ObjectiveAneurysmal bone cysts (ABC) rarely present in soft tissue locations (STABC). The 30 cases of STABC reported in the English literature were reviewed. Six new cases retrieved from the files... Show moreObjectiveAneurysmal bone cysts (ABC) rarely present in soft tissue locations (STABC). The 30 cases of STABC reported in the English literature were reviewed. Six new cases retrieved from the files of the Netherlands Committee on Bone Tumors were compared to the six cases described in the radiological literature.Materials and methodsImaging studies and histopathology of six new STABC cases were reviewed. Follow-up was recorded with respect to local recurrence. FISH for USP6 rearrangement and/or anchored multiplex PCR-based targeted NGS using Archer FusionPlex Sarcoma Panel were attempted.ResultsOn imaging, the six STABC cases presented as a solid or multicystic intramuscular soft tissue mass, usually with thin peripheral mineralized bone shell. On MRI, perilesional edema was visualized in nearly all cases. Fluid-fluid levels were observed in one case. All lesions had the distinct histologic features of STABC. In three cases suitable for NGS, the diagnosis of STABC was confirmed by a COL1A1-USP6 fusion gene. In one additional case, USP6 gene rearrangement was detected by FISH. After marginal excision, none of the six STABC recurred after a mean follow-up period of 50months (range, 39-187months).ConclusionsOn imaging, it can be difficult to discriminate between STABC and myositis ossificans. The presence of a thin bony shell and fluid-fluid levels can be helpful in discriminating these two entities. STABC is readily diagnosed after histopathologic examination of the resection specimen. STABC belongs to the spectrum of tumors with USP6 rearrangements, which includes ABC, myositis ossificans, and nodular fasciitis. Show less
Younger, E.; Litiere, S.; Cesne, A. le; Mir, O.; Gelderblom, H.; Italiano, A.; ... ; Graaf, W.T.A. van der 2018
Primary vascular tumors of bone are a heterogeneous group. Numerous terms have been introduced as well as different classification systems. However, so far none of them have been generally accepted... Show morePrimary vascular tumors of bone are a heterogeneous group. Numerous terms have been introduced as well as different classification systems. However, so far none of them have been generally accepted.Therefor, there is a need for more specific morphological, immunohistochemical and molecular tools to support the classification of different vascular tumors of bone. In this thesis, we attempt to delineate the high-grade malignant vascular tumors of bone based on histomorphological criteria, protein expression profile based on a large panel of oncogenes, tumor-suppressor genes and signaling molecules, and molecular data. Moreover, we compared our dataset with a small groups of angiosarcoma of soft tissue in order to see whether these are truly different tumors or whether they should be regarded as one entity with a different localization. Finally, we question whether the rare vascular tumor previously designated as "haemangiopericytoma of bone" is a true entity or should rather be regarded as a growth pattern. Show less
