Biomedical subjects
Publications and source records attributed to I Friedmann.
Cerebellar haemangioblastomata and angiomata of the retina are the most common vascular tumours seen in von Hippel-Lindau disease. A definite association between this condition and choroid plexus tumour has not been described previously and its presentation as a middle ear mass is unique.
Necrotizing sialometaplasia is a benign self-limiting disorder which can simulate malignancy. We report the third such case of nasal fossa involvement in the literature. The pathology of this condition is discussed.
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This chapter illustrates the use of mono- and polyclonal antibodies as diagnostic tools for neuroendocrine neoplasms of the larynx on the basis of data from the literature and the authors' personal experience. It is suggested that a panel of antibodies should be adopted, rather than to rely on the specificity and sensitivity of a single marker. It is also emphasized that immunohistochemical methods should serve not as a substitute but as a useful adjuvant in evaluating the morphological criteria of these neoplasms.
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A closer study of the pathology of the external auditory meatus shows that there are various non-neoplastic and neoplastic diseases of local origin which are of great clinico-pathological importance. These include Kimura's disease and angiolymphoid hyperplasia with eosinophilia; progressive necrotizing (or malignant) external otitis. Osteoma and ceruminoma are typical neoplasms of the external auditory meatus which is also the site of polypoid granulomatous or neoplastic structures arising from the middle ear cleft. These so-called 'aural polyps' often dismissed as of little or no diagnostic importance, may contain significant clues to a serious underlying infection or to a malignant neoplasm. Profuse bleeding may ensue on removal of such polyps. The histopathological features and their significance are described and discussed.
Immunoglobulins G (IgG) and A (IgA) were found in plasma cells, osteocytes, chondrocytes and in connective tissue of active otosclerotic (= otospongiotic) lesions. The application of antibodies against paramyxovirus and rubella virus antigens reproducibly determined the expression of these antigens at different sites in the otosclerotic stapes, but also in the epithelial cells of the overlying middle ear mucosa. By using specific T-lymphocyte and B-lymphocyte markers, about 80% of the lymphocytes present in the otosclerotic footplate were revealed to be T-lymphocytes. There was neither deposition of immunoglobulins nor any expression of viral antigens in non-otosclerotic footplates which were investigated as controls.
Lethal mid-line granuloma (LMG) is a clinical term, and it is histologically composed of Wegener's granulomatosis (WG), polymorphic reticulosis (PR), and malignant lymphoma (ML). WG is an inflammatory disease, and PR and ML are considered to represent a neoplastic proliferation of lymphoreticular cells. In the present report, a nation-wide study on LMG in Japan is compared with a study from the UK, to examine differences in frequencies of each disease in Eastern and Western countries. A total of 340 cases of LMG were examined. On the basis of histological and clinical findings, these cases were categorized as 68 WG, 129 PR, 92 ML, 44 chronic inflammation not specified, and 7 other related conditions. The crude frequencies of WG, PR, and ML per 100,000 outpatients of ENT clinics in Japan and England were 4, 8 and 6, respectively and 8, 4 and I, respectively. The predominance of malignant lymphoproliferative diseases to WG in Japan (3.5:1) was in marked contrast to the situation in the UK (1:1.6). A review of the pertinent literature suggests a clustering of malignant lymphoproliferative diseases of the nose among Mongolian ethnic groups.
Warthin's tumour arising from minor salivary glands of the nasopharynx is described in a patient with eosinophilic rhinitis and nasal polyposis. The microscopical features of this lesion were indistinguishable from those of Warthin's tumours of the parotid gland. The present case supports the histogenetic theory that the epithelial component of extraparotid Warthin's tumours represents reactive oncocytic metaplasia and hyperplasia induced by chronic inflammation rather than genuine neoplasia. It is suggested that the term Warthin's tumour is preferable to designations indicating neoplasia of these extraparotid lesions which are indistinguishable histologically and ultrastructurally from parotid and juxtaparotid adenolymphomas.
Neuroendocrine carcinomas of the larynx are uncommon tumors of considerable scientific interest and clinical importance. They include typical carcinoid tumors, atypical carcinoid tumors, and small cell neuroendocrine carcinomas. This paper considers these neoplasms from a personal experience and reviews the relevant medical literature. About 200 cases of neuroendocrine carcinomas of the larynx have been reported. The diagnosis is based on light microscopy and is confirmed by ultrastructural evidence of neurosecretory granules. Histochemical and immunocytochemical investigations may support it. Paraneoplastic syndromes associated with laryngeal neuroendocrine carcinomas have been reported occasionally. The histogenesis, treatment, and prognosis of these lesions also are discussed.
Fragments of otospongiotic and otosclerotic footplates were investigated by immunohistochemical methods. Antibodies IgG, IgA, IgM were found to be bound to the vascular connective tissue of the resorption lacunae, IgG also to osteocytes. The application of antibodies against mumps, measles and rubella antigens showed the expression of the relevant viral antigens in the large cells of the resorption lacunae, in the vascular connective tissue, and in osteocytes, osteoclasts and chondrocytes, present in or around the otospongiotic areas. In the sclerotic stage only the perivascular connective tissue and chondrocytes have expressed viral antigens whereas IgG was restricted to the osteocytes of the sclerotic focus and to the residual perivascular tissue. Two footplates with postinflammatory sclerosis serving as controls revealed only IgG in some chondrocytes. Healthy footplates showed neither a deposition of antibodies nor any expression of viral antigens. These results favour a viral aetiology of otosclerosis as an inflammatory vascular reaction of the otic capsule initiated or caused by the viruses of measles, rubella and mumps.
Carcinoid neoplasms, although rare, have to be considered in the differential diagnosis of neoplasms of the larynx. The total number of cases reviewed and listed in the present article is 47. The gross appearance of carcinoid tumours is not characteristic and does not differ from that of other malignant neoplasms of the larynx. Under the light microscope various types can be distinguished, which are described and illustrated in this review article. Available material of five previously published cases and of one new case have been re-examined under the electron microscope and by modern histochemical and immunohistochemical methods. The ultrastructural presence of neurosecretory granules and of argyrophilia by the Grimelius technique were uniformly positive, together with monoclonal antibodies for somatostatin and keratin. These special methods offer a reliable basis for the diagnosis of neuroendocrine neoplasms to which carcinoid tumours belong. The treatment of carcinoid neoplasms of the larynx is surgical.
The ultrastructure of the normal human endolymphatic sac (ES) has been observed by transmission electron microscopy (TEM). The role of the epithelium, the various regions of the subepithelial space and the general anatomy of the ES in pressure regulation were morphologically studied to generate testable hypotheses of human ES function. Light microscopic (LM) and TEM evidence of pressure regulatory mechanisms by endolymph resorption, mechanical factors, and secretory activity are presented. These mechanisms may be useful in designing experimental studies of the ES, and in interpretation of retrospective LM and TEM studies of patients with Menière's disease.
Paramyxoviruses have been described in the osteoclasts in Paget's disease and it has been suggested that the disease was caused by this virus. There is considerable similarity of the histological features of active otosclerosis and Paget's disease and the probable presence of viral antigens in the otosclerotic footplate of the stapes of 15 patients has been investigated by means of immunohistochemical methods. The active otosclerotic lesions of all 15 patients studied displayed a high concentration of antibodies of the IgG-class which was less pronounced in the cytoplasm of the cells of the adjacent enchondral layer. Using polyclonal antibodies against measles and rubella viruses, both antigens could be shown expressed by the chondrocytes of the enchondral layer and by occasional osteocytes and osteoclasts of the otosclerotic lesion. IgG antibodies or viral antigens were absent in the normal bone of the stapes specimens. The interpretation and significance of these findings is discussed, with particular reference to the pathogenesis of otosclerosis.
A 69-year-old white female had a left superficial parotidectomy for a papillary cystic oncocytoma. The histologic appearance was identical to Warthin's tumor except that it lacked a lymphoid component. This case suggests that the general morphology of a Warthin's tumor does not depend on the presence of lymphoid tissue or association with an intra-parotid lymph node. We reviewed 50 patients with Warthin's tumors to investigate the histogenesis of the lesion. We found that most Warthin's tumors (86%) appeared to be within lymph nodes, as indicated by the presence of a lymph node capsule or sinuses. The anatomy of intra-parotid lymph nodes in glands containing Warthin's tumors has been compared with that found in 11 parotid glands containing mucoepidermoid carcinomas. So-called heterotopic ductal inclusions may actually represent the normal intimate relationship of parotid gland to intra-parotid lymph nodes.
The structural basis of the combined conductive and sensorineural deafness has been described in two patients with Hurler's disease. All parts of the ear contained numerous large vacuolated Hurler cells, the vacuoles being distended lysosomes from which accumulated glycosaminoglycans had been dissolved during fixation of the tissue. The external and middle ears also showed chronic inflammation. There was resorption of the bone in the mastoid process by masses of Hurler cells and abnormal new bone with prominent cement lines. The blood vessels were surrounded by a 'blue mantle' of osteoid tissue similar to that which is usually associated with otosclerosis. The stapes appeared deformed and was covered by thickened mucosa and granulation tissue. The bone structure of the ossicles resembled that of the mastoid process. The organ of Corti was degenerate and the Reissner's and tectorial membranes were adherent to one another and covered by haemorrhagic material near the vascular striae. The blood vessels in the striae were congested and the scalae media and tympani contained some blood. The neurons in the basal coil of the spiral ganglion were replaced by Hurler cells. The vestibulo-cochlear nerves were disrupted by numerous Hurler cells. These pathological findings adequately explain the combined conductive and sensorineural deafness in these cases. They are also discussed in relation to some other clinical and pathological aspects of these two specific patients.