Relapsing lymphomatoid papulosis after allogenic bone-marrow transplant.

Lymphomatoid papulosis (LyP) is a rare cutaneous lymphoproliferative disorder in children, which can rarely be associated with a cutaneous or systemic lymphoma. We report a 13-year-old girl who presented with typical LyP and pathological features of subtype A. Six months later, the patient presented with rapidly progressive peripheral and systemic lymphadenopathy. On examination of a lymph-node biopsy, a lymphoid infiltrate negative for anaplastic lymphoma kinase (ALK) and positive for CD30 was found, suggestive of systemic anaplastic large T-cell lymphoma (S-ALCL). The patient was treated with chemotherapy, followed by allogeneic bone-marrow transplant (BMT). Over the following 6 years, she presented with biopsy-confirmed LyP relapses with complete cutaneous, peripheral-blood and bone-marrow chimerism. This is only the third reported paediatric association of S-ALCL with LyP to our knowledge, and seems to be the first paediatric case of recurrent relapses of LyP after bone-marrow allograft for S-ALCL with total (100%) cutaneous and bone-marrow chimerism. LyP occurring after allogenic BMT does not appear to be donor-derived.

[Xanthelasma associated with Wegener's granulomatosis]. / Xanthélasmas et granulomatose de Wegener.

BACKGROUND: There have been rare reports of normolipidaemic xanthelasma in Wegener's granulomatosis. Herein, we describe another case associated with a review of the literature. CASE REPORT: A 62-year-old woman was hospitalized for Wegener's disease with cutaneous, ENT, renal, joint and neurological signs. Physical examination showed bilateral palpebral xanthelasma with infiltrated lacrimal glands that had appeared simultaneously to the other clinical signs. No hyperlipidaemia was noted. Biopsy of the xanthelasma revealed a typical infiltrate of foamy histiocytes and focal vasculitis. On treatment with corticoids and cyclophosphamide, the yellow lid lesions gradually disappeared. DISCUSSION: Normolipidaemic xanthelasma is rarely reported in association with Wegener's granulomatosis. It accompanies local ophthalmological inflammation and its appearance during the course of this disease must be dealt with carefully.

[Congenital cyst and fistula of the face and neck]. / Kystes et fistules congénitaux de la face et du cou.

The authors propose an update on cervicofacial congenital cysts and fistulas' symptomatology. Embryological data, epidemiology and clinical manifestations are described. A reminder of the therapeutic principles is proposed as well as the evolution of these congenital pathologies, which may or may not involve the branchial system. branchial.

Mixed adenocarcinoma and squamous cell carcinoma arising in a gastric duplication cyst.

Malignant transformation of duplication cyst is a rare condition. The authors report the original case of a degenerated gastric duplication cyst in a 67-year-old patient. The histologic examination revealed a gastric duplication cyst infiltrated with both adenocarcinoma and squamous cell carcinoma. Local carcinomatosis was found at laparotomy. The patient died six months after complete macroscopic resection of the lesion, with metastatic disease.

[Recurrent adenomas in ileal pouch following ileoanal anastomosis for familial adenomatous polyposis]. / Adénomatose récidivante du réservoir iléal après anastomose iléoanale pour polypose adénomateuse familiale.

Polyadenoma occuring on ileal pouch is a rare event. The treatment and the frequency of the endoscopic examinations is still matter of controversies. The authors report the case of recurrent endoileal pouch adenoma.

[Pyodermatitis-pyostomatitis vegetans with nasal involvement]. / Pyostomatite-pyodermatite végétante avec atteinte nasale.

BACKGROUND: Pyodermatitis-pyostomatitis vegetans (PPV) is a rare chronic disorder often associated with inflammatory bowel disease. We report an isolated case involving the oral, labial and nasal mucosa. PATIENTS AND METHODS: A 28-year-old man, in good general condition, presented with a 2-year history of painless stomatitis. The physical examination revealed pustular and exophytic lesions of the jugal, gingival and palatine mucosa on an erythematous background, as well as some pustular and crusted lesions of the lower lip and nostrils. Histopathological analysis revealed epithelial hyperplasia and a suprabasal cleft with some signs of acantholysis and numerous neutrophils and eosinophils. Direct and indirect immunofluorescence assay was negative. There was no associated bowel disease. We concluded on a diagnosis of PPV of younger subjects. The lesions disappeared with oral corticosteroids but with steroid dependency. DISCUSSION: PPV is a rare dermatosis associated in more than 75% of cases with inflammatory bowel disease, usually ulcerative colitis. Lesions of the oral mucosa are a constant finding and are characterised by aseptic pustules on an erythematous background. Skin lesions are pustular and more or less exophytic. To our knowledge, there have been no reports to date of intranasal lesions of PPV.

[Rating of tumoral growth in non-operated primary or recurrent extra-abdominal aggressive fibromatosis]. / Etude de la croissance tumorale des tumeurs desmoïdes extra-abdominales (FDEA) primitives ou récidivées non opérées.

PURPOSE OF THE STUDY: Extra-abdominal aggressive fibromatosis (EAAF) is a benign desmoid tumor with a potentially aggressive behavior. Surgical treatment is compromised by a very high rate of recurrence, sometimes with significant morbidity. We conducted a prospective surveillance of our patients (clinical and MRI) with EAAF to search for prognostic factors. MATERIAL AND METHODS: This cohort included 17 patients with EAAF. For nine patients, biopsy alone was performed. For eight, the tumor was a recurrence after surgical removal. Patients were seen for a clinical assessment and MRI every six months. RESULTS: Median follow-up was 42 months (range 6-114). Three patients worsened clinically with pain or functional impairment. One patient required neurosurgery to control pain (good stable outcome). MRI showed progression for two tumors (12%) but with a short follow-up since diagnosis (9 and 14 months), in one case despite medical treatment. Three tumors regressed and twelve remained stable on successive MRI. On average the tumor growth lasted ten months. DISCUSSION: Tumor growth was never noted beyond 36 months. This notion of an interruption in tumor growth is mentioned sporadically in reports on EAAF, which have generally included recurrent tumors. To our knowledge this is the first series reporting tumors left in place a followed with modern imaging techniques. The high rate of spontaneous interruption of tumor growth must be counterbalanced with the difficult task of local treatment: the risk of recurrence is particularly high after surgery and functional sequelae can be significant when wide resection is proposed in an anatomically difficult localization. The precise role for surgery, and combined radiotherapy, remain to be determined. There are only scarce reports on general treatments. Considering these facts, we propose that surgical resection should not be considered the only solution for the treatment of EAAF. Further work is needed to define the useful contribution of simple surveillance of these benign tumors.

Two giant orf lesions in a heart/lung transplant patient.

Orf is an infectious ulcerative stomatitis of sheep and goats. The responsible pathogen, parapoxvirus, may be transmitted to humans. Orf lesions are often atypical in immunocompromised individuals. The present report describes two very large exophytic lesions in a 31-year-old transplant patient receiving oral tacrolimus, mycophenolate mofetil and prednisone. Early surgical excision was successful, with no relapse after 14 months.

Tk, a new colon tumor-associated antigen resulting from altered O-glycosylation.

Erythrocyte polyagglutination antigens T and Tn are truncated O-glycan chains that are also carcinoma-associated antigens. We investigated whether Tk polyagglutination antigen could similarly be a carcinoma-associated marker and a target of immunotherapy. Monoclonal antibody LM389 was raised against Tk erythrocytes and tested by immunohistochemistry. LM389 strongly reacted with 48% human colorectal carcinomas. Labeling of normal tissues was visible on epithelial cells, mainly digestive, but was confined at a supranuclear level. Expression of the antigen on cloned human carcinoma cells correlated with sialosyl-Tn expression. O-Sialoglycoprotein endopeptidase treatment revealed that on carcinomas and cell lines, the epitope was present on O-glycans. Antibody specificity was determined using synthetic carbohydrates. Direct binding and inhibition studies indicated that LM389 best ligands were terminated by two branched N-acetylglucosamine units. Screening of murine cellular cell lines with LM389 allowed development of an experimental model with Tk-positive and -negative cells in syngeneic BDIX rats. Vaccination of rats with Tk erythrocytes provided a protection against growth of rat Tk-positive, but not of Tk-negative, tumor cells in association with the development of antibodies. Taken together, the results indicate that Tk polyagglutination antigen is a new colorectal carcinoma-associated antigen, absent from the normal cell surface, resulting from alteration of O-glycans biosynthesis and with potential as a target of immunotherapy.

Human submucosal neurones regulate intestinal epithelial cell proliferation: evidence from a novel co-culture model.

The role of the human enteric nervous system (ENS) in the control of the intestinal epithelium organization and proliferation is unknown. To address this issue, we developed a novel co-culture model, consisting of human submucosa containing the submucosal plexus and a human colonic epithelial monolayer. After 3 days in basal conditions (i.e. in absence of neuronal activation) epithelium disorganization and proliferation occurred. In contrast, electrical activation of submucosal neurones maintained monolayer organization and decreased cell proliferation. These effects were blocked by tetrodotoxin and a vasoactive intestinal peptide (VIP) receptor antagonist, and reproduced by VIP. In conclusion, our study suggests that the human ENS is involved in the control of epithelial cell proliferation.

[Systemic vasculitis]. / Les vascularites systémiques.

Systemic vasculitis is a general term designating a wide range of sometimes overlapping vascular conditions with marked tropism for peripheral constituting a complex nosological structure. Angiologists must be aware of the different telltale clinical signs including purpura, polyarthritis, glomerulonephritis, polyneuritis and unexplained inflammatory syndrome. Medium-sized and small-sized vessels are involved in a wide range of visceral damage. Several typical clinical presentations have led to the individualization of recognized syndromes such as rheumatoid type purpura, Wegener disease, Churg disease, Strauss disease, or peri-arteritis nodosa. Anti-nuclear serologies (pANCA and cANCA) contribute to diagnosis and have been helpful in individualizing a new entity: microscopic polyangeitis. In angiological practice, macroscopic expression of systemic vasculitis can lead to Takayasu type granulomatous angeitis in yong women and giant-cell arteritis in the elderly. These different arteriopathies should be treated by corticosteroid therapy with regular laboratory tests to follow inflammation and angiograhic exploration in case of occlusive arterial complications. Although these conditions are quite rare, a better understanding of their pathogenesis and their clinical course should help the angiologist make the initial diagnosis and the therapist to monitor treatment efficacy.

[Sarcomatoid variant of chromophobe renal cell carcinoma. Report of 2 cases]. / Variante sarcomatoïde du carcinome rénal à cellules chromophobes. A propos de 2 observations.

In this paper, we report two cases of sarcomatoid carcinoma arising in chromophobe carcinoma. The sarcomatoid component appeared as white, shiny areas, occupying less than 10% of each tumor and containing numerous mitotic figures. Whereas both components expressed cytokeratin, only the carcinomatous cells were positive for epithelial membrane antigen and only the sarcomatoid cells were found to express vimentine. Sarcomatoid transformation in chromophobe cell carcinoma has been reported exceptionally. An aggressive behavior may be expected. The variable proportion of sarcomatoid component may lead to multiplication of the samples, especially when white, firm and shiny areas are detected on gross findings.

[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.

[Breast metastasis from a "tall cell variant" of papillary thyroid carcinoma]. / Métastase mammaire d'un carcinome papillaire à cellules hautes de la thyroïde.

The authors report a case of breast metastasis from a "tall cell" variant of papillary thyroid carcinoma in a 59-year-old woman. This metastasis was discovered two months after the diagnosis of an inextirpable thyroid tumor. Tall cell variant of papillary thyroid carcinoma is a sub-type of papillary carcinoma with an aggressive course and frequent metastases (in patients over 50 years of age). No mammary metastasis of this tumor type has been reported so far.

[Pulmonary endodermal tumor resembling fetal lung. Low grade adenocarcinoma of the fetal lung type]. / Tumeur pulmonaire endodermique ressemblant au poumon foetal. Adénocarcinome de type poumon foetal de bas grade.

Pulmonary endodermal tumor resembling fetal lung is a rare pulmonary neoplasm, classified either within the pulmonary blastomas spectrum or as a subtype of adenocarcinoma. We report a case revealed by a fever in a 24-year-old woman. The tumor measured 9 cm and extended into the lower right bronchus. The diagnosis was done on a biopsy performed during fiberoptic endoscopy. The patient was treated by lobectomy. She is well without disease 6 years after surgery. This type of predominantly epithelial tumor with neuroendocrine differentiation and a scanty non malignant stromal component should be identified in young women because of its favorable outcome after surgical resection. It must not be confused with ordinary adenocarcinoma nor metastatic adenocarcinoma, especially endometrioid type.

[Disclosure of Behcet disease by concomitant dental infection]. / Poussée inaugurale de maladie de Behçet concomitante d'un foyer infectieux dentaire.

BACKGROUND: Behçet's disease can be aggravated by chronic infection. Our case illustrates how the disease can be disclosed during an acute activation of a chronic infectious focus. CASE REPORT: A 40-year-old man had Behçet's syndrome associating fever, bipolar aphtosis, cutaneous pseudo folliculitis, anterior uveitis, and meningitis following an acute episode of a dental infection, with periapical granuloma. Antibiotic therapy was ineffective in calming this first flare-up of Behçet's disease. Corticosteroid and colchicine therapy were effective. DISCUSSION: Clinical and experimental work would implicat buccal streptococcal flora in the pathogenesis of Behçet's disease. Antibiotics offer interesting perspectives but further studies are needed to define their role in the treatment of Behçet's disease.