Difference between revisions of "Phosphaturic mesenchymal tumour, mixed connective tissue type"
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| Micro = spindle cells without atypia | | Micro = spindle cells without atypia | ||
| Subtypes = | | Subtypes = | ||
| LMDDx = | | LMDDx = [[osteosarcoma]], [[neurofibroma]], other [[soft tissue lesions]] | ||
| Stains = | | Stains = | ||
| IHC = vimentin +ve, pankeratin -ve, S-100 -ve | | IHC = vimentin +ve, pankeratin -ve, S-100 -ve | ||
| Line 20: | Line 20: | ||
| Signs = | | Signs = | ||
| Symptoms = | | Symptoms = | ||
| Prevalence = | | Prevalence = very rare | ||
| Bloodwork = low serum phosphate | | Bloodwork = low serum phosphate | ||
| Rads = | | Rads = | ||
| Line 47: | Line 47: | ||
*Osteoclast-type [[giant cell]]s. | *Osteoclast-type [[giant cell]]s. | ||
*+/-Calcifications. | *+/-Calcifications. | ||
DDx: | |||
*[[Osteosarcoma]]. | |||
*[[Neurofibroma]]. | |||
*Other [[soft tissue lesions]]. | |||
===Images=== | ===Images=== | ||
Revision as of 16:12, 12 June 2014
| Phosphaturic mesenchymal tumour, mixed connective tissue type | |
|---|---|
| Diagnosis in short | |
|
| |
| 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 in an eosinophilic matrix.
- Osteoclast-type giant cells.
- +/-Calcifications.
DDx:
- Osteosarcoma.
- Neurofibroma.
- Other soft tissue lesions.
Images
IHC
Features:[3]
- Vimentin +ve.
- Pankeratin -ve.
- S-100 -ve.
- CD34 -ve.
Others:[6]
- CD68 +ve.(???)
- Desmin -ve.
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 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/.