Sinonasal Phosphaturic Mesenchymal Tumor: Case Report and Systematic Review

Deep, Nicholas L.; Cain, Rachel B.; McCullough, Ann E.; Hoxworth, Joseph M.; Lal, Devyani

doi:10.2500/ar.2014.5.0100

Cited by 30 publications

(43 citation statements)

References 44 publications

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“…Only one of the tumors in our series was originally diagnosed as PMT, a pattern consistent with previously published studies [1,5,10]. Tumors arising in the head and neck may be more prone to misdiagnosis as these sites are often biopsied and the small amount of material provided may not demonstrate features that distinguish PMT from similar tumors.…”

Section: Discussionsupporting

confidence: 74%

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Phosphaturic Mesenchymal Tumor Involving the Head and Neck: A Report of Five Cases with FGFR1 Fluorescence In Situ Hybridization Analysis

Wasserman

Purgina

Lai

et al. 2016

Head and Neck Pathol

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Phosphaturic mesenchymal tumor (PMT) is a rare neoplasm; however, it is the most common cause of tumor-induced osteomalacia (TIO), a paraneoplastic syndrome characterized by renal phosphate wasting and hypophosphatemia. A subset of PMTs harbours an FGFR1 translocation although this alteration has not been demonstrated in PMT involving a head and neck site. We present a series of five PMTs involving the head and neck and demonstrate the diagnostic utility of fluorescence in situ hybridization (FISH) for detecting FGFR1 translocations. Patients' age and sex, tumor location, original diagnosis, the duration of symptoms, the presence of TIO, biochemical results, and medical management were reviewed. The median age at presentation was 45 (range, 24-58 years) and TIO was present in three cases. Four tumors involved soft tissue and one involved bone. Four out of the five tumors in our series were initially misdiagnosed. Three tumors were ultimately categorized as malignant PMT (two patients developed metastatic disease). FGFR1 translocation was present in two out of four cases and remained unknown in one case. In summary, we report on five cases of PMTs arising in the head and neck and confirm utility of FGFR1 FISH in the diagnosis of a subset of PMT.

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Section: Discussionsupporting

confidence: 74%

“…Tumors involving the head and neck are extremely rare [3,[5][6][7][8][9]. Half of all PMTs reported in the head and neck have involved the sinonasal tract; other sites include the pharynx, maxilla, mandible, tongue, floor of mouth, and posterior neck.…”

Section: Introductionmentioning

confidence: 99%

Phosphaturic Mesenchymal Tumor Involving the Head and Neck: A Report of Five Cases with FGFR1 Fluorescence In Situ Hybridization Analysis

Wasserman

Purgina

Lai

et al. 2016

Head and Neck Pathol

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“…The most common location for PMT involves the extremities (95 %) followed by head and neck (H&N) sites (5 %) with the sinonasal cavity representing more than half of the cases [9,10]. There is a roughly equal distribution between hard and soft tissue with perhaps a slight predilection in bone (53 %) [11][12][13].…”

Section: Introductionmentioning

confidence: 99%

Phosphaturic Mesenchymal Tumor: 2 New Oral Cases and Review of 53 Cases in the Head and Neck

Qari

Hamao-Sakamoto

Fuselier

et al. 2015

Head and Neck Pathol

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Phosphaturic mesenchymal tumor (PMT) is a rare neoplasm that secretes fibroblast growth factor-23 (FGF-23) and causes oncogenic osteomalacia. It occurs in adults with equal gender distribution and the most common location is the lower extremities, followed by the head and neck. Besides osteomalacia, the clinical presentation includes bone pain and multiple bone fractures. Microscopic features consist of spindle cells, multinucleated giant cells, and calcifications embedded in a chondromyxoid matrix. Laboratory findings indicate normal calcium and parathyroid levels, hypophosphatemia, and increased levels of FGF-23 that usually revert to normal after surgical removal. Due to its rarity, the purpose of the study was to report 2 new oral cases of PMT and to review the literature in the head and neck. The first case occurred in the gingiva and had been present for 6 years. The second case was a recurrence of a previously diagnosed PMT in the right mandible that metastasized to the lung and soft tissue. The literature review included 53 cases in the head and neck. There was a predilection for extra-oral sites (76 %) compared to intra-oral sites (24 %) with paranasal sinuses considered the most common location (38 %) followed by the mandible (15 %). There were 9 recurrences that included 3 malignant cases indicating a potentially aggressive tumor. Due to the indeterminate biological behavior of PMT and its rarity, a comprehensive evaluation of medical, laboratory, radiographic, and histological findings are crucial for a definitive diagnosis and treatment.

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“…A total of 4 cases (57.14%) had recurrence during the follow-up. [6,9,10] In our study, the biochemical indicators of case 1 showed no abnormities which made diagnosis di cult.…”

Section: Discussionmentioning

confidence: 53%

Phosphaturic mesenchymal tumor of the occipitocervical region: Report of two rare cases and literature review

Zhang

Yan

et al. 2020

Preprint

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BackgroundTumor-induced osteomalacia (TIO) is regarded as a rare paraneoplastic syndrome mainly caused by phosphaturic mesenchymal tumor (PMT). To our known, only 5 occipitocervical PMTs have been described in the world’s English literature. We reported two rare cases of occipitocervical PMT, and conducted a retrospective analysis of these 7 cases. The purpose of this study is to discuss the clinical characteristics and treatment of occipitocervical PMT.Case PresentationBoth patients were middle-aged females, and had a long-standing bone pain. In case 1, there were no abnormalities in biochemical indicators. The blood phosphorus was normal and alkaline phosphatase (ALP) was elevated in case 2. Magnetic resonance imaging (MRI) suggested that osteolytic bone destruction accompanied by a soft tissue mass in left C1-2 vertebra (case 1). In case 2, the bone destruction was located on the right C1-2 and the clivus. Then both patients underwent complete resection of tumor, and case 2 also received adjuvant radiotherapy, the histopathology revealed a PMT. Case 2 suffered recurrence during 5-year follow-up.ConclusionsOccipitocervical PMT is quite rare, and only 5 cases have been reported in the literature. Currently, complete resection of the tumor is the best option. The surgery is difficult, and requires delicate operation due to the complex anatomy of the occipitocervical region. Postoperative radiotherapy has little effect on local control. And further research is needed to confirm the effectiveness of the newly-emerged therapies.

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Sinonasal Phosphaturic Mesenchymal Tumor: Case Report and Systematic Review

Cited by 30 publications

References 44 publications

Phosphaturic Mesenchymal Tumor Involving the Head and Neck: A Report of Five Cases with FGFR1 Fluorescence In Situ Hybridization Analysis

Phosphaturic Mesenchymal Tumor Involving the Head and Neck: A Report of Five Cases with FGFR1 Fluorescence In Situ Hybridization Analysis

Phosphaturic Mesenchymal Tumor: 2 New Oral Cases and Review of 53 Cases in the Head and Neck

Phosphaturic mesenchymal tumor of the occipitocervical region: Report of two rare cases and literature review

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