Treatment with a direct acting antiviral (DAA) has revolutionized HCV therapy, as more than 95% of patients achieve a sustained virological response (SVR). Cryoglobulinemic vasculitis (CryoVas), however, can persist and recur after the HCV cure. In this systematic review, we include data from 19 studies that provided information on the persistence and recurrence of CryoVas after the HCV cure with DAAs. A complete clinical response (CR) was reported in 63.7% to 90.2% of the DAA-treated patients after achieving SVR. Relapse of CryoVas symptoms was reported in 4% to 18% of the patients. Neuropathy, nephropathy, and dermatological complications were the most common manifestations of CryoVas. B-cell clones persisted in 31–40% of the patients and could contribute to CryoVas relapse. INFL3-rs12979860, ARNTL-rs648122, RETN-rs1423096, and SERPINE1-rs6976053 were associated with a higher incidence of persistence and recurrence of CryoVas. Prospective multicenter studies with diverse patient populations are needed to validate these findings for the timely and effective management of this challenging condition.
The thyroid gland is found in the neck and corresponds to the 2-4 tracheal cartilages. It is rarely found in other sites and is termed ectopic thyroid tissue (ETT) once found. In this report, we present a case of a 19-year-old female who was diagnosed with ETT in the lumbar region and posterior mediastinal region after total thyroidectomy for a large dominant thyroid nodule. ETT has a unique presentation and physicians should be vigilant to diagnose it correctly and promptly to decrease morbidity associated with the disease.
Dermatofibrosarcoma protuberans (DFSP) is a low- to intermediate-grade dermal soft tissue malignant tumor (sarcoma) with a high local recurrence rate but low metastatic potential. DFSP is characterized by uniform spindle cell fascicles arranged classically in a storiform pattern and by CD34 immunoreactivity. On gross examination, DFSP usually manifests as a white or yellow soft tissue mass with a smooth outer surface and poor circumscription. In this study, we report a case of DFSP with fibrosarcomatous transformation, a rare but well-known phenomenon encountered in DFSP that is correlated with an increased risk of adverse outcomes in patients with DFSP. A 45-year-old male presented with a progressively enlarging lump on his left shoulder, initially suspected of being a lipoma but diagnosed as a fibrosarcomatous transformation of DFSP. Surgical resection was performed, with the subsequent identification of metastatic sarcoma in pulmonary nodules. Robotic-assisted thoracoscopy excised the nodules, confirming metastatic sarcoma with aggressive behavior. Despite negative adjuvant treatment plans, the patient remains under surveillance with imaging, showing no recurrence in recent scans. Continued follow-up with medical and surgical oncology is planned. DFSP is a rare soft tissue sarcoma characterized by indolent growth and low metastatic potential, except in fibrosarcomatous transformation cases. Molecularly, DFSP is defined by a COL1A1-PDGFB fusion transcript that is targetable with imatinib therapy. Treatment involves wide surgical resection, with adjuvant radiation therapy in select cases. Radiation therapy may be employed in cases with close or positive margins, while conventional chemotherapy has limited utility. Multidisciplinary collaboration is crucial for optimal management. Overall, this case underscores the challenges in diagnosing and managing aggressive sarcomas like fibrosarcomatous DFSP, emphasizing the importance of vigilant surveillance and multidisciplinary collaboration in optimizing patient outcomes. Further research is needed to understand the mechanisms underlying fibrosarcomatous transformation and to explore novel therapeutic avenues for this challenging malignancy.