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.

[Hemobilia caused by biliary cyst]. / Hémobilie par kyste biliaire.

Recurrent angiocholitis attacks occurred in an 80-year-old woman, and repeated investigations could not provide any diagnosis. Subsequently, the patient developed haematemesis. Angiography revealed haemobilia from an intrahepatic cause. Surgical constatations and pathological examination of the resected specimen revealed that haemobilia was due to a non-parasitic cyst of the liver. In this case, haemobilia resulted from two unusual complications of this hepatic lesion: intra-cystic bleeding and communication with an intrahepatic duct. Haemobilia has never been reported as a complication of non-parasitic cyst of the liver.

[Malignant endocrine tumor of the pancreas associated with high blood thyrocalcitonin levels]. / Tumeur maligne endocrine du pancréas associée à des taux sériques élevés de thyrocalcitonine.

A case of thyrocalcitonin-secreting endocrine tumour of the pancreas is reported. Endocrine pancreatic tumours are neoplasias evolving over a long period; most of them are multisecreting, usually with a predominant secretion responsible for clinical symptoms. In this particular case the retrospective diagnosis of a thyrocalcitonin-secreting tumour of the pancreas was made by immunocytochemical study of the pancreatectomy specimen. One year after pancreatectomy, the persistence of clinical signs and a plasma thyrocalcitonin level higher than 25,000 pg/ml led to the discovery of a liver metastasis. An immunocytochemical study of the segmental hepatectomy specimen showed a single population of thyrocalcitonin-secreting cells identical with those found in the pancreatic tumour. In this context, radioimmunological assays of peptides and immunocytochemistry seem to be the best diagnostic methods.

[Metastatic Darier-Ferrand dermatofibrosarcoma. Review of the literature apropos of a case]. / Dermatofibrosarcome de Darier-Ferrand métastasé. Revue de la littérature à propos d'un cas.

We report the case of a 56-year old male patient with pulmonary, abdominal and pelvic metastases from a dermatofibrosarcoma (DFS) which had developed 23 years previously in the right scapulo-humeral region and had recurred twice after the first excision, in 1983. Surgery was only performed once. The most remarkable feature of the disease was the progressive transformation, with each recurrence, of a typical DFS into a poorly differentiated and highly malignant tumour, about as active as a fibrosarcoma. A review of the literature yielded 31 cases of histologically proven metastatic DFS. Although the actual incidence of metastases is difficult to determine with accuracy, their frequency may be estimated at 3 p. 100 (most probably a maximum figure). The disease spreads mostly through the blood, but also sometimes through the blood and lymph. However, since lymphatic involvement is rare the usefulness of lymph node excision is extremely doubtful. Pulmonary lesions are the most frequent ones, but various other organs may be affected, although this seldom applies to the liver. Following a first excision, metastases occur within 6 years on average (range: 1 to 33 years). This mean delay is superior to the mean follow-up period for most of the important series without metastases. The histopathological picture is usually one of typical DFS but in some cases, including ours, it is much less typical and may even be frankly malignant. A few reports mention the presence of histiocytic infiltrates associated with the fibroblasts. The prognosis in patients with metastases is particularly poor: in most of the cases reported the patient died within the year following the discovery of metastatic lesions. The very high progressivity of the secondary lesions contrasts with the slow course of the primary tumour. None of the treatments tried (surgery, radiotherapy, chemotherapy) has resulted in a significant prolongation of life. No correlation can be established between the occurrence of metastases and the following parameters taken together: patient's age and sex, tim elapsed between the appearance of the tumour and the date of exicision, and histological features of the initial tumour. The development and number of recurrences seems to be the most significant predictive factor as regards the risk of metastasis. The noxious role of incomplete and/or repeated surgery creating vascular breaks, disrupting the defense barriers and encouraging malignancy in some cases has been mentioned by most authors; it emphasizes the value of the widest possible initial surgery.(ABSTRACT TRUNCATED AT 400 WORDS)

[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.

[Phoniatric and orthophonic management of patients during the awakening phase of coma]. / Prise en charge phoniatrique et orthophonique de patients en phase d'éveil de coma.

The creation of a structure adapted to serious head injuries, in the awakening phase of a coma, enables early treatment of these patients upon leaving intensive care. Along with a pluridisciplinary team, speech therapists will help establish non-verbal communication by setting up a yes-no code and by favouring and programming the resumption of deglutition before soliciting verbal communication and recuperation of the memory and all the cognitive functions.

[Enteropathogenic mechanisms involved in giardiasis in children (author's transl)]. / La giardiase intestinale chez l'enfant. Mécanismes de l'entéropathogénicité du parasite.

23 patients with gastroenteritis and 9 with severe malabsorption syndrome related to giardiasis were investigated in a semi-prospective fashion as follows: (1) conjugated bile acid levels measured in duodenal aspirate (thin layer chromatography) in 6 patients with steatorrhea. (2) intraepithelial lymphocytes count (results expressed as the number of intraepithelial lymphocytes per 100 epithelial cells) in small intestinal biopsies from the 32 patients, 11 of which had immunoglobulin deficiency (9 IgA deficiency). The results indicate that there is no decrease in the percentage of conjugated bile acids (mean percentage 90%; normal = 80); a significantly increased percentage of intra-epithelial lymphocytes is documented in giardiasis (11.1% +/- 6.7), versus 2.3% +/- 0.5 in acute gastroenteritis (9 patients) and 6.3 +/- 0.5 in chronic diarrheas (6 patients) (p less than 0,001). This percentage, however, is significantly lower than in untreated coeliac sprue (23 patients) (12.17 +/- 11.6) (p less than 0,01). Conversely a high intraepithelial lymphocyte count does not correlate with the degree of intestinal villous atrophy (3 patients had severe and 6 partial villous atrophy) (r = 0.170). IgA deficiency should be suspected in patients with giardiasis presenting with intestinal villous atrophy (5 patients). Steatorrhea in our patients does not appear related to bile acid deconjugation. To explain enterotoxicity in giardiasis, more than a direct effect of the ventral disk of the parasite on intestinal mucosa, one should incriminate the host immune cell mediated response as shown by lymphocytic infiltration of the epithelium on small bowel biopsies.

[Pseudo-tumorous panniculitis of the mesentery. An unusual initial stage of acute lupus erythematosus in a 10-year-old girl]. / Panniculite pseudo-tumorale du méso gastrosplénique. Forme inaugurale inhabituelle d'un lupus érythémateux aigu chez une fille de dix ans.

Lupus panniculitis usually affects the hypodermis and the dermis profundus (Irgang's lupus profundus). In our case, the systemic lupus erythematosus was preceded by an intraabdominal panniculitis, occurring as a voluminous tumor. Histological examination showed the usual picture of panniculitis, but there were also severe fibrinoid necrotic lesions, which should have alerted us. Lupus must be included among the etiologies of intraabdominal panniculitis.

[Wilson's disease. A clinical and pathological study on 6 cases (author's transl)]. / La maladie de Wilson. Etude anatomoclinique de six observations.

In connection with 6 cases of Wilson's disease, the authors recall the main features of this hereditary metabolic disorder at late onset (usually the second decade), treatable with a chelating agent, when diagnosed at an early stage. Wilson's disease is first of all a liver disease and the authors emphasize the fact that cirrhosis is usually present when neurologic symptoms, revealing the disease in 5 cases, appear, even if there is no clinical or biological evidence for liver disease. In one instance hemolytic anemia and chronic active hepatitis were observed at clinical onset. Copper metabolism usually gives the key for diagnosis but its interpretation may be difficult, a normal serum ceruleoplasmin level being found in two patients and evaluated at 6% in the literature. This fact brings up the puzzling question of the pathogesis of the disease. Wilson's disease is not a simple ceruleoplasmin synthesis defect, but a lysosomal disease responsible for the lack of copper biliary excretion. This is pointed out by histochemical studies using a special rubeanic acid preparation (revealing copper deposit on the biliary side of the hepatic cell), and by electron microscopy showing lysosomal dystrophy.

Mixed familial polyposis syndromes.

Three cases of polyposis consisting of adenomatous and hamartomatous elements are described. The literature on mixed polyposis syndromes is reviewed.

[Cholestatic icterus during parenteral feeding in the newborn ano infants]. / Ictères cholostatiques au décours de l'alimentation parentérale chez le nouveau-né et le nourrisson.

The authors observed six cases of cholestatis jaundice occuring during parenteral alimentation in the newborn and the infant. In 4 cases, local factors (duodenal atresia, necrosing enteritis of prematurity) or general factors (mucoviscidosis) probably played an essential role. In two cases, the parenteral alimentation seemed to be a main factor, but in one case with excessive and imbalanced supplies of amino acids. Three patients survived, with regression of the jaundice in 15 days to three weeks following the interruption of parenteral alimentation.

[Multilocular pancreatic cystadenomas in the child. Apropos of 1 case]. / Cystadénomes pancréatiques multiloculaires de l'enfant. A propos d'un cas.

One patient, 9 months old was operated upon for a pancreatic multicystic adenoma. 6 other cases only are reported. Emphasis is made on the microscopic pathology of this lesion among other pancreatic cysts in infancy. Conservative surgery is advocated.

[Benign pseudo-tumoral lymphoid hyperplasia with massive Destombes-Rosai-Dorfman histiocytosis]. / Hyperplasie lymphoïde bénigne pseudo-tumorale avec histiocytose massive de Destombes-Rosai et Dorfman.

The authors report one case of sinus histiocytosis with massive lymphadenopathy which occurred in a 70 years old woman. They remind the main features of this pseudolymphomatous benign disorder with three key microscopic findings : massive sinus histiocytosis, lymphophagocytosis, proliferation of mature plasma cells. There is no effective therapy and nothing definite about the etiology.

[Stewart-Treves syndrome. 2 cases with an ultrastructural study]. / Syndrome de Stewart-Treves. Deux cas avec étude ultrastructurale.

The authors present an ultrastructural study of two cases of Stewart-Treves syndrome in elderly women. Under light microscopy, the dermal and hypodermal proliferations showed vascular features which were more clearly preceptible in the second case than in the first. Ultrastructural studies showed, in the first case, clefts containing erythrocytes and surrounded by neoplastic cells which were linked by zonulae adherentes. A rudimentary form of basal membrane was also noted, with an exterior lining of pericyte-like cells. In the second case, typical capillary vessels were bordered by endothelial cells containing a few pinocytotic vesicles and microfilaments, in contact by zonulae adherentes. The walls of these vessels consisted of a clearly defined membrane lined with pericytes, and constituting, in some areas, an anastomotic network due to the swelling of the endothelial cells. Ultrastructural study suggests an angiosarcomatous origin of these lesions. Finally, the article includes a review of the literature.