Extrapulmonary lymphangioleiomyomatosis: an unusual cause of biliary tract obstruction.

We present a patient who was diagnosed with retroperitoneal lymphangioleiomyomatosis (LAM) and who developed biliary tract obstruction caused by LAM in the papilla of Vater. After endoscopic retrograde cholangiopancreatography (ERCP) and papillotomy, the patient's liver enzymes normalised. Disease progression was slowed down with gosereline and interferon alpha 2b (IF N-alpha 2b). In patients with LAM and signs of biliary tract obstruction, disseminated LAM should be considered. IFN alpha 2b can be a useful treatment in patients with widespread LAM.

Evaluation of a cytological scoring system for predicting histological grade and disease-free survival in primary breast cancer.

A simple cytological scoring system was evaluated as a method of predicting histological grade and disease-free survival in 79 patients with primary breast cancer. The mitotic activity index and oestrogen receptor status were also assessed for their predictive value. The concordance between cytological scores and histological grades was good (80%) for low-grade lesions, but poor (45%) for high-grade lesions. Similar results were found using the mitotic activity index as a prognostic indicator. Cytological grading was not found to be an independent prognostic indicator after a median follow up of 8 years.

[Lyme psychosis]. / Lyme-psychose.

A woman aged 64 was admitted to the psychiatric department because of a psychotic decompensation with visual hallucinations, disorientation in time and space and associative thinking. On psychotropic drugs the condition failed to improve; subsequently neurological symptoms developed. EEG abnormalities prompted a lumbar puncture. In the CSF a strong plasma cell reaction with atypical cells was observed. The enzyme immunoassay for Borrelia burgdorferi was positive and after treatment with penicillin the psychiatric and neurological signs and symptoms disappeared. From the history which could then be taken it appeared that the patient had been bitten by ticks. Her husband aged 66 years passed through a similar episode of disease.

Extra-adrenal phaeochromocytoma presenting as fulminant malignant disease with tumour positive sputum cytology.

A case of a 69-year-old man with an extra-adrenal malignant phaeochromocytoma is described. Sputum cytology revealed metastatic cells, which have not been reported previously in malignant phaeochromocytoma. This case is also remarkable for the short duration of disease, rapid progression and extensive spread of metastases, the radiological aspect of metastatic lesions shown by chest X-ray, hypercalcaemia and extremely high levels of circulating catecholamines and urinary metabolites.

Prognostic scoring systems in differentiated thyroid carcinoma: which is the best?

The usefulness of three prognostic scoring systems in differentiated thyroid carcinoma (the TNM system, the EORTC score and the AGES index) were evaluated for use in a medium size teaching hospital. Twenty-one patients with papillary (n = 13), mixed (n = 2) and follicular tumours (n = 6) were studied. Median follow-up was over seven years (range 10-194 months). Four patients died. The TNM system gives a poor discrimination between patients with a good prognosis and those with a lethal outcome and is of little value in these tumours. The EORTC score, which is easy to calculate, gives a good prediction of survival. The best discrimination between a good and a bad prognosis was found using the more complicated AGES index for papillary tumours. Of patients with a papillary or mixed papillary carcinoma, one of the two patients with a score of four or more actually died, while all with a good index of less than 4 (13/15 of this subgroup) survived, giving the best prognostic bisection of our patient group. Even in the combined group of all differentiated thyroid carcinomas, the AGES index gives the best prognostic bisection, with 33 percent of patients in the poor prognostic group, of whom 57 percent died. This makes the AGES index the most useful prognostic scoring system.

Intraneural lipofibroma of the median nerve.

A case of an intraneural lipofibroma of the median nerve is described. The patient was first seen with an enlarging mass on the flexor side of the wrist and in the palm. After interfascicular dissection of the tumor there was a permanent loss in sensibility and motor function. In most cases decompression of the nerve will suffice; resection by means of interfascicular dissection is rarely justified.

Legionnaires' disease: case report with special emphasis on electron microscopy and potential risk of infection at autopsy.

A case of Legionnaires' disease (LD) is presented in which the diagnosis was made initially by EM and later confirmed by specific immunofluorescence of formalin-fixed, paraffin-embedded lung tissue. The possibility of an infection with Legionella pneumophila during autopsy is suggested by the fact that the pathologist who performed the post-mortem examination developed signs and symptoms of an upper respiratory tract infection 2 weeks afterwards. Approximately 4 weeks after the onset of his illness a significant antibody titre against L. pneumophila was found in his serum, whereas 6 months later the antibody titre was insignificant.