Secondary angiosarcoma: A fatal complication of chronic lymphedema.

Secondary Angiosarcoma (Stewart-Treves Syndrome) is a rare malignant cutaneous lesion, which arises in chronic lymphedema of the extremity, often observed after breast cancer treatment. We reviewed the history and the oncological outcome of two patients with this disease. Multimodal therapy including hyperthermic isolated limb perfusion with TNF-alpha and Melphalan, combined with radical resection of the affected skin and subcutaneous tissue including the fascia, with large safety margins may probably lead to better survival.

Successful management of lymphangiosarcoma in a puppy using a tyrosine kinase inhibitor.

A puppy was diagnosed with lymphangiosarcoma associated with lymphedema based on lymphography and histopathology. The lesions resolved after toceranib therapy, and the dog remains in remission 1 year later. This is the first report of a successful outcome following oral toceranib as first-line therapy for lymphangiosarcoma in a dog.

Gestion réussie d'un lymphangiosarcome chez un chiot à l'aide d'un inhibiteur de la tyrosinekinase. Un chiot a été diagnostiqué avec un lymphangiosarcome associé à un lymphoedème en se basant sur une lymphographie et l'histopathologie. Les lésions se sont résorbées après un traitement au tocéranib et le chien demeurait en rémission un an plus tard. Il s'agit du premier rapport d'un résultat favorable après le recours au tocéranib oral comme traitement de premier recours pour le lymphangiosarcome chez un chien.(Traduit par Isabelle Vallières).

Lymphangiosarcoma: Is Stewart-Treves Syndrome a Preventable Condition?

BACKGROUND: Stewart-Treves syndrome is a rare complication of breast cancer treatment, representing a lymphangiosarcoma commonly associated with lymphedema and severely impacting patient's outcome. The tumor typically develops in the atrophic, pachydermatous, hyperkeratotic skin of limbs affected by long-standing lymphedema. Clinical data associated with Stewart-Treves syndrome and lymphedema management have rarely been published. METHODS AND RESULTS: In the period between 1980 and 2009, ten patients with Stewart-Treves syndrome were diagnosed and treated at the Foeldiklinik, Hinterzarten, Germany. Nine of the ten patients were female. Five patients had previously suffered from breast cancer (and were treated with mastectomy); two from other malignancies; two patients had primary lymphedema, and one had undergone lower extremity lymphadenectomy. All cancer patients had undergone radiation treatment. In all cases, the sarcoma developed in non-irradiated areas 6-48 years (average 16.3 years) after the onset of lymphedema. None of the patients had received complex decongestive physical therapy (CDT). Two patients had above-elbow amputation, one had shoulder exarticulation, two patients had wide excision and skin grafting, two patients had above-knee amputation procedure, two patients had a below-knee amputation procedure, and one patient had no surgical treatment at all. The time to recurrence after surgery, time to metastasis, patient survival and CDT were recorded. CONCLUSIONS: Patients with lymphedema should be closely examined starting 5 years from the time of lymphedema onset, paying special attention to those with associated malignancies. Only early diagnosis and treatment by radical ablative surgery confers a reasonable prognosis with this rare but aggressive disease. A potential effect of CDT on lymphangiosarcoma has to be studied in a greater patient cohort.

Stewart Treves Syndrome.

Stewart-Treves Syndrome is characterized by the presence of lymphangiosarcoma on limb extremities. Rare, it occurs in 0.5% of patients who have undergone radical mastectomy with axillary node dissection. The main cause is chronic lymphedema with endothelial and lymphatic differentiation, with no direct relationship to breast cancer. Seven years after a radical right-side mastectomy with lymph node dissection and adjuvant therapy, the patient developed a lesion on her right arm. The dermatological examination revealed an erythematous nodule with bleeding surface on chronic right forearm lymphedema. After the biopsy, a lymphangiosarcoma on chronic lymphedema was diagnosed. Infrequent, this syndrome is relevant because of its associated mortality. Early diagnosis is important to improve survival and reduce complications.

Stewart-Treves syndrome following idiopathic leg lymphoedema: remember sarcoma.

Patients with skin complaints secondary to oedema are commonly encountered and can be a diagnostic challenge. Here, we present the case of a 78-year-old lady with Stewart-Treves syndrome, a rare cutaneous angiosarcoma. The histology showed angiocutaneous sarcoma with poorly defined margins. The prognosis remains extremely poor. A successful transfemoral amputation reduced the massive tumour burden and cleared a source of sepsis from this fungating tumour. Our aim is to highlight this rare but high-grade sarcoma which results from chronic lymphoedema. The red flag signs of sarcoma are masses greater than 5 cm, tumours which are rapidly growing, deep to fascia or painful, and tumours that recur after previous surgery. Non-healing or progressive ulceration in the background of chronic lymphoedema should raise the possibility of underlying malignancy. We need a low threshold for biopsy and early referral to an appropriate multidisciplinary team for the optimum management of the patient.

Stewart Treves Syndrome

Stewart-Treves Syndrome is characterized by the presence of lymphangiosarcoma on limb extremities. Rare, it occurs in 0.5% of patients who have undergone radical mastectomy with axillary node dissection. The main cause is chronic lymphedema with endothelial and lymphatic differentiation, with no direct relationship to breast cancer. Seven years after a radical right-side mastectomy with lymph node dissection and adjuvant therapy, the patient developed a lesion on her right arm. The dermatological examination revealed an erythematous nodule with bleeding surface on chronic right forearm lymphedema. After the biopsy, a lymphangiosarcoma on chronic lymphedema was diagnosed. Infrequent, this syndrome is relevant because of its associated mortality. Early diagnosis is important to improve survival and reduce complications.

Post-mastectomy benign lymphangioendothelioma of the skin following chronic lymphedema for breast carcinoma: a teaching case mimicking low-grade angiosarcoma and masquerading as Stewart-Treves syndrome.

Benign lymphangioendothelioma (BL) represents a very rare lymphatic vascular proliferation. Our aim is to be aware that owing to its characteristic features, pathologists can easily misinterpret it as cutaneous low-grade angiosarcoma when examining only small specimens. In the present case, multiple small and yellowish to reddish soft nodules were noticed in the edematous left arm of a 54-year-old Japanese female 4 years after the radical mastectomy with axillary lymph nodes dissection and following radiotherapy to the chest for the left breast carcinoma. The biopsy specimen showed an ill-defined lesion composed of a proliferation of irregular and sometimes anastomosing vascular structures in the dermis, lined by endothelial cells having mildly hyperchromatic and pleomorphic nuclei, but no mitotic figures. As the lesion grew within deeper dermis, these proliferating vessels dissected dermal collagenous bands, occasionally arranged in low-papillary projections and/or characteristic hobnail cytomorphology. We first interpreted it as low-grade angiosarcoma following chronic lymphedema due to the operation, i.e., the so-called Stewart-Treves syndrome. Although additional treatments were performed for 7 years, she had neither local invasion nor metastases of these tumors, respectively, and was alive and well. Retrospective immunohistochemical findings demonstrated that these mildly atypical endothelial cells were strongly positive for lymphatic vessel endothelial hyaluronan receptor (LYVE)-1 as well, and MIB-1 labeling index was less than 1%. Therefore, we finally made a diagnosis of BL of the skin. MIB-1 labeling index might be useful and adjunctive aids for reaching the correct diagnosis of cutaneous BL, especially in case of small or inadequate specimens.Virtual Slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/13000_2014_197.

Lymphangiosarcoma of the scalp.

BACKGROUND: Lymphangiosarcoma is a rare, aggressive malignancy that originates from the endothelial cells lining lymphatic vessels and carries an extremely poor prognosis. Its clinical and histologic features are often indistinguishable from angiosarcoma. OBJECTIVE: We sought to better characterize the clinical and histologic features of lymphangiosarcoma. METHODS: Case report and review of the literature. RESULTS: A number of immunohistochemical markers, including Von Willebrand factor, Ulex europaeus agglutinin 1, CD31, VEGFR-3, D2-40, Prox-1, can be used to help differentiate lymphatic from vascular tissue. CONCLUSIONS: Recent characterization of several new biologic markers has allowed greater differentiation between these tumors and may provide new therapeutic targets for treatment.

Stewart-Treves syndrome: a case report.

We present a case of chronic lymphoedema that progressed to Stewart-Treves syndrome in a 63-year-old woman with a previous modified radical mastectomy, associated lymph node dissection, chemotherapy and radiotherapy. While producing stabilisation of most cutaneous lesions initially, chemotherapeutic treatment of the angiosarcoma did not prevent subsequent metastasis and patient death. We urge vigilance and regular follow-up appointments for patients following a mastectomy with chronic lymphoedema to facilitate prevention or early treatment of this devastating syndrome.

Use of surgery and mitoxantrone chemotherapy in a dog with disseminated lymphangiosarcoma.

CASE DESCRIPTION: A 5-year-old sexually intact male Giant Schnauzer was evaluated because of difficulty breathing and left pelvic limb swelling. Eighteen months previously, the patient had had intermittent left pelvic limb swelling, but the owner declined further testing at that time. CLINICAL FINDINGS: Physical examination revealed severe pitting edema of the left pelvic limb and prepuce and muffled heart sounds on thoracic auscultation. Results of thoracic radiography and thoracocentesis were consistent with chylothorax, and CT imaging of the thorax and abdomen revealed a mass involving the whole left sublumbar area. TREATMENT AND OUTCOME: In an attempt to treat the chylothorax, pleural omentalization and pericardectomy were performed. Histologic evaluation of several biopsy specimens harvested in the abdominal and thoracic cavities revealed disseminated lymphangiosarcoma. The patient recovered well from surgery, and mitoxantrone chemotherapy was administered. As of 10 months after surgery, the dog was clinically normal except for mild pelvic limb edema. CLINICAL RELEVANCE: The combination of clinical signs, multiple imaging features, surgical findings, and histologic examination findings enabled the final diagnosis of lymphangiosarcoma. Clinical management that included medical and surgical treatments and chemotherapy resulted in improved quality of life and extended survival time in a dog with metastatic lymphangiosarcoma.

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.

Resection of a large intra-abdominal tumor in the Mexican axolotl: a case report.

OBJECTIVE: This case report describes the surgical removal of an intra-abdominal tumor from a Mexican axolotl (Ambystoma mexicanum). The animal was admitted with left abdominal swelling that had increased over 4 months. METHODS: Surgical removal was performed under general anesthesia with MS222 under an operating microscope. Exploratory laparotomy was performed through 2.5 dorsocranial skin incision in the left flank, followed by subcutaneous dissection. RESULTS: The tumor involved the spleen, was adjacent to the descending colon, and supplied by vessels from the spleen, stomach, and colon. The mass was removed by clamping and transecting the spleen and the peritoneum was closed with a continuous suture pattern, while abdominal muscles and skin were closed in layers. After a total duration time of anesthesia of 90 minutes the animal was kept in prophylactic antibiotic baths. Tissue sections revealed characteristics of both lymphangiosarcoma and lymphosarcoma with an appearance typical for a malignant tumor. CONCLUSIONS: Abdominal surgery was performed in an axolotl and the surgical wound healed without complication.

Reoperation in the postoperative period of lung transplantation.

BACKGROUND: Lung transplantation (LTx) has been established as the last treatment option in certain lung diseases. It is not uncommon for complications to occur that require urgent reoperation. The objective of our study was to analyze the characteristics of lung transplant patients who required reoperation in the postoperative period. PATIENTS AND METHODS: We have conducted a retrospective descriptive study of 224 lung transplants from January 1999 to September 2008, excluding retransplants. A subgroup of 16 subjects (7.2%) required reoperations. RESULTS: These 16 individuals had a mean age of 49.38 +/- 14.32 years with 75% men and 25% women. The disease leading to LTx was emphysema in 6 (37.5%), pulmonary fibrosis in 5 (31.3%), pulmonary hypertension in 2 (12.5%) and bronchiectasis, cystic fibrosis, and lymphangioleiomyomatosis in 1 each (6.3%). Preoperatively, 40% were taking corticosteroids. Double lung transplantation was performed in 56.3% and single lung in 43.7%. LTx surgery was prolonged in 68.8% of patients and intraoperative complications were more frequent than in the other patients (P = .041). The causes for reoperation were bleeding in 13 (of these, 5 had severe adhesions and 4 required extracorporeal circulation during LTx); bronchial dehiscence in 1; wall dehiscence in 1; and vascular stenosis in 1. At the end of the study, 62.5% were alive and among the 6 who died, 3 succumbed as a result of the surgery. Most subjects underwent late reoperation after a mean of 16 days from transplantation (range, 1-55). The need for reoperation was not associated with greater perioperative mortality. CONCLUSIONS: The incidence of reoperation in the postoperative period was low in our series. The main cause was bleeding. In more than half of the cases, LTx surgery was prolonged and intraoperative complications were more frequent. The need for reoperation was not associated with greater perioperative mortality.

Lung transplantation for lymphangioleiomyomatosis: the European experience.

BACKGROUND: Lung transplantation has been accepted widely as therapy for end-stage pulmonary lymphangioleiomyomatosis (LAM); however, single-center and national experience is limited due to the rarity of LAM. METHODS: We report the recent European experience of lung transplantation for LAM. A self-administered questionnaire was distributed to 30 European lung transplant centers to evaluate patients who underwent primary lung transplantation for LAM (1997 to 2007). RESULTS: Seventy percent of centers responded to the questionnaire. A total of 61 lung transplants were undertaken in women only, with mean age at transplant 41.3 years (SD 5.1). Centers performed a median of 2 (0 to 9) transplant operations. Severe pleural adhesions were the most common intra-operative complication. Early deaths (N = 6) were due to primary graft or multiple-organ failure or sepsis. Twelve recipients were diagnosed with bronchiolitis obliterans syndrome at a median of 20 months (range 10 to 86 months) post-transplant. LAM-related complications included renal angiomyolipoma and pneumothorax in the native lung. Recurrence of LAM occurred in 4 recipients. As of December 2007, actuarial Kaplan-Meier survival was 79% at 1 year and 73% at 3 years post-transplant. CONCLUSIONS: Post-transplant outcome for pulmonary LAM in the recent era appears to have improved compared with the previous era. LAM-related complications remain common, but recurrence of LAM in the allograft is rare.

Lymphangiosarcoma in the nictitating membrane of a horse.

A 15-year-old Haflinger gelding presented with a mass in the left nictitating membrane. Two biopsies and the excised nictitating membrane were taken at different time points as a result of reoccurrence of the mass and submitted for histopathologic evaluation. The horse was euthanized as a result of poor prognosis following the reoccurrence of the mass after surgical removal. Histologically, the mass consisted of dilated, thin-walled vascular clefts and channels, lined by flattened to cuboidal endothelial cells with moderate cellular pleomorphism. There was up to 1 mitotic figure per high power field. The channels were empty or contained few erythrocytes. In the collagen-rich stroma, few lymphocytes, focal follicular lymphoid aggregations, and marked lymphangiectasia were observed. Immunohistochemically, the neoplastic cells stained positive for vimentin and partially positive for factor VIII-related antigen. Ultrastructural analysis revealed discontinuous endothelial lining vascular channels that partially lacked a basal membrane. Based on the histopathologic, immunohistochemical, and ultrastructural features lymphangiosarcoma was diagnosed.

Stewart-Treves syndrome as a rare complication of a hereditary lymphedema.

Lymphangiosarcoma (LAS) may occur as a rare complication of primary lymphedema. A case of LAS in hereditary lymphedema of the lower extremity in a 36-year old female is reported. Despite of chemotherapy, local hyperthermia and later amputation of the extremity the patient died of progressive disease due to pulmonary metastasis. In respect to this case, the different therapeutic concepts, as reported in the literature, and their results are presented and discussed.

Lymphangiosarcoma in a dog treated with surgery and chemotherapy.

A large subcutaneous mass at the left cervical site in a 9-year-old male Siberian husky was removed surgically. Histopathologically, the mass was mainly consisted of a proliferation of spindle-shaped neoplastic cells arranging in solid sheath and partially vascular channels containing few blood cells. The tumor cells exhibited highly invasive activity to the surrounding tissues. In addition, the tumor cells were immunopositive for Factor VIII-related antigen. On the basis of these findings, the tumor was diagnosed as lymphangiosarcoma. Recurrent mass was noticed 3 weeks after surgery but completely disappeared after the doxorubicin treatment. Neither recurrence nor metastasis was observed for 9 months after the remission.