Induction in rats of paranasal sinus carcinomas with radioactive cerium chloride.

A colloidal suspension of radioactive cerium stabilized in the form of hydroxide was inoculated into young Sprague-Dawley rats near the maxillary sinus. Well-differentiated epidermoid carcinomas of the paranasal sinus were obtained in 8 of 12 rats and in 8 of 9 animals that liver more then 200 days. Therefore, this istope was highly effectivein inducing tumors at the site of injection. The uniformity of the histologic type of tumors induced under these experimental conditions was remarkable.

Successful surgical removal of occult metastases of medullary thyroid carcinoma recurrences with the help of immunoscintigraphy and radioimmunoguided surgery.

Patients with recurrent or metastatic medullary thyroid carcinoma (MTC) were referred for pretargeted immunoscintigraphy (Affinity Enhancement System; AES) and radioimmunoguided surgery (RIGS). Data collected from 13 patients establish that whole-body AES immunoscintigraphy revealed metastases < 360 mg and RIGS detected micrometastases (5-15 mg). All tissue samples removed by the surgeon were diagnosed by histology and immunohistochemistry of calcitonin to check the accuracy of IS and RIGS results. AES immunoscintigraphy is very sensitive. Of 34 metastases or recurrences detected, 22 had escaped physical examination or conventional imaging. The accuracy of RIGS was 86%, its sensitivity 75%, and its specificity was 90% (n = 208). IS and RIGS detected occult tumors that would have escaped surgery, clearly demonstrating clinical benefit. Serum calcitonin (normal, 10 pg/ml) and carcinoembryonic antigen (normal, 5 ng/ml) of two patients were restored to normal. In patients whose tumors were discovered, progression of their disease was slowed, as evidenced by the large decrease in serum calcitonin and carcinoembryonic antigen, an important prognostic factor. Surgery was canceled in one case where IS detected distant metastases out of surgical reach. Thus, AES immunoscintigraphy and RIGS might be of valuable help for the surgical management of medullary thyroid carcinoma.

Sicca complex and infection with human immunodeficiency virus.

Five male patients with the persistent generalized lymphadenopathy syndrome also had a sicca complex. Salivary gland biopsy specimens showed diffuse lymphocytic infiltration of the glandular parenchyma. Serum autoantibodies and rheumatoid factor were not detected. All patients had IgG antibodies to human immunodeficiency virus and IgG to the viral capsid antigen of Epstein-Barr virus. These five patients had benign lymphocytic infiltrates in other organs (lung, liver, and kidneys). Sicca complex may be one of the various manifestations of the lymphoid hyperplasia noted in human immunodeficiency virus-infected patients. In these patients, the sicca complex showed specific features related to male predominance, lack of serum autoantibodies, and peripheral-blood T-lymphocyte subset distribution.

Systemic evaluation of Sjögren-like syndrome after bone marrow transplantation in man.

A systematic evaluation of Sjögren-like syndrome (SLS) was performed in 68 bone marrow transplant (BMT) recipients (60 allogeneic and 8 syngeneic recipients). At day 100, the patients underwent clinical evaluation, functional salivary scintigraphy, and lip biopsy. If any findings were abnormal, the examinations were repeated annually for 3 years. Twenty-two patients with SLS and extensive chronic graft-versus-host disease (CGVHD) had abnormal scintiscan and lip biopsy at day 100. Marked keratoconjunctivitis sicca and xerostomia developed between 12 and 24 months after BMT and, thereafter, progressively decreased. Twenty-seven irradiated recipients (7 syngeneic and 20 allogeneic recipients without CGVHD) had isolated xerostomia and disturbed scintiscan but normal biopsy. Seven other patients with limited CGVHD had a lymphocytic infiltrate on lip biopsy but no SLS and a normal scintiscan. Schirmer's test, functional salivary scintigraphy, and lip biopsy allowed us to distinguish SLS from radiotherapy sequelae. As early as day 100, these 3 tests have a predictive value for SLS, one of the criteria for extensive CGVHD.

Fulminant adenovirus hepatitis after allogeneic bone marrow transplantation.

Adenoviruses may cause severe infections in bone marrow transplant recipients. We report the case of a patient who developed fulminant hepatitis 5 months after bone marrow transplantation. Adenovirus type 2 was cultured from stool and blood samples. The patient died from liver failure. Histologic examination of post-mortem liver samples showed extensive necrosis with nuclear inclusions. Adenovirus was identified in liver cells by electron microscopy and immunohistochemical staining using a monoclonal anti-adenovirus antibody. No other pathogen was identified.

Scintigraphy of the salivary glands in Sjögen's syndrome.

Scintigraphy of the salivary glands with technetium-sodium pertechnetate (99mTc) was undertaken on 320 patients with oral dryness or connective tissue disease using a computer assisted method that gave quantitative results about the major salivary gland function. Compared with clinical and histological data, scintigraphy provides a sensitive method, even though it is not specific, for detecting minimal injuries to salivary glands in patients suspected of having Sjögen's syndrome. Moreover, it might differentiate between the Sjögen-like syndrome and the sequelae of radiotherapy in patients with bone marrow graft. Scintigraphy of the major salivary glands could therefore form part of the routine investigation of patients with Sjögen's syndrome.

Variability of immunohistochemical reactivity on stored paraffin slides.

AIMS: To investigate the effects of slide storage on immunohistochemical staining, since recent reports have indicated that storage of unstained paraffin slides for up to 12 weeks may lead to false negative immunostaining of tumour markers. METHODS: 11 antibodies (anti-cytokeratin, epithelial membrane antigen (EMA), vimentin, smooth muscle actin, PS100, chromogranin, CD45, CD20, CD3, CD30, and oestrogen receptor (OR) were tested on unstained paraffin slides of breast carcinomas, lymphomas, and neuroendocrine tumours that had been stored for three to 10 years. All the paraffin blocks were recut less than one week before immunostaining. Immunostainings of years old slides were compared with those of recent slides in at least five cases for each antibody. For three antibodies (antichromogranin, anti-CD3, and anti-OR) we also tested one year old and three months old slides. RESULTS: Intensity of staining on years old slides was strikingly reduced for chromogranin and CD3 in several cases and was slightly stronger for vimentin. In some cases a significant decrease of OR positivity was observed after three months storage, and a complete loss of OR immunostaining after 12 months. No significant difference was noted with the other antibodies. CONCLUSIONS: Immunohistochemical detection of some antigens located either in the nucleus, in the cytoplasm, or on the cytoplasmic membrane could be impaired by storage of paraffin slides as short a time as three months. One should be cautious of doing retrospective immunohistochemical studies on stored unstained slides.

MR of cerebral aspergillosis in patients who have had bone marrow transplantation.

PURPOSE: To assess the CT and MR appearance of cerebral aspergillosis in patients who have undergone bone marrow transplantation. METHODS: The imaging and clinical data of five patients with cerebral aspergillosis were reviewed retrospectively and compared with autopsy findings. RESULTS: Lesions are often located in the basal ganglia and demonstrate an intermediate signal intensity within surrounding high-signal areas on long-repetition-time MR scans. The lesions were multiple in four of the five patients and more numerous on MR images than on CT scans. The lesions (which demonstrate no parenchymal enhancement) are consistent with acute infarcts as confirmed at autopsy. In the large lesions, there is early intravascular and meningeal enhancement, as expected in acute infarcts involving an appreciable portion of the territory of a cerebral artery. CONCLUSION: The diagnosis of early cerebral infarction in a patient considered at risk for invasive aspergillosis, even without overt pulmonary disease, is an indication to institute aggressive antifungal therapy.

Cyclosporine-associated nephropathy in patients with heart and bone marrow transplants.

The morphology of kidneys from heart (n = 55) and bone marrow (n = 112) transplant recipients treated either with cyclosporine (CSA) or conventional immunosuppression was investigated at autopsy. The major findings were: In the bone marrow transplant recipients glomerular collapse, tubular atrophy, interstitial fibrosis, striped form, CSA-associated arteriolopathy and thrombi in glomeruli and/or arterioles were more often found in the CSA group as compared to conventional immunosuppression. In the heart transplant recipients glomerular collapse and obsolescence, tubular atrophy and intimal fibrosis in arteries were more frequent in the CSA group. Vascular interstitial toxicity known to be associated with CSA treatment from renal transplant patients was found in 54% (25% severe) of the bone marrow and 19.5% (9.7% severe) of the the heart transplant recipients. The prevalence of vascular interstitial toxicity in bone marrow versus heart transplant recipients is possibly due to higher CSA dosage and pretreatment with cytostatic drugs and irradiation. Analyses of the lesions from early stages to the full picture of vascular interstitial toxicity suggests that CSA causes a form of thrombotic microangiopathy with focal glomerular and/or arteriolar thrombosis followed by typical CSA-associated arteriolopathy which results in interstitial fibrosis with tubular atrophy.

WHO International Histological Classification of Tumours. Tentative Histological Classification of Salivary Gland Tumours.

The principles of the proposed modified WHO Histological Typing of Salivary Gland Tumours are based on the following: 1) The classification of tumours is oriented to the routine work of the practicing surgical pathologists, those who do not see tumours of the salivary glands very often. The inclusion of rare, but clearly defined tumour entities should be helpful to surgical pathologists consulting with clinical specialists. 2) The different types of carcinomas must be distinguished not only by precise histopathological definitions, but also considering differences in prognosis and treatment. For example, the polymorphous low-grade adenocarcinoma and the epithelial-myoepithelial carcinoma are characterized by a relatively good prognosis in contrast to the salivary duct carcinoma. 3) Special points of discussion are: subclassification and grading of carcinomas (e.g. acinic cell carcinoma, mucoepidermoid carcinoma and adenoid cystic carcinoma), the classification of basal cell tumours (basal cell adenoma, basal cell carcinoma, solid type of adenoid cystic carcinoma), malignant tumours in pleomorphic adenomas and the differential diagnosis between primary tumours and metastases.

Multiple endocrine neoplasia (MEN) type II b: report of a case observed at autopsy with immunohistochemical study of mucosal neuromas.

A case of multiple endocrine neoplasia (MEN) II b syndrome was studied in a 28-year-old Colombian woman. The patient presented initially with medullary carcinoma of the thyroid (MTC), an unusual habitus, numerous mucosal neuromas, and intestinal ganglioneuromatosis. Recurrent medullary carcinoma in the mediastinum produced compression. The tumor mass could not be removed surgically, and the patient died of post-operative complications. At autopsy metastatic MTC was present in the liver, lymph nodes, and lungs. In addition, multiple mucosal neuromas were present in the mouth, nasopharynx, larynx, digestive tract, peri-adrenal fat, and hepatic portal spaces. There was no evidence of pheochromocytoma. Immunofluorescence study of mucosal neuromas showed hyperplasia and hypertrophy of nerves, without evidence of tumor. Kindred screening was negative.

[Intraoral minor salivary gland tumors. Report of 296 cases (author's transl)]. / Tumerus des glandes salivaires accessoires de la cavité buccale. Etude de 296 cas.

296 intraoral minor salivary gland tumors were observed among 702 salivary tumors. The study emphasizes a frequent palatine location, observed in more than 50 per cent of theses tumors. Pleiomorphic adenomas (or so called mixed tumors), are frequently encountered; but it is noteworthy that the proportion of muco-epidermoid tumors or adenoid cystic carcinoma is higher among theses tumors than among major salivary glands; this fact explain a poor overall prognosis of minor salivary gland tumors.

[Recurrent ventricular tachycardia and parchment right ventricle in the adult. Anatomical and clinical report of 2 cases]. / Tachycardies ventriculaires récidivantes et ventricule droit papyracé de l'adulte. A propos de 2 observations anatomo-cliniques.

Two patients, aged 52 and 55 years, were admitted to hospital for attacks of recurrent ventricular tachycardia for which preventive treatment was difficult. The crises were of one type, with left-sided delay. The findings on clinical examination, electrocardiography, haemodynamic and arteriographic investigation and at post-mortem were those of the parchment right ventricle syndrome of the adult. Electrophysiological investigation in the 2 cases indicated that a re-entry mechanism underlay the attacks of tachycardia. Late activation of the pulmonary infundibulum was responsible for a post-excitation potential on the surface and intra-cavitary leads. Mapping carried out during ventricular tachycardia in one case demonstrated the pathway for re-entry at the level of the pulmonary infundibulum.

[Consequences of radiation on tissues]. / Conséquences des irradiations sur les tissus.

Tissular and cellular radiation-induced injuries. The authors examine morphological, tissular and cellular radiation-induced injuries utilizing anatomo-clinical case reports and experiments in the pig. One must distinguish early and delayed tissular lesions. In the latter, appearing in general after at least six months, vasculo-connective changes are the most important, leading to sclerosis. Indeed, because of the vascular damages the histo-haematic barrier is enlarged leading to a decrease of tissular resistance to injury, long and difficult tissular repairs after a surgical operation. At the end the authors mention some special morphological features concerning the site of lesions.

[Pigmented lesions of the oral cavity]. / Lésions pigmentées de la cavité buccale.

Pigmented lesions of the oral cavity are of multiple origin. They can be subdivided as follows: non tumoral pigmentations, non melanin pigmented tumors or tumor-like lesions, benign melanin pigmented tumors and malignant melanomas. Among non tumoral pigmented lesions, some of them show melanin deposits: they can be associated with a systemic disease (Peutz Jeghers syndrome, Addison's disease) or have a medicamentous origin, or belong to a lichen migricans. Non tumoral and non melanin pigmentations are principally due to a heavy metal accumulation or an accidental tatoo arising after tooth treatment. Peripheral giant cell granuloma, so-called giant cell epulis is the major non pigmented non melanin pseudotumoral lesion; pigmentation is due to hemosiderin deposits. In the oral cavity nevi are principally of the intramucosal type. Blue nevus, the second type in frequency, is usually located on the hard palate. Primary malignant melanomas are rare in the oral cavity, but it is--because its very bad prognosis--the most important lesion. In order to improve the survival it is necessary to do the diagnosis as early as possible.

[Cutaneous-mucous hyalinosis (Urbach-Wiethe disease). Histologic and ultrastructural study of a case]. / Hyalinose cutaneo-muqueuse (maladie d'Urbach-Wiethe). Etude histologique et ultra-structurale d'une observation.

Hyalinosis cutis et mucosae is a rare genetic disorder, primarily involving skin and mucous membranes, that has been described under various titles as lipoidosis lipoproteinosis and Urbach-Wiethe disease. A 17-year-old girl presented cutaneous macular lesions, skin scars, thickened lips and enlarged firm tongue; hoarseness was known since birth. The labial biopsy specimen was investigated by light and electron microscopy. Histologically there are deposits of amorphous, extra-cellular eosinophilic hyaline material in the upper thickened connective tissue. In the lower part changes are focal with hyaline mantle around the vessels. Histochemical studies have revealed that the deposits were PAS-positive and amylase-resistant, indicating the presence of neutral mucopolysaccharides. Lipid stains were positive, especially in vessel walls. Ultrastructurally hyaline substance is composed of 1 to 2 nm protofilaments, larger ones (5 to 10 nm wide) and amorphous material. Fibroblasts contain in their dilated cisternae amorphous material. A frequent feature is marked multilamination of the capillary basal lamina which is interpenetrated by hyaline substance. Pathogenesis is still unknown but recently it is suggested hyalinosis cutis et mucosae could be a systemic disease of basal lamina.

[Leiomyosarcoma of the larynx. Histological, immunohistochemical and ultrastructural study of a case with review of the literature]. / Léiomyosarcome du larynx. Etude histologique, immunohistochimique et ultrastructurale d'une observation avec revue de la littérature.

Laryngeal leiomyosarcoma: histological, immunohistochemical and ultrastructural study of one case with review of the literature. Leiomyosarcoma of the larynx have rarely been reported and a review of the literature has yielded only 8 cases. Authors report a case in a 45-year-old patient. Histological exam of the surgical specimen showed a malignant spindle cell tumor. Ultrastructurally, neoplastic cells presented some features of smooth-muscle cells. Immunohistochemical studies revealed that most tumor cells coexpressed vimentin and smooth-muscle actin.

[Craniofacial granulomatous lesions]. / Processus granulomateux cranio-faciaux.

Granulomatous lesions of the cranio-facial area are frequent and various in their nature: lymphohistiocytic with or without eosinophils, tuberculoid-like with epithelioid and giant cells, or sometimes made essentially of giant cells. Their etiology can be known or easy to find: foreign body granuloma, sarcoidosis, leprosy, rhinoscleroma, fungal diseases especially zygomycosis and rhinosporidiosis, parasitic diseases. The lethal midline granuloma is a clinical entity characterized by its necrotic and relentlessly progressive destructive presentation. After elimination of a malignant process, especially lymphoid, and of a Wegener's granulomatosis the diagnosis will be "idiopathic midline non-healing granuloma". Some of them will stay located at the facial area; others will disseminate as a malignant disease. Central giant cell granuloma and histiocytosis X, especially eosinophilic granuloma, are two other varieties of granuloma, different of the former granulomatous infiltrates by their clinical presentation and their evolution.

[Carcinosarcoma of the submaxillary gland]. / Carcinosarcome de la glande sous-maxillaire.

Salivary gland carcinosarcoma is a rare neoplasm, with a poor prognosis, that should not be confused with the more common carcinoma ex-pleomorphic adenoma, in which the epithelial component alone is malignant. The authors report one case of carcinosarcoma of the submandibular gland, with no previous history of pre-existent tumor. The tumor exhibited two intermingled and very atypical cellular components, one undifferentiated and the other with a chondroid pattern. Immunostaining with epithelial markers in the undifferentiated area allowed to distinguish this tumor from salivary gland sarcomas. In addition, the positivity for EMA, vimentin, and S100 protein in the two predominant components suggested a common origin for the different tumoral cell types, and led to discuss the nomenclature and the still unclarified cellular origin of these tumors.

[Prenatal diagnosis of a harlequin fetus using electron microscopy]. / Diagnostic prénatal par microscopie électronique d'un foetus Arlequin.

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.