Cancer-related secondary lymphoedema due to cutaneous lymphangitis carcinomatosa: clinical presentations and review of literature

Abstract

Lymphoedema is a clinical condition caused by impairment of the lymphatic system, leading to swelling of subcutaneous soft tissues. As a result, accumulation of protein-rich interstitial fluid and lymphostasis often causes additional swelling, fibrosis and adipose tissue hypertrophy leading to progressive morbidity and loss of quality of life for the patient. Lymphoedema can be distinguished as primary or secondary. Lymphoedema is a complication frequently encountered in patients treated for cancer, especially after lymphadenoectomy and/or radiotherapy based on destruction of lymphatics. However, although lymphatic impairment is sometimes caused by obstructive solid metastasis, we present three cases of secondary lymphoedema with minor dermatological features without detectable solid metastasis. Sometimes this type of lymphoedema is mistakenly called malignant lymphoedema. All patients were previously treated for cancer without clinical signs of recurrence, presented with progressive lymphoedema and minor dermatological features of unknown origin. Clinical and histopathological examination of the skin revealed diffuse lymphangitis carcinomatosa, leading to secondary lymphoedema and adjustment of the therapeutic approach and prognosis. We reviewed literature on these rare presentations of cancer recurrence and recommend, where appropriate, consulting a dermatologist when discrete skin abnormalities are seen in patients with a history of cancer and developing lymphoedema.

Cutaneous lymphangitis carcinomatosa made cervicofacial oedema intractable in a patient with superior vena cava syndrome

“… 9 Lung cancer is the quickest to metastasise to the skin (15.75 months) among solid tumours. 3 The average survival time from the appearance of CLC in lung cancer (less than 3 months) is shorter than that from the appearance of cutaneous metastasis (3 to 5 months). 3 7 12 The only known treatment for CLC is systemic chemotherapy, but early diagnosis can lead to earlier anticancer treatment.…”

“…This subcutaneous lymphatic invasion results in lymphoedema and diffuse skin erythema mimicking an infectious process, which differs from the typical form of cutaneous metastases such as nodules or masses. 3–6 The incidences of cutaneous metastases in patients with metastatic cancer and with lung cancer are approximately 5% and 1.7%–3.1%, respectively; CLC accounts for approximately 5% of all cutaneous metastases. 2 7 The most common histological feature observed in patients with lung cancer who develop CLC is adenocarcinoma, as in the present case.…”

“… 1 Cutaneous lymphangitis carcinomatosa (CLC), a rare condition that accounts for less than 5% of cutaneous metastases, also causes cervicofacial oedema, 2 and patients with CLC usually present with progressive lymphoedema and erythema. 3 Here, we report a rare case of lung adenocarcinoma with CLC and SVC stenosis in a patient whose cervicofacial oedema and dyspnoea remained despite improving SVC blood flow via balloon dilation.…”

Therapeutic challenge: severe dyspnea in a patient with metastatic breast cancer and lymphangitis carcinomatosis syndrome

“…Lymphangitis carcinomatosis is a rare clinical state in which the malignant cells are infiltrating the lymphatics. The appearance of the syndrome has been reported in skin [8], duodenum [9] and lungs [4]. In the pulmonary manifestation the most predominant symptom is intense breathlessness that cannot be relieved by standard procedures [4].…”

Cutaneous metastases of lung cancer

“…Reported cases of cutaneous metastases of lung cancer were 1.78% with poor prognosis . Cutaneous metastases accompanied with lymphangitis carcinomatosa shows various erythemas with upper limb edema, similar to SVC syndrome which is a major cause of upper limb edema occurring in patients with lung cancer. Systemic chemotherapy is a standard therapy for cutaneous metastases, but is rarely performed due to poor performance status with limited life expectancy.…”