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Squamous-cell carcinoma on long-lasting lymphoedema
Case report Squamous-cell carcinoma on long-lasting lymphoedema Miguel Echenique-Elizondo and José Elorza Lancet Oncol 2002; 3: 319
A 63-year-old white male living in a rural area, with previously untreated congenital lymphoedema, was admitted to our clinic (figure 1). A histological analysis of a biopsy from the lesion showed squamous-cell carcinoma cells. Metastases were also found in the ulcerated inguinal-lymphnodes. The patient underwent lymphoedema-reducing surgery, with wide excision of the ulcerated area and inguinal lymphadenectomy. Unfortunately, he died of a massive pulmonary embolism 4 days after surgery even though adequate pharmacological and physical measures were taken. Lymphoedema1 may be a primary or secondary occurrence following either disease or surgery. Primary lymphoedema is sometimes congenital (Milroy’s disease) but it may also occur at any time of life, particularly puberty. Secondary lymphoedema is the most common form however, and often the result of filariasis, which is particularly common in southeast Asia and Africa. In developed countries, postsurgical lymphoedema of the extremity is common, and complications of chronic limb-lymphoedema include recurrent cellulitis and lymphangiosarcoma. Although lymphangiosarcoma2 is a recognised complication of chronic lymphoedema, squamous-cell carcinoma has only been reported on seven previous occasions.3 Multiple epithelial tumours on a single limb have also been reported,4 as have other cancers such as primary cutaneous B-cell lymphoma.5 Lymphangiosarcoma is an aggressive, malignant, vascularised tumour which occurs after chronic lymphoedema. Because of improvements in breast-cancer surgery, chronic lymphoedema is now seen less often, and the number of patients diagnosed with lymphangiosarcoma has substantially reduced in recent years. The true incidence of lymphangiosarcoma after post-mastectomy lymphoedema remains controversial and may be under-diagnosed. A large review, conducted between 1954 and 1983, studied 7620 patients treated for breast carcinoma at the Institut Gustave Roussy (Paris, France).6 Of these patients, 6919 were followed for at least 1 year, and 11 were diagnosed with sarcomas thought to be induced by radiation therapy—2 were StewardTreves Syndrome and 9 were sarcomas arising within the irradiated field. The sites of these sarcomas were: parietal wall (1 case), second costal cartilage (1 case), infraclavicular region (1 case), supraclavicular region (2 cases), internal third of the clavicle (2 cases), axillary region (2 cases), and the internal side of the upper arm (Stewart-Treves syndrome; 2 cases). The cumulative incidence of sarcoma following irradiation was 0·2% (0·09–0·47) at 10 years. The standardised incidence ratio of sarcoma (observed number of cases divided by the expected number of cases) was 1·81 (95% CI 0·91–3·23). This was significantly higher than 1·0 (p=0.03; one-tailed exact
THE LANCET Oncology Vol 3 May 2002
http://oncology.thelancet.com
Figure 1. A patient suffering from congenital lymphoedema.
test). The mean annual excess per 100 000 person-years at risk during the same period was 9·92. This study suggests that patients treated by radiation for breast cancer have a higher risk of developing a sarcoma than the general population. Adequate management of epidermoid carcinoma arising on long-lasting lymphoedema is poorly defined because it is rarely reported. Treatment options include radical ablative surgery, radiation therapy, and chemotherapy. Early detection is essential and we now recommend radical ablative surgery for this type of cancer. References
1 Szuba A, Rockson SG. Lymphoedema: classification, diagnosis, and therapy. Vasc Med 1998; 3: 145–56. 2 Heitmann C, Ingianni G. Stewart-Treves syndrome: lymphangiosarcoma following mastectomy. Ann Plast Surg 2000; 44: 72–75. 3 Lister RK, Black MM, Calonje E, Burnand KG. Squamous-cell carcinoma arising in chronic lymphoedema. Br J Dermatol 1997; 136: 384–87. 4 Dandurand M, Bernard F, Barneon G, et al. Multiple spinocellular carcinomas on chronic lymphoedema: association with vitiligo. Ann Dermatol Venereol 1990; 117: 953–56. 5 Torres-Paoli D, Sanchez JL. Primary cutaneous B-cell lymphoma of the leg in a chronic lymphoedematous extremity. Am J Dermatopathol 2000; 22: 257–60. 6 Taghian A, de Vathaire F, Terrier P, et al. Long-term risk of sarcoma following radiation treatment for breast cancer. Int J Radiat Oncol Biol Phys 1991; 21: 361–67. ME-E is an Associate Professor of Surgery, and both authors are members of the Department of Surgery, Basque Country University, Spain. Correspondence: Dr Miguel Echenique-Elizondo, Basque Country University, School of Medicine, P Dr Begiristain, 105, 20010 San Sebastián, Spain. Tel: +34 943 01 73 19. Fax: +34 943 01 73 30. Email: [email protected]
319
For personal use. Only reproduce with permission from The Lancet Publishing Group.
A 63-year-old white male living in a rural area, with previously untreated congenital lymphoedema, was admitted to our clinic (figure 1). A histological analysis of a biopsy from the lesion showed squamous-cell carcinoma cells. Metastases were also found in the ulcerated inguinal-lymphnodes. The patient underwent lymphoedema-reducing surgery, with wide excision of the ulcerated area and inguinal lymphadenectomy. Unfortunately, he died of a massive pulmonary embolism 4 days after surgery even though adequate pharmacological and physical measures were taken. Lymphoedema1 may be a primary or secondary occurrence following either disease or surgery. Primary lymphoedema is sometimes congenital (Milroy’s disease) but it may also occur at any time of life, particularly puberty. Secondary lymphoedema is the most common form however, and often the result of filariasis, which is particularly common in southeast Asia and Africa. In developed countries, postsurgical lymphoedema of the extremity is common, and complications of chronic limb-lymphoedema include recurrent cellulitis and lymphangiosarcoma. Although lymphangiosarcoma2 is a recognised complication of chronic lymphoedema, squamous-cell carcinoma has only been reported on seven previous occasions.3 Multiple epithelial tumours on a single limb have also been reported,4 as have other cancers such as primary cutaneous B-cell lymphoma.5 Lymphangiosarcoma is an aggressive, malignant, vascularised tumour which occurs after chronic lymphoedema. Because of improvements in breast-cancer surgery, chronic lymphoedema is now seen less often, and the number of patients diagnosed with lymphangiosarcoma has substantially reduced in recent years. The true incidence of lymphangiosarcoma after post-mastectomy lymphoedema remains controversial and may be under-diagnosed. A large review, conducted between 1954 and 1983, studied 7620 patients treated for breast carcinoma at the Institut Gustave Roussy (Paris, France).6 Of these patients, 6919 were followed for at least 1 year, and 11 were diagnosed with sarcomas thought to be induced by radiation therapy—2 were StewardTreves Syndrome and 9 were sarcomas arising within the irradiated field. The sites of these sarcomas were: parietal wall (1 case), second costal cartilage (1 case), infraclavicular region (1 case), supraclavicular region (2 cases), internal third of the clavicle (2 cases), axillary region (2 cases), and the internal side of the upper arm (Stewart-Treves syndrome; 2 cases). The cumulative incidence of sarcoma following irradiation was 0·2% (0·09–0·47) at 10 years. The standardised incidence ratio of sarcoma (observed number of cases divided by the expected number of cases) was 1·81 (95% CI 0·91–3·23). This was significantly higher than 1·0 (p=0.03; one-tailed exact
THE LANCET Oncology Vol 3 May 2002
http://oncology.thelancet.com
Figure 1. A patient suffering from congenital lymphoedema.
test). The mean annual excess per 100 000 person-years at risk during the same period was 9·92. This study suggests that patients treated by radiation for breast cancer have a higher risk of developing a sarcoma than the general population. Adequate management of epidermoid carcinoma arising on long-lasting lymphoedema is poorly defined because it is rarely reported. Treatment options include radical ablative surgery, radiation therapy, and chemotherapy. Early detection is essential and we now recommend radical ablative surgery for this type of cancer. References
1 Szuba A, Rockson SG. Lymphoedema: classification, diagnosis, and therapy. Vasc Med 1998; 3: 145–56. 2 Heitmann C, Ingianni G. Stewart-Treves syndrome: lymphangiosarcoma following mastectomy. Ann Plast Surg 2000; 44: 72–75. 3 Lister RK, Black MM, Calonje E, Burnand KG. Squamous-cell carcinoma arising in chronic lymphoedema. Br J Dermatol 1997; 136: 384–87. 4 Dandurand M, Bernard F, Barneon G, et al. Multiple spinocellular carcinomas on chronic lymphoedema: association with vitiligo. Ann Dermatol Venereol 1990; 117: 953–56. 5 Torres-Paoli D, Sanchez JL. Primary cutaneous B-cell lymphoma of the leg in a chronic lymphoedematous extremity. Am J Dermatopathol 2000; 22: 257–60. 6 Taghian A, de Vathaire F, Terrier P, et al. Long-term risk of sarcoma following radiation treatment for breast cancer. Int J Radiat Oncol Biol Phys 1991; 21: 361–67. ME-E is an Associate Professor of Surgery, and both authors are members of the Department of Surgery, Basque Country University, Spain. Correspondence: Dr Miguel Echenique-Elizondo, Basque Country University, School of Medicine, P Dr Begiristain, 105, 20010 San Sebastián, Spain. Tel: +34 943 01 73 19. Fax: +34 943 01 73 30. Email: [email protected]
319
For personal use. Only reproduce with permission from The Lancet Publishing Group.