Primary Renal Epithelioid Angiosarcoma

Waqas, Muhammad; Rahim, Waqas; Shohab, Durre; Khawaja, Muhammad Athar; Zafar, Atif; Mamoon, Nadira

doi:10.29271/jcpsp.2018.03.s66

Cited by 6 publications

(8 citation statements)

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“…Angiosarcoma is a malignant vascular neoplasm which may occur in any location of the body, including the skin, soft tissue, breast, bone and visceral organs including the liver. Primary renal angiosarcoma is very rare, with about 70 cases described in the literature to date [49][50][51][52][53][54][55][56][57]. Most of these cases are seen in older men, with a peak incidence in the sixth to seventh decades and a pronounced male predilection (male-to-female ratio of 7:1) [49].…”

Section: Angiosarcomamentioning

confidence: 99%

“…CT imaging demonstrates a hypodense mass with varying levels of contrast enhancement ranging from none, to heterogeneous, to intense peripheral, to diffuse enhancement [53,54,58,59]. Central areas of necrosis or hemorrhage may be detected [55]. MRI studies have shown intermediate to high signal intensity on T1-weighted images, and some cases show alternating areas of high and low signal intensity on T2-weighted images, creating a striated pattern corresponding to alternating necrotic and solid areas of the tumor [59].…”

Section: Angiosarcomamentioning

confidence: 99%

“…Generally, a diffusely infiltrative growth pattern is seen, with some cases displaying invasion of the perinephric adipose tissue [49]. On immunohistochemistry, the neoplastic cells should show positivity for at least one endothelial marker (CD34, CD31, ERG, FLI-1) and are negative for epithelial markers; however, cases of epithelioid angiosarcoma have been shown to stain positively for keratins [2,49,55]. The Ki-67 proliferative index is usually very high, ranging from 30% to over 80% [51].…”

Section: Angiosarcomamentioning

confidence: 99%

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Vascular Neoplasms and Related Lesions of Kidney

Lin¹,

Ro²

2020

ann urol oncol

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Vascular tumors arising in the kidney are distinctly uncommon occurrences and have not been well-characterized in the literature; most cases have been documented in single reports or small case series. These tumors often exhibit nonspecific clinical and radiologic features and may mimic malignant epithelial tumors such as renal cell carcinoma (RCC) on imaging studies, necessitating nephrectomy with pathologic examination for definitive diagnosis. In addition, since primary renal vascular tumors are rare and infrequently encountered in surgical pathology specimens, they are often not considered in the differential diagnosis and therefore may be systematically under-diagnosed. In this review, we discuss the clinical and morphologic features of benign, borderline, and malignant vascular neoplasms and related lesions arising in the kidney, with a focus on recent, clinically relevant advances in imaging, histopathologic diagnosis, and treatment.

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Section: Angiosarcomamentioning

confidence: 99%

Section: Angiosarcomamentioning

confidence: 99%

Section: Angiosarcomamentioning

confidence: 99%

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Vascular Neoplasms and Related Lesions of Kidney

Lin¹,

Ro²

2020

ann urol oncol

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“…On the other hand, primary renal epithelioid angiosarcoma; which mimics epithelial tumors, both morphologically and immunohistochemically, is an extremely rare and aggressive malignancy with a rapidly metastatic and fatal course, which presents a great diagnostic challenge as it mimics RCC, and it is associated with rapid local and bloodstream dissemination. Consequently, patients usually have advanced disease when diagnosed and a high risk for recurrence (3). Lastly, primary well-differentiated renal neuroendocrine tumors (carcinoid) area also exceedingly rare neoplasms, considered a distinct entity within the latest World Health Organization Classification scheme (4), with histological features identical to carcinoid tumors found at other anatomical locations (5).…”

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confidence: 99%

Rarely Described Renal Malignancies Associated With Venous Tumor Thrombus

et al. 2022

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Background/Aim: Collecting duct carcinoma, epithelioid angiosarcoma and neuroendocrine/carcinoid tumor are uncommon renal malignancies, and their association with tumor thrombus extending into the inferior vena cava is extremely rare. Owing to the rarity of the above-mentioned malignancies and short follow-up of the cases published in the literature, the prognosis and clinical behavior of these tumors remains unclear. Up to date, the culprit of treatment is surgical management with radical nephrectomy, lymph node dissection, thrombectomy and vascular reconstruction if necessary. Patients and Methods: We herein describe in detail the first cases published of the above-mentioned renal malignancies associated with extensive inferior vena cava (IVC) thrombus, in which complex vascular reconstruction was performed. Results: Three male patients were identified as having collecting duct carcinoma, epithelioid angiosarcoma and neuroendocrine/ carcinoid tumor with IVC involvement. Tumor thrombus levels were II, I and IIIc respectively. Patient ages were 42, 60 and 47 years and tumor sizes were 9.2, 10.9 and 3.7 cm correspondingly. Patient 2 underwent cavectomy, IVC replacement using polytetrafluoroethylene (Gore-Tex ® ) vascular graft and IVC filter deployment inside the graft. None of the patients developed any pulmonary emboli postoperatively. At the last follow-up, IVC graft for patient 2 remained patent. Conclusion: Owing to the rarity of the aforementioned malignancies and short follow-up of cases

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“…metastasis and tumor size (11,12). The mainstay of treatment of PRA is surgical resection (12). Sequential chemotherapy with/without radiotherapy may be applied but the survival rate remains low (13).…”

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confidence: 99%

Primary Angiosarcoma of the Kidney: Literature Review of a Rare Nosologic Entity

et al. 2020

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Primary angiosarcoma of the kidney is a rare tumor, hence little is known concerning its diagnostic features and therapeutic management. We conducted this survey to present a complete literature review with emphasis on clinicopathological features, diagnosis and therapy. A thorough search was conducted in MEDLINE/PubMed. All relevant studies concerning primary renal angiosarcomas in adults were thoroughly reviewed. Primary renal angiosarcoma is characterized by an overall poor prognosis, is of unknown etiology and occurs most commonly in males between 60 and 70 years old. Presence of distant metastasis at the time of diagnosis is prevalent. Histopathological examination and immunohistochemical studies are the most important diagnostic tools. Treatment options include surgery, chemotherapy, radiotherapy and immunotherapy. Conclusion: Primary renal angiosarcoma is a rare but aggressive malignancy with low response to available therapeutic regimens and dismal survival rates.Angiosarcoma is an extremely rare and aggressive malignant tumor occurring in fewer than 2% of all soft-tissue sarcomas (1). Primary angiosarcoma arises most commonly from the skin, superficial soft tissue, liver, spleen, bone, and breast (2, 3). An exceedingly infrequent location is the kidney, which accounts for 1% of the total of angiosarcomas diagnosed (4). Primary renal angiosarcoma (PRA) has an overall dismal prognosis, with an estimated average life expectancy of 6 months. It is also referred to as renal hemangiosarcoma and was first described in 1942, with few reported cases since then. Its etiology is still unknown and its predisposing factors remain ill-defined (5, 6). However, case reports show development of PRA in patients with pre-existing renal angiomyolipoma or multicystic kidney disease (7,8). White males between 60-70 years old are most commonly affected (9). Presenting symptoms in the majority of patients include flank pain, hematuria, anemia and palpable mass (3).Initial diagnostic approach involves abdominal computed tomography (CT) and magnetic resonance imaging which may reveal a solitary renal mass. Further total body scan, which is indicated for TNM staging, may unveil distant metastatic lesions in the lungs, liver, bone, spleen, abdominal lymph nodes, peritoneum, or soft tissues (3). Histopathologically, it is defined by an angiomatous structure composed of atypical proliferation of endothelial cells, with epithelioid, spindle or histiocytoid characteristics, and anastomotic vascular channels (10). Biopsy and cytological characteristics combined with a positive immunohistochemical staining for CD31, CD34, factor VIII, vimentin, and friend leukemia integration 1 (FLI1) are the gold standard methods for the definitive diagnosis of PRA (6, 10).

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Primary Renal Epithelioid Angiosarcoma

Cited by 6 publications

References 0 publications

Vascular Neoplasms and Related Lesions of Kidney

Vascular Neoplasms and Related Lesions of Kidney

Rarely Described Renal Malignancies Associated With Venous Tumor Thrombus

Primary Angiosarcoma of the Kidney: Literature Review of a Rare Nosologic Entity

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