ABSTRACT
Two case histories are described of pleural and anterior mediastinal fibrosis presenting as a continuous fibrotic process with thick parietal pleural plaques extending from one pleura to the contralateral pleura through the retrosternal area, and with retroperitoneal fibrosis. Follow-up over 4 years in one case demonstrated rapid progression of disease, with pleural fibrosis preceding retrosternal and retroperitoneal fibrosis. Histopathological analysis in both cases showed non-tumoral fibrosis with broad fibrous bundles surrounding fibroblasts (and lymphocytes in one case). Possible causes such as infections and exposure to ergot derivatives were excluded. Both patients had been slightly or moderately exposed to asbestos. These cases represent an unusual new presentation of pleural and retrosternal fibrosis extending beyond the anatomical structures and associated with retroperitoneal fibrosis.
Subject(s)
Asbestos/toxicity , Occupational Exposure/adverse effects , Pleural Diseases/pathology , Retroperitoneal Fibrosis/pathology , Aged , Biopsy, Needle , Disease Progression , Fibrosis , Follow-Up Studies , Humans , Immunohistochemistry , Male , Middle Aged , Pleural Diseases/etiology , Retroperitoneal Fibrosis/etiology , Risk Factors , Syndrome , Tomography, X-Ray ComputedABSTRACT
INTRODUCTION: Low grade endometrial stromal sarcoma (ESS) often expresses oestrogen (ER) and progesterone (PR) receptors, even in metastatic disease. These receptors may also be hormone dependent. CASE REPORT: Two years after the institution of oestrogen replacement therapy (HRT) a woman of 56 presented with haemoptysis which led to the discovery of multiple pulmonary nodules. Twelve years previously the patient had had a hysterectomy for a low grade endometrial stromal sarcoma, ER and PR positive. Surgical resection of the nodules on the right side confirmed the diagnosis of metastatic endometrial stromal sarcoma. The metastases expressed oestrogen and progesterone receptors. Three months after the withdrawal of HRT and treatment with an aromatase inhibitor (letrozole) the contralateral metastases had disappeared and this complete response was maintained for more than 2 years of follow-up. CONCLUSION: Care should be taken in the institution of HRT in a woman with a history of low grade ESS. Hormonal treatment with aromatase inhibitors may be considered in cases where ER and PR are expressed by the primary tumour and metastases, with possible benefits even in metastatic disease.
