En bloc resection of visceral aorta and right kidney due to aortic sarcoma using temporary extracorporeal bypass grafting

There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

Abstract

Aortic sarcomas have not been linked to Lynch syndrome in humans, although other soft tissue malignancies have been. We report the case of a 31-year-old man with Lynch syndrome, who presented with abdominal pain and severe claudication. The clinical and diagnostic workup revealed near occlusion of the infrarenal aorta due to aortic angiosarcoma. En bloc resection of the visceral and infrarenal aorta with right nephrectomy was performed, facilitated by temporary extracorporeal bypass to the visceral arteries. The aorta was reconstructed with a bifurcated Dacron graft. At the 24-month follow-up examination, the patient was free of disease but was experiencing chronic diarrhea.

Related collections

Most cited references 15

Record: found
Abstract: found
Article: not found

Undifferentiated intimal sarcoma of large systemic blood vessels: report of 14 cases with immunohistochemical profile and review of the literature.

Andrew Ricci, Sakari Knuutila, Peter Pytel … (2005)

Intimal sarcoma (IS) is defined as a malignant tumor arising in the tunica intima of large blood vessels. In systemic circulation, the majority of IS develop in the aorta, where close to three fourths of published cases lack specific differentiation and are called undifferentiated intimal sarcomas (UIS). The remaining cases are intima-associated sarcomas of recognized types, also called differentiated intimal sarcomas (DIS). In this report, we further characterize UIS, including its immunohistochemical profile and results of comparative genomic hybridization. A total of 14 cases of UIS were collected from 17 medical institutions, including slides, blocks, electron photomicrographs, clinical abstracts, and reports of surgical pathology specimens and autopsies. The patients, 7 women and 7 men, were 41 to 85 years of age (median, 65.6 years). Twelve tumors arose from the aorta, one from the left external iliac and femoral arteries, and one in a large systemic vein (the venous tumor was included due to histologic similarity with the arterial lesions). Tumors ranged from 1 cm to over 10 cm in diameter. Histopathology was that of a largely necrotic, poorly differentiated epithelioid and pleomorphic malignant neoplasm relating to the tunica intima. Usually there was only a thin layer of viable tumor cells overlying a large thrombus. All tumors stained at least focally with the endothelial markers CD31 and Fli-1; however, there was otherwise considerable variability in immunophenotype. The distinctive histopathologic appearance of the primary luminal lesion was lost whenever tumor invaded outside the vessel wall (into adventitia and beyond) or in metastatic sites. Such extravascular tumors assumed a variety of patterns reminiscent of undifferentiated pleomorphic sarcoma (UPS; in older literature also known as pleomorphic malignant fibrous histiocytoma, MFH) or other distinct types of sarcomas, including osteosarcoma, angiosarcoma, and rhabdomyosarcoma. The results of comparative genomic hybridization were nonspecific. Eleven patients died of the disease, in an average of 11 months after diagnosis. Three patients are still alive and free of disease at 4, 16, and 27 years. UIS of large systemic vessels represents a distinct clinical entity where intraluminal sarcoma presents with thrombosis and occlusion of large vessels. It is associated with a highly characteristic, although not entirely specific, histology and immunohistochemical phenotype. The histogenesis of UIS is not certain; however, it seems that the cell of origin must leave the confines of the vessel wall to show altered morphology. Although there are rare long-term survivors, UIS behaves as a fully malignant neoplasm that is almost uniformly associated with metastases and tumor-related death.

0 comments Cited 30 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: not found

Primary tumors of the thoracoabdominal aorta: surgical treatment of 5 patients and review of the literature.

Isabelle Brocheriou, Edouard Kieffer, Benoit Mongrédien … (2003)

The objectives of this study were to describe five cases involving primary tumors of the thoracoabdominal aorta and to review the pertinent literature. Between April 1990 and April 2000, we performed surgery on five patients with primary tumors of the aorta (PTA). There were three men and two women ranging in age from 37 to 65 years (mean, 49.8 years). The presenting manifestations were renovascular hypertension in four cases, including three associated with abdominal angina and lower extremity embolism in one case. In all patients aortograms identified atherosclerotic-like occlusive lesions in the thoracoabdominal aorta extending to the descending thoracic aorta in three cases, visceral arteries in four cases, and infrarenal aorta in one case. Preoperative histological diagnosis of PTA was achieved in two patients following open repair with placement of an aortoaortic graft in one case and peripheral embolectomy in one case. In two cases, diagnosis of PTA was strongly suspected before or during the procedure. In the remaining case, diagnosis was not achieved until the definitive histological report. In two patients surgical treatment was carried out with curative intent (aortic resection with graft replacement). In two cases surgical treatment was incomplete (endarterectomy of the aorta and visceral arteries). In the remaining case surgical treatment was purely palliative (aortic and superior mesenteric artery bypass). Histological findings demonstrated intimal-type sarcoma in two cases, leiomyosarcoma in one case, and angiosarcoma in one case. In the remaining case, histological analysis was unfeasible for technical reasons. One patient died due to massive cerebral embolism 2 days after surgical treatment involving revascularization of the aortic arch carried out with hypothermic circulatory arrest. One patient developed secondary paraplegia. All four patients who survived the immediate postoperative period died of tumor-related complications and cachexia at 5, 7, 16, and 24 months after the initial surgical procedure. The results of this small series as well as those of 130 previously reported cases confirm the extremely dismal prognosis of PTA. Mean overall survival for patients presenting PTA was less than 16 months. Survival at 5 years was 8%. Survival rates appear to be higher after surgical treatment and were significantly improved by adjuvant chemotherapy. The main factors correlated with poor prognosis were intimal type, involvement of the ascending aorta, aortic arch, or visceral aorta, and incomplete resection.

0 comments Cited 19 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: not found

Sarcomas associated with hereditary nonpolyposis colorectal cancer: broad anatomical and morphological spectrum.

Inge Bernstein, Christina Therkildsen, Ida A. Nissen … (2008)

Hereditary nonpolyposis colorectal cancer (HNPCC) is primarily linked to colorectal and endometrial cancer, but is associated with a broad tumor spectrum. Though not formally part of the syndrome, occasional sarcomas have been reported in individuals with HNPCC. We used the national Danish HNPCC-register to identify HNPCC families in which sarcomas had been diagnosed. Fourteen sarcomas were identified in families with mutations in MSH2, MSH6, and MLH1. The median age at sarcoma diagnosis was 43 (15-74) years. Soft tissue sarcomas predominated followed by uterine sarcomas and eight histopathological subtypes were represented with recurrent diagnoses of liposarcoma, leiomyosarcoma, and carcinosarcoma. Tumor tissue from eight cases was available for analysis of mismatch-repair (MMR) status using immunohistochemical staining and analysis of microsatellite instability, which revealed MMR defects in six of the eight tumors investigated. This suggests that sarcomas may be part of the HNPCC tumor spectrum and that colorectal cancer should be considered in the family history of sarcoma patients.

0 comments Cited 18 times – based on 0 reviews      Review now

Bookmark

All references

Author and article information

Contributors

Patrick Björkman

Journal

Journal ID (nlm-ta): J Vasc Surg Cases Innov Tech

Journal ID (iso-abbrev): J Vasc Surg Cases Innov Tech

Title: Journal of Vascular Surgery Cases and Innovative Techniques

Publisher: Elsevier

ISSN (Electronic): 2468-4287

Publication date PMC-release: 26 November 2019

Publication date Collection: December 2019

Publication date (Electronic): 26 November 2019

Volume: 5

Issue: 4

Pages: 589-592

Affiliations

[a ]Department of Vascular Surgery, Abdominal Center, Helsinki University Hospital, Helsinki, Finland

[b ]Comprehensive Cancer Center, Helsinki University Hospital, Helsinki, Finland

Author notes

[∗ ]Correspondence: Patrick Björkman, MD, PhD, Department of Vascular Surgery, Abdominal Center, Helsinki University Hospital, Helsinki FI-00029, Finland Patrick.bjorkman@ 123456hus.fi

Article

Publisher ID: S2468-4287(19)30109-1

DOI: 10.1016/j.jvscit.2019.08.002

PMC ID: 6881628

SO-VID: 97c051f7-bafc-41c4-a171-57a5e939b7cc

License:

This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

History

Date received : 10 March 2019

Date accepted : 2 August 2019

Comments

Comment on this article

scite_

Smart Citations

Citing PublicationsSupportingMentioningContrasting

View Citations

See how this article has been cited at scite.ai

scite shows how a scientific paper has been cited by providing the context of the citation, a classification describing whether it supports, mentions, or contrasts the cited claim, and a label indicating in which section the citation was made.

En bloc resection of visceral aorta and right kidney due to aortic sarcoma using temporary extracorporeal bypass grafting

Read this article at

Abstract

Related collections

Genome Engineering using CRISPR

Most cited references 15

Undifferentiated intimal sarcoma of large systemic blood vessels: report of 14 cases with immunohistochemical profile and review of the literature.

Primary tumors of the thoracoabdominal aorta: surgical treatment of 5 patients and review of the literature.

Sarcomas associated with hereditary nonpolyposis colorectal cancer: broad anatomical and morphological spectrum.

Author and article information

Contributors

Journal

Affiliations

Author notes

Article

History

Categories

Comments

Comment on this article

Similar content 14

Cited by 4

Most referenced authors 168