We describe a 74-year-old male patient with an intimal sarcoma of the descending aorta mimicking aortitis. The patient presented with lower back pain, fever, and increased C-reactive protein, erythrocyte sedimentation rate, and immunoglobulin G4 (IgG4) serum levels, together with Staphylococcus epidermidis-positive blood cultures. These findings, together with evidence of a 49-mm pseudoaneurysm of the descending thoracic aorta, caused us to suspect aortitis. However, postoperative histology and immunohistochemistry demonstrated the presence of an intimal aortic sarcoma. At the 8-month follow-up, local recurrence of the neoplasm and lung metastases were noted

Basolo, Fulvio

Berchiolli, RAFFAELLA NICE

Bortolotti, Uberto

DE MARTINO, Andrea

De Martino, Andrera

Levantino, Maurizio

Pucci, Angela

English

PubMed

BERCHIOLLI, RAFFAELLA NICE

BASOLO, FULVIO

BORTOLOTTI, UBERTO

DE MARTINO, ANDREA

Archivio della Ricerca - Università di Pisa

Received: May 3, 2016Accepted: June 29, 2016 Published online: August 2016  Case Report AORTA, August 2016, Volume 4, Issue 4:142-145DOI: http://dx.doi.org/10.12945/j.aorta.2016.16.022* Corresponding Author: Uberto Bortolotti, MDUniversity HospitalDivision of Cardiac SurgeryVia Paradisa, 2, 56124 Pisa, ItalyTel.: +1 39 50 995250; Fax: +1 39 50 541647; E-Mail: uberto.bortolotti@med.unipi.itFax +1 203 785 3552 E-Mail: aorta@scienceinternational.orghttp://aorta.scienceinternational.org© 2016 AORTAPublished by Science International Corp.ISSN 2325-4637Accessible online at: http://aorta.scienceinternational.orgAbstractWe describe a 74-year-old male patient with an  intimal sarcoma of the descending aorta mimicking aortitis. The patient presented with lower back pain, fever, and  increased C-reactive protein, erythrocyte sedimenta-tion rate, and immunoglobulin G4 (IgG4) serum levels, together with  Staphylococcus epidermidis-positive blood cultures. These findings, together with evidence of a 49-mm pseudoaneurysm of the descending tho-racic aorta, caused us to suspect aortitis. However, postoperative histology and immunohistochemistry demonstrated the presence of an intimal aortic sarco-ma. At the 8-month follow-up, local recurrence of the neoplasm and lung metastases were noted.Copyright © 2016 Science International Corp.Key WordsAorta • Aortitis • Aortic tumorIntroductionIntimal sarcomas are extremely uncommon  tumors involving the major vessels, mostly the  pulmonary ar-tery and the aorta, with a dismal prognosis [1]. The clinical presentation is usually nonspecific and mostly related to tumor embolism resulting in limb or viscer-al ischemia [1, 2]. Preoperative diagnosis is difficult and most neoplasms are recognized only upon histo-logical analysis of the surgical specimen. Here, we de-scribe an unusual case of intimal sarcoma of the de-scending thoracic aorta mimicking aortitis.Case PresentationA 74-year-old male was admitted to our depart-ment complaining of abdominal and lower back pain and fever for a duration of three months. The patient had also previously been diagnosed with hyperten-sion, hypercholesterolemia, stage 3 chronic kidney disease, and monoclonal gammopathy of unknown significance. Laboratory tests revealed increased erythrocyte sedimentation rate and serum levels of C- reactive protein and IgG4 with mild anemia. Blood cultures were positive for Staphylococcus epidermidis. Computed tomography (CT) with contrast-enhanced angiography showed a disruption of the anterior wall of the aorta with a maximum diameter of 49 mm sur-rounded by contrast enhancing solid tissue at the thoraco-abdominal transition and close to the origin of the celiac trunk, which was interpreted as a pseu-doaneurysm (Figure 1A and 1B). To better define the aortic lesion, 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) was performed, revealing isolated localization of the radionuclide at the level of the pseudoaneurysm (Figure 1C and 1D).A differential diagnosis between infective aortitis and aortitis associated with IgG4-related sclerosing disease was considered. Accordingly, steroid and anti-biotic therapy was started with no clinical improve-ment or improvement of laboratory test findings  after 10 days.The patient underwent surgical resection of the pseudoaneurysm through a left thoracotomy in the Intimal Sarcoma of the Descending Aorta  Mimicking AortitisAngela Pucci, MD, PhD1, Andrea De Martino, MD2, Maurizio Levantino, MD2,  Raffaella Berchiolli, MD3, Fulvio Basolo, MD1, Uberto Bortolotti, MD2*1 Division of Pathology, University Hospital, Pisa, Italy2 Division of Cardiac Surgery, University Hospital, Pisa, Italy3 Division of Vascular Surgery, University Hospital, Pisa, ItalyPucci, A. et al.             Aortic Sarcoma143             Case Report7th intercostal space combined with a laparotomy and a retroperitoneal approach. The operation was performed with left heart bypass by cannulating the left femoral artery and the left inferior pulmonary vein under moderate hypothermia (32°C). The  pseudoaneurysm was isolated and transected and the aorta was reconstructed with the interposition of a 24-mm cryopreserved homograft. The postopera-tive course was uncomplicated and the patient was discharged home on the 8th postoperative day.Both histology and microbiological examinations were performed on the resected aortic tissue. By rou-tine histology and immunohistochemistry, an intimal aortic sarcoma was diagnosed (Figure 2). The aortic wall was extensively infiltrated by a  poorly- differentiated spindle cell tumor with nuclear pleomorphism, abundant mitoses, and microfoci of necrosis originating from the intimal surface and  infiltrating the aortic wall including the resected  margins. Immunohistochemistry, performed using an automated immunostainer and specific antibodies (Ventana Medical Systems, Inc., Tucson, AZ, USA), showed diffuse staining for vimentin andfocal Mouse double minute 2 homolog (MDM2) positivity. Markers for epithelial cells, smooth and skeletal muscle cells, leukocytes, and melanocytes were otherwise nega-tive.The patient was treated with a specific protocol of chemotherapy with close clinical follow-up. However, the tumor relapsed soon after surgery with develop-Figure 1. Panel A. Angio-computed tomography (CT) showing the pseudoaneurysm in the descending thoracic aorta. Panel B. Vol-ume-rendered image showing the close relation of the pseudoaneurysm with the celiac trunk (arrow). Panels C and D. CT positron emission tomography demonstrating intense tracer uptake in the aortic wall.AORTA, August 2016            Volume 4, Issue 4:142-145Case Report             144ment of local recurrence and lung metastases at 8-month follow-up.DiscussionThe present case is, to our knowledge, the first  reported aortic intimal sarcoma mimicking aortitis and presenting as a descending aortic pseudoaneu-rysm. Intimal aortic sarcomas are extremely rare high-grade malignant neoplasms of large blood vessels, sub-classified into intimal tumors characterized main-ly by intraluminal growth, mural tumors of medial or adventitial origin, and mixed tumors [3]. Such neo-plasms are poorly differentiated and usually have a nonspecific presentation. The differential diagnosis requires histological analysis demonstrating a malig-nant mesenchymal tumor, and immunohistochemis-try revealing immunoreactivity only for vimentin and MDM2. Vimentin is a sensitive marker for many neo-plasms, while MDM2 is found in over 70% of intimal sarcomas and is a negative prognostic marker.  Indeed, most cases have been diagnosed either at necropsy or at examination of aortic surgical specimens [1, 2]. An intimal sarcoma must be differentiated from a more common angiosarcoma, which shows definite and diffuse endothelial differentiation, and from a ley-Figure 2. Histologic sections showing the sarcoma originating from the aortic intima, characterized by high-ly atypical cells with nuclear pleomorphism (asterisk) and mitoses (arrows), and diffuse immunoreactivity for vimen-tin and focal positivity for mouse double minute 2 homolog (MDM2). Panel A. Haematoxylin and eosin stain, origi-nal magnification 2×. Panel B. Haematoxylin and eosin stain, original magnification 20×; Panel C. Immunoperoxidase staining and haematoxylin counterstaining using a specific antibody against Vimentin, original magnification 10×; Panel D. Immunoperoxidase staining and haematoxylin counterstaining using a specific antibody against MDM2, original magnification 20×.Pucci, A. et al.             Aortic Sarcoma145             Case Reportomiosarcoma, which is characterized by smooth mus-cle marker immunoreactivity [4-6]. The pathogenesis of intimal sarcoma is still unknown, but genetic fac-tors, including changes in the MDM2-p53pathway, seem to play important roles [7].Intimal aortic sarcomas are usually localized in the abdominal aorta between the celiac artery and the il-iac bifurcation, and in 30% of cases the descending aorta is involved as seen in the present case [1, 2, 4, 6]. The most common clinical presentation is related to tumor embolization with peripheral or mesenteric ischemia and necrotic skin lesions, or to metastasis, which occurs mainly to bones, liver, and lungs. Less common symptoms are secondary to intraluminal oc-clusion of the aorta, mass effect, or pseudoaneurysm formation with or without secondary aortic rupture [6].Imaging studies of the aorta, such as a CT scan, can seldom distinguish aortic intimal tumors from ad-vanced atherosclerotic disease. Even 18F-FDG PET may not specifically differentiate a tumor from an infec-tious aortic lesion as observed in the present case. This has been emphasized by Naughton et al. [6] who reported a patient with an aortic neoplasm present-ing as an abdominal aortic rupture with clinical and radiologic findings suggestive of aortitis. In tumors of the pulmonary artery, the presence of intraluminal filling defect enhancement on MRI with  gadolinium has been shown to be the most sensitive diagnostic tool to differentiate such tumors from non-neoplastic lesions [8]; it can be speculated that this finding could also be applied to aortic lesions.Primary malignant tumors of the aorta have a poor prognosis, with a mean survival time of 14 months [6]. The most effective treatment is surgical resection with graft interposition, but radical surgery is often not feasible because of either tumor volume or local extension with adjacent tissue infiltration. In the  present case, operation consisted of excision of the pseudoaneurysm and reconstruction of the aorta by interposition of a homograft because of the suspicion of an infective nature of the lesion. Postoperative his-tological and immunoistochemical analyses ruled out the presence of aortitis, revealing instead a rare malig-nant neoplasm.In conclusion, intimal sarcomas of the aorta are rare and aggressive neoplasms which occasionally may mimic aortitis. The preoperative diagnosis appears of utmost importance because an unsuspected malig-nancy, as in the case herein reported, may lead to in-complete wide margin resection, thus influencing the long-term prognosis. Indeed, our patient has shown tumor recurrence at 8-month follow-up.Conflict of Interest The authors have no conflict of interest relevant to this publication.Comment on this Article or Ask a Question 1. Bode-Lesnievska B, Debiec-Rychter M. In-timal sarcoma. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F. WHO classi-fication of tumors of soft tissue and bone, 4th Edition. Lyon: IARC; 2013, p. 232-233.2. Sebenik M, Ricci A Jr, DiPasquale B, Mody K, Pytel P, Jee KJ, et al. Undifferentiated intimal sarcoma of large systemic blood vessels: report of 14 cases with immuno-histochemical profile and review of litera-ture. Am J Surg Pathol. 2005;29:1184-1193. DOI: 10.1097/01.pas.0000159774.70288.7d3. Wright EP, Glick AD, Virmani R, Page DL. Aortic intimal sarcoma with embolic me-tastases. Am J Surg Pathol. 1985;9:890-897. DOI: 10.1097/00000478-198512000-000054. Staats P, Tavora F, Burke AP. Intimal sarco-mas of the aorta and iliofemoral arteries: a clinicopathological study of 26 cases. Pathology. 2014;46:596-603. DOI: 10.1097/PAT.00000000000001825. Lazar A, Evans HL, Shipley J. Leiomyosar-coma. In: Fletcher CDM, Bridge JA, Hogen-doorn PCW, Mertens F. WHO classification of tumors of soft tissue and bone, 4th Edi-tion. Lyon: IARC; 2013, p. 111-113.6. Naughton PA, Wandling M, Phade S, Gar-cia-Toca M, Carr JC, Rodriguez HE. Intimal angiosarcoma causing abdominal aortic rupture. J Vasc Surg. 2011;53:818-821. DOI: 10.1016/j.jvs.2010.10.0907. Zhang H, MacDonald WD, Erickson- Johnson M, Wang X, Jenkins RB, Oliveira AM. Cytogenetic and molecular cytoge-netic findings of intimal sarcoma. Cancer Genet Cytogenet. 2007;179:146-149. DOI: 10.1016/j.cancergencyto.2007.08.0138. Restrepo CS, Betancourt SL, Marti-nez-Jimenez S, Gutierrez FR. Tumors of the pulmonary artery and veins. Semin Ultrasound CT MR. 2012;33:265-272. DOI: 10.1053/j.sult.2012.04.002Cite this article as: Pucci A, DeMartino A, Levantino M, Berchiolli R, Basolo F, Bortolotti U. Intimal Sarcoma of the Descending Aorta Mimicking Aortitis. AORTA (Stamford) 2016;4(4):142-145. DOI: http://dx.doi.org/10.12945/j.aorta.2016.16.022References

Intimal Sarcoma of the Descending Aorta Mimicking Aortitis

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Intimal Sarcoma of the Descending Aorta Mimicking Aortitis

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