Epithelioid Angiosarcoma Research Articles

Multifocal vascular neoplasm with an EWSR1::NFATC2 gene fusion and progression to epithelioid angiosarcoma - a case report.

Multifocal vascular neoplasm with an EWSR1::NFATC2 gene fusion and progression to epithelioid angiosarcoma - a case report.

Molecular Testing Prevents Overdiagnosis of Epithelioid Hemangioma

Abstract Introduction/Objective Epithelioid hemangiomas (EH) are mostly solitary benign vascular tumors commonly involving the dermal and subcutaneous areas of the head and neck. However, reports of multifocality are increasing, specially in same anatomic region. Some reported cases of EH show morphologic features that may mimic malignant vascular tumors such as hemangioendotheliomas or angiosarcomas. However, there are no reported overlaps in the molecular characteristics of these tumors. We describe a multifocal EH with atypical features, suspicious for angiosarcoma, which needed molecular testing to resolve the diagnosis. Methods/Case Report The patient is a previously healthy 42-year-old male with a 5-month history of multiple growing painless penile nodules. Biopsy of one of the nodules was done. Histologic examination showed multifocal dermal- based ill-circumscribed nodules with interspersed inflammatory infiltrates and focal areas of necrosis. Tumor periphery shows vasoformative growth with intraluminal and extravasated red blood cells. Neoplastic cells were large and ovoid with abundant eosinophilic cytoplasm, large vesicular pleomorphic nuclei and prominent nucleoli. Immunohistochemical stains showed strong expression of ERG, FLI-1, and CD31 and negativity for AE1/AE3, CD34, HHV8 and EMA. The morphologic and immunohistochemical features of the specimen showed significant overlap of two primary differential diagnoses - epithelioid angiosarcoma and epithelioid hemangioma. Ultimately, molecular testing revealed ZFP36-FOSB fusion, confirming the diagnosis of EH. Results (if a Case Study enter NA) NA Conclusion Differentiating benign and malignant epithelioid vascular neoplasms can sometimes be challenging. There can be overlap in terms of architectural variety, presence of necrosis, vasoformation, inflammatory cell components and cytologic features, especially in EH with atypical features and epithelioid angiosarcoma (EA). Both of these tumors show positive immunoreactivity for CD31, CD34, FLI1 and ERG, and negativity for most keratins. Currently, the most reliable way to distinguish each tumor is molecular testing, with EH having FOS/FOSB rearrangements and EA with MYC or FLT4 amplification. Benign and malignant epithelioid vascular tumors can be indistinguishable morphologically and immunohistochemically. Molecular tests can help differentiate these neoplasms to better tailor patient management.

EVERY KERATIN-POSITIVE MALIGNANT TUMOR IN THE ADRENAL GLAND IS NOT METASTATIC CARCINOMA- A CASE REPORT

Abstract Introduction/Objective Angiosarcoma is a rare subtype of sarcoma arising from the vascular endothelial cells. Primary adrenal angiosarcoma (PAA) is an exceedingly rare entity, with only 53 cases documented in the literature so far. Methods/Case Report A 78-year-old female presented with left upper quadrant abdominal pain and early satiety. A computed tomography scan of the abdomen revealed a 15 x 13 x 16 cm, heterogeneous enhancing mass with interspersed calcifications, possibly originating from the left adrenal gland. The mass was found to be displacing the spleen and the left kidney. An ultrasound-guided biopsy of the mass was inconclusive, and the patient subsequently underwent resection of the left retroperitoneal mass, left adrenalectomy, splenectomy, and left partial nephrectomy. Gross examination revealed a slightly ruptured, red-brown mass (19.5 x 12.7 x 12.6 cm, 1784g), which was serially sectioned to reveal diffusely hemorrhagic and necrotic cut surfaces. On microscopic examination, the tumor was almost entirely hemorrhagic and necrotic, with a few viable areas that were composed of solid sheets of markedly pleomorphic epithelioid cells with a high nuclear-cytoplasmic ratio, prominent nucleoli, and increased mitotic figures. There were also a few ectatic vascular spaces lined by these markedly pleomorphic epithelioid cells. Immunohistochemical stains of the tumor cells showed positive results for cytokeratins, CD31, ERG, and friend leukemic integration 1 (FLI-1), while Inhibin and SF-1 were negative. A diagnosis of epithelioid angiosarcoma was rendered. Results (if a Case Study enter NA) NA Conclusion PAA can be difficult to diagnose since it is rare, and thus it is not typically considered in the initial differential diagnosis. Most PAA have an epithelioid morphology, and they frequently test positive for cytokeratins. Biopsy diagnosis can also be challenging, as the mass may exhibit extensive necrosis and hemorrhage. Considering its rarity, large adrenal masses with heterogeneous features on imaging PAA should be considered as a differential diagnosis.

A unique presentation of pericardial epithelioid angiosarcoma with multifaceted complications

Introduction and importance: Angiosarcomas are rare tumors of endothelial origin and may arise in any organ. Epithelioid angiosarcomas are a subtype of angiosarcoma that are rapidly progressive and typically fatal. Case presentation: The authors report a case of a 25-year-old previously healthy female who presented initially for dyspnea and palpitations, on further evaluation she was found to have large bilateral pleural effusions and cardiac tamponade. Clinical discussion: Pericardiocentesis and thoracentesis were performed alongside biopsies that revealed atypical cellular proliferation. Fluorodeoxyglucose-positron emission tomography (FDG-PET) showed avid uptake in the anterior mediastinum, perivascular, paratracheal, subcarinal and pleural lymph nodes with large FDG uptake in the bilateral pleural effusion. Mediastinoscopy was done and biopsies showed an overtly malignant, epithelioid neoplasm with foci of vaso-formation; Keeping with high-grade epithelioid angiosarcoma of the pericardium. She received six cycles of weekly paclitaxel, but imaging for abdominal pain incidentally showed evidence of metastasis to the liver and spine so she was switched to Adriamycin-Ifosfamide for which she received one cycle so far. Her hospital course was complicated by high-output pleural effusions, chylothorax, left atrial thrombus formation and an intensive care unit stay for septic shock. Conclusion: Pericardial epithelioid angiosarcoma has been reported rarely in the literature. The authors aim to report a case of extensive metastatic pericardial epithelioid angiosarcoma in a young patient; which we believe can be an addition to the literature of a malignancy associated with poor prognosis and no definitive proven treatment regimen.

Epithelioid angiosarcoma arising from pleomorphic xanthoastrocytoma, CNS WHO grade 3.

Epithelioid angiosarcoma arising from pleomorphic xanthoastrocytoma, CNS WHO grade 3.

Molecular Analysis of Renal/Adrenal Angiosarcomas Reveals High Frequency of Recurrent Genetic Alterations.

Angiosarcomas of the kidney and adrenal gland are rare, highly aggressive vascular neoplasms. Their genomic profile has not been systematically studied to date. We report the clinicopathologic and molecular features of six angiosarcomas centered in the kidney/adrenal gland. All patients were male adults, ranging from 58 to 77 years of age. Tumor sizes ranged from 2.5 to 22.5 cm. Half of the cases demonstrated hot spot mutations in the KDR gene, while one-third demonstrated mutations in the PIK3CA gene; both of these gene alterations being previously described, preferentially in breast angiosarcomas. In addition, two cases each demonstrated BRIP1 gene amplification, CTNNB1 and ETV6 mutations, which have not been previously reported in angiosarcoma. Notably, molecular studies were critical in establishing the correct diagnoses in three cases: one was an epithelioid angiosarcoma originally misdiagnosed as metastatic adenocarcinoma to the adrenal gland, the second was a vasoformative angiosarcoma that mimicked hemangioma, and the third was a collision tumor between a high-grade angiosarcoma and a chromophobe renal cell carcinoma which was originally diagnosed as a sarcomatoid renal cell carcinoma. In summary, angiosarcomas of the kidney and adrenal gland have a high frequency of recurrent genetic alterations, some of them being shared with other angiosarcoma subtypes, while other appear to be novel. In particular, activating hot spot KDR and PIK3CA mutations represent potential therapeutic targets for these highly aggressive cancers.

Comparison of clinical and MRI features of hepatic angiosarcoma and epithelioid hemangioendothelioma.

Differentiating hepatic epithelioid hemangioendothelioma (EHE) and angiosarcoma (AS), the two most common vascular tumors in the liver, is important due to disparities in their prognosis and treatment. We aimed to compare clinical and MRI features of the two tumors. This retrospective study included patients with pathologically-confirmed AS or EHE who underwent MRI using gadoxetate disodium between 2008 and 2023. Two radiologists independently reviewed MR images. Wilcoxon rank sum and Fisher's exact tests were used to compare clinical and imaging features. Overall survival was compared using restricted mean survival time at 3years. 32 patients with AS (18 women [56.3%]; median age, 68years) and 38 with EHE (24 women [63.2%]; 51years) were included. Patients with AS were generally older (81.3% ≥ 60years; P < 0.001), had more frequent laboratory abnormalities (P ≤ 0.018), and poorer overall survival (11.2 vs. 31.8months; P < 0.001) than those with EHE. On MRI, a large dominant mass accompanied by smaller nodules (14/32, 43.8%), often with ill-defined margins (15/32, 46.9%) was prevalent in AS; compared with nodules of similar sizes (24/38, 63.2%; P = 0.015) with well-defined margin (30/38, 78.9%; P = 0.002) in EHE. Cirrhotic appearance of the liver was more frequent in AS (62.5%, P < 0.001), along with decreased parenchymal enhancement on hepatobiliary phase (31.3%, P < 0.001) and ascites (37.5%, P = 0.010). AS frequently presented with avid enhancement of bizarrely-shaped foci, with a centrifugal enhancement pattern. In comparison, targetoid appearance was characteristic of EHE (78.9% on T2-weighted, 54.1% on diffusion-weighted, 65.8% on multiphase images) (P ≤ 0.002), with enhancement degree typically lower than that of the aorta. On hepatobiliary phase, all the AS exhibited hypointensity, while 39.5% of EHE showed targetoid appearance (P < 0.001). Patients aged ≥ 60years presenting with laboratory abnormalities, typically with a large dominant mass accompanied by smaller nodules, exhibiting avid, bizarre, and centrifugal enhancement-particularly in the cirrhotic-appearing liver-suggests the likelihood of AS over EHE.

Assessing spatial distribution of bioindicator elements in various cutaneous tumors using correlative imaging with laser-ablation-based analytical methods

Correlative imaging of cutaneous tumors provides additional information to the standard histopathologic examination. However, the joint progress in the establishment of analytical techniques, such as Laser-Induced Breakdown Spectroscopy (LIBS) and Laser Ablation Inductively Coupled Plasma Mass Spectrometry (LA-ICP-MS) in clinical practice is still limited. Their combination provides complementary information as it is also shown in our study in terms of major biotic (Ca, Mg, and P) and trace (Cu and Zn) elements. To elucidate changes in the elemental composition in tumors, we have compiled a set of malignant tumors (Squamous Cell Carcinoma, Basal Cell Carcinoma, Malignant Melanoma, and Epithelioid Angiosarcoma), one benign tumor (Pigmented Nevus) and one healthy-skin sample. The data processing was based on a methodological pipeline involving binary image registration and affine transformation. Thus, our paper brings a feasibility study of a practical methodological concept that enables us to compare LIBS and LA-ICP-MS results despite the mutual spatial distortion of original elemental images. Moreover, we also show that LIBS could be a sufficient pre-screening method even for a larger number of samples according to the speed and reproducibility of the analyses. Whereas LA-ICP-MS could serve as a ground truth and reference technique for preselected samples.

Angiosarcoma Arising in a Vagal Schwannoma - Report and Literature Review.

Schwannoma and angiosarcoma are rare occurrences. Angiosarcoma's occurrence in a preexisting schwannoma is a very rare event with only fifteen cases reported in the literature. We report the sixteenth case of angiosarcoma arising in a schwannoma, a 30-year-old male patient with a long-standing (15 years) history of right neck lump. The lump measured around 10x7 cm at the time of presentation and all cranial nerves were intact. We performed a fiberoptic laryngoscopy and a computed tomography scan of the head and neck with contrast. Our provisional diagnosis was a parapharyngeal space neoplasm, most likely a salivary gland malignancy. The tumor was excised surgically. On histopathology it contained two distinct tissue architectures representing a schwannoma and epithelioid angiosarcoma, also confirmed on immunohistochemistry. Literature review of these limited cases implicates a poor prognosis of the disease. The pathogenesis is uncertain, but the theories put forward suggest chronic vascular stasis or vascular endothelial proliferation as possible etiologies. The main takeaway of our report is to consider the potential of malignancy in long standing cases of schwannomas. Prompt surgical treatment should be offered, and the patient and their family be counselled for postoperative adjuvant treatment for better prognosis.

Epithelioid Angiosarcoma: A Diagnostic Dilemma with Clinically and Histopathologically Overlapping Features

Cutaneous angiosarcoma is a rare malignancy of endothelial cell origin that commonly presents in the head and neck region of elderly individuals and is known for high rates of lymph node metastasis and mortality. There is significant variability in the histopathologic features, and immunohistochemistry is necessary to help differentiate epithelioid angiosarcoma from lesions it may clinically mimic. Here, we report a rare case of poorly differentiated epithelioid angiosarcoma on the trunk initially diagnosed as poorly differentiated squamous cell carcinoma (SCC) on shave biopsy. A 56-year-old man presented for a full-body skin examination significant for a 5.6 x 2.8 mm brown-black macule on the left superomedial posterior trunk and a 5.0 x 2.7 mm pink, ulcerated papule on the right inferior posterior trunk. On histopathology, one lesion was found to be melanoma in situ, and the other showed ulcerated and infiltrative, poorly differentiated SCC. Post-excision histologic analysis of the right inferior back lesion revealed tumor cells immunoreactive to vimentin, ERG, and CD31 (PECAM-1). These findings suggested that the lesion previously diagnosed as SCC was an invasive, poorly differentiated epithelioid angiosarcoma. The patient underwent 40 sessions of radiotherapy. Epithelioid angiosarcoma occurring outside of the conventional epidemiologic profile is believed to portend higher mortality rates. This case highlights the importance of thorough clinical evaluation and appropriate histopathologic diagnosis of an aggressive neoplasm unlikely to present in this location. Further studies are required to explore potential risk factors, effective treatments, and long-term outcomes in patients with epithelioid angiosarcoma.

Proposed diagnostic and prognostic markers of primary malignant hepatic vascular neoplasms

IntroductionPrimary malignant hepatic vascular tumors with various malignant potentials include epithelioid hemangioendothelioma (EHE) and angiosarcoma (AS), which may overlap pathologically. This study aimed to compare the pathological findings of hepatic EHE with those of AS, in association with patient outcomes.MethodsFifty-nine histologically confirmed patients with 34 EHE and 25 AS were admitted to a tertiary hospital from 2003 to 2020. Their EHE and AS pathological features were compared. Immunohistochemistry for CD31, ERG, CAMTA-1, TFE3, P53, and Ki-67 labeling was performed on paraffin-embedded blocks. Markers, along with histological findings, were analyzed for the purposes of diagnostic and prognostic significance by multivariate analysis.ResultsCAMTA-1 was 91.2% positive in EHE, but negative in AS (p = < 0.001). AS was significantly correlated to an aberrant p53 expression, high Ki-67 labeling, and high mitotic activity, compared to EHE (all, p = < 0.001). EHE can be classified as low grade (LG) and high grade (HG) using the prognostic values of mitotic activity and ki-67 labeling (sensitivity = 1, specificity = 1). Low grade-EHE showed significantly better overall survival than high grade-EHE (p = 0.020).ConclusionsImmunohistochemistry for CAMTA-1, P53, and Ki-67 labeling may help distinguish EHE and AS in histologically ambiguous cases, especially small biopsied tissue. Moreover, the combination of mitotic activity and Ki-67 labeling can be a prognostic factor for EHE with various clinical features.

Epithelioid hemangioma of the acromion causing shoulder pain: a case report.

Epithelioid hemangioma of bone is a rare benign vascular tumor. Because of the locally destructive clinical presentation and the presence of atypical histologic features with increased mitotic activity and necrosis, it is often misdiagnosed as low-grade malignant epithelioid hemangioendothelioma or high-grade malignant epithelioid angiosarcoma. Correct diagnosis through imaging studies and histopathological examination is mandatory to determine the appropriate course of treatment, as the prognosis differs from that of other malignant hemangioma tumors. A 69-year-old male who presented with intractable shoulder pain caused by epithelioid hemangioma in the acromion of the scapula was treated with tumor curettage. This paper reports a good result with a review of the relevant literature.

Aortic Angiosarcoma Manifesting as Multiple Musculoskeletal Metastases: A Case Report.

Aortic angiosarcomas are rare. Due to its rarity and metastatic presentation, it is difficult to diagnose metastatic aortic angiosarcoma. We describe the clinicopathological and radiologic features of a metastatic aortic angiosarcoma presenting as musculoskeletal metastases. A 59-year-old male patient presented with left thigh pain. Plain radiographs revealed multifocal osteolytic lesions in the left femur shaft. Abdominopelvic computed tomography showed a lobulated osteolytic lesion in the left iliac bone. Magnetic resonance images revealed multifocal soft tissue lesions in the thigh musculature. A positron emission tomography/computed tomography (PET/CT) scan demonstrated multiple foci of increased uptake in the left femur bone, pelvis, left thigh, and calf musculature. Focal increased uptake in the lower abdominal aorta was newly detected. Pelvis biopsy showed tumor cell nests of epithelioid cells. The tumor cells showed vasoformative features. Immunohistochemically, the tumor cells showed positivity for vimentin, CD31, and ERG. The pathologic diagnosis of epithelioid angiosarcoma was established. The origin of the tumor was presumed to be the aorta. This case underscores the importance of PET scans in identifying primary lesions. In terms of the histopathologic diagnosis of biopsy samples with tumor cells exhibiting epithelioid neoplastic morphology, employing appropriate ancillary techniques such as immunocytochemistry with vascular markers may assist in accurately diagnosing metastatic angiosarcoma.

A155 METASTATIC EPITHELIOID ANGIOSARCOMA AND RECURRENT GASTROINTESTINAL BLEEDING: A CASE REPORT

Abstract Background Angiosarcomas are aggressive tumors of the vascular endothelium that represent less than 3% of all sarcomas. Epithelioid angiosarcomas are a distinct subtype, and in exceedingly rare circumstances can involve the gastrointestinal tract leading to significant bleeding. Aims Report a case of metastatic epithelioid angiosarcoma as a cause of recurrent gastrointestinal bleeding and the utility of endoscopy in obtaining a diagnosis. Methods A retrospective case report. Results A 71 year old female with a history of type two diabetes and coronary artery disease presented to a peripheral hospital with a two week history of fatigue, abdominal pain, and intermittent fevers for which she was diagnosed with pyelonephritis. Computed tomography (CT) scan of the abdomen/pelvis was unremarkable on admission. Her stay was complicated by cardiac ischemia prompting the initiation of dual antiplatelet therapy and anticoagulation. After a few days she developed melena and an acute hemoglobin drop requiring red blood cell (RBC) transfusion. Gastroscopy revealed a few superficial erosions in the stomach and duodenum. She went on to develop hypercalcemia prompting a repeat CT abdomen/pelvis which showed multiple lytic bone lesions, innumerable small lung nodules, and areas of hypoattenuation in the liver. Contrast enhanced MRI abdomen was performed to better assess the liver, showing a geographic area of signal abnormality without mass like features. The patient was transferred to a tertiary centre for ongoing work-up and management. Shortly after arrival, a bone biopsy from a lytic lesion of the iliac crest was taken. Initial histopathology was inconclusive for malignancy. During this time, she had ongoing melena and need for intermittent RBC transfusions. Enteroscopy revealed multiple centrally ulcerated, hemorrhagic, erythematous/purpuric lesions throughout the stomach and proximal small bowel (Figure 1a). A duodenal lesion with fresh clot was clipped and multiple biopsies were taken from the stomach and duodenum. Histopathology from a gastric biopsy confirmed the diagnosis of epithelioid angiosarcoma (Figure 1b). PET scan revealed uptake in bone, lungs, and liver. The patient received 1 cycle of palliative chemoradiation, however this was discontinued due to frailty. Overt bleeding and intermittent RBC transfusions continued. The patient was transferred to full comfort care and died within 4 months of initial presentation to hospital. Conclusions Epithelioid angiosarcoma involving the gastrointestinal tract should be considered as a cause for upper gastrointestinal bleeding. Our case highlights the endoscopic characteristics and the importance of endoscopic biopsy in obtaining a histopathologic diagnosis. Figure 1a. Enteroscopy showing metastatic angiosarcoma to the stomach and small bowel. A. Gastroesophageal junction; B. Cardia and fundus; C. Duodenal bulb; D. Second portion of duodenum. Figure 1b. Metastatic epithelioid angiosarcoma to the stomach. A-B. Low and medium power H&amp;E; C. Cytokeratin AE1/3; D. Ber-EP4; E. CD31; F. ERG. Funding Agencies None

Combining nanoparticle albumin-bound paclitaxel with camrelizumab in advanced soft tissue sarcoma: activity, safety, and future perspectives.

Background: It is still uncertain whether Nanoparticle albumin-bound paclitaxel (nab-paclitaxel) and programmed cell death protein 1 (PD-1) inhibitor have synergistic effects on metastatic soft tissue sarcomas (STSs). The purpose of this study was to evaluate the safety and activity of nab-paclitaxel plus camrelizumab (a PD-1 inhibitor) in patients with advanced STS who had previously failed chemotherapy. Methods: In this single-center, open-label, single-arm phase II clinical trial, patients with advanced (unresectable or metastatic) STS who had previously failed chemotherapy received up to six cycles of nab-paclitaxel plus camrelizumab, whereas camrelizumab treatment was continued for up to 1 year. The median progression-free survival (PFS), objective response rate (ORR) and safety were collected and evaluated. Results: This trial included 40 patients (28 men and 12 women). The overall ORR was 22.5%, and the median PFS was 1.65months (95% confidence interval [CI], 1.3-2.0months). Patients with epithelioid sarcoma demonstrated a longer PFS compared with those with other histological subtypes (2.3months vs. 1.5months, respectively); however, this difference was not significant. Patients who had received only one line of previous chemotherapy had a significantly longer PFS compared with those who had undergone two or more lines of previous chemotherapy (2.8months vs. 1.3months, respectively, p = 0.046). In terms of safety, the toxicity of this combination therapy is mild and no serious adverse events have occurred. Conclusion: Nab-paclitaxel plus camrelizumab exhibited modest activity and mild toxicity in treating epithelioid sarcoma, angiosarcoma, and fibrosarcoma. The overall effectiveness of this treatment regimen for advanced STS is relatively low. Further research on combining nab-paclitaxel with effective drugs, including chemotherapy and targeted agents, for these specific STS subtypes is needed.

Pseudolymphomatous primary breast epithelioid angiosarcoma: a case report with presentation as a monoclonal B-cell lymphocytosis

Pseudolymphomatous primary breast epithelioid angiosarcoma: a case report with presentation as a monoclonal B-cell lymphocytosis

Vascular Neoplasms With NFATC1/C2 Gene Alterations : Expanding the Clinicopathologic and Molecular Characteristics of a Distinct Entity.

Despite significant advances in their molecular pathogenesis, skeletal vascular tumors remain diagnostically challenging due to their aggressive radiologic appearance and significant morphologic overlap. Within the epithelioid category and at the benign end of the spectrum, recurrent FOS/FOSB fusions have defined most epithelioid hemangiomas, distinguishing them from epithelioid hemangioendothelioma and angiosarcoma. More recently, the presence of EWSR1/FUS :: NFATC1/2 fusions emerged as the genetic hallmark of a novel group of unusual vascular proliferations, often displaying epithelioid morphology, with alternating vasoformative and solid growth, variable atypia, reminiscent of composite hemangioendothelioma. In this study, we further our understanding and morphologic spectrum of NFATC -fusion positive vascular neoplasms by describing 9 new cases, including soft tissue locations and novel fusion partners. Combining with the initial cohort of 5 cases, a total of 14 patients were analyzed, showing slight female predilection and an age range of 10 to 66 (mean 42y). Twelve patients had solitary lesions, while 2 had multifocal polyostotic (pelvic bones) disease. Overall, 12 lesions were intra-osseous and 2 in soft tissue. By targeted RNA Fusion panels or FISH, there were 6 cases of EWSR1::NFATC1 , 4 EWSR1::NFATC2 , 2 FUS::NFATC2 , 1 EWSR1 rearrangement, and 1 with a novel FABP4::NFATC2 fusion. Follow-up was available in 4 patients. One patient experienced 2 local recurrences, 11 and 15 years postdiagnosis, and one patient experienced progressive disease despite multimodality treatment (curettings, embolization, radiation) over 3 years. In summary, our extended investigation confirms that NFATC -related fusions define a distinct group of vascular neoplasms with variable architecture, epithelioid phenotype, and cytologic atypia, commonly located in the bone, occasionally multifocal and with potential for local recurrence and aggressive behavior but no metastatic potential. Molecular analysis is recommended in diagnostically challenging cases with atypical histology to exclude malignancy.

Epithelioid angiosarcoma of the pericardium: Case report of the challenges in the management of a rare malignancy with poor prognosis.

A primary epithelioid angiosarcoma of the pericardium is a rare soft tissue malignancy. This report describes a young adult woman who presented with progressive dyspnea and pericardial effusion. She was found to have pericardial mass on imaging along with extensive myocardial infiltration. The biopsy of the mass revealed epithelioid angiosarcoma, which was CD34 and CD31-immuno-positive. Due to unresectable disease, she was given a trial of immunotherapy followed by conventional chemotherapy. She showed partial response on interim assessment, but progressed soon after the completion of six cycles and succumbed to her rapidly progressive illness within nine months of diagnosis. This report discusses the diagnostic and therapeutic challenges faced while managing this disease of aggressive tumor biology.

Misdiagnosis of Cutaneous Epithelioid Angiosarcoma as Diabetic Foot Ulcer: A Case Study

Background. AS is a malignant tumor that originates from vascular endothelial cells and is known for a high rate of local recurrence and metastasis. Case Report. A 48-year-old male presented with cutaneous epithelioid AS. Cutaneous AS of the foot is quite rare, especially in the absence of predisposing factors, and in this patient it was previously misdiagnosed as a DFU. Conclusion. Physicians should be aware of this rare presentation of cutaneous AS. The authors of the current report advise regular clinical reassessment of chronic ulcers and biopsies of nonhealing wounds, even when adequate wound treatment has been administered, with the goal of identifying ulcerated skin malignancies and preventing delay in providing appropriate treatment.

A Wolf in Sheep's Clothing - A Rare Case of an Angiosarcoma Arising from a Schwannoma in the Posterior Mediastinum: A Case Report

Introduction: Schwannoma is one of the benign tumors occurring in the posterior mediastinum. However, angiosarcoma is a malignant tumor of vascular origin and presents as an infiltrating mass. Primary angiosarcoma of the mediastinum is very rare. Few cases have been reported in the atrium, pulmonary vein, and lung and present with chest pain. Angiosarcoma arising from a benign tumor like a schwannoma is very rare; only a handful of cases have been reported in the literature. This case is presented for its rarity and awareness that even a schwannoma presenting as well-circumscribed mediastinal mass can harbor a life-threatening malignancy. Case Report: A 51-year-old male presented with chest pain, and on investigation, there was an extrapulmonary and posterior mediastinal mass in the left paravertebral region with a benign appearance and his serum tumor markers were normal. However, a needle biopsy showed evidence of an angiosarcoma. Hence, with these contradicting results, a surgical exploration was done and since it was well delineated was excised completely. Grossly, it was a well-circumscribed mass measuring 12.5 cm × 9.5 cm × 6.5 cm with a variegated appearance with pale–yellow and hemorrhagic areas. A diagnosis of an epithelioid angiosarcoma arising from a schwannoma was made by light microscopic and immunohistochemical findings. Conclusion: Epithelioid angiosarcoma is a rare malignant transformation occurring in a schwannoma. It can also have a deceptively benign appearance as in our case, and in any symptomatic long-standing posterior mediastinal schwannoma, this differential should be kept in mind when planning surgery and further treatment.