"Lymphangiosarcoma Management and Outcomes: A Retrospective Study from a Single Institution"

SDCC 

Introduction:
Lymphangiosarcoma (LAS) is an aggressive cutaneous angiosarcoma, often occurring in association with chronic lymphedema. While LAS most often diagnosed in the upper extremities following axillary lymph node dissection or local radiation therapy for breast carcinoma, it may also affect other organs. However, due to its rarity, neither pathogenesis nor a standard treatment strategy has been established. This retrospective analysis aims to contribute insights into the management and outcomes of lymphangiosarcoma through a comprehensive review of cases from a single institute.
Methods:
A retrospective medical record review of patients with a diagnosis of lymphangiosarcoma from 1976 to 2022 was done. Clinical details including demographics, history, risk factors and comorbidities, operative course, postoperative complications, outcomes, and recovery were recorded. Descriptive analysis was done on the data points.
Results:
37 patients were included in the final analysis. The mean age at the diagnosis of this patient population was 61.6 ±16.6 years, with a mean BMI of 29.7±8.6 kg/m2. 11 (29.7%) patients were male. Hypertension was noted in 21 out 32 cases, cardiovascular disease in 7 out of 33 cases, and chronic lymphedema in 29 out of 35 cases where data were available. LAS location included 15 cases (40.5%) in the upper extremities alone, 12 cases (32.4%) in the lower extremities alone, and 10 cases (27.0%) in other areas. Among these patients, 17 out of 37 (45.9%) had history of prior surgeries, with 8 out of 29 (27.6%) received prior chemo and 17 out of 33 (51.5%) underwent prior radiation where data was available. Surgical intervention was done in 31 out of 37 (83.8%) diagnosed cases, with a median time from diagnosis to surgery of 29 days (range: 2-1246). Additionally, chemotherapy and radiation therapy were administered in 19 cases (51.4%) and 9 cases (24.3%), respectively. The median survival time from date of diagnosis to either death or available last follow up is 28.4 months (range: 0.2-563.2), with recurrence of 24 (64.9%) out of 37 cases.
Conclusion:
In conclusion, this study highlights the diverse management approaches and outcomes of Lymphangiosarcoma (LAS) at our institute. With no standardized treatment protocol due to its rarity, surgical intervention emerged as a prominent therapeutic modality, with proportion of patients undergoing chemotherapy, and radiation. The median survival time highlights the challenges in managing this condition, with a notable recurrence rate.

Hand and Upper Extremity

PSTM 2024