Phosphaturic Mesenchymal Tumor of the Cerebellopontine Angle

Walsh, Erika M.; Jacob, Jeffrey T.; Lucas, David R.; Sargent, Eric W.

doi:10.1001/jamaoto.2018.4045

Cited by 5 publications

(5 citation statements)

References 6 publications

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“…25 On MR imaging, tumors are isointense to soft tissues on T1-weighted imaging and demonstrate enhancement. 26 Intralesional T2 signal is variable. Most commonly, tumors are T2-hyperintense, though Broski et al 22 noted that PMTs characteristically have small foci of intralesional T2 hypointensity.…”

Section: Discussionmentioning

confidence: 99%

“…Thus, they may present a diagnostic conundrum for radiologists, particularly when the diagnosis of TIO is unknown, or, as in this case, if the PMT mimics the appearance of a more common entity. 26 The only known definitive treatment for TIO is surgical resection of the tumor. Fortunately, removal of PMTs results in complete resolution of the biochemical and physical sequelae of the paraneoplastic syndrome.…”

Section: Discussionmentioning

confidence: 99%

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Phosphaturic Mesenchymal Tumor

Benson

Trejo‐Lopez

Nassiri

et al. 2022

AJNR Am J Neuroradiol

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Phosphaturic mesenchymal tumors (PMTs) are neoplasms associated with tumor-induced osteomalacia. Patients typically present with pathologic fractures in the setting of chronic hypophosphatemic hyperphosphaturic osteomalacia, as well as gradual muscle weakness, bone pain, and difficulty walking. Because of their rarity and nonspecific symptomatology, phosphaturic mesenchymal tumors often go undiagnosed for years. Even when discovered on imaging, the tumors can be diagnostically challenging for radiologists. Phosphaturic mesenchymal tumors often tend to be small and can be located nearly anywhere in the body, and, therefore, can mimic many other tumors. This case highlights the imaging and pathologic markers of a phosphaturic mesenchymal tumor, often found in a patient with tumor-induced osteomalacia.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Phosphaturic Mesenchymal Tumor

Benson

Trejo‐Lopez

Nassiri

et al. 2022

AJNR Am J Neuroradiol

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“…There have only been 89 reported cases of head and neck PMTs. 4,[7][8][9][10][11][12][13][14][15] Sinonasal PMTs are particularly rare, representing only 32 of the 89 (36%) reports. [8][9][10][11]13,15,16 The vast majority of sinonasal PMT cases present with TIO.…”

Section: Discussionmentioning

confidence: 99%

“…[4][5][6] Only 89 cases of PMTs involving the head and neck region have been described in the literature. 4,[7][8][9][10][11][12][13][14][15] Of the reported head and neck cases, sinonasal PMTs are extremely rare, representing only 32 of the 89 (36%) reports. [8][9][10][11]13,15,16 Of the 32 reported sinonasal PMT cases, only 4 (12%) were diagnosed without evidence of TIO.…”

Section: Introductionmentioning

confidence: 99%

Phosphaturic Mesenchymal Tumors of the Sinonasal Area and Skull Base: Experience at a Single Institution

Argersinger

Haring

Hanks

et al. 2021

Ann Otol Rhinol Laryngol

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Objectives: Phosphaturic mesenchymal tumor (PMT) is a rare, polymorphous neoplasm with a highly variable presentation and natural history and unpredictable clinical course. The primary objective was to describe our clinical experience with and management of 4 markedly different cases of sinonasal and skull base PMT. Methods: A retrospective case series with chart review, and relevant literature review, was performed at a tertiary academic medical center between 1998 and 2020. Adult patients treated for PMTs of the sinonasal area and skull base were included. Our main outcome measures included postoperative laboratory findings and radiological evidence of disease remission. Results: Four patients (2 Males, 2 Females; Mean Age: 63.5 years) with PMTs of the skull base have been managed at our institution since 1998. Patient presentations varied, ranging from severe phosphorus wasting and osteoporosis to symptoms secondary to mass effect, including nasal obstruction and rhinorrhea. All 4 patients were eventually found to have elevated levels of fibroblast growth factor 23. Tumors were located in the sinonasal area (right frontal sinus, right ethmoid sinus, and right nasal cavity, respectively) in 3 patients and in the lateral skull base (right jugular foramen) in 1 patient. All 4 patients underwent complete surgical resection of their tumors. PMT tissue pathology was confirmed in all cases. Gross total resection was achieved in all patients. There was no chemical or radiological evidence of disease recurrence in any patients at follow-up. Conclusions: The presentation of skull base PMT is variable, and it may mimic other mass pathologies of the head and neck. Complete surgical resection with negative margins is potentially curative.

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“…4 Of the approximately 500 cases of OITs described in previous reports to date, 5 relatively few have been intracranial. 4,[6][7][8][9][10][11][12][13][14][15][16][17][18][19] This report summarizes a case of intracranial OIT and presents a review of the literature.…”

Section: Introductionmentioning

confidence: 99%

Intracranial phosphaturic mesenchymal tumors. A case report and review of literature

et al. 2022

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Most osteomalacia-inducing tumors (OITs) are phosphaturic mesenchymal tumors (PMTs) that secrete fibroblast growth factor 23 (FGF23). These tumors usually occur in the bone and soft tissues, and intracranial OITs are rare. Therefore, intracranial OIT is difficult to diagnose and treat. This paper presents a case of intracranial OIT and shows a review of previous cases. A 45-year-old man underwent nasal cavity biopsy and treatment with active vitamin D 3 and neutral phosphate for hypophosphatemia. Amplification of FGF23 mRNA level within the tumor was detected. Subsequently, the surgical specimen was diagnosed with a PMT and was considered the cause of the patient's osteomalacia. The patient was referred to a neurosurgery department for the excision of the intracranial tumor extending to the nasal cavity. After tumor removal, the serum levels of FGF23 and phosphorus were normalized as compared to preoperative those. The patient remains disease-free, without additional treatment, approximately 10 years after surgery, with no tumor recurrence. As per the literature, intracranial OITs usually occur in patients aged 8-69 years. Bone and muscle pain are major complaints. Approximately 60% of the patients reported previously had symptoms because of intracranial tumors. In some cases, it took several years to diagnose OIT after the onset of the osteomalacia symptoms. Laboratory data in such cases show hypophosphatemia and elevated FGF23 levels. Because FGF23 levels are associated with the severity of osteomalacia symptoms, total tumor resection is recommended. PMT and hemangiopericytoma (HPC) are histologically similar, but on immunochemistry, PMT is negative for signal transducer and activator of transcription 6 (STAT6), whereas HPC is positive. FGF23 amplification is seen in PMTs but not in HPCs. Therefore, the analysis of FGF23 and STAT6 was helpful in distinguishing PMTs from HPCs. In cases of hypophosphatemia and osteomalacia without a history of metabolic, renal, or malabsorptive diseases, the possibility of oncogenic osteomalacia should be considered.

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Phosphaturic Mesenchymal Tumor of the Cerebellopontine Angle

Cited by 5 publications

References 6 publications

Phosphaturic Mesenchymal Tumor

Phosphaturic Mesenchymal Tumor

Phosphaturic Mesenchymal Tumors of the Sinonasal Area and Skull Base: Experience at a Single Institution

Intracranial phosphaturic mesenchymal tumors. A case report and review of literature

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