[Necrotizing sialometaplasia].
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Biomedical subjects
Publications and source records attributed to C Brocheriou.
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Recurrence of an aneurysmal cyst and cementifying fibroma in a 13 year old adolescent required treatment by mandibular resection. Published data on combined aneurysmal cysts and other lesions are reviewed and a new entity, the desmoosteoblastoma of the psammomatous type, discussed.
Concerning three specimens of Bourneville tuberous sclerosis, after a brief recall of general statements about this disease, the authors describe in two out of the three cases published, lesions of buccal mucosis typical of this disease. Besicle hyperplasic lesions, quite common with these patients who are epileptic most of the time, thise lesions mostly consist of fibrous, non inflamatory nodule more frequents on gums, especially one the fore-part. The histologic aspects without being pathognomonic are still quite specific.
A 17-year-old young girl presented with a rare maxillary tumor, an osteocementoblastoma, which from the ectopic type of position of the wisdom tooth had apparently been present for many years pathologic findings are reported. Post-operative course was normal but regular follow-up is necessary because of the tendency for recurrence of the few cases reported in the literature of this benign tumor.
The authors report a case of a 53 year old woman with Riedel's thyroiditis and retroperitoneal fibrosis. The thyroiditis has been diagnosed a year before the retroperitoneal fibrosis. This one was idiopathic and has been responsible of the death of the patient during an operation performed for ureterolysis. Fifteen other similar cases have been reported in the literature: four of them associated also mediastinal and biliary ducts fibrosis. The existence in one patient of multiple fibrosclerosis locations and the similitude of histological patterns (inflammatory diffuse fibrosis) lead to the concept of a multiple sclerosing disorder involving Riedel's thyroiditis, Ormond's disease, mediastinal fibrosis, sclerosing cholangitis, and the pseudotumors of the orbit. The etiopathogenic processes remain unclear.
Cholesterol ester storage disease, a mild form of Wolman's disease, is caused by a cholesterol ester lysosomial acid hydrolase deficiency leading to an accumulation of lipids in numerous cells of the organism. The authors have studied the liver parenchyma of two patients. The lipid deposits are found in the hepatocytes, macrophages and Küpffer cells, and in the walls of the biliary canaliculi. Histological study shows them to be rich in triglycerides and cholesterol esters. Ultrastructural study confirms their intralysosomal nature. Finally, examination of a tendinous xanthoma in one of the patients revealed deposits of the same nature in the macrophages.
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.
Three cases of bowel angiodysplasia with digestive bleeding were reported. In all cases, diagnosis was confirmed by arteriographic and/or histological data. Angiodysplasia of the colon was the most often an acquired and degenerative disease, and most commonly affecting patients after the seventh decade of life. This condition was a frequent and misinterpreted cause of bleeding per rectum in patients after fifty years. Selective superior mesenteric angiography, after colonoscopy (except during major bleeding) was the best investigation, showing pathognomonic images and specifing the site, often located in the caecum and/or ascending colon. Treatment was surgical, but endoscopic methods provided encouraging results.
We have diagnosed a Harlequin fetus at 22 weeks' gestation. Harlequin syndrome is a fatal, congenital disorder of keratinization whose biochemical basis is unknown. The parents were second cousins and had had four children, two with the Harlequin syndrome who had died at birth and two normal children. During the fifth pregnancy the parents asked us to find out if the fetus was affected. Multiple skin biopsies were done by fetoscopy during the 22nd week of gestation. On binocular loupe examination the skin appeared thickened and fragile to the touch; light and electron microscopy revealed a remarkably thickened stratum corneum with involvement of both follicular and interfollicular epidermis, a feature characteristic of a Harlequin fetus at term. The pregnancy was terminated in the 24th week of gestation: the fetus had all the characteristic clinical features of Harlequin fetus.
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The case of a 42 years old man who had a monoblastic acute leukemia treated by a bone-marrow graft is described. The patient died of a cerebral toxoplasmosis and a cerebral computed tomography done two days before is correlated with anatomopathologic slices done in the same planes than the computed tomagraphy examination. There is a good correlation between necrotic abceded areas and hypodense areas accompanied by serpiginous fixations of contrast media. The immunocompromised patient is very favorable for the development of a cerebral toxoplasmosis. CT, while not specific, has the advantage to suspect the diagnosis which must be discussed with other oportunistic infections, leukemic infiltrates, methotrexate encephalopathy, progressive multifocal encephalopathy, and small infarcts.
Intramandibular salivary inclusions and ectopia concern the main salivary glands. The most common takes the form of a regular well-defined lacunar appearance situated at the mandibular angle or molar region. This asymptomatic inclusions is in fact only the imprint of a lobule of the sub-mandibular gland on the mandible. More rarely, the parotid gland and sublingual gland may also be responsible for a lacunar radiological appearance in the mandible. By contrast, a few ectopic glandular lobules of the sublingual may be completely induced at the site of the mandibular symphsis. Salivary inclusions and ectopia thus result from two different aerio-pathogenic mechanisms.
The authors report 4 cases of parotid cyst seen in the department of maxillo-facial surgery (Pr Crépy): -one cystic lymphangioma, -one dermoid cyst, -two lympho-epithelial cysts. They stress the hazards of needle puncture and the essential value of exploratory parotidectomy.
Pseudo-sarcomatous spindle cell carcinomas are a variety of squamous carcinoma which must be recognized since they may raise the difficult problem of histological identification. They may be confused with a sarcomatous tumour, in particular fibroblastic. Buccal involvement, somewhat rarer than other sites in the upper respiratory and digestive tract, is nevertheless not exceptional. Clinically the lesion may be either a relative superficial polypoid mass, where the prognosis is relatively favourable, or a highly infiltrating lesion, which applied in the five cases reported here and which then has a rapid course and bad prognosis.
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