Melanocytic schwannomas arising within localized pigmented neurofibromatosis of the sympathetic chain.
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Biomedical subjects
Publications and source records attributed to J M Theaker.
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The clinical, light microscopic and immunohistochemical features of six cutaneous heterotopic meningeal nodules (primary, or type I, cutaneous meningiomas) are described. These are rare lesions of the scalp and back which generally present at birth or in childhood. They appear as small subcutaneous fibrous nodules, with no specific clinical features although they can be associated with abnormalities of spinal closure. Microscopically, they show a variable architecture but have similar cytological features. Important diagnostic features include psammoma and small collagenous bodies. An intimate relationship to nerves is seen in some cases, and this may be of significance with regard to the development of these lesions. As with intracranial meningiomas, there is widespread vimentin expression, and most cases show epithelial membrane antigen expression.
Widespread soreness and focal ulceration of the oral mucosa developed in a woman with vitamin B12 deficiency shortly after hydroxocobalamin therapy had commenced. An oral mucosal biopsy showed disordered epithelial maturation with marked cytologic atypia. However, during the next month of treatment, the patient's oral mucosa became clinically and histologically normal. Pathologists should be aware that vitamin B12 deficiency may be associated with epithelial changes similar to those associated with premalignancy.
We have previously shown that a range of anti-epithelial membrane antigen monoclonal antibodies show immunoreactivity with the perineurial fibroblast, both in normal nerves and within a range of common peripheral nerve tumours. We have extended these observations by studying a further collection of peripheral nerve lesions, including some which have previously been thought to have an origin from the perineurial cell. The results provide further evidence that these antibodies reliably stain perineurial cells and that in conjunction with antisera to S-100 protein and neurofilaments, the relative contributions of the perineurial fibroblast, Schwann cell and neurone can be assessed within a nerve-related tumour/lesion. The perineurial fibroblast is an important component of some peripheral nerve lesions.
Neural tumours composed solely of Pacinian corpuscles or showing focal Pacinian differentiation are extremely rare and have only occasionally been reported in the literature. All such lesions to date have been benign. Three lesions are described herein which presented as painful digital masses in middle-aged adults and which were composed of abnormal aggregates of morphologically mature Pacinian corpuscles and intervening small nerves. Only five similar cases have been previously recorded. Possible pathogenetic mechanisms of this unusual hyperplastic phenomenon are discussed.
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In an immunohistochemical study of six cases of giant cell myocarditis, the typical giant cells have been shown to express up to four different macrophage-associated antigens, but not desmin, the intermediate filament protein characteristic of muscle cells. These results support the view that the giant cells have a macrophage rather than a myogenic origin. In contrast, the giant cells found in regenerating skeletal muscle have the immunophenotype of muscle cells and not of macrophages supporting their muscle origin.
This paper describes a case of oral extramammary Paget's disease associated with widespread in situ carcinoma of minor salivary gland ducts and invasive, poorly differentiated carcinoma. Clinically, the case was characterized by unusually extensive and rapidly spreading erythroplakia of the oral mucosa, accompanied by a relatively small invasive salivary gland carcinoma. Despite the similarities of breast and salivary tissue, oral extramammary Paget's disease appears to be extremely rare.
Specimens of normal peripheral nerve and a series of peripheral nerve lesions have been immunostained with three different anti-epithelial membrane antigen (EMA) monoclonal antibodies. Sites of EMA immunoreactivity have been confirmed within perineurial cells of peripheral nerve, noted within the capsule of Schwannomas and palisaded encapsulated neuromas, and also detected with traumatic neuromas and plexiform neurofibromas. No expression was detected within simple neurofibromas, diffuse neurofibromas or within malignant Schwannomas. These sites of EMA expression concur with the suggested involvement of perineurial cells in the formation of the particular lesions. The relationship between EMA expression by the perineurium and the piaarachnoid membrane is discussed.
Approximately 5% of cases of dermatofibrosarcoma protuberans contain dendritic melanocytes; such lesions are often known as Bednar tumours. These neoplasms have received little attention in the literature but seem to show no great clinicopathological differences from conventional dermatofibrosarcoma protuberans except for the presence of melanocytes. The existence of such tumours, combined with ultrastructural evidence, has led some leading authors to regard them all as being of neuroectodermal origin. Seven examples of the pigmented variant are presented herein, of which six have been studied immunohistochemically and one has been examined ultrastructurally. Except for the presence of melanocytes in each tumour, no evidence of neuroectodermal (in particular perineural fibroblastic) differentiation has been demonstrated. The histogenesis of dermatofibrosarcoma protuberans and its pigmented variant is discussed. The possibility that the pigmentation may simply reflect secondary melanocyte colonization from the epidermis should be considered.
A patient presented initially with a testicular mass, which on biopsy had morphological features consistent with malignant histiocytosis. The tumour cells labelled strongly with EBM/11, a murine monoclonal antibody with high specificity for cells of the human mononuclear phagocyte system. Subsequent clinical and laboratory studies confirmed the diagnosis. As poorly differentiated tumour cells reacted with EBM/11, this antibody may be useful in positively identifying malignant tumours with histiocytic differentiation from malignancies of other types where morphological detail alone is inconclusive in tumour classification.
A case of a submandibular mass due to a granulomatous reaction to vegetable matter of dietary origin is presented. The histology of the lesion and its pathogenesis are described. It is compared with previous reports of 'hyaline bodies' in relation to chronic mandibular periostitis.
The expression of cytokeratin intermediate filaments by a tumour has been accepted as evidence of an epithelial origin. Although there have been anecdotal reports of cytokeratin expression within tissues and neoplasms of non-epithelial origin, particularly muscle, there have been no comprehensive studies of its frequency and distribution. In order to investigate this we have studied 51 cases of normal smooth muscle and benign and malignant smooth muscle tumours using a panel of monoclonal antibodies against a range of intermediate filaments (cytokeratins, desmin and vimentin). Cytokeratin expression was noted overall in 50% of normal, benign and malignant smooth muscle tissues. Such expression tended to have a focal or patchy distribution. No case expressed cytokeratins in the absence of both desmin and vimentin. The implication of these findings for diagnostic immunocytochemistry is that intermediate filaments alone are not completely reliable markers of tumour histogenesis and should be used as part of a larger panel of monoclonal antibodies.
We report a case of a long-standing subcutaneous nodule of the scalp in a young man, with the histological features of a primary cutaneous meningioma. The results of an immunohistological study using a panel of monoclonal antibodies gave a similar pattern to that seen in intracranial meningiomas, and distinct from other cutaneous lesions which may be considered in the differential diagnosis.
A patient receiving long-term haemodialysis developed systemic amyloidosis, which was shown immunohistochemically to be of beta 2-microglobulin type, a previously unrecognised form of systemic amyloidosis. Histologically, the amyloid deposits were closely associated with foci of acute and granulomatous inflammation and vasculitis, although it was not clear if the amyloid deposits directly caused the inflammatory process, or if amyloid was deposited preferentially in areas of inflammation of uncertain aetiology.
A case of giant cell myocarditis in a patient with non-Hodgkin's lymphoma is reported. To our knowledge, this is a previously unrecorded association and supports the hypothesis that the aetiology of giant cell myocarditis is related to a changed immune state. Immunohistochemical investigation of this case with a panel of monoclonal antibodies against a range of leucocyte and muscle antigens supports the view that the giant cells have a histiocytic rather than a myogenic origin.
Immunohistology was used for the detection of Legionella pneumophila serogroup I in necropsy tissue. Study of pneumonic lung from the recent Stafford outbreak has shown that this technique has a high sensitivity. A retrospective postmortem examination showed that L pneumophila serogroup 1 was an unusual cause of pneumonia in Oxfordshire during the study period. L pneumophila serogroup 1 can be successfully subgrouped, using a panel of monoclonal antibodies on formalin fixed paraffin embedded sections. Immunohistological methods have a potentially useful role in the diagnosis of Legionellosis at postmortem examination and in the epidemiological investigation of individual cases and outbreaks.
A 62 year old woman presented with subacute dementia and hemiparesis which progressed to death with terminal seizures over 5 months. A cerebral biopsy specimen and necropsy showed the features of neoplastic angioendotheliosis which proved to be lymphomatous. Cerebral CT showed multiple low density areas in the white matter with striking enhancement after high-dose intravenous contrast administration. There was temporary clinical improvement with prednisolone therapy. The clinical features, cerebral CT appearances and partial response to treatment are discussed, with a review of the relevant literature.