Phosphaturic mesenchymal tumour, mixed connective tissue type

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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

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 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]

Images

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

  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. //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. 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. 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. //www.ncbi.nlm.nih.gov/pmc/articles/PMC4013303/.