DSS 2010-1
-
Upload
ayanna-gibbs -
Category
Documents
-
view
38 -
download
0
description
Transcript of DSS 2010-1
![Page 1: DSS 2010-1](https://reader036.fdocuments.in/reader036/viewer/2022062408/5681353a550346895d9c9e59/html5/thumbnails/1.jpg)
DSS 2010-1DSS 2010-1
Sarah E. Martin, M.D.Eyas M. Hattab, M.D.
Indiana University School of Medicine86th Annual Meeting of the AANP
June 10-13, 2010
![Page 2: DSS 2010-1](https://reader036.fdocuments.in/reader036/viewer/2022062408/5681353a550346895d9c9e59/html5/thumbnails/2.jpg)
Clinical HistoryClinical History
61 yo woman with a history of multiple fractures and hypophosphatemia for 6 yrs
Presented with bilateral weakness and shooting pains in her legs after a fall
MRI: multi-lobulated, vividly enhancing, heterogeneous T12 mass with extension into epidural space and paraspinal musculature
![Page 3: DSS 2010-1](https://reader036.fdocuments.in/reader036/viewer/2022062408/5681353a550346895d9c9e59/html5/thumbnails/3.jpg)
![Page 4: DSS 2010-1](https://reader036.fdocuments.in/reader036/viewer/2022062408/5681353a550346895d9c9e59/html5/thumbnails/4.jpg)
![Page 5: DSS 2010-1](https://reader036.fdocuments.in/reader036/viewer/2022062408/5681353a550346895d9c9e59/html5/thumbnails/5.jpg)
Diagnosis?Diagnosis?
![Page 6: DSS 2010-1](https://reader036.fdocuments.in/reader036/viewer/2022062408/5681353a550346895d9c9e59/html5/thumbnails/6.jpg)
Phosphaturic mesenchymal Phosphaturic mesenchymal tumor, mixed connective tissue tumor, mixed connective tissue
variant (PMTMCT)variant (PMTMCT)
![Page 7: DSS 2010-1](https://reader036.fdocuments.in/reader036/viewer/2022062408/5681353a550346895d9c9e59/html5/thumbnails/7.jpg)
PMTMCTPMTMCT
Definition– Largely benign, morphologically distinct
mesenchymal neoplasm almost invariably associated with oncogenic osteomalacia
Incidence– Extremely rare, ~ 150 cases described in the
literature
Historical perspective– 1987: Weidner and Santa Cruz coined term
– 2004: Folpe et al. fully characterized PMTMCT
![Page 8: DSS 2010-1](https://reader036.fdocuments.in/reader036/viewer/2022062408/5681353a550346895d9c9e59/html5/thumbnails/8.jpg)
![Page 9: DSS 2010-1](https://reader036.fdocuments.in/reader036/viewer/2022062408/5681353a550346895d9c9e59/html5/thumbnails/9.jpg)
PMTMCTPMTMCT Location
– Soft tissues and bones of the extremities
– Only rarely in axial skeleton (index case)
Etiology
– FGF-23 overexpression: inhibits trans-epithelial phosphate transport in renal tubules, resulting in renal phosphate wasting and subsequent osteomalacia
Clinical features:
– 25-77 years; female predominance
– Bone pain, multiple fractures
– Hypophosphatemia, hyperphosphaturia, and osteomalacia; fail vitamin D therapy
– Chronic, protracted history before tumor is discovered
![Page 10: DSS 2010-1](https://reader036.fdocuments.in/reader036/viewer/2022062408/5681353a550346895d9c9e59/html5/thumbnails/10.jpg)
PMTMCT: pathologyPMTMCT: pathology Gross:
– Superficial and deep soft tissues– Well-circumscribed
Microscopic – Low cellularity, bland spindled cells– Myxochondroid/osteoid-like matrix– Dystrophic calcification, incomplete rim of ossification – HPC-like vessels, microcysts, hemorrhage– Osteoclast-like giant cells
Immunohistochemistry – FGF-23
![Page 11: DSS 2010-1](https://reader036.fdocuments.in/reader036/viewer/2022062408/5681353a550346895d9c9e59/html5/thumbnails/11.jpg)
![Page 12: DSS 2010-1](https://reader036.fdocuments.in/reader036/viewer/2022062408/5681353a550346895d9c9e59/html5/thumbnails/12.jpg)
![Page 13: DSS 2010-1](https://reader036.fdocuments.in/reader036/viewer/2022062408/5681353a550346895d9c9e59/html5/thumbnails/13.jpg)
![Page 14: DSS 2010-1](https://reader036.fdocuments.in/reader036/viewer/2022062408/5681353a550346895d9c9e59/html5/thumbnails/14.jpg)
![Page 15: DSS 2010-1](https://reader036.fdocuments.in/reader036/viewer/2022062408/5681353a550346895d9c9e59/html5/thumbnails/15.jpg)
![Page 16: DSS 2010-1](https://reader036.fdocuments.in/reader036/viewer/2022062408/5681353a550346895d9c9e59/html5/thumbnails/16.jpg)
![Page 17: DSS 2010-1](https://reader036.fdocuments.in/reader036/viewer/2022062408/5681353a550346895d9c9e59/html5/thumbnails/17.jpg)
![Page 18: DSS 2010-1](https://reader036.fdocuments.in/reader036/viewer/2022062408/5681353a550346895d9c9e59/html5/thumbnails/18.jpg)
![Page 19: DSS 2010-1](https://reader036.fdocuments.in/reader036/viewer/2022062408/5681353a550346895d9c9e59/html5/thumbnails/19.jpg)
PMTMCT: pathologyPMTMCT: pathology Differential diagnosis:
– Hemangiopericytoma– Giant cell tumor– Osteoblastoma– Osteosarcoma– Mesenchymal chondrosarcoma– Hemangioma
Keys to diagnosis:– Awareness of entity– Clinical history of osteomalacia (not universal)– Morphologic heterogeneity
![Page 20: DSS 2010-1](https://reader036.fdocuments.in/reader036/viewer/2022062408/5681353a550346895d9c9e59/html5/thumbnails/20.jpg)
PMTMCT: managementPMTMCT: management Treatment
– Surgical resection is curative of both tumor and osteomalacia
Prognosis– Generally excellent after complete resection– Rare malignant forms have been reported
Increased mitoses, high nuclear grade and high cellularity
Local recurrence; lung and skeletal metastases reported
![Page 21: DSS 2010-1](https://reader036.fdocuments.in/reader036/viewer/2022062408/5681353a550346895d9c9e59/html5/thumbnails/21.jpg)
![Page 22: DSS 2010-1](https://reader036.fdocuments.in/reader036/viewer/2022062408/5681353a550346895d9c9e59/html5/thumbnails/22.jpg)
The end.The end.