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Review
. 2009 Dec;108(6):925-32.
doi: 10.1016/j.tripleo.2009.07.005. Epub 2009 Oct 14.

Phosphaturic mesenchymal tumor, mixed connective tissue variant, of the mandible: report of a case and review of the literature

Victoria L Woo  1 , Regina Landesberg, Erik A Imel, Steven R Singer, Andrew L Folpe, Michael J Econs, Taeyun Kim, Lara R Harik, Thomas P Jacobs
Affiliations
Review

Phosphaturic mesenchymal tumor, mixed connective tissue variant, of the mandible: report of a case and review of the literature

Victoria L Woo et al. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2009 Dec.

Abstract

Tumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome that results in renal phosphate wasting with hypophosphatemia. In most cases, the underlying cause of TIO is a small mesenchymal neoplasm that is often difficult to detect, resulting in delayed diagnosis. One such neoplasm is the phosphaturic mesenchymal tumor, mixed connective tissue variant (PMTMCT), an unusual entity with unique morphologic and biochemical features. Most of these tumors are found at appendicular sites with only rare cases reported in the jaws. We describe a PMTMCT involving the mandible in a patient with a protracted history of osteomalacia. A review of the current literature is provided with emphasis on the clinical and histologic features, etiopathogenesis, and management of PMTMCT in the setting of TIO.

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Figures

Figure 1
Figure 1
Panoramic radiograph taken 9 years prior to presentation showing a radiolucent lesion with interradicular scalloping.
Figure 2
Figure 2
Panoramic radiograph taken at presentation showing a multilocular lesion with internal opacities.
Figure 3
Figure 3
A mandibular cone-beam computerized tomography scan. Bucco-lingual expansion is evident. A, axial. B, coronal.
Figure 3
Figure 3
A mandibular cone-beam computerized tomography scan. Bucco-lingual expansion is evident. A, axial. B, coronal.
Figure 4
Figure 4
Microscopic images of lesion. A, A cellular spindle cell proliferation exhibiting a fascicular pattern (hematoxylin-eosin, original magnification X100). B, “Grungy”-type calcifications and osteoclast-like giant cells (arrows) (hematoxylin-eosin, original magnification X100). C, Positive staining with vimentin (original magnification X100).
Figure 4
Figure 4
Microscopic images of lesion. A, A cellular spindle cell proliferation exhibiting a fascicular pattern (hematoxylin-eosin, original magnification X100). B, “Grungy”-type calcifications and osteoclast-like giant cells (arrows) (hematoxylin-eosin, original magnification X100). C, Positive staining with vimentin (original magnification X100).
Figure 4
Figure 4
Microscopic images of lesion. A, A cellular spindle cell proliferation exhibiting a fascicular pattern (hematoxylin-eosin, original magnification X100). B, “Grungy”-type calcifications and osteoclast-like giant cells (arrows) (hematoxylin-eosin, original magnification X100). C, Positive staining with vimentin (original magnification X100).
See this image and copyright information in PMC

References

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