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. 2014:2014:729387.
doi: 10.1155/2014/729387. Epub 2014 Aug 24.

Tumor-Induced Osteomalacia: Increased Level of FGF-23 in a Patient with a Phosphaturic Mesenchymal Tumor at the Tibia Expressing Periostin

Anke H Hautmann  1 , Josef Schroeder  2 , Peter Wild  3 , Matthias G Hautmann  4 , Elisabeth Huber  2 , Patrick Hoffstetter  5 , Martin Fleck  6 , Christiane Girlich  7
Affiliations

Tumor-Induced Osteomalacia: Increased Level of FGF-23 in a Patient with a Phosphaturic Mesenchymal Tumor at the Tibia Expressing Periostin

Anke H Hautmann et al. Case Rep Endocrinol. 2014.

Abstract

In our case, a 45-year-old male patient had multiple fractures accompanied by hypophosphatemia. FGF-23 levels were significantly increased, and total body magnetic resonance imaging (MRI) revealed a tumor mass located at the distal tibia leading to the diagnosis of tumor-induced osteomalacia (TIO). After resection of the tumor, hypophosphatemia and the increased levels of FGF-23 normalized within a few days. Subsequent microscopic examination and immunohistochemical analysis revealed a phosphaturic mesenchymal tumor mixed connective tissue variant (PMTMCT) showing a positive expression of somatostatin receptor 2A (SSTR2A), CD68, and Periostin. Electron microscopy demonstrated a poorly differentiated mesenchymal tumor with a multifocal giant cell component and evidence of neurosecretory-granules. However, the resected margins showed no tumor-free tissue, and therefore a subsequent postoperative radiotherapy was performed. The patient is still in complete remission after 34 months. Tumor resection of PMTMCTs is the therapy of choice. Subsequent radiotherapy in case of incompletely resected tumors can be an important option to avoid recurrence or metastasis even though this occurs rarely. The prognostic value of expression of Periostin has to be evaluated more precisely in a larger series of patients with TIO.

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Figures

Figure 1
Figure 1
(a) MRI of the giant cell tumor at the distal tibia and (b) an ultrasound examination of the tumor.
Figure 2
Figure 2
Phosphaturic mesenchymal tumor mixed connective tissue variant. (a) Histopathology of the tumor consisting of monomorphic cells with round cell nuclei without increased mitotic activity (hematoxylin-eosin stain: HE 200x), (b) scattered multinucleated giant cells (HE 400x), and ((c)–(e)) expression of CD68, SSTR2, and POSTN in tumor tissue (immunohistochemistry).
Figure 3
Figure 3
Primary formalin fixed tumor tissue and electron microscopic images. (a) Ultrastructural features of spindle shaped stromal cells with organelle-poor cytoplasm and few dense granules (arrow). Orig. mag. × 3125. (b) The granules resemble neurosecretory granules, diameter approx. 200 nm (not typical “dense core” granules), orig. mag. × 10,000. (c) Rounded tumor cell with a nuclear indentation, scant organella, and any cell surface specialized structures. Note singular electron dense granules in cytoplasm (arrows). Orig. mag. × 4,000. (d) Ultrastructural aspect of a giant multinuclear cell displaying in the organelle-rich cytoplasm some vesicular structures with pericentric dense inclusions (arrows). Orig. mag. × 3125.
See this image and copyright information in PMC

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