Oncogenic osteomalacia: strange tumours in strange places

Weiss, Daniel M.; Bar, Robert S.; Weidner, Noel; Wener, Mark H.; Lee, F.

doi:10.1136/pgmj.61.714.349

Cited by 29 publications

(17 citation statements)

References 12 publications

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“…PMT may be small in volume, and in unusual locations (e.g. great toe), and therefore can be difficult to localize [15,17]. The tumors can be radiographically localized by FDG PET/CT; scans should specifically include hands, feet, and the head.…”

Section: Discussionmentioning

confidence: 99%

“…PMTMCT can occur either in the soft tissues or bone. Relevant to this discussion, a number of PMTMCT involving the craniofacial bones and sinonasal tract have been reported and illustrated [17][18][19][20][21][22][23][24][25][26][27][28][29]. Grossly, PMTMCT appear well-circumscribed, but microscopically, all tumors infiltrate the surrounding connective tissues.…”

Section: The Diverse Histologies Of Phosphaturic Mesenchymal Tumorsmentioning

confidence: 99%

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Striking Pathology Gold: A Singular Experience with Daily Reverberations: Sinonasal Hemangiopericytoma (Glomangiopericytoma) and Oncogenic Osteomalacia

Brandwein-Gensler

Siegal

2012

Head and Neck Pathol

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Section: Discussionmentioning

confidence: 99%

Section: The Diverse Histologies Of Phosphaturic Mesenchymal Tumorsmentioning

confidence: 99%

Striking Pathology Gold: A Singular Experience with Daily Reverberations: Sinonasal Hemangiopericytoma (Glomangiopericytoma) and Oncogenic Osteomalacia

Brandwein-Gensler

Siegal

2012

Head and Neck Pathol

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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.…”

mentioning

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

587

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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“…En una semana el dolor lumbar y la impotencia funcional habían desaparecido, el paciente fue dado de alta con jarabe de fosfato y y Friedman demostraron que la resección del tumor normalizaba la fosfatemia 2,3 .…”

Section: Tabla 1 Estudio Complementariounclassified

“…Hasta ahora se han comunicado algo más de 100 casos de osteomalacia oncogénica cuyos elementos esenciales son hipofosfatemia, hiperfosfaturia, normocalcemia, niveles normales de 25 OH D3 y disminuidos de 1-25 OH D3, concentraciones séricas de PTH y péptido relacionado con PTH normales y ausencia de otros defectos en la reabsorción tubular [1][2][3][4][5][6][7][8][9][10][11][12][13][14] . En la Tabla 2 se mencionan los tipos histológicos más frecuentes y en la Tabla 3 sus localizaciones.…”

Section: Tabla 1 Estudio Complementariounclassified

Hipofosfatemia revertida al extirpar hemangioendotelioma compuesto del dedo mayor del pie

Roa²,

A³

2003

Rev. méd. Chile

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Reversion of hypophosphatemia after the excision of a composite hemangioendothelioma in a great toe. Report of one case a hipofosfatemia es un hallazgo relativamente frecuente en pacientes hospitalizados, pero que excepcionalmente tiene relevancia clínica. La hipofosfatemia puede ser el resultado de pérdidas renales, reducción en la absorción intestinal o redistribución del fosfato al interior de la célula. Las pérdidas renales de fosfato explican la mayor parte de las hipofosfatemias y, a su vez, pueden ser consecuencia de defectos intrínsecos de la función tubular, de cambios en la volemia, del efecto de drogas o de anomalías en la secreción de hormonas que regulan la reabsorción tubular de fosfato 1 .A propósito de un caso de hipofosfatemia asociada a un tumor de origen vascular, se discuten recientes avances en la comprensión de la homeostasis del fosfato. CASO CLÍNICOVarón de 43 años, agricultor de etnia mapuche, residente en área rural próxima a Chol-Chol en la Cordillera de la Costa de la provincia de Cautín, que consultó, en julio de 2000 en el policlínico de Reumatología del Hospital Regional de Temuco, por dolor lumbar recurrente de 20 años de evolución, no asociado con traumatismo ni con actividad física, sin irradiación y tratado habitualmente con paracetamol

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Oncogenic osteomalacia: strange tumours in strange places

Cited by 29 publications

References 12 publications

Striking Pathology Gold: A Singular Experience with Daily Reverberations: Sinonasal Hemangiopericytoma (Glomangiopericytoma) and Oncogenic Osteomalacia

Striking Pathology Gold: A Singular Experience with Daily Reverberations: Sinonasal Hemangiopericytoma (Glomangiopericytoma) and Oncogenic Osteomalacia

Most Osteomalacia-associated Mesenchymal Tumors Are a Single Histopathologic Entity

Hipofosfatemia revertida al extirpar hemangioendotelioma compuesto del dedo mayor del pie

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