Treatment of Stewart-Treves syndrome

The Stewart-Treves syndrome is the most dangerous complication of chronic lymphedema following mastectomy. Prognosis is poor. In the world literature 278 cases are described. At the Surgical Clinic of the University of Heidelberg 3 cases of Stewart-Treves syndrome have been treated so far. Decisive for the prognosis of this fast growing tumor of high malignancy is early detection and initiation of therapy. The only curative therapy seems to be radical surgery in the form of interthoracic scapular exarticulation of the arm at an early stage of this disease. Radio- and chemotherapy offer palliation in advanced tumor stage.     

Stewart-Treves syndrome

Lymphangiosarcoma is an uncommon vascular tumor that usually develops in longstanding lymphedema. We gathered the cases of lymphangioma observed in a hospital and attempted to analyze their characteristics: age, sex, localization, treatment and follow-up data. We studied five cases: three cases of Stewart-Treves syndrome after mastectomy and radiotherapy and two cases that developed in patients with late-onset congenital lymphedema. There were four women and one man. Radical surgery was performed in four patients. The techniques employed were: above-knee amputation (one patient), hip disarticulation (one patient), scapulo-humeral disarticulation (two patients) and gemcitabine and radiotherapy in one patient with metastatic disease at diagnosis. Three patients died in the first 14 months of follow-up, while two are disease free after 46 and 86 months respectively. This study confirms the poor prognosis of patients with Steward-Treves syndrome.     

Isolated limb perfusion with tumor necrosis factor and melphalan for nonresectable Stewart-Treves lymphangiosarcoma

Background Cutaneous Stewart-Treves lymphangiosarcomas represent a rare group of tumors characterized by a high grade of vascularization and by localization in an extremity with lymphedema. The multifocality and the localization makes these tumors eligible for treatment with isolated limb perfusion (ILP). ILP with tumor necrosis factor (TNF) and melphalan is a safe and highly effective procedure that can achieve limb salvage in ≥80% of all patients with nonresectable extremity soft tissue sarcoma or melanoma. Methods In 10 patients with multifocal Stewart-Treves lymphangiosarcoma of the extremities, 16 ILPs with TNF plus melphalan were performed. All patients would have been candidates for exarticulation of the extremity. Results We observed an 87% overall response rate (complete and partial responses); one patient had a mixed response, and one patient did not respond to the therapy. In nine perfusions (56%), a complete response was achieved, and five perfusions (31%) resulted in a partial response. Limb salvage was achieved in eight patients (80%), with a mean follow-up duration of 34.8 months (range, 3 to ≥115 months). Regional toxicity was limited and systemic toxicity minimal to moderate, with no toxic deaths. Conclusions Multifocal Stewart-Treves lymphangiosarcomas in extremities with chronic lymphedema can be successfully treated by ILP with TNF and melphalan.     

Stewart-Treves syndrome and the use of positron emission tomographic scanning

In this article, we provide an account of two rare cases of Stewart-Treves syndrome, that is, cutaneous angiosarcoma secondary to lymphedema, treated at our center. Unusually, both occurred in the lower extremity. The first case was treated initially with a wide local excision, followed by a further re-excision, and eventually an above-the-knee amputation because of recurrence. In the second case, a hindquarter amputation was undertaken after a positron emission tomographic scan, which revealed the extent and spread of the lesions. In cases of cutaneous angiosarcoma, a positron emission tomographic scan can be extremely helpful in demonstrating the extent of subcutaneous spread and planning surgical management.     

Cutaneous angiosarcoma following breast-conserving surgery and radiation: an analysis of 27 cases

Iatrogenic angiosarcomas (AS), following treatment of breast carcinomas and attributed to chronic lymphedema, were first described by Stewart and Treves. With emphasis on breast-conserving therapy combined with adjuvant radiation, a recently recognized form of cutaneous postradiation angiosarcoma of the breast (CPRASB) has emerged. To more completely characterize CPRASB, 27 cases were analyzed. Histologic features studied included pattern of growth (vasoformative, sieve-like, or solid), nuclear grade, necrosis, and mitotic rate. Clinical and follow-up information was obtained. The patients received relatively standard radiation treatment. The median interval to diagnosis of CPRASB was 59 months; 5 occurred in less than 3 years. Lymphedema was largely absent, and when present was only mild in nature. CPRASB was frequently multifocal at presentation (13 of 27). All tumors had a vasoformative pattern of growth; the majority (16 of 27) had areas with a sieve-like pattern. The solid pattern was less frequent (7 of 27). The majority had high-grade nuclear features (16 grade 3, 8 grade 2, 3 grade1). The mean mitotic rate was 9/10 HPF. Necrosis was rare (2 of 27). All were treated with wide excision or mastectomy. Follow-up was available on 22 of 27 cases (median 44 months). Fourteen experienced local recurrence and 6 had multiple recurrences. Metastasis was documented in 9 of 22 patients and involved lungs (6), contralateral breast (3), skeleton (2), lymph nodes (1), and soft tissue (1). Eight patients died of disease, 2 died with disease, 4 were alive with disease, and 8 are alive without disease. The median interval to death was 33.5 months. All 4 patients with disease have documented metastasis. CPRASB differs from Stewart-Treves AS by its shorter latency period and lack of association with lymphedema.     

Primary malignant lymphoma arising in postmastectomy lymphedema. Another facet of the Stewart-Treves syndrome

In rare cases, primary malignant lymphomas may arise in the soft tissues. Only one previous case has arisen in the context of chronic lymphedema. Because of the clinical appearance of such lesions, which resemble violaceous nodular or plaquelike tumors, they may be confused clinically with lymphedema-associated angiosarcomas occurring after radical mastectomy (Stewart-Treves syndrome). Furthermore, the histologic appearance of some lymphomas and angiosarcomas may also be similar. We studied two women with primary postmastectomy lymphedema-related malignant lymphoma in the soft tissues of the upper arm. These tumors arose 11 and 30 years, respectively, after radical removal of ductal mammary carcinomas. Histologically, one neoplasm mimicked metastatic carcinoma or epithelioid angiosarcoma; whereas the other was initially confused with a variety of pathologic entities, including vasculitis, epithelioid hemangioma, and malignant fibrous histiocytoma. The lymphoid nature of both lesions was confirmed by immunoreactivity for leukocyte common antigen in addition to the B-lymphocyte marker, L26. Conversely, vascular and epithelial determinants were absent. One patient's disease pursued an indolent course; she died of unknown causes but with no evidence of lymphoma at last follow-up. The second patient is currently in remission on chemotherapy. Awareness of the existence of lymphedema-related malignant lymphoma and familiarity with methods used for its distinction from epithelioid vascular sarcomas should prevent unnecessary surgery.     

Squamous Cell Carcinoma in Chronic Lymphedema: Case Report and Review of the Literature

BACKGROUND: Squamous cell carcinoma (SCC) arising in chronic lymphedema is rare; only nine cases have been reported. OBJECTIVE: To present the evolution of SCC in chronic lymphedema. METHODS: Case report and literature review. RESULTS: The tumor was treated by wide excision and covered by a skin graft. CONCLUSION: In most of the other reported SCC cases in lymphedema, there are additional factors for carcinogenesis. There is no additional carcinogenic factor except for chronic lymphedema in our case. This strongly supports that lymphedema itself is one of the carcinogenic factors for not only angiosarcoma but also SCC.     

Stewart-Treves syndrome after treatment for breast cancer

This study reviews 3 cases of angiosarcoma of the upper extremity after mastectomy and radiotherapy for breast cancer (Stewart-Treves syndrome). Angiosarcoma was diagnosed an average 14 years (from 6.5 to 26 years) after treatment for breast cancer. Presenting signs included a red raised lesion, a palpable mass, a blister appearance (in one case). Two of our three patients underwent surgical treatment: one patient underwent local excision followed by chemotherapy, and the other patient wide excision, followed by external beam radiotherapy. Local recurrence occurred in one of these two patients and was followed by the development of lung metastases. The second patient who had treatment is free of disease without problems. The third patient refused any treatment and died 5 months later. The purpose of this article is to add to the literature 3 new cases of Stewart-Treves syndrome and to discuss some specific problems of this rare tumour.     

Stewart-Treves syndrome (angiosarcoma on lyphoedema): A rare complication of lymphoedema

Stewart-Treves syndrome (SST) or former lymphangiosarcoma is a rare complication of chronic lymphoedema mainly related to the breast cancer (90% of cases). It occurs in 0.03% of patients surviving 10 or more years after radical mastectomy. The prognosis is very poor with a five-year survival close to 10% despite the various treatment modalities. The treatment of choice is a large resection, but some authors recommend radical resection in the form of shoulder disarticulation or forequarter amputation. Surgical treatment can be preceded or followed by radiation therapy. Locally advanced tumors or metastatic forms can be treated with mono or polychemotherapy, systemic or local.     

Thoracic surgical implications of the yellow nail syndrome

Idiopathic lymphedema associated with yellow discoloration of the nail beds constitutes the yellow nail syndrome. Pleural effusions and chronic sinusitis are also frequently present. This report describes a case of yellow nail syndrome in a 65-year-old woman.     

乳癌术后上肢淋巴水肿的微波治疗

采用微波烘绑疗法治疗肢体淋巴水肿已有报道，但对乳癌根治术后所致的治疗效果报道甚少。对６４例患者采用微波治疗，每天微波照射１小时，２０天为一个疗程治疗，经过二个疗程治疗。治疗后随访１个月～２年。结果显示：症状明显消退，患侧肢体的水肿消退明显，经统计学分析有显著差异（Ｐ＜０．０１）；“丹毒”样发作的频率较治疗前明显减少，也有显著差异（Ｐ＜０．０１）；患肢的弹性得到恢复。无一例出现治疗后并发症。认为，对乳癌根治术后引起的上肢淋巴水肿，采用微波治疗，可获得较好效果，是保守治疗淋巴水肿的有效方法。     

Update on Post‐mastectomy Lymphedema Management

Lymphedema is a chronic, progressive condition caused by an imbalance of lymphatic flow. Upper extremity lymphedema has been reported in 16–40% of breast cancer patients following axillary lymph node dissection. Furthermore, lymphedema following sentinel lymph node biopsy alone has been reported in 3.5% of patients. While the disease process is not new, there has been significant progress in the surgical care of lymphedema that can offer alternatives and improvements in management. The purpose of this review is to provide a comprehensive update and overview of the current advances and surgical treatment options for upper extremity lymphedema.     

Reconstruction of penile and scrotal lymphedema.

Acquired genital chronic lymphedema and elephantiasis are not common problems in the United States. Most noncongenital cases are due to lymph node dissection, injury, or irradiation. Genital lymphedema is a functionally disabling and emotionally incapacitating entity, especially in the elderly population. Methods of reconstruction reported in the literature involve either lymphangioplasty or direct excision of the involved tissue with local tissue reconstruction. We report a case of acquired genital elephantiasis in an elderly man following radical cystectomy and pelvic irradiation for transitional cell bladder cancer. Our technique of reconstruction consisted of (1) excision of all involved lymphedematous skin of the penis and scrotum, and (2) use of posterior scrotal flaps, superiorly based flap of the pubic area for testicular coverage, and split-skin graft to the penis. Genital lymphedema presents a formidable challenge for reconstructive surgeons. The low morbidity and successful outcome observed in this case, however, indicate that the procedure described herein can and should be offered to elderly patients to improve function and quality of life.     

Chronic penile strangulation

Chronic penile strangulation is exceedingly rare with only 5 cases previously reported. We report an additional case of progressive penile lymphedema due to chronic intermittent strangulation caused by a rubber band applied to the penile base for 6 years. A 49-year-old man presented incapacity to exteriorize the glans penis. For erotic purposes, he had been using a rubber-enlarging band placed in the penile base for 6 years. With chronic use, he noticed that his penis swelled. Physical examination revealed lymphedema of the penis, phimosis and a stricture in the penile base. The patient was submitted to circumcision and the lymphedema remained stable 10 months postoperatively. Chronic penile incarceration usually causes penile lymphedema and urinary disturbance. Treatment consists of removal of foreign devices and surgical treatment of lymphedema.     

Lymphedema of the hand following a fracture of the distal radius

A case of chronic lymphedema of the hand and forearm following a fracture of the distal radius is reported. None of the classic causes of secondary lymphedema appeared to be the cause. A strong psychogenic component appeared to be the underlying mechanism. This rare phenomenon was thought to be self-induced and not genetic in nature.     

Stewart-Treves syndrome: lymphangiosarcoma following mastectomy

Lymphangiosarcoma (LAS) is an aggressive, malignant vascular tumor following long-lasting chronic lymphedema. Patients with LAS demonstrate a history of breast cancer treated by radical mastectomy in the majority of patients. In the 1960s the incidence of LAS in patients with a 5-year survival after radical mastectomy varied from 0.07 to 0.45%. Today, due to changes in the operative techniques of breast cancer, less chronic lymphedema is seen with only a scant number of LAS patients. The etiology of this enigmatic tumor is not yet completely understood. Histologically, LAS arises from vascular endotheliocytes, and all vascular sarcomas originating in the setting of a chronic lymphedema are categorized as LAS. There is no standard treatment of LAS. The treatment options include radical ablative surgery, radiation therapy, and chemotherapy. The prognosis of LAS is poor; long-term survival is the exception. Only early recognition and radical surgery offer a chance of cure.     

A modified therapeutic option for a long lasting success in the treatment of genital lymphedema

In cases of chronic genital lymphedema with an excessive proliferation of scrotal or labial tissue, lymph cysts, and fistulae, complex decongestive therapy (CDT) alone is not enough. After decongestion has been achieved by CDT, a major reconstructive procedure followed by a renewed phase of CDT is required. We report on our good cosmetic and functional long-time results achieved in 24 patients suffering from lymphedema of the genitalia treated by this combination of conservative CDT and surgery. In 17 male patients (16 primary and one secondary lymphedema), reduction of the scrotum was performed. Bilateral reduction of the labia was carried out in seven female patients (five secondary and two primary lymphedema). The mean follow-up was 67.8 months (36–84 months). The etiology of the lymphedema had no effect on the operation procedure or the operative outcome.An invited commentary on this paper is available at .     

A model of experimental lymphedema in rats' limbs

The authors' modified Olszewski method (J Cardiovasc Surg 9:178-183, 1968) for preparing chronic lymphedema with rats' limbs is presented. The incidence of chronic lymphedema formation after 9 months of follow-up was 64%. The experiment demonstrates that there is a brisk regeneration of the lymphatics under the lymphedema. The regeneration observed took place on the lateral wall of the blocked lymphatics, except for regeneration of capillary lymphatics, and the newborn lymphatics grew about 1 mm each day. There are indeed preexisting lymphatics in the body. They are of significant importance in the establishment of the collateral circulation because of their great number and early opening. Lymphatic regeneration and opening could be prevented by tissue scarring. Effective prevention of the formation of tissue scarring has important significance in preventing the development of chronic lymphedema.     

手法淋巴引流综合治疗肢体慢性淋巴水肿

目的 探讨采用手法淋巴引流治疗慢性淋巴水肿的新方法.方法 50例慢性肢体淋巴水肿患者接受了手法淋巴引流综合消肿治疗,对体表(包括躯干和肢体)淋巴系统沿淋巴回流方向和途径做轻柔的按压和按摩.50例中原发性淋巴水肿29例,继发性21例;下肢患病42例,上肢患病8例.采用多频生物电阻人体成分分析仪检测组织水肿和测量肢体周径检验治疗效果.结果 经过1～2个疗程的治疗,50例患病肢体的水肿均有不同程度的减轻,表现为患肢组织水肿程度显著减轻(P＜0.05)和患肢周径的显著缩小(P＜0.05).肢体周径与组织水肿变化的相关性检验表明两者有非常显著的相关性(rs=0.774,P＜0.01).结论 手法淋巴引流综合治疗的有效地促进滞留组织淋巴水肿的回流,减轻肢体的肿胀,改善患肢的外形,是治疗慢性淋巴水肿有效的治疗手段.     

Microsurgery for treatment of peripheral lymphedema: long-term outcome and future perspectives

Authors report over 30 years of their own clinical experience in the treatment of chronic peripheral lymphedemas by microsurgical techniques performed at the Center of Lymphatic Surgery of the University of Genoa, Italy. Over 1,500 lymphedema patients were treated with microsurgical techniques. Derivative lymphatic-venous techniques were most often used. For those cases where a venous disease was associated to lymphedema, reconstructive lymphatic microsurgery techniques were performed (lymphatic-venous-lymphatic-plasty). Objective assessment was undertaken by water volumetry and lymphoscintigraphy. Volume changes showed a significant improvement in over 83%, with an average follow-up of more than 10 years. There was an 87% reduction in the incidence of cellulitic attacks after microsurgery. Microsurgical lymphatic-venous anastomoses have a place in the treatment of peripheral lymphedema and should be the therapy of choice in patients who are not sufficiently responsive to nonoperative treatment. Improved results can be expected with operations performed at earlier lymphedema stages.