Phosphaturic mesenchymal tumor with chondromyxoid fibroma-like feature: an unusual morphological appearance

Suryawanshi, Pallavi; Agarwal, Manish; Dhake, Rahul; Desai, Saral; Rekhi, Bharat; Dr, K B Vijaya Mohan Reddy; Jambhekar, Nirmala A.

doi:10.1007/s00256-011-1159-6

Cited by 14 publications

(8 citation statements)

References 23 publications

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“…There is a roughly equal distribution between hard and soft tissue with perhaps a slight predilection in bone (53 %) [11][12][13]. Clinical presentation is related to anatomic site affected and may include bone pain, multiple bone fractures, gait disturbance, atrophy of proximal muscles, and osteopenia.…”

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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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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“…Although we believe the morphological features of PMTMCT to be sufficiently characteristic to allow correct diagnosis in the great majority of cases, even without ancillary FGF-23 testing,1 9 there is some morphological overlap between PMTMCT, ABC and CMF, in particular CMF in juxtacortical locations 20 21. Obviously, FGF-23 testing is not of value in the distinction of PMTMCT from these two entities.…”

Section: Discussionmentioning

confidence: 86%

Frequent expression of fibroblast growth factor-23 (FGF23) mRNA in aneurysmal bone cysts and chondromyxoid fibromas

et al. 2012

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Osteomalacia has multiple aetiologies including the least common, tumour-induced osteomalacia (TIO). Recently, most cases of TIO have been confirmed to be due to phosphaturic mesenchymal tumour of mixed connective tissue type (PMTMCT). Most cases of TIO are the result of production of the fibroblast growth factor-23 (FGF-23) by the tumour. The authors recently showed reverse transcriptase PCR (RT-PCR) for FGF-23 to be valuable in the diagnosis of PMTMCT. However, the authors also noted FGF-23 expression in some cases of aneurysmal bone cyst (ABC) and chondromyxoid fibroma (CMF). For the present study, the authors studied FGF-23 expression by RT-PCR in 19 cases of ABC and eight cases of CMF, all with typical clinical and radiographic features and without evidence of TIO. Seven of 16 (44%) ABC and two of seven (29%) CMF were positive for FGF-23. These results confirm that ABC and CMF not uncommonly express FGF-23. These results strongly suggest caution and careful integration with all other clinical and radiographic data in the use of FGF-23 RT-PCR for the diagnosis of PMTMCT.

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“…The expression level of the FGF23 gene transcript in the chondromyxoid fibroma was also lower than observed in PMTs with TIO; however, it was comparable to PMTs without TIO. These results raise a concern as to whether chondromyxoid fibroma can be completely distinguished from the non-phosphaturic variant of PMT, because it is occasionally challenging to differentiate chondromyxoid fibroma from PMT [ 24 , 25 ]. Chondromyxoid fibromas typically show a distinct lobular architecture and rarely contain dilated blood vessels.…”

Section: Resultsmentioning

confidence: 99%

Immunohistochemical and molecular detection of the expression of FGF23 in phosphaturic mesenchymal tumors including the non-phosphaturic variant

et al. 2016

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BackgroundPhosphaturic mesenchymal tumors (PMTs) are rare neoplasms that are often associated with tumor-induced osteomalacia (TIO) due to excessive serum levels of fibroblast growth factor 23 (FGF23). PMTs share overlapping histologic features with other types of tumors; thus, accurate pathological diagnosis may be challenging. We performed an immunohistochemical examination of FGF23 expression in PMTs and other types of tumors, together with pertinent molecular analyses.MethodsSeven PMTs (5 with TIO and 2 without TIO) and 46 other types of bone and soft tissue tumors were retrieved, and immunohistochemistry was performed using a commercially available anti-FGF23 antibody. In addition, FGF23 mRNA expression was detected by reverse transcription-polymerase chain reaction (RT-PCR), using RNA extracted from formalin-fixed, paraffin-embedded tissues.ResultsImmunohistochemical analysis of FGF23 expression showed distinct, punctate staining in the cytoplasm in 5 PMTs with TIO, whereas FGF23 expression was negative in the 2 PMTs without TIO and the other 46 tumors. FGF23 mRNA expression was detected in all 4 PMTs examined, as well as in 1 chondromyxoid fibroma and 1 myxoid liposarcoma. The real-time RT-PCR data showed that the relative expression levels of the FGF23 mRNA tended to be higher in PMTs with TIO than in PMTs without TIO, or in the chondromyxoid fibroma specimen.ConclusionsOur data suggested that the feasibility of immunohistochemical detection of FGF23 may depend on the level of secreted FGF23 from tumor cells. Thus, immunohistochemistry for FGF23 is an useful diagnostic adjunct for PMT, although its utility appears to be limited in cases without TIO.

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Phosphaturic mesenchymal tumor with chondromyxoid fibroma-like feature: an unusual morphological appearance

Cited by 14 publications

References 23 publications

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

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

Frequent expression of fibroblast growth factor-23 (FGF23) mRNA in aneurysmal bone cysts and chondromyxoid fibromas

Immunohistochemical and molecular detection of the expression of FGF23 in phosphaturic mesenchymal tumors including the non-phosphaturic variant

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