Difference between revisions of "Phosphaturic mesenchymal tumour, mixed connective tissue type"

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| Prognosis  =
| Prognosis  =
| Other      =
| Other      =
| ClinDDx    =
| ClinDDx    = autosomal dominant hypophosphatemic rickets
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'''Phosphaturic mesenchymal tumour, mixed connective tissue type''', abbreviated '''PMTMCT''', is a rare tumour.  
'''Phosphaturic mesenchymal tumour, mixed connective tissue type''', abbreviated '''PMTMCT''', is a rare tumour.  
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*Hypophosphatemia.<ref name=pmid23722193>{{Cite journal  | last1 = Papierska | first1 = L. | last2 = Ćwikła | first2 = JB. | last3 = Misiorowski | first3 = W. | last4 = Rabijewski | first4 = M. | last5 = Sikora | first5 = K. | last6 = Wanyura | first6 = H. | title = Unusual case of phosphaturic mesenchymal tumor. | journal = Pol Arch Med Wewn | volume = 123 | issue = 5 | pages = 255-6 | month =  | year = 2013 | doi =  | PMID = 23722193 | URL = http://pamw.pl/sites/default/files/85_Papierska.pdf}}</ref>
*Hypophosphatemia.<ref name=pmid23722193>{{Cite journal  | last1 = Papierska | first1 = L. | last2 = Ćwikła | first2 = JB. | last3 = Misiorowski | first3 = W. | last4 = Rabijewski | first4 = M. | last5 = Sikora | first5 = K. | last6 = Wanyura | first6 = H. | title = Unusual case of phosphaturic mesenchymal tumor. | journal = Pol Arch Med Wewn | volume = 123 | issue = 5 | pages = 255-6 | month =  | year = 2013 | doi =  | PMID = 23722193 | URL = http://pamw.pl/sites/default/files/85_Papierska.pdf}}</ref>
*Most common cause of ''oncogenic osteomalacia'' (tumour-induced osteomalacia).<ref name=pmid22927293>{{Cite journal  | last1 = William | first1 = J. | last2 = Laskin | first2 = W. | last3 = Nayar | first3 = R. | last4 = De Frias | first4 = D. | title = Diagnosis of phosphaturic mesenchymal tumor (mixed connective tissue type) by cytopathology. | journal = Diagn Cytopathol | volume = 40 Suppl 2 | issue =  | pages = E109-13 | month = Aug | year = 2012 | doi = 10.1002/dc.21647 | PMID = 22927293 }}</ref>
*Most common cause of ''oncogenic osteomalacia'' (tumour-induced osteomalacia).<ref name=pmid22927293>{{Cite journal  | last1 = William | first1 = J. | last2 = Laskin | first2 = W. | last3 = Nayar | first3 = R. | last4 = De Frias | first4 = D. | title = Diagnosis of phosphaturic mesenchymal tumor (mixed connective tissue type) by cytopathology. | journal = Diagn Cytopathol | volume = 40 Suppl 2 | issue =  | pages = E109-13 | month = Aug | year = 2012 | doi = 10.1002/dc.21647 | PMID = 22927293 }}</ref>
Clinical DDx:
*Autosomal dominant hypophosphatemic rickets.<ref name=pmid19828339/>


==Gross==
==Gross==
*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==

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
Clin. DDx autosomal dominant hypophosphatemic rickets

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]

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

  • Vimentin +ve.[3]

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.
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