Forner-Cordero I, Herrero-Manley L, Garcia-Marcos R, Munoz-Langa J
Lymphedema Unit, Hospital Universitari i Politècnic La Fe, University of Valencia, Valencia, SPAIN.
Physical Medicine and Rehabilitation Service, Hospital Arnau de Vilanova, Valencia, SPAIN.
Lymphology. 2022;55(4):144-154.
Diagnosing malignant lymphedema is a challenge in daily clinical practice. Clinically, patients may show clear signs of malignancy, but this is not always the case, and at times the diagnosis is not straightforward. In some patients, pain, hardness of the tissues, joint stiffness, proximal involvement, collateral circulation, or an acute onset will provide the clue to determining malignancy. Our aim is to describe several diverse scenarios of Secondary Malignant Lymphedema (SML) with the etiopathogenesis. One possible cause is lymphatic obstruction due to extrinsic compression of lymphatic vessels and/or nodes by either the primary tumor or metastatic masses. Lymphatic obstruction can also be caused by tumoral infiltration. This infiltration can affect both deep and regional nodes as well as cutaneous and subcutaneous vessels and is commonly known as lymphangitis carcinomatosa. Malignant lymphedema can also be secondary to obstruction of the venous flow due to tumoral venous thromboembolism or to extrinsic compression of the veins by tumors or adenopathic masses. Nevertheless, the most frequent cause of this illness is a mixed mechanism of compression of the lymphatic and venous systems. Frequently, SML is the first manifestation of relapse. When lymphedema appears abruptly, is progressive, with intense pain, associated with collateral circulation, or with hard and infiltrated skin or joint stiffness, SML must be ruled out with an urgent referral to the oncologist and an imaging evaluation.
在日常临床实践中，诊断恶性淋巴水肿是一项挑战。临床上，患者可能会表现出明显的恶性迹象，但情况并非总是如此，有时诊断并不简单直接。在一些患者中，疼痛、组织硬度、关节僵硬、近端受累、侧支循环或急性发作将为确定恶性肿瘤提供线索。我们的目的是描述几种不同的继发性恶性淋巴水肿（SML）情况及其发病机制。一个可能的原因是原发性肿瘤或转移瘤对淋巴管和/或淋巴结的外在压迫导致淋巴阻塞。淋巴阻塞也可由肿瘤浸润引起。这种浸润可影响深部和区域淋巴结以及皮肤和皮下血管，这通常被称为癌性淋巴管炎。恶性淋巴水肿也可能继发于肿瘤性静脉血栓栓塞导致的静脉血流阻塞，或肿瘤或肿大淋巴结对静脉的外在压迫。然而，这种疾病最常见的原因是淋巴和静脉系统受压的混合机制。通常，SML是复发的首发表现。当淋巴水肿突然出现、呈进行性、伴有剧烈疼痛、伴有侧支循环、或伴有皮肤坚硬浸润或关节僵硬时，必须紧急转诊至肿瘤学家并进行影像学评估以排除SML。
