Spindle cell xanthogranuloma is a rare variant of juvenile xanthogranuloma that most commonly presents in adults as papulonodules. We describe a vascular-appearing case of spindle cell xanthogranuloma on the nose of a 10-year-old boy. The lesion was a dark red, well-demarcated, dome-shaped papule. Histopathology revealed spindle-shaped histiocytes in a storiform pattern that stained positive for cluster of differentiation 68 (CD68) and the nuclear antigen Ki-67. No vascular features were found. To our knowledge, this is the first reported spindle cell xanthogranuloma to mimic an angiomatous lesion.
Juvenile xanthogranuloma (JXG) is a benign proliferative disorder of dermal histiocytes that typically presents as yellowish to reddish papulonodules . Spindle cell xanthogranuloma (SCXG) is a rare form of JXG and most often occurs in adulthood. Here, we present a pediatric and vascular-appearing case of SCXG.
A 10-year-old male patient presented with a 13-mm well-demarcated, dome-shaped, dark red nodule on the left ala (Figure 1). It had been present for eight months. During that time, it had increased in size and bled. The lesion received no prior treatment. The remainder of the physical exam was unremarkable.
A shave biopsy was performed, and histopathology revealed a diffuse infiltrate of spindle-shaped histiocytes in a storiform pattern (Figure 2), few multinucleated giant cells, scattered lymphocytes, and eosinophils (Figure 3). Immunohistochemical studies showed tumor cells positive for cluster of differentiation 68 (CD68) and the proliferation marker Ki-67 (Figure 4). The lesion was negative for S-100 protein, anti-melanoma antibody (HMB45), protein Melan-A, and smooth muscle actin (SMA). These histologic features supported the diagnosis of SCXG. The nodule resolved spontaneously several months later.
SCXG is a rare variant of JXG, originally described in 1995 by Zelger et al. who reported 12 solitary cases of SCXG . Since 1995, only a few reports of SCXG have been described [3-5]. A literature review of previous case reports, including our report, is summarized in Table 1. SCXG classically presents as brownish to yellowish papulonodules involving the head, neck, upper trunk, and extremities in decreasing occurrence . SCXG most often affects those between the ages of 20 - 40 years without preference for gender .
In contrast to the typical SCXG presentation of brownish to yellowish papules appearing in adulthood, we describe a pediatric case of SCXG that presented with dark red vascular features appearing similar to a hemangioma. The histopathology failed to reveal vascular features and confirmed the diagnosis of SCXG.
Spitz nevus (SN) was also high on our differential diagnosis since it also presents as a rapidly growing reddish nodule in children . Nakamura et al. reported a case of SCXG in a 10-year-old, initially diagnosed as an SN due to the nodule’s dark red to bluish clinical appearance and peripheral blue background with white streaks evident upon dermoscopy . However, histologic features of SN were not seen in our case.
Histological examination of SCXG typically reveals Touton-type multinucleated giant cells and spindle-shaped histocytes in a storiform pattern . The macrophage and dendritic cell ancestry of SCXG is confirmed through immunohistochemistry as SCXG stains positive for histiocyte markers: mature macrophage marker monoclonal antibody (HAM-56), CD68, and Factor XIIIa [2-3]. SCXG is histologically similar to progressive nodular histiocytosis; both tumors display a predominance of spindle cells in a storiform arrangement and multinucleated giant cells [2, 4]. However, progressive nodular histiocytosis appears in a disseminated pattern in the elderly, which is markedly different from the presentation of SCXG [2, 4]. Dermoscopy findings of SCXG appear as an orange-yellow structureless pattern with an erythematous border . The tumors usually resolve spontaneously in six to 36 months .
SCXG is a rare form of JXG that may clinically masquerade as various other neoplasms, including angiomatous lesions and Spitz nevi.
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A Vascular-appearing Spindle Cell Xanthogranuloma in a Child
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Cite this article as:
Morse D C, Tschen J A, Migden M R, et al. (May 08, 2018) A Vascular-appearing Spindle Cell Xanthogranuloma in a Child . Cureus 10(5): e2595. doi:10.7759/cureus.2595
Received by Cureus: April 19, 2018
Peer review began: April 23, 2018
Peer review concluded: May 05, 2018
Published: May 08, 2018
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Morse et al. This is an open access article distributed under the terms of the Creative Commons Attribution License CC-BY 3.0., which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.