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FGF23 Producing Mesenchymal Tumor

DOI: 10.1155/2014/492789

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Abstract:

A 40-year-old patient was referred to Clinic of Endocrinology due to hypophosphatemia causing pain, cramps, and weakness of muscles. Moreover, his bone mineral density was very low. The previous treatment with phosphorus and active vitamin D metabolites was ineffective. In lab tests the hypophosphatemia, hyperphosphaturia, and elevated FGF23 levels were found. Somatostatin receptor scintigraphy (SRS) showed increased radiotracer uptake in the right maxillary sinus and CT scans confirmed presence of tumor in this localization. Biopsy and cytological examination created suspicion of mesenchymal tumor—glomangiopericytoma. Waiting for surgery the patient was treated with long acting Somatostatine analogue, and directly before operation short acting Octreotide and intravenous phosphorus were used. Histology confirmed the cytological diagnosis and the phosphatemia return to normal values in 10 days after the tumor removal. 1. Case Report A 40-year-old man was referred to Clinic of Endocrinology due to prolonged, deep hypophosphatemia causing pain, cramps, and weakness of proximal muscles. One year before, during the previous hospitalization (in neurological ward) primary muscle disease had been excluded and diagnosis of osteomalacia had been established. Diagnosis was made on basis of symptoms, low calcium and phosphorus level (2.1?mmol/L and 0.6?mmol/L, resp.), and very low 24?h calcium urine excretion (80?mg/24?h). PTH was then surprisingly normal that is, 57?pg/mL (normal ranges 15–65?pg/mL), and 25OHD3 level was undetectable (<4?ng/mL). There were no fractures in patient’s medical history; however bone mineral density was very low (in all localizations -scores and -scores <?3). Patient was treated for more than one year with calcitriol 1?μg, alfacalcidol 1?μg, calcium 1000?mg, and phosphorus 1500?mg per day. This medication had no effect on serum concentrations of phosphorus and only a moderate effect on clinical symptoms. Patient was under dental treatment (caries and periodontitis). At admission the levels of CPK, calcium, and PTH were normal; 25OHD3 level raised to 29.1?ng/dL (normal ranges 30–80?ng/dL) despite using only little amount of D3 contained in Ca/D3 preparations apart from active metabolites. Alkaline phosphatase (AP) was slightly elevated (137?U/L; normal ranges 40–129?U/L) and serum phosphorus concentration was very low (0.41?mmol/L; normal ranges 0.81–1.45?mmol/L). 24?h urine collection showed high phosphorus excretion (66.5?mmol/24?h, normal ranges 12.00–65.00?mmol/24?h). Bone mineral density was still very low: lumbar spine -score =

References

[1]  R. Seijas, O. Ares, J. Sierra, and M. Pérez-Dominguez, “Oncogenic osteomalacia: two case reports with surprisingly different outcomes,” Archives of Orthopaedic and Trauma Surgery, vol. 129, no. 4, pp. 533–539, 2009.
[2]  W. H. Chong, A. A. Molinolo, C. C. Chen, and M. T. Collins, “Tumor-induced osteomalacia,” Endocrine-Related Cancer, vol. 18, no. 3, pp. R53–R77, 2011.
[3]  P. Komínek, I. Stárek, I. Geierová, P. Matou?ek, and P. Zeleník, “Phosphaturic mesenchymal tumour of the sinonasal area: case report and review of the literature,” Head & Neck Oncology, vol. 3, article 16, 2011.
[4]  N. Koriyama, K. Nishimoto, T. Kodama et al., “Oncogenic osteomalacia in a case with a maxillary sinus mesenchymal tumor,” American Journal of the Medical Sciences, vol. 332, no. 3, pp. 142–147, 2006.
[5]  S. I. Cho, N. Y. Do, S. W. Yu, and J. Y. Choi, “Nasal hemangiopericytoma causing oncogenic osteomalacia,” The Clinical and Experimental Otorhinolaryngology, vol. 5, no. 3, pp. 173–176, 2012.
[6]  A. L. Folpe, J. C. Fanburg-Smith, S. D. Billlings, et al., “Most osteomalacia associated mesenchymal tumors are a single histopathologic entity, the, “phosphaturic mesenchymal tumor, mixed connective tissue variant”: an analysis of 32 cases and a comprehensive review of the literature,” The American Journal of Surgery, vol. 28, pp. 1–30, 2004.
[7]  Y. Jiang, W. Xia, X. Xing et al., “Tumor-induced osteomalacia: an important cause of adult-onset hypophosphatemic osteomalacia in China: report of 39 cases and review of the literature,” Journal of Bone and Mineral Research, vol. 9, pp. 1967–1975, 2012.
[8]  W. H. Chong, S. Yavuz, S. M. Patel, C. C. Chen, and M. T. Collins, “The importance of whole body imaging in tumor-induced osteomalacia,” Journal of Clinical Endocrinology and Metabolism, vol. 96, no. 12, pp. 3599–3600, 2011.
[9]  S. M. Jan de Beur, E. A. Streeten, A. C. Civelek et al., “Localisation of mesenchymal tumours by somatostatin receptor imaging,” The Lancet, vol. 359, no. 9308, pp. 761–763, 2002.
[10]  M. S. Elston, I. J. Stewart, R. Clifton-Bligh, and J. V. Conaglen, “A case of oncogenic osteomalacia with preoperative secondary hyperparathyroidism: description of the biochemical response of FGF23 to octreotide therapy and surgery,” Bone, vol. 40, no. 1, pp. 236–241, 2007.
[11]  A. Ishii, Y. Imanishi, K. Kobayashi et al., “The levels of somatostatin receptors in causative tumors of oncogenic osteomalacia are insufficient for their agonist to normalize serum phosphate levels,” Calcified Tissue International, vol. 86, no. 6, pp. 455–462, 2010.
[12]  A. Mékinian, M. Ladsous, A. Balavoine et al., “Curative surgical treatment after inefficient long-acting somatostatin analogues therapy of a tumor-induced osteomalacia,” Presse Medicale, vol. 40, no. 3, pp. 309–313, 2011.
[13]  J. Seufert, K. Ebert, J. Müller et al., “Octreotide therapy for tumor-induced osteomalacia,” The New England Journal of Medicine, vol. 345, no. 26, pp. 1883–1888, 2001.

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