Thoracic endometriosis syndrome manifesting as atraumatic haemothorax causing difficult ventilation under general anaesthesia.

A 31-year-old woman underwent elective hysteroscopy and laparoscopy for investigation of primary infertility. The procedure was abandoned owing to intraoperative desaturation and reduced right-sided chest movement. Postoperative chest x-ray showed an opacified right hemithorax with tracheal deviation to the left. Retrospectively on direct questioning, she admitted to dry cough, mild dyspnoea and significant weight loss. Diagnostic thoracocentesis and contrast CT scan confirmed heavily blood-stained exudative pleural effusion but were otherwise non-diagnostic. After chest drain insertion, 4.6 litre drained but a hydropneumothorax developed upon drain removal, with no improvement on repeat drain insertion. Right video-assisted thoracic surgery was performed, converted to thoracotomy, with repair of diaphragmatic fenestrations, pleurectomy and decortication of trapped lung undertaken. Histological examination of samples of pleura and lung cortex was pathognomic of endometriosis. She was started on norethisterone with no recurrence to date.

The clinical significance of the FUS-CREB3L2 translocation in low-grade fibromyxoid sarcoma.

BACKGROUND: Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft-tissue neoplasm with a deceptively benign histological appearance. Local recurrences and metastases can manifest many years following excision. The FUS-CREB3L2 gene translocation, which occurs commonly in LGFMS, may be detected by reverse-transcriptase polymerase chain reaction (RT-PCR) and fluorescence in situ hybridisation (FISH). We assessed the relationship between clinical outcome and translocation test result by both methods. METHODS: We report genetic analysis of 23 LGFMS cases and clinical outcomes of 18 patients with mean age of 40.6 years. During follow-up (mean 24.8 months), there were no cases of local recurrence or metastasis. One case was referred with a third recurrence of a para-spinal tumour previously incorrectly diagnosed as a neurofibroma. RESULTS: Results showed 50% of cases tested positive for the FUS-CREB3L2 translocation by RT-PCR and 81.8% by FISH, suggesting FISH is more sensitive than RT-PCR for confirming LGFMS diagnosis. Patients testing positive by both methods tended to be younger and had larger tumours. Despite this, there was no difference in clinical outcome seen during short and medium-term follow-up. CONCLUSIONS: RT-PCR and FISH for the FUS-CREB3L2 fusion transcript are useful tools for confirming LGFMS diagnosis, but have no role in predicting medium-term clinical outcome. Due to the propensity for late recurrence or metastasis, wide excision is essential, and longer-term follow-up is required. This may identify a difference in long-term clinical outcome between translocation-positive and negative patients.