British Journal of Radiology (2007) 80, e30-e32
© 2007 British Institute of Radiology
doi: 10.1259/bjr/77258375
A case of a periaortic lymphoma presenting with the features of descending thoracic aorta dissection
A A Lotto, MD, MRCSI
1
S W Kendall, FRCS
1
R Hartley, FRCR
2 and
P Walker, FRCR
2
1Cardiothoracic and 2Radiology Departments, James Cook University Hospital, Marton Road, Middlesbrough TS4 3BW, UK
Correspondence: Mr Simon W Kendall, Cardiothoracic Department, James Cook University Hospital, Marton Road, Middlesbrough TS4 3BW, UK. E-mail: Simon.kendall{at}stees.nhs.uk
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Abstract
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We report the case of a 68-year-old male in whom an intrathoracic non-Hodgkin's lymphoma was diagnosed late after he presented with the clinical and radiological features of a descending aortic dissection due to penetrating ulcer. An endovascular stent was implanted in the descending aorta. At follow up, a CT scan showed the presence of a mediastinal mass thought to be a periaortic haematoma as a consequence of the endovascular stent implantation. A further CT scan showed an increase in size of the mediastinal mass encasing the whole descending aorta. A biopsy of the mass was performed which was shown to be non-Hodgkin's lymphoma. This is the first report of a penetrating ulcer of the descending aorta due to lymphoma, which probably caused the dissection.
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Introduction
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Intrathoracic non-Hodgkin's lymphomas usually present with a clinical picture of a systemic disease accompanied with lymphoadenopathy and the radiographic evidence of lymph node enlargement, pulmonary masses and pleural effusions [1]. Extrapleural space involvement is rare [2] and the presence of periaortic lymphoma may be misinterpreted at CT scan mimicking a thoracic aortic aneurysm or an intramural haematoma [3].
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Case report
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We report the case of a 68-year-old male in whom an intrathoracic non-Hodgkin's lymphoma presented with the clinical features of descending aorta dissection. He presented at an A&E department, following the onset of a severe acute on chronic back pain, at another hospital where he underwent urgent investigations. A CT scan of the chest revealed an aortic dissection, a penetrating ulcer in the descending thoracic aorta with extensive aortic haematoma and a bilateral pleural effusion (larger on the right side) (Figure 1
). Transferred to a teaching hospital, the patient then underwent an emergency endovascular stent insertion to the descending thoracic aorta. The procedure was uneventful and he was discharged after 5 days. 2 months later, a control CT scan confirmed the good position of the intravascular stent and the absence of contrast leakage, but revealed the presence of a large periaortic and mediastinal soft tissue mass, few small pre-tracheal lymph nodes were also noted. The presence of a bilateral pleural effusion, more evident on the right, was reported as well (Figure 2). The soft mass surrounding the aorta was interpreted as periaortic haematoma consequent to the endovascular stent implantation.
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Figure 1. Penetrating ulcer in the descending aorta(arrow) with extensive surrounding soft tissue, presumed to be haematoma, and bilateral pleural effusions. There is also abnormal soft tissue around the right internal mammary vessels (arrowhead).
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Figure 2. 2 months after aortic stent: well position of the intravascular stent (arrow); large periaortic and mediastinal soft tissue mass (arrowhead); bilateral pleural effusion, more evident on the right.
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1 month later the patient complained of shortness of breath and nocturnal dyspnoea, and a chest radiograph showed a large right pleural effusion. A pleurocentesis was performed and nearly 3 l of clear fluid were aspirated from the right chest, cytology of the pleural fluid revealed no malignant cells; this was assumed to be secondary to the haematoma.
The effusion recurred within 3 weeks and, after being referred to our Department, a further CT scan of the chest revealed the presence of soft tissue encasing the descending thoracic aorta with enlarged pre-vascular and aortic-pulmonary window lymph nodes (Figure 3a). On the right chest, there was a moderate pleural effusion with collapse of the lower lobe, multiple metastatic pleural lesions were present along with a lobulated mass in the right pericardial region with a diameter of 6 cm (Figure 3b). A 2.7 cm left retrocrural node and a 4.8 cm mass inseparable from the right crus were reported (Figure 3c). On comparison with earlier CT scans, these findings represented a definitive progression of previous lesions.
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Figure 3. (a) 4 months post-aortic stent: bilateral pleural effusions, nodular pleural thickening (arrowhead) and abnormal soft tissue around the right internal mammary vessels (arrow) which was biopsied. (b) Large right paracardial soft tissue mass (arrow) and nodular pleural thickening (arrowhead). (c) Soft tissue mass inseparable from the right crus (arrow).
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A deeper investigation into the patient's past medical history revealed a 1-year history of intermittent pain around the right costal margin associated with a dry cough, low grade fever, sweats, increasing shortness of breath and generally feeling unwell, but no weight loss in the last year.
Clinical examination revealed enlarged right groin lymph nodes, with no other lymphoadenopathy or hepatosplenomegaly. Laboratory findings did not demonstrate remarkable changes.
An ultrasound guided biopsy of the right chest mass revealed a low-grade follicular non-Hodgkin's lymphoma.
He was started on chemotherapy with oral chlorambucin and dexamethasone with adequate response to it. He subsequently underwent a right video-assisted thoracoscopic (VAT) talc pleurodesis for the presence of persistent right pleural effusion. The patient is at present stable, his clinical conditions are improving and the lymphoma is under control.
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Discussion
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This is a rare case of a patient with a low-grade intrathoracic follicular non-Hodgkin's lymphoma with extrapleural space involvement encasing the aorta which had the initial presentation of an aortic dissection and radiological features of a penetrating ulcer of the descending aorta with extensive intramural haematoma.
Extrapleural space involvement is a rare feature of lymphoma where the extrapleural fat is either displaced or invaded by the proliferative tissue [2]. Because the descending thoracic aorta is an extrapleural structure, there is a potential for the lymphoma to invade the soft tissue surrounding the aorta, encasing the whole vessel. One case has been reported so far of such disease which was initially misdiagnosed as a descending aortic aneurysm [3].
In our case, the acute onset of severe back pain was typical of an acute aortic syndrome, and the initial clinical and radiological features were consistent with aortic dissection with a penetrating aortic ulcer and intramural haematoma. In the absence of characteristic clinical picture of a systemic disease without lymphoadenopathy, the patient underwent an endovascular stent graft to the descending aorta. Patients who undergo endovascular graft procedure will have follow up with a CT scan after 2–3 months to detect possible complications, such as stent graft migration or endoleaks. Pleural effusions are reported to be common complications after the procedure and usually resolve in 3 months [5]. In our case the 2-month follow up CT scan was performed, and the images of tissue surrounding the descending aorta were thought to be a haematoma resulting, probably, from a perigraft leak. However, the presence of a bilateral pleural effusion, which was persistent on the right side after 3 months, should have raised suspicion of an alternative diagnosis, in particular the presence of a mediastinal soft tissue mass and pre-tracheal lymph nodes. This is particularly true if we consider that our patient had a bilateral pleural effusion, more evident on the right side. In contrast, in patients following endovascular stent graft implant the effusion is usually confined to the left side [5].
A further CT scan was then performed for the presence of a persistent and recurrent right pleural effusion, accompanied by severe shortness of breath and systemic symptoms. This showed the typical radiological appearance of a proliferative disease which, at histology examination, was shown to be an intrathoracic non-Hodgkin's follicular lymphoma.
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Conclusions
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Intrathoracic non-Hodgkin's lymphoma is a rare condition which can be difficult to diagnose and could be responsible for an acute aortic syndrome, in particular when there is extrapleural invasion of the lymphoma.
In this report, in the absence of any other evidence of aortic atherosclerotic disease, the penetrating ulcer could be assumed to be secondary to the lymphoma. In that case, this is the first report of a penetrating ulcer of the descending aorta due to lymphoma, which probably caused the dissection.
Received for publication February 2, 2006.
Revision received February 23, 2006.
Accepted for publication March 6, 2006.
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References
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Abstract
Introduction
