Phosphaturic mesenchymal tumor and related wound problem

Abstract

Introduction: Phosphaturic mesenchymal tumor mixed connective tissue type (PMT/MCT) is the most common type (up to 90%) of phosphaturic mesenchymal tumor (PMT), a rare clinicopathologic entity. Besides overproduction of fibroblast growth factor 23 (FGF23), there is a big variation of immunohistochemical characteristic across types of PMT, which makes it difficult to obtain an early diagnosis of PMT/MCT. As a benign tumor, PMT/MCT usually happens in subcutaneous tissues and leads to nonhealing of wound. A complete excision of PMT/MCT facilitates wound healing.

Conclusions: Review of the existing evidence indicates that early diagnosis of PMT/MCT is critically important when treating PMT/MCT wound. Hence standardization of early diagnosis for PMT/MCT is mandated.

Figure 1

The mechanism of osteomalacia caused by FGF23 (arrows in square brackets denote abnormality seen occasionally, which means upward indication of an increase and downward indication of a decrease); NPT2a type II sodium-phosphate cotransporter.

Figure 2

Flowchart of the differential diagnosis and characteristics of hypophosphaturic osteomalacia (N normal; arrows in square brackets denote abnormality seen occasionally, which means upward indication of an increase and downward indication of a decrease); ADHR = autosomal dominant hypophosphatemic rickets, ARHR = autosomal recessive hypophosphatemic rickets, FH = family history, FGF23 = fibroblast growth factor 23, FD = fibrous dysplasia, HHRH = hereditary hypophosphatemic rickets with hypercalciuria, Mc Alb = McCune Albright syndrome, PTH = parathyroid hormone, Prim. HPT = primary hyperparathyroidism, RF = rheumatoid factor, Sec. HPT = secondary hyperparathyroidism, TRP = fractional tubular reabsorption of phosphate, TMP/GFR = tubular maximum reabsorption of phosphate to glomerular filtration rate, TIO = tumor-induced osteomalacia, XLH = X-linked hypophosphatemic rickets.