[Intracranial ependymomas in adult patients. Diagnosis and histological prognostic factors]. / Ependymomes intracrâniens de l'adulte. Diagnostic histologique et facteurs histopronostiques.

BACKGROUND: Intracranial ependymomas are rare in adults and histopathological prognostic factors are poorly determined. PURPOSE: A retrospective multicentric study was conducted in France in order to assess the prognostic value of histology. MATERIAL: Between 1990 and 2004, 216 adult patients with newly diagnosed ependymomas were treated in 19 French centers. Eligibility required institutional histopathological confirmation of an ependymoma and available clinical history and MRI features (see comparison paper). METHODS: Histological preparations and one paraffin embedded block from each patient were sent to Pr D. Figarella-Branger in Marseille. Central review by four neuropathologists (D. Figarella-Branger, A. Maues de Paula, C. Fernandez and A. Jouvet) was performed. Specimens for which all pathologists agreed with the histological diagnosis of ependymomas were included, whereas cases for which all disagree were excluded and reclassified. In the event of doubt and/or discrepancies between pathologists immunostaining was performed in order to reach a consensus diagnosis. Diagnostic of ependymomas was confirmed in 121 cases (56%). In theses cases, ependymomas were classified according to the WHO system (subtype and grade). The potential prognostic value (overall survival OS and disease free survival DFS) of the following histological parameters was examined: perivascular pseudorosettes, ependymal rosettes, hyalinized vessels, mitotic index, microvascular proliferation, necrosis, area of increased cellularity, nuclear atypia, brain invasion and Mib-1 labelling index. RESULTS: Among the 121 ependymomas, 88 were grade II (47 classic, 17 cellular, 2 papillar, 6 clear cells and 16 tanicytic) and 33 grade III. WHO grading, occurrence of microvascular proliferation, necrosis, nuclear atypia and high proliferative index were correlated with both OS and DFS. Moreover, quantification of certain parameters enabled a reproducible grading system correlated with both OS and DFS.

[Perforating granuloma annulare with transfollicular perforation]. / Granulome annulaire perforant unique avec phénomène d'élimination transfolliculaire.

INTRODUCTION: The perforating granuloma annulare is a rare form of granuloma annulare. The clinical diagnosis is difficult and the confirmation is histological. The localisation is unique in less than 10% of all cases. We report a documented case with a histological suspicion of transfollicular perforation. OBSERVATION: A 36 year-old woman, without any particular antecedent, presented on her upper arm a single ulcerated nodular lesion that had evolved for one year. The clinical examination and biological investigations were normal. The histological examination led to the diagnosis of perforating granuloma annulare with a large epidermic ulceration. Treatment with a topical corticosteroid was disappointing and the removal was decided. DISCUSSION: In our observation, the single localization of this lesion raises the problem of differential diagnosis such as cutaneous tuberculosis, atypical mycobacteriosis, skin sarcoidosis, foreign body granuloma, epidermoid carcinoma or perforating dermatitis. The histological examination permitted diagnosis of a perforating granuloma annulare with large epidermic ulceration. The infiltration and destruction of a hair follicle evoked the possible transfollicular elimination of the necrotic material. Other pathologies with the histological aspect of a palisading granuloma were excluded from this context. No associated pathology such as diabetes nor any other favouring factors such as ultraviolet light or insect bites or traumas were identified. The physiopathology of perforating granuloma annulare and the process of perforation remain unknown. Numerous therapies have been proposed with variable results.

The dural crossing of the vertebral artery.

The aim of this study was to present the anatomic and macroscopic aspects of the vertebral artery at the level of the dural crossing. Twenty vertebral arteries in 10 cadavers were dissected. The preparations were photographed and all the samples at the level of the dural crossing were submitted to a histologic study. Macroscopic results allow specification of the relationships between the dural crossing of the vertebral artery and the nervous elements (C1 and C2 nerves, mixed nerves), the vascular elements (postero-inferior cerebellar artery), the dural sheath and the first two dentate ligament tips. Microscopic results show the intricate relationship of the different layers of the vertebral artery with the dura at its crossing. Dural fibers reinforce the adventitial fibers not only by continuing them in their contact, much as the pages of a book, but also by being encrusted into the adventitia up to the tunica media, thus creating a very efficient mooring system. This work provides some information for an understanding of the symptoms induced by foramen magnum meningiomas and the onset of clinical signs after the surgical excision of these tumors. It also explains why dissections of the suboccipital segment of the vertebral artery stop at the level of the dural crossing, and never spread towards the intracranial segment.

Postoperative bilateral vertebral artery dissection: a case report.

With the improvement of medical imaging and surgical techniques, surgery on cervical vertebral is more frequent. Some cases of complications of this type of surgery have been described. We report a case of postoperative bilateral vertebral artery dissection. It concerns a 58 year-old woman who suffered from a left cervico-brachial C6 neuralgia with paresthesiae of the thumb. She underwent discectomy at C5-C6 and C6-C7 followed by setting up intersomatic cages. In subsequent days, an irreversible coma developed. Supra-aortic echographic study revealed bilateral vertebral artery thrombosis. CT scan revealed ischemic lesions of the brain stem and cerebellum. Cerebral death was declared five days after the operation. Autopsy was performed to determine whether death was the consequence of the intervention. The cause of death was determined to be ischemic brain injury of the brain stem and cerebellum resulting from bilateral traumatic occlusion of the vertebral arteries caused by the surgery.

[Difficulties in diagnosis of human dirofilariasis outside known enzootic areas]. / Problèmes soulevés par le diagnostic de la dirofilariose humaine à distance d'une région enzootique connue.

INTRODUCTION: Human non-visceral dirofilariasis, a mosquito-borne disease of carnivores (dogs), is chiefly due to Dirofilaria repens in France and is well known in the Mediterranean basin. This zoonosis can be misdiagnosed in northern areas of France. We present and discuss two human cases diagnosed in Abbeville and Amiens (Picardy) by histological examination. EXEGESIS: The former case appeared as an axillary tumefaction occurring in a 41-year-old women 6 months after holidays in Montauban (southwestern France), the other as an intraorbital 'tumor' in a 53-year-old man who travelled for professional purposes in Central Europe and North America. Morphological, clinical, and epidemiological data of these human infections are discussed and the diagnostic features in tissue sections for species identification are reviewed. CONCLUSION: Increasing travel customs during the last decades favour the emergence of zoonotic parasites unusually in human hosts. Outside known enzootic areas, diagnosis is often delayed until pathological examination. In France, the incidence of human dirofilariasis has steadily increased and must be considered in the workup of cutaneous or intraorbital nodules.

Angiosarcoma variant of Kasabach-Merritt syndrome.

Kasabach-Merritt syndrome is characterized by the occurrence of disseminated intravascular coagulation (DIC) usually caused by benign angiomatous tumours. Here we report the case of a 70-year-old man in whom DIC revealed a locally advanced hepatic tumour. Although DIC resolved with heparin, antithrombin III, fresh frozen plasma and corticosteroids, the patient died from haemoperitoneum following a fall, 3 months after the initial observation. Histopathological examination by autopsy allowed the diagnosis of hepatic angiosarcoma. The physiopathogenic mechanisms and treatment options are discussed.

[Cellular angiofibroma. A rare vulvar tumor. Report of a case]. / L'angiofibrome cellulaire, une tumeur vulvaire rare. A propos d'un cas.

Cellular angiofibroma is a rare tumor. We report a vulvar case in a 37 year old woman. This nodular, well circonscribed tumor consists of bland spindle cells, numerous thin or thick often hyalinized vessels and adipocytes. The stromal cells are positive for vimentin and negative for CD34, protein S100, smooth muscle actin, desmin, epithelial membrane antigen and cytokeratin. Cellular angiofibroma is a benign tumor that has to be differentiated from aggressive angiomyxoma, angiomyofibroblastoma, glomangiopericytoma, spindle cell lipoma, solitary fibrous tumor and perineurioma.

[Epithelioid leiomyosarcoma of the uterine cervix. Report of a case]. / Léiomyosarcome épithélioïde du col utérin. A propos d'un cas.

We report a case of an epithelioid leiomyosarcoma of the uterine cervix in a 42 year-old woman. This is a very rare tumor. Usually, the presenting symptoms are vaginal bleeding and abdominal pain. Two problems have to be solved by the microscopy: to prove the smooth muscle differentiation of the tumor and to assert the malignancy. Surgery remains the basis of therapy. Prognosis is poor.

Giant cell arteritis localized to the colon associated with Crohn's disease.

AIMS: Intestinal vasculitis is uncommon and usually accompanies systemic vasculitis. Although intestinal vascular changes including vasculitis have been studied intensively, and are found regularly in Crohn's disease, giant cell arteritis is distinctly unusual. We describe a case of giant cell arteritis localized to the colon of an 18-year-old girl suffering from Crohn's disease. METHODS AND RESULTS: After three years of medical treatment, the patient underwent a proctocolectomy. The medium-sized arteries of the mesocolon demonstrated striking changes characterized by intimal fibrous thickening and an inflammatory infiltrate with giant cells, most predominant at the intima-media junction. Epithelioid cells and sarcoid-like granulomas were not observed. The internal elastic lamina was fragmented. Neither clinical symptoms nor laboratory findings showed evidence of systemic vasculitis. Neither the chest CT scan nor the echo-Doppler of the temporal arteries, supra-aortic and abdominal vessels revealed any abnormality. CONCLUSIONS: This case illustrates an extremely rare feature in the spectrum of vascular lesions in Crohn's disease which have to be differentiated from temporal and Takayasu's arteritis.

[Lipid pneumonia and pleural-pulmonary atypical mycobacteriosis]. / Pneumopathie lipidique et mycobactériose atypique pleuro-pulmonaire.

We report a case of fat deposit pneumonia extending to the parietal pleura with a granulomatous reaction centered on the fat vaculoles. This case was complicated by an atypical and fatal mycobacterial infection caused by Mycobacterium chelonae. The severity of Mycobacterium chelonae infection in fat deposit pneumonia warrants early empirical antibiotic therapy.

[Cellular neurothekeoma, a rare cutaneous tumor. Anatomo-clinical and immunohistochemical study of 2 cases]. / Le neurothécome cellulaire, une tumeur cutanée rare. Etude anatomo-clinique et immunohistochimique de deux observations.

Cellular neurothekeoma is an unusual cutaneous tumor described in 1986. We report two new cases in 14-year-old girls. Both tumors measured less than one centimeter and were located on the forearm and the shoulder. They had a plexiform architecture and were developed in the dermis and the dermo-hypodermic junction. Spindle and epithelioid tumor cells were immunoreactive for vimentin, NKI-C3, NSE, CD68 and smooth muscle actin. There was no recurrence 2 and 7 months after the operation. Cellular neurothekeoma is a benign tumor. Its histogenesis is still unknown. Histological and immunohistochemical features, with positivity of the tumor cells for NKI-C3, a non specific melanocytic marker, and negativity for S-100 protein and HMB-45, allow to differentiate cellular neurothekeoma from myxoid neurothekeoma and melanocytic tumors.

[Mesothelial cyst of the adrenal gland]. / Kyste surrénalien d'origine mésothéliale.

We report a case of a mesothelial adrenal cyst which was incidentally discovered in a 38-year old woman. The diagnosis was confirmed by an immunohistochemistry study. Adrenal cysts are rare. They occur more commonly in female patients during the 4th and 5th decades and are usually asymptomatic. Adrenal cysts are divided into four groups : parasitic, epithelial, endothelial cysts and pseudocysts. The management is still discussed : follow-up, surgical resection or fine-needle aspiration.

[Traumatic pulmonary pseudocysts. Mechanisms of formation]. / Les pseudokystes pulmonaires traumatiques. Mécanismes de formation.

Pulmonary pseudocysts (PPC) classically relate to chest trauma. It is a rare entity in adults, with multiple differential diagnosis. PPC most often evolve favorably. The clinical diagnosis is difficult to assess due to the poor and non specific clinical data. Chest radiographs are usually unsufficient for the diagnosis and the imaging modality of choice is computed tomography (CT). CT patterns of PPC relate to single or numerous cavities surrounded by air space consolidations. The physiopathological mechanisms of PPC remains uncertain. The histological study of this reported case affords some worth data to highlight the pathogenesis of this acquired abnormality.

[Pathological anatomy of tumors of the third ventricle]. / Anatomie pathologique des tumeurs du troisième ventricule.

The walls of the third ventricle contain a large variety of tissues, and the pathological processes in this area are extremely varied. The pathological lesions encountered in the third ventricle are analyzed with interest to their incidence in the national series. Colloid cysts have a single layer of epithelial cells, mimicking the bronchus epithelium, which may reveal their probable endodermic origin. Xanthogranulomas are not true neoplasms but are only reactional to local hemorrhages. Histological diagnosis may be difficult for glial tumors if the biopsy sampling is insufficient : pilocytic astrocytomas can be overgraded and confused with high grade astrocytomas. Subependymomas should be separated from ependymomas, and giant cell subependymal astrocytomas are specific lesions occurring in tuberous sclerosis. Craniopharyngiomas often show a papillary type when located in the third ventricle. Germinal tumors are associated with immunological markers. The variety of the encountered lesions in the third ventricle needs a close collaboration between neuropathologists and neurosurgeons, and a confrontation of histological data with clinical and radiological data.

[Plurifocal testicular hamartomas and testicular feminization syndrome]. / Hamartomes testiculaires plurifocaux et syndrome du testicule féminisant.

We report the case of a 14-year-old girl with a testicular feminization syndrome. The inguinal cryptorchid testis contained plurifocal hamartomas ranging from 0.5 to 1 cm. They were composed of tubules lined by cylindrical Sertoli cells immunoreactive for alpha-inhibin and p30/32(MIC2). The stroma contained few Leydig cells. Ultrastructural study showed tubules with immature Sertoli cells. The testicular feminization syndrome is caused by mutations of the androgen receptor gene. Patients with male genotype 46, XY have a female morphotype with external sexual organs without ambiguity. They have neither uterus nor ovary but two cryptorchid testis in which sex-cord stromal tumors can develop. Their malignant transformation is rare but requires preventive bilateral orchidectomy.

Primary central nervous system lymphomas in 72 immunocompetent patients: pathologic findings and clinical correlations. Groupe Ouest Est d'étude des Leucénies et Autres Maladies du Sang (GOELAMS)

We reviewed 72 primary central nervous system lymphomas occurring in immunocompetent patients. The cases were reviewed for clinical data, histology, immunophenotype, bcl-2 and p53 expression, and Epstein-Barr virus association. Follow-up was available for 40 patients included in the Groupe Ouest Est d'étude des Leucénies et Autres Maladies du Sang (GOELAMS) lymphomes cérébraux primitifs (LCP 88) trial. Each diagnosis, requiring a consensus among at least 3 pathologists, was performed according to the recent Revised European-American Lymphoma classification and equivalents in the updated Kiel classification. Tumors were predominantly classified as diffuse large B-cell lymphomas. There were 3 T-cell lymphomas and 1 Hodgkin lymphoma. The proteins bcl-2 and p53 were expressed in 35% and 16% of the tested cases, respectively. Epstein-Barr virus was not found by in situ hybridization except in the case classfied as a cerebral localization of Hodgkin disease. No significant association was found between subtypes, bcl-2 or p53 expression, and patient survival. From the standpoint of their biologic characteristics, primary central nervous system lymphomas are very similar to systemic diffuse large B-cell lymphomas. In contrast to AIDS-related primary central nervous system lymphomas, primary central nervous system lymphomas are rarely associated with Epstein-Barr virus and in immunocompetent patients they express bcl-2 at a relatively low rate.