J Pathol Transl Med.  2015 Sep;49(5):403-408. 10.4132/jptm.2015.05.28.

Paediatric Primary Pachymeningeal Xanthogranuloma with Scattered Foci Displaying Reticulohistiocytoma-like Features

• Salazar MF
• Estrada Hernandez MdR
• Gomez Apo
• Chavez Macias LG
• Rodriguez Alvarez CA

• KMID: 2381396
• DOI: http://doi.org/10.4132/jptm.2015.05.28

Abstract

We report a unique case of a 4-year-old girl with an intriguing fibrohistiocytic tumour. Magnetic resonance imaging scans showed a dural mass of variegated intensity compressing the left occipital pole and apparently extending toward the superior sagittal sinus. Grossly, the cut surface of the surgical specimen was yellow, pale, and soft with reddish kernel-like crusts. Histologically, the yellow areas resembled cholesterol granulomas with widespread coagulative necrosis, cholesterol clefts, powdery calcification, foreign body-type giant cells, and foamy macrophages, while the scattered red spots contained numerous multinucleated giant cells of foreign-body and Touton types, the former with amphophilic to slightly eosinophilic cytoplasm. Immunoperoxidase reactions confirmed the expression of histiocytic markers and vimentin. As far as we know, no tumour displaying these peculiar morphological features has yet been described.

Keyword

CNS fibrohistiocytic tumour; Dural tumour; Cholesterol granuloma; Solitary reticulohistiocytoma; Fibroxanthoma

MeSH Terms

Child, Preschool
Cholesterol
Cytoplasm
Eosinophils
Female
Giant Cells
Giant Cells, Foreign-Body
Granuloma
Humans
Macrophages
Magnetic Resonance Imaging
Necrosis
Superior Sagittal Sinus
Vimentin
Cholesterol
Vimentin

Figure

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  Fig. 1. Magnetic resonance imaging scans and gross surgical resection specimen. (A) T1-weighted sagittal scan demonstrating a tumour of heterogeneous intensity attached to the dura mater. (B) T1-weighted axial image. The arrow points to an area probably invaded by the tumour. (C) Transverse cut surface with a butter-covered raspberry slice of bread appearance.

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  Fig. 2. Microscopic features. (A) Panoramic photomicrograph showing the boundary zone between the cholesterol granuloma area (right field) and one of the reticulohistiocytoma-like nodules (left field). (B) Boundary zone at higher magnification. Cholesterol clefts, xanthocytes, multinucleated foreign-body type giant cells, and strands of thin fibrous tissue can be identified. (C) Panoramic view of one of the reticulohistiocytoma-like nodules: “chaotic pattern” of lipidized and amphophilic multinucleated giant cells. (D) Multinucleated giant cells with amphophilic cytoplasm and empty vacuoles. (E) Touton-type giant cell next to a cannibal cell (arrow). (F) Amphophilic multinucleated giant cell with a finely granular cytoplasm filled with lipids (Oil Red). (G) Mononucleated giant cell with nuclear atypia.

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  Fig. 3. Immunohistochemistry panel. (A) CD68 immunolabeling of foamy macrophages (left) and multinucleated giant cells (right). (B) Ubiquitous vimentin expression. (C) Delicate intranodal capillary branching unveiled by CD34. (D) Multinucleated giant cells’ circumference immunostained with epithelial membrane antigen.

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