Spindle cell xanthogranuloma D71.6

Solitary spindle cell xanthogranuloma as well as progressive nodular histiocytosis are distinct entities within the disease spectrum of the xanthogranuloma family, which is characterized by a predominance of spindle-shaped histiocytes.

No known sex predilection.

Young adults (aged 20-40 years), isolated cases have also been described in children (Morse DC et al. 2018).

Solitary spindle cell xanthogranuloma occurs with decreasing frequency on the head (including the eyelid area - DeStafeno JJ et al 2002), neck, upper trunk, or occasionally on the extremities (Zelger BW et al 1995).

Clinically, the clinical picture is characterized by a solitary brown-yellowish or yellow-reddish papule.

Histologically, spindle cell xanthogranuloma (like progressive nodular histiocytosis) is characterized by a predominance (>90%) of spindle-shaped histiocytes arranged in a stadial pattern. Other mononuclear (vacuolated, xanthomatized, "scalloped," oncocytic) and multinucleated (touton) histiocytes are also seen regularly.

Immunohistochemically, the classic features of the macrophagic/dendritic cell line are found with positivity for KP1/Ki-M1p (CD68), HAM 56, and factor XIIIa, as well as smooth muscle specific actin and HHF35.

Ultrastructurally, dense, regularly laminated, myeloid or pleomorphic cytoplasmic inclusions can be found. No evidence of Birbeck granules.

1. DeStafeno JJ et al (2002) Solitary spindle-cell xanthogranuloma of the eyelid. Ophthalmology 109:258-261.
2. Morse DC et al (2018) Vascular-appearing spindle cell xanthogranuloma in a child. Cureus 10:e2595.
3. Zelger BW et al (1995) Solitary and generalized variants of spindle cell xanthogranuloma (progressive nodular histiocytosis). Histopathology 27:11-19.
4. Zelger BG et al (1998) Scalloped cell xanthogranuloma. Histopathology 32:368-374.