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Omiyale AO. Primary vascular tumours of the kidney. World J Clin Oncol 2021; 12:1157-1168. [PMID: 35070735 PMCID: PMC8716994 DOI: 10.5306/wjco.v12.i12.1157] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/28/2021] [Revised: 07/01/2021] [Accepted: 11/25/2021] [Indexed: 02/06/2023] Open
Abstract
Primary vascular tumours of the kidney are rare and may pose diagnostic difficulties because of their similar clinical, morphological, and immunohistochemical features. This article summarizes the clinical and pathological features of primary renal angiosarcoma and anastomosing haemangioma of the kidney including epidemiology, genetics, and prognosis. Renal anastomosing haemangiomas are benign neoplasms characterized by anastomosing capillary-sized vascular channels. These tumours are rare, with about 75 cases reported in the literature. Most anastomosing haemangiomas are found incidentally on ultrasound, computed tomography, or magnetic resonance imaging. Common symptoms include abdominal pain, haematuria, and abdominal mass. Renal anastomosing haemangiomas are characterized by recurrent mutations in GNAQ and GNA14 genes. The prognosis of anastomosing haemangioma is excellent. Primary renal angiosarcomas are malignant tumours showing endothelial differentiation. To date, 76 cases have been described in the literature. Primary renal angiosarcomas are frequently symptomatic. The clinical features of renal angiosarcomas are similar to those of renal anastomosing haemangiomas, including abdominal pain, haematuria, and abdominal mass. Angiogenesis-related genes and vascular-specific receptor tyrosine kinases such as KDR, TIE1, SNRK, TEK, and FLT1 are upregulated in angiosarcomas. Primary renal angiosarcomas are highly aggressive neoplasms with a poor prognosis despite surgical treatment, chemotherapy, radiotherapy, or targeted therapy.
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Affiliation(s)
- Ayo O Omiyale
- Department of Cellular Pathology, Imperial College Healthcare NHS Trust, Charing Cross Hospital, London W6 8RF, United Kingdom
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2
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Boni A, Cochetti G, Sidoni A, Bellezza G, Lepri E, Giglio AD, Turco M, Vermandois JARD, Zingaro MD, Cirocchi R, Mearini E. Primary Angiosarcoma of the Kidney: Case Report and Comprehensive Literature Review. Open Med (Wars) 2019; 14:443-455. [PMID: 31410364 PMCID: PMC6689208 DOI: 10.1515/med-2019-0048] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/14/2019] [Accepted: 04/24/2019] [Indexed: 02/01/2023] Open
Abstract
PURPOSE Angiosarcoma (AS) is an aggressive malignant tumour of endothelial origin, most frequently compelling the skin. The kidney is a rare site of primary occurrence of AS with a quite challenging diagnosis and poor prognosis, mainly due to its raw metastasising power. We described our first case of AS treatment. A comprehensive literature review in this field is also carried out. PATIENTS AND METHODS We report the case of a 56-years-old man with radiological appearance of an 11 cm poor vascularised left renal mass. The pre-operative imaging ruled out distant metastases and inferior vena cava thrombosis. Thus, after radioembolization, we performed a transperitoneal open radical nephrectomy with regional lymphadenectomy with the removal of the middle portion of the ileo-psoas muscle, followed by two lines of adjuvant chemotherapy. We analysed all the papers concerning primary kidney AS until December 2018. RESULTS Estimated intraoperative blood loss was 200 mL with an operative time of 100 minutes. No postoperative complications were recorded, with the patient discharge in the 8th postoperative day. The pathological assessment showed a pT3a N0, M0 tumour compatible with AS of the kidney. An immune-histochemical study showed a vascular positive for CD31, CD34, Fli-1 with a Ki-67/Mib 1 of 50%. One month after surgery the patient began adjuvant chemotherapy with gemcitabine. After 45 days from surgery, a Computed Tomography scan showed lung and liver metastases with disease relapse in the left kidney lodge. The response to the first-line treatment was excellent, unlike the second-line, encountering a chemo-refractory disease. The cancer-specific survival was of 15 months. We included 66 cases in our review. CONCLUSION This is the first case of treatment of kidney AS performed at our Institute. Our findings added new information about the unclear biology and progression of this subset of tumours, demonstrating an abysmal prognosis.
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Affiliation(s)
- Andrea Boni
- Department of Surgical and Biomedical Sciences, Urologic Clinic, University of Perugia, University Hospital of Perugia, Perugia, Italy
| | - Giovanni Cochetti
- Department of Surgical and Biomedical Sciences, Urologic Clinic, University of Perugia, University Hospital of Perugia, Perugia, Italy
| | - Angelo Sidoni
- Department of Experimental Medicine, Section of Anatomic Pathology and Histology, Perugia Medical School, University of Perugia, Perugia, Italy
| | - Guido Bellezza
- Department of Experimental Medicine, Section of Anatomic Pathology and Histology, Perugia Medical School, University of Perugia, Perugia, Italy
| | - Emanuele Lepri
- Department of Surgical and Biomedical Sciences, Urologic Clinic, University of Perugia, University Hospital of Perugia, Perugia, Italy
| | - Andrea De Giglio
- Department of Experimental Medicine, Section of Oncology, University of Perugia, Perugia, Italy
| | - Morena Turco
- Department of Surgical and Biomedical Sciences, Urologic Clinic, University of Perugia - S.M. della Misericordia Hospital, P.le Menghini 1, 06132, Perugia, Italy
| | - Jacopo Adolfo Rossi De Vermandois
- Department of Surgical and Biomedical Sciences, Urologic Clinic, University of Perugia, University Hospital of Perugia, Perugia, Italy
| | - Michele Del Zingaro
- Department of Surgical and Biomedical Sciences, Urologic Clinic, University of Perugia, University Hospital of Perugia, Perugia, Italy
| | - Roberto Cirocchi
- Department of Surgical and Biomedical Sciences, Urologic Clinic, University of Perugia, University Hospital of Perugia, Perugia, Italy
| | - Ettore Mearini
- Department of Surgical and Biomedical Sciences, Urologic Clinic, University of Perugia, University Hospital of Perugia, Perugia, Italy
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Abstract
Primary renal angiosarcoma is an exceedingly rare malignancy of the kidney. It usually presents in the elderly with metastatic disease and dismal prognosis. Treatment protocols are not standardized for this very rare renal malignancy. We report the case of a young man who was incidentally diagnosed with primary renal angiosarcoma. Preoperative imaging was suggestive of renal cell carcinoma; however, postoperative histopathological examination confirmed it to be an angiosarcoma. The patient was treated with surgical excision followed by adjuvant chemotherapy and is doing well at one-year follow-up.
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Affiliation(s)
- Danny Darlington
- Urology, Government Stanley Medical College and Hospital, Chennai, IND
| | - Fatima Shirly Anitha
- Pediatrics, Church of South India Kalyani Multispeciality Hospital, Chennai, IND
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Lodhi HT, Inayat F, Munir A, Ilyas G. Primary renal angiosarcoma: a diagnostic and therapeutic challenge. BMJ Case Rep 2018; 2018:bcr-2018-225484. [PMID: 30244222 DOI: 10.1136/bcr-2018-225484] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/22/2022] Open
Abstract
Primary renal angiosarcoma is an exceedingly rare and aggressive neoplasm. Although it may occur in youth, this tumour is frequently reported in the sixth and seventh decades of life. The clinical presentation is frequently varied. Pathogenesis remains largely unknown and it has overlapping features with other tumours of the kidney. Current treatment options include variable combinations of surgery, chemotherapy and radiotherapy. Reports regarding the disease prognosis and natural history are limited. In this article, we chronicle the case of a patient with primary renal angiosarcoma presenting at an advanced stage as a widely metastasised tumour. Additionally, we undertake here a brief literature review highlighting the rarity and aggressiveness of this condition, its poor prognosis, and the lack of specific management guidelines.
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Affiliation(s)
| | | | - Ahmed Munir
- Services Institute of Medical Sciences, Lahore, Punjab, Pakistan
| | - Ghulam Ilyas
- SUNY Downstate Medical Center, Brooklyn, New York, USA
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Guan H, Zhang L, Zhang Q, Qi W, Xie S, Hou J, Wang H. Primary angiosarcoma arising in an angiomyolipoma of the kidney: case report and literature review. Diagn Pathol 2018; 13:53. [PMID: 30111336 PMCID: PMC6094454 DOI: 10.1186/s13000-018-0730-z] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/24/2018] [Accepted: 07/30/2018] [Indexed: 12/25/2022] Open
Abstract
BACKGROUND Primary angiosarcoma of the kidney is a rare and aggressive malignant tumor presenting with a recognizable vascular differentiation. Its prognosis is fatal and the pathogenesis remains unclear. Renal angiomyolipoma is a relatively infrequent renal cortical neoplasm and is composed of variable proportions of adipose tissue, spindle cells, epithelioid smooth muscle cells and abnormal thick-walled blood vessels. CASE PRESENTATION Here, we reported a case in which a 64-year-old woman presenting with the chief complaint of a progressively enlarged mass in the left abdomen. Abdominal computed tomography confirmed presence of a tumor measuring 18 cm × 11 cm in the left posterior renal fascia. Microscopic examination and immunohistochemical staining confirmed co-existence of angiomyolipoma and angiosarcoma. Furthermore, the two components interspersed with each other and there were transitional zones between them. CONCLUSIONS In this case, we described for the first time a primary renal angiosarcoma possibly arising in a pre-existing angiomyolipoma of the kidney.
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Affiliation(s)
- Hongwei Guan
- Department of Pathology, Dalian Medical University First Hospital, 222 Zhongshan Road, Xigang District, Dalian, 116011, Liaoning, China
| | - Lizhi Zhang
- Department of Pathology, Dalian Medical University First Hospital, 222 Zhongshan Road, Xigang District, Dalian, 116011, Liaoning, China
| | - Qiuping Zhang
- Department of Pathology, Dalian Medical University First Hospital, 222 Zhongshan Road, Xigang District, Dalian, 116011, Liaoning, China
| | - Wenjing Qi
- Department of Pathology, Dalian Medical University First Hospital, 222 Zhongshan Road, Xigang District, Dalian, 116011, Liaoning, China
| | - Suling Xie
- Department of Pathology, Dalian Medical University First Hospital, 222 Zhongshan Road, Xigang District, Dalian, 116011, Liaoning, China
| | - Jinping Hou
- Department of Pathology, Dalian Medical University First Hospital, 222 Zhongshan Road, Xigang District, Dalian, 116011, Liaoning, China
| | - Huali Wang
- Department of Pathology, Dalian Medical University First Hospital, 222 Zhongshan Road, Xigang District, Dalian, 116011, Liaoning, China.
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Abstract
PURPOSE OF REVIEW Primary angiosarcoma of the kidney is extremely rare; hence, relatively little is known regarding its clinicopathologic features and prognosis. Herein, we review the literature on primary renal angiosarcoma with emphasis on the clinical and pathologic features. RECENT FINDINGS Approximately 64 cases have been reported in the literature, and most cases occur in the 6th-7th decade with a strong male predominance. The aetiology is unknown. Patients present with flank pain, haematuria, abdominal mass and weight loss. A considerable number of patients develop metastatic disease at diagnosis or shortly afterwards. Grossly, the tumour comprises ill-defined haemorrhagic spongy masses often with necrosis. Microscopically, the tumour is composed of anastomosing capillary-sized vessels which are lined by malignant endothelial cells. The mainstay of treatment is surgery followed by radiation therapy with or without chemotherapy. Renal angiosarcomas are highly aggressive tumours with dismal outcome, and they must be distinguished from morphologically similar lesions of the kidney.
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Affiliation(s)
- Ayo O Omiyale
- Department of Cellular Pathology, Imperial College Healthcare NHS Trust, London, UK.
| | - James Carton
- Department of Cellular Pathology, Imperial College Healthcare NHS Trust, London, UK
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Iannaci G, Crispino M, Cifarelli P, Montella M, Panarese I, Ronchi A, Russo R, Tremiterra G, Luise R, Sapere P. Epithelioid angiosarcoma arising in schwannoma of the kidney: report of the first case and review of the literature. World J Surg Oncol 2016; 14:29. [PMID: 26842370 PMCID: PMC4739400 DOI: 10.1186/s12957-016-0789-5] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/05/2015] [Accepted: 01/26/2016] [Indexed: 12/24/2022] Open
Abstract
Background Schwannoma and angiosarcoma are infrequent pathologies that have been rarely reported in the kidney. Angiosarcoma is an uncommon malignant tumor presenting a recognizable vascular differentiation. It can develop in any site but the most common locations include the skin, soft tissues, breast, bone, liver, and spleen while renal localization has been very rarely reported in the literature. Schwannoma is a benign peripheral nerve sheath tumor composed of cells with the immunophenotype and ultrastructural features of differentiated Schwann cells. It has a wide anatomical distribution but the most frequent locations include subcutaneous tissues of the extremities and the head and neck region and the retroperitoneal and mediastinal soft tissues. The occurrence of an angiosarcoma in a pre-existing schwannoma is an extremely rare event with <20 cases reported in worldwide literature. In the present study, a renal case of angiosarcoma arising in schwannoma is presented with a detailed review of the pertinent literature. Case Presentation A 56-year-old man was admitted with a few days history of lower back pain and hematuria. Abdominal ultrasound showed a mass inside the left renal medulla. Subsequent imaging investigations with computed tomography and magnetic resonance confirmed the presence of the lesion and showed a pulmonary metastasis. Conclusions The final histopathological examination led to the diagnosis of epithelioid angiosarcoma arising in a schwannoma. The patient came to death a few months later due to a massive hemothorax. To the best of our knowledge, the present is the first case of an angiosarcoma arising in a schwannoma of the kidney.
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Affiliation(s)
- G Iannaci
- Division of Pathology, S. Maria del Popolo degli Incurabili Hospital ASL Na1, Naples, Italy.
| | - M Crispino
- Division of Urology, S. Maria del Popolo degli Incurabili Hospital ASL Na1, Naples, Italy
| | - P Cifarelli
- Division of Urology, S. Maria del Popolo degli Incurabili Hospital ASL Na1, Naples, Italy
| | - M Montella
- Division of Pathology, School of Medicine, University Federico II, Naples, Italy
| | - I Panarese
- Division of Pathology, School of Medicine, University Federico II, Naples, Italy
| | - A Ronchi
- Division of Pathology, School of Medicine, University Federico II, Naples, Italy
| | - R Russo
- Division of Pathology, School of Medicine, University Federico II, Naples, Italy
| | - G Tremiterra
- Division of Pathology, School of Medicine, University Federico II, Naples, Italy
| | - R Luise
- Division of Pathology, School of Medicine, University Federico II, Naples, Italy
| | - P Sapere
- Division of Pathology, S. Maria del Popolo degli Incurabili Hospital ASL Na1, Naples, Italy
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Omiyale AO. Clinicopathological features of primary angiosarcoma of the kidney: a review of 62 cases. Transl Androl Urol 2016; 4:464-73. [PMID: 26816844 PMCID: PMC4708591 DOI: 10.3978/j.issn.2223-4683.2015.05.04] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/01/2023] Open
Abstract
Angiosarcoma (AS) is a malignant tumor occurring in less than 2% of soft tissue sarcomas. Primary involvement of the kidney is rare, its pathogenesis remains largely unknown and it has overlapping features with other tumors of the kidney. The objective of this paper is to review the case reports of primary AS of the kidney in the literature. The search terms were primary AS of the kidney, primary renal AS and primary renal hemangiosarcoma. The total cohort of the cases reviewed was 62. The mean age of presentation was 61 years old with a predilection for the male sex. Metastatic disease at the time of diagnosis accounted for 44.9% (22/49) of the cases reported and 44.4% (12/27) of patients with non-metastatic disease at diagnosis, subsequently developed metastasis. Primary AS of the kidney is a rare malignant tumor with a poor prognosis. Local recurrence and distant metastasis is common. Primary AS of the kidney shares similar clinical presentation with other renal tumors and imaging does not allow for tumor specific diagnosis. Histopathological examination and immunohistochemistry is very important for the confirmation of the diagnosis. Current treatment options include a variable combination of surgery, radiotherapy and chemotherapy.
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Iacovelli R, Orlando V, Palazzo A, Cortesi E. Clinical and pathological features of primary renal angiosarcoma. Can Urol Assoc J 2014; 8:E223-6. [PMID: 24839487 DOI: 10.5489/cuaj.1585] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/28/2022]
Abstract
INTRODUCTION We review all cases of renal angiosarcoma described in the medical literature to describe its clinical and histological features. METHODS The search term was "angiosarcoma of the kidney." All articles or case reports in English, French, German or Spanish published until March 15, 2013 were considered. When available, data that focused on clinical and pathological features were extracted. RESULTS In total, the final cohort included 42 cases. The median overall survival (OS) was 5.0 months (95% confidence interval [CI], 2.1-7.9). Patients with metastatic disease had more than a threefold increase in the risk of death compared to patients without meta-static disease (hazard ratio: 3.27, 95% CI, 1.48-7.24; p = 0.004). Non-metastatic patients had dismal disease-free survival (DFS) rates, with a median DFS of 6.0 months (95% CI, 4.3-7.7); despite this, chemotherapy was effective to increase survival in eligible patients (4.0 vs. 1.0 months; p = 0.001). Microscopic examination found epithelioid and spindled cell in 44% and 56% of cases, respectively; there was a statistically insignificant increase in survival in patients with epithelioid patterns compared to spindled ones (9.0 vs. 4.0 months; p = 0.077). The tumour grading was related to OS; the lower the grade, the longer the survival (13 vs. 4 months; p = 0.029). CONCLUSIONS Renal angiosarcoma is a rare disease with defined clinical and pathological features. It has a very poor prognosis in patients with metastatic disease and a high recurrence rate in patients with non-metastatic disease.
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Affiliation(s)
- Roberto Iacovelli
- Sapienza University of Rome; Department of Radiology, Oncology and Human Pathology, Oncology Unit B, Rome, Italy
| | - Valentina Orlando
- Sapienza University of Rome; Department of Radiology, Oncology and Human Pathology, Oncology Unit B, Rome, Italy
| | - Antonella Palazzo
- Sapienza University of Rome; Department of Radiology, Oncology and Human Pathology, Oncology Unit B, Rome, Italy
| | - Enrico Cortesi
- Sapienza University of Rome; Department of Radiology, Oncology and Human Pathology, Oncology Unit B, Rome, Italy
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10
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Abstract
Primary angiosarcoma of the kidney is a rare tumor with only a few case reports in the literature. Management is not standardized and the prognosis is poor. However, clinicians need to be aware of this uncommon entity.
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Affiliation(s)
- Sagar Sabharwal
- Department of Urology, Christian Medical College, Vellore, Tamil Nadu, India
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Chaabouni A, Rebai N, Chabchoub K, Fourati M, Bouacida M, Slimen MH, Bahloul A, Mhiri MN. Primary renal angiosarcoma: Case report and literature review. Can Urol Assoc J 2013; 7:E430-2. [PMID: 23826057 DOI: 10.5489/cuaj.1396] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
Angiosarcoma is a rare malignant tumour occurring in less than 2% of soft tissue sarcomas. Angiosarcoma involving the kidney usually represents metastasis from skin or visceral primary lesions, while angiosarcoma primarily occurring in the kidney is a very rare neoplasm. We report a case of angiosarcoma of the right kidney in a 59-year-old male. The computed tomography scan showed a solid tumour with a low increased density after administration of contrast medium. Histological examination of the piece of nephrectomy confirmed the diagnosis.
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Affiliation(s)
- Ahmed Chaabouni
- Department of Urology, Habib Bourguiba University Hospital, Tunisia
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