What Is Your Diagnosis?

Abstract

Accessible online at: www.karger.com/res A 21-year-old female smoker of Afro-Caribbean origin presented with vague episodes of non-specific abdominal pain and underwent a laparotomy 3 years later. Pre-operative thoracic imaging was unremarkable. At surgery, a mass arising from the left kidney, involving the spleen, gastric wall and pancreatic tail was completely excised. Histology was compatible with a diagnosis of intraabdominal desmoid, but after re-evaluation was reclassified as a hyalinized variant of retroperitoneal fibrosis. The patient remained asymptomatic on follow-up until 11 years later (aged 32 years), when she developed an intractable cough. Chest X-ray (fig. 1) demonstrated a number of pulmonary masses. Computerized tomography (CT; fig. 2) revealed that the largest of these measured 6 ! 3 cm, and was cavitating (fig. 2). There were no other radiological abnormalities. Full blood count, serum electrolytes, autoantibody screening, angiotensin-converting enzyme level, paired serology for atypical organisms, viral titres, and sputum analysis for bacteria including acid fast bacilli were within normal limits. Serum precipitins were also absent. However, the erythrocyte sedimentation rate was elevated at 61mm in the 1st h. Fibre-optic bronchoscopy with broncho-alveolar lavage showed normal appearances with no evidence of acid fact bacilli, fungi, or malignancy. Thoracotomy and open biopsy demonstrated hyalinized fibrous tissue with a patchy mononuclear inflammatory cell infiltrate, consistent with reactive fibrosis. There was no evidence of malignancy. The patient’s symptoms remained stable over the next 4 years, although serial CT imFig. 1. Chest X-ray demonstrating mass in right mid-zone with further lesions at left apex. Fig. 2. Thoracic CT showing a cavitating mass in the right lung extending towards the right hilum (white arrow).