The autopsy findings in a clinically and biochemically documented case of adult-onset acid maltase deficiency presenting with limb girdle myopathy are presented. The skeletal muscles, tongue, extraocular and smooth muscles of gut and arterioles showed a vacuolar myopathy, most severely affecting proximal skeletal muscles. Muscle spindles were severely affected in all muscles. The heart showed basophilic degeneration and a vacuolar myopathy. The visceral organs and nervous system were morphologically normal. Possible mechanisms for this differential involvement of muscles and tissues are discussed.

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Fallopian tube carcinoma can be histologically indistinguishable from and has a similar clinical behavior to epithelial ovarian carcinoma. However, it is considerably less common; only approximately 1000 cases have been recorded in the literature. In the prevalence screen of 22000 women participating in The Royal London Hospital, London, UK, ovarian cancer screening project, three cases of early stage primary fallopian tube carcinoma were diagnosed following the finding of an elevated serum level of the CA 125 antigen. The ratio of epithelial ovarian : tubal cancer developing in these postmenopausal volunteers was 6:1. This is 25-fold greater than the expected ratio. It is difficult to attribute this finding to population selection bias. However, it is possible that the screening test was particularly effective in detecting tubal carcinoma or that, in clinical practice, the true primary site of origin of some tumors classified as widely disseminated ovarian cancer is in the fallopian tube.

Elevated serum levels of the tumour-associated antigen CA 125 occur in more than 80% of cases of ovarian carcinoma. The antigen can be demonstrated in formalin-fixed tissue using the monoclonal antibody OC 125, which localizes it to the surface membrane or cytoplasm. This study was performed to determine the relationship between pre-operative serum levels of CA 125 and the subsequent immunocytochemical findings in the surgical specimen. Paraffin-wax embedded sections from 40 consecutive borderline and frankly malignant ovarian epithelial tumours were stained with OC 125. The pattern and distribution of immunostaining were investigated in relation to histological appearances. Serous tumours showed a 100% correlation between immunocytochemical findings and elevated serum levels of CA 125. Amongst the other histological types, correlation was less good; mucinous tumours and undifferentiated carcinomas showed a poor correlation. Immunostaining within tumours was heterogeneous and only loosely related to morphological appearances. Our finding suggests that, with the exception of serous tumours, immunolocalization of CA 125 is insufficiently sensitive to provide reliable clinical guidance to the likely value of serum CA 125 monitoring on follow-up.

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