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Case report
peer-reviewed

A Vascular-appearing Spindle Cell Xanthogranuloma in a Child



Abstract

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.

Introduction

Juvenile xanthogranuloma (JXG) is a benign proliferative disorder of dermal histiocytes that typically presents as yellowish to reddish papulonodules [1]. 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.

Case Presentation

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.

 
 
 

Discussion

SCXG is a rare variant of JXG, originally described in 1995 by Zelger et al. who reported 12 solitary cases of SCXG [2]. 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 [2]. SCXG most often affects those between the ages of 20 - 40 years without preference for gender [2].

Case No. Author, year Age (years) Gender Location Size (mm) Color Recurrence
1 Zelger BW et al., 1995 11 F Chin n/a n/a No
2 Zelger BW et al., 1995 27 F Neck n/a n/a n/a
3 Zelger BW et al., 1995 21 M Occiput n/a n/a No
4 Zelger BW et al., 1995 59 M Back n/a n/a No
5 Zelger BW et al., 1995 18 F Eyebrow n/a n/a n/a
6 Zelger BW et al., 1995 31 F Upper Trunk n/a n/a No
7 Zelger BW et al., 1995 38 F Abdomen n/a n/a No
8 Zelger BW et al., 1995 41 M Neck n/a n/a No
9 Zelger BW et al., 1995 29 F Back n/a n/a No
10 Zelger BW et al., 1995 24 M Calf n/a n/a No
11 Zelger BW et al., 1995 54 F Thigh n/a n/a n/a
12 Zelger BW et al., 1995 15 M Lower Arm n/a n/a No
13 DeStafeno JJ et al., 2002 3 M Eyelid 7x7 Yellowish Brown n/a
14 Kim CR et al., 2012 0.92 (11 months) F Occiput n/a Yellowish Brown n/a
15 Nakamura Y et al., 2013 10 F Hip 10x5 Dark Red No
16 Morse DC et al., 2018 10 M Nose 13 Dark Red No
 

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 [6]. 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 [5]. 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 [2]. 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 [7]. The tumors usually resolve spontaneously in six to 36 months [1].

Conclusions

SCXG is a rare form of JXG that may clinically masquerade as various other neoplasms, including angiomatous lesions and Spitz nevi.


References

  1. Dehner LP: Juvenile xanthogranulomas in the first two decades of life: a clinicopathologic study of 174 cases with cutaneous and extracutaneous manifestations. Am J Surg Pathol. 2003, 27:579-93. 10.1097/00000478-200305000-00003
  2. Zelger BW, Staudacher C, Orchard G, et al.: Solitary and generalized variants of spindle cell xanthogranuloma (progressive nodular histiocytosis). Histopathology. 1995, 27:11-19. 10.1111/j.1365-2559.1995.tb00285.x
  3. DeStafeno JJ, Carlson JA, Meyer DR: Solitary spindle-cell xanthogranuloma of the eyelid. Ophthalmology. 2002, 109:258-61. 10.1016/S0161-6420(01)00879-X
  4. Kim CR, Kim HJ, Jung MY, et al.: A case of congenital spindle cell xanthogranuloma. Am J Dermatopathol. 2012, 34:672-73. 10.1097/DAD.0b013e3182327e40
  5. Nakamura Y, Nakamura A, Muto M: Solitary spindle cell xanthogranuloma mimicking a spitz nevus. Am J Dermatopathol. 2013, 35:865-67. 10.1097/DAD.0b013e3182840d2d
  6. Luo S, Sepehr A, Tsao H: Spitz nevi and other spitzoid lesions. Part I. background and diagnoses. J Am Acad Dermatol. 2011, 65:1073-84. 10.1016/j.jaad.2011.04.040
  7. Palmer A, Bowling J: Dermoscopic appearance of juvenile xanthogranuloma. Dermatology. 2007, 215:256-59. 10.1159/000106586
Case report
peer-reviewed

A Vascular-appearing Spindle Cell Xanthogranuloma in a Child


Author Information

Daniel C. Morse

Mcgovern Medical School, University of Texas Mcgovern Medical School at Houston, Houston, USA

Jaime A. Tschen

Dermatology, St. Joseph Dermatopathology, Houston, USA

Michael R. Migden

Dermatology, Head and Neck Surgery, The University of Texas MD Anderson Cancer Center, Houston, USA

Sirunya Silapunt Corresponding Author

Dermatology, University of Texas McGovern Medical School, Houston, USA


Ethics Statement and Conflict of Interest Disclosures

Human subjects: Consent was obtained by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.


Case report
peer-reviewed

A Vascular-appearing Spindle Cell Xanthogranuloma in a Child


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