Extrapulmonary anaplastic small cell tumours (EPSCC) of the gastrointestinal tract are generally aggressive neoplasms with a poor prognosis. Recent work has demonstrated that EPSCC may be sensitive to the chemotherapeutic regimens employed to treat small cell lung cancer.In this report we present the case of a patient with a primary anaplastic small cell gastric carcinoma treated with combination chemotherapy. Plasma calcitonin, somatostatin, gastrin releasing peptide, total enolase and non-muscle creatine kinase levels were measured before and, if elevated, after completion of induction chemotherapy. Doxorubicin, cyclophosphamide and etoposide treatment resulted in a complete remission which lasted for 11 months. Calcitonin levels, elevated prior to treatment, fell to within the normal range following completion of induction therapy. Extrapulmonary gastric small cell cancer is sensitive to cytotoxic chemotherapy. Like small cell lung cancer, these tumours may produce plasma/serum markers which may be useful in monitoring response to therapy.Case report. A 54-year-old man presented with a 3-month history of severe epigastric pain in October 1992. The pain was waking the patient from his sleep, was aggravated by moving and eased by vomiting. The symptoms were associated with a weight loss of 16 kg. There was no history of dysphagia. He had stopped smoking cigarettes 14 years earlier. Physical examination revealed exquisite tenderness in the epigastrium. On the day following admission to hospital he had an episode of haematemesis.At oesophago-gastroscopy examination a protruberant ulcerating lesion was noted in the gastric cardia below the oesophagogastric junction. The oesophegus appeared normal. Biopsies of the gastric mass (Figs la and b) revealed an infiltrating neoplasm composed of mitotically active small cells with hyperchromatic nuclei, inconspicuous nucleoli and scanty cytoplasm resembling small cell carcinoma of the lung. The cells were uniformly negative for common leukocyte antigen and weakly positive for keratin and neuron-specific enolase. Further biopsies were taken of the gastric antrum which revealed helicobacter pylori positive antral gastritis.A full blood count, renal, liver and bone biochemistry were within normal limits. Bone marrow aspirate and bone biopsy showed no evidence of malignant infiltration. Radiology included a CXR and CT scan of the thorax, a CT brain scan and isotope bone scan all of which were normal. A CT examination of the abdomen revealed evidence of a tumour at the cardia of the stomach with suhmucosal extension to involve the lower oesophagus. A subsequent barium swallow confirmed these findings. A radiolabelled somatostatin analogue scan, using "'In-[DTPA-DPhe'l-octreotide as the radioligand, failed to localise either the primary tumour or show evidence of metastases.Plasma and serum samples were obtained for analysis for the presence of a number of markers. Gastrin releasing peptide, somatostatin, total enolase and non-muscle creatine kinase levels were within normal lim...

Objective: Diagnosis of congenital cystic adenomatoid malformation of the lung (CCAM) in association with recombinant chromosome 18. Method: Ultrasound diagnosis of a CCAM and hydrops was made. The mother was known to have a pericentric inversion of chromosome 18 and had a previous pregnancy with a recombinant chromosome 18 (partial deletion of 18p and partial duplication of 18q). Cordocentesis for karyotype was therefore performed. Result: Fetal karyotype revealed a recombinant chromosome 18, this time with partial deletion of 18q and partial duplication of 18p. Postmortem confirmed a type III CCAM and a septum primum atrial defect. Conclusions: Although deletion/duplication of chromosome 18 is commonly associated with a wide variety of anomalies, the association with CCAM is an unusual one. Fetal and parental karyotyping should be considered in cases of CCAM, because fetal therapy is increasingly being considered in these pregnancies. Current management of parents with pericentric inversions must rely on invasive diagnostic testing in the second trimester because predicting the likelihood of an unbalanced karyotype and phenotype in a fetus is difficult.

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SUMMARY Telangiectasia-associated hepatic fibrosis (TAHF) in a 68-year-old woman with hereditary haemorrhagic telangiectasia (HHT) is described. The patient died of oat-cell carcinoma of the lung. In addition to the structural alterations which have been described previously in HHT, the liver exhibited focal midlobular hepatocytic necrosis and tumour metastases. The possibility that treatment of HHT was causally related to some of the hepatic abnormalities found in our patient and the differentiation of TAHF from true cirrhosis are discussed.

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