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Abdominal Radiology (New York) Jun 2024
Topics: Humans; Fluorodeoxyglucose F18; Positron Emission Tomography Computed Tomography; Radiopharmaceuticals; Prognosis; Fibromatosis, Aggressive; Predictive Value of Tests; Watchful Waiting; Fibromatosis, Abdominal
PubMed: 38748093
DOI: 10.1007/s00261-024-04262-3 -
Abdominal Radiology (New York) Jun 2024
Reply to letter to the editor re: D.N. Liu, C.P. Li, H.W. Li, et al. volume-based 18F-FDG PET/CT predicts prognosis and outcome of active surveillance for intra-abdominal desmoid tumor, abdominal radiology, 2024.
Topics: Humans; Fluorodeoxyglucose F18; Positron Emission Tomography Computed Tomography; Radiopharmaceuticals; Prognosis; Watchful Waiting; Fibromatosis, Aggressive
PubMed: 38748091
DOI: 10.1007/s00261-024-04354-0 -
Clinical Nuclear Medicine Jul 2024Prostate-specific membrane antigen (PSMA) PET/CT is widely used in the evaluation of suspected metastasis for initial definitive therapy and suspected recurrence of...
Prostate-specific membrane antigen (PSMA) PET/CT is widely used in the evaluation of suspected metastasis for initial definitive therapy and suspected recurrence of prostate cancer. We outline a case report of a 62-year-old man with history of prostate cancer treated with surgery, salvage radiation, and hormonal therapy presenting with rising PSA levels. There was incidental detection of a PSMA-avid subcutaneous abdominal wall mass on PSMA PET/CT study, which was consistent with desmoid fibromatosis on an ultrasound-guided biopsy.
Topics: Humans; Male; Positron Emission Tomography Computed Tomography; Middle Aged; Glutamate Carboxypeptidase II; Abdominal Wall; Antigens, Surface; Fibromatosis, Aggressive; Abdominal Neoplasms; Tomography, X-Ray Computed
PubMed: 38739529
DOI: 10.1097/RLU.0000000000005269 -
Current Opinion in Oncology Jul 2024This article discusses the evolving approaches to desmoid tumors management, shedding light on recent developments. (Review)
Review
PURPOSE OF REVIEW
This article discusses the evolving approaches to desmoid tumors management, shedding light on recent developments.
RECENT FINDINGS
Active surveillance has become the primary approach for managing primary peripheral desmoid tumors. This strategy was initially based on evidence from retrospective studies. Roughly 50% of cases managed with active surveillance show spontaneous stabilization or regression. Recent prospective trials conducted in Italy, The Netherlands, and France (2022-2023) confirm the efficacy of active surveillance, revealing 3-year progression-free survival rates ranging from 53.4 to 58%. For the patients under active surveillance, decisions regarding treatment are based on significant tumor growth or progressive symptoms. Moreover, three contemporary randomized trials investigated medical treatments for progressive or recurrent desmoid tumors. Sorafenib, pazopanib, and nirogacestat demonstrated clinical activity, as evidenced by favorable progression-free survival and objective response rates.
SUMMARY
Active surveillance has solidified its position as the primary management approach for desmoid tumors, validated by three robust prospective studies. Three recent randomized trials explored medical treatment for progressive or recurrent desmoid tumors, revealing promising clinical activities.
Topics: Humans; Fibromatosis, Aggressive; Watchful Waiting; Randomized Controlled Trials as Topic; Sorafenib; Indazoles; Pyrimidines; Sulfonamides
PubMed: 38726846
DOI: 10.1097/CCO.0000000000001049 -
JCO Global Oncology May 2024Desmoid fibromatosis (DF) is a locally aggressive tumor with low mortality but significant morbidity. There is a lack of standard of care, and existing therapies are...
PURPOSE
Desmoid fibromatosis (DF) is a locally aggressive tumor with low mortality but significant morbidity. There is a lack of standard of care, and existing therapies are associated with significant barriers including access, cost, and toxicities. This study aimed to explore the efficacy and safety of the metronomic therapy (MT) in DF in a large, homogenous cohort from India.
PATIENTS AND METHODS
This study involved histologically confirmed DF cases treated with MT comprising vinblastine (6 mg) and methotrexate (15 mg) both once a week, and tamoxifen (40 mg/m) in two divided doses once daily between 2002 and 2018.
RESULTS
There were 315 patients with a median age of 27 years; the commonest site was extremity (142 of 315; 45.0%). There were 159 (50.1%) male patients. Of the 123 (39.0%) prior treated patients, 119 had surgery. Of 315 patients, 263 (83.5%) received treatment at our institute (MT-151, 77-local treatment, 9-tyrosine kinase inhibitor, and 26 were observed). Among the MT cohort (n = 163, 61.2%), at a median follow-up of 36 (0.5-186) months, the 3-year progression-free and overall survival were 81.1% (95% CI, 74.3 to 88.4) and 99.2% (95% CI, 97.6 to 100), respectively. There were 35% partial responses. Ninety-two patients (56.4%) completed 1-year therapy, which was an independent prognosticator ( < .0001; hazard ratio, 0.177 [95% CI, 0.083 to 0.377]). MT was well tolerated. Predominant grade ≥3 toxicities were febrile neutropenia, 12 (7.4%) without any chemotoxicity-related death. The annual cost of MT was $130 US dollars.
CONCLUSION
The novel, low-cost MT qualifies as one of the effective, less toxic, sustainable, standard-of-care options for the treatment of DF with global reach and merits wide recognition.
Topics: Humans; Male; Female; Adult; Fibromatosis, Aggressive; India; Tertiary Care Centers; Young Adult; Middle Aged; Administration, Metronomic; Adolescent; Methotrexate; Standard of Care; Child; Vinblastine; Aged; Antineoplastic Combined Chemotherapy Protocols; Tamoxifen; Retrospective Studies
PubMed: 38723218
DOI: 10.1200/GO.23.00308 -
JCO Global Oncology May 2024@Thalcin explains how a metronomic regimen for desmoid tumors fits in the current treatment landscape.
@Thalcin explains how a metronomic regimen for desmoid tumors fits in the current treatment landscape.
Topics: Humans; Fibromatosis, Aggressive; Administration, Metronomic
PubMed: 38723215
DOI: 10.1200/GO.23.00488 -
Kyobu Geka. the Japanese Journal of... May 2024A man in his 50s who presented an abnormal shadow on chest X-ray was diagnosed with posterior mediastinal tumor that had grown compared to the previous chest X-ray....
A man in his 50s who presented an abnormal shadow on chest X-ray was diagnosed with posterior mediastinal tumor that had grown compared to the previous chest X-ray. Computed tomography showed a 5.7×3.9 cm solid mass with a smooth surface in the posterior mediastinum. A neurogenic tumor was suspected, and the mediastinal tumor was resected through thoracotomy because it was strongly adherent. The postoperative course was good, and he was discharged from the hospital on postoperative day 3. Contrary to preoperative expectations, the tumor was pathologically diagnosed as a desmoid tumor. After 6 months postoperatively without any complications, no recurrence was observed.
Topics: Humans; Male; Mediastinal Neoplasms; Fibromatosis, Aggressive; Middle Aged; Thoracotomy; Tomography, X-Ray Computed
PubMed: 38720608
DOI: No ID Found -
Cirugia Espanola May 2024
PubMed: 38704147
DOI: 10.1016/j.cireng.2024.03.011 -
Cancer May 2024Nirogacestat is a targeted gamma secretase inhibitor approved in the United States for adults with progressing desmoid tumors. In the phase 3 DeFi study (NCT03785964) of...
INTRODUCTION
Nirogacestat is a targeted gamma secretase inhibitor approved in the United States for adults with progressing desmoid tumors. In the phase 3 DeFi study (NCT03785964) of nirogacestat, ovarian toxicity (OT) was identified as a safety signal among females of reproductive potential (FORP). This analysis further describes the incidence, presentation, and resolution of OT.
METHODS
Patients were randomized to twice-daily oral nirogacestat (150 mg) or placebo, taken in continuous 28-day cycles. Investigator-identified OT in FORP was based on abnormal reproductive hormone values or perimenopausal symptoms (or both). Adverse event follow-up was conducted to assess OT resolution. Post hoc analyses included return of menstruation and return of follicle-stimulating hormone (FSH) to within normal limits (WNL) (≤20.4 mIU/mL).
RESULTS
Of 92 randomized females, 73 in the safety population were FORP (n = 36 nirogacestat, n = 37 placebo). OT was identified in 75% (27 of 36) receiving nirogacestat and 0% (0 of 37) receiving placebo. As of October 24, 2022, investigators reported OT resolution in 78% (21 of 27) of patients, with median OT duration of 19.1 weeks. Off-treatment resolution was reported in all 11 patients (100%) who stopped nirogacestat treatment; of these, all nine with available menstruation information experienced return of menstruation and eight had FSH WNL at last reported assessment. Resolution was reported in 10 of 14 (71%) while on nirogacestat; of these, all 10 experienced return of menstruation and seven had FSH WNL. Two patients were lost to follow-up.
CONCLUSION
Most FORP treated with nirogacestat experienced OT, with the majority resolving, including all who stopped treatment, suggesting that OT is transient.
PubMed: 38703010
DOI: 10.1002/cncr.35324