Primary renal angiosarcoma: a diagnostic and therapeutic challenge

Lodhi, Hanan T.; Inayat, Faisal; Munir, Asma; Ilyas, Ghulam

doi:10.1136/bcr-2018-225484

Cited by 4 publications

(5 citation statements)

References 24 publications

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“…Based on limited case reports, initial routine laboratory blood tests mainly show the presence of anemia while remaining blood tests and tumor marker levels, including that of carcinoembryonic antigen (CEA), are within standard limits. Apart from confirmed hematuria, urinalysis is normal (6,33,34). Ultrasound, revealing a solid renal mass with heterogeneous echo, cannot reliably differentiate benign from malignant behavior nor histological subtypes of the tumor (30,35,36).…”

Section: Diagnostic Approachmentioning

confidence: 99%

“…PRA is commonly depicted as a well-demarcated heterogeneous renal mass with rapid peripheral enhancement on corticomedullary phase. It is also characterized by fast wash out of the contrast agent followed by delayed central filling on nephrographic or excretory phase (6,13,33,38,39). Interestingly, in contrast to sarcomas, RCC has the pathological tendency to invade the renal vein or inferior vena cava (40).…”

Section: Diagnostic Approachmentioning

confidence: 99%

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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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Section: Diagnostic Approachmentioning

confidence: 99%

Section: Diagnostic Approachmentioning

confidence: 99%

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

et al. 2020

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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%

“…The most common sites of metastasis were lung and liver, but reports of metastasis to the bone, soft tissue, peritoneum, spleen, and lymph nodes have also been documented [49]. Important prognostic factors include the presence of metastatic disease at presentation and tumor size, with patients whose tumors were less than 5 cm generally demonstrating a higher survival rate [52,57]. The management of renal angiosarcoma is controversial and not wellestablished; most patients were treated with radical nephrectomy followed by varying combinations of radiation and chemotherapy [51].…”

Section: Angiosarcomamentioning

confidence: 99%

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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“…Still, others like angiosarcoma, collecting duct carcinoma, renal medullary carcinoma, and sarcomatoid variants have a poorer prognosis. [4, 5] The aim of the present study is to analyze the incidence, clinico-pathological features, surgical treatment, and survival of non-clear cell RCC at our institution.…”

Section: Introductionmentioning

confidence: 99%

Oncological outcomes of primary renal malignancies other than clear cell renal carcinoma: A retrospective study from a tertiary center

Mehra

Manikandan

Narayanan

et al. 2020

Preprint

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IntroductionA lot of research is available about clear cell renal carcinomas (ccRCC). But there are lesser known facts about other subtypes of renal malignancies. With advances in immunohistochemical and cytogenetic techniques, new variants of renal tumors are being increasingly reported. The treatment and prognosis of such rare malignancies is still an enigma. We performed this study to analyze the incidence, clinico-pathological features, surgical treatment, and survival of non-clear cell RCC at our institution.Materials and MethodsThe histopathological reports of 77 Nephrectomy specimens who underwent surgical treatment for suspected renal tumors from 2013-2018 were retrospectively reviewed. 19 (24%) of patients had documented uncommon histologic variants of RCC. The clinical, demographic, and histologic characteristics of these patients were analyzed, and survival was evaluated. The characteristic light microscopy and immunohistochemical features of these lesions were documented.ResultsMean age was 45 years (21-67 years). Out of 19 patients, 14 (73.6%) were males, and 5 (26.4%) were females. Mean tumor size was 12 (6-31) cm in the largest dimension. 17 (22%) patients underwent radical nephrectomy, and 2 (2.5%) were treated with partial nephrectomy. Patients with collecting duct, synovial sarcoma, and primitive neuro-ectodermal tumor (PNET) had associated inferior vena caval thrombus and underwent venous thrombectomy. Adjuvant treatment in the form of chemotherapy was instituted in collecting duct, adult Wilms and pure sarcomas. There was no mortality in the papillary carcinoma, and the worst prognosis was encountered inConclusionSarcomatoid and collecting duct variants were associated with worse prognosis. Presently, aggressive surgical extirpation is the mainstay in the management of these histologic variants. Adjuvant chemotherapy and TKI inhibitors have a limited role.

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Primary renal angiosarcoma: a diagnostic and therapeutic challenge

Cited by 4 publications

References 24 publications

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

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

Vascular Neoplasms and Related Lesions of Kidney

Oncological outcomes of primary renal malignancies other than clear cell renal carcinoma: A retrospective study from a tertiary center

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