Case Of Angiosarcoma Research Articles

Primary vascular bone tumors in the spine: a challenge for pathologists and spine oncology surgeons

PurposePrimary vascular bone tumors of the spine represent a challenge for oncology surgeons, due to the need of planning a surgical strategy appropriate to the tumor behavior. But these tumors represent a challenge also for pathologists, as immunohistochemical and molecular analyses have recently refined the terminology.MethodsA cohort of 81 cases was retrospectively reviewed, targeting the evolution of diagnoses and the treatment-related outcome. Sixty-six cases (including one case originally diagnosed as chordoma) were diagnosed before 2011, and 15 were diagnosed and treated in the period 2011–2017. Fully documented outcome studies are available for 46 patients whose immunohistochemical and molecular analyses were available. The follow-up ranges from 6 months of the early patient death to 300 months of the longest disease-free survival.ResultsThe outcome was related not only to the treatment performed, but also to the evolution of diagnoses. The term Hemangioendothelioma that defined a benign aggressive (Enneking stage 3) tumor is now obsolete and replaced by the diagnosis of Epithelioid Hemangioma for benign aggressive tumor and Epithelioid Hemangioendothelioma for low-grade malignant tumor. En bloc resection was appropriate for local control, but 2 out of 7 cases of epithelioid Hemangioendotheliomas died due to the spread of the disease None of the Hemangiomas recurred after intralesional excision with or without radiotherapy, or after vertebroplasty. Both cases of Angiosarcoma had a fast and lethal evolution.ConclusionsSurgeons must be aware of the evolution of terminology to decide the most appropriate treatment options.Graphical abstractThese slides can be retrieved from Electronic Supplementary Material.

Primary Hepatic Angiosarcoma Revealed by a Hemorrhagic Parietal Metastasis about a Case in Lomé

Angiosarcoma is a malignant tumor developed at the expense of endothelial cells. Primary hepatic localization is rare. Chronic toxic exposure is classically described as responsible for the development of this tumor. The diagnosis is based on the pathological examination. The authors report a case of hepatic angiosarcoma in a 28-year-old woman who received for a thoraco-abdominal hemorrhagic parietal secondary lesion. A thoracic abdominal and parietal ultrasound was performed followed by thoraco-abdomino-pelvic CT. The diagnosis was confirmed by histology. Palliative treatment combining surgery and chemotherapy has been established with a poor prognosis.

A case of cutaneous angiosarcoma of the scrotum

A case of cutaneous angiosarcoma of the scrotum

Cutaneous angiosarcoma of the leg

Angiosarcomas are malignant neoplasms arising from endothelial cells. Cutaneous angiosarcoma is the most common form, typically occurring in the setting of chronic lymphedema or previous radiation. The head and neck are the most common locations for cutaneous angiosarcoma, with rare occurrence on the trunk and extremities. Herein, we present a case of angiosarcoma on the lower extremity in an elderly man. This 71-year-old man presented with a two-year history of red-yellow discoloration of the left lower leg with subsequent development of black nodules over the past several months prior to presentation. He denied any itching, bleeding, or ulceration. Past medical history included diabetes, hypertension, and non-melanoma skin cancer. Physical examination showed erythematous, violaceous, non-blanchable papules with yellow atrophic regions and overlying black crusted nodules and plaques. Biopsies were taken and the patient was diagnosed with angiosarcoma and referred to a tertiary care center for further evaluation.

CAMTA-1 Expression in 24 Cases of Hepatic Epithelioid Hemangioendothelioma in a Single Institute: Diagnostic Utility for Differential Diagnosis from Hepatic Angiosarcoma

Epithelioid hemangio-endothelioma (EHE) of the liver is an uncommon vascular tumor with variable clinical courses ranging from stable disease to fatal outcome. EHE can mimic epithelioid angiosarcoma, which has a more aggressive behavior, especially in a small biopsy sample. EHEs are known to have the WWTR1-CAMTA1 fusion gene, and nuclear expression of CAMTA1 by immunohistochemistry (IHC) has been reported in about 90% of EHEs in multiple organs. Our study aimed to validate the diagnostic utility of CAMTA1 expression in EHEs, especially in the liver. IHC was performed using anti-CAMTA1 antibody in 34 tumors (24 hepatic EHEs and 10 angiosarcomas). In CAMTA1-negative EHEs, TFE3 IHC was performed. Of the 24 hepatic EHEs, 22 (91.6%) showed nuclear staining for CAMTA1. One of two CAMTA1-negative cases showed TFE3 positivity. The other case was negative for TFE3. Meanwhile, all 10 angiosarcoma cases had no CAMTA1 expression. CAMTA1 is a highly sensitive and specific marker for diagnosis of hepatic EHE. It is helpful for differentiation of hepatic EHE and angiosarcoma, especially in small biopsy samples.

Angiosarcoma originating in the anterior mediastinum

Rationale:Angiosarcomas are malignant vascular tumors, and angiosarcoma occurring in the anterior mediastinum is rare. Here we report a case of angiosarcoma that originated in the anterior mediastinum treated with surgery, followed by radiotherapy and synchronous chemotherapy.Patient concerns:A 56-year-old female was admitted to our hospital with chest pain for 3 days. Chest computerized tomogram (CT) examination showed a heterogeneous mass in the anterior superior mediastinum, and after injection of contrast agent, the mass showed obvious heterogeneous enhancement. Magnetic resonance imaging (MRI) with T1 weighted image (T1WI) showed isointensity and T2 weighted image (T2WI) showed heterogeneous signal intensity, the mass showed an obvious heterogeneously enhancement after intravenous administration of contrast material.Diagnosis and interventions:Surgical resection operation was carried out. According to its morphologic and immunohistochemic feature of tumor cells which expressing CD31, CD34, and ERG, the tumor was categorized as an angiosarcoma. After operation, the patient received radiotherapy and synchronous chemotherapy.Outcomes:At present, 8 months postoperatively, no signs of recurrence have been observed.Lessons:Although angiosarcoma in anterior mediastinum is rare, when a mass located in this area, a more careful immunohistological analysis should be performed to avoid overlooking the presence of angiosarcoma.

Life experience of 1 angiosarcoma patient with amputation who possesses "strong trait"

Objective By interview with 1 young angiosarcoma and amputated patient who has trait, the resonance of the patients with the same situation was aroused and it is helpful for the clinical nursing staff to give the patient's comprehensive and pertinent guidance. Methods One case of young angiosarcoma and amputation who has trait were interviewed and the interview data were analyzed by the method of narrative study in qualitative study. Results A total of 4 themes were summed up: to be optimistic in the life, rational acceptance the fact of illness; by trait to shape tough will; gratitude and strong pluralistic support; and the right way of life. Conclusions The 4 subjects have a positive impact on improving the quality of life of the young angiosarcoma. At the same time, it can make the clinical nurses actively guide the other young angiosarcoma and set up a correct life attitude so as to get out of the shadow of the disease. Key words: Angiosarcoma; Qualitative research; Life experience

Two cases of radiation-associated angiosarcoma of the breast

BackgroundThe incidence of radiation-associated angiosarcoma (RAA) of the breast has been increasing, and its prognosis is reportedly poor. It is important to remove tumor tissues completely to prevent recurrence.Case presentationWe report two cases of patients with RAA of the breast. Both patients had a nodule in their remaining breast a few years after undergoing breast-conserving surgery and radiation therapy for breast cancer. The nodules were diagnosed as angiosarcoma by skin biopsy and open biopsy, respectively. To determine the extent of lesion spread, mapping biopsy was performed before surgery. Both patients underwent mastectomy, extensive skin resection, and split skin grafting. Pathological findings showed that their tumors could be completely resected. After surgery, chemotherapy was performed.ConclusionIn our cases, no local or distant recurrence has been detected in either patient for over 4 years. We identified the range of tumor invasion by preoperative mapping biopsy and completely resected all tumor tissue.

A case of radiation-associated angiosarcoma after breast cancer

BackgroundRadiation-associated angiosarcoma (RAAS) is a rare subtype of secondary angiosarcoma that is characterized by rapid proliferation and extensive tissue infiltration. Although various treatments for RAAS (such as surgery, chemotherapy, and radiation therapy) have been reported, there is no consensus as to which approach is the best.Case presentationA 76-year-old woman presented with right breast cancer (T1N0M0, stage I) 9 years ago. She had undergone breast-conserving surgery and sentinel lymph node biopsy and was receiving adjuvant chemotherapy and radiation therapy for the malignancy. Six years after presenting with the tumor, she developed pigmented skin and was diagnosed with a RAAS; this angiosarcoma recurred three times within 2 years. The angiosarcoma was resected each of the three times, after which adjuvant radiation therapy was performed. At 76 years old, the patient developed a new mass on her chest skin in the vicinity of the scar. Angiosarcoma was diagnosed following a pathology report, which resulted in a second diagnosis of recurrent RAAS again since the diagnostic criteria were met. After extensive resection of the irradiated area, the patient has remained free of angiosarcoma for the last 3 years.ConclusionResection of the entire irradiated field is critical for successful treatment of RAAS.

TAA8. Aortic Epithelioid Angiosarcoma After Endovascular Treatment of Descending Aortic Ulcer

Aortic epithelioid angiosarcoma is a rare disease. We report a case of epithelioid angiosarcoma of the abdominal aortic wall after endovascular treatment of descending aortic ulcer. A 55-year-old man, diagnosed with descending aortic ulcer through regular health examination, was implanted with a Gore Conformable TAG (W. L. Gore & Associates, Flagstaff, Ariz) at the suprarenal aorta, with celiac trunk covered. During 4-month follow-up, the patient had no complaints, and no graft migration was shown by computed tomography angiography (CTA) examination, yet superior mesenteric artery occlusion was observed. During 10-month follow-up, the patient presented with abdominal back pain, general fatigue, and fever. CTA examination showed abnormal density around the graft, which was assumed to be a graft infection with periaortic tissue compatible with an inflammatory reaction. After invalid treatment for infection, positron emission tomography-computed tomography was performed and showed avid fluorodeoxyglucose uptake at the descending aorta, retroperitoneum, right lung, pancreas, spleen, left kidney, mesenteric glands, and left whirl bone. Computed tomography-guided biopsy confirmed it as epithelioid angiosarcoma. On review of the CTA study, no sign of malignant tumor was found on the preoperative images; however, left renal metastases were seen on the 4-month postoperative images. There may have been an association between angiosarcoma and artificial graft observed in animal experiments or rare cases before. However, this is the first case of epithelioid angiosarcoma after Conformable TAG aortic endograft placement. In addition, the rapid onset of the tumor also confused us as to whether the cause of the descending aortic ulcer was arteriosclerosis or epithelioid angiosarcoma and whether the epithelioid angiosarcoma was primary or secondary to the artificial graft.

Radiotherapy-associated intra-abdominal angiosarcoma after prostatic adenocarcinoma: Case reports.

Angiosarcoma is a rare soft tissue neoplasm, which accounts for <1% of all soft tissue tumours. It has been previously reported that the incidence rate of angiosarcomas increases following radiotherapy. The present study reports two cases of intra-abdominal angiosarcoma associated with previous radiotherapy treatment. To the best of our knowledge, these associations have not been previously described in English literature. The patients aged 71 and 83 years were admitted to the center for abdominal pain and diarrhea. Each patient had previously had treatment with radiotherapy for prostate adenocarcinoma. During their hospitalization, biopsies were obtained and the diagnosis of angiosarcoma was reached. In each patient the tumors had irregular proliferating vascular channels, lined by atypical endothelial cells, which varied from elongated and spindle-shaped to large and plump. Immunocytochemistry revealed that the tumor cells were positive for the cytoplasmic endothelial markers cluster of differentiation (CD)31 and CD34. The recognition of these associations is important and their occurrence in this rare type of neoplasm should not mislead the pathologist and cause a misdiagnosis of the sarcoma.

Angiosarcoma of Breast in Pregnancy: A Rare Case Report

Angiosarcoma of the breast is a rare aggressive malignancy of the endothelial origin with a tendency for local regional recurrence. A frequent clinical presentation is a painful palpable smooth mass or diffuse enlargement of the breast without a palpable mass but with purple discoloration of the overlying skin with apparent bruising. The aim is to give optimal treatment to the mother to maximize the chances of survival, while minimizing the risk of harm of the fetus. Here, we present a rare case of angiosarcoma of breast during pregnancy. Twenty-seven-year-old primigravida at 25 weeks of gestation presented to our hospital with complaints of lump in right breast with rapid increase in size and associated with pain. Ultrasound-guided FNAC showed features of angiosarcoma; hence, right-sided modified radical mastectomy was performed. Histopathological findings and epithelial markers of the removed tumor confirmed as angiosarcoma with no evidence of lymph node involvement. HRCT lungs showed metastasis in right lung middle lobe. In view of metastasis, she was treated with adjuvant chemotherapy before and after delivery.

Diagnostic difficulties of primary angiosarcoma of the breast: a case report

BackgroundAngiosarcoma of the breast is a rare tumor, which may be primary or secondary to breast surgery or irradiation. It is characterized by polymorphic and nonspecific clinical and radiological features. A pathologist plays a key role in positive and differential diagnosis and in establishing the prognosis: only a histological examination can confirm the diagnosis, and the histologic grade is the most important prognostic factor. In fact, angiosarcomas of the breast constitute a very heterogeneous group and they are classified into three grades based on the degree of differentiation. We will illustrate diagnostic challenges through this new case of primary angiosarcoma of the breast. Microscopic findings were initially interpreted as a benign vascular tumor. We will also discuss the relevant medical literature.Case presentationA 56-year-old Arabian woman presented with a palpable right breast mass that had been enlarging for 2 months, measuring 5 cm, without axillary lymphadenopathy. She had no personal or family history of breast surgery or breast irradiation. A mammography showed no evidence of spiculation. No suspicious calcifications were seen. A needle core biopsy was performed. Microscopic findings were initially interpreted as a benign vascular tumor. However, as the mass measured 5 cm, the diagnosis of angiosarcoma was more appropriate, and mastectomy without axillary dissection was performed. Microscopic examination found mild to moderately scattered pleomorphic cells, and scattered mitotic figures. It also showed papillary formations, solid foci of spindle cells, and hemorrhagic necrosis. The margins of the tumor were infiltrative. The diagnosis of primary intermediately differentiated angiosarcoma of the breast (grade II) was made. No distant metastases were found. Our patient was lost to follow-up and further treatment after mastectomy until she developed local tumor progression 4 months later.ConclusionsThrough this case report, we emphasize the importance of clinicopathological confrontation in angiosarcoma of the breast.

A case of angiosarcoma occurring after total hip replacement and review of the literature

A case of angiosarcoma occurring after total hip replacement and review of the literature

Li-Fraumeni Syndrome-related Malignancies Involving the Genitourinary Tract: Review of a Single-institution Experience

ObjectiveTo report a case of pelvic angiosarcoma in a 27-year-old man with Li-Fraumeni Syndrome (LFS) and evaluate the presentation and timeline of genitourinary (GU) tract involvement in LFS patients. MethodsWe retrospectively identified 39 LFS patients treated at our institution between 2000 and 2014; 7 (18%) had experienced a GU malignancy or an LFS-related malignancy involving the GU tract. Clinical characteristics, including dates of onset of first GU tract malignancies; pathologic findings; multimodal management; and familial history of LFS were reviewed. ResultsMedian age at first malignancy was 14.0 years (interquartile range [IQR] 5.5-24.0). There was a slight male predominance (4 of 7). Median time between first malignancy and the malignancy involving the GU tract was 10.1 years (IQR 8.0-19.5). Six of the 7 patients (86%) had a form of sarcoma involving the GU tract; 1 developed adrenocortical carcinoma. The cancer pedigree of all patients showed LFS-associated malignancies in family members. Multimodal management included surgical resection in 6 patients with adjuvant chemotherapy or radiotherapy in 1 patient each. One patient received chemotherapy only. Following diagnosis of malignancy involving the GU tract, 5 of the 7 patients developed additional primary malignancies. At a median follow-up of 4.7 years (IQR 3.0-12.1), 2 patients are alive, 3 died of disease, and 1 died of unknown cause. One patient was lost at follow-up. ConclusionContinued follow-up of LFS cancer patients aimed at the determination of optimal screening, management, and surveillance protocols is recommended and may result in longer survival expectations.

Effectiveness of Vascular Markers (Immunohistochemical Stains) in Soft Tissue Sarcomas.

To ascertain the effectiveness of IHC markers of vascular origin like CD31, CD34, FLI1 and ERG in vascular soft tissue sarcomas including angiosarcomas, Kaposi sarcomas, epithelioid hemangioendothelioma and a non-vascular soft tissue sarcoma (Epithelioid sarcoma). Descriptive study. Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore, from 2011 to 2017. Diagnosed cases of angiosarcomas (n=48), epithelioid hemangioendothelioma (n=9), Kaposi sarcoma (n=9) and epithelioid sarcoma (n=20) were selected. Immunohistochemical staining as performed on formalin fixed paraffin embedded sections. The sections were stained for the following markers: CD34 (VENTANA clone Q Bend 10), CD31 (Leica clone 1 A 10), FLI1 (CELL MARQUE clone MRQ-1) and ERG (CELL MARQUE clone EP111). A complete panel of CD34, CD31 and ERG was applied on 8/48 cases of angiosarcomas with triple positivity in 6 cases. Eight cases showed positivity for only CD31 and ERG and 2 cases showed positivity for only ERG. A complete panel of CD34, CD31 and ERG was applied on 3/9 cases of epithelioid hemangioendothelioma with positivity for all markers in 2 cases. Combined positivity for ERG and CD34 was seen in 2 cases and on 4 cases only CD31 immunohistochemical was solely applied with 100% positivity. FLI1 was not applied on any case. Among 9 cases of Kaposi sarcoma, ERG, CD34 and CD31 in combination were applied on only 1 case with triple positivity. Remaining cases show positivity for either CD34, CD31 or FLI1. Majority of cases of epithelioid sarcomas were diagnosed on the basis of cytokeratin and CD34 positivity with loss of INI1. The other vascular markers showed negativity in all cases. Among these four markers, ERG immunohistochemical stain is highly effective for endothelial differentiation due to its specific nuclear staining pattern in normal blood vessel endothelial cells (internal control) as well as neoplastic cells of vascular tumors and lack of background staining.

A case of angiosarcoma of the nasal cavity

&lt;p class="abstract"&gt;Angiosarcoma of the nasal cavity is extremely uncommon accounting for less than 0.1% of all sinonasal malignancies. Here we report a case of angiosarcoma of nasal cavity. A 45 year old male patient with c/o right sided nasal bleeding since 20days, on examination showed mass in nasal cavity. Patient underwent septoplasty with right inferior turbinectomy with submucosal diathermy. The histological examination showed features of angiosarcoma epitheloid variation of nasal cavity, diagnosis was confirmed by immunohistological stain with factor CD 31 and cytokeratin.&lt;/p&gt;

Distinguishing papillary endothelial hyperplasia and angiosarcoma on core needle biopsy of the breast: The importance of clinical and radiologic correlation.

Papillary endothelial hyperplasia (PEH) is a rare non-neoplastic exuberant organizing hematoma that can closely mimic angiosarcoma due to a resemblance to malignant anastomosing blood vessels. It could be particularly difficult to distinguish PEH from angiosarcoma in breast core needle biopsies. We identified all cases of these lesions diagnosed on core needle biopsy in order to identify clinical, radiologic, and pathologic features that could prove helpful to arrive at the correct diagnosis. Four cases of PEH and 4 cases of angiosarcoma were identified. The mean age at diagnosis was 62 for PEH and 33 for primary angiosarcoma. All cases of PEH formed small masses with circumscribed or lobulated margins by imaging (mean size 0.9cm). In 3 cases, the masses were difficult or impossible to identify after the biopsy. Angiosarcomas presented as larger masses with ill-defined margins (mean size 2.8cm) that were unchanged in size after biopsy. PEH was surrounded by adipose tissue, whereas angiosarcoma invaded into fibrous stroma and involved lobules. The pseudopapillary structures of PEH were composed mainly of collagen, and thus, additional histologic stains for fibrin were not helpful for diagnosis. The 4 patients with PEH received no further treatment and are alive and disease-free at 2-11years of follow-up. In contrast, the patients with angiosarcoma underwent mastectomy and chemotherapy or radiation therapy. Two of the patients with angiosarcoma died 3years after diagnosis and the other 2 patients are alive without disease at 5 and 6years. Therefore, distinguishing PEH and angiosarcoma is essential for appropriate management. This is the first series to compare these lesions on core needle biopsy and the first to note important clinical, imaging, and histologic differences that aid in making a diagnosis of PEH with confidence on breast core needle biopsy.

Primary angiosarcoma of the breast-series of 11 consecutive cases-a single-centre experience.

Angiosarcomas of the breast are rare parenchymal malignancies of the chest wall. Surgery is the main treatment modality with chemotherapy and radiotherapy used in case of recurrence. With generally unfavourable prognosis and lack of clear treatment guidelines due to its rarity and scarcity of available data, angiosarcoma of the breast is a challenging clinical situation for both oncologist and patient. We present here the results of a series of 11 consecutive primary angiosarcoma cases treated at our institute between 2000 and 2015.

Epithelioid angiosarcoma of bone and soft tissue: a report of seven cases with emphasis on morphologic diversity, immunohistochemical features and clinical outcome.

Epithelioid angiosarcoma is a rare histopathologic variant of angiosarcoma characterized by an epithelioid morphology. This subset can histologically mimic non-vascular neoplasms and impose serious challenges in reaching a correct diagnosis, especially in the context of limited tissue sampling (e.g., needle core biopsy). To improve recognition of epithelioid angiosarcoma - and the spectrum of morphologic diversity associated with this rare variant - and to avoid a misdiagnosis, we describe the clinical, histopathologic, and immunohistochemical findings of cases of epithelioid angiosarcoma diagnosed at our institution. Seven cases of epithelioid angiosarcoma with appropriate pathologic material were identified from our archives. Immunohistochemistry was used to detect the expression of CD31, CD34, Factor VIII, cytokeratin, epithelial membrane antigen, vimentin, HMB45, CD1a, CD68, lysozyme, CD45, desmin, and smooth muscle actin in all cases. Follow-up information was obtained by reviewing medical records or by direct communication with family members. The lesions involved the bone (n = 4) and soft tissues (n = 3). Microscopically, all tumors had a predominantly diffuse growth pattern, with a focal nested architecture in 6 cases, which closely mimicked metastatic carcinoma. The initial biopsy was performed in 2 of 6 patients and revealed the presence of a malignant neoplasm suggestive of metastatic carcinoma. Immunohistochemically, the epithelioid endothelial cells usually showed strong reactivity for CD31 (7/7), variable or focal positive staining for CD34 (5/7), Factor VIII (4/7), cytokeratin (6/7), epithelial membrane antigen (2/7), vimentin (7/7), and CD68 (3/7). In contrast, they were negative for CD1a, HMB45, lysozyme, CD45, desmin, and smooth muscle actin. Three patients died of disease within one year of the diagnosis, 2 patients developed local recurrence or metastases, and another 2 were disease-free at this writing. With any unusual epithelioid neoplasm displaying some or all of the morphologic features described above, epithelioid angiosarcoma should be included in the differential diagnosis. In such an instance, endothelial markers should be incorporated in the immunohistochemical analysis to avoid misdiagnosis, particularly with limited sampling.