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Varied presentation of sinonasal phosphaturic mesenchymal tumour: report of a case series with follow-up

  • Regi KurienEmail author
  • Vedantam Rupa
  • Meera Thomas
Rhinology
  • 33 Downloads

Abstract

Purpose

Phosphaturic mesenchymal tumour (PMT) of the paranasal sinuses is a rare tumour that is associated with oncogenous osteomalacia causing predominant musculoskeletal symptoms. We present a series of eight patients diagnosed to have PMT of the paranasal sinuses with varied presentation and highlight the appropriate evaluation required to arrive at the diagnosis.

Methods

Retrospective review of eight patients diagnosed to have PMT-causing tumour-induced osteomalacia with follow-up data.

Results

Eight patients, 4 males and 4 females, aged 36–67 years (mean = 44 years) presented with vague musculoskeletal symptoms (6 patients) or epistaxis (3 patients). Six patients were found to have hypophosphatemia, phosphaturia and raised FGF-23 levels preoperatively. All eight patients were found to have a tumour in the nose and/ paranasal sinuses with one patient having intracranial extension. All patients were treated with endoscopic excision of these tumours which resulted in resolution of symptoms and normalisation of biochemical abnormalities. In addition, one patient required a craniofacial resection. Histopathological features were consistent with PMT mixed connective tissue variant. Two atypical patients were seen. The longest follow-up was for 5 years and there was no evidence of recurrence during the follow-up period in any patient.

Conclusion

Diagnosis of PMT of the paranasal sinuses causing oncogenous osteomalacia requires a high index of suspicion when there are no ENT symptoms. Appropriate biochemical tests and histopathology lead to the correct diagnosis. Total endoscopic surgical excision leads to a good outcome.

Keywords

Oncogenic osteomalacia Paranasal sinus Hypophosphatemia Hemangiopericytoma 

Notes

Funding

None.

Compliance with ethical standards

Conflict of interest

The authors declare no conflict of interest.

Ethical approval

All procedures performed in this study were in accordance with the ethical standards of the institutional research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. Institutional review board approval was obtained prior to the commencement of the retrospective study (IRB No:11,448).

Informed consent

Informed consent was obtained from all the participants in the study.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of ENTChristian Medical College and HospitalVelloreIndia
  2. 2.Department of PathologyChristian Medical College and HospitalVelloreIndia

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