CASE REPORT
Ausual presentation of an unusual diagnosis
H. Al Hashimi, M.F. Scholten, J. McGhie
Chest pain is one of the most common acute medical emergencies and a significant proportion of cases will manifest in one ofthe acute coronary syndromes. We report a case in which an intermittent non-specific chest pain led to the detection of primary mediastinal large B-cell lymphoma. (Neth Heart J 2002;10:326-8.) Key words: aortic aneurysm, tumour A 28-year-old female was referred by her general Mpractitioner (GP) to the University Hospital of Rotterdam with an abnormal chest X-ray showing a possible dissecting aortic aneurysm. She was investigated for a non-specific chest pain radiating to the back; the pain was in the inter-scapula region and had been present for the last three months. The pain was vague and dull in nature. There were no relieving or exacerbating factors and no other associated symptoms. The patient had no past medical history of ischaemic heart disease (IHD) and no significant family history. She was not on any regular medication. The patient was a non-smoker and a social drinker.
Examination The patient was not acutely ill. There were no peripheral stigmata indicative ofconnective tissue disease (e.g. Marfan syndrome). Her blood pressure was 130/70 mmHg (equal in both arms), pulse rate 75 beats per minute (equal and symmetrical in both arms) and respiratory rate 20 per minute. The jugular venous pressure was not raised and there was no peripheral oedema.
H. AN Hadsm.
M.F.S d. J. Mchb.
Department of Cardiology, Erasmus University Medical Centre, Rotterdam. Address for correspondence: H. Al Hashimi.
E-mail: [email protected]
326
On cardiovascular examination, the apex beat was normal in character, slightly displaced at the 6th intercostal space, mid clavicular line. The heart sounds were normal. A soft systolic murmur, grade 3 out of 6, was detected at the left 3rd intercostal space. The rest of the physical examination including chest, abdomen and central nervous system was unremarkable.
Investigations The full blood count revealed a haemoglobin of 6.1, haematocrit 0.32, MCV 75, platelets 368, leucocytes 3.6, reticulocytes 1.4%, basophils 1%, eosinophils 2%, neutrophils 73, lymphocytes 14, monocytes 11, and erythrocyte sedimentation rate (ESR) 60 mm/h. Urea, electrolytes, liver function test and thyroid function test were all normal, except that the LDH was remarkably high at 906 U/L. The chest X-ray showed a normal cardiothoracic ratio (CTR) with dear lung fields. There was a large well-defined opacity projected over the aorta with a diameter of 8 cm. Figure la shows the anteroposterior view and figure lb the lateral view. The ECG showed sinus rhythm at a rate of 75 beats per minute, with a normal axis. No abnormalities were found. Echocardiography revealed normal dimensions and function ofthe heart and structurally normal valves. There was a large mass in the anterior mediastinum, measuring about 8 cm in diameter, which compressed the left pulmonary artery (LPA), as seen in figures 2, 3 and 4. An irregular filling defect in the anterior mediastinum measuring about 8 cm in diameter with a hypo-dense centre was revealed on the CT scan of the chest; no other abnormalities were seen (figure 5). CT scan of the abdomen was normal. In conclusion, we report a young healthy female referred by her GP with a history of non-specific chest pain and abnormal chest X-ray in the form of anterior mediastinal mass. The deferential diagnosis ofthis mass was aortic aneurysm, lymphoma, teratoma, parathyroid gland and thymoma.1 We ruled out the possibility of aortic aneurysm echocardiographicaly by CT scan. The most likely diagnosis was mediastinal lymphoma. We arranged for her to have a tissue biopsy for histopathological examination. The histopathological report Netherlands Hcart Journal, Volume 10, Number 7/8, August 2002
A usual presentation of an unusual diagnosis
Figure 1. Chest X-ray; large well-defined opacity projected over the aorta with a diameter of 8 cm.
was consistent with a primary mediastinal large B-cell lymphoma. We referred the patient to the haematologist for further management including staging and chemotherapy treatment combined with radiotherapy.
Discussion The most common mediastinal tumours are lymphoma, malignant germ cell tumour and thymomas.2 Primary mediastinal large B -cell tumour, which is the diagnosis Figure 2. 2D echocardiography; the tumour compressing the left
pulmonaryartery (LPA).
Nctherlands Heart Journal, Volume 10, Number 7/8, August 2002
327
A usual presentation of an unusual diagnosis
Figure 3. 2Dechkocar-
diograpby; the tumour
witb a nor
~
A Fiur 5.II
no
h bjl
rglriln efc
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II_ .1
.
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S
Figure 5. CTrscan oftheclwsf imgularfiuingdfetin theanerio mediastnum measured about 8 cm in diameter with a bypo-dense centre.
,-.F -
I~~~ I, -
tumour arising in an unusual site in a cohort ofyoung adult patients (more commonly women) in whom large cell lymphoma is infrequent and noteworthy.3 Pathologically, it is characterised by a sclerotic pattern and the unusual surface immunoglobin (SIg)-negative B-cell phenotype of a tumour arising in a T-cell organ (thymus).3 Malignant primary mediastinal tumours often require multimodality treatment.4 This type of tumour shows a good response to a combination of chemotherapy and radiotherapy.3 U RIfem 1
Finure 4hcaserdiographis pulscetwae DlnioppthlergincMalb siubtye lare use vel inA,b lymphoarecgie )a)clPA 4Bofardiffuby cotnosuDtpper origin ofrg symhowmn inreasegnivelociny ficatinon.DopkIrtisan aggreAssoive, invraseinve,btlocaised
2
3 4
328
Edwards CRW, et al. Tumour of the mediastinum. Davidson's Principles and Practice of Medicine. Seventeenth edition. pp3779. Temes R, et al. Primary mediastinal malignancies: findings in 219 patients. WestJMed 1999;170:161-6. Aisenberg AC. Primary large cell Lymphoma ofthe mediastinum. Semin Oncol 1999;26:251-8. Whooley BP, et al. Primary tumours of the mediastinum. J Surg Oncol 1999;70:95-9.
Nctherlands Hcart Journal, Volume 10, Number 7/8, August 2002
Ausual presentation of an unusual diagnosis
H. Al Hashimi, M.F. Scholten, J. McGhie
Chest pain is one of the most common acute medical emergencies and a significant proportion of cases will manifest in one ofthe acute coronary syndromes. We report a case in which an intermittent non-specific chest pain led to the detection of primary mediastinal large B-cell lymphoma. (Neth Heart J 2002;10:326-8.) Key words: aortic aneurysm, tumour A 28-year-old female was referred by her general Mpractitioner (GP) to the University Hospital of Rotterdam with an abnormal chest X-ray showing a possible dissecting aortic aneurysm. She was investigated for a non-specific chest pain radiating to the back; the pain was in the inter-scapula region and had been present for the last three months. The pain was vague and dull in nature. There were no relieving or exacerbating factors and no other associated symptoms. The patient had no past medical history of ischaemic heart disease (IHD) and no significant family history. She was not on any regular medication. The patient was a non-smoker and a social drinker.
Examination The patient was not acutely ill. There were no peripheral stigmata indicative ofconnective tissue disease (e.g. Marfan syndrome). Her blood pressure was 130/70 mmHg (equal in both arms), pulse rate 75 beats per minute (equal and symmetrical in both arms) and respiratory rate 20 per minute. The jugular venous pressure was not raised and there was no peripheral oedema.
H. AN Hadsm.
M.F.S d. J. Mchb.
Department of Cardiology, Erasmus University Medical Centre, Rotterdam. Address for correspondence: H. Al Hashimi.
E-mail: [email protected]
326
On cardiovascular examination, the apex beat was normal in character, slightly displaced at the 6th intercostal space, mid clavicular line. The heart sounds were normal. A soft systolic murmur, grade 3 out of 6, was detected at the left 3rd intercostal space. The rest of the physical examination including chest, abdomen and central nervous system was unremarkable.
Investigations The full blood count revealed a haemoglobin of 6.1, haematocrit 0.32, MCV 75, platelets 368, leucocytes 3.6, reticulocytes 1.4%, basophils 1%, eosinophils 2%, neutrophils 73, lymphocytes 14, monocytes 11, and erythrocyte sedimentation rate (ESR) 60 mm/h. Urea, electrolytes, liver function test and thyroid function test were all normal, except that the LDH was remarkably high at 906 U/L. The chest X-ray showed a normal cardiothoracic ratio (CTR) with dear lung fields. There was a large well-defined opacity projected over the aorta with a diameter of 8 cm. Figure la shows the anteroposterior view and figure lb the lateral view. The ECG showed sinus rhythm at a rate of 75 beats per minute, with a normal axis. No abnormalities were found. Echocardiography revealed normal dimensions and function ofthe heart and structurally normal valves. There was a large mass in the anterior mediastinum, measuring about 8 cm in diameter, which compressed the left pulmonary artery (LPA), as seen in figures 2, 3 and 4. An irregular filling defect in the anterior mediastinum measuring about 8 cm in diameter with a hypo-dense centre was revealed on the CT scan of the chest; no other abnormalities were seen (figure 5). CT scan of the abdomen was normal. In conclusion, we report a young healthy female referred by her GP with a history of non-specific chest pain and abnormal chest X-ray in the form of anterior mediastinal mass. The deferential diagnosis ofthis mass was aortic aneurysm, lymphoma, teratoma, parathyroid gland and thymoma.1 We ruled out the possibility of aortic aneurysm echocardiographicaly by CT scan. The most likely diagnosis was mediastinal lymphoma. We arranged for her to have a tissue biopsy for histopathological examination. The histopathological report Netherlands Hcart Journal, Volume 10, Number 7/8, August 2002
A usual presentation of an unusual diagnosis
Figure 1. Chest X-ray; large well-defined opacity projected over the aorta with a diameter of 8 cm.
was consistent with a primary mediastinal large B-cell lymphoma. We referred the patient to the haematologist for further management including staging and chemotherapy treatment combined with radiotherapy.
Discussion The most common mediastinal tumours are lymphoma, malignant germ cell tumour and thymomas.2 Primary mediastinal large B -cell tumour, which is the diagnosis Figure 2. 2D echocardiography; the tumour compressing the left
pulmonaryartery (LPA).
Nctherlands Heart Journal, Volume 10, Number 7/8, August 2002
327
A usual presentation of an unusual diagnosis
Figure 3. 2Dechkocar-
diograpby; the tumour
witb a nor
~
A Fiur 5.II
no
h bjl
rglriln efc
ntea&ro
II_ .1
.
-l~~~~S.-~1
2:
S
Figure 5. CTrscan oftheclwsf imgularfiuingdfetin theanerio mediastnum measured about 8 cm in diameter with a bypo-dense centre.
,-.F -
I~~~ I, -
tumour arising in an unusual site in a cohort ofyoung adult patients (more commonly women) in whom large cell lymphoma is infrequent and noteworthy.3 Pathologically, it is characterised by a sclerotic pattern and the unusual surface immunoglobin (SIg)-negative B-cell phenotype of a tumour arising in a T-cell organ (thymus).3 Malignant primary mediastinal tumours often require multimodality treatment.4 This type of tumour shows a good response to a combination of chemotherapy and radiotherapy.3 U RIfem 1
Finure 4hcaserdiographis pulscetwae DlnioppthlergincMalb siubtye lare use vel inA,b lymphoarecgie )a)clPA 4Bofardiffuby cotnosuDtpper origin ofrg symhowmn inreasegnivelociny ficatinon.DopkIrtisan aggreAssoive, invraseinve,btlocaised
2
3 4
328
Edwards CRW, et al. Tumour of the mediastinum. Davidson's Principles and Practice of Medicine. Seventeenth edition. pp3779. Temes R, et al. Primary mediastinal malignancies: findings in 219 patients. WestJMed 1999;170:161-6. Aisenberg AC. Primary large cell Lymphoma ofthe mediastinum. Semin Oncol 1999;26:251-8. Whooley BP, et al. Primary tumours of the mediastinum. J Surg Oncol 1999;70:95-9.
Nctherlands Hcart Journal, Volume 10, Number 7/8, August 2002
