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.

Primary renal angiosarcoma with extensive hemorrhage: CT and MRI findings

ABSTRACT Primary angiosarcomas of the kidney are very rare, but highly aggressive tumors showing poor prognosis. Patients frequently complain of flank pain, hematuria, or a palpable mass. We present a case of primary renal angiosarcoma occurring in a 61-year-old man. CT images depicted a huge exophytic mass (16 cm in diameter) in the right kidney, exhibiting extensive hemorrhage. The mass showed centripetal peripheral nodular enhancement on dynamic contrast-enhanced images. Furthermore, MR imaging revealed a tangled mesh of tumor vessels in the periphery of the mass. We suggest its inclusion in the differential diagnosis of cases of hemorrhagic renal tumors with prominent vasculature.

Primary cardiac angiosarcoma with rare presenting feature and successful surgical treatment

Abstract Primary angiosarcoma is a rare clinical entity, it's typically located within the right atrium and known to be rapidly fatal. A 37-year-old female was presented with a history of recurrent facial paralysis and left hemiparesis. A cranial mass was identified at cranial magnetic resonance imaging and she underwent neurosurgery operation. The immunohistochemical examination was determined as metastatic cardiac angiosarcoma. The tumor, as well as part of the right pericardium, were resected. A piece of bovine pericardium was used to reconstruct the right atrial wall. Tricuspid valve was reconstructed with ring annuloplasty. Due to resection of right coronary artery with the tumor, coronary bypass surgery was performed successfully. The patient is currently healthful without any recurrence and complaint 12 months after the diagnosis as followed up.

Angiosarcoma cutáneo en la región nasal tratado con radioterapia: reporte de caso / Cutaneous Angiosarcoma Nasal Region treated with Radiation Therapy: Case Report

El angiosarcoma cutáneo es un tumor poco frecuente con tendencia a localizarse en la cabeza y el cuello, en especial en el cuero cabelludo y la cara. Es considerado un tumor de rápido crecimiento y frecuentemente hace metástasis a distancia. Su tratamiento no está claramente definido y es basado en estudios retrospectivos y reportes de caso. Al parecer, la mejor opción de manejo es la cirugía con resección local amplia y, en lo posible, con bordes negativos; posteriormente, radioterapia adyuvante, debido a la alta tasa de recaída que presentan estos tumores. Se presenta el caso de un paciente de 91 años quien recibió manejo exclusivo con radioterapia hipofraccionada, quien presentó respuesta completa a los tres meses de finalizar y permaneció libre de enfermedad hasta la fecha de este reporte, ló meses después de la radioterapia.

Cutaneous angiosarcoma is a rare tumor with a tendency to be located in the head and neck especiallv on the scalp and face. It is considered a rapidly growing tumor and often causes distant metastases. The treatment of this tumor is not clearly defined and is based on retrospective studies and case reports, still seems the best option handling surgery with wide local excisión and possibly with negative margins and subsequently adjuvant radiotherapy due to the high rate relapse presented by these tumors. We report the case of a patient of 91 years who received exclusive management with radiotherapy hypofractionated presenting complete response at 3 months after completion and remaining free of disease until the date of this report, 16 months after radiotherapy.

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.

Angiosarcoma in HIV-negative patients is not associated with HHV-8

Abstract: BACKGROUND: Angiosarcoma is an aggressive, malignant neoplasm of vascular or lymphatic origin. Herpes virus 8 (HHV-8) is a member of the herpes family with a tropism for endothelial cells and it has been proven to induce vascular neoplasms, such as Kaposi's sarcoma. The role of HHV-8 in the pathogenesis of angiosarcoma has not been well defined. OBJECTIVE: To investigate the relationship between the presence of HHV-8 and angiosarcoma. METHODS: In this study, the team investigated the relationship between the presence of HHV-8, as determined by polymerase chain reaction, and angiosarcoma, using samples from patients with epidemic Kaposi's sarcoma as controls. RESULTS: While all control cases with epidemic Kaposi's sarcoma were positive for HHV-8, none of the angiosarcoma cases was. CONCLUSION: These findings support most previous studies that found no association between HHV-8 and angiosarcoma.

Metástasis ovárica única de angiosarcoma mamario como localización infrecuente / Single ovarian metastases of breast angiosarcoma as unusual location

Los angiosarcomas son tumores vasculares extremadamente raros con alta tasa de proliferación e invasión. Están caracterizados por células anaplásicas que se originan en las paredes de los vasos sanguíneos. Poseen mal pronóstico debido a su comportamiento altamente agresivo, el cual condiciona una elevada probabilidad de recurrencia local y extensión multiorgánica. Se presentan principalmente en: pulmón, hígado, ganglios linfáticos y huesos. Su tratamiento es controvertido debido escasa casuística disponible acerca de esta estirpe tumoral, aunque parece claro que el tratamiento principal es la cirugía y que el tratamiento posterior puede ser la quimioterapia adyuvante(AU)

Angiosarcomas are extremely rare vascular tumors with high rate of proliferation and invasion. They are characterized by anaplastic cells originating in the walls of blood vessels. They have poor prognosis because of their highly aggressive behavior, which determines a high probability of local recurrence and spreading to multiple organs. They occur mainly in lung, liver, lymph nodes and bones. Treatment is controversial due to small number of cases available about this tumor type, although it seems clear that the main treatment is surgery and subsequent treatment can be adjuvant chemotherapy(AU)

Angiosarcoma of the lung / Angiossarcoma pulmonar

Angiosarcoma is a rare malignant vascular tumor. Pulmonary involvement is usually attributable to metastasis from other primary sites, primary pulmonary angiosarcoma therefore being quite uncommon. We report a case of angiosarcoma with pulmonary involvement, probably primary to the lung, which had gone untreated for more than two years. We describe this rare neoplasm and its growth, as well as the extensive local invasion and hematogenous metastasis at presentation. We also discuss its poor prognosis.

O angiosarcoma é um tumor vascular maligno. O envolvimento pulmonar é geralmente atribuído à metástase de outros sítios primários, sendo o angiossarcoma pulmonar primário extremamente raro. Relatamos um caso de angiossarcoma com envolvimento pulmonar, provavelmente primário no pulmão com mais de dois anos de evolução. Descrevemos seu crescimento e sua extensa invasão local e hematogênica na apresentação. Documentamos ainda seu mau prognóstico.

Stewart-Treves Syndrome of the Lower Extremity

Stewart-Treves syndrome is a rare cutaneous angiosarcoma that develops in long-standing chronic lymphedema. Though most commonly this angiosarcoma is a result of post mastectomy lymphoedema, it also develops in Milroy disease, idiopathic, congenital, traumatic and filarial lymphoedema. Despite the rarity of this syndrome and its poor prognosis, early diagnosis associated with radical surgery can provide improved survival. We report a case of angiosarcoma in the lower limb in a patient with chronic lymphedema associated with history of repeated erysipela episodes.

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.

Angiosarcoma in previously irradiated breast in patient with Li-Fraumeni syndrome. A case report / Angiossarcoma em mama previamente irradiada em paciente portadora da síndrome de Li-Fraumeni. Um relato de caso

CONTEXT: Li-Fraumeni syndrome is a rare disease with an autosomal dominant inheritance pattern and high penetrance that defines a 50% chance of developing cancer before the age of 30 years, including cases of breast sarcoma. Patients with this syndrome who require radiotherapy have an increased risk of developing secondary malignancies including angiosarcomas. CASE REPORT: This was a case report on a female patient with Li-Fraumeni syndrome. In October 2005, she was diagnosed with invasive ductal carcinoma of the right breast and underwent sectorectomy. She then received chemotherapy and adjuvant radiotherapy. Trastuzumab and tamoxifen were also part of the treatment. She recently sought care at our hospital, complaining of hyperemia and nodulation in the right breast, and underwent surgical resection that revealed epithelioid angiosarcoma. CONCLUSIONS: When genetic predisposition due to Li-Fraumeni syndrome is documented, the therapy should be adapted so as to minimize the risk. Thus, conservative surgical treatments should be avoided and mastectomy without radiation should be prioritized. In cases in which use of radiotherapy is justified, patients should be followed up intensively. .

CONTEXTO: A síndrome de Li-Fraumeni é doença rara que apresenta padrão de herança autossômica dominante e alta penetrância, definindo possibilidade de 50% no desenvolvimento de neoplasias antes dos 30 anos, incluindo nesses casos os sarcomas em mama. Pacientes portadoras dessa síndrome que requerem tratamento radioterápico têm risco aumentado de desenvolver neoplasias secundárias, incluindo os angiossarcomas. RELATO DE CASO: Este é um relato de caso de paciente feminina, portadora da síndrome de Li-Fraumeni. Em outubro de 2005, ela teve diagnóstico de carcinoma ductal invasor da mama direita, sendo submetida à setorectomia. Recebeu quimioterapia e radioterapia adjuvante; trastuzumabe e tamoxifeno também fizeram parte do tratamento. Recentemente, procurou atendimento em nosso serviço, com queixa de hiperemia e nodulação em mama direita, e foi submetida a ressecção cirúrgica que revelou angiossarcoma epitelioide. CONCLUSÕES: Quando a predisposição genética da síndrome de Li-Fraumeni está documentada, devese adequar a terapêutica a fim de minimizar riscos, evitando tratamentos cirúrgicos conservadores e priorizando a mastectomia sem radioterapia. Nos casos em que se justifica o uso de radioterapia, os pacientes devem ser acompanhados de forma intensiva. .

Angiossarcoma pouco diferenciado primário de glândula salivar parótida: relato de caso / Primary poorly differentiated angiosarcoma of the parotid gland

As neoplasias mesenquimais primárias das glândulas salivares são raras. Os autores descrevem um caso de angiossarcoma primário da glândula parótida em um homem com 84 anos (AU)

Primary mesenchymal neoplasms of the salivary glands are rare. The authors report a case of primary angiosarcoma of the parotid gland in a 84-year old male (AU)

Angiosarcoma primario de mama y carcinoma papilar de tiroides sincrónico: presentación de un caso / Primary angiosarcoma of breast and synchronous thyroid papillary carcinoma: a case report

Antecedentes: El angiosarcoma primario de mama constituye un tumor infrecuente que se desarrolla de forma predominante en mujeres en la tercera y cuarta décadas de la vida. Los nódulos tiroideos incidentales son aquellos que se objetivan en pruebas de imagen realizadas por otra causa y se observan en menos de un 10 por ciento con la tomografía por emisión de positrones (PET). Objetivo: Presentar el caso excepcional de una paciente con angiosarcoma primario de mama y un carcinoma papilar de tiroides sincrónicos. Caso clínico: Mujer de 34 años con angiosarcoma primario en la mama derecha sometida a mastectomía simple. En el estudio de extensión se halló un incidentaloma tiroideo derecho compatible con carcinoma papilar que requirió tiroidectomía total y linfadenectomía del compartimento central cervical en un segundo tiempo. Comentarlo: Los angiosarcomas primarios suponen menos del 0,05 por ciento de todos los tumores primarios malignos de la mama. El diagnóstico definitivo de estos tumores viene definido por el estudio anatomopatológico, que establece tres grados: alto, bajo e intermedio, los cuales se relacionan de forma directa con la supervivencia. La prevalencia de los incidentalomas malignos tiroideos objetivados por PET varía del 14-30,9 por ciento, según diferentes estudios. La planificación del tratamiento quirúrgico de las neoplasias incidentales tiroideas en pacientes con otro tumor primario debe analizarse de forma individualizada, según la supervivencia esperada del tumor primario.

Background: The primary angiosarcoma of breast is a rare tumor that develops predominantly in women in the third and fourth decades of life. Incidental thyroid nodules are observed in imaging tests performed for other reasons and it's observed in less than 10 percent with the positron emission tomography (PET). Aims: To report the exceptional case of a patient with primary angiosarcoma of the breast and synchronous thyroid papillary carcinoma. Clinical case: Female patient 34 year old with primary angiosarcoma in the right breast, submitted to a simple mastectomy. In the extension study we found an incidental thyroid papillary carcinoma that required a total thyroidectomy and central cervical lymphadenectomy in a second time. Comments: The primary angiosarcoma are less than 0.05 percent of all primary malignant tumors of the breast. The definitive diagnosis is determined by the pathology, which defines three grades: high, low and intermediate, which relate directly to survival. Prevalence of malignant thyroid incidentalomas observed by PET varies from 14 to 30.9 percent, according different studies. Planning the surgical treatment of incidental thyroid malignancies in patients with other primary tumor should be analyzed individually, according the expected survival of the primary tumor.

Clinical and pathological aspects of multicentric hemangiosarcoma in a Pinscher dog / Sinais neurológicos multifocais devido ao hemangiossarcoma multicêntrico em um cão Pinscher: aspectos clínicos e patológicos

A 3-year-old female Pinscher dog was presented with a history of lameness and pain in hind limbs for one week which acutely progressed to non-ambulatory paraparesis. The dog had been apathetic and anorexic during the six days prior to presentation. The neurological findings were compatible with upper motor neuron signs to the left hind limb due to a spinal cord compressive mass on the twelfth thoracic vertebral body. On the other hand, signs of lower motor neurons to the right hind limb were due to a mass with a pathological fracture in the right proximal femur, compressing the sciatic nerve. A histopathological analysis of all organs revealedthe proliferation of endothelial cells showingin filtrative growth and organization into vascular structures with a solid pattern. In addition, immunohistochemycal analysis revealed low proliferation index and citoplasmatic positivity for CD-31, confirming the vascular nature of the neoplasm. Clinical, histopathological and immunophenotipical findings were consistent with hemangiosarcoma.

Uma cadela Pinscher de três anos de idade foi atendida em Hospital Veterinário Escola com histórico de dor nos membros pélvicos com progressão aguda para paraparesia não ambulatória. O animal estava apático e anoréxico há seis dias. Os achados neurológicos foram compatíveis com sinais de lesão de neurônios motores superiores para o membro pélvico esquerdo devido a uma massa no 12º corpo vertebral comprimindo a medula espinhal. Por outro lado, foram observados sinais de lesão de neurônios motores inferiores para o membro pélvico direito devido a uma fratura patológica no colo proximal do fêmur direito que comprimiu o nervo isquiático. Análises histopatológicas de todos os órgãos revelaram proliferação de células endoteliais com crescimento infiltrativo e organização sólida em estruturas vasculares. Adicionalmente, a análise imuno-histoquímica revelou baixa proliferação e marcação citoplasmática positiva para CD-31, confirmando a natureza vascular da neoplasia. Os achados clínicos, histopatológicos e imuno-histoquímicos foram consistentes com hemangiossarcoma.

Angiosarcoma de mama: a propósito de un caso / Angiosarcoma of the breast: apropos of one case

El angiosarcoma de mama constituye una neoplasia vascular maligna muy poco frecuente en la mama, con una incidencia inferior al 0,05 por ciento de entre todos los tumores primarios que asientan en la mama. Teniendo en cuenta que su edad de aparición es a partir de la tercera-cuarta década de la vida, resulta preocupante la aparición de casos a edades cada vez más tempranas. Se presenta el caso clínico de una paciente de 25 años de edad con antecedente de fibroma mamario benigno desde los 17 años que degenera en un angiosarcoma mamario...

Breast Angiosarcoma is a malignant vascular neoplasm rare in the breast with an incidence of less than 0.05 percent of all primary tumors in the breast that seat. Given its age of onset is from the third to fourth decade of life, it is disturbing to the occurrence of increasingly early ages. We describe the case of a patient 25 years old with a history of benign breast fibroid from the 17 years that degenerates into a breast angiosarcoma...

Angiosarcoma primario uterino: revisión de la literatura y presentación de caso / Primary uterine angiosarcoma: clinical case and literature review

Antecedentes: Los angiosarcomas son infrecuentes y corresponden a menos del 1 por ciento de tumores de tejidos blandos, pueden presentarse en cualquier localización fundamentalmente en la piel. El angiosarcoma primario uterino fue por primera vez reportado en 1902 por Silberberg con informes ulteriores esporádicos que suman un total de 33 casos incluyendo el presente. Objetivo: Exponer un caso diagnosticado en el Departamento de Patología de la Universidad Industrial de Santander (Bucaramanga-Colombia), y revisar la literatura de los casos previamente descritos. Caso clínico: Paciente de 25 años, con 2 años de evolución de menometrorragias y dolor pélvico crónico. Se realiza ablación endometrial encontrándose tumor maligno de endometrio pobremente diferenciado, infiltrante, el cual no es posible categorizar. Se efectúa una histerectomía ampliada, salpingooforectomía bilateral, linfadenectomía pélvica y omentectomía. El examen microscópico reveló lesión tumoral pobremente diferenciada compatible con un angiosarcoma uterino de alto grado.

Background: Angiosarcomas are rare and account for less than 1 percent of soft tissue tumors can occur at any location primarily in the skin. The primary uterine angiosarcoma was first reported in 1902 by Silberberg with sporadic subsequent reports, with a total of 33 cases including the present. Objective: To present a case diagnosed in the Department of Pathology, Universidad Industrial de Santander (Bucaramanga, Colombia) and review the literature of the cases previously described. Case report: A 25 years old woman, with 2 years of chronic pelvic pain and menometrorrhagia. Endometrial ablation is performed and an endometrial malignancy was found, poorly differentiated and infiltrating, which is not possible to categorize. It makes an extended hysterectomy, bilateral salpingo-oophorectomy, pelvic lymphadenectomy, and omentectomy. Microscopic examination revealed poorly differentiated tumor, compatible with a high-grade uterine angiosarcoma.