1 / 22

DSS 2010-1

This article discusses the diagnosis, pathology, and management of phosphaturic mesenchymal tumor (PMT), a rare neoplasm associated with oncogenic osteomalacia. It covers the clinical features, gross and microscopic characteristics, differential diagnosis, and treatment options for PMT.

erincollins
Download Presentation

DSS 2010-1

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. DSS 2010-1 Sarah E. Martin, M.D. Eyas M. Hattab, M.D. Indiana University School of Medicine 86th Annual Meeting of the AANP June 10-13, 2010

  2. Clinical 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

  3. Diagnosis?

  4. Phosphaturicmesenchymal tumor, mixed connective tissue variant (PMTMCT)

  5. PMTMCT • 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

  6. PMTMCT • 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

  7. PMTMCT: 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

  8. PMTMCT: 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

  9. PMTMCT: 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

  10. The end.

More Related