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.

Secondary breast angiosarcoma: A multicentre retrospective survey by the national Italian association of Breast Surgeons (ANISC).

BACKGROUND: Breast angiosarcoma is a malignant mesenchymal neoplasm, which accounts for approximately 2% of all soft tissue sarcomas. Secondary breast angiosarcoma (SBA) may be related to chronic lymphedema after a mastectomy with lymph node dissection (Stewart Treves syndrome) and previous radiotherapy for complications from breast radiation treatment. It is a very rare condition; therefore, diagnosis and management are still a challenge. METHODS: The ANISC collected SBA data by means of a survey sent to all Italian breast centres in the ANISC. The clinicopathological characteristics and the management of this disease were analysed. RESULTS: Twenty-four centres participated in this survey in which 112 cases of SBA were analysed. The median age of the women with SBA was 68.9 years and it appeared approximately 90 months after the first irradiation for breast cancer. In 92% of cases, a mastectomy was performed without axillary dissection for those patients having a high grade of SBA (74.2%). The prognosis was worse in the high-grade cases (overall survival-OS: 36 months) as compared with the low-grade cases (OS: 48 months). After a follow-up of 5 years, 50.5% of the patients were still alive. Disease-free survival (DFS) was 35 months, and there were no differences between the groups of patients with either high- or low-grade histology. CONCLUSIONS: Secondary breast angiosarcoma is a very aggressive disease associated with a short survival outcome. The surgical approach still remains an important step in the course of treatment; furthermore, an accurate histological examination is helpful in establishing the prognosis of the patient. A mastectomy is mandatory. A longer OS was observed in patients with low-grade angiosarcoma as compared to high-grade angiosarcoma (C.I. 40-57 vs. 31-41 months).

Stewart-Treves Syndrome on the Lower Extremity Associated to Idiopathic Chronic Lymphedema Visualized on FDG PET/CT.

Angiosarcomas are highly malignant and rare tumors of vascular or lymphatic endothelial cell origin with a poor prognosis. Lymphangiosarcoma associated with chronic lymphedema is known as Stewart-Treves syndrome. Stewart-Treves syndrome is primarily described in patients with lymphedema of an upper extremity occurring after breast cancer surgery including radical axillary lymph node dissection and subsequent radiotherapy. It is rarely described in the presence of idiopathic chronic lymphedema of the lower extremities. We present a case of lymphangiosarcoma visualized on F-FDG PET/CT, where Stewart-Treves syndrome is secondary to probably a combination of idiopathic chronic lymphedema of the lower extremities and systemic immunosuppressive treatment.

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.

Transformación maligna de linfedema crónico en miembro inferior en linfangiosarcoma / Malignant transformation of lower limb chronic lymphodema into lymphangiosarcoma

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(18)F-FDG PET/CT in a rare case of Stewart-Treves syndrome: future implications and diagnostic considerations.

BACKGROUND: The aim of this article is to illustrate the possible applications of (18)F-fluorodeoxyglucose positron emission tomography/computer tomography ((18)F-FDG PET/CT) in chronic extremity lymphedema and its complications. METHODS AND RESULTS: (18)F-FDG PET/CT findings in a rare case of Stewart-Treves Syndrome (STS), angiosarcoma secondary to chronic extremity lymphedema, are presented. Lymphedema of the extremities is a debilitating disease characterized by chronic swelling due to interstitial edema caused by insufficient lymphatic drainage capacity. Progression with skin thickening, subcutaneous fibrosis, and increased adipose tissue volume is common. Chronic inflammation has been suggested as a key pathophysiologic component. STS is a rare complication with a very poor prognosis; however, early diagnosis and radical treatment is associated with increased survival. Thus, accurate pretreatment staging is paramount. (18)F-FDG PET/CT is highly sensitive in detecting increased glucose metabolism as seen in many types of cancer and inflammation. The role of (18)F-FDG PET/CT in the management of lymphedema and its complications has to our knowledge yet to be described. This case documents high (18)F-FDG uptake in STS, but is at the same time an example of the low specificity of this imaging modality. CONCLUSIONS: We suggest that (18)F-FDG PET/CT has the potential to become an important tool in the staging and treatment planning of Stewart-Treves syndrome. Furthermore, (18)F-FDG-accumulation may be a sensitive tool in detecting low grade inflammation in the skin and subcutis, which has been suggested to cause tissue remodeling in lymphedema progression. However, further studies are needed to elucidate this theory.

Lymphangiosarcoma complicating extensive congenital mixed vascular malformations.

Pediatric hepatic angiosarcoma is a very rare malignant vascular tumor. A few cases have shown pediatric hepatic angiosarcoma occurring on a background of preexisting vascular lesions. We report the case of a newborn girl who presented extensive limbs and upper trunk cutaneous mixed vascular malformations at birth. These malformations were associated with thrombocytopenia. Cutaneous biopsies revealed complex vascular malformations with a significant lymphatic component. Compressive body suit therapy led to regression of the limbs' cutaneous vascular malformations. At the age of 9 months, the patient presented multiple heterogeneous hepatosplenic nodules. Aggressive treatment with prednisone, vincristine, and hepatosplenic embolizations resulted in initial improvement of the hepatosplenic lesions for few months, followed by an increase of the lesions with failure of response to treatment despite adding alpha-interferon-2b to treatment. The patient died at the age of 19 months. The autopsy's pathological examination revealed a hepatic-based angiosarcoma with plurimetastatic dissemination to the spleen, lungs, peritoneum, pleura, mesenteric linings as well as the serosa of the stomach and small intestine. Multiple cutaneous and visceral complex capillaro-lymphatico-venous malformations were also identified. We hypothesize that these multiple extensive mixed vascular malformations were associated with chronic lymphedema which probably predisposed to the development of the angiosarcoma in our patient.

Paresis in an Asian small clawed otter (Aonyx cinereus) associated with vertebral and ischial osteolysis caused by a malignant lymphangiosarcoma.

A 10-yr-old male intact Asian small clawed otter (Aonyx cinerus) was presumptively diagnosed by histopathology and immunohistochemistry with lymphangiosarcoma after bony destruction of the ischium and spinal column from local tumor invasion had caused progressive signs of hind limb lameness and paresis/paralysis, which led to humane euthanasia. At necropsy, the primary tumor was identified as a flocculent mass present under the caudal lumbar vertebrae. Multiple nerves were seen to run from the spinal cord into the wall of the mass. This mass had locally invaded the surrounding muscle, vertebral column, and spinal cord, which led to the clinical signs noted at presentation. Bony destruction was severe with almost complete obliteration of the right ischium and osteolysis of L6, exposing the spinal cord beneath. The tumor had metastasized to at least two different sites within the spleen. The abdominal tumor was confirmed to be of endothelial origin by the use of immunohistochemical staining for factor VIII-related antigen and was confirmed as lymphatic origin versus vascular origin because of the lack of red blood cells within the vessels. The length of time from initial presentation with hind limb lameness to euthanasia because of hind limb paralysis was 4 mo. This is the first report of lymphangiosarcoma, an uncommon malignant neoplasm of lymphatic origin, in a mustelid and the first report of neoplastic disease in an Asian small clawed otter. In addition, the presentation of hind limb paresis associated with bony lysis because of local tumor invasion has not been previously reported with lymphangiosarcoma in humans, domestic animals, or nondomestic animals.

Linfangiosarcoma en paciente mastectomizada (síndrome de Stewart-Treves) / No disponible

El linfangiosarcoma postmastectomía (LPM) es un tumorvascular muy infrecuente y agresivo, que asienta habitualmenteen brazos portadores de un linfedema de larga evolución,tras una mastectomía radical por cáncer. Su incidencia es del0,45% en pacientes que sobreviven 5 años tras la mastectomíaradical. La etiología de estos tumores es aún completamentedesconocida. En la actualidad no existe un tratamientoestandarizado. Las opciones terapéuticas incluyen la exéresisquirúrgica, amputación de la extremidad afectada, desarticulación,radioterapia y quimioterapia. El pronóstico es malo. Lasupervivencia tras el diagnóstico oscila entre 8 y 15 meses.Presentamos el caso de una paciente con linfangiosarcomapostmastectomía radical y radioterapía(AU)

Lymphangiosarcoma postmastectomy is an uncommonvascular tumor, arising in the area of chronically lymphoedematousextremity, after radical mastectomy and radiotherapyin patients with breast cancer. It shows an incidence of 0,45%among patients that survive more than five years after radicalmastectomy. The etiology of this enigmatic tumor is not yetcompletely understood. There is no standard treatment. Thetreatment options include radical ablative surgery, amputation,radiation therapy and chemotherapy. The prognosis is poor.Survival after diagnosis ranged from 8 to 15 months. We reportthe case of patients with LPM and RT(AU)

Linfangiosarcoma sobre un linfedema crónico postmastectomía: síndrome de Stewart-Treves / No disponible

En 1948, Stewart y Treves comunicaron la aparición de unlinfangiosarcoma en el brazo edematoso de 6 pacientes sometidasa mastectomía radical por cáncer de mama. El síndromede Stewart-Treves describe la presentación de un linfangiosarcomao de un hemangiosarcoma sobre un linfedema crónico.Comunicamos el caso de una mujer de 74 años que fue sometidaa mastectomía radical derecha con radioterapia 23 añosantes y que consultó por la aparición de lesiones cutáneas enforma de placas violáceas en el brazo homolateral que llegarona hacerse nodulares. Tras la biopsia, el análisis histopatológicoconfirmó el diagnóstico de linfangiosarcoma. El estudio de extensiónfue negativo para metástasis. Se decidió la amputaciónde la extremidad. Seis meses más tarde, se diagnosticóuna metástasis cerebral y la paciente falleció poco tiempo después.Las opciones terapéuticas en estos casos (amputación,radioterapia y quimioterapia) son muy agresivas y ofrecen pobresresultados. Sólo un diagnóstico muy precoz, basado en lasospecha clínica, puede mejorar el pronóstico de esta complicaciónde la linfadenectomía axilar. La biopsia es obligatoriaante cualquier lesión sospechosa(AU)

In 1948, Stewart and Treves reported a lymphangiosarcomain 6 patient’s edematous arm after radical mastectomy forbreast cancer. Stewart-Treves syndrome describes a cutaneouslymphangiosarcoma or hemangiosarcoma that develops inlong-standing chronic lymphedema. We report a 74-year-oldwoman who was submitted to a right mastectomy and radiotherapy23 years before. She developed a chronic lymphedemain the same limb and fast-growing purplish lesions on thearm. These lesions became nodular. After the biopsy examinationthey were diagnosed as lymphangiosarcoma. The studyfor metastatic extension was negative. It was decided the amputationof the limb. Six months after the treatment, a brainmetastasis was diagnosed and the patient died. The therapeuticpossibilities (amputation, radiotherapy, chemotherapy) arevery aggresive and offer poor results. The physician mustmaintain a high index of suspicion in diagnosing this complicationof lymphadenectomy. If there is any doubt, biopsy is mandatory(AU)

Lymphangiosarcoma in a 3.5-year-old Bullmastiff bitch with vaginal prolapse, primary lymph node fibrosis and other congenital defects.

Lymphangiosarcoma is an extremely rare tumour in dogs with only 16 cases reported in the literature. Lymphoedema, which may be primary due to defects in the lymphatic system, or secondary to various other pathologies, often precedes malignancy. Of the 16 canine reports, only 1 dog was confirmed as having had prior primary lymphoedema due to aplasia of the popliteal lymph nodes. A case of lymphangiosarcoma is described in a 3.5-year-old purebred, Bullmastiff bitch which presented with vaginal blood 'spotting' for 3 weeks after cessation of oestrus, during which intromission by the male had been unsuccessful. During ovariohysterectomy a large multicystic, proliferative, spongy, fluid-filled, brownish-red mass surrounding the cervix and projecting into the abdominal space was removed with the cervix, and a diagnosis of lymphangiosarcoma made on histological and electron microscopic examination of the tissue. Ultrastructurally, no basement membrane or pericytes were found, only some of the neoplastic endothelial cells were linked by tight junctions while there were gaps between others, and neither micropinocytotic vesicles nor Weibel-Palade bodies occurred in the cells examined. Very few of the endothelial cells lining the many interlinking, tortuous maze of channels, stained slightly positive immunohistochemically for factor VIII-related antigen. The channels were filled mostly with serous fluid, and occasionally mixed leucocytes and some erythrocytes. The endothelium was often associated with underlying blocks of collagenous material, as well as loosely-arranged aggregates of lymphocytes, other mononuclear cells and occasional neutrophils in the connective tissue septae and more prominently perivascularly. The bitch was discharged on antibiotic treatment but returned 2 weeks later with apparent prolapsed vagina which failed to reduce over the next week. Laparotomy revealed the tumour to have spread extensively in the caudal abdomen to involve the broad ligament and the ventral rectal serosa, and the 'prolapsed' tissue was found to be expanded vaginal wall. The bitch was euthanased and necropsied, Histological examination confirmed lymphangiosarcomatous invasion of the submucosal and muscular layers of the retroperitoneal, traumatised, prolapsed part of the vagina, the urethra and the ventral rectal wall. The broad ligament was diffusely invaded with tumour which had proliferated into the caudal abdominal space, and 3 small intra-trabecular foci of tumour were found in the right popliteal lymph node near the hilus. Mitotic figures were generally scarce. There was mild subcutaneous oedema of the ventral trunk extending from the axillae to the inner proximal thighs, which had not been evident clinically, and the lymph nodes (peripheral more so than internal) microscopically showed marked trabecular and perivascular fibrosis especially in hilar regions. Other congenital defects were hepatic capsular and central venous fibrosis with lymphatic duplication and dilatation in all areas of connective tissue, ventrally-incongruous half-circular tracheal rings, and multifocal renal dysplasia affecting the right kidney. There was locally-extensive subacute pyelonephritis of the left kidney.

Linfedema postmastectomía / Postmastectomy lymphedema

El linfedema postmastectomía es una complicación de la extirpación de la mama y territorios ganglionares. Su incidencia varía en función de la técnica empleada en la extirpación de la mama y de la aplicación o no de la radioterapia. Ante la presencia del mismo, desde las fases más iniciales, debe diagnosticarse correctamente, diferenciarlo de los edemas de otra etiología y tratarlo como tal. La linfografía isotópica y el eco-Doppler son métodos diagnósticos de elección para el estudio de las lesiones del sistema linfático y la posible afectación del sistema venoso, respectivamente. La linfografía y la flebografía convencionales quedan hoy en día relegadas como técnicas de recurso. El linfedema postmastectomía responde muy bien al tratamiento conservador: fundamentalmente a las Benzopironas (cumarina), vendaje y masaje de drenaje linfático manual. Sólo ante el fracaso del mismo o frente a eventuales complicaciones, -como la linfangitis-, es susceptible de tratamiento quirúrgico: anastomosis linfovenosas. Ante el linfefibredema postmastectomía, la técnica de resección o exéresis se impone como profilaxis de la degeneración neoplásica: linfangiosarcoma de Stewart-Treves

The postmastectomy lymphedema is a complication of breast removal and lymph nodule territory. Its incidence varies in accordance to the technique used in the removal of the breast and the application or not of radiotherapy. Faced with its presence, from the most initial stages, it must be correctly diagnosed, distinguished from oedemas of other etiologies and be treated as such. The isotope lymphography and the eco Doppler are chosen methods for the study of lymphatic system lesions and the possible affectation of the venal system respectively. The conventional lymphography and phlebography are nowadays relegated to being supplementary techniques. The postmastectomy lymphedema responds very well to conservative treatment: basically to the Benzopyrones (coumarins), bandages and massage of manual lymphatic drainage. Only if this fails or faced with eventual complications, - for example lymphangitis-, it is susceptible to surgery: lymphovenous anastomosis. In the case of postmastectomy fibrolymphedema, the resection technique or exeresis prevails as prophylaxis of the neoplastic degeneration: lymphangiosarcoma of STEWART-TREVES

Feline lymphangiosarcoma--definitive identification using a lymphatic vascular marker.

Three cases of feline exudative dermatitis associated with lymphangiosarcoma are described. The animals, an 11-year-old, neutered male and two 10-year-old, neutered female short hair European cats, presented with a 2-month history of transparent liquid oozing from the skin of the groin and caudal abdomen. On physical examination the neutered male cat and one of the females were slightly depressed and showed loss of weight. Skin lesions were similar in all cats and characterized by the presence of alopecia and moist dermatitis in the ventral abdomen, groin and inner thigh. The hair at the periphery appeared matted by the fluid. In all three cases, histopathological examination of skin biopsies from the abdomen identified poorly defined neoplasia involving dermis and subcutis, characterized by proliferation of spindle cells aligned along pre-existing collagen bundles. The dissection of collagen bundles gave rise to irregular shaped anastomosing, often blind-ending vascular channels and trabeculae. Vascular spaces were mostly optically empty. These histological features were strongly suggestive of lymphangiosarcoma. Neoplastic cells were positive for the blood vascular marker Von Willebrand factor, and a lymphatic vascular marker LYVE-1 (Lymphatic Vessel Endothelial receptor - 1), demonstrating the mixed vascular origin of the tumour. Ultrastructural findings confirmed the final diagnosis of lymphangiosarcoma.

Síndrome de stewart-Treves: linfangiosarcoma en linfedema crónico posmastectomía / Stewart-treves syndrome: lymphangiosarcoma in chronic post-mastectomy lymphedema

Introducción. El linfangiosarcoma es un angiosarcoma desarrollado sobre zonas de linfedema crónico, casi siempre posmastectomía (síndrome de Stewart-Treves). Presenta una incidencia del 0,45 per cent, entre los pacientes que sobreviven más de cinco años desde la intervención. Se caracteriza por una aparición lenta de nódulos rojizos o violáceos, duros, con tendencia a la hemorragia espontánea, que crecen y se multiplican con rapidez y producen metástasis pulmonares. Las posibilidades terapéuticas (radioterapia, quimioterapia, amputación) son muy agresivas y proporcionan resultados pobres, con una supervivencia inferior al 5 per cent desde el momento del diagnóstico. Caso clínico. Paciente de 71 años que en 1989 se sometió a una mastectomía derecha con vaciamiento axilar, quimioterapia y radioterapia de los ganglios linfáticos supraclaviculares, axilares y de la pared torácica. Lentamente desarrolló un linfedema en esa extremidad. Diez años después de la intervención, aparecieron unas lesiones violáceas en el brazo que presentaron un crecimiento rápido, con una transformación nodular y tendencia a la hemorragia. Tras la biopsia de las mismas, se llegó al diagnóstico de linfangiosarcoma. El estudio de extensión metastásica fue negativo. Tras evaluar a la paciente en el Servicio de Oncología, se acordó como tratamiento más resolutivo la amputación de la extremidad. La paciente rechazó dicho tratamiento y recibió cuidados paliativos hasta su fallecimiento seis meses después del diagnóstico. Conclusiones. Los tratamientos instaurados no han mejorado la supervivencia a pesar de la agresividad que conllevan: radioterapia, quimioterapia y amputación de la extremidad. (AU)