[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.

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.

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.

[Double breast and vertebral plasmacytoma. Acute myeloblastic leukemia 8 years later]. / Double plasmocytome mammaire et vertébral. Leucémie aiguë myéloblastique 8 ans plus tard.

We report in a 40 year-old woman a bifocal mammary and vertebral plasmocytoma attended by surgery and "preventive" chemotherapy. Eight years later, an acute and apparently non secondary myeloblastic leukemia is observed without sign of diffuse myelomatosis. Plasma cell tumors of the breast are uncommon. Eleven cases have been published, combining solitary plasmocytoma and infiltration occurring in multiple myeloma.

[Familial cardiomyopathy caused by carnitine deficiency]. / Myocardiopathie familiale par déficit en carnitine.

The authors report a familial case of carnitine insufficiency presenting in two out of seven children as a severe, isolated, hypertrophic and hypokinetic cardiomyopathy. The etiology was confirmed by histological study and measurement of carnitine concentrations in the blood and muscle. The evolution was spectacular with specific therapy. Left ventricular hypokinesia regressed completely within 18 months (fractional fibre shortening increased from 10 to 33% and the SCI from 26 to 55% in the more severe of the two cases). Hypertrophy and dilatation decreased significantly. This is a so-called intermediary form of carnitine insufficiency and very unusual because of the isolated cardiac involvement. These cases underline the value of systematic muscle biopsy with measurement of carnitine concentrations in the investigation of all cases of supposed primary cardiomyopathy, especially as a rapid improvement can be obtained by specific replacement therapy.

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.

[Pulmonary metastasis of meningioma of the falx cerebri]. / Métastases pulmonaires d'un méningiome de la faux du cerveau.

We report the case of a woman who had been operated upon for a recurrent meningioma of the falx cerebri which, 22 years after it was discovered, produced pulmonary metastases. Metastases from meningiomas are extremely rare; they affect the lungs in 60% of the cases or, less frequently, the liver or lymph nodes. The factors predictive of secondary dissemination are the site of the tumour, the quality of excision, the histological type (haemangiopericytic meningioma) and the presence of cell necrosis, hypercellularity and strong mitotic activity.

[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.

[Malignant dermoid cyst of the ovary with double tumor cell population, revealed by an umbilical metastasis]. / Kyste dermoïde cancérisé de l'ovaire avec double contingent tumoral, révélé par une métastase ombilicale.

The authors report a case of multiple malignancies (squamous cell carcinoma and carcinoid) arising in a dermoid cyst of the ovary. The tumor being revealed by an umbilical metastasis. They insist on the rarity of this entity and discuss the elements of diagnosis and prognosis through a review of literature.

[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.

[Pseudotumoral amyloidosis of the orbit]. / Amylose orbitaire pseudotumorale.

A case of orbital amyloid pseudotumour is reported; this rare condition typically present as a ptosis followed by slowly growing orbital neoplasm, in middle-aged women. CT-scan disclose an extra-conal, extra-osseous, calcified mass; preoperative diagnosis of this rare condition might help in avoiding recurrence, probably due to incomplete excision. Pathophysiology of these amyloid deposits remain unknown.

[Rare tumors of the prostate or seminal vesicles. Report of a case of leiomyosarcoma]. / Les tumeurs rares de la prostate ou des vésicules séminales. A propos d'un cas de lélomyosarcome.

An unusual case of leiomyosarcoma of the prostate presented as a recurrent pelvic cystic mass. Prostatic sarcoma are rare in adults with a poor prognosis. It is often difficult to determine a definite origin. The authors reviewed the differential diagnosis with pseudosarcoma, inflammatory fibrosarcoma and others rare tumors of the prostate and the seminal vesicle.

[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.

[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.

[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.

[Non-discal sciatica secondary to a cystic lesion of the posterior longitudinal ligament. 10 cases]. / Les sciatiques non discales secondaires à une lésion kystique du ligament longitudinal postérieur. 10 observations.

Cyst of the longitudinal posterior ligament of the spine is a new entity described here on the basis of 10 cases. This is a lesion, perhaps of traumatic origin, which occurs in young, athletic subjects suffering from classical lumbosciatica. Computerized tomography showed an image resembling nucleus pulposus herniation. In one of our cases magnetic resonance imaging provided the preoperative diagnosis. Following surgery the outcome was favourable, with less postoperative complications than with discal herniation. The macroscopic and histological findings were the same in all cases, making the anatomico-pathological diagnosis easy.