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Presented by Pedram Argani, M.D. and prepared by Mohammed Lilo, M.D.
Case 3: a 49 year old female with a thigh mass
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1. Question
Week 591: Case 3
This is a 49 year old female with a thigh massimages/lilo/Argani/Arg-3-A.jpg
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images/lilo/Argani/Arg-3-e.jpgCorrect
Answer: Phosphaturic mesenchymal tumor
Histology: This is a rare lesion with a characteristic morphology. The most typical feature is the grungy, basophilic calcification which is located centrally. This amorphous material is surrounded by bland ovoid spindle cells, which are associated with hemangiopericytomatous vasculature. The cells are lack significant cytologic atypia or mitotic activity.
Discussion: The hemangiopericytoma pattern is one seen in a variety of tumors, and true hemangiopericytoma are thought to lie within the spectrum of solitary fibrous tumors. Tumoral calcinosis would lack the mesenchymal spindle cell proliferation of the current lesion. Synovial sarcomas may be heavily calcified; however, the calcification is generally not of this extensive amorphous character, and the lesions are generally more cellular and mitotically active.
Phosphaturic mesenchymal tumor is a well-known cause of oncogenic osteomalacia. The lesional cells express fibroblastic growth factor 23, which prevents reabsorption of phosphate in the renal tubules and results in osteomalacia.
Incorrect
Answer: Phosphaturic mesenchymal tumor
Histology: This is a rare lesion with a characteristic morphology. The most typical feature is the grungy, basophilic calcification which is located centrally. This amorphous material is surrounded by bland ovoid spindle cells, which are associated with hemangiopericytomatous vasculature. The cells are lack significant cytologic atypia or mitotic activity.
Discussion: The hemangiopericytoma pattern is one seen in a variety of tumors, and true hemangiopericytoma are thought to lie within the spectrum of solitary fibrous tumors. Tumoral calcinosis would lack the mesenchymal spindle cell proliferation of the current lesion. Synovial sarcomas may be heavily calcified; however, the calcification is generally not of this extensive amorphous character, and the lesions are generally more cellular and mitotically active.
Phosphaturic mesenchymal tumor is a well-known cause of oncogenic osteomalacia. The lesional cells express fibroblastic growth factor 23, which prevents reabsorption of phosphate in the renal tubules and results in osteomalacia.
