Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion · 2024
Clinical Outcomes of Pediatric Acute Lymphoblastic Leukemia in the Bajio Region of Mexico: A Retrospective Cohort Study.
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Martínez Villegas Octavio, Alatoma Medina Norma Eryca, Romero Vázquez Marcos Javier, Andrade Colmenero Juan Carlos, Tirado López Bertha Elizabeth, Toala Fernández Ana Isabel, Valdez Garibay Betzayda, Murillo Ortíz Blanca Olivia, Amador Medina Lauro Fabián
Аннотация
BACKGROUND: Fibromatosis is a rare, benign, yet locally aggressive monoclonal fibroblastic proliferation. While current management paradigms increasingly favour non-operative strategies to avoid surgical morbidity and high recurrence rates, the role of surgery remains debated.
OBJECTIVE: This study aimed to evaluate the clinical presentation, management strategies, and oncological outcomes of fibromatosis at a single tertiary care centre.
METHODS: We conducted a retrospective, observational study of patients with histologically confirmed fibromatosis treated between January 2006 and September 2021. Data on patient demographics, tumour characteristics, treatment modalities, and disease status at last follow-up were analysed.
RESULTS: Of 57 identified patients, 33 had complete records and were included. The cohort was predominantly female (81.82%) with a median age at diagnosis of 25 years. The most common site was the lower limb (mid-leg). The majority (93.94%) of tumours were deep to the fascia. Surgery was the primary treatment in 75.76% of patients, with wide, marginal, and intralesional resections performed in 72%, 24%, and 4% of cases, respectively. At a mean follow-up of 80 months, the progression-free rate for patients treated with primary surgery was 72% (18/25), compared to 87.5% (7/8) for those managed primarily with non-operative strategies (active surveillance or systemic therapy). Overall, 75.76% of patients were disease-free. Statistical analysis revealed no significant correlation between disease-free status and the type of surgical procedure (p = 0.45), surgical margin status (p = 0.72), or tumour location (p = 0.61).
CONCLUSION: In our cohort, both surgery and initial non-operative management resulted in favourable medium-term disease control. Although not statistically significant in this small cohort, a trend towards higher recurrence was observed with marginal (50%) compared to wide (22%) margins. Our data suggest that in selected patients, less radical resection may be a viable option to reduce morbidity, but wider margins may be preferable when feasible without functional compromise. These findings contribute to the ongoing reevaluation of management strategies for fibromatosis.
OBJECTIVE: This study aimed to evaluate the clinical presentation, management strategies, and oncological outcomes of fibromatosis at a single tertiary care centre.
METHODS: We conducted a retrospective, observational study of patients with histologically confirmed fibromatosis treated between January 2006 and September 2021. Data on patient demographics, tumour characteristics, treatment modalities, and disease status at last follow-up were analysed.
RESULTS: Of 57 identified patients, 33 had complete records and were included. The cohort was predominantly female (81.82%) with a median age at diagnosis of 25 years. The most common site was the lower limb (mid-leg). The majority (93.94%) of tumours were deep to the fascia. Surgery was the primary treatment in 75.76% of patients, with wide, marginal, and intralesional resections performed in 72%, 24%, and 4% of cases, respectively. At a mean follow-up of 80 months, the progression-free rate for patients treated with primary surgery was 72% (18/25), compared to 87.5% (7/8) for those managed primarily with non-operative strategies (active surveillance or systemic therapy). Overall, 75.76% of patients were disease-free. Statistical analysis revealed no significant correlation between disease-free status and the type of surgical procedure (p = 0.45), surgical margin status (p = 0.72), or tumour location (p = 0.61).
CONCLUSION: In our cohort, both surgery and initial non-operative management resulted in favourable medium-term disease control. Although not statistically significant in this small cohort, a trend towards higher recurrence was observed with marginal (50%) compared to wide (22%) margins. Our data suggest that in selected patients, less radical resection may be a viable option to reduce morbidity, but wider margins may be preferable when feasible without functional compromise. These findings contribute to the ongoing reevaluation of management strategies for fibromatosis.