Difference between revisions of "Phosphaturic mesenchymal tumour, mixed connective tissue type"
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*Soft tissue ''or'' [[bone]].<ref name=pmid22532501>{{Cite journal | last1 = Jiang | first1 = Y. | last2 = Xia | first2 = WB. | last3 = Xing | first3 = XP. | last4 = Silva | first4 = BC. | last5 = Li | first5 = M. | last6 = Wang | first6 = O. | last7 = Zhang | first7 = HB. | last8 = Li | first8 = F. | last9 = Jing | first9 = HL. | title = Tumor-induced osteomalacia: an important cause of adult-onset hypophosphatemic osteomalacia in China: Report of 39 cases and review of the literature. | journal = J Bone Miner Res | volume = 27 | issue = 9 | pages = 1967-75 | month = Sep | year = 2012 | doi = 10.1002/jbmr.1642 | PMID = 22532501 }}</ref> | *Soft tissue ''or'' [[bone]].<ref name=pmid22532501>{{Cite journal | last1 = Jiang | first1 = Y. | last2 = Xia | first2 = WB. | last3 = Xing | first3 = XP. | last4 = Silva | first4 = BC. | last5 = Li | first5 = M. | last6 = Wang | first6 = O. | last7 = Zhang | first7 = HB. | last8 = Li | first8 = F. | last9 = Jing | first9 = HL. | title = Tumor-induced osteomalacia: an important cause of adult-onset hypophosphatemic osteomalacia in China: Report of 39 cases and review of the literature. | journal = J Bone Miner Res | volume = 27 | issue = 9 | pages = 1967-75 | month = Sep | year = 2012 | doi = 10.1002/jbmr.1642 | PMID = 22532501 }}</ref> | ||
*Often small - thus hard to identify.<ref name=pmid23868423>{{Cite journal | last1 = Ledford | first1 = CK. | last2 = Zelenski | first2 = NA. | last3 = Cardona | first3 = DM. | last4 = Brigman | first4 = BE. | last5 = Eward | first5 = WC. | title = The Phosphaturic Mesenchymal Tumor: Why is Definitive Diagnosis and Curative Surgery Often Delayed? | journal = Clin Orthop Relat Res | volume = | issue = | pages = | month = Jul | year = 2013 | doi = 10.1007/s11999-013-3178-1 | PMID = 23868423 }}</ref> | *Often small - thus hard to identify.<ref name=pmid23868423>{{Cite journal | last1 = Ledford | first1 = CK. | last2 = Zelenski | first2 = NA. | last3 = Cardona | first3 = DM. | last4 = Brigman | first4 = BE. | last5 = Eward | first5 = WC. | title = The Phosphaturic Mesenchymal Tumor: Why is Definitive Diagnosis and Curative Surgery Often Delayed? | journal = Clin Orthop Relat Res | volume = | issue = | pages = | month = Jul | year = 2013 | doi = 10.1007/s11999-013-3178-1 | PMID = 23868423 }}</ref> | ||
Clinical DDx: | |||
*Autosomal dominant hypophosphatemic rickets.<ref name=pmid19828339/> | |||
==Microscopic== | ==Microscopic== | ||
| Line 46: | Line 49: | ||
Image: | Image: | ||
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2783479/figure/F4/ PMTMCT (nih.gov)].<ref name=pmid19828339>{{Cite journal | last1 = Woo | first1 = VL. | last2 = Landesberg | first2 = R. | last3 = Imel | first3 = EA. | last4 = Singer | first4 = SR. | last5 = Folpe | first5 = AL. | last6 = Econs | first6 = MJ. | last7 = Kim | first7 = T. | last8 = Harik | first8 = LR. | last9 = Jacobs | first9 = TP. | title = Phosphaturic mesenchymal tumor, mixed connective tissue variant, of the mandible: report of a case and review of the literature. | journal = Oral Surg Oral Med Oral Pathol Oral Radiol Endod | volume = 108 | issue = 6 | pages = 925-32 | month = Dec | year = 2009 | doi = 10.1016/j.tripleo.2009.07.005 | PMID = 19828339 }}</ref> | *[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2783479/figure/F4/ PMTMCT (nih.gov)].<ref name=pmid19828339>{{Cite journal | last1 = Woo | first1 = VL. | last2 = Landesberg | first2 = R. | last3 = Imel | first3 = EA. | last4 = Singer | first4 = SR. | last5 = Folpe | first5 = AL. | last6 = Econs | first6 = MJ. | last7 = Kim | first7 = T. | last8 = Harik | first8 = LR. | last9 = Jacobs | first9 = TP. | title = Phosphaturic mesenchymal tumor, mixed connective tissue variant, of the mandible: report of a case and review of the literature. | journal = Oral Surg Oral Med Oral Pathol Oral Radiol Endod | volume = 108 | issue = 6 | pages = 925-32 | month = Dec | year = 2009 | doi = 10.1016/j.tripleo.2009.07.005 | PMID = 19828339 | PMC = 2783479 }}</ref> | ||
==IHC== | ==IHC== | ||
*Vimentin +ve.<ref name=pmid19828339 | *Vimentin +ve.<ref name=pmid19828339/> | ||
==References== | ==References== | ||
Revision as of 11:48, 19 August 2013
| Phosphaturic mesenchymal tumour, mixed connective tissue type | |
|---|---|
| Diagnosis in short | |
|
| |
| LM | spindle cells without atypia |
| IHC | vimentin +ve |
| Site | soft tissue, bone |
|
| |
| Blood work | low serum phosphate |
Phosphaturic mesenchymal tumour, mixed connective tissue type, abbreviated PMTMCT, is a rare tumour.
General
- Extremely rare.
- Hypophosphatemia.[1]
- Most common cause of oncogenic osteomalacia (tumour-induced osteomalacia).[2]
Gross
Clinical DDx:
- Autosomal dominant hypophosphatemic rickets.[5]
Microscopic
- Spindle cells without atypia in an eosinophilic matrix.
- Osteoclast-type giant cells.
- +/-Calcifications.
Image:
IHC
- Vimentin +ve.[5]
References
- ↑ 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.
- ↑ 2.0 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.
- ↑ 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.
- ↑ 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.
- ↑ 5.0 5.1 5.2 5.3 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/.