Tubulovillous adenoma of the vulva.

BACKGROUND: We report the second case of enteric-type villous adenoma of the vulva. The differential diagnosis, histogenesis, and pathogenesis are discussed. CASE: A 66-year-old woman had a tumor resected from the posterior aspect of the vulva. The tumor was characterized by its villous architecture, with columnar epithelium and goblet cells. This vulvar tubulovillous adenoma was identical to a tumor resected 6 months before from this patient's rectal wall. CONCLUSION: These two tumors behaved benignly, and the vulvar adenoma possibly originated with the müllerian vestige.

Lissencephaly type III, stippled epiphyses and loose, thick skin: a new recessively inherited syndrome.

We report on two new cases of syndromic lissencephaly in two consanguineous sibs, with skeletal abnormality, born to young, healthy, second cousin parents with healthy children. In Case 1, fetal ultrasound screening at 32 weeks of gestation showed microcephaly, skin infiltration and equinovarus feet. MRI disclosed cerebral agyria, hypoplastic cerebral mantle and posterior agenesis of the corpus callosum. The propositus, a boy, died soon after birth at term. In Case 2, fetal ultrasound study performed at 16 weeks of gestation disclosed skin infiltration. MRI at 22 weeks of gestation showed microcephaly with agenesis of corpus callosum and cerebellar hypoplasia. Pregnancy was terminated at 22 weeks of gestation. The fetus had normal 46, XY karyotype and similar anomalies found in the index case, with cranio-facial edema and arthrogryposis. X-ray films showed epiphyseal stippling of cervical vertebrae, feet and sacrum. Metacarpal bones were shortened with hypoplastic distal phalanges. Neuropathological findings were concordant with the pattern described in type III lissencephaly: an agyric brain with hypoplastic brain stem and cerebellum, severe neuronal loss of the cortical plate, matrix zone, basal ganglia, brainstem nuclei and spinal cord with axonal swelling and microcalcification. This entity seems to be a new syndromic lissencephaly type III, because of epiphyseal calcifications and metacarpophalangeal bone dysplasia.

[Granulosa cell tumour: review of the literature]. / Tumeur à cellules de la granulosa: revue de la littérature.

Granulosa cell tumours account for only 5% of ovarian malignancies and there is currently no standard treatment. It is considered as a low-grade malignancy with a favorable prognosis but relapse and extraovarian spread may occur as late as 20 years after diagnosis. It is thus important to better define the characteristics and prognostic factors of this histological subtype in order to recommend appropriate therapy. We performed a review of the literature. This disease is most commonly diagnosed in patients around 50 years old or at puberty. In most cases patients present identical symptoms associated with endometrial hyperplasia or neoplasia. Call-Exner bodies and "coffee bean" cells are characteristic of the histopathology of these tumors. Various prognostic factors are currently being evaluated. Surgery is the treatment for local disease. Patients with locally advanced, recurrent or metastatic tumors require chemotherapy, although the optimal regimen remains to be determined.

[Olfactory esthesioneuroma manifesting as Schwartz-Bartter syndrome]. / Esthésioneurome olfactif révélé par un syndrome de Schwartz-Bartter.

INTRODUCTION: Olfactory esthesioneuroblastoma is an uncommon neuroectodermal tumor originating from the olfactory epithelium, which is rarely associated with hormone excess syndrome. EXEGESIS: Asymptomatic olfactory esthesioneuroblastoma was diagnosed in a 22-year-old man who presented a syndrome of inappropriate antidiuretic hormone secretion. Following surgery, the immunohistochemical analysis demonstrated the existence of neurophysin hormone in tumoral cells. CONCLUSION: This case provides evidence that olfactory esthesioneuroblastoma can be uncovered by inappropriate antidiuretic hormone secretion.

Lymphomatoid granulomatosis--a report on four cases: evidence for B phenotype of the tumoral cells.

Four cases of lymphomatoid granulomatosis are reported, three of them involving the lung. Histological features included a true angiocentric and angiodestructive polymorphic cellular proliferation. This included histiocytes, plasma cells, many reactive T-cells and rare large, atypical cells which were of the B phenotype. Epstein-Barr virus was detected in the atypical cells by in situ hybridization in three cases, with expression of both latent membrane proteins (LMP)-1 and Epstein-Barr nuclear antigen-2 in two cases and expression of only LMP-1 in the third case. Expression of both of these proteins suggests a defect in the T-cell-mediated immunity and that Epstein-Barr virus is not only a silent passenger but may also be involved in the pathogenesis of the disease. This could have implications for therapy.

[Enormous lingual lymphangioma]. / Lymphangiome lingual monstrueux.

The authors report a huge lymphangioma of the tongue in a sixty-seven years old female patient. The remarkable progression in dimensions of this lesion, leading to the inevitable protrusion of the tongue, led to the realization of an extended glossectomy, with a functional objective in mind. This simple procedure showed an excellent five years follow-up results. From this case report, the authors stress up on the etiopathogenic, pathological, clinical as well as therapeutic aspects of lingual lymphangioma.

[Histopathologic study of melanoma of the choroid after proton therapy]. / Etude histopathologique des mélanomes choroïdens après protonthérapie.

To evaluate irradiation effects on choroidal melanomas, histopathologic findings of 18 eyes whose primary treatment was enucleation were compared to 15 eyes enucleated after proton beam irradiation. Irradiated tumors showed more likely necrosis (p = 0.01) had balloon cells (p = 0.01), and inflammatory infiltrate (p = 0.05). In the irradiated group, the prevalence of tumor blood vessel damage was higher (p = 0.0002) and mitotic figures were fewer (p = 0.01). These findings suggest that proton beam irradiation damages tumor cells and alters the tumor's capacity for cellular reproduction. It damages blood vessels leading to tumor necrosis. It induces an inflammatory response of unknown effects. Radiosensitivity of choroidal melanomas cannot be assessed using conventional histologic methods. However, tumor necrosis, mitotic activity and rate of balloon cells can help to establish tumoral sensitivity to irradiation.

Woodchuck hepatitis virus-induced carcinoma as a relevant natural model for therapy of human hepatoma.

BACKGROUND: Eastern American woodchuck (Marmota monax), naturally infected with woodchuck hepatitis virus, a virus similar to human hepatitis B virus, develops liver cancer with a high prevalence. AIMS: The aim of this work was to assess Marmota monax as a model of human hepatocellular carcinoma, especially to assess new potential adjuvant therapies after surgical resection. METHODS: Forty-four woodchuck hepatitis virus-infected animals were regularly screened by ultrasound examination from the age of 18 months and for a 30-month period. One or more liver tumors were diagnosed in 31 animals (70%). Five of them with multifocal tumor or poor general status were considered unsuitable for surgery. The other 26 were operated on. At laparotomy no tumor was found in three. RESULTS: The 18 liver tumors studied were hepatocellular carcinomas, grossly and microscopically similar to human hepatocellular carcinoma. Peritumoral parenchyma studied in 13 specimens was always non-cirrhotic but adequate staining demonstrated patterns of fibrosis in four cases. Clear evidence of chronic active hepatitis, periportal hepatitis and steatosis were demonstrated in five, seven and one of the 13 specimens, respectively. Tumors were treated by tumorectomy in eight animals, by alcoholization in seven and by laser photocoagulation in one. A simple tumor biopsy was performed in the other seven. Ten animals died postoperatively. All the survivors in the tumorectomy group died from tumor recurrence within 10-18 months after surgery. CONCLUSIONS: It is concluded that woodchuck hepatitis virus-induced liver carcinoma is a natural model of human hepatocellular carcinoma with similar pathology and natural history, including early ultrasonic detection and tumor recurrence after resection. Tumor excision is feasible in this animal model, which now provides the basis for assessment of new potential adjuvant therapies for human hepatocellular carcinoma in an attempt to reduce the high recurrence rate after surgical resection in humans.

[Pure embryonal carcinoma of the ovary. A review of the literature apropos of a case]. / Carcinome embryonnaire pur de l'ovaire. Revue de la littérature à propos d'un cas.

A 25-year-old woman with a stage IA pure embryonal carcinoma of the left ovary, without elevated serum markers namely AFP and HCG (nor-positive immuno-histologic marking at the later pathological analysis) underwent surgery alone (unilateral salpingo-oophorectomy). She relapsed seven weeks later with peritoneal carcinomatosis. She still did not have elevated tumor markers. She then received five courses of a cis platinum-based chemotherapy (bleomycin, etoposide and cisplatinum, BEP) and achieved pathological complete response (as attested by a coelioscopic third look). This response is still lasting, fourteen months after the end of the procedure. Malignant ovarian germ cells tumors account for 2 to 5% of all ovarian cancers and embryonal carcinoma is rare. It may be associated with high serum levels of AFP and/or HCG, but not when the embryonal carcinoma is really pure. These markers are very helpful for patient follow-up but not in our patient's history. These tumors offer a very good chemosensitivity especially when treated with cisplatinum. This treatment dramatically improved their prognosis and extensive debulking surgery is yet unnecessary. Some questions still remains is there any specificity for embryonal carcinoma within malignant germ cell tumors of the ovary group? What is the best treatment for stage IA disease? When is a surgical second look (or a third look as for our patient) justified?

[Wilson-Jones tumors of proliferating tricholemmal cysts. Apropos of 2 cases]. / Tumeurs de Wilson-Jones ou kystes tricholemmaux proliférants. A propos de deux cas.

The authors report two new cases of proliferating tricholemmal cyst. Physiopathological, clinical and histological findings are described. Differential diagnosis with squamous cell carcinoma is discussed.

[Condylar osteochondroma: apropos of 3 cases with arthritic lesions]. / Ostéochondromes condyliens: à propos de 3 observations avec lésions arthrosiques.

Three cases of osteochondroma of the mandibular condyle were observed with arthrosis type lesions. Based on a review of the literature, the epidemiologic, symptomatologic, pathologic and diagnostic characteristics of this rare benign tumour with facial localization are presented together with the therapeutic approach.

[Use of diode laser in transcleral retinal photocoagulation]. / Utilisation du laser à diode dans la photocoagulation rétinienne transsclérale.

Transscleral retinal photocoagulation using a diode laser emitting infrared radiation (810 nm) was performed on 8 rabbits. Examination was performed at 2 days, one month and three months after photocoagulation. The retinal burns were examined by light and electron microscopy and were found to be similar to those produced by argon and krypton photocoagulations. Histopathologic evaluation of the lesions demonstrated an intact sclera overlying the chorioretinal lesions. The results of the present experimental study support the hypothesis that transsccleral retinal photocoagulation using diode laser in selected indications may be a valuable alternative to cryotreatment and diathermy in the human eye. The absence of scleral damage and pigmented epithelium cell dispersion as well as the decreased break down of the blood ocular barrier after transscleral diode laser photocoagulation are the main advantages of the technique as compared to transscleral diathermy and cryotreatment.

[Pulmonary metastases from a hormone-induced clear cell adenocarcinoma of the vagina]. / Métastases pulmonaires d'un adénocarcinome vaginal à cellules claires hormon-induit.

The authors report the case of a patient aged 27 suffering from a clear cell adenocarcinoma of the vagina which was related to her mother taking Diethylstilboestrol or Distilbene (DES) during her pregnancy. This young woman presented with pulmonary metastases for which several trials of chemotherapy ended in failure. The case raises several problems amongst which are epidemiologies of cancers induced by Diethylstilboestrol (DES), their prognosis as well as the therapeutic strategies at the time of diagnosis and the recurrences which occur as metastases.

Acute silicosis due to inhalation of a domestic product.

A 20-yr-old woman with no history of pulmonary disease presented with acute dyspnea and fever. After various investigations and treatments the symptoms persisted and a lung biopsy was performed. Pathologic analysis disclosed the presence of silicotic crystals within lung tissue and mediastinal lymph nodes. The patient admitted having repeatedly inhaled a domestic scouring powder rich in silica during the preceding 6 months. This is the first report of acute pulmonary silicosis developing after exposure to a common household product.

[Cystic lymphangioma of the mediastinum. 2 personal cases and a review of the literature]. / Lymphangiomes kystiques du médiastin. 2 cas personnels, revue de la littérature.

Two mediastinal lymphangiomas (KLM) are described, in 36 and 41 year-old women. They presented as an incidental finding on chest radiograph. The mediastinal mass was anterior or posterior, compressing the adjacent vital structures, and cystic on computed tomography. The complete surgical removal was possible, but complicated by the size (12 x 7 x 4 cm and 10 x 7 x 5 cm), the hypervascularization and the infiltrative tendency of the lesions. The patients are well 36 and 30 months after removal. We compare these cases with 109 previously reported. They account for 1 p. 100 of the whole lymphangiomas and for 0.5 to 4.5% of all mediastinal tumours. The congenital or infantile cervico-mediastinal lymphangiomas are more frequent (10%). The general signs, symptoms, pathological findings, treatment and pathogenetic theories are discussed.

[Surgery of a voluminous palatal Kaposi's sarcoma developing in AIDS]. / Traitement chirurgical d'un volumineux sarcome de Kaposi palatin développé dans le cadre d'un SIDA.

The authors report of a case of voluminous palate Kaposi's Sarcoma in a patient with A.I.D.S. Oral Kaposi's Sarcoma occurs in a large percentage of patients with A.I.D.S. (30 to 50%). The therapeutic of this voluminous Kaposi's Sarcoma is not easy. Surgery is the best therapeutic it can bring, as in this case, functional and durable effect.

[Extra-pulmonary lymphangiomyomatosis presenting as an exudative enteropathy with a rapid fatal course]. / Lymphangiomyomatose extra pulmonaire révélée par une entéropathie exsudative d'évolution rapidement fatale.

We report a case of lymphangiomyomatosis (LAM) which occurred in a twenty six year old woman, presenting with protein losing enteropathy and pancreatic fibrosis due to lymphostasis. Despite having no pulmonary lesion, the patient died quickly. The proliferation of smooth muscle involved the mesenteric, peripancreatic lymphatic channels but also the submucosal lymphatic vessels of the duodenum. Only one other case of LAM presenting with protein losing enteropathy has been reported, in which there were no abnormalities in the intestinal tractus or in the pancreas.