Intestinal obstruction complicating a mesenteric cystic lymphangioma.

Mesenteric cystic lymphangioma is a rare benign tumor. Diagnosis is suggested by radiology and confirmed by histology. Treatment is surgical and the prognosis is good.

[Cutaneous vasculitis, myelodysplasia and relapsing polychondritis]. / Vascularite cutanée et myélodysplasie au cours d'une polychondrite chronique atrophiante.

BACKGROUND: Relapsing polychondritis is a rare autoimmune disease, characterized by recurrent inflammation of cartilaginous tissues. In some cases, many other tissues can be involved. CASE REPORT: We describe the case of a 64 year-old man with relapsing polychondritis, whose first symptoms were papulonodular and mucosal aphthous lesions. The skin biopsy revealed vasculitis without leukocytoclasic features. The diagnostic of relapsing polychondritis was made only two years later, thanks to recurrent auricular chondritis. Since the beginning, the disease was associated with a myelodysplasia. DISCUSSION: Dermatological manifestations are noticed in less than 50 p. 100 of cases during the evolution of relapsing polychondritis. An association with a myelodysplasia has already been reported, and it could be a particular form of relapsing polychondritis. Myelodysplasic syndromes are more frequently associated with relapsing polychondritis than with other vasculitis. It must be sought especially when cutaneous and mucosal manifestations exist.

Does paraaortic lymphadenectomy have a benefit in the treatment of ovarian cancer that is apparently confined to the ovaries?

We conducted a retrospective review of all epithelial ovarian carcinoma patients with disease that is apparently confined to the ovaries who were treated in the Obstetric and Gynecologic Hospital of the University of Tours. In our hospital, no lymphadenectomies for such epithelial ovarian carcinoma patients are carried out. We studied the survival of these patients that were operated upon from 1 December 1975 until 1 August 1997. 43 epithelial ovarian carcinoma patients were studied; 22 were stage Ia, 1 was stage Ib and 20 were stage Ic. The average age was 58 years (range 27-86 years). 5% (2/43) developed recurrent disease and the rates of disease-free and overall survival after 5 years were 83% and 90.3% respectively. These results are very close to those described in literature for patients who underwent paraaortic and pelvic lymphadenectomy. As no series to date has demonstrated the benefit of paraaortic lymphadenectomy on survival and we know that paraaortic lymphadenectomy increases morbidity, we think it reasonable to propose surgery without lymphadenectomy for the treatment of early ovarian epithelial cancer patients whose disease is apparently confined to the ovaries.

Low alpha-linolenic acid content of adipose breast tissue is associated with an increased risk of breast cancer.

Data derived from experimental studies suggest that alpha-linolenic acid may have a protective effect in breast cancer. Observations obtained from epidemiological studies have not allowed conclusions to be drawn about a potential protective effect of dietary alpha-linolenic acid on breast cancer, possibly because of methodological issues. This case-control study conducted in an homogeneous population from a central area in France was designed to explore the hypothesis that alpha-linolenic acid inhibits breast cancer, using fatty acid levels in adipose breast tissue as a biomarker of past qualitative dietary intake of fatty acids. Biopsies of adipose breast tissue at the time of diagnosis were obtained from 123 women with invasive non-metastatic breast carcinoma. 59 women with benign breast disease served as controls. Individual fatty acids were analysed by capillary gas chromatography. An unconditional logistic regression model was used to obtain odds ratio estimates whilst adjusting for age, menopausal status and body mass index (BMI). No association was found between fatty acids (saturates, monounsaturates, long-chain polyunsaturates n-6 or n-3) and the disease, except for alpha-linolenic acid which showed an inverse association with the risk of breast cancer. The relative risk of breast cancer for women in the highest quartile of adipose breast tissue alpha-linolenic acid level was 0.36 (95% confidence interval=0.12-1.02) compared with those in the lowest quartile (P trend=0.026), suggesting a protective effect of alpha-linolenic acid in the risk of breast cancer. The effects of dietary alpha-linolenic on the risk of breast cancer warrant further study.

Emphysematous pyelonephritis related to specific gas-forming Escherichia coli without diabetes mellitus.

A 60-year-old-man without a history of diabetes mellitus, or invasive manipulation or obstruction of the urinary tract was admitted for septic shock. Type I emphysematous pyelonephritis was clear in this case: gas within the renal parenchyma extending into the subcapsular region and the perirenal space was present on spiral computerised tomography (CT). Surgical nephrectomy was performed because biochemistry, urography and CT identified a damaged non-functioning left kidney. The outcome was favourable. All urine, blood and nephrectomy specimen cultures were positive for a specific Escherichia coli which produced a high level of gas compared to a reference E. coli strain in the same standard medium, despite the absence of diabetes mellitus. Certain strains of bacteria are able to produce high levels of nitrogen, carbon dioxide and hydrogen and such fermentation in the absence of a high glucose serum level might explain the acute gas-producing bacterial renal infection.

Herpetic salpingitis and fallopian tube prolapse.

AIM: We describe the unusual association of fallopian tubal prolapse and herpetic infection, an occurrence not previously reported to our knowledge. METHODS AND RESULTS: A 37-year-old woman presented with a small polypoid mass of the vaginal vault, 3 months after abdominal hysterectomy and abdominoplasty. The vaginal mass proved to be the fimbriated end of a fallopian tube, herniated into the vagina. Reintervention 3 months later with resection of a small vaginal 'polyp' revealed a residual portion of fallopian tube, with superimposed herpes simplex virus (HSV) infection and marked cytological atypia of surface epithelial cells. HSV-2 immunostaining of viral nuclear inclusions and of atypical cells confirmed the herpetic nature of the infection. CONCLUSION: Involvement of the genito-urinary tract by HSV may occur via an ascending infection from the cervix, but the fallopian tube, deeply located in the pelvis, is generally spared from herpetic infection. In the setting of fallopian tubal prolapse, direct exposure of the herniated fallopian tube to various pathogens in the vagina provides an unique clinical model for salpingitis. In herpetic tubal infections, special attention must be paid to cytological atypia of probable viral cytopathogenic origin, to avoid a misdiagnosis of malignancy.

[An unusual variant of urothelial carcinoma: the "nested variant of urothelial carcinoma." Report of two cases]. / Une variante particulière de carcinome urothélial: le "nested variant of urothelial carcinoma". A propos de deux cas.

We report two cases of a poorly known variant of transitional cell carcinoma, the "nested variant of urothelial carcinoma". This tumor is composed of small islands or nests of transitional cells, presenting little atypia and mimicking von Brünn's nests. This low grade tumoral variant seems to behave as a high grade tumor of the same stage. Deep biopsies are necessary to display tumoral invasion, which allows the diagnosis. Importance of the knowledge of this entity is highlighted in order to avoid misdiagnoses that could delay appropriate therapy.

[Intraneural perineurioma: an unusual nerve sheath tumor]. / Le périneurome intra-neural: une tumeur nerveuse inhabituelle.

We report a case of intraneural perineurioma, developed in the left cubital nerve, in a five-year old girl. This intraneural tumor is rare and shows a typical histologic appearance: concentric whorls of perineurial cells EMA+, PS100-, around nerve fibers. This tumor must be distinguished from extraneural or soft tissue perineurioma, also composed of perineurial cells, with distinct clinical presentation and histological appearance.

[Idiopathic granulomatous mastitis. Review of the literature illustrated by 4 cases]. / Mastite granulomateuse idiopathique. Revue de la littérature illustrée par 4 observations.

INTRODUCTION: Idiopathic granulomatous mastitis (IGM) was described as a specific entity in 1972 by Kessler and Wolloch. Despite the 120 cases reported in the international literature, this pathology remains quite unknown. MATERIAL AND METHODS: Four cases of idiopathic granulomatous mastitis are reported in this article in order to outline the main clinical features of this affection. Data in the literature were used to discuss diagnostic and therapeutic particularities. DISCUSSION: The histologic findings of a non-caseating granulomatous inflammation, centered on breast lobules, composed of epithelioid cells and multinucleated giant cells, allow establishing the diagnosis of granulomatous mastitis (GM). The main presenting symptom of GM is a single inflammatory mass of the breast; the diagnosis is thus often mistaken for breast carcinoma (more than 50% of the reported cases). Radiologic and cytologic findings alone do not enable reaching certain diagnosis because they cannot resolve the differential diagnosis of inflammatory process and malignancy. The diagnosis of IGM can be established after the etiological work up remains negative. A course of oral corticosteroid therapy, non-steroidian anti-inflammatory drugs, or colchicine can be used in order to shrink the breast mass, allowing more conservative surgery. Local excision is of limited benefit as there is a strong tendency for recurrence. CONCLUSION: Different therapeutic options of IGM are explained by its clinical variability. A more pragmatic therapeutic approach would be enabled by a new classification based on course and severity.

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.

[Desmoplastic recurrence of a melanoma of the female urethra]. / Récidive desmoplastique d'un mélanome de l'urètre féminin.

We report a case of melanoma of the female urethra, recurring on the vulva as a desmoplastic invasive melanoma. The urethral tumor was an unclassifiable melanoma with an invasive epithelioid, and an intraepidermal atypical melanocytic component. The desmoplastic recidive was made of intersecting fascicles of spindled, moderately atypical cells, scattered over a dense collagenous matrix. Tumor cells were immunoreactive for S100 protein and vimentin, negative for HMB45. The morphological and immunophenotypic differences with the initial tumor, the absence of melanocytic dysplasia within the overlying epidermis, could lead to a misinterpretation of this lesion as a fibrous scar or a fibrohistiocytic tumor. Desmoplastic melanoma shares many homologies with acrolentiginous and mucous membrane melanoma and could occur more frequently on the genitourinary tract than it is generally assumed.

Solitary fibrous tumour of the larynx: report of a case.

Solitary fibrous tumour is a particular kind of mesenchymal tumour, classically arising in the pleura. We report the first case arising in the larynx, associated with a metastasizing adenocarcinoma. The diagnosis is mainly histopathological, especially when strong immunoreactivity for the CD34 antibody is present.

[MALT lymphoma with parotid and gastric involvement during Gougerot-Sjögren syndrome]. / Lymphome du MALT à localisation parotidienne et gastrique au cours du syndrome de Gougerot-Sjögren.

Sjören syndrome favors the development of lymphoma, particularly in the salivary glands with MALT lymphomas. The differential diagnosis with benign lymphoepithelial sialadenitis can be difficult. A 78-year-old woman had an oculo-buccal sicca syndrome for 10 years and developed parotid hypertrophy. The first biopsy, performed 7 years before the present investigation had showed chronic lymphoepithelial sialadenitis. A second biopsy showed MALT lymphoma. Search for extension revealed a second gastric localization of the lymphoma. This patient had a particular immunophenotype, showing a CD5+ tumoral population frequently observed in mantel lymphomas and usually lacking in MALT lymphomas. Recently, however, another case similar to our own, has been reported in the literature. The observations raise the problem of distinguishing between mantel lymphoma and MALT lymphoma.

Microanatomy of the hypophyseal fossa boundaries.

OBJECT: The authors studied the heads of 17 adult cadavers and one fetus to clarify the anatomy of the sellar region, particularly the lateral boundaries of the hypophyseal fossa. METHODS: Vascular injections and microdissection or histological techniques were used in this study. The roof of the cavernous sinuses and diaphragma sellae were part of a single horizontal dural layer that joined the two anterior petroclinoid folds. Laterally, the direction of this layer changed; it became the lateral wall of the cavernous sinus and joined the dura mater of the middle cerebral fossa. On the midline, this layer ballooned toward the sella through the diaphragmatic foramina, created a dural bag containing the hypophysis, and attached to the inferior aspect of the diaphragma sellae. As a consequence, no straight sagittal dural wall existed between the pituitary gland and cavernous sinus; the lateral border of the hypophyseal fossa was part of this anteroposterior and superoinferior convex bag. The authors stress the importance of the venous elements of the region and discuss the structure of the cavernous and coronary sinuses. CONCLUSIONS: Invasion of the cavernous sinus makes surgery more risky and difficult and may necessitate modification of the surgical treatment plan. The preoperative diagnosis of cavernous sinus invasion is thus of great interest, but the possibility of normal lateral expansions of the pituitary gland must be kept in mind. A lateral expansion of this gland into the cavernous sinus was encountered in 29% of the specimens, and an adenoma that developed in such an expansion could easily mimic cavernous sinus invasion.

[Similarities between pleuropulmonary blastoma and Askin tumor]. / Similitude du blastome pleuropulmonaire de l'adulte et de la tumeur d'Askin.

Adult pleuropulmonary blastoma is an uncommon mesenchymal tumor of the peripheral lung parenchyma composed of undifferentiated cells. The differential diagnosis with Askin-Rosaï's tumor, an extrapulmonary pleuroparietal neuroectodermal tumor, may be a difficult task. We propose a similar therapeutic approach for these two malignant conditions: surgical excision associated with chemotherapy and autologous bone marrow transplant with adjuvant radiotherapy in case of recurrence. These two entities have many epidemiological, clinical, histological and immunohistochemical similarities.

[Adenoid basal carcinoma and adenoid cystic carcinoma of the uterine cervix]. / Carcinome adénoïde basal et carcinome adénoïde kystique du col utérin.

Adenoid basal carcinoma and adenoid cystic carcinoma are rare primary cervical neoplasms occurring in postmenopausal women. They are generally regarded as distinct entities on the basis of their distinctive morphology and a different biological behaviour. Although the separation of these two entities is still mandatory, some rare complex cases may show overlapping pathological features and are difficult to classify. We report a case of composite cervical tumor with both types of carcinoma, an in situ epidermoid carcinoma and a hitherto undescribed clear cell component, each with a different morphology and immunophenotype. The adenoid basal component is mainly immunoreactive for cytokeratin KL1. The adenoid cystic component is strongly stained for smooth muscle actin and is associated with a hyaline stroma immunostained for collagen IV.

[Spinal leptomeningeal tumor spread disclosing a pineocytoma]. / Essaimage tumour leptomeninge spinal révélateur d'un pineocytome.

A 39-year-old female was admitted to the hospital because of a sudden meningeal syndrome followed by diplopia, cervical, dorsal and sciatic nerve pains, and right peripheral facial palsy. Cerebrospinal fluid obtained by lumbar puncture showed a protein level at 23 g/l. Myelography and magnetic resonance imaging (MRI) were in favor of a lumbar arachnoiditis. A meningeal biopsy revealed a tumour infiltration with foci of cells that were stained with anti-glial fibrillary acidic protein antibody. Cerebral MRI was performed to search for a central nervous system (CNS) primary tumour, and disclosed a pineal mass. Five months after the onset of the disease, the patient worsened her clinical state and died. Necropsy confirmed the presence of a pineocytoma with astrocytic differentiation and diffuse leptomeningeal spread. This exceptional occurrence leads us to discuss about primary tumours of the CNS with leptomeningeal spread.