Phosphaturic Mesenchymal Tumors from Head to Toe: Imaging Findings and Role of the Radiologist in Diagnosing Tumor-Induced Osteomalacia

Kawthalkar, Ameya Shirish; Janu, Amit; Deshpande, Mrunmayee; Gala, Kunal; Gulia, Ashish; Puri, Ajay

doi:10.1007/s43465-019-00005-5

Cited by 10 publications

(7 citation statements)

References 12 publications

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“…The use of USG in the diagnosis of PMT has rarely been described in the literature (24,29,30). No specific ultrasonographic diagnostic features have been associated with these tumors.…”

Section: Discussionmentioning

confidence: 99%

“…However, currently, the major role of cross-sectional imaging, including ultrasound (USG), CT, and MRI, is for better local anatomical definition of the mass before surgery (18). Although functional nuclear medicine scans are well-established for tumor localization in the work-up of TIO, only few publications in the radiologic literature have focused on the imaging features of PMTs (22)(23)(24)(25)(26). Hence, the aim of the present study was to describe the radiological characteristics of histologically proven PMTs that presented as TIO.…”

Section: Introductionmentioning

confidence: 99%

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Imaging characteristics of phosphaturic mesenchymal tumors

et al. 2023

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Background Tumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome associated with phosphaturic mesenchymal tumors (PMTs). Localization of the causative tumor in these cases is an arduous task since the culprit lesions are usually small, slow-growing, and can be located almost anywhere from head to toe. Purpose To describe the morphological characteristics of histologically proven PMTs on various radiological modalities. Material and Methods After institutional ethical approval, this retrospective study analyzed 20 cases with a histopathological evidence of PMT. Various imaging characteristics of the tumors on available computed tomography (CT) and magnetic resonance imaging (MRI) scans were evaluated. Descriptive statistical analyses were conducted. Results The tumors were located in diverse locations: lower extremities (n = 10); head and neck (n = 5); vertebral column (n = 3); pelvis (n = 1); and upper extremities (n = 1). Bone lesions seen on CT had variable morphology: sclerotic (n = 3/8, 37.5%); lytic (n = 3/8, 37.5%), and both lytic and sclerotic (n = 2/8, 25%) with presence of narrow zone of transition in all cases (n = 8/8) and amorphous internal matrix calcifications in 25% of cases (n = 2/8). Of the tumors, 68.4% (n = 13/19) were hypointense on T1 and all of them showed hyperintense signal on T2-weighted and STIR images (n = 19/19) and contrast enhancement (n = 16/16). Of the tumors, 66.7% (n = 6/9) showed restricted diffusion. DOTANOC PET/CT showed tumor uptake in all cases (n = 8/8). Conclusion PMTs may have variable and non-specific tumor appearances on various imaging modalities. However, in an appropriate clinical scenario and a background of suggestive biochemical work-up, the radiologist should keep a high index of suspicion.

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“…The use of USG in the diagnosis of PMT has rarely been described in the literature (24,29,30). No specific ultrasonographic diagnostic features have been associated with these tumors.…”

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Imaging characteristics of phosphaturic mesenchymal tumors

et al. 2023

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“…Noninvasive imaging examination plays an increasingly prominent role in the localization of TIO. X-ray manifestations of TIO are not significantly different from other osteomalacia, which are typically characterized by blurred trabeculae, multiple false fracture lines or fractures, “double concave” changes in the vertebral body and “triangular” changes in the pelvis [ 18 ]. CT and MRI can find hidden fracture and intraosseous edema, and make suggestive diagnosis for adult hypophosphorus osteomalacia patients who have ruled out other causes, but lack specificity [ 19 , 20 ].…”

Section: Discussionmentioning

confidence: 99%

Tumor-induced osteomalacia characterized by “painful knee joint with difficulty in moving”: a case report

et al. 2022

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Background Tumor-related osteomalacia (TIO) is a rare paraneoplastic syndrome characterized by severe hypophosphatemia and osteomalacia. The diagnosis of TIO can be very difficult because of its nonspecific nature of clinical manifestations. Here we reported a case of young TIO patient with “painful knee joint with difficulty in moving” to improve the clinical diagnosis and treatment levels. Case presentation The patient’s clinical features were consistent with TIO. A tumor was successfully located in left tibial by 68Ga-DOTATATE PET/CT, and then was surgically resected. Upon pathologic assessment, the tumor was diagnosed as phosphaturia stromal tumor (PMT) with positive Vim staining. After the surgery, serum phosphate level rapidly recovered and symptoms significantly improved. Conclusion TIO should be considered in patients with chronically hypophosphorus osteomalacia in the setting of no family history. Early removal of the responsible tumors is clinically essential for the treatment, and imaging examination is of great significance for tumor localization.

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“…X-rays are often the first radiological technique performed in symptomatic patients. Common plain radiographic findings include coarse trabeculae, thinned-out cortex, and Looser’s zones, manifestations of generalized osteopenia in adults, and rickets in children [ 19 ]. X-rays of the foot and pelvis commonly demonstrate non-united osteomalacic fractures [ 18 ] (Fig.…”

Section: Radiological Featuresmentioning

confidence: 99%

Phosphaturic mesenchymal tumors: radiological aspects and suggested imaging pathway

et al. 2021

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Phosphaturic mesenchymal tumors (PMTs) are rare mesenchymal neoplasms of soft tissue or bone origin that can give rise to a challenge in diagnostic imaging. These tumors are frequently associated with tumor-induced osteomalacia, also called oncogenic osteomalacia, which is a rare paraneoplastic syndrome characterized by ectopic secretion of fibroblast growth factor 23, a hormone that regulates serum phosphate level. PMTs show polymorphic features on both radiological findings and histological examination, causing problems in diagnosis owing to their similarity with other mesenchymal tumors. Thus, this paper aims to describe radiological aspects of PMTs and suggest an imaging pathway for accurate diagnosis throughout the evidence from the literature review.

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Phosphaturic Mesenchymal Tumors from Head to Toe: Imaging Findings and Role of the Radiologist in Diagnosing Tumor-Induced Osteomalacia

Cited by 10 publications

References 12 publications

Imaging characteristics of phosphaturic mesenchymal tumors

Imaging characteristics of phosphaturic mesenchymal tumors

Tumor-induced osteomalacia characterized by “painful knee joint with difficulty in moving”: a case report

Phosphaturic mesenchymal tumors: radiological aspects and suggested imaging pathway

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