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Year : 2010  |  Volume : 53  |  Issue : 2  |  Page : 384-385
Phosphaturic mesenchymal tumor of ethmoid sinus


1 Department of Pathology, SDM College of Medical Sciences & Hospital, Manjushree Nagar, Dharwad - 580 009, India
2 Department of Radiodiagnosis, SDM College of Medical Sciences & Hospital, Manjushree Nagar, Dharwad - 580 009, India

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Date of Web Publication12-Jun-2010
 

How to cite this article:
Parshwanath H A, Kulkarni PR, Rao R, Joshi S K, Patil P. Phosphaturic mesenchymal tumor of ethmoid sinus. Indian J Pathol Microbiol 2010;53:384-5

How to cite this URL:
Parshwanath H A, Kulkarni PR, Rao R, Joshi S K, Patil P. Phosphaturic mesenchymal tumor of ethmoid sinus. Indian J Pathol Microbiol [serial online] 2010 [cited 2014 Oct 22];53:384-5. Available from: http://www.ijpmonline.org/text.asp?2010/53/2/384/64317


Sir,

'Phosphaturic mesenchymal tumor: A case report' was published in this journal in 2004. [1] We also have seen a similar case, which is rare and hence would like to report. A 42-year-old woman with history of osteomalacia since three and half years was admitted to the department of ear, nose and throat with a mass in the left nasal cavity. The biochemical profile showed hypophosphatemia, normal serum calcium, slightly elevated alkaline phosphatase (serum phosphorus - 1.3 mg/dl, serum calcium - 9.4 mg/dl, ALP - 195 IU/L) and urine examination showed only abnormal phosphaturia, which was 976.7 mg/day.

X-ray pelvis showed Looser's zone suggesting osteomalacia [Figure 1]. Plain and contrast enhanced computed tomography (CECT) of the paranasal sinuses showed a moderately vascular heterogenous necrotic soft tissue mass with cystic areas within, having epicentre in the nasal cavity and ethmoid sinus with destruction of surrounding bones [Figure 2]. Within about three weeks after excision of the tumor, the clinical symptoms began to resolve and biochemical parameters came to normal levels (serum phosphorus - 4.3 mg/dl, serum calcium - 10.6 mg/dl, ALP - 190 IU/L), and after three months she was totally asymptomatic and X-ray pelvis was normal.

Grossly, the tumor tissue consisted of multiple gray-brown and gray-yellow fragments admixed with blood clots weighing 100 gm and ranging from 3 x 2 x 0.5 cm to 1 x 0.5 x 0.5 cm.

Microscopically, hematoxylin and eosin stained sections showed tumor composed of small round to oval fairly uniform cells arranged in compact sheets, having moderate amount of pale eosinophilic cytoplasm and ill-defined cytoplasmic borders. Cytoplasmic vacuolation was seen in few of the cells [Figure 3]. There was no atypia of cells. Mitotic figures were seen occasionally. Numerous osteoclastic giant cells were present with areas of hemorrhage and collagenous tissue with increased vascularity and a few dilated blood vessels [Figure 3]. Routine special stains and immunohistochemical stains were noncontributory [Figure 4].

In view of these histologic findings and clinical settings, diagnosis of phosphaturic mesenchymal tumor was made.

Oncogenic osteomalacia is a heterogenous group of disorder most often caused by phosphaturic mesenchymal tumor. A wide variety of other tumors have been reported to cause this condition including hemangiopericytoma, chondroblastoma, chondroma, osteoblastoma, fibroangioma and chondrosarcoma. [2] Oncogenic osteomalacia usually affects adults of both sexes with a peak incidence in fourth decade (mean 33 years, range 5-63 years). [3] The tumor is commonly seen in the extremities and head and neck regions i.e., mandible, sinuses and nasopharynx. It is also described in the temporal bone, oral and maxillofacial region. [3],[4]

The pathogenic mechanism of this syndrome is still unclear; recently it has been shown that these tumors overexpress fibroblast growth factor-23(FGF-23) capable of inhibiting renal tubular epithelial phosphate transport. [5]

Oncogenic osteomalacia should be suspected in any patient presenting with a metabolic bone disease associated with hypophosphatemia and inappropriate phosphaturia. She should be carefully investigated for the presence of an underlying tumor, which should be excised and subjected for histopathological examination for confirmation. Despite the rarity of this condition, it is of significant clinical importance as its recognition has profound implications for early and successful treatment.


   Acknowledgments Top


The authors express sincere thanks to Dr. Lakshmi Rao, Professor and Head, Department of Pathology, KMC Manipal - 576104, for helping in the immunohistochemical study and Dr. Raghunath Shanbhag and Dr. Ashok Naik, Department of ENT, and Dr. Suresh Dugani, Department of Surgery (Neuro), SDM College of Medical Sciences & Hospital, Dharwad - 580 009.

 
   References Top

1.Nayak A, Sharma SG, Tandon N, Ray R. Phosphaturic mesenchymal tumour: A case report. Indian J Pathol Microbiol 2004;47:530-3.  Back to cited text no. 1      
2.Kaylie DM, Jackson CG, Gardner EK. Oncogenic osteomalacia caused by phosphaturic mesenchymal tumour of the temporal bone. Otolaryngol Head Neck Surg 2006;135:653-4.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]  
3.Weidner N, Santa Cruz D. Phosphaturic mesenchymal tumours: A polymorphous group causing osteomalacia or rickets. Cancer 1987;59:1442-54.  Back to cited text no. 3  [PUBMED]    
4.Ahn J, Kim H, Cha C, Kim J, Yim S, Kim H. Oncogenic Osteomalacia: induced by tumour, cured by surgery. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007;103:636-41.  Back to cited text no. 4      
5.Folpe AL, Fanburg-Smith JC, Billings SD, Bisceglia M, Bertoni F, Cho JY, et al. Most Osteomalacia-associated Mesenchymal Tumors Are a Single Histopathologic Entity: An Analysis of 32 Cases and a Comprehensive Review of the Literature. Am J Surg Pathol 2004;28:1-30.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]  

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Correspondence Address:
Padmaja R Kulkarni
Department of Pathology, SDM College of Medical Sciences & Hospital, Manjushree Nagar, Dharwad - 580 009
India
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DOI: 10.4103/0377-4929.64317

PMID: 20551573

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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