[Carcinoma cuniculatum. Apropos of 4 cases with orofacial involvement]. / Epithélioma cuniculatum. A propos de 4 cas de localisation oro-faciale.

Carcinoma cuniculatum, a rare variant of verrucous carcinoma, was first described in the foot. The authors report 4 cases of uncommon localizations of this tumor, involving oral cavity and face, 3 of them having a 6-year follow-up. The specific histological and clinical features of this tumor are remembered and the difficulties of its diagnosis are emphasized. This slow-growing, ulcerated proliferation, invading the surrounding tissues, is often responsible for chronic suppuration, but very rarely metastasizes to the regional lymph nodes. The surgical treatment consists in a wide excision; neck dissection is theoretically useless, and radiotherapy is strictly contraindicated because of the risk of transformation into an anaplasic carcinoma. For all these reasons, carcinoma cuniculatum must be considered as an anatomoclinic entity, and deserves to be known by the clinicians and the pathologists.

[Arterial thrombotic complications in Crohn's disease]. / Accidents thrombotiques artériels au cours de la maladie de Crohn.

In a series of 230 observations of Crohn's disease, the authors describe 4 cases of arterial thrombosis; two of them involving cerebral arteries. These complications occurred in young women without any notable risk factor for atheroma. All patients had highly active Crohn's disease when arterial thrombosis occurred: two of them had several episodes of thrombosis and three, extraintestinal manifestations. As the arterial thromboses are often severe, rarely foreseeable and the venous thromboses frequent, the point is whether to use anticoagulants. When Crohn's disease is highly active, but only if there are no hemorrhagic lesions, anticoagulants at prophylactic doses may be recommended. How to define more exactly a high risk thromboses population deserves further investigation.

Is there an increased incidence of adenomatous polyps in breast cancer patients?

Using flexible proctosigmoidoscopy, the incidence of adenomatous polyps was studied in 161 patients previously operated on for breast cancer and also in 147 hospitalized controls not presenting with colorectal symptoms. The acceptance and tolerance of the examination were good in both populations. The mean age and length of the colons explored were not statistically different for the two groups. The incidence of adenomas was high in the breast cancer group (14.2%) in comparison to the control group (4.7%) (P less than 0.01). This strong incidence especially concerned very small polyps with a diameter smaller than 3 mm. There were also two polyps with superficial carcinomas in the breast cancer group. Breast cancer does indeed seem to represent a condition with a high risk of colorectal adenomas. Our results prompt us to propose the adoption of systematic screening measures and a surveillance schedule as soon as the breast cancer is found.

[Primary malignant melanoma of the digestive tract: esophagus and rectum. Apropos of 2 cases]. / Les mélanomes malins primitifs du tractus digestif: oesophage et rectum. A propos de 2 observations.

The authors report two cases of primary malignant melanoma. These primary neoplasms are exceptional on the GI tract. The diagnostical problems are discussed. The radiological features are a nodular lesion like an under-mucosa tumor.

[Abetalipoproteinemia. Apropos of 2 cases]. / L'abêtalipoprotéinémie. A propos de deux cas.

The cases of two sisters with abetalipoproteinemia are reported. Both presented the complete clinical and biological features of the disease: ataxia, retinitis pigmentosa, lack of apolipoprotein B, chylomicrons, LDL and VLDL, reduced titers of serum cholesterol and triglycerides, acanthocytosis, very low levels of serum vitamin A and E. Abetalipoproteinemia is a rare autosomal inherited disease. It is usually revealed during early childhood by steatorrhea and failure to thrive; ataxia and retinitis pigmentosa appear later. The originality of these two cases stems from: 1) their late and fortuitous diagnosis: the first sister was investigated at the age of 42 after the discovery of a vitamin K induced coagulation disorder. The other sister was 39 when she was routinely examined as a family member; 2) the presence of constipation without any other suggestive digestive complaint. However, white discoloration of the duodenal mucosa seen at endoscopy and lipid droplets within the intestinal absorptive cells at biopsy were characteristic. Barium studies showed diffuse involvement of the small bowel which was displaced by an enlarged sigmoid. Treatment consists of administration of vitamin A and vitamin E which prevent or delay ocular and neurologic symptoms. Vitamin K is associated whenever necessary.

[Specific gastric localization of Wegener's disease]. / Localisation gastrique spécifique de la maladie de Wegener.

We report an unusual gastric ulcer disease in a 52 year old white man which relapsed despite antacids and anti-H2 treatment. During the course of the disease, fever, weight loss, and ocular, oropharyngeal as well as systemic manifestations occurred. Gastric biopsies were normal until macrobiopsy was performed 5 months later demonstrating necrotizing granulomatous vasculitis. Further identical oropharyngeal mucosal lesions and a pulmonary infiltrate occurred supporting the diagnosis of Wegener's disease. The subsequent course was complicated by a massive hemorrhage which required total gastrectomy. Despite specific treatment with cyclophosphamide and steroids, the patient died. Most of the gastrointestinal lesions consistent with Wegener's disease are usually found at necropsy. Clinical gastrointestinal manifestations are scarce and rarely due to the underlying disease. Prognosis depends upon the renal lesions. However, as well, involvement of the digestive tract seems to be a factor of poor evolution of the disease.

[Esophagogastroduodenal localizations of Crohn's disease are frequent]. / Les localisations oesogastroduodénales de la maladie de Crohn sont fréquentes.

The purpose of this study was to determine the endoscopic features, actual frequency and consequences of upper digestive involvement in Crohn's disease (CD). Between April 1973 and April 1985, one or several upper digestive tract endoscopies with biopsies were performed in 129 of 195 patients admitted for ileal and/or colorectal CD. Endoscopic lesions were small and superficial: aphtous and superficial ulcerations, or erythematous areas. The diagnosis of upper digestive involvement was probable in 36 of the 129 patients who underwent upper endoscopies (28 p. 100), certain (microscopic evidence of granuloma) in 21 patients (16 p. 100). Lesions were isolated or multifocal but their preferential sites were the antrum and proximal duodenum. Histologic study of biopsies showed that granuloma were as frequent in upper as in lower intestinal lesions of CD. A frequency of 20 p. 100 seems to us to be more correct than the 5 p. 100 usually admitted. In our experience, upper digestive involvement in CD could not be regarded as a pejorative prognostic factor. It was sometimes possible to identify colitis or a peptic ulcer disease as a lower or upper digestive manifestation of CD, respectively.

[Malignant diffuse peritoneal mesothelioma: clinical and therapeutic aspects. Apropos of a case with gelatinous ascites]. / Mésothéliome péritonéal malin diffus: aspects cliniques et thérapeutiques. A propos de 1 cas accompagné d'ascite gélatineuse.

Peritoneal mesothelioma ranks second in frequency after pleural mesothelioma. A well-established clinical entity since 1960, mesothelioma is increasingly frequent due to improvements in diagnostic methods and to the fairly wide industrial use of asbestos, a well-known carcinogenic substance for mesothelial cells. The diagnosis of malignant peritoneal mesothelioma rests on a corpus of convergent data. Treatment consists of surgery combined with radiotherapy and chemotherapy. The mean survival from the time of diagnosis is 8-10 months.

Primary malignant melanoma of the esophagus.

Primary malignant melanoma of the esophagus is rare, and its symptoms are similar to those of squamous cell carcinoma. This tumor tends to be polypoid, pediculated, and irregular. Hematogenic and lymphogenic metastases are common. Surgical resection with reestablished continuity of the gastrointestinal tract is the treatment of choice, and postoperative irradiation may be useful. Despite these measures, however, the prognosis is poor, with a 5-year survival of 4.2%. The case of a 47-year-old man with esophageal melanoma is described, and a review of the world literature is presented.

[Complex recto-colic adenomatous and hamartomatous polyposis with hyperplastic gastric polyps in a 13-year-old girl]. / Polypose recto-colique complexe adénomateuse et hamartomateuse avec polypes gastriques hyperplasiques chez une jeune fille de 13 ans.

We report the case of a 13-year-old girl who presented mixed polyposis coli and gastric polyps. The rectal and colonic polyps were of the adenomatous and hamartomatous type (i. e. juvenile and Peutz-Jeghers polyps) without any intermediate features; in the stomach, the polyps were of the hyperplastic type. Microadenomas of the appendix and lymphoid hyperplasia of the ileum were also found. The karyotype was normal and the HLA group was A2 B12. No family history of polyps was discovered. To our knowledge no such case (complex polyposis) has been previously described. The present findings suggest that the usual pathological classification of polyposis may be arbitrary and that various pathological types may represent different aspects of the same spectrum of disease. Absence of "intermediate" polyps rule out the hypothesis of the sequence "hyperplastic polyp-juvenile polyp-adenoma".

[Chondrosarcoma of soft tissue. Apropos of a case]. / Chondrosarcome des tissus mous. A propos d'un cas.

This article reports a case of soft-tissue chondrosarcoma in the subcutis of the right supraspinal fossa of an 18-year-old woman who had undergone irradiation in infancy. A review of 123 published cases of extraskeletal chondrosarcoma raises the question of whether or not these tumors are really malignant in patients other than children. Their relationship with previous irradiation is also examined.

[Contribution to the study of cholecystoses. Clinical, radiological and pathological analysis (author's transl)]. / Contribution à l'étude des cholécystoses. Analyse clinique, radiologique et anatomo-pathologique.

Cholecystoses is a generic term introduced by Colesson and Jutras for a group of abnormalities of the gallbladder that appear to be separate from inflammatory diseases. Cholecystoses imply a benign proliferation of normal tissue elements, whereas cholecystosis indicates a pathologic process that is distinct from inflammation. 1 072 cholecystectomies were examined. 180 cholecystoses were diagnosed, 162 were associated with gallstones. There was no difference concerning the clinical symptom between cholecystoses and cholelithiasis. Radiological study confirms and clarifies the nature and character of these gallbladder lesions. In the histological examination 49,4 per cent of the cases were cholesterolosis, 2,9 per cent polyposis and 47,7 per cent adenomatosis. The treatment of cholecystoses is first and foremost medical, exception in case of calcareous and porcelain gallbladders. Surgery is only indicated in cases in which medical treatment is unsuccessful and when a cancerous degeneration is suspected.

[The intestinal polyposes (author's transl)]. / Les polyposes recto-coliques.

In a general review of the different varieties of the intestinal polyposes, emphasis is laid upon the main three types of the so-called "genetic polyposes" (juvenile polyposis, Peutz-Jeghers syndrome, and adenomatous polyposis) because of their peculiar interest concerning the pathological diagnosis, prognosis and genetic background. A correct histological diagnosis is of the utmost importance before an appropriated treatment can be initiated.

[Papillary and adenomatous polyps of the gallbladder. Clinical, radiological, and anatomical study, and report on 6 cases (author's transl)]. / Adénomes papillaires et polypeux de la vésicule biliaire. Etude clinique, radiologique et anatomique. A propos de 6 observations.

Papillary and adenomatous polyps of the gallbladder are frequently confused with cholesterol pseudotumors. They are rare tumors and are usually diagnosed only after histological examination of a specimen removed at operation. Their course is uncertain and they are potentially malignant lesions, as shown by one of these cases. The frequent association with lithiasis, which in fact can induce malignant changes, is a point in favour of the need for surgical therapy.