PMC6611013_01
A 14-year-old male presented with bilateral mandibular enlargement and affecting his speech and mastication, and this enlargement did not subsequently involute or regress (A). CT shows expansile osseous remodeling with multilocular appearance and coarse trabecular pattern of the mandible, prominent dental derangement involving the mandible, without orbital involvement or clear involvement of the maxilla (B). Multiple bilateral enlarged regional lymph nodes are noted in CT as well (arrow) (C). CT indicates computed tomography.
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This enlargement did not subsequently involute or regress (Figure 1A). Computed tomography (CT) shows expansile osseous remodeling with multilocular appearance and coarse trabecular pattern of the mandible, prominent dental derangement involving the mandible, without orbital involvement or clear involvement of the maxilla, although there is mild derangement of the maxillary teeth (Figure 1B). Multiple bilateral enlarged regional lymph nodes are noted (Figure 1C).
Gross examination of mandible resection specimen shows 15 cm x 15 cm x 4 cm deformed mandible with focal cystic defects on the exterior surface, poorly aligned premolar and molar teeth, and soft yellow tissue interspersed with patchy hemorrhage and solid white bone (A and B).
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Gross examination of mandible resection specimen shows a 15 cm x 15 cm x 4 cm deformed mandible with focal cystic defects on the exterior surface, poorly aligned premolar and molar teeth, and soft yellow tissue interspersed with patchy hemorrhage and solid white bone (Figure 2A and B).
Histological examination of the mandible showed large areas of bland spindle-cell fibrous tissue with perivascular eosinophilic fibrin cuffing (arrow), with moderately abundant collagen and multiple nodules of giant cell-rich tissue (arrow) resembling a central giant cell granuloma (A and B). The mandible lesion also shows focal areas of hemosiderin deposition (arrow) and calcification (arrow) (C and D).
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Histological examination of the mandible was consistent with the clinical diagnosis of cherubism, showing large areas of bland spindle-cell fibrous tissue with perivascular eosinophilic fibrin cuffing, moderately abundant collagen, and multiple nodules of giant cell-rich tissue resembling a central giant cell granuloma (Figure 3A and B). The mandible lesion also shows focal areas of hemosiderin deposition and calcification (Figure 3C and D).
Regional lymph nodes demonstrated hemosiderin-laden macrophages (arrow) and basophilic laminated concretions (arrow) primarily localized to the cortical interfollicular space and along the periphery of the follicular marginal zone (A and B). A normal lymph node is shown in (C) for comparison.
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Regional lymph nodes were also sampled for interoperative consultation due to enlargement (2.3 cm x 2.0 cm x 0.5 cm) (normal lymph node size <1 cm), and these demonstrated hemosiderin-laden macrophages and basophilic laminated concretions primarily localized to the cortical interfollicular space and along the periphery of the follicular marginal zone, findings which have not been previously reported in the literature for this disease (Figure 4A and B).