Lymphedema as an initial manifestation of paraneoplastic rheumatic syndromes: a case series of six patients
- 1Department of Rheumatology and Clinical Immunology, Beijing Shijitan Hospital, Capital Medical University
- 2Department of Lymphoma, Beijing Shijitan Hospital, Capital Medical University
Abstract
Objective: Lymphedema as the initial symptom poses significant challenges in the early diagnosis of underlying malignancies masquerading as rheumatic diseases.
Methods: This study retrospectively analyzed six patients who were hospitalized at Beijing Shijitan Hospital between October 2015 and October 2025 with paraneoplastic rheumatic syndromes (PRS) presenting with lymphedema as the initial manifestation. Data on clinical manifestation, laboratory and imaging findings, diagnoses, treatments, and prognosis were collected.
Results: Six patients (3 males, 3 females; median age 66 years) presented with lymphedema as the initial manifestation of PRS. The initial rheumatologic diagnoses were leukocytoclastic vasculitis (n=2), undifferentiated connective tissue disease (n=2), Sjögren’s syndrome (n=1), and anti-synthetase syndrome (n=1). The median interval from lymphedema onset to rheumatic diagnosis was 5.5 months. All patients received glucocorticoids, and four received immunosuppressants; none achieved durable benefit. The median interval from rheumatic diagnosis to therapy failure was 1 month, and from therapy failure to cancer diagnosis was 1 month. The underlying malignancies included lymphoma (n=3), lung adenocarcinoma (n=2), and gastric adenocarcinoma (n=1). During a follow-up of 1 to 13 months, two patients showed significant improvement in lymphedema, whereas the remaining four died.
Conclusion: Malignancies may present with lymphedema as the initial symptom, mimicking rheumatic diseases. Underlying malignancy should be highly suspected, particularly when there is a poor response to immunosuppressive therapy. Early recognition and screening for malignancies are crucial for improving patient outcomes.
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BibTeX
@article{zhang2026lymphedema,
title = {{Lymphedema as an initial manifestation of paraneoplastic rheumatic syndromes: a case series of six patients}},
author = {Guo-Hua Zhang and Ling-Ling Zhang and Min-Na Jiang and Wei-Xia Wang and Kai-Li Zhong and Yu-Hua Wang},
journal = {Frontiers in Oncology},
year = {2026},
month = oct,
doi = {10.3389/fonc.2026.1964817},
url = {https://www.frontiersin.org/articles/10.3389/fonc.2026.1964817/full},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Lymphedema as an initial manifestation of paraneoplastic rheumatic syndromes: a case series of six patients
AU - Guo-Hua Zhang
AU - Ling-Ling Zhang
AU - Min-Na Jiang
AU - Wei-Xia Wang
AU - Kai-Li Zhong
AU - Yu-Hua Wang
PY - 2026
DA - 2026/10/26
JO - Frontiers in Oncology
DO - 10.3389/fonc.2026.1964817
UR - https://www.frontiersin.org/articles/10.3389/fonc.2026.1964817/full
AB - Objective: Lymphedema as the initial symptom poses significant challenges in the early diagnosis of underlying malignancies masquerading as rheumatic diseases. Methods: This study retrospectively analyzed six patients who were hospitalized at Beijing Shijitan Hospital between October 2015 and October 2025 with paraneoplastic rheumatic syndromes (PRS) presenting with lymphedema as the initial manifestation. Data on clinical manifestation, laboratory and imaging findings, diagnoses, treatments, and prognosis were collected. Results: Six patients (3 males, 3 females; median age 66 years) presented with lymphedema as the initial manifestation of PRS. The initial rheumatologic diagnoses were leukocytoclastic vasculitis (n=2), undifferentiated connective tissue disease (n=2), Sjögren’s syndrome (n=1), and anti-synthetase syndrome (n=1). The median interval from lymphedema onset to rheumatic diagnosis was 5.5 months. All patients received glucocorticoids, and four received immunosuppressants; none achieved durable benefit. The median interval from rheumatic diagnosis to therapy failure was 1 month, and from therapy failure to cancer diagnosis was 1 month. The underlying malignancies included lymphoma (n=3), lung adenocarcinoma (n=2), and gastric adenocarcinoma (n=1). During a follow-up of 1 to 13 months, two patients showed significant improvement in lymphedema, whereas the remaining four died. Conclusion: Malignancies may present with lymphedema as the initial symptom, mimicking rheumatic diseases. Underlying malignancy should be highly suspected, particularly when there is a poor response to immunosuppressive therapy. Early recognition and screening for malignancies are crucial for improving patient outcomes.
SN - 2234-943X
ER -