Isolated intracraniospinal non-Langerhans cell histiocytoses (non-LCHs) without cutaneous lesion are rare.5,10,11,14,19 The most frequently reported lesion in these cases is juvenile xanthogranuloma (JXG), with several cases reported as fibrous xanthoma, xanthoma, fibroxanthoma, and xanthogranuloma (XG).1–3,5–9,11–18,21–39,41–44 Most reported intracraniospinal non-LCHs have been pediatric cases, and histopathological investigations show that they are composed mainly of xanthomatous histiocytes. In addition, several cases of intracraniospinal non-LCHs presented with dural-based lesions. We present an 8-month-old boy with isolated non-LCH attached to the dura mater in the left occipital region. Interestingly, the residual tumor regressed without treatment after surgery. We report this unique case and review the literature on isolated intracraniospinal non-LCHs, especially in cases of dural-based lesion.
Case Report
History and Examination
An 8-month-old boy who had experienced seizures with loss of consciousness and cyanosis at the age of 5 and 7 months was admitted to our hospital due to convulsive status. His growth and development had been normal, including head circumference. He had no cutaneous lesions. Physical examination and chest radiographs showed no definite abnormality. Head CT scans demonstrated a mass lesion approximately 5 cm in diameter in the left occipital region. Portions of the lesion showed high radiographic attenuation, and there was strong medial and rim enhancement (Fig. 1). The cerebral parenchyma was compressed but surrounding edema was mild. Magnetic resonance imaging showed hyperintense areas in the medial portion and rim of the tumor on T1-weighted studies (Fig. 2A). The mass demonstrated heterogeneous hypointensity on T2-weighted imaging (Fig. 2B). Diffusion-weighted imaging (Fig. 2C) showed hypointense areas, suggesting low cell density. The mass showed homogeneous enhancement contiguous with the dura mater, resembling meningioma (Fig. 2D and E).

Preoperative CT scans. Plain CT scan (left) demonstrating a mass lesion with an approximately 5-cm diameter in the left occipital region. Enhanced head CT scan (right) showing a well-enhanced area in the medial portion and rim of the mass.

Preoperative MRI studies. Axial T1-weighted MR image (A) demonstrating hyperintense areas in the medial portion and rim of the mass. The mass shows heterogeneous hypointensity on T2-weighted MR (B) and diffusion-weighted (C) images. Axial (D) and coronal (E) T1-weighted MR images obtained after contrast enhancement showing homogeneous enhancement contiguous with the dura mater.
Operation
The patient underwent tumor resection by left occipital craniotomy. The tumor was yellow colored, and strongly attached to the tentorium and falx; its consistency was very hard, and it was difficult to debulk even using an ultrasonic aspirator. Considering the surgical stress on the infant, we performed a partial (approximately 60%) resection, and a staged operation was planned at that time.
Histological Examination
Macroscopically, the tumor was colored white-gray with areas of yellow, and appeared to arise from the cranial dura mater of the left occipital region.
On microscopic examination (Fig. 3), the tumor revealed abundant xanthomatous cells that had clear or slightly eosinophilic granular cytoplasm with thin fibrous stroma, aggregates of fairly uniform foamy histiocytes with scattered thin-walled blood vessels and occasional multinucleated giant cells (but not Touton giant cells), and dense fibrocollagenous tissue in which were embedded spindle-shaped cells occasionally arranged in a storiform pattern. No overt meningothelial whorls were seen. No significant mitotic activity or necrosis was found in the tumor. Scattered inflammatory cells composed predominantly of eosinophils were noted in the tumor, and some plasma cells and lymphocytes were also observed. Although an accumulation of foamy histiocytes was observed in the granulomatous lesion of the tumor, no cholesterol cleft, hemosiderin deposits, or multinucleated foreign body giant cells were noted.

Microscopic findings. The tumor reveals abundant xanthomatous cells with clear or slightly eosinophilic granular cytoplasm (left) and dense fibrocollagenous tissue in which are embedded spindle-shaped cells (right). H & E, original magnification ×400. Figure is available in color online only.
On immunohistochemical examination (Fig. 4), CD68, HAM56, and CD163 were diffusely positive for xanthomatous cells and foamy histiocytes. These lipid-laden cells were focally positive for S100 protein. Vimentin was strongly and diffusely positive for spindle-shaped cells, but negative for CD1a, S100 protein, CD207 (Langerin), glial fibrillary acidic protein (GFAP), cytokeratin, epithelial membrane antigen (EMA), neurofilament protein (NFP), α–smooth muscle actin (α-SMA), CD34, and leukocyte common antigen (LCA). The MIB-1 labeling index was approximately 1%–2%.

Immunohistochemical findings. Staining for CD68 (A), HAM56 (B), and CD163 (C) demonstrates diffuse positivity for xanthomatous cells. Vimentin staining (D) shows strongly and diffusely positive results for the spindle-shaped cells, but negative results for CD1a (E) and CD207 (Langerin) (F). Original magnification ×400. Figure is available in color online only.
Ultrastructurally, the tumor cells were relatively rich in cytoplastic organelles, but lacked cell-to-cell attachments and intercellular junctions. No overt Birbeck granules were detected.
Based on these histological findings, the lesion was diagnosed as non-LCH, because it was positive for CD68, HAM56, and CD163, but negative for CD1a and CD207. The tumor did not present Touton giant cells, which are typical of JXG; hence, we described the lesion as a fibroxanthogranuloma. Based on the results of the immunohistological examination, the lesion is unlikely to be xanthomatous meningioma, solitary fibrous tumor, or pleomorphic xanthoastrocytoma.
Postoperative Course
Compared with a postoperative CT scan obtained at 1 week (Fig. 5A), the CT scan performed 3 months after surgery demonstrated spontaneous regression of the residual lesion (Fig. 5B). A follow-up CT scan performed approximately 1 year after surgery showed further spontaneous regression (Fig. 5C). The residual lesion continued to regress gradually, and as of approximately 5 years after surgery, only a slight residual lesion along the tentorium remained (Fig. 5D and E). The patient is presently achieving normal developmental milestones, is not receiving medications, and has experienced no seizures.

Postoperative neuroimaging. Axial CT scans performed 1 week (A), 3 months (B), and 1 year (C) after surgery. The residual tumor shows spontaneous regression. Gadolinium-enhanced MRI studies performed 5 years after surgery (D and E) demonstrate only a slight residual lesion remaining along the tentorium.
Discussion
Cases of intracraniospinal non-LCH without cutaneous lesions are rare.5,10,11,14,19 The most common form of isolated non-LCH is JXG.3,5 To our knowledge, 29 cases of isolated intracraniospinal JXG have been reported.2,3,5–8,11–15,17,18,24,25,29,31–36,38,39,41–43 H istopathologically, J XG e xhibits abundant lipid-laden histiocytes associated with mononuclear inflammatory infiltrates and a variable quantity of Touton giant cells.10,19 The immunohistochemical examination is typically positive for CD68, but negative for CD1a and S100 protein.8,10,11,19 Of the 29 cases, 17 were intracranial,2,3,5,11,13–15,18,29,31,33,34,36,38,39,42 9 were intraspinal,6,7,11,17,24,25,32,35,43 a nd 3 s howed b oth i ntracranial a nd i ntraspinal l esions.8,12,41 Only one case was in an adult.17 Among the 29 cases, 22 followed a benign course, whereas 7 showed progression of the disease.8,12,29,31,33,39,41 These 7 cases occurred in Meckel's cave or presented with multiple lesions. Orsey et al.31 reported a JXG diffusely involving the CNS that subsequently underwent malignant transformation and dissemination to the peritoneum and bone marrow.
In addition to the 29 cases of JXG, 8 cases of isolated intracraniospinal xanthomatous tumors have been reported as fibrous xanthoma, xanthoma, and fibroxanthoma.1,9,21–23,27,28,44 Histopathological examination in these cases revealed a collection of foamy histiocytes with variable amounts of fibrous tissue. Four of these cases were immunohistochemically considered to be non-LCH, based on negative staining for CD1a or S100 protein.9,22,23,27 The pathological findings in the other cases also resembled those of JXG.1,21,28,44 Most cases occurred in children and essentially followed favorable courses.
Another related lesion is XG; we found 4 cases of XG that were histopathologically similar to JXG.16,26,30,37 These cases were positive for CD68 and negative for CD1a, or showed Touton giant cells. The location was intracranial in 2 cases,16,30 and intraspinal in the other 2 cases.26,37 One case was in an infant,37 and the other 3 cases were in adults.16,26,37
Among the 42 cases of isolated intracraniospinal non-LCHs (reported as JXG, fibrous xanthoma, xanthoma, fibroxanthoma, and XG) including our case (Table 1), 18 cases (43%) were dural-based lesions.1,2,5,9,11,16,21,22,25–28,30,32,35,37,38 Eleven of these 18 cases were intracranial1,2,5,9,16,21,27,28,30,38 and the other 7 were spinal.11,22,25,26,32,35,37 No cases showed recurrence during the follow-up period and essentially followed benign courses.
Previous reports of isolated intracraniospinal non-LCHs (JXG, fibrous xanthoma, xanthoma, fibroxanthoma, and XG)
Authors & Year | Reported Pathology | Age, Sex | Site | Dural-Based | Touton GC | Immunohistochemical Examination | Treatment | Outcome |
---|---|---|---|---|---|---|---|---|
Abiko & Orita, 1981 | Fibrous xanthoma | 6 yrs, M | Bilat temporal | Yes | No | Not described | PR | Uneventful, 4 mos |
Wertz et al., 1982 | JXG | 20 mos, F | ON | No | Yes | Not described | Resection | NR, 15 mos |
Morimura et al., 1986 | Fibrous xanthoma | 46 yrs, M | Gasserian ganglion | Yes | No | (−) NSE | TR, RT | NR |
Kamiryo et al., 1988 | Fibrous xanthoma | 6 yrs, M | Bilat temporal | Yes | No | (−) GFAP | PR | NR, 6 yrs; spontaneous regression |
Yamataki et al., 1990 | Fibrous xanthoma | 77 yrs, F | Frontal, clival region | No | No | (−) GFAP | Biopsy | Died of cerebral infarction |
Kimura et al., 1991 | Xanthoma | 9 yrs, M | Meckel's cave | No | No | (−) S100 | PR | NR, 6 mos |
Paulus et al., 1992 | JXG | 7 yrs, M | Meckel's cave | No | Yes | (+) CD68, HAM56; (−) S100, GFAP, EMA | PR | PD, 6 mos; STR, RT; NR, 6 mos |
Shimosawa et al., 1993 | XG | 13 mos, F | T6–9 | Yes | Yes | (+) lysozyme, vimentin; (−) S100 | TR | NR, 9 mos |
Kitchen et al., 1995 | JXG | 15 yrs, F | S-1 nerve root | No | Yes | (+) HAM56; (−) S100 | TR | Not described |
Kim et al., 1996 | Xanthoma | 16 mos, M | C7–T3 | Yes | No | (+) CD68, lysozyme; (−) S100, GFAP | TR | NR, 3 mos |
Oyama et al., 1997 | JXG | 18 yrs, F | C-1 | Yes | Yes | (+) CD68, lysozyme; (−) S100, NSE | TR | NR, 6 mos |
Schultz et al., 1997 | JXG | 13 yrs, M | Temporal | No | Yes | (+) HAM56, LCA; (−) S100 | TR | NR, 12 mos |
Miyazono et al., 1999 | Fibroxanthoma | 19 mos, M | Occipital | Yes | No | (−) S100 | STR | NR, 6 mos |
Boström et al., 2000 | JXG | 4 yrs, M | MF, lat vents, tentorium, orbita (xanthofibroma | Yes | Yes | (+) CD68, vimentin, LCA; (−) CD1a, S100, EMA, GFAP | PR | NR, 7 yrs |
Rampini et al., 2001 | JXG | 34 mos, F | C5–7 | Yes | Yes | (+) CD68; (−) CD1a, S100, GFAP, CD34, vimentin, CK | TR | NR, 4 mos |
Gutnik et al., 2001 | JXG | 15 yrs, F | Parietal | No | Yes | (+) CD68, S100; (−) CD1a | Resection | Not described |
Ernemann et al., 2002 | JXG | 18 yrs, F | Lat vent, spinal lesions | No | No | (+) CD68; (−) CD1a, S100 | Biopsy, chemo, RT | PD, 1 yr |
Ashley et al., 2005 | JXG | 7 yrs, M | Frontal | No | No | (+) CD68, factor XIIIa; (−) CD1a, S100 | TR | NR, 1 yr |
Nakasu et al., 2007 | JXG | 2 yrs, M | Meckel's cave | No | Yes | (+) CD68; (−) S100 | PR | PD, 2 mos; RT; NR, 8 mos |
Orsey et al., 2008 | JXG | 11 yrs, M | Brainstem, cerebral peduncles | No | Yes | (+) CD68, factor XIIIa; (−) CD1a, S100 | Biopsy, chemo, RT | PD, dead at 29 mos |
Cao et al., 2008 | JXG | 18 yrs, F | C-2 nerve root | No | Yes | (+) CD68, vimentin; (−) NSE | TR | NR, 12 mos |
Fulkerson et al., 2008 | JXG | 8 yrs, M | Frontal | No | No | (+) CD68, factor XIIIa; (−) CD1a, S100 | TR | NR, 3 yrs |
Chung et al., 2009 | Fibroxanthoma | 6 mos, F | Occipital | Yes | No | (+) CD68; (−) CD1a, EMA, vimentin | Resection | Died just after surgery |
Castro-Gago et al., 2009 | JXG | 14 yrs, M | Multi in spinal canal | No | Yes | (+) CD68; (−) CD1a, S100 | PR, chemo | AWD, 12 mos |
Sun et al., 2009 | JXG | 5 mos, M | Parietal | Yes | Yes | (+) CD68; (−) CD1a, S100 | TR | NR, 6 mos |
Rajaram et al., 2010 | JXG | 3 yrs, M | Frontal | No | Yes | (+) CD68, S100; (−) CD1a | Resection | NR, 6 mos |
Naren et al., 2011 | XG | 40 yrs, M | Cerebellar | Yes | Yes | (+) CD68, vimentin; (−) S100, EMA | STR | Not described |
Inoue et al., 2011 | JXG | 38 yrs, M | C-8 nerve root | No | Yes | (+) CD68; (−) S100, GFAP, EMA, CD34 | TR | NR, 2 yrs |
Husain et al., 2012 | XG | 38 yrs, F | Frontoparietal | Yes | Yes | (+) CD68, vimentin, S100; (−) CD1a, GFAP, EMA, α-SMA | TR | Not described |
Lee et al., 2012 | XG | 29 yrs, M | C1–2 | Yes | No | (+) CD68, lysosome; (−) CD1a, S100, GFAP | TR | NR, 2 yrs |
Vijapura & Fulbright, 2012 | JXG | 13 yrs, M | Posterior fossa, spinal cord | No | Yes | (+) CD68, S100, factor XIIIa; (−) CD1a | PR, chemo | PD; chemo, RT; NR, 11 mos |
Abla et al., 2013 | JXG | 9 yrs, F | Occipital | Yes | Yes | (+) CD68, factor XIIIa; (−) CD1a, S100 | Biopsy, chemo | NR, 22 mos |
Deisch et al., 2013 | JXG | 14 yrs, F | Gasserian ganglion | No | Yes | (+) CD68, LCA; (−) CD1a, S100 | PR, chemo | NR, 9 mos |
JXG | 15 yrs, F | T-9 | Yes | Yes | (+) CD68, factor XIIIa, S100; (−) CD1a | TR | NR, 8 mos | |
Tamir et al., 2013 | JXG | 3.5 yrs, M | Occipital | No | No | (+) CD68, factor XIIIa; (−) CD1a, S100 | TR | NR, 28 mos |
JXG | 3.5 yrs, F | Meckel's cave | No | Yes | (+) CD68, vimentin, factor XIIIa; (−) CD1a, S100, EMA, GFAP, α-SMA | PR | PD, 1 yr; chemo; NR, 2.9 yrs | |
Chiba et al., 2013 | JXG | 4 yrs, M | Multi intracranial, spine | No | No | (+) CD68, vimentin, S100; (−) CD1a, GFAP | Biopsy, chemo | PD |
Gressot et al., 2013 | JXG | 6 wks, M | Brainstem, thalamus, BG, ONs, lat vents | No | No | (+) CD68, S100; (−) CD1a | Biopsy, chemo | NR, 7 mos |
Wille et al., 2013 | JXG | 6 mos, ? | C3–7 | No | No | (+) CD68; (−) CD1a, S100 | TR | NR, 2 yrs |
Jain & Mehta, 2013 | JXG | 8 yrs, M | Lat vents | No | Yes | (+) CD68, S100; (−) CD1a, GFAP | PR | Not described |
Konar et al., 2014 | JXG | 18 yrs, M | C3–5 | Yes | Yes | Not described | TR | NR, 3 mos |
Present study | Fibroxanthogranuloma | 8 mos, M | Occipital | Yes | No | (+) CD68, HAM56, CD163, vimentin, S100; (−) CD1a, CD34, LCA, CK, GFAP, NFP, α-SMA, CD207, EMA | PR | NR, 4 yrs; spontaneous regression |
AWD = alive with disease; BG = basal ganglia; chemo = chemotherapy; CK = cytokeratin; GC = giant cell; MF = middle fossa; multi = multiple; NR = no recurrence; NSE = neuron-specific enolase; ON = optic nerve; PD = progressive disease; PR = partial resection; RT = radiation therapy; STR = subtotal resection; TR = total resection; vent = ventricle; ? = unknown.
Interestingly, in our case, the residual tumor underwent spontaneous regression after surgery. Cutaneous JXGs are known to involute spontaneously.5,10 In addition, spontaneous resolution of extracutaneous JXG has been reported: these lesions occurred in the larynx,4,40 orbit,20 temporalpetrous bone,10 and intraabdomen.10 Dehner suggested that JXG may have the capacity for self-healing or spontaneous regression. Of intracraniospinal cases, 1 case of regressive fibrous xanthoma has been reported.21 Non-LCHs may have the potential to regress spontaneously. Because all reported dural-based cases, including ours, have followed benign courses, they could be followed up without additional treatment after resection. However, close follow-up is mandatory in patients with lesions.
Among the non-LCHs that we reviewed, clinically similar tumors have been diagnosed as different pathological entities. Moreover, one pathological diagnosis, especially in JXG, has included both benign and malignant cases. Further investigations and the accumulation of more cases are needed to elucidate the pathogenesis of the non-LCHs.
Acknowledgment
Dr. Kiyoshi Gomi, Department of Pathology, Kanagawa Children's Medical Center, contributed greatly to this report. The authors dedicate this report to his memory.
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