Phosphaturic mesenchymal tumor
The initial presenting symptoms and signs of PMT usually relate to hypophosphatemia and osteomalacia and include muscle weakness, bone pain, and pathologic fractures. These paraneoplastic manifestations may predate diagnosis of the underlying PMT by an average of 3 years, as reported in 1 study. Occasionally, a PMT may be detected prior to paraneoplastic manifestations, especially if in the soft tissue.
PMT most often presents in middle-aged adults, although cases throughout life have been reported. In the elderly, cases have been detected more frequently in women. PMTs have been reported in many soft tissue locations or bones; however, soft tissue tumors are most frequently reported on the extremities or acral areas, and bony tumors involve the extremities, shoulder and pelvic girdle, cranial bones, and the paranasal sinuses.
Tumors are typically solitary and occur sporadically, with no specific risk factors or exposures noted in existing literature. The vast majority are benign, but malignant cases with sarcomatous histopathology and metastases have rarely been reported. In 1 study, the elderly were noted to have a higher risk of metastasis.
D16.9 – Benign neoplasm of bone and articular cartilage, unspecified
D21.9 – Benign neoplasm of connective and other soft tissue, unspecified
703649004 – Phosphaturic mesenchymal tumor, benign
703650004 – Phosphaturic mesenchymal tumor, malignant
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- Dermatofibrosarcoma protuberans (DFSP)
- Giant cell tumor of bone and soft tissue
- Aneurysmal bone cysts of bone and soft tissue
- Benign and malignant cartilaginous tumors
- Benign osteogenic tumors
- Endocrine neoplasms
- Biphasic tumor of the jaw
- Epidermal nevus syndrome.
- Small cell carcinoma of the lung.
- Neurofibromatosis type 1, associated with diffuse neuromas as well as axillary freckling.
- McCune-Albright syndrome, associated with unilateral café au lait spots and peripheral precocious puberty.