Difference between revisions of "Phosphaturic mesenchymal tumour, mixed connective tissue type"
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{{ Infobox diagnosis | {{ Infobox diagnosis | ||
| Name = {{PAGENAME}} | | Name = {{PAGENAME}} | ||
| Image = | | Image = Phosphaturic mesenchymal tumour -- high mag.jpg | ||
| Width = | | Width = | ||
| Caption = | | Caption = Phosphaturic mesenchymal tumour, mixed connective tissue type. [[H&E stain]]. | ||
| Micro = | | Micro = spindle cells without atypia | ||
| Subtypes = | | Subtypes = | ||
| LMDDx = | | LMDDx = [[osteosarcoma]], [[neurofibroma]], other [[soft tissue lesions]] | ||
| Stains = | | Stains = | ||
| IHC = | | IHC = vimentin +ve, pankeratin -ve, S-100 -ve | ||
| EM = | | EM = | ||
| Molecular = | | Molecular = | ||
| Line 20: | Line 20: | ||
| Signs = | | Signs = | ||
| Symptoms = | | Symptoms = | ||
| Prevalence = | | Prevalence = very rare | ||
| Bloodwork = low serum phosphate | | Bloodwork = low serum phosphate | ||
| Rads = | | Rads = | ||
| Line 26: | Line 26: | ||
| Prognosis = | | Prognosis = | ||
| Other = | | Other = | ||
| ClinDDx = | | ClinDDx = autosomal dominant hypophosphatemic rickets | ||
}} | }} | ||
'''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. | ||
| Line 32: | Line 32: | ||
==General== | ==General== | ||
*Extremely rare. | *Extremely rare. | ||
*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 }}</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== | ||
| Line 40: | Line 43: | ||
==Microscopic== | ==Microscopic== | ||
Features:<ref name=pmid22927293/> | Features:<ref name=pmid22927293/><ref name=pmid19828339/> | ||
*Spindle cells without atypia | *Spindle cells without atypia and moderate lightly eosinophilic cytoplasm without distinctive cell borders. | ||
*Eosinophilic matrix - described as "gungy" (shabby).<ref name=pmid14707860/> | |||
*[[HPC-like vessels]]. | |||
*Osteoclast-type [[giant cell]]s. | *Osteoclast-type [[giant cell]]s. | ||
*+/-Hemorrhage. | |||
*+/-Calcifications. | *+/-Calcifications. | ||
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> | DDx:<ref name=pmid14707860/> | ||
*[[Osteosarcoma]] - has cellular atypia. | |||
*[[Hemangiopericytoma]]. | |||
*[[Giant cell tumour of bone]]. | |||
*[[Neurofibroma]]. | |||
*Other [[soft tissue lesions]]. | |||
===Images=== | |||
<gallery> | |||
Image: Phosphaturic mesenchymal tumour -- intermed mag.jpg | PMTMCT - intermed. mag. (WC) | |||
Image: Phosphaturic mesenchymal tumour -- high mag.jpg | PMTMCT - high mag. (WC) | |||
Image: Phosphaturic mesenchymal tumour - alt -- high mag.jpg | PMTMCT - high mag. (WC) | |||
Image: Phosphaturic mesenchymal tumour -- very high mag.jpg | PMTMCT - very high mag. (WC) | |||
Image: Phosphaturic mesenchymal tumour - alt -- very high mag.jpg | PMTMCT - very high mag. (WC) | |||
</gallery> | |||
www: | |||
*[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> | |||
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4013303/figure/cjc-30-11-794-g003/ PMTMCT (nih.gov)].<ref name=pmid22035861/> | |||
==IHC== | |||
Features:<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> | |||
*FGF-23 +ve -- key immunostain (17 +ve/21 cases).<ref name=pmid14707860>{{cite journal |author=Folpe AL, Fanburg-Smith JC, Billings SD, ''et al.'' |title=Most osteomalacia-associated mesenchymal tumors are a single histopathologic entity: an analysis of 32 cases and a comprehensive review of the literature |journal=Am. J. Surg. Pathol. |volume=28 |issue=1 |pages=1–30 |year=2004 |month=January |pmid=14707860 |doi= |url=}}</ref> | |||
*Vimentin +ve. | |||
*Pankeratin -ve. | |||
*S-100 -ve. | |||
*CD34 -ve. | |||
Others:<ref name=pmid22035861>{{cite journal |author=Hu FK, Yuan F, Jiang CY, ''et al.'' |title=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 |journal=Chin J Cancer |volume=30 |issue=11 |pages=794–804 |year=2011 |month=November |pmid=22035861 |pmc=4013303 |doi=10.5732/cjc.011.10013 |url=}}</ref> | |||
*CD68 +ve.(???) | |||
*Desmin -ve. | |||
*Actin -ve/+ve (3 +ve/21 cases).<ref name=pmid14707860/> | |||
==Sign out== | |||
<pre> | |||
LEFT CALF, BIOPSY: | |||
- SPINDLE CELL LESION WITHOUT SIGNIFICANT NUCLEAR ATYPIA AND SHABBY HYALINE | |||
MATERIAL WITH CALCIFICATIONS, SEE COMMENT. | |||
COMMENT: | |||
The tumour stains as follows: | |||
POSITIVE: vimentin, CD68 (focal). | |||
NEGATIVE: pankeratin, S-100, CD34. | |||
PROLIFERATION (Ki-67): 3% of lesional cells. | |||
This probably represents a recurrence of the "phosphaturic mesenchymal tumour, mixed | |||
connective tissue type". | |||
The case was compared to the previous pathology. Clinical correlation is recommended. | |||
</pre> | |||
==References== | ==References== | ||
Latest revision as of 23:13, 2 October 2015
| Phosphaturic mesenchymal tumour, mixed connective tissue type | |
|---|---|
| Diagnosis in short | |
 Phosphaturic mesenchymal tumour, mixed connective tissue type. H&E stain. | |
|
| |
| LM | spindle cells without atypia |
| LM DDx | osteosarcoma, neurofibroma, other soft tissue lesions |
| IHC | vimentin +ve, pankeratin -ve, S-100 -ve |
| Site | soft tissue, bone |
|
| |
| Prevalence | very rare |
| 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
Microscopic
- Spindle cells without atypia and moderate lightly eosinophilic cytoplasm without distinctive cell borders.
- Eosinophilic matrix - described as "gungy" (shabby).[6]
- HPC-like vessels.
- Osteoclast-type giant cells.
- +/-Hemorrhage.
- +/-Calcifications.
DDx:[6]
- Osteosarcoma - has cellular atypia.
- Hemangiopericytoma.
- Giant cell tumour of bone.
- Neurofibroma.
- Other soft tissue lesions.
Images
PMTMCT - intermed. mag. (WC)
PMTMCT - high mag. (WC)
PMTMCT - high mag. (WC)
PMTMCT - very high mag. (WC)
PMTMCT - very high mag. (WC)
www:
IHC
Features:[3]
- FGF-23 +ve -- key immunostain (17 +ve/21 cases).[6]
- Vimentin +ve.
- Pankeratin -ve.
- S-100 -ve.
- CD34 -ve.
Others:[7]
- CD68 +ve.(???)
- Desmin -ve.
- Actin -ve/+ve (3 +ve/21 cases).[6]
Sign out
LEFT CALF, BIOPSY: - SPINDLE CELL LESION WITHOUT SIGNIFICANT NUCLEAR ATYPIA AND SHABBY HYALINE MATERIAL WITH CALCIFICATIONS, SEE COMMENT. COMMENT: The tumour stains as follows: POSITIVE: vimentin, CD68 (focal). NEGATIVE: pankeratin, S-100, CD34. PROLIFERATION (Ki-67): 3% of lesional cells. This probably represents a recurrence of the "phosphaturic mesenchymal tumour, mixed connective tissue type". The case was compared to the previous pathology. Clinical correlation is recommended.
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.
- ↑ 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/.
- ↑ 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.
- ↑ 6.0 6.1 6.2 6.3 Folpe AL, Fanburg-Smith JC, Billings SD, et al. (January 2004). "Most osteomalacia-associated mesenchymal tumors are a single histopathologic entity: an analysis of 32 cases and a comprehensive review of the literature". Am. J. Surg. Pathol. 28 (1): 1–30. PMID 14707860.
- ↑ 7.0 7.1 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/.