Porcine-derived Acellular Dermal Matrix for Temporary Soft Tissue Coverage in Locally Advanced Angiosarcoma of the Face - A Soft Tissue Odyssey?

BACKGROUND/AIM: Angiosarcomas of the face are rare but present significant treatment challenges due to their origin in the supportive tissues of blood or lymphatic vessels. Achieving optimal balance between oncological efficacy and aesthetic outcomes requires a multidisciplinary approach, particularly in cases where radical R0 resection is necessary. Delays often occur, especially during histopathological examinations, which can complicate primary plastic reconstruction before definitive pathological findings. CASE REPORT: To address this issue, we present a case with the use of porcine-derived acellular dermal matrix for temporary soft tissue coverage as a viable option in a case of angiosarcoma of the face. This is particularly useful in situations where frozen sections risk the loss of critical anatomical structures and intraoperative diagnosis is not feasible. This approach allowed for satisfactory wound coverage and granulation during diagnostic phases, paving the way for oncologically manageable situations and functional rehabilitation. CONCLUSION: Temporary soft tissue coverage with porcine-derived acellular dermal matrix is a valuable option in tumor surgery of rare and complex situations.

Prognostic value of tumour-related factors associated with canine retroperitoneal hemangiosarcoma in comparison with other anatomic presentations: A retrospective observational study.

BACKGROUND: Dogs with retroperitoneal hemangiosarcoma (HSA) exhibit variable postoperative median survival times (MST). OBJECTIVE: To retrospectively evaluate the prognostic value of selected tumour-related factors, such as tumour size, rupture, invasion into adjacent tissue, involvement of lymph node and distant metastasis, they were analysed in dogs with retroperitoneal HSA. METHODS: Ten dogs with retroperitoneal HSA managed solely with surgical excision were reviewed and compared with spleen (71) and liver (9) HSA. The Kaplan-Meier method and log-rank analysis were used compare MSTs between factors. Multivariable Cox proportional-hazard analysis was used to compare differences between arising sites. RESULTS: Retroperitoneal HSA showed comparatively longer postoperative MST compared with that of spleen and liver HSA and demonstrated significantly longer MST (p = 0.003) for tumours ≥5 cm (195 days) than <5 cm (70 days). Spleen HSA revealed significantly shorter MSTs in involvement of distant lymph nodes (23 days) and distant metastasis (39 days) than those in negative (83 days, p = 0.002 and 110 days, p < 0.001, respectively). Liver HSA also revealed significantly shorter MST (16.5 days compared with 98 days, p = 0.003) for distant metastasis. Additionally, hazard ratios (HRs) and their forest plot for overall HSA revealed as poor prognostic factors, arising sites (spleen; HR 2.78, p = 0.016 and liver; HR 3.62, p = 0.019), involvement of distant lymph nodes (HR 2.43, p = 0.014), and distant metastasis (HR 2.86, p < 0.001), and as better prognostic factor of tumour size ≥5 cm (HR 0.53, p = 0.037). CONCLUSION: In combination with overall HSA, retroperitoneal HSA shows comparatively longer postoperative MST compared to spleen and liver HSA, associated with tumour size ≥5 cm suggesting better prognostic factor.

Right-sided ureteral hemangiosarcoma in a paraplegic dog.

BACKGROUND: This study aims to describe a rare case of primary ureteral hemangiosarcoma, in which surgical intervention preserved the kidney and ureter after tumor removal. CASE PRESENTATION: A 13-year-old, neutered male dog, weighing 14 kg, mixed-breed, presented with apathy, anorexia, acute-onset vomiting, and abdominal discomfort during the physical examination. Ultrasonography and pyelography revealed a right-sided dilation of the renal pelvis and ureter due to complete obstruction in the middle third of the ureter. A mass obstructing the lumen of the right ureter was completely resected, and ureteral suturing was performed, preserving the integrity of the involved structures. Histopathology confirmed primary ureteral hemangiosarcoma. Due to the local and non-invasive nature of the mass, chemotherapy was not initiated. The patient's survival was approximately two years, and normal renal function was preserved throughout this period. CONCLUSIONS: Considering this type of tumor in the differential diagnosis of upper urinary tract obstructive disorders. Furthermore, the preservation of the ureter and kidney is a suitable therapeutic option after surgical resection of non-invasive tumors.

Recurrent fever leading to the diagnosis of an angiosarcoma of the adrenal gland: a case report.

BACKGROUND: Angiosarcoma of the adrenal gland is a very rare malignant vascular neoplasm. The clinical symptoms are atypical or completely absent. Angiosarcomas of the adrenal gland are therefore often discovered incidentally, and the diagnosis is made histologically after resection. CASE PRESENTATION: A 46-year-old white Spanish male who was a previous smoker and nondrinker and was slightly overweight (92 kg, 176 cm, body mass index 29.7 kg/m2) with no relevant medical history presented to the internal medicine emergency department of our hospital with an unclear 12 cm tumor of the right adrenal gland. Prior to the computed tomography scan, he had had persistent evening fevers for 4 months and unintentional weight loss of 5 kg. The laboratory results showed anemia and an elevated C-reactive protein, but no hormone production. We performed an open adrenalectomy of the right adrenal gland. Finally, the histologic findings revealed an angiosarcoma of the adrenal gland. CONCLUSION: Even though angiosarcomas of the adrenal gland are rare, the differential diagnosis of an angiosarcoma should be considered if a malignant tumor of the adrenal gland is suspected. Treatment decisions should be made on an interdisciplinary basis and preferably in a specialized center. Owing to the rarity of angiosarcomas of the adrenal gland, it is necessary to continue to share clinical experience to gain a better understanding of this particular tumor entity.

A rare case of epithelioid angiosarcoma.

Epithelioid angiosarcoma is a rare high-grade vascular neoplasm with a poor prognosis. We present an anticoagulated 77-year-old man, with a history of popliteal/soleal vein thrombosis in the previous month, complaining of ipsilateral persistent lower limb pain and claudication. Absent popliteal/distal pulses prompted an arterial doppler ultrasound (DUS), revealing thrombosis of the distal superficial femoral artery and a popliteal mass. As the arterial wall's integrity could not be appropriately evaluated by DUS, adventitial cystic disease of the popliteal artery was suspected. Computed tomography angiography and magnetic resonance imaging findings were also suggestive. Due to refractory pain, he was submitted to a popliteal mass excision along with a femoral-posterior tibial bypass. Pathology revealed an epithelioid angiosarcoma. He was referred to a Sarcoma Center, requiring hospitalization for agitation and fever. A positron emission tomography (PET) scan revealed extensive lower limb disease persistence and distant metastases. He died on the 56th day after surgery. To our knowledge, there are only 15 cases of angiosarcoma of the popliteal artery described in the literature. Ours stands out as the first one unrelated to a popliteal aneurysm. Being a highly-aggressive tumor, an early diagnosis is challenging but essential to a successful treatment, warranting the need for suspicion of this neoplasm. An early core biopsy or surgical sample may expedite the diagnosis.

Improved predictability is needed for calculators used to preoperatively determine the etiology of splenic masses in dogs: an external validation study of the HeLP score and T-STAT.

OBJECTIVE: To assess the predictability of the hemangiosarcoma likelihood prediction (HeLP) score and the Tufts Splenic Tumor Assessment Tool (T-STAT) for hemangiosarcoma and malignancy, respectively. ANIMALS: 261 dogs undergoing splenectomy for a splenic mass. METHODS: Medical records were retrospectively reviewed; variables for the HeLP score and T-STAT were collected, and scores were assigned. Area under the curve (AUC) was calculated for each score. RESULTS: The HeLP score included 141 dogs; hemangiosarcoma was diagnosed in 87 (61.7%) dogs. The median cumulative HeLP score was 51 (range, 17 to 82; IQR, 39 to 58) for dogs with hemangiosarcoma and 28 (range, 0 to 70; IQR, 17 to 41) for dogs without hemangiosarcoma. The categorical HeLP score was low (28; 32.2%), medium (31; 35.6%), and high (28; 32.2%) for dogs with hemangiosarcoma and was low (41; 75.9%), medium (9; 16.7%), and high (4; 7.4%) for dogs without hemangiosarcoma. The AUC of the cumulative and categorical HeLP scores for diagnosis of hemangiosarcoma were 0.79 (95% CI, 0.71 to 0.86) and 0.73 (95% CI, 0.65 to 0.82), respectively. The T-STAT included 181 dogs. Lesions were benign in 95 (52.5%) and malignant in 86 (47.5%) dogs. The median T-STAT score was 62% (range, 5% to 98%; IQR, 36% to 77%) for dogs with malignant lesions and 38% (range, 5% to 91%; IQR, 24% to 59%) for dogs with benign lesions. The T-STAT had an AUC of 0.68 (0.60 to 0.76) for diagnosis of malignancy. CLINICAL RELEVANCE: The HeLP score had acceptable performance, and the T-STAT had poor performance for diagnosis prediction. A tool with excellent or outstanding discrimination is needed to more reliably predict the presence of hemangiosarcoma or a malignant lesion preoperatively.

Unusual presentation and delayed diagnosis of cardiac angiosarcoma.

BACKGROUND: Primary cardiac angiosarcomas are very rare and present aggressively with high rates of metastasis. Given the poor prognosis, particularly once disease has spread, early diagnosis and multidisciplinary treatment is essential. CASE PRESENTATION: We present the case of a 46-year-old male who presented with chest pain, intermittent fevers, and dyspnea. Workup with computed tomography scan and transesophageal echocardiography demonstrated a right atrial pseudoaneurysm. Given the concern for rupture, the patient was taken to the operating room, where resection of the pseudoaneurysm and repair using a bovine pericardial patch was performed. Histopathology report initially demonstrated perivascular lymphocyte infiltrate. Six weeks later, the patient represented with chest pain and new word finding difficulty. Workup revealed multiple solid lung, pericardial, brain, and bone nodules. Eventual biopsy of a cardiophrenic nodule demonstrated angiosarcoma, and rereview of the original pathology slides confirmed the diagnosis of primary cardiac angiosarcoma. CONCLUSIONS: Primary cardiac angiosarcomas are often misdiagnosed given the rarity of these tumors, but early diagnosis and initiation of treatment is essential. The unique presentation of our case demonstrates that clinical suspicion for cardiac angiosarcoma should be maintained for spontaneous pseudoaneurysm originating from the right atrium.

Treatment of retroperitoneal sarcoma results in improved outcomes.

OBJECTIVE: To report the clinical characteristics, treatments, and outcomes in a cohort of dogs with histologically confirmed retroperitoneal sarcoma (RPS) and to identify potential variables of prognostic significance. ANIMALS: 46 client-owned dogs from 10 clinics with histopathologic diagnosis of a sarcoma originating from the retroperitoneal space. METHODS: Medical records were retrospectively reviewed to obtain information regarding clinical characteristics, treatments, and outcomes. Recorded variables were analyzed to report descriptive data for all cases and overall survival time. Multivariate analysis was utilized to evaluate prognostic factors for overall survival. RESULTS: Hemangiosarcoma was the most common histologic subtype diagnosed (76.1%). Cytoreductive and curative intent surgical excision of the RPS was attempted in 12 and 22 dogs, respectively; 12 dogs underwent no surgery or had an exploratory laparotomy with incisional biopsy only. Nineteen dogs received adjuvant chemotherapy, either injectable or metronomic, and 1 dog received adjuvant radiation therapy. Fourteen of the 34 (41.2%) surgically treated dogs developed evidence of local recurrence, but there was no difference in local recurrence when comparing dogs categorized as curative intent versus cytoreductive surgery. The median overall survival time was 238 days. On multivariable analysis, treatment approach was associated with survival with surgical excision (vs palliative treatment) and adjuvant chemotherapy following surgery being protective against death. A diagnosis of hemangiosarcoma was associated with a greater hazard of death. CLINICAL RELEVANCE: This study demonstrates a substantially greater survival time than previously published and suggests a survival benefit from surgical excision and adjuvant chemotherapy.

Successful resection of a primary angiosarcoma of the azygos vein: A case report.

A 63-year-old woman was admitted to our department for the investigation of superior vena cava (SVC) syndrome. Computed tomography revealed an azygos tumor extending into the SVC. Video-assisted thoracic surgery (VATS) was performed to remove the distal end of the azygos vein in the left lateral position, followed by complete resection of the entire tumor under median sternotomy in the supine position. The histological diagnosis was a primary angiosarcoma of the azygos vein. The patient was discharged without any complications and is now alive and tumor-free 24 months after surgery. In addition, contrast-enhanced computed tomography revealed no graft occlusion in the two reconstructed brachiocephalic veins. Thoracoscopic surgery in the lateral position is useful for safe and reliable complete resection of a tumor arising from the azygos vein.

Chalazion-mimicked eyelid angiosarcoma in a young Asian with good prognosis: a case report.

BACKGROUND: Angiosarcoma is an extremely rare malignant tumor. So far, only about 42 cases of angiosarcoma involving the eyelids have been reported. Eyelid angiosarcoma occurs more frequently in elderly Caucasian males and is prone to misdiagnosis. We present a case report in a young Asian male patient with eyelid angiosarcoma that was misdiagnosed as a chalazion. CASE PRESENTATION: A 46-year-old South Korean male with no underlying disease had a right lower lid mass. The lesion was initially misdiagnosed as a chalazion at a local clinic, but a diagnosis of eyelid angiosarcoma was made after the first biopsy trial. PET-CT was performed to ensure that there was no metastasis in the whole body. Surgical excision with enough surgical margin was used alone for treatment and reconstruction was performed with a tarsoconjunctival advancement flap (modified Hughes procedure), which helped ensure good cosmesis. No recurrence was observed 4 years and 5 months after the surgery. CONCLUSIONS: The current study presents the first case of chalazion-mimicked eyelid angiosarcoma in a young Asian male aged under 50 years. This case shows that even if a benign eyelid disease is suspected in a young patient, an incisional biopsy must be performed to confirm whether the lesion is malignant. Since the prognosis is good for the case of eyelid angiosarcoma, if there is no clear evidence of distal metastasis, surgical resection should be performed with an enough safety margin.

Angiosarcoma of gallbladder, a literature review.

BACKGROUND: Angiosarcoma of the gallbladder is a rare diagnostic entity rarely encountered by pathologists and has rarely been reported in literature. This review aimed to examine the clinicopathological features, immunohistochemistry, treatment, and outcomes of gallbladder angiosarcoma. METHODS: A search of the PubMed, Science Direct and Google Scholar was done with the search terms ("angiosarcoma" OR "angiosarcomas") AND ("gallbladder" OR "gallbladders"). Based on inclusion and exclusion criteria, only case reports could be used for this review. RESULT: 8 case reports were chosen in the end for analysis. The mean age of the patients at presentation was 65 years. It was most frequently observed in males. Abdominal pain and palpable mass were the most commonly reported symptoms. Cholelithiasis and anemia were also reported. On histopathology morphologically epithelioid appearance of angiosarcoma was evident. Cytokeratin (CK) AE1/AE3, Von willebrand factor, Factor VIII antigen, Vimentin, CD31 were positive. Meanwhile, UEA, CD34, CD117, S-100, Keratin, EMA, and CEA showed negative outcome. Surgery was the preferred method of treatment and a mean 10-months follow-up was done. CONCLUSION: Despite the unavailability of convincing data, histological and immunohistochemical analyses play a major role in the diagnosis of gallbladder angiosarcoma. Nevertheless, more comprehensive clinical studies are required to provide universal guidelines for the treatment and diagnosis of angiosarcoma of the gallbladder.

Primary small intestine angiosarcoma mimicking Crohn's disease.

A man in his 40s presented to an emergency department after experiencing worsening abdominal pain for 2 days. Contrast-enhanced CT of the abdomen and pelvis revealed circumferential mural thickening and luminal narrowing of the distal ileum and upstream dilatation of the small intestine, indicating small intestine obstruction. This prompted emergency laparotomy, where two lesions in the distal ileum were identified as the source of his bowel obstruction and resected. Immunohistochemistry of the resected segment revealed a primary small intestine angiosarcoma acting positively for vascular markers ERG and CD31. A subsequent positron emission tomography (PET) scan revealed positive mediastinal metastatic lymphadenopathy without organ metastases.Following his surgery, the patient recovered well and was promptly referred to an oncology unit at a specialised health centre for further treatment. Primary small intestine angiosarcoma is a rare entity in which patients present with non-specific symptoms requiring prompt tissue diagnosis to facilitate multidisciplinary management.

Primary angiosarcoma of the Pancreas - A case Report and review of the literature.

BACKGROUND: Angiosarcomas are malignant neoplasms that originate from endothelial cells. The symptoms exhibit a non-specific nature, and achieving a preoperative diagnosis is frequently challenging. They are seldom encountered in the abdomen, and their occurrence in the pancreas is even rarer. METHODS: Here we document a 67-year-old man with pancreatic angiosarcoma and analyse the literature to outline the clinicopathologic characteristics of this rare phenomenon. RESULTS: This patient with family history of pancreas cancer presented with abdominal pain, and the CT-scan revealed a 4 cm mass at the neck of the pancreas but CA19-9 was normal. Radiologic findings were unusual for ordinary pancreas cancer. Fine-needle aspiration biopsy through endoscopic ultrasound revealed "undifferentiated malignant cells for which the diagnosis of "carcinoma" was favoured. Total pancreatectomy, splenectomy and portal vein reconstruction were performed and epithelioid angiosarcoma were diagnosed. Despite an uneventful postoperative period, discharge on postoperative day 8 without any complications, as well as diligent post-discharge clinical care, the patient died 65 days postoperatively, attributed to the presence of extensive metastasis. A comprehensive literature search has identified a limited number of documented cases of primary pancreatic angiosarcoma, with only ten cases reported to date. CONCLUSIONS: Pancreatic angiosarcomas are very rare and prone to misdiagnosis. The formation of a more demarcated but high-grade tumour with necrosis is a feature that distinguishes angiosarcomas from ordinary carcinomas of this organ. Pathologic diagnosis is also highly challenging closely resembling undifferentiated carcinomas. Angiosarcomas are highly aggressive when they occur in the pancreas. Prompt diagnosis at an early stage is crucial as surgery with curative intent serves as the primary treatment approach.

Surgery with curative intent is the mainstay treatment for pancreatic angiosarcoma when diagnosed at an early stage.Oncological treatment options should be taken into consideration according to the follow-up data.Why does this paper matter?This article is important in that it is the most comprehensive review of the literature on pancreatic angiosarcoma, which is a very rare pathology, from the perspective of radiology, pathology and surgery.

Clinical characteristics and treatment outcomes of angiosarcoma of the head and neck: A 17-year single-centre experience.

INTRODUCTION: Angiosarcomas in the head and neck region are aggressive tumours associated with high local recurrence and metastatic rates. We present our 17-year experience at the North of England Bone and Soft Tissue Tumour Service. METHODS: A retrospective review of our prospectively maintained database was undertaken, looking for patients diagnosed with angiosarcomas affecting the head and neck. Data were gathered using a pre-defined proforma to include demographics, histological characteristics, treatment modalities, adjuvant therapies, local recurrence, distant spread, and disease-specific survival. RESULTS: A total of 23 patients (17 males, 6 females) were identified, with a mean age of 76 years at presentation. Fourteen presented with scalp lesions, whereas the remainder arose on the face. Eighteen patients underwent resection with curative intent, whereas three received palliative radiotherapy and two received comfort-based care only. Of the patients undergoing surgery, 12 had local flap reconstruction and 6 underwent free tissue transfer. Clear resection margins were obtained in nine cases (50%). Fourteen patients (78%) presented with local recurrence after surgery, and 11 (61%) developed distant metastases. The median disease-specific survival time for patients treated with curative intent was 38 months. Eight patients had mapping biopsies ahead of their resection; however, complete resection was achieved in only two cases. DISCUSSION: Angiosarcomas of the head and neck are associated with a poor prognosis, with most patients rapidly developing local recurrence, resulting in adverse clinical outcomes. Mapping biopsies do not demonstrate a clear advantage for achieving complete surgical resection. A radical surgical approach is warranted, given the aggressiveness of the pathology. However, there remains no consensus on optimal surgical management; we recommend further synthesising studies to determine the most appropriate treatment pathway.

Pediatric primary intracranial angiosarcoma with epithelioid differentiation: a surgeon's dilemma.

Angiosarcoma is a rare form of soft tissue sarcoma originating from endothelial tissue, accounting for < 1% of all sarcomas. Primary epithelioid angiosarcomas of the central nervous system (CNS) are even more elusive, with only four reports described in the literature. In this article, we describe the first case in pediatric population, with a brief literature review regarding this entity. A 13-year-old girl presented to emergency services with raised intracranial pressure. MRI demonstrated a heterogenous lesion in the temporal lobe. She underwent emergency craniotomy and subtotal excision of the tumor. Eventually the patient developed multiple infarcts and succumbed post operatively. Pre-operative diagnosis on radiology is difficult considering the rarity of this entity and heterogeneity in radiological appearance. One needs to have a high degree of suspicion to consider angiosarcoma as a radiological differential. Overall prognosis remains poor. Early adjuvant treatment may improve overall survival.

Radiation-induced angiosarcoma of the breast: retrospective analysis at a regional treatment centre.

BACKGROUND: Radiation-induced angiosarcoma (RIA) is an uncommon but morbid complication after radiotherapy for breast cancer. METHODS: Retrospective analysis of breast RIA patients at Cambridge University Hospital (CUH), a regional treatment centre in the East of England. RESULTS: 22 patients were identified between 2010 and 2022. Median age of diagnosis was 65 years (range 41-78). Median time from breast radiotherapy to RIA diagnosis was 6.5 years (range 2.4-16.0)-this interval has decreased over the last 24 years (r2 = 0.6601). 9% had metastasis at presentation. All patients underwent surgery (55% at CUH, 45% at local hospitals). 27% received peri-operative pegylated liposomal doxorubicin in the first-line setting. 62% relapsed following their primary curative-intent treatments after a median of 28 months. Metastases occurred in 36%, the commonest sites being lung (100%) and lymph node (50%). 2-year and 5-year overall survival (OS) rates for all patients were 73% and 60%, respectively. No correlation between progression-free survival (PFS) and OS was found with tumour size, margin, peri-operative chemotherapy, and whether surgery was performed at CUH. Patients with multifocal disease on their breasts had shorter PFS following surgery compared to single-lesion disease (median 10 vs 65 months; HR = 4.359 [95% CI 1.342-14.16]; P = 0.0143). Patients aged > 72 years had a median OS of 45 months vs 102 months for those ≤ 72 years (HR = 7.129 [95% CI 1.646-30.88]; P = 0.0086). CONCLUSION: RIA has high rates of recurrence and mortality and appears to be occurring sooner after breast radiotherapy. Further studies on its pathogenesis and effective treatment are warranted.

Cardiac angiosarcoma: a case report.

Angiosarcoma is the most invasive and malignant cardiac tumor and most commonly originates from the right atrium. Early diagnosis is essential, and echocardiography has an important role in diagnosis. This tumor grows aggressively, and metastases to other sites makes it difficult to control. Surgical treatment remains the best option for patients who do not respond to chemoradiotherapy. We herein report a case of a 17-year-old patient with cardiac angiosarcoma who presented with dyspnea, chest pain, dry cough, and fever. Although we considered the most probable diagnosis to be constrictive pericarditis, pathologic examination revealed a primary angiosarcoma originating from the pericardium. The patient underwent total pericardiectomy. However, despite receiving chemotherapy for 2 weeks postoperatively, she developed complications including leukopenia and eventually died of respiratory failure. Late diagnosis of angiosarcoma often occurs, resulting in progression to end-stage disease and a very poor prognosis. Therefore, a thorough understanding of this entity, knowledge of its pitfalls in management, and establishment of an accurate treatment guideline would help to develop a reliable and life-saving treatment approach for these patients.