We report the first case of primary intraosseous smooth muscle tumor of uncertain malignant potential (STUMP) which is analogous to borderline malignant uterine smooth muscle tumors so designated. The tumor presented in the femur of an otherwise healthy 30-year-old woman. Over a 3-year period, the patient underwent 11 biopsies or resections and 2 cytologic procedures. Multiple pathologists reviewed the histologic material including musculoskeletal pathologists but could not reach a definitive diagnosis. However, metastases eventually developed and were rapidly progressive and responsive to gemcitabine and docetaxel. Molecular characterization and ultrastructural analysis was consistent with smooth muscle origin, and amplification of unmutated chromosome 12p and 12q segments appears to be the major genomic driver of this tumor. Primary intraosseous STUMP is thought to be genetically related to leiomyosarcoma of bone, but likely representing an earlier stage of carcinogenesis. Wide excision and aggressive follow-up is warranted for this potentially life-threatening neoplasm.
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| http://dx.doi.org/10.4081/rt.2016.6507 | DOI Listing |
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Article Synopsis
- Uterine leiomyomas, although rare in adolescents, can present with symptoms like abnormal bleeding and pelvic pain, with smooth muscle tumors of unknown malignant potential (STUMP) being even rarer.
- In a hospital case study, three 19-year-old patients presented with significant symptoms, leading to the identification of varying sizes of uterine myomas; one was diagnosed as a STUMP tumor while the others were benign fibroids.
- Despite their rarity, it is important for healthcare providers to consider uterine myomas and STUMP tumors as potential diagnoses in young patients experiencing pelvic symptoms.
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