A retrospective analysis of Stewart-Treves syndrome in the context of chronic lymphedema

Abstract Background stewart-treves syndrome (STS) is an angiosarcoma associated with chronic lymphedema. Objectives This article analyses the characteristics of twenty-two patients and proposes active intervention in lymphedema and the early diagnosis of STS. Methods Twenty-two patients with STS were diagnosed at the centre over an 11-year period. Clinical manifestations, a series of conventional analyses, and histopathology were used to study these cases retrospectively. Results The age range of 22 patients with STS was 15 to 78 years. The main clinical manifestations included multiple skin and subcutaneous nodules and scattered red or purplish-red rashes in the lymphoedematous limbs. These patients often showed clinical symptoms such as lymphedema, weakness, emaciation, pain, mass, lymphadenopathy and so on. The positive rates of ultrasonography, MRI and radionuclide imaging were 66.7% (6/9), 92.3% (12/13) and 18.2% (2/11), respectively. The main points regarding active intervention in lymphedema and early diagnosis of STS were summarized. Study limitations Since this was a retrospective study, the main points summarized by the author need to be further quantified in clinical work to guide the diagnosis of this kind of disease more conveniently. In addition, further clinical trials are needed to evaluate the role of lymphedema in the occurrence and development of malignant tumors. Conclusions STS can appear in lymphoedematous tissue many years after lymphedema onset. To avoid delays in the diagnosis and therapy of STS, physicians should actively look for signs or symptoms of malignant lymphedema during the follow-up period and promptly manage patients developing problems.

A retrospective analysis of Stewart-Treves syndrome in the context of chronic lymphedema.

BACKGROUND: stewart-treves syndrome (STS) is an angiosarcoma associated with chronic lymphedema. OBJECTIVES: This article analyses the characteristics of twenty-two patients and proposes active intervention in lymphedema and the early diagnosis of STS. METHODS: Twenty-two patients with STS were diagnosed at the centre over an 11-year period. Clinical manifestations, a series of conventional analyses, and histopathology were used to study these cases retrospectively. RESULTS: The age range of 22 patients with STS was 15 to 78 years. The main clinical manifestations included multiple skin and subcutaneous nodules and scattered red or purplish-red rashes in the lymphoedematous limbs. These patients often showed clinical symptoms such as lymphedema, weakness, emaciation, pain, mass, lymphadenopathy and so on. The positive rates of ultrasonography, MRI and radionuclide imaging were 66.7% (6/9), 92.3% (12/13) and 18.2% (2/11), respectively. The main points regarding active intervention in lymphedema and early diagnosis of STS were summarized. STUDY LIMITATIONS: Since this was a retrospective study, the main points summarized by the author need to be further quantified in clinical work to guide the diagnosis of this kind of disease more conveniently. In addition, further clinical trials are needed to evaluate the role of lymphedema in the occurrence and development of malignant tumors. CONCLUSIONS: STS can appear in lymphoedematous tissue many years after lymphedema onset. To avoid delays in the diagnosis and therapy of STS, physicians should actively look for signs or symptoms of malignant lymphedema during the follow-up period and promptly manage patients developing problems.

Síndrome de Stewart-Treves no membro inferior com apresentação exuberante: relato de caso / Profuse Stewart-Treves syndrome in the right leg: a case report

A Síndrome de Stewart-Treves é uma rara condição onde um angiossarcoma se desenvolve sobre área de linfedema crônico. Afeta mais frequentemente os membros superiores e representa 5% dos casos de angiossarcoma. Apesar de ser mais comum em mulheres submetidas a mastectomia radical e esvaziamento axilar por neoplasia de mama, pode ocorrer a partir de linfedema de outras etiologias. Clinicamente as lesões são caracterizadas por nódulos únicos ou múltiplos de cor avermelhada ou azulada no estágio inicial e, em estágio avançado, podem evoluir para massas hemorrágicas e até gangrena. O prognóstico é ruim e a conduta cirúrgica com excisão ampla ou amputação oferecem uma maior chance de sobrevida. Relata-se um caso de Síndrome de Stewart-Treves na perna direita, apresentando-se de forma exuberante e com acometimento de linfonodos pélvicos e abdominais.

Stewart-Treves syndrome is a rare condition characterized by the development of an angiosarcoma at sites of chronic lymphedema. It most often affects the upper limbs and accounts for 5% of cases of angiosarcoma. Although the syndrome is more common in women undergoing radical mastectomy and axillary dissection for breast cancer, it may result from lymphedema of other etiologies. Clinically, the lesions are characterized by single or multiple bluish-red nodules in the early stage and may progress to hemorrhagic masses and even gangrene in advanced stages. The prognosis is poor, and surgical management with wide excision or amputation offers a greater chance of survival. We report a case of profuse Stewart-Treves syndrome in the right leg, with involvement of pelvic and abdominal lymph nodes.

Cutaneous angiosarcoma: report of three different and typical cases admitted in a unique dermatology clinic

Abstract: Angiosarcoma is a rare and aggressive tumor with high rates of metastasis and relapse. It shows a particular predilection for the skin and superficial soft tissues. We report three distinct and typical cases of angiosarcoma that were diagnosed in a single dermatology clinic over the course of less than a year: i) Angiosarcoma in lower limb affected by chronic lymphedema, featuring Stewart-Treves syndrome; ii) a case of the most common type of angiosarcoma loated in the scalp and face of elderly man and; iii) a skin Angiosarcoma in previously irradiated breast. All lesions presented characteristic histopathological findings: irregular vascular proliferation that dissects the collagen bundles with atypical endothelial nuclei projection toward the lumen.

Vulvar lymphangiosarcoma in a bitch

Background: Lymphangiosarcoma (LSA) is a rare, highly malignant and infiltrative neoplasm of the lymphatic endothelium of dogs and cats. It is mostly reported in medium to large breed dogs, over 5-year-old, with no sexual predisposition. Affected animals present fluctuating and diffuse swelling, covering both dermis and subcutaneous tissue, spreading through lymphatic and haematic vessels. Histologically, LSA is characterized by connected channels devoid of conspicuous haematic elements. Treatment depends on location of the neoplasm, staging, and possibility of curative surgical excision. Chemotherapy and radiotherapy can increase survival time. In this report, we describe a rare case of vulvar LSA in a dog. This is the first Brazilian report of LSA in dogs so far.Case: A 3-year-old, female, mixed breed dog was presented for evaluation of vesicle-bullous lesions in the vulvar and perivulvar region with progressive growth along 6 month. Histopathology revealed neoplastic proliferation in the superficial dermis, advancing through the profound dermis. The histological lesion pattern was consistent with angiosarcoma, which united along with macroscopic pattern of the tumor, and the presence of multiple anastomosed vascular structures without erythrocytes within it at microscopy, was compatible with LSA. No evidence of metastasis or lymphadenopathy was found on survey radiography and ultrasound. We performed a surgical excision, and remaining wound was reconstructed with an advancement skin flap. Despite wide surgical resection, neoplastic cells could be found in surgical borders, as well as a metastatic inguinal lymph node. Postoperative chemotherapy based on doxorubicin as a single agent was administrated. Disease free interval (DFI) was one month after surgery, when small bullous lesions were observed near the surgical site, and histopathological exam confirmed LSA...

Vulvar lymphangiosarcoma in a bitch

Background: Lymphangiosarcoma (LSA) is a rare, highly malignant and infiltrative neoplasm of the lymphatic endothelium of dogs and cats. It is mostly reported in medium to large breed dogs, over 5-year-old, with no sexual predisposition. Affected animals present fluctuating and diffuse swelling, covering both dermis and subcutaneous tissue, spreading through lymphatic and haematic vessels. Histologically, LSA is characterized by connected channels devoid of conspicuous haematic elements. Treatment depends on location of the neoplasm, staging, and possibility of curative surgical excision. Chemotherapy and radiotherapy can increase survival time. In this report, we describe a rare case of vulvar LSA in a dog. This is the first Brazilian report of LSA in dogs so far.Case: A 3-year-old, female, mixed breed dog was presented for evaluation of vesicle-bullous lesions in the vulvar and perivulvar region with progressive growth along 6 month. Histopathology revealed neoplastic proliferation in the superficial dermis, advancing through the profound dermis. The histological lesion pattern was consistent with angiosarcoma, which united along with macroscopic pattern of the tumor, and the presence of multiple anastomosed vascular structures without erythrocytes within it at microscopy, was compatible with LSA. No evidence of metastasis or lymphadenopathy was found on survey radiography and ultrasound. We performed a surgical excision, and remaining wound was reconstructed with an advancement skin flap. Despite wide surgical resection, neoplastic cells could be found in surgical borders, as well as a metastatic inguinal lymph node. Postoperative chemotherapy based on doxorubicin as a single agent was administrated. Disease free interval (DFI) was one month after surgery, when small bullous lesions were observed near the surgical site, and histopathological exam confirmed LSA...(AU)

Síndrome de Stewart-Treves / Stewart-Treves Syndrome

El síndrome de Stewart-Treves es un angiosarcoma cutáneo, poco frecuente, que se produce en pacientes con linfedema crónico. Se describe clásicamente luego de realizar mastectomía radical con vaciamiento ganglionar y/o radioterapia. Su mortalidad es elevada. Comunicamos el caso de una paciente de 76 años de edad, sometida a mastectomía radical con vaciamiento ganglionar axilar, seguido de quimioterapia y radioterapia. Consulta 13 años después por dolor intenso y lesión tumoral exofitica, ulcerada, angiomatosa, sangrante y maloliente en cara anterior del brazo izquierdo. Presentaba edema del miembro de varios años de evolución. Luego de realizar biopsia por punch de la lesión, los resultados histopatológicos resultaron compatibles con linfangiosarcoma. La familia junto con el servicio de Cirugía decide realizar la extirpación del tumor, con conservación del miembro. Falleció luego de once meses de realizado el diagnóstico de síndrome de Stewart-Treves, en nuestro Servicio.

The Stewart-Treves Syndrome is a rare cutaneous angiosarcoma, that occurs in patients with chronic lymphedema. It is classically described after performing radical mastectomy with lymph node dissection and/or radiation therapy. Its mortality is high. We have reported the case of a 76 years old patient, who underwent radical mastectomy with axillary lymph node dissection, followed by chemotherapy and radiotherapy. She came 13 years later with an intense pain and exophytic, ulcerated, angiomatous, bleeding and smelly tumor injury in front side of the left arm. She had an edema of the left upper limb with several years of evolution. After performing punch biopsy of the lesion the histopathological results were compatible with lymphangiosarcoma. The family, jointly with the surgery service decided to remove the tumor with limb preservation. She died 11 months after the diagnosis of Stewart-Treves syndrome, performed in our department.

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.

Linfangiosarcoma en un varón con linfedema crónico / Lymphangiosarcoma in a male with chronic lymphedema

Comunicamos un caso de un paciente de sexo masculino de 54 años de edad, con un linfangiosarcoma símil síndrome de Stewart-Treves post-linfedema crónico, secundario a un traumatismo. El enfermo rechazó la amputación por lo que fue irradiado y luego recibió quimioterapia, interferón y talidomida; no obstante ello, fallece debido a metástasis pulmonares a los cuatro años de efectuado el diagnóstico. Se efectúa una revisión de los angiosarcomas en general, del linfangiosarcoma, como asimismo una nota conceptual sobre los llamados hemangioendoteliomas.

A 54 years-old man with a lymphangiosarcoma, associated to a post traumatic chronic lymphedema Stewart-Treves like, was reported. The patient refused the amputation of the superior left arm so radiation therapy was done. Despite the adjuvant chemotherapy and subcutaneous interferon, together with thalidomide the patient death four years after the diagnosis due to disseminated lung metastasis. A brief review of the cutaneous angiosarcoma and a conceptual nosological position of the so called hemangioendotheliomas, were made.

Linfangiosarcoma en un varón con linfedema crónico / Lymphangiosarcoma in a male with chronic lymphedema

Comunicamos un caso de un paciente de sexo masculino de 54 años de edad, con un linfangiosarcoma símil síndrome de Stewart-Treves post-linfedema crónico, secundario a un traumatismo. El enfermo rechazó la amputación por lo que fue irradiado y luego recibió quimioterapia, interferón y talidomida; no obstante ello, fallece debido a metástasis pulmonares a los cuatro años de efectuado el diagnóstico. Se efectúa una revisión de los angiosarcomas en general, del linfangiosarcoma, como asimismo una nota conceptual sobre los llamados hemangioendoteliomas.(AU)

A 54 years-old man with a lymphangiosarcoma, associated to a post traumatic chronic lymphedema Stewart-Treves like, was reported. The patient refused the amputation of the superior left arm so radiation therapy was done. Despite the adjuvant chemotherapy and subcutaneous interferon, together with thalidomide the patient death four years after the diagnosis due to disseminated lung metastasis. A brief review of the cutaneous angiosarcoma and a conceptual nosological position of the so called hemangioendotheliomas, were made.(AU)

Síndrome de Stewart-Treves: Caso clínico / Stewart-Treves syndrome: Report of one case

Angiosarcomas are malignant tumors derived from the endothelium of blood vessel (hemangiosarcomas) or lymph vessels (lymphangiosarcomas). Lymph edema of the limbs is considered secondary when extrinsic injuries are observed and primary when these injuries are not present. Stewart-Treves syndrome or a Lymphangiosarcoma, developed over a chronic lymph edema, is a rare complication described in mastectomized patients but it can be observed in lymph edemas located elsewhere. It appears as nodular skin lesions that grow, multiply quickly and frequently metastasize. We report a 40-year-old mole with an angiosarcoma associated with primary chronic lower limb lymph edema. The patient consulted for a history of weight loss and malaise and appearance of violaceous lesions over the zone of lymph edema and inguinal lymph node involvement. A CT scan showed bilateral lung lesions and enlargement of inguinal and iliac lymph nodes. A biopsy of one of the skin lesions disclosed an angiosarcoma, diagnosis that was confirmed with immuno-histochemistry Chemotherapy was started but the patient died five months after the diagnosis.