journal of Internal Mcdicine 1990: 227 : 2 1 5-1 7

Case report Mediastinal tumour as the presenting manifestation of W egener ' s gr anulomatosis

P. BOUDES From the Department of Nephrology. Section of Clinical Immunology. Henri Mondor Hospital. Criteil. France

Abstract. Boudes P (Department of Nephrology. Section of Clinical Immunology, Henri Mondor Hospital, Cretail, France). Mediastinal tumour as the presenting manifestation of Wegener's granulomatosis. lournul of lnternrrl Medicine 1990: 227: 2 15-1 7 .

A 42-year-old man presented with a mediastinal tumour. On histological examination, tuberculosis was evoked, but no definitive conclusion could be drawn. The patient thereafter developed throat ulcerations and rapidly progressing renal insufficiency due to necrotizing glomerulonephritis. After reviewing the histological material, it was concluded that all of these manifestations were related to Wegener's granulomatosis. The presentation of Wegener's granulomatosis as a mediastinal tumour is very rare, but this form of the disease must be recognized, as early treatment with cyclophosphamide is essential for a favourable outcome.

Keywords: necrotizing glomerulonephritis. Wegener's granulomatosis.

Introduction Wegener's granulomatosis is a sytemic vasculitis, prompt recognition of which is essential as rapid treatment of the disease with cyclophosphamide is necessary for a favourable prognosis [1].

We evaluated a patient who had presented with a mediastinal tumour as the initial manifestation of Wegener's granulomatosis. Because this aspect of the disease is very unusual, diagnosis was delayed and treatment was only initiated when the vasculitis had caused irreversible renal insufficiency.

Case reports A 42-year-old healthy white man developed a constrictive left thoracic chest pain. Electrocardio- graphic and enzymatic evaluation showed no ab- normalities. One week later, fever, weight loss and asthenia occurred. Three weeks later, a chest X-ray revealed a left mediastinal mass (Figs 1 and 2). A physical examination showed no abnormalities, and there was no history of upper respiratory tract, cutaneous, central nervous system, or locomotor

system symptoms. A tuberculin skin test was nega- tive, and serum creatinine and urinary sediment were normal. Fibre optic bronchoscopy revealed no lesions. An arteriographic procedure disclosed a lacunar non-vascular area corresponding to the tumour.

A left anterior thoracotomy was performed, in- volving excision of most of the tumour, which was fibrous with central necrosis. Histological exam- ination revealed a granuloma with central necrosis, and no evidence of vasculitis. This was believed to be consistent with tuberculosis. Stains for acid-fast bacilli and fungus were negative, as were cultures. No definitive diagnosis was made and the patient was discharged without treatment.

Three months later he was admitted to our unit with rapidly progressing renal insufficiency. The serum creatinine level was 630 pmol I-' and the urinary protein level was 0.3 g d-' (300 mg/24 h). Haematuria was 3 + . A renal biopsy contained 20 glomeruli and revealed a segmental necrotizing glomerulonephritis, with crescent formation in nearly 75% of glomeruli. Fibrinoid necrosis of an afferent arteriole was demonstrated. Physical exam-

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216 P.BOUDES

Figs I and 2. First admission chest X-ray film showing a mediastinal tumour.

ination also revealed a right tonsil ulceration and a budding lesion of the epiglottis. Biopsies of these lesions revealed the presence of necrotizing granu- lomatous lesions without vasculitis.

A diagnosis of Wegener’s granulomatosis was suspected, and the histological material of the mediastinal tumour was reviewed. Fibrinoid necrosis was noted, and granulomatous lesions without caseous necrosis were present within and outside the arterial wall, confirming that the previous medi- astinal tumour was a manifestation of Wegener’s granulomatosis.

Treatment with prednisone and cyclophosphamide was initiated. Symptoms in the upper airway dis-

appeared, and the patient’s general status improved. Renal function remained deteriorated, and the patient began continuous haemodialysis treatment.

Discussion Respiratory involvement is a frequent manifestation of Wegener’s granulomatosis, as most patients pres- ent with symptoms of the upper or lower respiratory systems [ 11. Characteristic findings are multiple bilateral nodal infiltrates with a tendency to cavitate [ l , 21. However, hilar or mediastinal manifestations, both as presenting events and in the course of the disease, are exceptional [ l , 3, 4. 51.

Nichols et al. reported the case of a 31-year-old woman with Wegener’s granulomatosis and bilateral hilar adenopathy [6], and in an extensive review of the literature of 2 78 cases of the disease, they found only three cases with unilateral hilar mass. However, on studying their references [l, 4, 7-91 we found only one case that was consistent with a mediastinal involvement in Wegener’s granulomatosis [8]. This case presented with a mass between the oesophagus and the trachea, but it was not clear from the report whether the abnormality was large enough to appear on a chest X-ray.

In fact, only the report of Gohel et al. [2] describes hilar manifestations in the context of Wegener’s granulomatosis. These authors studied 20 cases of Wegener’s granulomatosis, and observed two cases presenting with hilar masses that were indistinguish- able from pulmonary malignancies. One of these cases had an accompanying lesion in the pulmonary parenchyma. Wechsler et al. [lo], in their study of 17 cases of Wegener’s granulomatosis, observed three cases with hilar or parahilar masses, but provided no further details.

Mediastinal manifestations viewed by chest X-ray in Wegener’s granulomatosis are so rare that, when they are isolated, as in the case described here, they are particularly misleading. In this disease, granu- loma formation can become tumorous and, with the exception of the lungs, such tumours are mainly found in the upper airway [ l l ] and the orbit [12, 131. They may also occur in the salivary glands [14] or the kidney [8, 151. The case presented in this report is to our knowledge the first example of a mediastinal tumour occurring as the presenting manifestation of Wegener’s granulomatosis.

It is important to recognize this presenting feature of the disease, because early treatment with

MEDIASTINAL TUMOUR I N WEGENER'S GRANULOMATOSIS 217

cyclophosphamide is essential for a favourable out- come [l]. In this context, the use of the anticyto- plasmic antibodies against polymorphonuclear neutrophils and monocytes may have been a useful diagnostic tool [16].

In conclusion, the case described in this report suggests that a mediastinal tumour may be an unusual presenting manifestation of Wegener's granulomatosis. This feature of the disease must be recognized to prevent delay in initiation of cyclophosphamide therapy.

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Received 11 July 1989. accepted 8 August 1989.

Correspondence: Dr Pol Boudes. Department of Nephrology. HBpital Henri Mondor. 51 avenue du Marechal de Lattre de Tassigny. 9401 0 Creteil. France.