 months
a Laviv et al., 2013 [19] 58 yr./M Frontal Solitary 6.5 Surgery DOD 4 months after presentation
aGomi et al.. 2012 [15] 17 mo/F Cerebellum, dissemination to spine Solitary 4.7 × 4.3 × 4.3 Surgery+ Chemotherapy AWPD at 16 months
aGentzler et al., 2011 [13] 52 yr./F Parietal Solitary 1.7 Palliative Care Expired from lung cancer
a Perez-Ruiz et al., 2013 [23] 41 yr./F Temporal and leptomeninges Solitary 1.5 × 2 Surgery+ TMZ + IMRT: 61.2Gy ANED 42 months
aChalasani et al., 2013 [7] 44 yr./M Corpus callosum Multiple 3.5 and 2.6 Chemotherapy+ WBRT: 26 Gy + Boost to 46 Gy DOD 27 weeks after presentation
aIdbaih et al., 2014 [17] 40 yr./M Temporal Solitary ND Chemotherapy DOD 6 months after presentation
aMoulinger et al., 2014 [22] 63 yr./F Pons Multiple ND Chemotherapy DOD 20 days after completion of treatment
a Bai et al., 2014 [2] 52 yr./M Frontal Solitary ND Surgery+ TMZ + IMRT: 54 Gy ANED 16 months
aFoster et al., 2015 [11] 15 yr./F Frontal Solitary 5.8 × 4.7 × 4.0 Surgery+ TMZ + 3D CRT ANED 23 months
a Chen et al., 2015 [8] 61 yr./M Meckel’s Cave Solitary 1.5 × 1.1 × 1.8 Radiotherapy 66Gy in 33 fractions + 1 cycle CVP + 2 cycles CHOP AWPD 31 months
aBrown et al., 2015 [4] 23 yr./M Cerebellopontine Solitary 6 Chemotherapy+ IMRT AWPD 60 months
aSo et al., 2015 [25] 59 yr./M Parietal, Corpus callosum, Frontal and Spine Multiple ND Chemotherapy DOD 8 months after presentation
Zanelli et al., 2017 [33] 45 yr./F Leptomeninges N/A N/A No therapy DOD 2 months after presentation
a Ueno et al., 2016 [30] 65 yr./M Frontal, parietal, spine, and leptomeninges Multiple ND Radiotherapy AWPD 11 months
Kim et al., 2017 [18] 16 yr./M Corpus callosum, frontoparietal Solitary 6.5 × 5.3 Surgery+ Chemotherapy+ Radiotherapy 60Gy DOD 12 months after presentation
Marguet et al., 2018 [21] 67 yr./M Periventricular, hypothalamic and sellar region, leptomeningeal involvement. Multiple 2.1, 1.4 No therapy DOD 7 months after presentation
Present case 47 yr/ F Cerebellar vermis and leptomeninges Multiple 3.0 × 2.9 × 2.3 Surgery + WBRT: 30.6 Gy + Boost to 45 Gy + Chemotherapy DOD 8 months after presentation
  1. ANED Alive and no evidence of disease, AWPD Alive with progressive disease, DOD Dead of disease, ND Not Determined, PTV Planning target volume, WBRT Whole Brain Radiation Therapy, 3D CRT 3D Conformal Radiotherapy, IMRT Intensity modulated radiotherapy, TMZ Temozolomide, CVP Cyclophosphamide, vincristine, and prednisone, CHOP Cyclophosphamide, doxorubicin, vincristine, and prednisone
  2. aIndicates patients from manuscript Zanelli et al., 2017 [33]