Case report: Oncogenous osteomalacia: A new case secondary to a malignant tumor

Rico, H.; Fernandez-Miranda, E.; Sanz, Julia; Gómez-Castresana, F.; Escribá, A.; Hernandez, Enrique; Krsnik, Isabel

doi:10.1016/8756-3282(86)90251-6

Cited by 12 publications

(5 citation statements)

References 21 publications

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“…62,103 There are also 4 cases reported under other names that we think are most likely histologically malignant PMTMCT. 36,47,68,80 Although it seems likely that the great majority of cases of OO are caused by a single entity, PMTMCTs are clearly not the only mesenchymal tumors that may cause OO. In the current series, both of the craniofacial sinus lesions lacked typical features of PMTMCT and, instead, more closely resembled sinonasal HPC.…”

Section: Discussionmentioning

confidence: 97%

Most Osteomalacia-associated Mesenchymal Tumors Are a Single Histopathologic Entity

Folpe

Fanburg‐Smith²,

Billings

et al. 2004

The American Journal of Surgical Pathology

586

547

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Oncogenic osteomalacia (OO) is a rare paraneoplastic syndrome of osteomalacia due to phosphate wasting. The phosphaturic mesenchymal tumor (mixed connective tissue variant) (PMTMCT) is an extremely rare, distinctive tumor that is frequently associated with OO. Despite its association with OO, many PMTMCTs go unrecognized because they are erroneously diagnosed as other mesenchymal tumors. Expression of fibroblast growth factor-23 (FGF-23), a recently described protein putatively implicated in renal tubular phosphate loss, has been shown in a small number of mesenchymal tumors with known OO. The clinicopathological features of 32 mesenchymal tumors either with known OO (29) or with features suggestive of PMTMCT (3) were studied. Immunohistochemistry for cytokeratin, S-100, actin, desmin, CD34, and FGF-23 was performed. The patients (13 male, 19 female) ranged from 9 to 80 years in age (median 53 years). A long history of OO was common. The cases had been originally diagnosed as PMTMCT (15), hemangiopericytoma (HPC) (3), osteosarcoma (3), giant cell tumor (2), and other (9). The tumors occurred in a variety of soft tissue (21) and bone sites (11) and ranged from 1.7 to 14 cm. Twenty-four cases were classic PMTMCT with low cellularity, myxoid change, bland spindled cells, distinctive "grungy" calcified matrix, fat, HPC-like vessels, microcysts, hemorrhage, osteoclasts, and an incomplete rim of membranous ossification. Four of these benign-appearing PMTMCTs contained osteoid-like matrix. Three other PMTMCTs were hypercellular and cytologically atypical and were considered malignant. The 3 cases without known OO were histologically identical to the typical PMTMCT. Four cases did not resemble PMTMCT: 2 sinonasal HPC, 1 conventional HPC, and 1 sclerosing osteosarcoma. Three cases expressed actin; all other markers were negative. Expression of FGF-23 was seen in 17 of 21 cases by immunohistochemistry and in 2 of 2 cases by RT-PCR. Follow-up (25 cases, 6-348 months) indicated the following: 21 alive with no evidence of disease and with normal serum chemistry, 4 alive with disease (1 malignant PMTMCT with lung metastases). We conclude that most cases of mesenchymal tumor-associated OO, both in the present series and in the reported literature, are due to PMTMCT. Improved recognition of their histologic spectrum, including the presence of bone or osteoid-like matrix in otherwise typical cases and the existence of malignant forms, should allow distinction from other mesenchymal tumors. Recognition of PMTMCT is critical, as complete resection cures intractable OO. Immunohistochemistry and RT-PCR for FGF-23 confirm the role of this protein in PMTMCT-associated OO.

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Section: Discussionmentioning

confidence: 97%

Most Osteomalacia-associated Mesenchymal Tumors Are a Single Histopathologic Entity

Folpe

Fanburg‐Smith²,

Billings

et al. 2004

The American Journal of Surgical Pathology

586

547

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“…Since PTH is phosphaturic [23], it is logical that there would be hypophosphatemia. To determine how many of these patients with hypophosphorémie osteomalacia have typ ical oncogenous osteomalacia is another problem pend ing solution, but, as we and other authors have com mented [24,25], it could be more frequent than is thought. In our cases high PTH levels account for the hypophosphatemia and do not suggest oncogenous os teomalacia, which courses with normal PTH levels [22,24,25].…”

Section: Resultsmentioning

confidence: 99%

“…To determine how many of these patients with hypophosphorémie osteomalacia have typ ical oncogenous osteomalacia is another problem pend ing solution, but, as we and other authors have com mented [24,25], it could be more frequent than is thought. In our cases high PTH levels account for the hypophosphatemia and do not suggest oncogenous os teomalacia, which courses with normal PTH levels [22,24,25]. Hypocalcemia and hypophosphatemia are also reported to result from the treatment of these patients with estrogens [26], which have a phosphaturic effect [27], In our cases these findings cannot be attributed to treatment since both groups received estrogens and the only patients with hypocalcemia were those who had increased bone resorption and elevated OHP excretion, and it is known that estrogens inhibit the effect of PTH on bone [28] and thus diminish the urinary elimination of OHP [29],…”

Section: Resultsmentioning

confidence: 99%

Hyperparathyroidism in Metastases of Prostatic Carcinoma: A Biochemical, Hormonal and Histomorphometric Study

Rico

Uson²,

Hernández³

et al. 1990

Eur Urol

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“…A characteristic histologic feature of such tumors is a background of spindle cells that tend to have low mitotic activity, prominent vascularity, osteoclast-like giant cells, or the presence of bony tissue. Although most of these tumors are thought to have a benign histologic appearance, malignant presentation and metastases can occur (86)(87)(88)(89)(90). While metastases are rare, infiltration of surrounding connective tissue is typically present, which has significant implications for surgical management and emphasizes the importance for wide surgical margins to avoid persistence or recurrence.…”

Section: Tumor-induced Osteomalacia (Tio)mentioning

confidence: 99%

FGF23 and Phosphate Wasting Disorders

2013

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A decade ago, only two hormones, parathyroid hormone and 1,25(OH)2D, were widely recognized to directly affect phosphate homeostasis. Since the discovery of fibroblast growth factor 23 (FGF23) in 2000 (1), our understanding of the mechanisms of phosphate homeostasis and of bone mineralization has grown exponentially. FGF23 is the link between intestine, bone, and kidney together in phosphate regulation. However, we still do not know the complex mechanism of phosphate homeostasis and bone mineralization. The physiological role of FGF23 is to regulate serum phosphate. Secreted mainly by osteocytes and osteoblasts in the skeleton (2-3), it modulates kidney handling of phosphate reabsorption and calcitriol production. Genetic and acquired abnormalities in FGF23 structure and metabolism cause conditions of either hyper-FGF23 or hypo-FGF23. Hyper-FGF23 is related to hypophosphatemia, while hypo-FGF23 is related to hyperphosphatemia. Both hyper-FGF23 and hypo-FGF23 are detrimentalto humans. In this review, we will discuss the pathophysiology of FGF23 and hyper-FGF23 related renal phosphate wasting disorders (4 IntroductionAccording to pathogenesis, ricketsis classified into calicopenic rickets, phosphopenicrickets, and a miscellaneous group associated with direct inhibitors of mineralization ( 5). In general, most instances of nutritional rickets are calicopenic, whereas heritable causes are usually phosphopenic. In this review we focus on hypophosphatemic rickets or osteomalacia. The causes of hypophosphatemia are various, of whichthe most prominent one is decreased reabsorption of phosphate in the proximal tubule.Although renal tubular disease can result in excessive renal phosphate wasting, in most hypophosphatemic disordersno abnormalities are found in the proximal tubule (6-7). It is speculated that an unknown factor is responsible for this phenomenon. The discovery of fibroblast growth factor 23 (FGF23), a member of the Phosphate homeostasisPhosphate comprises about 1% of total body weight. About 85% of total body phosphate resides in the bone, 14% in the cells, and only 1% in the serum and extracellular fluids. Maintenance of serum phosphate within its normal range allows for optimal mineralization of bone without deposition in vascular and other soft tissues. Serum phosphate concentration is determined Xianglan Huang et al. www.boneresearch.org | Bone Research 121by the balance among intestinal absorption of phosphate from the diet, its storage in bone, and its excretion in the urine. The proximal tubule is responsible for the reabsorption of phosphate filtered at the glomerulus and is the primary regulator of phosphate balance in the body. Transportation in the proximal tubule is driven primarily by sodium-potassium ATPase, which is located in the baso-lateral membrane of the cell (8-11). Under normal conditions, about 85% of the filtered phosphate is reabsorbed via the sodium-phosphate co-transporter (NaPi2a and NaPi2c) in the proximal tubule (9, 11). Parathyroid hormone (PTH) is one of the most potent horm...

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Case report: Oncogenous osteomalacia: A new case secondary to a malignant tumor

Cited by 12 publications

References 21 publications

Most Osteomalacia-associated Mesenchymal Tumors Are a Single Histopathologic Entity

Most Osteomalacia-associated Mesenchymal Tumors Are a Single Histopathologic Entity

Hyperparathyroidism in Metastases of Prostatic Carcinoma: A Biochemical, Hormonal and Histomorphometric Study

FGF23 and Phosphate Wasting Disorders

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