Adult-onset hypophosphatemic osteomalacia secondary to neoplasm. A case report and review of the pathophysiology.

Gitelis, Steven; Ryan, Will G.; Rosenberg, A G; Templeton, Angela

doi:10.2106/00004623-198668010-00018

Cited by 16 publications

(3 citation statements)

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“…This is reflected in the wide variety of diagnoses given in the roughly 20 cases reported between 1972 and 1987, including hemangiopericytoma, giant cell tumor of bone, sclerosing hemangioma, and angioliIn 1987, as noted above, Weidner and Santa Cruz 103 published the first comprehensive study of OO-associated mesenchymal tumors and coined the term "phosphaturic mesenchymal tumor, mixed connective tissue variant." As part of this seminal series, those cases previously published under a variety of diagnoses19,26,33,85,102,105 were re-reviewed and reclassified as PMTMCT. Additionally, Weidner and Santa Cruz 103 reclassified those cases previously reported by others10,30,110 as osteoblastoma-like PMT, the cases reported by others 6,70 as nonossifying fibroma-like PMT, and the case of Nomura as an ossifying fibroma-like PMT.…”

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confidence: 99%

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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confidence: 99%

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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“…[3][4][5][6][7] OO-associated mesenchymal tumors are in most cases histologically distinctive, 3,8 although a wide variety of diagnoses have been given to these tumors such as hemangiopericytoma, giant cell tumor of bone, sclerosing hemangioma, and angiolipoma. [9][10][11][12][13][14] The term ''phosphaturic mesenchymal tumor'' (PMT) was coined by Weidner and Santa Cruz who described the pathologic features of 17 mesenchymal tumors that caused osteomalacia or rickets. 1 They proposed classifying OO-associated tumors into the following categories: primitive-appearing mixed connective tissue tumors; osteoblastoma-like tumors; nonossifying fibroma-like tumors; and ossifying fibroma-like tumors.…”

Section: Discussionmentioning

confidence: 99%

Phosphaturic mesenchymal tumor diagnosed by fine‐needle aspiration and core biopsy: A case report and review of literature

Policarpio‐Nicolas

Abbott

Dalkin

et al. 2008

Diagnostic Cytopathology

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Oncogenic (tumor-induced) osteomalacia is a rare paraneoplastic syndrome of phosphate wasting that is frequently associated with phosphaturic mesenchymal tumor (PMT). As the cytologic features of this tumor apparently have not been reported, we describe the fine-needle aspiration (FNA) findings for PMT that arose from the gluteal soft tissue in a patient with hypophosphatemia and multiple fractures secondary to osteomalacia. Smears from the computerized tomography (CT)-guided FNA showed groups of spindle cells having elongated nuclei, fine to moderately coarsely granular chromatin, inconspicuous nucleoli, and delicate cytoplasm. Marked nuclear atypia, mitotic figures, and necrosis were absent. The differential diagnosis included a variety of benign and malignant spindle cell neoplasms such as monophasic synovial sarcoma, leiomyoma, peripheral nerve sheath tumor, fibrosarcoma, and, less likely, metastatic melanoma and sarcomatoid carcinoma. The bland-appearing cytologic features of a spindle cell tumor in a patient with osteomalacia should suggest the diagnosis of PMT.

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“…However, there are difficulties in the histological diagnosis of haemangiopericytoma because other soft tissue neoplasms may have areas of rich "haemangiopericytoma-like" vascularity 6,7 . It occurs commonly in fourth to fifth decade of life and shows no sex predilection 8 . Spread is not a common feature of the tumour and reports of spread occur frequently after incomplete surgical resection 4.…”

Section: Introductionmentioning

confidence: 99%

Malignant haemangiopericytoma of the left thigh with metastases to the bones and lungs: A case report

Atalabi¹,

Ibinaiye²,

Obajimi³

et al. 2010

Nigerian Journal of Surgical Research

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Adult-onset hypophosphatemic osteomalacia secondary to neoplasm. A case report and review of the pathophysiology.

Cited by 16 publications

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Most Osteomalacia-associated Mesenchymal Tumors Are a Single Histopathologic Entity

Most Osteomalacia-associated Mesenchymal Tumors Are a Single Histopathologic Entity

Phosphaturic mesenchymal tumor diagnosed by fine‐needle aspiration and core biopsy: A case report and review of literature

Malignant haemangiopericytoma of the left thigh with metastases to the bones and lungs: A case report

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