Fatal cytophagic panniculitis and haemophagocytic syndrome.

We present two fatal cases of cytophagic panniculitis (CP) and haemophagocytic syndrome (HPS). In the first of these, there was an underlying T-cell lymphoma but in the second no associated disease was found. HPS is a frequently fatal disorder of immune regulation, characterized by fever, histiocytic haemophagocytosis, hepatosplenomegaly, pancytopenia, hypertriglyceridaemia and coagulopathy; CP is a less common manifestation. A number of benign and malignant conditions may present with HPS, the clinical findings and investigations aiding in determining an underlying disorder. Therapy is both supportive and directed at any associated illness, but often very difficult as diagnosis is delayed.

Fibroelastoma: case report and review of the literature.

A 64 year old woman presented with right hemianaesthesia and was found to have a pansystolic apical murmur with systolic and diastolic posture related plops. Echocardiography revealed a mobile mass on the mitral valve apparatus that was confirmed by magnetic resonance imaging. This was successfully excised and was proven on histopathological examination to be a fibroelastoma. Other cases of fibroelastoma from the literature are reviewed.

Mycobacterium chelonei keratitis: a case report and review of previously reported cases.

A 56-year-old woman who wore hard contact lenses developed a keratitis due to Mycobacterium chelonei. The organism was only sensitive to imipenem and partially to ciprofloxacin and erythromycin. After an initial response to topical therapy with these antibiotics the infection relapsed and a penetrating keratoplasty was performed, with resulting cure. M. chelonei has not previously been reported as a cause of keratitis associated with hard contact lens wear; neither has its treatment with imipenem and/or ciprofloxacin. A detailed photographic record showing the natural history of the keratitis is presented. Previously reported cases of M. chelonei keratitis are reviewed.

Carcinosarcoma arising in eccrine spiradenoma. A clinicopathologic and immunohistochemical study of two cases.

Malignant change in a benign eccrine spiradenoma is rare. Only 13 such cases have been previously reported. Two further cases are described herein, both of which were carcinosarcomas and arose in middle-aged women. In each case continuity was demonstrated between the benign and malignant epithelial components and the sarcomatous element. In case 1 the sarcomatous component showed osteocartilaginous and rhabdomyoblastic differentiation, whereas in case 2 only osteosarcoma was evident. To our knowledge, carcinosarcoma has not been previously described arising in eccrine spiradenoma. The literature regarding malignant eccrine spiradenoma is reviewed.

Metastasis to the temporal bone, secondary to carcinoma of the bladder.

An 80-year-old man was seen in the clinic with a two month history of pain, bleeding and deafness in his left ear; accompanied by a left facial palsy. Six months earlier, anaplastic carcinoma of the bladder had been diagnosed and treated with radiotherapy. On examination there was a mass in his left external auditory canal and VII, VIII, IX and X left cranial nerve palsies. Histology of a biopsy was identical with his original bladder tumour. He received a course of palliative radiotherapy but died shortly after. Post-mortem demonstrated the left temporal bone metastasis extending into the middle and posterior cranial fossae. There was no evidence of residual bladder tumour, or other primary tumour. Temporal bone metastases are probably more common than generally thought. Presentations like this may become more common as cancer therapy improves, with patients surviving longer and presenting later with metastatic disease.

Treatment of acromegaly by external irradiation.

Despite the place of hypophysectomy as the primary treatment in acromegaly, external radiotherapy maintains a role as a relatively slow but effective therapy for inadequately treated patients or those unsuitable for operation. Over the last 25 years our radiotherapy regimen has differed from the published series in that we give a larger dose per fraction, with fewer treatments. We have analysed the efficacy and side-effects of this regimen in 27 subjects with acromegaly. Growth hormone levels have fallen by, on average, 27% per year in the first five years, 83% of subjects achieving a basal growth hormone of less than 10 mU/l. The acute and chronic side-effects of irradiation are discussed, including the relevance of estimates of biological potency, for example the Time Dose Fraction (TDF). One patient suffered visual loss that was most likely to be secondary to the radiotherapy. We also report the histological appearances of the pituitary fossa in five subjects previously treated with radiotherapy.

Sjögren's syndrome and fibrosing alveolitis complicated by pulmonary lymphoma.

The case of a middle aged woman who presented with fibrosing alveolitis, in her mid-forties, followed by a sicca syndrome and who subsequently developed a pulmonary lymphoma (B cell) while receiving azathioprine therapy is recorded. Of interest was the absence of polyclonal B cell activation, e.g., production of rheumatoid factor, hypergammaglobulinaemia, high titre antinuclear antibodies or antibodies to extractable nuclear antigens (ENA) during most of her illness. Persistently raised IgM levels and low IgA levels were demonstrated. The relevance of azathioprine to development of the lymphoma is discussed.

Cyanotic tetralogy of Fallot in a 77 year old man.

We report the clinical details of a 77 year old man with classical tetralogy of Fallot. The patient had clubbing and cyanosis at birth, and exertional squatting in childhood. He was asymptomatic as an adult until the seventh decade, when he developed biventricular failure and had an episode of bacterial endocarditis. He finally died of cerebral infarction and bronchopneumonia following abdominal surgery. In spite of investigations, the diagnosis was not made in life, but only discovered at post-mortem. A persistent ductus arteriosus was also found. Increased pulmonary blood flow via the ductus is believed to have facilitated this patient's unusual longevity.

Epithelial markers in thyroid carcinoma: an immunoperoxidase study.

Ten cases each of papillary, follicular, anaplastic and medullary carcinoma of the thyroid were stained for thyroglobulin, calcitonin, epithelial membrane antigen (EMA), carcinoembryonic antigen (CEA) and cytokeratin (CAM 5.2). Monoclonal or affinity purified polyclonal antibodies, and an indirect immunoperoxidase technique were used. All the papillary and follicular tumours, 5/10 anaplastic and 3/10 medullary carcinomas contained thyroglobulin. Only the 10 medullary carcinomas stained positively for calcitonin. Three out of 10 papillary, 1/10 follicular, 0/10 anaplastic and 10/10 medullary carcinomas were positive for CEA. Nine out of ten papillary, 7/10 follicular, 2/10 anaplastic and 3/10 medullary carcinomas were positive for EMA. Ten out of 10 papillary, 10/10 follicular, 5/10 anaplastic and 10/10 medullary carcinomas were positive for cytokeratin. The presence of calcitonin and CEA is of value in the diagnosis of medullary carcinoma, and enable its distinction from anaplastic thyroid carcinoma. Thyroglobulin is a useful marker in thyroid carcinomas.

Multicentric extra-abdominal 'myofibromatosis': report of a case with ultrastructural findings.

A case of multicentric extra-abdominal fibromatosis, a rare entity, arising in a young female is presented. Three separate lesions developed in the same limb over a period of 11 years, one of which recurred on two occasions. Electron microscopy of the most recent tumour showed a predominance of myofibroblasts. The literature regarding extra-abdominal fibromatosis is reviewed and discussed. Redesignation of both abdominal and extra-abdominal fibromatoses as myofibromatoses is suggested.

Soft tissue chondroma--a study of 15 tumours.

Fifteen cases of soft tissue chondroma have been reviewed. This lesion shows an equal sex incidence and occurs predominantly in middle-aged individuals. The majority arise in the hands and feet. Histologically these tumours are largely composed of adult-type hyaline cartilage, but in all cases foci show nuclear atypia and pleomorphism. In cartilaginous lesions of the axial skeleton or large limb bones this would probably have led to a diagnosis of well differentiated chondrosarcoma. Despite these worrying features none of the seven cases for which follow-up information is available recurred. The importance of recognizing the benign nature of these little-known tumours is stressed. The differential diagnosis of soft tissue cartilaginous tumours is briefly discussed.

Suction biopsy in Hirschsprung's disease.

Seventy two children with symptoms and signs consistent with Hirschsprung's disease had full thickness and suction rectal biopsies performed. Results were identical with both methods, except for one case of total aganglionosis of the colon. Full thickness biopsy no longer has a place as a screening method.

Glomus tumours of the coccygeal body associated with coccydynia. A preliminary report.

Two cases of coccydynia are presented. Their definitive treatment was excision of the coccyx and the pericoccygeal tissues. The histology in both cases revealed a glomus tumour of the coccygeal body and the symptoms were completely relieved after operation. Glomus tumours of the coccygeal body may be the cause of some cases of coccydynia.

Astrocytic differentiation in medulloblastoma.

A case of cerebellar medulloblastoma with transitional features towards malignant fibrillary astrocytoma is described. In the cerebellum the tumour is characterised by extensive subpial infiltration with cells of undifferentiated type, and the astrocytic component could only be identified by a positive glial fibrillary acidic protein reaction. In the brainstem the character of the growth transforms to that of diffuse astrocytoma. This demonstrates the potential for differentiation existing in a "primitive" neuroepithelial neoplasm.

Angiosarcoma of pericardium. Problems in diagnosis and management.

The clinical histories of two patients with angiosarcoma of the pericardium are described. Both were previously well young men who presented with cardiac tamponade caused by haemorrhagic pericardial effusions. If a pericardiectomy is undertaken for diagnosis, wide excision of the pericardium is recommended. This will provide a large specimen for histological examination and may prevent subsequent development of constriction.