Gingival metastasis of angiosarcoma of the breast as a first manifestation of spreading disease: Case report and review of the literature.

BACKGROUND: Angiosarcoma is an aggressive malignant neoplasm of vascular origin. Oral metastases of angiosarcoma are rare and have a non-specific clinical presentation, thus the diagnosis may be challenging. CASE REPORT: Herein we report a case of a 34-year-old female patient after treatment of a high-grade angiosarcoma of the breast, who presented an asymptomatic bleeding purplish nodule in the maxillary interdental papilla between the first and second premolar. A biopsy was performed, and the histological examination revealed infiltration by malignant neoplasm of epithelioid and fusocellular pattern. Immunohistochemical analysis demonstrated that neoplastic cells were positive for ERG and CD31, and negative for cytokeratins AE1/AE3, confirming the diagnosis of metastatic angiosarcoma. After investigation, multiple metastases were discovered. The patient is under management with chemotherapy and palliative radiotherapy for the bone lesions. CONCLUSION: Metastases should be considered in the differential diagnosis of oral lesions in patients with a previous history of cancer. Due to the morphology of angiosarcomas, the metastatic lesions may resemble benign vascular lesions, therefore, biopsy is mandatory to exclude malignancy.

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.

Angiosarcoma de cavidad nasal: reporte de caso y revisión de literatura / Nasal cavity angiosarcoma: case report and literature review

Los tumores malignos de cavidad nasal y cavidades paranasales son poco frecuentes y sus síntomas de presentación suelen ser unilaterales e inespecíficos; éstos incluyen: epistaxis, rinorrea y obstrucción nasal. Encontramos una amplia variedad histológica de tumores, entre ellos tenemos el angiosarcoma nasal, que es considerado una rareza y el diagnóstico definitivo lo da la confirmación anatomopatológica complementado con estudios inmunohistoquímicos. Su modalidad de tratamiento incluye múltiples opciones, pero la cirugía radical temprana con márgenes negativos asociado a radioterapia adyuvante, ofrece el mejor pronóstico. Se presenta a un paciente adulto masculino con un angiosarcoma nasal izquierdo que fue detectado de forma precoz y se manejó con cirugía radical y radioterapia.

Malignant tumors of the nasal cavity and paranasal cavities are rare, while presenting symptoms are usually unilateral and nonspecific like, epistaxis, rhinorrhea, and nasal obstruction. There are many histological varieties of tumors, among them we have nasal angiosarcoma, which is considered a rarity pathology. The definitive diagnosis is given by biopsy confirmation complemented with immunohistochemical studies. Its treatment modality includes multiple options, but early radical surgery with negative margins associated with adjuvant radiotherapy offers the best prognosis. We present a male adult patient with a left nasal angiosarcoma that was detected early and managed with radical surgery and radiotherapy.

Histopathological case study of canine hemangiosarcoma with multiple organ metastases

We studied a 14-year-old male dog necropsied. Gross findings were hemorrhagic nodules in the spleen, liver, heart and abdominal and thoracic lymph nodes. Histologically, we homogeneously observed tumor cells often with prominent, bulging and mitotic nuclei that were pleomorphic and hyperchromatic, forming small blood vessels. Tumors in all organs were diagnosed as capillary hemangiosarcomas. No tumor cells were detected in the lungs. We presume that the primary tumor was present in the spleen, from where it metastasized multiple organs via lymphatic vessels.(AU)

Tumor microenvironment and its clinicopathological and prognostic associations in surgically resected cutaneous angiosarcoma.

PURPOSE: Cutaneous angiosarcoma (CAS) is a rare but typically aggressive malignant vascular neoplasm of the skin. Tumor microenvironment (TME) of CAS and its associations with baseline clinicopathological features and patient outcomes are very important, especially when considering the recent advances in understanding of the tumor biology. METHODS/PATIENTS: We retrospectively reviewed medical records of patients who underwent surgical resection for CAS at a tertiary Hospital. The pretreated specimens were evaluated by immunohistochemistry for programmed cell death protein 1 (PD-1) and its ligand (PD-L1), densities of tumor infiltrative lymphocytes (TILs) (CD3+, CD4+, CD8+, CD45RO+, FoxP3+), as well as c-MYC and Ki-67 expressions. Overall survival (OS) was estimated by Kaplan-Meier method and compared with Log-rank test. RESULTS: A total of 21 CAS patients were identified. Median age was 67 (ranges: 20-81) years, 14 (66.7%) were male, and over 50% had lesions of scalp. Histopathological examination showed a predominantly spindle cell type (57.1%). All patients underwent surgery, 16 (76.2%) were treated further. PD-L1 was positively stained (> 1%) in tumor cells (42.9%) and TILs (23.8%). PD-1 expression (> 1%) was identified in TILs of 11 (52.4%) cases. PD-1/PD-L1 expressions were significantly associated with the higher densities of CD3+, CD4+, CD8+, CD45RO+, and Foxp3+ TILs, but not with patient characteristics or c-MYC or Ki-67 expression. Median OS was 18.5 months (95% CI 6.0-35.9), although no prognostic significance was observed with respect to any clinicopathological features. CONCLUSION: We characterized TME and its clinical and prognostic association in CAS. PD-1/PD-L1 expressions were significantly associated with TILs subtypes but not with OS.

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.

Hemangiossarcoma na língua de um gato / Hemangiosarcoma in a cats tongue

Background: Hemangiosarcoma is a primary malignant neoplasm of vascular endothelial cells, common in dogs and uncommon in cats. It is characterized by being aggressive and presenting high metastatic behavior. It corresponds to 0.5% to 2% of all feline neoplasms, with more reports in shorthaired cats aged between 8 and 12.2 years old, with no predisposition for sex or breed. Among the diagnostic methods, histopathological and immunohistochemical exams stand out because they are more accurate, since the result of fine-needle aspiration biopsy is usually hidden by blood contamination. This report aims to describe a case of a hemangiosarcoma in a cats tongue. Case: A 9-year-old, female, shorthair cat with a history of weight loss, lethargy, chewing difficulty with the evolution of three months and two-day oral bleeding was attended. Physical examination revealed blood in the oral cavity, enlargement, and darkening of the tongue surface, normalized oral and ocular mucous membranes, estimated dehydration of 7%, a rectal temperature of 38.8OC, and pulmonary and cardiac auscultation without changes. The cat was hospitalized, and euthanasia was elected. Cytological and histopathological exams of the tongue were requested, which revealed a neoplastic proliferation of endothelial cells expanding and replacing the muscular layer of the tongue, forming primitive vascular structures filled with a large number of red blood cells and supported by a delicate fibrovascular stroma. There were moderate anisocytosis and anisocariosis and rare figures of mitosis. Immunohistochemical examination showed the expression of von Willebrand factor, smooth muscle vimentin, and actin in the cytoplasm of neoplastic cells, and negative staining for pancitokeratin. Discussion: According to the results of cytological, histopathological and immunohistochemical…(AU)

Hemangiossarcoma na língua de um gato / Hemangiosarcoma in a cat’s tongue

Background: Hemangiosarcoma is a primary malignant neoplasm of vascular endothelial cells, common in dogs and uncommon in cats. It is characterized by being aggressive and presenting high metastatic behavior. It corresponds to 0.5% to 2% of all feline neoplasms, with more reports in shorthaired cats aged between 8 and 12.2 years old, with no predisposition for sex or breed. Among the diagnostic methods, histopathological and immunohistochemical exams stand out because they are more accurate, since the result of fine-needle aspiration biopsy is usually hidden by blood contamination. This report aims to describe a case of a hemangiosarcoma in a cat’s tongue. Case: A 9-year-old, female, shorthair cat with a history of weight loss, lethargy, chewing difficulty with the evolution of three months and two-day oral bleeding was attended. Physical examination revealed blood in the oral cavity, enlargement, and darkening of the tongue surface, normalized oral and ocular mucous membranes, estimated dehydration of 7%, a rectal temperature of 38.8OC, and pulmonary and cardiac auscultation without changes. The cat was hospitalized, and euthanasia was elected. Cytological and histopathological exams of the tongue were requested, which revealed a neoplastic proliferation of endothelial cells expanding and replacing the muscular layer of the tongue, forming primitive vascular structures filled with a large number of red blood cells and supported by a delicate fibrovascular stroma. There were moderate anisocytosis and anisocariosis and rare figures of mitosis. Immunohistochemical examination showed the expression of von Willebrand factor, smooth muscle vimentin, and actin in the cytoplasm of neoplastic cells, and negative staining for pancitokeratin. Discussion: According to the results of cytological, histopathological and immunohistochemical

Oral masses in African pygmy hedgehogs.

African pygmy hedgehogs (Atelerix albiventris) frequently develop oral neoplasms, and most of these neoplasms are malignant. We characterized oral masses detected in hedgehogs at clinical examination. During a 1-y period, we diagnosed oral cavity masses in 27 privately owned hedgehogs; 16 were female and 11 were male, with ages of 2-7 y (mean: 4.3 y). Eight masses were non-neoplastic and were diagnosed as gingival hyperplasia (GH). Nineteen masses were neoplastic, of which 17 were squamous cell carcinomas (SCCs) and 2 were mesenchymal tumors (1 spindle cell tumor of probable neural origin, and 1 hemangiosarcoma). The GHs were noninvasive, exophytic, and did not recur after surgical excision. The SCCs were highly invasive tumors that induced facial deformation and were located in the caudal portion of the oral cavity, with 12 of them arising from the right-caudal maxilla. Thus, clinical signs, growth pattern, and anatomic location can be used to suspect a diagnosis of SCC among the other possible diagnoses, such as GH, in this location. However, histopathology is necessary for confirmation. Also, hemangiosarcoma should be considered among the differential diagnoses.

Photodynamic therapy for cutaneous hemangiosarcoma in dogs.

Cutaneous hemangiosarcoma is a malignant neoplasia that frequently occurs in dogs. The most effective treatment requires wide surgical excision of the tumor. To avoid mutilating surgeries, photodynamic therapy (PDT) could serve as an alternative treatment. This study aimed to treat cutaneous hemangiosarcomas in dogs using PDT with aluminium-chloride-phthalocyanine nanoemulsion (AlClPc-nano) as photosensitizer. Eight dogs with histopathological diagnosis of naturally occurring cutaneous hemangiosarcoma were treated. Animals were given intra and peritumoral injections of AlClPc-nano (13.3 µM). After 15 min, the masses were LED irradiated at a wavelength of 658-662 nm (80 mW potency) for 25 min (120 J/cm2 fluency). The number of sessions was based on lesion observations, with PDT sessions repeated every 7 days until the mass was no longer macroscopically visible. On that occasion, an excisional biopsy of the area was taken for histopathology analysis. Blood was collected from each animal before each PDT session and excisional biopsy for hematological analysis (blood counts; liver and kidney function). The number of PDT sessions varied from 2 to 4, depending on the size of the initial mass. Seven of the eight cases demonstrated complete remission of neoplasia. Microscopic analysis of the excisional biopsies showed necrosis and hemorrhage only, with no cancer cells, except in one case. During the treatment, inflammation and necrosis were macroscopically observed in the treated areas. The dogs did not show any alteration in blood parameters that could be related to the PDT. In conclusion, PDT with AlClPc-nano is a safe and effective treatment for cutaneous hemangiosarcoma in dogs.

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 renal angiosarcoma with extensive hemorrhage: CT and MRI findings.

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.

Unsuspected Stewart-Treves syndrome clinically mimicked by apparent bullous erysipelas and a systematic review of dermatological presentations of the classical Stewart-Treves syndrome.

BACKGROUND: Stewart Treves-Syndrome (STS) was first characterized as angiosarcoma in the homolateral limb of a patient with breast cancer and lymphedema. Now, other conditions represent STS. It's a rare condition. The diagnosis is easier in the presence of single or multiple purple nodules. Even though other dermatological aspects have been reported, no study has grouped its characteristics. AIM: Evaluate the dermatological characteristics of classical STS (c-STS). METHODS AND RESULTS: We report a patient with chronic lymphedema with a history of recurrent erysipelas that rapidly developed multiple papules in the superior limb. It was initially diagnosed as bullous erysipelas, but unsatisfactory evolution led to biopsy, which demonstrated an unsuspected epithelioid angiosarcoma. We have also performed a review of dermatologic aspects of c-STS using PubMed and Lilacs databases. PICTOS methodology and PRISMA flow chart were considered. The main dermatological aspects associated with c-CTS were summarized. Using a systematic evaluation from 109 articles, 29 were selected and 44 patients were described to whom we added one case. The mean time with lymphedema was 10 years. Of the patients analyzed, 97.2% were female; 95.6% were submitted to radical mastectomy; 81.2% presented with multiple lesions, 67.4% of the lesions were reported as nodules or tumors, 53.4% were purple, 33.4% were associated with an ecchymotic halo, and 33.4% were ulcerated lesions. CONCLUSION: When evaluating patients with chronic lymphedema with new dermatological abnormalities, clinical suspicion, or unfavorable evolution, the knowledge of clinical signs is important for diagnosis, and a biopsy must be considered. Papules associated with erythematous-wine color and bluish hematoma aspect must raise clinical suspicion.

Malignant Vascular Tumors of the Liver in Adults.

Hepatic angiosarcoma and epithelioid hemangioendothelioma (EHE) might be clinically considered a spectrum since, although more frequently presenting indolent behavior, EHE occasionally evolves to high-grade neoplasms. However, in most circumstances, pathological and immunohistochemical patterns define this differential diagnosis. More recently, molecular pathways for angiosarcoma and for EHE from other organs and from soft tissue have been proved different, paving the way for future morpho-molecular assessment of their hepatic counterpart. The frequency of liver involvement by Kaposi sarcoma in HIV-infected patients is lower nowadays. Histological findings and immunostaining for HHV-8 Ag are characteristic. Hepatic small vessel neoplasms have been recently recognized as important mimickers of angiosarcoma. The criteria for this differential diagnosis and the clinical behavior, up to now considered favorable, must be further studied.

Primary Cardiac Angiosarcoma with Rare Presenting Feature and Successful Surgical Treatment.

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.

Soft tissue angiosarcoma of the breast in a patient with Klippel-Trenaunay-Weber syndrome.

Angiosarcomas are neoplasms of blood or lymphatic vessels with aggressive behavior. We report the coexistence of this malignancy within soft tissue of the breast in a 49-year-old woman who was diagnosed with Klippel-Trenaunay-Weber syndrome (KTW-S) during childhood. The patient has no previous history of radiation therapy on the chest and does not have any known risk factor for developing angiosarcoma, except for her congenital disease. To the best of our knowledge, the association between soft tissue angiosarcoma of the breast and KTW-S has never been previously reported.

Los angiosarcomas son neoplasias de los vasos sanguíneos y linfáticos con comportamiento agresivo. Reportamos la coexistencia de esta malignidad dentro del tejido blando de la mama en una mujer de 49 años que fue diagnosticada de síndrome de Klippel-Trenaunay-Weber desde la infancia. La paciente no tiene antecedentes de radioterapia en el tórax ni factores de riesgo conocidos para desarrollar angiosarcoma, a excepción de su enfermedad congénita. A nuestro saber, la asociación entre el angiosarcoma del tejido blando de la mama y el síndrome de Klippel-Trenaunay-Weber no había sido reportada previamente.

A rare case of primary breast angiosarcoma in a male: a case report.

BACKGROUND: Sarcomas account for less than 1% of primary breast cancers, and breast angiosarcomas are responsible for only 0.05% of all breast malignancies. The male breast has the same potential for malignant transformation as the female breast. However, due to anatomical differences in the breast and the low incidence of angiosarcoma, it is difficult to determine how male breasts can be affected by this type of tumor. CASE PRESENTATION: A 36-year-old male patient was admitted to the hospital with a palpable lump in his right breast. Lymphadenopathy was negative. Ultrasonography showed a hypoechoic mass with partially defined contours, measuring 4.0 × 3.0 cm, with muscle infiltration. Histological examination revealed a malignant tumor. Radical mastectomy was then performed with clear surgical margins. The patient began chemotherapy with paclitaxel. Following the second cycle of chemotherapy, he presented with headache and seizures due to a frontal lobe metastasis. Twenty days after the onset of neurological symptoms, the patient died. CONCLUSIONS: Primary angiosarcomas of the male breast are extremely rare. This is the sixth case published in the literature. It is in agreement with other studies in the literature concerning clinical presentation and poor prognosis. Treatment consists in surgical removal of the tumor with clear margins and without axillary lymphadenectomy.

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.