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

May, Jackson M.; Waddle, Mark R.; Miller, Daniel H.; Stross, William C.; Kaleem, Tasneem A.; May, Byron C.; Miller, Robert C.; Jiang, Liuyan; Strong, Gerald W.; Trifiletti, Daniel M.; Chaichana, Kaisorn L.; Reimer, Ronald; Tun, Han W.; Peterson, Jennifer L.

doi:10.1186/s13014-018-1115-x

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

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
^aIndicates patients from manuscript Zanelli et al., 2017 [33]

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