An unusual case of mycosis fungoides presenting as sarcoidosis or granulomatous mycosis fungoides.

The authors describe a case of cutaneous and lymph node granulomas first reported as sarcoidosis. As skin sarcoidlike reactions disappeared, the development of typical histologic and immunopathologic features of cutaneous mycosis fungoides suggested granulomatous mycosis fungoides. This case illustrates the difficulties in differentiating true systemic sarcoidosis associated with mycosis fungoides from sarcoidlike reactions when extensive granulomas obscure the underlying cutaneous lymphoma. This report emphasizes the utility of immunohistochemical analysis to identify the early cutaneous T-lymphomatous infiltrate, initially admixed with epithelioid and giant cell granulomas. This technique also made it possible to characterize a Ki-1-positive anaplastic large-cell lymphoma when the transformation of mycosis fungoides into highly malignant lymphoma occurred in the lymph node.

Magnetic resonance imaging of von Meyenburg complexes: report of a pathologically documented case.

Von Meyenburg complexes (VMC) are rare malformations of the bile ducts. They are usually associated with adult dominant polycystic disease (ADPCD). Although generally benign, they may give rise to cholangiocarcinomas. Herein, we report the third MR documented case of VMC. The comparative diagnostic value of ultrasound (US), computed tomography (CT), and magnetic resonance (MR) examinations is discussed.

Differential effects of butyrate derivatives on human breast cancer cells grown as organotypic nodules in vitro and as xenografts in vivo.

The antiproliferative and cytodifferentiating effects of a new stable butyric derivative, monobut-3, were compared using human MDA-MB-231 breast cancer cells grown in three dimension as either in vitro tumor nodules or in vivo xenograft tumors. In in vitro tumor nodules, monobut-3 exhibited marked growth inhibitory effects consistent with the results obtained in monolayer cell cultures. Some functional cell differentiation was also detected in treated nodules. In in vivo xenografts, monobut-3 significantly decreased MDA-MB-231 tumor take but did not affect the rate of tumor growth. No difference was noted in the histological characteristics of the xenografts between untreated and treated mice. Moreover, once monobut-3 treatment was discontinued, tumor growth rapidly resumed in tumor-free animals. The decreased efficacy of monobut-3 in in vivo MDA-MB-231 xenografts as compared to in vitro tumor nodules indicates that factors related to host environment may still limit the clinical effectiveness of this compound.

Virginal breast hypertrophy at puberty; a case report.

Plastic surgery reduction with free nipple graft was performed on an eleven and a half-year-old girl referred to us with massive bilateral macromastia. Hormonal studies revealed no abnormalities. Histopathological examination confirmed the diagnosis of virginal hypertrophy, an infrequent etiology in adolescent macromastia.

[Effects of LAK cells activated by IL-2 on MCF-7 human breast cancer cell line maintained in organotypic culture]. / Effets des cellules LAK en présence d'IL-2 sur la lignée carcinomateuse mammaire humaine MCF-7 maintenue en culture organotypique.

Lymphokine Activated Killer (LAK) cells, stimulated by interleukin 2 (IL-2) have a pronounced antitumor effect in the therapy of melanoma and renal cancers. LAK cells were cultivated in presence of the nodules of the human breast adenocarcinoma cell line MCF-7 maintained in organotypic culture to study the interactions between lymphocytes and breast tumor cells. After two days of co-culture, the proliferation of MCF-7 nodules and that of LAK cells was diminished about five folds. The cytotoxic effect of the latter, appreciated by Chrome 51 release was unchanged after the coculture. In histological sections, the penetration of the LAK cells into the MCF-7 nodules was accompanied by an increase of tumor necrosis but also by a glandular differentiation of cancerous tissue. Polarized epithelial cell formations bording neoplasic lumens with intracytoplasmic vacuoles filled with mucus, appeared in the nodules. The immunohistochemistry underlines the presence of T lymphocytes marked by UCHL1 and CD3 antibodies and of Natural Killer (NK) cells marked by IOT10, located between the MCF-7 cancer cells. In electron microscopy, the membrane contacts were tight and were accompanied by the appearance of secondary lysosomes and nuclear alterations. The relatively low infiltration level of the nodules may lead to the supposition that an indirect mechanism will intervene in this dual action of a LAK cells: increase of necrosis, although partially, and development of glandular and functional differentiation.

[Meningoradiculitis disclosing T-lymphocyte leukemia in HTLV-1 infection]. / Méningo-radiculite révélatrice d'une leucémie à lymphocytes T au cours d'une infection à HTLV1.

A case of HILV1-associated adult T cell leukaemia/lymphoma (ATLL) in à 21-year olf African woman is reported. The patient presented with lymphomatous meningoradiculopathy. The usual clinical features of ATLL were absent. Lumbar MRI showed a pial enhancement by DTPA-gadolinium of the conus medullaris which extended to the proximal cauda equina. Under systemic chemotherapy coupled with intrathecal chemotherapy the patient progressively improved, and at the second MRI examination complete disappearance of the lumbar enhancement was observed. MRI of the brain using axial and coronal T2-weighted sequences detected multifocal lesions of high-intensity signal in the subcortical white matter. ATLL is unusual in people of African origin. The ATLL-strongyloïdes infestation association has previously been reported, suggesting that parasitic infestation may be an important co-factor leading to the development of ATLL.

[Adrenal medullary hyperplasia: a rare etiology of arterial hypertension--report of a case]. / L'hyperplasie médullosurrénalienne: une étiologie rare de l'hypertension artérielle--rapport d'un cas.

Adrenal medullary hyperplasia is often misdiagnosed. The clinical features may resemble those of pheochromocytoma, with paroxysmal hypertension and elevated urinary catecholamine and metanephrine levels. Pathologic study shows diffuse or nodular adrenal medullary hyperplasia, determined by morphometric analysis: increased adrenal gland weight, increased relative medullary volume, increased relative medullary weight, decreased cortico-medullary ratio. Adrenal medullary hyperplasia may be primary or sporadic, but is often associated with multiple endocrine neoplasia (MEN) type II.

[Apropos of 1 case of paratesticular papillary serous cystadenocarcinoma]. / A propos d'un cas de cystadénocarcinome papillaire séreux paratesticulaire.

The occurrence within the testis or paratesticular tissue of serous tumors, similar to ovarian tumors, is rare. This article reports a primary serous paratesticular cystadenocarcinoma in a 39 year-old man. From data of the literature, we offer guidelines for diagnosis, histogenesis and treatment of this rare tumor.

[Congenital hepatic fibrosis. Five cases with late occurrence in adults]. / Fibrose hépatique congénitale. Cinq cas à révélation tardive chez l'adulte.

Congenital hepatic fibrosis (CHF), is an autosomal recessive disease, presenting principally in childhood with portal hypertension and/or cholangitis, and often associated with renal malformations. The forms presenting later in adults are rare, and illustrated herein by 4 cases with dominant cholangitis, and one latent form. Biological tests and radiological imaging are often normal. The histopathologic diagnosis, sometimes difficult on liver needle biopsy is based on fibrous enlargement of portal areas, with numerous and tortuous bile ducts, lined by regular, cuboidal epithelium. Interportal fibrosis can mimic cirrhosis. In CHF, cholangitis are favoured by intrahepatic biliary dilatation, sometimes related to Caroli's disease, associated in 25% of cases. Suppurative complications, sometimes fatal explain the severity of cholangitis forms of CHF, contraindicating inopportune cholangiography and biliary surgery.

[Botryoid rhabdomyosarcoma of the cervix. Clinico-pathologic study of a case]. / Rhabdomyosarcome botryoïde du col utérin. Etude anatomo-clinique d'un cas.

The botryoid rhabdomyosarcoma of the cervix is a rare tumour occurring in young woman or during genital activity. It mainly causes vaginal bleeding or appears as a polypoid grape-like mass with a gelatinous cut-surface. The diagnosis is based on the presence of a submucosal cambium layer and a rhabdomyoblastic differentiation, corresponding to an intracytoplasmic double cross-striation. Metaplastic cartilaginous islands are sometimes observed. The immunostaining ensures the muscular origin of the tumour, characterized by the expression of actin and desmin. Apart from mullerian adenosarcomas, the main differential diagnosis is represented by the benign polypoid formations of the cervix: the genital rhabdomyoma and the fibroblastic lesion, called fibro-epithelial polyp with atypical stroma. The treatment actually includes both chemotherapy and surgery which is often limited to a conization.

[Bacillary peliosis in AIDS. Anatomo-clinical study of 2 cases]. / Péliose bacillaire au cours du SIDA. Etude anatomo-clinique de 2 observations.

The authors report two cases of peliosis hepatis, occurring in patients with AIDS, who presented a persistent fever and an hepatomegaly. The liver biopsies showed areas of peliosis, where bacilli were observed by Warthin-Starry stain. In one case, techniques of molecular biology allowed the identification of Rochalimaea henselae, pathogen involved in bacillary angiomatosis. This rickettsia has been newly recognized in the United-States, where 17 cases of bacillary peliosis have been published in immunocompromised hosts and mainly in patients with AIDS. These observations illustrate the clinical and histological features of this new opportunistic infection, as it is described in the literature. The clinical signs include an unexplained fever, an hepatomegaly, and in 75% of the cases, a splenomegaly. The cutaneous lesions of bacillary angiomatosis are associated in 40% of the cases. An antibiotic treatment by erythromycin ensures a complete recovery.

[Cardiovascular complications in chronic atrophic polychondritis. Apropos of an anatomo-clinical case. Review of the literature]. / Les complications cardiovasculaires de la polychondrite chronique atrophiante. A propos d'un cas anatomo-clinique. Revue de la littérature.

The case of a man, 25 years of age and presenting with chronic atrophic polychondritis (CAP), complicated by a complete atrioventricular block, double mitral valve incompetence and aneurysm of the ascending aorta, offered the possibility of investigating the various clinical manifestations and cardiovascular complications of this common disorder. CAP is a connectivitis of unknown etiology, it corresponds to ubiquitous and recurrent cartilage inflammation, leading to characteristic chondritis of the ears and nose, joint disease and laryngo-trachco-bronchial disorders. Other systemic impact is seen at sites containing high levels of proteoglycans, such as the eye, inner ear and cardiovascular system. Respiratory problems are the main cause of death, but cardiovascular effects occur in 25% of cases and constitute the second most frequent cause of mortality. These effects consist mainly of aortic and/or mitral valve incompetence. Annular dilatation, which is often associated with ectasia of the ascending aorta, is the main cause of aortic incompetence. Several cases of isolated AVB or AVB secondary to Al have been reported. Aneurysms develop along the aorta and the large and medium caliber arteries (sub-clavicular, coronary, mesenteric arteries). These are characterized by destruction of the elastic fibers and a reduction in the proteoglycan content of the walls, which is also observed when dystrophy of the cartilage occurs. Other vascular disorders reported include arteritis of the legs, superficial migratory varices and vascularitis, which in some cases gave rise to skin, renal or neurological reactions.

[A rare ovarian pseudotumor: massive edema of the ovary]. / Une pseudo-tumeur ovarienne rare: l'oedème massif de l'ovaire.

The authors report a case of massive ovarian edema which declared itself by pain in the abdomen and pelvis and an ovarian mass measuring 13 cm in diameter, occurring in a 22-year-old woman. Since it was not possible to make a diagnosis by any frozen-section examination, histology was carried out on the ovary that had been removed. This showed that the stroma of the ovary had become separated by massive edema preserving the albuginea and the superficial cortex. This case history of massive edema of the ovary shows the characteristics of this ovarian pseudotumour as described in the literature. The principal differential diagnoses of the condition are oedematous fibroma, and myxoma of the ovary. Apart from the fact that torsion of the adnexae can occur in some of these cases, the pathogenesis is still unexplained. When an ovarian tumour is found in a young woman a frozen-section examination must be carried out to make the diagnosis and perhaps avoid oophorectomy, particularly when untwisting a torsion can lead to resorption of the edema.

[Rochalimaea henselae infection. Febrile pancytopenia and hepatic peliosis in a patient with HIV infection]. / Infection par Rochalimaea henselae. Pancytopénie fébrile et péliose hépatique révélatrices chez un malade infecté par le VIH.

A case of Rochalimaea henselae infection in an AIDS patient is reported. The R. henselae infection was revealed by febrile pancytopenia associated with liver and spleen enlargement. The diagnosis was made on the finding at histology of hepatic peliosis lesions. Within these lesions Warthin Starry staining displayed bacilli that were identified as R. henselae. This case shows that all HIV-infected patients with these symptoms might be infected with this organism.