Phosphaturic mesenchymal tumour, mixed connective tissue type

From Libre Pathology
Revision as of 17:52, 6 June 2014 by Michael (talk | contribs)
Jump to navigation Jump to search
Phosphaturic mesenchymal tumour, mixed connective tissue type
Diagnosis in short
LM spindle cells without atypia
IHC vimentin +ve, pankeratin -ve, S-100 -ve
Site soft tissue, bone
Blood work low serum phosphate
Clin. DDx autosomal dominant hypophosphatemic rickets

Phosphaturic mesenchymal tumour, mixed connective tissue type, abbreviated PMTMCT, is a rare tumour.

General

Clinical DDx:

  • Autosomal dominant hypophosphatemic rickets.[3]

Gross

  • Soft tissue or bone.[4]
  • Often small - thus hard to identify.[5]

Microscopic

Features:[2][3]

  • Spindle cells without atypia in an eosinophilic matrix.
  • Osteoclast-type giant cells.
  • +/-Calcifications.

Image:

IHC

Features:[3]

  • Vimentin +ve.
  • Pankeratin -ve.
  • S-100 -ve.
  • CD34 -ve.

Others:[6]

  • CD68 +ve.(???)
  • Desmin -ve.

References

  1. ↑ Papierska, L.; Ćwikła, JB.; Misiorowski, W.; Rabijewski, M.; Sikora, K.; Wanyura, H. (2013). "Unusual case of phosphaturic mesenchymal tumor.". Pol Arch Med Wewn 123 (5): 255-6. PMID 23722193.
  2. ↑ 2.0 2.1 William, J.; Laskin, W.; Nayar, R.; De Frias, D. (Aug 2012). "Diagnosis of phosphaturic mesenchymal tumor (mixed connective tissue type) by cytopathology.". Diagn Cytopathol 40 Suppl 2: E109-13. doi:10.1002/dc.21647. PMID 22927293.
  3. ↑ 3.0 3.1 3.2 3.3 Woo, VL.; Landesberg, R.; Imel, EA.; Singer, SR.; Folpe, AL.; Econs, MJ.; Kim, T.; Harik, LR. et al. (Dec 2009). "Phosphaturic mesenchymal tumor, mixed connective tissue variant, of the mandible: report of a case and review of the literature.". Oral Surg Oral Med Oral Pathol Oral Radiol Endod 108 (6): 925-32. doi:10.1016/j.tripleo.2009.07.005. PMC 2783479. PMID 19828339. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2783479/.
  4. ↑ Jiang, Y.; Xia, WB.; Xing, XP.; Silva, BC.; Li, M.; Wang, O.; Zhang, HB.; Li, F. et al. (Sep 2012). "Tumor-induced osteomalacia: an important cause of adult-onset hypophosphatemic osteomalacia in China: Report of 39 cases and review of the literature.". J Bone Miner Res 27 (9): 1967-75. doi:10.1002/jbmr.1642. PMID 22532501.
  5. ↑ Ledford, CK.; Zelenski, NA.; Cardona, DM.; Brigman, BE.; Eward, WC. (Jul 2013). "The Phosphaturic Mesenchymal Tumor: Why is Definitive Diagnosis and Curative Surgery Often Delayed?". Clin Orthop Relat Res. doi:10.1007/s11999-013-3178-1. PMID 23868423.
  6. ↑ Hu FK, Yuan F, Jiang CY, et al. (November 2011). "Tumor-induced osteomalacia with elevated fibroblast growth factor 23: a case of phosphaturic mesenchymal tumor mixed with connective tissue variants and review of the literature". Chin J Cancer 30 (11): 794–804. doi:10.5732/cjc.011.10013. PMC 4013303. PMID 22035861. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4013303/.
Retrieved from "https://librepathology.org/w/index.php?title=Phosphaturic_mesenchymal_tumour,_mixed_connective_tissue_type&oldid=31959"