[Primary angiosarcoma of the heart. Histoenzymological and ultrastructural study of a case].
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Biomedical subjects
Publications and source records attributed to M Auriol.
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Among 873 salivary gland tumors, 15 cases of acinic cell tumors were found (frequency of 1.6%). They occurred approximately evenly in man and women, the mean age being 41 years. They were located principally in the major salivary glands (12 cases in the parotid and 1 case in the submandibular gland) and only twice in accessory glands. Histologically, these tumors were divided into two groups: group I = highly differentiated tumors (less than 20% undifferentiated cells) and group II = poorly differentiated tumors (more than 20% undifferentiated cells). These histological feature seems to play a role in the clinical behaviour of these neoplasms. Thus, the 9 patients with highly differentiated tumors were alive and well 5 to 22 years after the treatment. On the other hand, 5 of the 6 patients with poorly differentiated tumors had local recurrences (4 cases) and lethal metastases to distant lymph nodes and bone (3 cases). The ultrastructural study corroborated the analogy of differentiated tumoral cells with normal acinic cells (numerous secretory granules and ergastoplasmic cisternae). Moreover, the undifferentiated cells had numerous ribosomes and their morphological structure was similar to that of intercalated duct cells. The histogenesis of this rare tumor is discussed. The acinic cell tumor seems more likely to arise from immature cells of intercalated ducts than from adult acinic cells.
Histoenzymologic and ultrastructural examinations were conducted on a doubly located calcified odontogenic epithelial tumor. The cells of the tumor, developing in both the maxilla and mandible, differed from those of the common ameloblastoma, being all of the malpighian type without any secretory polarity. Furthermore, optical and electronic microscopy showed evidence of marked alkaline phosphatases and ATPases activity in their numerous peripheral microvilli. These identical activities to those of the stratum intermedium could suggest the origin of the tumor from these latter cells. But, like the preameloblasts of the dental germ, these tumoral cells produce in their intracytoplasmic vesicles and then excrete into the stroma, a granulofilamentous substance, pseudo-amyloid, which could thus represent an abnormal protein of the enamel matrix. The calcifications, mainly present in this amyloid-like substance, are the expression of an attempt to mineralize this abnormal matrix. Stromal calcifications may result from a sketchy odontogenetic induction, as shown by the intense activity of ATPases and alkaline phosphatases on the fibroblastic cytoplasm membranes.
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Following the death of a patient with calcific aortic stenosis and complete heart block, five days after surgical replacement of the aortic valve, the autopsy demonstrated multiple calcific emboli in the brain, surrounded by hemorrhagic necrosis. Coronary, renal and splenic emboli were also found. This article discusses the incidence and topographical distribution of these emboli as well as the complications to which they may give rise.
The six cases of malignant histiocytosis reviewed in this article all presented with unusual manifestations (muscular masses, renal failure, dysenteriform syndrome, neurological symptoms, or isolated splenomegaly). With one exception, correct diagnosis was only made post mortem. In addition to the usual hematological localizations, the autopsy revealed unusual topographical features in each case, explaining the atypical clinical manifestations.
The case of Shulman's syndrome reported in this article is unusual, because of the topography of the lesions and the lethal course. The perivertebral fasciitis, which was pseudotumoral in appearance, induced a myelomalacia by compression of the cervical spinal cord. In addition, the autopsy revealed severe tissue eosinophilia, cutaneous sclerodermiform sclerosis and pharyngoesophageal lesions unusual in this disease. The pathogenesis of the syndrome is discussed.
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The ameloblastic fibrosarcoma is a rare variety of neoplasm. Three new cases reported here occurred within preexistent benign odontogenic tumors (ameloblastic fibroma or fibro-odontoma). These large, osteolytic tumors, spreading to adjacent soft parts, recurred after surgical treatment in two cases. One of them had a lethal course, with pleuro-pulmonary, mediastinal lymph node and hepatic metastases. Histologically, these sarcomas show a malignant mesenchymal component and few benign ameloblastic islands, which often disappear after one or several recurrences. Histoenzymologically, a high level of alkaline phosphatase and ATPase activities is always present, a feature not present in common fibrosarcomas. The ultrastructural study demonstrates, in analogy with odontogenic myxomas, clear cells provided with numerous microfilaments, secretory cells and also some fibroblasts and myofibroblast-like cells. In addition to these pleomorphic cells, a great number of peculiar granular cells with numerous lysosomal bodies were also found. The histogenesis of these tumors in unknown. Perhaps the epithelial component, being unable to assume its functions of organization, may initiate the malignant transformation of its odontogenic mesenchyme.
The authors discuss a large series of biopsy and necropsy findings in patients with giant cell arteritis (Buerger's thromboangiitis, Horton and Takayasu's diseases), and then in patients with other forms of arteritis with an inconstant plasmodial factor, such as the Chürg and Strauss syndrome, Wegener's disease, the pseudo-sarcoidosis form of lymphomatoid granulomatosis. The histology, correlated when necessary with ain immunofluorescent study, was completed by a topographical and evolutional study of the lesions in all cases. The pathogenesis of these different forms of arteritis is discussed in the light of the ultrastructural data concerning the formation of the various types of giant cell and the methods of identification of the vascular walls.
One case of myoepithelioma of the submandibular gland is reported. The tumor was composed of mixed spindle-shaped and plasmacytoid cells. The electron microscopy showed intracytoplasmic myofilaments, with variations in number and in repartition from one cell to another. Histoenzymologically, ATPasic and alkaline phosphatase activities could not be demonstrated in these poor differentiated myoid cells. Usually, the tumor has a good behavior. It represents a rare tumor (7 cases - 0,8% - in a retrospective study of 850 salivary gland tumors). Without demonstration of myofilaments by ultrastructural analysis, the diagnosis of such a tumor is very difficult. Taking into consideration new concepts about the myoepithelial cell, the histogenesis of this neoplasm is discussed.
A 5.1/2-year-old girl presented with an isolated diffuse gingival hypertrophy and an abnormal mobility of teeth (alveolysis on roentgenograms). The diagnosis of a plasmacytoma was suspected after pathological and immunohistological examinations of a gingival biopsy. Plasma cells were secreting monoclonal immunoglobulins (heavy chain IgA and light chain lambda), quite different from polyclonal secretions of a reactive plasmacytosis. The electron microscopic study demonstrated polymorphic plasma cells (well-differentiated, dysplastic or undifferentiated cells). The isolated nature of this tumor was confirmed by biological tests and radiological investigations. The extremely rare diffuse gingival locations of extraosseous solitary plasmacytomas was proved by a review of the published litterature. The evolution of such gingival tumors is unknown. It seems to be variable, and, perhaps, diffuse myelomas may develop later on (as in other sites of extra-osseous solitary plasmacytoma).
139 salivary gland biopsies have been studied in patients with Sjögren's syndrome. Histologically, we classified labial salivary gland injuries (127 cases) in 3 groups based on striated duct distension: first, some tubular ectasia with few interstitial lymphoid cells; second, much ectasia associated with many lymphoid cells and early pericanalar sclerosis; third, intensive ectasia, scarce lymphocytes, severe peri and intralobular sclerosis and extensive destruction of acini. This grading is positively correlated with that of parotid biopsies and also with abnormalies on 60 parotid sialographic radiogramms performed simultaneously. Histoenzymological and ultrastructural studies demonstrate, at an early stage of the disease, epithelial and myoepithelial alterations in striated ducts, with tubular ectasia. Simultaneously, an intensive regeneration from intercalated ducts takes place. Nevertheless that regeneration fails to repair myoepithelial cells. Furthermore, young myoid cells, leaving tubular walls, migrate in interstitial tissue and are responsible for accumulation of membranoid material and later of collagen sclerosis.
A case of sarcoid-like granulomatosis involving the lung, skin and a large cervical vessel is presented. The patient died 4 years after the beginning of the disease with a diffuse angioimmunoblastic lymphadenopathy and a sarcomatous change. Although peculiar histological lesions were seen, with a majority of epithelioid and giant Langhans cells, the extra-pulmonary involvement and the poor prognosis were similar to those of the typical lymphomatoid granulomatosis (Liebow's disease).
An histoenzymological study (including oxidative enzymes, diaphorases, acid and alkaline phosphatases and naphtolesterases) of 41 biopsy and operation specimens revealed interesting factors in the diagnosis of some lesions of the buccal mucosa, and also of cysts and ameloblastomas of the jaw. When compared with normal buccal mucosa and epidermis, the enzymatic activities found in the oral lesions, fell into three different types. In non dysplasic leukoplakia, enzymatic activities were found that were similar to those of the epidermis (high oxidative activities, particularly prominent in basal cells and in the granular layer and esterasic activity beneath the keratinised layer). In lichen planus, some vacuolized or necrotic basal cells occurred which lacked enzymatic activity. In the upper layers, the distribution of the enzymes was irregular. In severe dysplasia and epidermoid carcinoma, numerous variations of oxidative, esterasic and acid phosphatase activities were seen from one cell to another. Among the lesions of the jaws, radicular cysts as well as dentigerous cysts, had low enzymatic activities, similar to those of normal buccal epithelium. The epidermoid cysts (keratocysts), because of their highly differentiated keratinization, like leukoplakia, had the same enzymatic activities as epidermis. The enzymatic activities of common ameloblastoma differed from those of malpighian tissues (low oxidative activities without decreasing gradient). Besides round epithelial nests, the stroma showed a high and widespread alkaline phosphatase activity, which indicated a low degree of odontogenic induction. Thus, this peculiar stromal activity may be useful in differentiating between cystic epidermoid varieties of ameloblastoma and the other epidermoid cysts of the jaws.
3 case reports of ectopic salivary gland tissue in the neck are presented. A localized swelling was found in the upper or the middle neck along the anterior border of the sternocleidomastoid muscle. It was not associated with any draining sinus opening on the skin. After surgical excision, the histopathological study demonstrated around a large "cystic" duct few or many lobules of salivary gland tissue containing serous acini and ducts. The embryogenesis of these heterotopies within remanants of the branchial apparatus could be demonstrated in one of our cases by the association of a true branchial cleft cyst with an ectopic salivary gland.
Two types of salivary monomorphic adenomas, the so-called adenolymphoma and oncocytoma (75 cases in a series of 873 salivary gland tumors) were studied. These tumors were almost always located in major salivary glands (essentially in the parotid gland). They were much more common in men (85%) than in women. The oncocyte represented the characteristic cell in these two neoplasms. By electron microscopy, they were seen to contain numerous and abnormal mitochondriae and well-developed lysosomal systems. These findings were correlated with a high level of activity of oxidative enzymes and of acid phosphatases. The histogenesis of these tumors was discussed. They seemed to arise from aberrant striated ducts embedded in heterotopic lymph nodes. The tumoral oncocytes would suffer a primary disturbance of their oxidative metabolism followed by a compensative mitochondrial hypertrophy.
In Midline malignant granuloma, a clinical entity characterized by necrotic ulcerations of the upper respiratory tract, two different histological features can be observed. The first one, called "idiopathic" granuloma, looks like simple granulation tissue and remains a strictly localized disease. In the second type (midline malignant reticulosis or histiocytosis), the granulation tissue contains atypical histiocytic cells. The 3 cases reported here belong to this last group. The early lesions were located in the nose (2 cases) or in the maxillary sinus (1 case). The histological analysis showed in all cases 20 to 40% of atypical histiocytes associated with other "inflammatory" cells. Radiotherapy was used in association with chemotherapy or surgery in the treatment. One patient is still alive at 3 years. We have no follow-up data for another patient. The third patient died after 18 months. An autopsy demonstrated multifocal visceral involvement by atypical histiocytes (malignant histiocytic lymphoma). The histogenesis of this disease, in light of histoenzymological and ultrastructural findings, and its possible relationship to lymphomatoid granulomatosis are discussed.