Lymphangiosarcoma in chronic lymphoedema. Stewart-Treves syndrome

A case of post-mastectomy lymphangiosarcoma is reported. Lymphangiosarcoma is an extremely rare but highly lethal complication of chronic lymphoedema. Our patient was treated by amputation and died 6 months later. Treatment of post-mastectomy lymphangiosarcoma is still unsatisfactory. Early recognition and radical ablative surgery seem to provide best chance for survival.     

Postmastectomy angiosarcoma

Six cases of postmastectomy angiosarcoma of the upper extremity have been treated at the North Carolina Baptist Hospital/Wake Forest University Medical Center over the past 25 years. Five patients had typical ductal carcinoma; one patient had mammary Paget's disease. Two patients, including the patient with Paget's disease, had bilateral mastectomies. The five patients with ductal carcinoma had received postoperative radiation therapy. The mean interval between mastectomy and the appearance of angiosarcoma was 17.5 years. Significant lymphedema of the upper extremity preceded the appearance of angiosarcoma in all six patients, and it appears important that special care be taken during mastectomy to preserve the integrity of the lymphatic system. The patient treated with interscapulothoracic amputation is alive at 2 years postamputation; the five patients whose angiosarcomas were treated with wide excision or radiotherapy died an average of 4.5 years later. Interscapulothoracic amputation is recommended as the initial treatment of postmastectomy angiosarcoma of the upper extremity.     

上肢透明细胞肉瘤的治疗

目的 探讨上肢透明细胞肉瘤的疗效.方法 对3例上肢透明细胞肉瘤患者采用了肿块扩大切除术进行治疗,并采用带蒂尺动脉腕上皮支皮瓣覆盖创面.结果 术后3例皮瓣全部存活,伤口Ⅰ期愈合.随访时间例1为2年,例2为4年,2例患者至今存活,局部肿块无复发,无局部和远处转移;例3患者于前臂中段截肢术后4个月死亡.结论 透明细胞肉瘤是一种高度恶性的软组织肿瘤,早期手术是主要的治疗手段.     

上肢透明细胞肉瘤的治疗

目的 探讨上肢透明细胞肉瘤的疗效.方法 对3例上肢透明细胞肉瘤患者采用了肿块扩大切除术进行治疗,并采用带蒂尺动脉腕上皮支皮瓣覆盖创面.结果 术后3例皮瓣全部存活,伤口Ⅰ期愈合.随访时间例1为2年,例2为4年,2例患者至今存活,局部肿块无复发,无局部和远处转移;例3患者于前臂中段截肢术后4个月死亡.结论 透明细胞肉瘤是一种高度恶性的软组织肿瘤,早期手术是主要的治疗手段.     

Postmastectomy lymphangiosarcoma: experience with three patients and electron microscopic observations in one.

The clinical features are presented of three patients in whom lymphangiosarcoma developed after radical mastectomy followed by local irradiation. The median time between radical mastectomy and the diagnosis by biopsy of lymphangiosarcoma was 9.9 years. No form of therapy, including high-dose combination chemotherapy, appeared able to control the disease. All three patients died with residual lymphangiosarcoma and in two it was the direct cause of death. These results stress that prevention of postmastectomy lymphangiosarcoma is vital. This can be accomplished by avoiding radical mastectomy followed by local irradiation. Ultrastructural observations in one case suggest that the tumour has a primitive vasoformative origin.     

Treatment of advanced and inaccessible sarcomas with continuous intra-arterial chemotherapy prior to definitive surgery or radiotherapy--a possible alternative to amputation or disabling radical surgery

Four patients with advanced and inaccessible soft tissue sarcomas were treated with a regimen of intra-arterial chemotherapy followed by radiotherapy and/or surgical excision. Two of the patients had advanced sarcomas in the buttock and thigh regions which would otherwise have required hindquarter amputation in one case or disarticulation of the hip in the other case. These sarcomas responded significantly to intra-arterial chemotherapy to the extent that subsequent local surgery was effective in eradicating the residual tumours. No viable tumour cells were found in the resected specimens. In both patients amputation was avoided and local tumour eradication was achieved. In the other two patients, advanced and non-resectable sarcomas in the head were first treated with a similar regimen of intra-arterial chemotherapy. In both cases the tumours regressed in size prior to administration of local radiotherapy. After completion of chemotherapy and radiotherapy no viable tumour cells were detected in either lesion. In one case (originally a very extensive sarcoma of the jaw in a 5 year old child) a residual lump was resected but no viable tumour was detected in the resected specimen. These four patients represent our total experience with this plan of management. All responded well and there has been no evidence of local disease recurrence in any of the four patients. One patient (Case 2) did develop pulmonary and bone metastases from which she died 2 years later but the other three patients remain well with no evidence of residual disease, 11 years, 4 years and 20 months after presentation.     

TREATMENT OF ADVANCED AND INACCESSIBLE SARCOMAS WITH CONTINUOUS INTRA-ARTERIAL CHEMOTHERAPY PRIOR TO DEFINITIVE SURGERY OR RADIOTHERAPY—A POSSIBLE ALTERNATIVE TO AMPUTATION OR DISABLING RADICAL SURGERY

Four patients with advanced and inaccessible soft tissue sarcomas were treated with a regimen of intra-arterial chemotherapy followed by radiotherapy and/or surgical excision. Two of the patients had advanced sarcomas in the buttock and thigh regions which would otherwise have required hindquarter amputation in one case or disarticulation of the hip in the other case. These sarcomas responded significantly to intra-arterial chemotherapy to the extent that subsequent local surgery was effective in eradicating the residual tumours. No viable tumour cells were found in the resected specimens. In both patients amputation was avoided and local tumour eradication was achieved. In the other two patients, advanced and non-resectable sarcomas in the head were first treated with a similar regimen of intra-arterial chemotherapy. In both cases the tumours regressed in size prior to administration of local radiotherapy. After completion of chemotherapy and radiotherapy no viable tumour cells were detected in either lesion. In one case (originally a very extensive sarcoma of the jaw in a 5 year old child) a residual lump was resected but no viable tumour was detected in the resected specimen. These four patients represent our total experience with this plan of management. All responded well and there has been no evidence of local disease recurrence in any of the four patients. One patient (case 2) did develop pulmonary and bone metastases from which she died 2 years later but the other three patients remain well with no evidence of residual disease, 11 years, 4 years and 20 months after presentation.     

Lymphangiosarcoma with postmastectomy edema of the arm

A case of a lymphangiosarcoma (LAS) in a chronic postmastectomy lymphedematous arm in a 67 y.o; women is reported. The LAS was found 8 years after a radical left mastectomy for cancer and 7 years after the development of a lymphedema in the left arm. Because of a rapid spread of the disease no surgical treatment was done. The patient died 14 months after the diagnosis. Lymphangiosarcoma is a rare neoplasia that usually arise in chronic lymphedematous limbs mostly in post-mastectomy lymphedema of the arms. Chronic lymphedema is an important neoplastic stimuli decreasing the local immunity, as well leading to lymphoproliferative and degenerative changes of collagenous and fat tissues. Usually it appears as a multicentric lesion like bluish nodules, sclerotic plaques, bullous lesions. Lungs, pleura and thoracic wall are the most common sites of metastatic disease. The DD should be done with Kaposi sarcoma, hemangioma, hemangiopericytoma. The prognosis is always poor and after treatment the mean survival time is 18 months. The best treatment that gives a temporary result is the ablative surgery. Therefore because of unsuccessful therapeutic procedures a particular care should be paid to avoid postmastectomy lymphedema leaving, when possible, a reasonable lymphatic drainage of the arm.     

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.     

Leiomyosarcomas of the rectum. Amputation of the rectum or local resection?]

Two cases of high grade leiomyosarcoma of the rectum treated by local excision are reported. The first patient presented a local recurrence associated with liver and pulmonary metastases and eventually died, respectively 11 and 22 months after resection. The second patient's tumor recurred 3 months after excision and was treated with pelvic exenteration and CYVADIC regimen as chemotherapy and died 3 years later from local recurrence. A review of the literature indicates that local surgical excision in comparison to a more radical surgical approach, such as abdominoperineal resection, is as effective in improving survival. Lesions less than 2.5 cm in diameter, confined to the bowel can be treated by local surgical excision. To date no adjuvant therapy is effective in terms of survival or local control.     

Extremity epithelioid sarcoma. Amputation vs local resection.

Amputation has traditionally been advised for extremity epithelioid sarcoma because of its pattern of innocuous presentation and relentless soft-tissue and nodal metastasis. To assess the role of amputation in extremity epithelioid sarcoma, we reviewed our experience with 42 patients treated between 1961 and 1986. On presentation with localized primary tumor (n = 18), nine of 11 patients who underwent wide local excision and four of six patients who underwent excisional biopsy were free of disease, and one patient who underwent amputation died. After presentation with localized recurrence (n = 12), four of six patients who underwent wide local excision and two patients who underwent excisional biopsy were free of disease; three other patients who underwent wide local excision had margins that tested positive on pathologic examination, of whom one was free of disease; one patient who underwent amputation died of disease. On presentation with regional metastasis (n = 12), only one of five patients who underwent wide local excision and one of seven patients who underwent amputation were free of disease. Primary amputation offered no apparent overall survival benefit to patients presenting with regional metastasis. The favorable outcome after local resections for localized disease indicates that wide local excision with margins that test negative on pathologic examination is preferable to radical amputation in these patients.     

乳腺分叶状肿瘤14例的诊断和治疗

目的：探讨乳腺分叶状肿瘤的临床病理特点、治疗原则和影响预后的因素。方法：回顾1993年6月至2001年2月收治的14例乳腺分叶状肿瘤患者的临床资料。结果：14例患者平均年龄35岁，按WHO分类标准，其中良性5例，交界性5例，亚性4例。7例行局部切除术，4例行乳腺单纯切除术，3例行乳腺癌改良根治术。随访11例，平均随访时间19个月（6-96个月），1例行乳腺癌改良根治术后2年死于远处转移，3例行局部切除术后复发。结论：保证1-2cm切缘的扩大局部切除术是治疗乳腺分叶状肿瘤的首选方案。     

寰枢椎肿瘤的手术治疗（附22例报告）

目的：探讨不同手术入路,切除术式及内固定治疗寰椎,枢椎肿瘤的效果,方法：回顾性分析22例寰椎和枢椎肿瘤患者的病理类型,临床表现,各种手术途径,术式及其预后,结果：22例中,寰椎肿瘤4例,枢椎肿瘤18例;原发性肿瘤19例,转移性肿瘤3例,9例行囊内切除,8例包膜切除,3例广泛切除,单纯枕颈后路植骨CD Cervical或Cervifix内固定术2例,1例骨巨细胞瘤患者术后10d因呼吸循环衰竭死亡,1例转移癌患者术后13d因肺部感染,呼吸循环衰竭死亡,术后随访3个月至4年,20例术后近期疗效较满意,局部疼痛和神经症状改善或缓解,2例转移癌患者分别于术后13个月和19个月全身多处转移,全身衰竭死亡,1例骨母细胞瘤,1例软骨肉瘤术后1年局部复发。结论：应根据寰枢椎肿瘤的具体部位,范围选择相应的手术入路和术式,肿瘤的性质,手术方式及术后综合治疗对患者预后具有重要影响。枕颈CD Cervical或Cervifix内固定术有利于保持上颈椎的稳定。     

Lymphangiosarcoma (Stewart-Treves syndrome) in postmastectomy patients

Stewart-Treves syndrome (STS) is a rare but aggressive upper extremity lymphangiosarcoma in postmastectomy patients. Unfamiliarity with this disease and the innocuous appearance of the tumor often lead to delayed diagnosis. A comprehensive search of the databases at a single tertiary-care academic institution revealed only 3 cases of STS in the last 63 years. The latency time between breast cancer treatment and diagnosis of STS was 11 to 21 years. Survival after diagnosis of STS ranged from 8 to 15 months. One patient underwent radical surgery. The extensive lymphangiosarcoma in the other 2 patients precluded surgical resection and they underwent chemotherapy. All patients had adjuvant radiation therapy at the time of the original breast cancer resection. This report includes a discussion of the epidemiology, etiology, presentation, treatment, and prognosis of STS.     

Penis conserving treatment for T1 and T2 penile carcinoma: clinical implications of a local recurrence

PURPOSE: We evaluated our experience with primary tumor treatment for T1 and T2 penile squamous cell carcinoma and discussed the clinical implications of a local recurrence. MATERIALS AND METHODS: The primary tumor treatment and clinical course of 257 patients with T1 or T2 penile carcinoma were evaluated. Primary tumor treatment consisted of penis preservation in 157 and (partial) amputation in 100 patients. Median followup was 106 months (range 16 to 541). RESULTS: The 5-year local recurrence-free estimate after penis preservation was similar for T1 and T2 tumors (log rank test p = 0.1) and overall 63% (CI: 54%-72%) compared to 88% (CI: 81%-95%) for partial amputation (log rank test p = 0.0003). In case of a local recurrence after penis preserving treatment, local control could be achieved in 94% (51 of 54) of cases. Of patients with T1 tumors treated with penis preservation, regional recurrence developed in 33% (7 of 21) of patients with local recurrence compared to only 6% (3 of 47) of patients without local recurrences (Fisher's exact test p = 0.005). Of the patients with T2 tumors treated with penis preservation, regional recurrence developed in 27% (9 of 33) of patients with local recurrence compared to 27% (12 of 45) of patients without local recurrence (chi-square test p = 0.96). Of 10 patients with a local recurrence after partial amputation of the penis, 9 died of disease. CONCLUSIONS: The incidence of local recurrence increases with penis preservation but can be treated accurately in most cases. Local recurrences can signify lymphatic regional spread. A local recurrence after penile amputation carries a poor prognosis.     

Infected femorodistal bypass: is graft removal mandatory?

Infected lower extremity bypass grafts have been associated with high rates of limb loss. Traditionally treatment has included graft excision. To compare aggressive local treatment, without graft removal, with more conventional graft excision, we reviewed 38 consecutive patients with 39 infected lower extremity bypasses treated during the last 10 years. The grafts used were prosthetic in 33 cases, vein in 4, and composite in 2. Median follow-up was 2.7 years. Twenty-eight infected grafts were treated with either complete (14) or partial (14) graft removal. Nine new grafts were placed. Recurrent infection developed in five cases, and two patients died of complications of graft infection. Ten of 20 limbs at risk were lost. Eleven patients with patent bypasses (4 vein, 2 composite, 5 prosthetic) were treated without graft excision. Treatment of five patients in this group included muscle transposition. Five patients were treated with incision and drainage of abscesses, and one had excision of a persistent sinus tract. One patient underwent major amputation 6.3 years after treatment of graft infection. Limb salvage was significantly higher (p = 0.012, log-rank test) than in patients treated with graft excision. One patient died, and no recurrent infections developed; these were not significant differences compared with those having graft excision. We conclude that aggressive local treatment of infected lower extremity bypass grafts, including drainage, debridement, and muscle transposition may treat infection in selected patients without the need for graft removal and with rates of limb salvage superior to those obtained with excisional therapy.     

枢椎肿瘤切除与重建技术探讨

目的：研究枢椎肿瘤的不同手术入路，肿瘤切除术及内固定重建的治疗效果。方法：对枢椎肿瘤的性质，病理类型，临床表现，各种手术途径，术式及其预后进行分析，本组18例中，骨巨细胞瘤6例，骨母细胞瘤1例，软骨肉瘤1例，骨髓瘤2例，脊索瘤2例，血管内皮细胞瘤1例，恶性淋巴瘤1例，神经纤维瘤1例，转变癌3例。5例行囊内切除，5例包膜切除，6例广泛切除，单纯枕颈后路植骨CD Cervical或Cervifix内固定术2例。结果：1例骨巨细胞瘤患进术后10d因四肢瘫痪状加重，呼吸循环衰竭死亡，1例转移癌患者术后13d因肺部感染，高热，呼吸循环衰竭死亡，术后随访6个月－4年，16例术后近期疗效较满意，局部疼痛和神经症状改善或缓解，2例转移癌患者分别于术后13个月和19个月全身多处转移，全身衰竭死亡，1例骨细胞瘤、1例软骨肉瘤术后1个局部复发。结论：应根据枢椎肿瘤具体部位，范围选择相应的手术入路和术式。肿瘤的性质，手术方式极术后综合治疗对患者预后具有重要影响。枕颈CD Cervical或Cervifix内固定术有利于保持上颈椎的稳定。     

上肢软组织恶性肿瘤的手术治疗和预后

目的 总结10年来上肢软组织恶性肿瘤的分类、发生、发展、治疗及预后。方法 对22例上肢软组织恶性肿瘤，采用手术治疗将肿瘤及边缘的正常组织一并切除，尽量保留肢体及其重要血管和神经，以保存上肢功能。行神经移植修复3例，带蒂皮瓣覆盖创面8例。对10例复发病例再次切除肿瘤组织后，进行化学治疗和放射治疗等综合治疗。结果 术后随访最短3个月，最长10年半，平均5年；其中2例滑膜肉瘤于术后1年因远处转移而死亡，其余病人无远处转移。5年生存率为87．5％，生存5年以上者共14例。局部复发14例，复发率为70％；由于多次复发最终上肢截肢1例，手指截指2例，肢体保存率为85％。结论 上肢软组织恶性肿瘤以手术治疗为主，辅以化学治疗和放射治疗等综合治疗。     

胃肠道神经鞘瘤的诊断及治疗

目的 分析胃肠道神经鞘瘤的发病概况并探讨其诊治方法。方法 对１２例胃肠道神经鞘瘤进行回顾性分析并文献复习。结果 术前确诊仅１例,手术切除１０例,术中活检２例;良性５年,２年内死于伴发病２例,存活已〉２年３例;恶性４例,１年及３年内死亡各为３例及１例;恶变倾向３例,２年内死亡１例,已存活〉６年２例。结论 胃肠道神经鞘瘤术前诊断困难,确诊靠病理,免疫组化检查有助于与平滑肌瘤鉴别。良性者可行局部切除,恶     

骨巨细胞瘤手术治疗的临床观察

目的：观察骨巨细胞瘤患者手术治疗的疗效。方法：23例均手术治疗，其中肿瘤切除 碘酊、酒精局部灭活 骨水泥填塞术14例，肿瘤瘤段切除 人工金属假体置换术4例，截肢术2例，肿瘤切除 病灶清除术2例，肿瘤刮除 植骨术1例。结果：治愈20例，死亡3例。术后随访2年～7年5个月，平均3年6个月。结论：我们提倡GCT采用临床、X线和病理三结合方法，临床疗效满意。对提高诊断和治疗有很大的价值。