Dysembryoplastic neuroepithelial tumour: the Western Australian experience.

Dysembryoplastic neuroepithelial tumour (DNET) is a rare tumour that is usually seen in the context of epilepsy surgery. The purpose of this study was to provide a profile of DNET in an unbiased population. The 2020 brain tumours diagnosed in Western Australia in the 10-year period 1982-1991 were reviewed; 5 cases of DNET were identified. All patients were neurologically intact and presented with complex partial seizures from a young age. Surgery was performed for tumour resection or biopsy in all cases; no patient underwent surgery specifically for epilepsy control. The lesions were intracortical, multinodular and were composed of astrocytes, oligodendrocytes and neurons. Three patients underwent total or subtotal resection and their seizures were either abolished or reduced in frequency. Two patients had biopsies only and were clinically unchanged. There has been no evidence of recurrence in up to 8 years' follow-up. The diagnosis of DNET is important because it is a surgically treatable benign tumour.

Cerebral and spinal cord myxopapillary ependymomas: a case report.

We report the case of a 16 year old male with a left temporal myxopapillary ependymoma which did not demonstrate any connection to the lateral ventricles. The patient had complained of headaches for ten months and signs of increased intracranial pressure for one month. One month post-operatively, because of lower back pain, further investigations disclosed two separate intraspinal myxopapillary ependymomas, one at T12-L1 level and the other at L5-S1 level. It is postulated that the cerebral myxopapillary ependymoma was in fact a secondary from one of the spinal cord tumors. There are only three reports of this type of ependymoma in the cerebrum.

Epidermal inclusion cysts of the clitoris as a complication of female circumcision and pharaonic infibulation.

Female circumcision and pharaonic infibulation is still performed on young females in many parts of the world. It is estimated that two thousand young women living in Britain undergo this ritual every year. The most common complication of this procedure is epidermal inclusion cysts within the infibulation cicatrix. We report the clinical and pathological findings in 10 cases of clitorial inclusion cysts seen at the North West Armed Forbes Hospital, Tabuk, Saudi Arabia between 1989 and 1993. The clinical diagnosis in six cases was that of "clitoral tumor". Two patients presented with complaints of localized pain, one patient presented with dyspareunia and one patient presented with the complaint of a sporadic discharge of white fluid from the circumcision scar. The pathological findings were those of an implantation dermoid in all cases except in two patients where cysts had ruptured and had become inflamed and/or infected. Four cysts were multilocular.

High-grade cerebral neuroblastoma: a case study.

The case of a 48-year-old male patient is reported in whom a primary malignant cerebral neoplasm was cured by its neurosurgical removal and by postoperative radiotherapy and chemotherapy. Initially, from the results of the examination of frozen and paraffin section, the tumour was considered to be a glioblastoma multiforme. Electronmicroscopy, immunohistochemistry and review of the light microscopy of the original biopsy sample after his death by suicide four-and-a-half years later showed the neoplasm to have been a primary cerebral neuroblastoma rather than a glioblastoma. The diagnosis of glioblastoma multiforme, which depends upon multiple non-specific microscopic findings, such as necrosis, abnormal blood vessels, anaplasia and the pleomorphism of tumour cells, often is imprecise. Our experience underlines the need for comprehensive neuropathological studies of malignant cerebral neoplasms, including transmission electronmicroscopy and immunohistochemistry. This is of particular importance in view of the dismal prognosis of glioblastoma multiforme and of the palliative, rather than curative, treatment programmes that frequently are indicated for this tumour. The value of our report is to demonstrate that a cerebral neuroblastoma, which potentially is curable, may be mistaken easily for a glioblastoma-even by competent neuropathologists.

Myopathies associated with hypothyroidism: a review based upon 13 cases.

The clinical and myopathological features of 13 patients with a myopathy occurring in association with hypothyroidism are presented. Seven patients had hypothyroid myopathy, including two with the Hoffmann syndrome and one with the Kocher-Debre-Semelaigne syndrome. Five patients had an inflammatory myopathy and one had polymyalgia rheumatica. Serum CK activity was elevated up to 12-fold in the patients with hypothyroid myopathy and returned to normal after treatment with thyroxine. Pathological changes in these cases included type 1 or type 2 fibre atrophy or hypertrophy, myofibre necrosis and regeneration in four cases and, in one case, prominent core-like areas containing amorphous granulo-filamentous material. The findings in this series of cases illustrate the clinical and histopathological heterogeneity of patients with hypothyroid myopathy and the need to consider other myopathies in hypothyroid patients who present with muscular symptoms.

Paget's disease of the groin associated with adenocarcinoma of the urinary bladder.

A 70-year-old Caucasian male developed a left groin tumor with epidermal infiltration of Paget's type. Five months later the ureter was found to be obstructed by a bladder adenocarcinoma cytologically identical to the skin lesion. Both tumors contained carcinoembryonic antibodies, using the immunoperoxidase method. Despite radiation therapy, the patient died a month later. There are no previous reports of association between urinary bladder adenocarcinoma and extramammary Paget's disease of the skin.

Pineal neoplasms and third-ventricular teratomas in Niigata (Japan) and Western Australia. A comparative study of their incidence and clinicopathological features.

It is claimed that Japan has the highest incidence of pineal neoplasms in the world. To test this hypothesis, we reviewed all pineal neoplasms and third-ventricular teratomas that were diagnosed histopathologically and treated neurosurgically over 10 years (1975-1984) in Niigata City, Japan (12 patients) and in Perth, Western Australia (9 patients). The age-standardized incidence rates per million person-years were 0.61 for Niigata and 0.70 for Western Australia; thus, the contention that Japan has the highest incidence in the world of pineal neoplasms is not confirmed.

Cerebral gliomas with palisading necrosis: glioblastoma multiforme or high grade cerebral neuroblastoma? A light and electron microscopical study of three cases.

Three adult patients had cerebral malignant gliomas composed predominantly of small cells and displaying many areas of palisading necrosis, which are usually characteristic of glioblastoma multiforme. Light and electron microscopy, however, revealed tumour cells with neuritic processes, while astrocytic differentiation was convincingly excluded. It is suggested that palisading necrosis is not pathognomonic of glioblastoma multiforme and that high grade cerebral neuroblastoma should be considered in the differential diagnosis. The need for comprehensive light and electron microscopic studies for the accurate classification of gliomas is stressed.

Palisades in primary cerebral neuroblastoma simulating so-called polar spongioblastoma. A light and electron microscopical study of an adult case.

A frontal lobe neoplasm in a 25-year-old Caucasian man showed the typical histological pattern of a "polar spongioblastoma." Immunoperoxidase staining for glial fibrillary acidic protein (GFAP) was negative while silver stains in paraffin-embedded tissue, and electron microscopy displayed neoplastic cells with neuritic processes. Ultrastructurally there were microtubules, synapses and dense-core neurosecretory granules, all features of a neuroblastic neoplasm. It is suggested that this new growth with its polar spongioblastic appearance is, in fact, a moderately malignant primary cerebral neuroblastoma.

Meningioma occurring twenty-three years after a successfully treated cerebellar medulloblastoma: case report.

A 6 year old boy had a mid-line cerebellar medulloblastoma treated by local resection followed by craniospinal irradiation. Twenty-three years later he underwent temporal craniotomy to remove a cystic temporal meningioma. It is suggested that this is a radiation induced neoplasm.

Cystic fibrosis, Crohn's colitis, and adult meconium ileus equivalent.

A 23-year-old man with cystic fibrosis developed massive rectal bleeding that continued intermittently, necessitating an emergency laparotomy and total colectomy. The resected colon was shortened by fibrosis and displayed widespread excessive production of mucus, forming a carpetlike layer over the mucosa as well as segmental discrete areas of mucosal ulceration with fissures, transmural inflammation, and multiple epithelioid granulomas, which are in keeping with Crohn's disease. One case, in a seven-year-old girl, had been reported previously.

Causes of organic dementia: a necropsy survey of 60 cases.

The neuropathological findings in all demented patients who were necropsied at the Sir Charles Gairdner Hospital during a 10-year period were reviewed. Of a total of 60 cases, 44 (73.3%) had the typical changes of Alzheimer's disease, while a variety of other conditions accounted for the remaining 16 cases--neoplasms (four patients); lacunar disease (four patients); viral infections (three patients: herpes simplex in two and Creutzfeldt-Jakob disease in one); non-specific brain atrophy (two patients); and single cases of Huntington's chorea, subdural haematomas and methotrexate-induced leucoencephalopathy. Associated cerebral pathologies were found in 17 (38.6%) of the cases of Alzheimer's disease and included vascular lesions (eight cases), subdural haematomas (six cases) and Wernicke's encephalopathy (two cases).

Pleuropulmonary necrobiotic rheumatoid nodules. A review and clinicopathological study of six patients.

Pleuropulmonary rheumatoid nodules were diagnosed histologically in six patients of whom five were known to have rheumatoid arthritis; the pulmonary lesion preceded the development of arthritis in the sixth patient. Pulmonary lesions are commonly found in patients with rheumatoid arthritis. These lesions are either non-specific (effusions, pleurisy, fibrosis, arteritis and obliterative bronchiolitis) or the specific necrobiotic nodules that constitute Caplan's syndrome in association with pneumoconiosis. The necrobiotic nodules are usually pleural or subpleural and rarely occur in the bronchial tree. Pulmonary necrobiotic nodules can appear before, coincident with, or after the onset of arthritis. It is essential to distinguish these lesions from infections or neoplasia.

Angiocentric pulmonary granulomas in tuberculosis.

An open-lung biopsy performed in a 15-year-old girl because of left sided pulmonary nodules revealed striking angiocentric necrotising granulomas. No acid fast bacilli (AFB) were cultured or demonstrated in tissue sections, however, the diagnosis of tuberculosis was suggested. Anti-tuberculous therapy resulted in both clinical and radiological cure within 12 months. The differential diagnosis of this type of lesion should include pulmonary tuberculosis as well as the non-infective angiocentric granulomas such as lymphomatoid granulomatosis and has major therapeutic implications.

Scleromyxedema and inflammatory myopathy: a clinicopathologic study of three patients.

Scleromyxedema (lichen myxedematosus) is a rare cutaneous manifestation in patients with idiopathic inflammatory myopathy. The clinical and histological findings in three patients with this association are presented. Two patients had a severe inflammatory polymyopathy which responded incompletely to corticosteroid therapy, while in the third, who developed esophageal carcinoma, the myopathy was relatively mild and the skin changes were the dominant feature. The occurrence of scleromyxedema in patients with inflammatory myopathy appears to carry a poor prognosis.

The neuropathology of progressive autonomic failure of central origin (the Shy-Drager syndrome).

The neuropathological features of the "Shy-Drager syndrome" have, in the past, been unsettled. The position was recently clarified by Oppenheimer who reviewed the 51 reported patients with progressive autonomic failure in whom neuropathological findings were given. He divided these cases into two groups. Group I included those with lesions of the pigmented nuclei of the brain stem which contained Lewy bodies. These he further subdivided into subgroups IA or IB according to whether or not the patient showed clinical evidence of Parkinson's disease in addition to the common denominator of orthostatic hypotension. In group II he placed those patients in whom multiple system atrophy was found at necropsy. These were the majority. Loss of neurons from the intermediolateral columns of the thoracic spinal cord, thought to be the cause of the postural hypotension, was present in all cases. To illustrate the new classification the necropsy findings in 3 patients taken from the files of the Royal Perth Hospital are described. One of these showed the lesions of Parkinson's disease with Lewy bodies, and thus conformed to Oppenheimer's type IA. The two other patients showed multiple system atrophy and thus belonged to Oppenheimer's type II. All three showed loss of neurons in the intermediolateral columns of the thoracic spinal cord. We believe that Oppenheimer's classification of progressive orthostatic hypotension improves the clinicopathological understanding of the syndrome and is thus useful as well as informative to the practising neurologist.

Primary cerebral neuroblastomas. A clinicopathological study of one adolescent and five adult patients.

Neuronal differentiation was demonstrated by light microscopy, immunohistochemistry and electron microscopy in the cerebral neoplasms of one adolescent and five adult patients. The tumours exhibited a wide spectrum of cellular differentiation from low to high grade malignancy which seems to correlate well with their biological behaviour. The natural history of these 6 cerebral neuroblastomas is rather long compared to that of other malignant primary cerebral neoplasms of adulthood; however, 2 patients died, one survived about 5 yr after initial symptoms whilst an untreated patient survived more than 12 yr. It is suggested that morphological grading along the lines of Kernohan's system may be useful in determining the prognosis and postoperative management of patients with cerebral neuroblastomas.