Solitary fibrous tumor (hemangiopericytoma) metastases

Case contributed by Lukas Valkovic
Diagnosis almost certain

Presentation

Patient with a history of meningeal haemangioperictyoma 5 years ago.

Patient Data

Age: 40 years
Gender: Female

Initial CT showed a bony destruction of the right temporal bone, followed by a contrast-enhanced study with a depiction of tumor expansion in the temporal region. 

MRI shows a well-circumscribed lobular vividly enhancing tumor with multiple cystic regions arising from the dural surface of the petrosal part of the temporal bone. It is growing extraaxially with mass effect on the ventricular system and other midline structures. Small perifocal edema. SWI depicts rich vascularization. 

MRI shows multiple vividly enhancing lesions in the liver, lungs, muscles of right abdominal/thoracic wall and next to the right kidney. They have a variable T1/T2 signal, but they are mostly isointense to adjacent parenchyma. Similarly, after early enhancement, they remain mainly isointense in later postcontrast phases.

FDG-PET/CT shows an additional finding - multiple enhancing lesions in breast parenchyma bilaterally. However, none of them show increased glucose metabolism.

Case Discussion

Patient with sensations in the right ear during pregnancy, which worsened after childbirth (tingling of the face). Initial CT and MRI with the finding of a large tumor in the right temporal lobe with infiltration of the petrosal part of the temporal bone. Subsequently treated with preoperative embolization than with a partial surgical resection followed by radiotherapy 5 years ago.

The recent follow-up study shows multiple lesions in both breasts, muscles of the abdominal/thoracic wall, liver, lungs and probably kidney which show similar characteristics. They are not easily recognizable in native CT/MRI and show no increase in glucose metabolism on PET. Their vivid early enhancement, distribution and patients history points to hemangiopericytoma metastases. 

 

Note: Due to molecular/genetic similarities hemangiopericytomas are no longer recognized as distinct entities. Instead, they represent cellular higher-grade solitary fibrous tumors. In the 2016 revised 4th Edition of the WHO classification of CNS tumors, they were grouped together under one diagnosis (solitary fibrous tumor/hemangiopericytoma), and in the 2021 5th edition the term hemangiopericytoma was dropped entirely. 

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