Thyroglobulin in medullary thyroid carcinoma: immunohistochemical study with polyclonal and monoclonal antibodies.

The presence of thyroglobulin (TG) was investigated by immunohistochemistry with one polyclonal and three distinct monoclonal anti-TG antibodies in 19 primary and 15 metastatic medullary thyroid carcinoma (MTC) cases. In two primary tumors possessing a mixed trabecular and follicular growth pattern, a significant number of tumor cells were positive with the four antibodies in all parts of the tumor; these tumors were considered to be genuine mixed follicular and parafollicular tumors. The 17 other primary tumors looked like "classical" (ie, predominantly compact) MTC. Eight contained tubular or microfollicle-like structures that were consistently TG-negative. Eight contained residual entrapped normal follicles positive with the four anti-TG antibodies. In six cases isolated foci of tumor cells reacted with polyclonal antibody or monoclonal antibody 11. In 10 cases circulating TG stained positively with polyclonal antibody in vascular channels, five cases stained positively for TG with monoclonal antibody 11, four cases stained positively for TG with monoclonal antibody 7, and three cases stained positively for TG with monoclonal antibody 1. In metastases TG was found in tumor cells in only one instance, but positive reactions were obtained in vascular channels and macrophages in several cases. We conclude from this study that there is no genuine secretion of TG in classical, compact MTC, which differs morphologically and functionally from mixed follicular and parafollicular tumors secreting both TG and thyrocalcitonin.

Parathyroid carcinoma, adenoma and hyperplasia in a case of chronic renal insufficiency on dialysis.

A 62 year-old female patient with polycystic disease and chronic renal insufficiency who had required dialysis for 3 years, presented with diffuse but predominantly lumbar bone pain associated with lassitude. Radiologic studies showed diffuse osteoporosis and destruction of the 4th lumbar vertebra. Calcium levels were normal (2.30-2.50 mmoles) with increased PTH levels (1820 microliter Eq/ml). The presence of a cervical mass suggested a diagnosis of hyperparathyroidism. A cervical exploration was performed with ablation of 4 parathyroid glands and autotransplantation of a portion of the left inferior gland into the arm. Pathological studies showed the existence of carcinoma in the right superior and inferior glands, adenoma of the left superior gland and hyperplasia of the left inferior gland. The association of hyperplasia, adenoma and carcinoma could be explained by an initial hyperplasia of the 4 glands due to renal insufficiency, with subsequent transformation of 3 into adenomas of which 2 became cancerous. The patient's status was significantly improved following surgery.

PCR analysis of hepatitis B virus DNA in paraffin-embedded liver tissue from patients with chronic liver disease.

We described a nested polymerase chain reaction protocol to detect hepatitis B viral DNA in paraffin-embedded liver tissue and tried to determine whether this virus was associated with non-B chronic liver disease. Fifty-five samples were obtained from 28 patients with B, and 27 patients with non-B chronic liver disease (35 cirrhosis, 4 hepatocellular carcinoma and 16 chronic hepatitis). The two sets of primers amplify a sequence located in a conserved polymerase/surface region of the viral genome. Reaction products were analysed using a nonisotopic hybridization method. None of the 27 (0%) seronegative samples and 20 of the 28 (71%) seropositive specimens were positive for hepatitis B virus DNA. There were 4 false negatives in which beta-globin PCR was positive. Although its sensitivity is reduced in formalin-fixed paraffin-embedded tissue, nested PCR allows rapid detection of HBV DNA sequences and can be a useful tool if no frozen tissue is available.

Clear cell sarcoma of the kidney in children: a distinct entity.

Four cases of clear cell sarcoma of the kidney in children are reported. The specificity of the tumor is examined. The pathologic features, the clinical presentation, and the occurrence of bone metastasis are reasons to distinguish clear cell sarcoma from the Wilms' tumor group. The poor prognosis may be improved in the future by radical resection associated with polyvalent chemotherapy.

[Celiac disease, autoimmune cirrhosis, chronic pancreatitis: apropos of a triple association]. / Maladie coeliaque, cirrhose auto-immune, pancréatite chronique: à propos d'une triple association.

We report the case of a 60 year old lady suffering from celiac disease and autoimmune cirrhosis who developed exocrine pancreatic insufficiency with canalicular lesions consistent with chronic pancreatitis. Celiac disease is known to be associated with either pancreatic insufficiency or liver disease, but association of all three diseases has not yet been described. We suggest that chronic pancreatitis be added to the list of idiopathic inflammatory pancreatitis of possible autoimmune origin, enabling to explain the pathophysiology of all three disorders with one hypothesis.

[Pseudomyxoma peritonei: a case with multiple metastases. Ultrastructural study and chemical analysis of the mucoid substance]. / Pseudomyxome péritonéal: un cas avec métastases multiples. Etude ultrastructurale et analyse chimique de la substance mucoïde.

A patient with spreading pseudomyxoma peritonei probably caused by a tumor of the appendix showing suggestive clinical features is presented. The long evolution was marked by the discovery of gelatinous substance in the stools and in the lumen of the gut, and the appearance of distant metastasis in the lungs, adrenal glands and lymph nodes. Histochemical studies showed that periodic Shiff acid and Alcian blue were fixed by the mucoid substance. The use of toluidin blue (pH 1) revealed metachromosia. Electron microscopy ascertained the presence of enterocytes and mucous cells and revealed a more obvious nuclear disorganisation than by histological microscopy. Biochemical study showed that the mucoid substance extracted from the pseudomyxoma was rich in carbohydrates and could be identified as a mucin on the basis of the composition of its monosaccarides and of the alkali-lability of its glycanprotein linkages. The mucin was weakly hydrolysed by trypsin in vitro but was deeply attacked by the same enzyme after desialylation. These results: a) confirm the possibility of observing spreading distant metastasis in the pseudomyxoma peritonei; b) underline the potential interest of electron microscopic study in case of wavering diagnosis; c) show that the mucoid substance belongs to the group of neutral sulfomucins; d) legitimate, in the future, a trial of palliative treatment by neuraminidase in such cases.

[Uterine metastasis revealing gastric adenocarcinoma]. / Métastase utérine révélatrice d'un adénocarcinome gastrique.

We report a case of metastasis to the uterine corpus revealing a primary gastric adenocarcinoma. A 26-year-old woman suffered from weight loss, vaginal bleeding, abdominal pain. An endometrial curettage showed apparently metastatic adenocarcinoma. The primary site of the tumour was gastric. The upper gastrointestinal endoscopy revealed an ulcus and aspect of linitis plastica in the fundus. Biopsies showed diffuse type adenocarcinoma. Because of extensive disease, laparotomy was not performed and exclusive palliative chemotherapy was started. The patient died 10 months after the diagnosis. Metastasis from primary gastric cancer to the female genital tract are rare and are usually observed in young premenopausal women with diffuse type gastric adenocarcinoma. This case report underlines the interest, for those patients of careful gynaecologic examination at the initial staging and after treatment.

[Necrotizing hepatic granuloma of brucellosis origin. Apropos of a case]. / Granulome nécrosant hépatique d'origine brucellienne. A propos d'un cas.

The authors report on a case of febrile necrotizing brucellosis hepatic granulomatosis in a patient living in brucellosis endemia areas. This hepatic localization of brucellosis is rare: to our knowledge only 28 cases have been described in the literature. The bacteriological diagnosis was not established by blood or abscess pus cultures, but by Brucella serology only. Surgery completed by biantibiotherapy have permitted a complete recovery.

[Morphologic study of different types of venous grafts. Correlation trial with parietal fibrinolytic activity]. / Etude morphologique de différents types de greffons veineux. Essai de corrélation avec l'activité fibrinolytique pariétale.

In this experimental work, morphological aspects of different vein grafts were studied using both light microscopy, transmission electron microscopy and scanning electron microscopy. The correlation was made with their fibrinolytic activity ; 21 dogs were operated on under general anaesthesia ; "venous grafts" (perfusion of the vascular segments with venous blood) were performed by excision of the femoral vein with reanastomosis at the same site (protocol P1). "Arterialised grafts" (perfusion of a segment of vein with arterial blood at arterial pressure) were performed according to two protocols : P2 in which a segment of femoral artery was replaced with a segment of reversed femoral vein ; P3 in which a segment of femoral artery was by passed with the femoral vein left in situ after destroying the valves using a Fogarty balloon without damage of the intima. The factors affected by surgery are, therefore, disconnection of the vasa vasorum by excision of the vessel in protocols P1 and P2 and increased oxygenation and luminal pressure in protocols P2 and P3. In all the cases, endothelial cells retained the morphological characteristics of venous endothelium. However, the vein grafts in the arterial circulation were thickened and the fibrinolytic activity was changed. Pathogenic mechanisms of these changes are discussed.

[A new case of Urbach-Wiethe disease. Electron-microscopy study]. / Un nouveau cas de maladie d'Urbach Wiethe. Etude en microscopie électronique.

A new case of Urbach Wiethe disease is reported. The diagnosis of this disorder, despite the existence of typical histological connective tissue deposits, was recognized late. The authors review the morphological characteristics of the disease, emphasizing that atypical clinical presentations may occur which underline the importance of the histological examination.

[Melanic proliferations of the conjunctiva. Histopathologic study of a series of 40 cases]. / Les proliférations mélaniques de la conjonctive. Etude histopathologique à propos d'une série de 40 cas.

A retrospective study of 40 melanocytic conjunctival tumors (22 naevi and 18 malignant melanoma) is reported. Cases of non-degenerated conjunctival melanosis were not observed. The particular aspects of diagnosis and the prognostic behaviour of these proliferations are discussed and compared with their cutaneous homologues. Epithelial inclusions within tumor cell proliferation are considered as the best criterion of benignity. Because conjunctival melanomas are rather uncommon and technical difficulties in their study are very frequent (poor quality of tissue samples, small size of specimens) many reports are contradictory. Some diagnostic signs are specific for this kind of tumor in the conjunctiva: nevertheless, the particular conjunctival anatomy makes it impossible to classify according to criteria defined for cutaneous malignant melanoma. No criteria would have a predictive value. The severe prognosis of conjunctival melanoma (44% deaths in the present series over 10 years after diagnosis) has been underestimated. Most often carcinologic therapies have not been used in previously reported cases.

[Survival after gastrectomy for cancer. 209 cases]. / Survie après gastrectomie pour cancer. 209 observations.

The long-term results of 209 gastrectomies performed for adenocarcinoma, including 117 which were prospectively collected, are presented. Resection was curative in 154 cases (73.6 percent). The TNM distribution of the tumours was: stage I (TxNOMO) 75 cases, stage II (TxN1MO) 46 cases, stage III (TxN2MO) 33 cases and stage IV (TxNxM1) 55 cases. Lymph node involvement was more frequent in the prospective than in the retrospective study. With a more than 5 years' follow-up of 80 percent of the patients operated upon, the actuarial survival rate at 5 years (operative mortality included) was 38 percent for all lesions, 52 percent for curative resection and 2 percent for palliative resection. Following curative resection, the survival rates for tumours of the upper, middle and lower thirds of the stomach were 40, 60 and 55 percent respectively. These rates were 60 percent for stage I tumours, 54 percent for stage II tumours and 25 percent for stage III tumours. The results obtained in this series, where most of the curative gastrectomies included excision of N1 and N2 lymph nodes, show that lymph node involvement has no significant importance for the prognosis when it is proximal (N1) and is not incompatible with prolonged survival when it is pedicular (N2).

[Wegener's granulomatosis. Contribution of computed tomography and nuclear magnetic imaging]. / Granulomatose de Wegener. Apport de l'imagerie par tomodensitométrie et résonance magnétique.

Amongst the endothoracic forms of Wegener's granulomatosis, tracheal, bronchial and above all mediastinal localisations are unusual. Such a case having a bad outcome is reported here. Computed Tomography (TDM) was used to assess the extend of pulmonary parenchymal involvement. Magnetic Resonance Imaging (MRI) was superior to TDM for showing transmural infiltration of the tracheal and the origin of main bronchi.

[Blue rubber bleb naevus and glomangiomatosis, unicity or duality? About two cases (author's transl)]. / Blue rubber bleb naevus et glomangiomatose unicité ou dualité? A propos de 2 cas.

About 2 personal cases, a review concerning Blue Rubber Bleb Naevus syndrome and glomangiomatosis is presented. Attention is drawn to the different histological pictures of these disease. Importance of a clear differentiation from the viewpoint of prognosis is emphasized.

[Crosti's reticulosis. Report on three cases, one with ultrastructural study (author's transl)]. / La réticulose de Crosti. A propos de 3 cas dont un avec étude ultrastructurale.

Crosti's reticulosis is defined as a peculiar form of well-circumscribed, slow developing lymphomas in adults, with a malignant histological picture. Three cases with typical dorsal localisation according to Crosti's first description are reported and the cardinal features of this type of tumour are recalled. One observation includes an ultrastructural study which confirms the malignant nature of histiocytes in this disease. Localisation in back seems not to be exclusive and a study of 32 cases of the literature about other localised cutaneous reticulum cell lymphomas show them similar to Crosti's reticulosis-which authorizes their classification, as was proposed by Thiers in a single group of slow developing circumscribed reticuloses.

[Retraction pockets, pathological entity?]. / Les poches de rétraction, entité pathologique?

Retraction pockets are not a pathological entity per se but take after various ear diseases, with which they share the same morphological eardrum alterations. The authors believe that any holistic evaluation of retraction pockets, as though these were forming a single group of like pathogenic origin, i.e., tubal dysfunction, would be artificial and raise therapeutic problems. The statistical analysis of the causes for retraction pocket formation provides little information. Otologists are still looking into chronic otitis media and cholesteatoma as a possible, long-suspected, unproved etiology. Electron microscopy and, more particularly, istological-enzymological analyses of mounts prepared by the authors have shown, in some cases, the anomalous presence, in the pocket, of Langerhans' cells, which the authors consider as strongly indicative of cholesteatoma. While confirming the clinical diagnosis, anatomopathological examination allows to differentiate poor-prognosis retraction pockets from benign ones. The pathogenesis of these pockets is still poorly understood. It is the authors' contention that tympanic invagination is more likely traceable to some biopathological/biochemical phenomenon than to occupational mechanical disorders involving the tube. The clinical characteristics of retraction pockets are the basis for their classification into three groups, according to their evolutional tendency. Thus, developing cholesteatomas bear a poor prognosis; sequelae of benign otitis are associated with small risk; lastly, there is a small group of pockets the evolution of which is hard to specify. The authors believe that such differentiation between retraction pockets is mandatory to have a clear picture of therapeutic indications, and to assess the various outcomes.

[Neurinomas of the cochlear nerve]. / Neurinomes du nerf cochléaire.

Usually, schwannomas of the eight nerve arise from the vestibular nerve. Yet, the authors have operated from September 1993 to September 1995 three neuromas whose origin is certainly the cochlear nerve. Surgery was performed by retro-sigmoid approach magnified by endoscopic procedure of the cerebello-pontine angle before and after removing the tumor. We report here the cases and the literature is documented. Similar cases of observing cochlear neuromas at the moment of the procedure are exceptional. Because of more and more early diagnosis of these tumors, advances of microsurgery in ponto-cerebellar angle and endoscopic improvements in this area, observation of this kind of pathology could be more and more frequent.

[Partial nodular transformation of the liver with portal hypertension. A new case. Review of the literature]. / Transformation nodulaire partielle du foie avec hypertension portale. Une nouvelle observation. Revue de la littérature.

Based on a case of partial nodular transformation of the liver (PNT) with portal hypertension and splenic microaneurysms in a 24 year old man, the authors review the literature on this subject which only consists of 7 female cases. The authors point out the rarity of this entity which, together with hyperplastic nodular regeneration, is one of the causes of the essential portal hypertension syndrome. They stress the early onset of this disease (mean age of 35 years), on the severity of its clinical course and on the surgical possibilities of porto-caval shunts. The pathogenesis of PNT remains unknown.