[Primary angiosarcoma of the kidney: case report and literature review]. / Angiosarcome primitif du rein : à propos d'un cas et revue de la littérature.

The primary angiosarcoma of the kidney is a rare tumor. We report a case of angiosarcoma of the right kidney in a man of 60 years. The CT-scan appearance is the one of a solid tumor compatible with renal cell carcinoma. Histological examination of the piece of nephrectomy straightens diagnosis and reveals the angiosarcomatous nature. In this patient with bone and lung synchronous metastasis, evolution has been a lightning death in less than three months. The literature review confirms the high potential of malignancy of these tumors (metastases almost constant and very short survival in spite of local and systemic treatment).

[Comparative study of periprostatic tissues thickness after retropubic or laparoscopic radical prostatectomy]. / Comparaison de l'épaisseur des tissus périprostatiques après prostatectomie rétropubienne ou laparoscopique.

OBJECTIVE: Analysing periprostatic tissue (PPT) thickness after retropubic (RP) or laparoscopic (LP) prostatectomy. MATERIAL: From January to December 2007, 114 consecutives prostatectomies were performed in our institution (38 RP, 76 LP). Clinical data were prospectively collected in a database. Gardner et al.'s (1988) procedure was used for pathological analysis. PPT thickness was measured on pathological specimens by a single observer on a single microscope. The observer had no knowledge of either clinical data or surgical approach. Four levels were chosen (at the base, the proximal part, the distal part, the apex) and 12 standardized measures were performed on each level, 48 measures: a prostate. We compared PPT thickness and surgical margins according to surgical approach and clinical data. RESULTS: Comparative analysis confirmed that LP and RP groups were similar as far as it concerns preoperative and pathological findings. Positive margin rate was also similar in LP and RP groups (4% versus 5.3%; P=0,37). Overall PPT thickness was thinner after LP than after RP except at the apex and the anterior face. Nevertheless, in the "complete preservation" group, PPT thickness was thinner at the apex in the RP group, thinner at the base in the LP group. CONCLUSION: Measuring PPT thickness was an original objective and reproducible way to compare different techniques and new technologies for radical prostatectomy. PPT sparing was different but not better with the laparoscopic approach.

[Bladder located gastric heterotopy: a case report confronting embryology to histopathology]. / Hétérotopie gastrique de siège vésical: un cas clinique au carrefour de l'embryologie et de l'histopathologie.

We report a case of bladder located gastric heterotopy, which has never been described, to our mind in the scientific literature. We discuss the diagnosis and the physiopathological mechanisms that may have been involved in the genesis of such a lesion.

Effectiveness of modified radical neck dissection and postoperative radiotherapy.

BACKGROUND AND OBJECTIVE: The aim of this study was to evaluate the effectiveness of a modified radical neck dissection with preservation of non-lymphatic structures usually removed in advanced-stage head and neck epidermoid carcinoma associated with postoperative radiotherapy (PORT). METHODS: We analyzed retrospectively the files of 109 patients, presenting with epidermoid carcinoma of the upper digestive/respiratory tract staged N2 or N3, over a 6-year period. The rates of regional control, mortality, and recurrence were analyzed and linked to the kind of neck-dissection (usual radical neck dissection [RND], modified radical neck dissection [MRND], selective neck-dissection [SND]) performed. RESULTS: Forty-three neck dissections were RND, 92 were MRND, and 21 were SND. PORT was used in all cases. The mean follow-up was 57.3 months. The overall rate of regional control was 93.6% (97.7% for RND and 93.5% for MRND; p=0.35). Patients having undergone MRND had a better prognosis and less recurrence then patients having undergone RND (respectively p=0.007, and p=0.0004). DISCUSSION: MRND in association with PORT is an effective treatment in patients with advanced head and neck epidermoid carcinoma staged N2 and N3.

Mandibular reconstruction using induced membranes with autologous cancellous bone graft and HA-betaTCP: animal model study and preliminary results in patients.

Providing an acceptable quality of life for patients after reconstruction of mandibular segmental defects is challenging because the surgical techniques available have limitations. The authors evaluated two-stage mandibular reconstruction in rabbits and provide preliminary results in humans. 21 rabbits underwent bilateral segmental mandibulectomy and the defect was filled with methylmethacrylate. The methylmethacrylate was removed after 4 weeks and an iliac autograft performed on the right-hand side and an autogenous graft with hydroxyapatite and triphasic calcium phosphate (HA-betaTCP) on the left-hand side. Four patients with severe mandibular osteoradionecrosis underwent a two-stage reconstruction. No clinical or paraclinical complications were noted. Hematoxylin-eosin-saffron staining revealed an induced membrane lining the cavity of all samples with dense vascularity. Decalcified, undecalcified and histomorphometric analysis showed new bone formation in the biomaterial and the autograft. Calcium uptake was higher inside the new cortical bone, notably at sites with HA-betaTCP. CT scans at 6 months showed that two patients had a favorable outcome with cortico-cancellous bone. Microscopic and immunochemical analysis confirmed the experimental data. This study demonstrates the feasibility of mandibular reconstruction using the induced membranes technique. This technique is efficient, and the results would be better in non-irradiated patients with good general health.

Mandibular metastases from an ileum stromal tumor.

INTRODUCTION: Metastatic disease of the jaws is unusual and accounts for 1 to 4% of oral cavity malignancies. Jaw metastases from the gastrointestinal (GI) tract usually evolve from adenocarcinoma of the esophagus, colon, and rectum. Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the GI tract defined by a positive C-Kit (CD117). These tumors are thought to arise from Cajal cells in GI tract walls, essential for intestine motor function. The small intestine harbors only 30% of GIST. After reviewing the literature, no case of jaw metastases from GIST was found. The purpose of this study was to report the first case of mandibular metastases arising from a stromal tumor of the ileum. CASE REPORT: A 68-year-old man presented with a painful swelling in the parasymphysis and left molar mandibular area having grown progressively for 3 weeks. The oral mucosa was macroscopically normal. The orthopantomograph showed radiolucency. A CT-Scan revealed an irregular osteolytic lesion with invasion of soft-tissues. Biopsy proved a stromal tumor. A complete CT-Scan analysis revealed an ileum tumor. Biopsies and immunochemistry proved an ileum stromal tumor. All tumoral cells expressed the C-Kit in the ileum and the mandible. The patient was treated with imatinib but died 11 months after the diagnosis. DISCUSSION: The prevalence of GIST is low but the true incidence may be higher because of under-diagnosis. To our knowledge, this is the first well-documented case report of jaw metastasis from ileum GIST. GIST should be included in the differential diagnosis of intramandibular tumor in patients with prior or current non-oral malignancy.

Use of gutta percha cores in CT scan imaging for patent nasopalatine duct.

Gutta percha cores were inserted in nasopalatine ducts to improve their visualization on CT scans. This simple method enhances the diagnostic quality of these images so that surgery can be performed more precisely.

[Lambert-Eaton Myasthenic Syndrome associated with vocal cord carcinoma]. / Syndrome myasthénique de Lambert-Eaton associé à un carcinome épidermoïde de la corde vocale.

INTRODUCTION: Lambert-Eaton Myasthenic Syndrome (LEMS) is an autoimmune channelopathy in which patients produce autoantibodies directed against voltage-gated calcium channels. LEMS is paraneoplastic in 50% of patients, most frequently associated with small cell lung carcinoma. We describe a case of paraneoplastic LEMS associated with a vocal cord carcinoma. OBSERVATION: A 64-year-old man developed in five months muscle weakness affecting gait. Clinical examination showed proximal muscular deficiency, areflexia and dysphonia. Electrophysiologic study showed potentiation greater than 500% after post exercise facilitation and 76 percent increment response at high-rate repetitive nerve stimulation (20Hz). Diagnosis of LEMS was confirmed by electrophysiologic study and anti-voltage gated calcium channel antibodies (90pM, positive value greater or equal to 70pM). Left vocal cord lesion histology showed epidermoid carcinoma. A combination of vocal cord tumor removal by endoscopy and treatment by pyridostigmine, 3-4 diaminopyridine and intravenous human immunoglobulin improved neurological symptoms. CONCLUSION: Paraneoplastic syndromes in association with cancers of the larynx and hypopharynx are unusual. Only two cases are reported with LEMS associated with larynx carcinoma. We describe an unusual case of LEMS associated with a left vocal cord carcinoma.

[Mouth floor and mobile tongue epidermoid carcinomas thickness: prognostic value]. / Epaisseur de l'infiltration tumorale des carcinomes épidermoïdes du plancher buccal et de la langue mobile : valeur pronostique.

INTRODUCTION: Most cancers of the oral cavity are epidermoid carcinomas. The prognosis is made on the patient's general health status and the tumoral stage. The UICC TNM staging classification system is one of the most important factors taken in consideration for the prognosis. But this classification in oral epidermoid carcinomas does not include the tumor thickness (except for T4 tumors). Several studies demonstrated that tumor thickness could influence the prognosis in epidermoid carcinoma and other types of cancers. The aim of our retrospective study was to assess the prognostic value of tumor thickness in oral epidermoid carcinoma. PATIENTS AND METHODS: The study included patients with mouth floor or mobile tongue epidermoid carcinoma classified T1N0, T2N0 and T3N0 between 1985 and 2005. All patients were treated with a curative intention. A pathologist analysed the tumor thickness and cervical lymph nodes. All the slides were examined to measure tumor thickness in millimetres. RESULTS: Three hundred and five patient files were reviewed and 124 patients were included, with 94 men (75.8%), and a mean age of 59.3 years (17-93). The mean and median tumor thickness were respectively 7.7 and 6.5 mm (0.4-30). The median tumor thickness was chosen for the study. There was a statistically significant link between the five-year probability of global survival and the initial tumor thickness and between neck node invasion and tumor thickness (p<0.05). DISCUSSION: This study suggests that tumor thickness should be taken in consideration in T1N0, T2N0 and T3N0 mouth floor and mobile tongue epidermoid carcinomas. In the future, the clinical evaluation of tumor thickness will help determine the therapeutic management.

Imaging of renal leiomyomas.

BACKGROUND: Renal leiomyomas are rare benign tumors of the kidney which can be found at autopsy as small capsular nodules in about 5% of cases. The clinical incidence of such lesions is much smaller, and only case reports or small series have been reported in the imaging literature. PURPOSE: To describe the imaging characteristics observed in a series of eight patients with pathology-proven asymptomatic leiomyomas of the kidney. MATERIAL AND METHODS: We reviewed the imaging findings observed in eight patients with pathologically proven asymptomatic renal leiomyomas discovered during studies performed for reasons unrelated to the kidney. All patients had undergone computed tomography (CT), two ultrasonography, and one magnetic resonance imaging (MRI). RESULTS: Lesions ranged in size from 1.2 to 13 cm. Six were at the periphery of the kidney, compressed its outer surface, but did not cause disruption of the cortex; two involved the renal cortex. All had regular outer margins. A cleavage plane between the tumor and the kidney was revealed at CT and/or ultrasonography in three of the cases located at the periphery. At ultrasonography, leiomyomas appeared hypoechogenic. At CT, they were slightly hyperdense before contrast medium injection; all were hypodense to the renal cortex after contrast medium. Four were homogeneous, two were slightly heterogeneous, and the remaining two were frankly heterogeneous. The lesion studied by MRI, which was homogeneous at the postcontrast CT study, had a heterogeneous structure on both T1- and T2-weighted images, with internal areas of hypointensity on T1. CONCLUSION: There are some imaging findings that can help to suggest the diagnosis of renal leiomyomas. First, their density: all tumors examined before contrast were hyperdense to the kidney, with density similar to that of muscles, and all had lower enhancement than the adjacent renal parenchyma. Second, the location and margins of the tumors: most were peripheral, without involvement of the renal cortex and with well-defined margins. Although not pathognomonic for a renal leiomyoma, the combination of these findings should include leiomyoma in the list of differential diagnoses.

[Immunohistochemistry expression of 3 markers (CEA, UEA-I and Ki-67) in nasal inverted papillomas]. / Expression immunohistochimique de l'ACE, de l'UEA-I et du Ki-67 dans les papillomes inversés naso-sinusiens.

OBJECTIVES: Immunohistochemistry evaluation of the expression of degeneration and proliferation markers of the benign form of Schneiderian inverted papillomas in the ORL sphere, in the nondysplastic, dysplastic and degenerated forms. Seeking out an expression profile with a prognostic value. MATERIALS AND METHOD: 44 surgical specimens were analyzed in two groups: A= 33 benign and B= 11 degenerated. Group A included: 10 dysplastic lesions, 4 septal lesions and 2 isolated sphenoidal lesions. A simultaneous bipolar localization belonged to the two groups (nasal, benign and otologic malignant). A control group consisted often biopsies of healthy mucous membranes harvested during septoplasties. The carcinoembryonic antigen (CEA), the ulex europaeus agglutinin I (UEA-I) and the monoclonal antibody Ki-67 were revealed by immunoreaction with peroxidase and enhanced by streptavidine-biotine. RESULTS: There was a significant difference in the marking of the CEA between the control group and groups A (p< 0.023) and B (p< 0.045). This expression was fulfilled only in the superficial layers in 84.1% of cases. There was no difference in expression between groups A and B, the dysplastic and non-dysplastic inverted papillomas and between the various degrees of dysplasias. There was no significant statistical difference in expression of UEA-I between all the groups and the sub-groups. The expression of Ki-67 was significantly increased in groups A (p< 0.00023) and B (p< 0.05) when compared to the control group. But, no difference existed between groups A and B, the various sub-groups and the benign specific localizations. This expression was present in the basal cells. An expression encompassing all the thickness of the epithelium was generally associated with a degenerated or dysplastic lesion, without being systematic. CONCLUSION: It was not possible using this specimen and for these three glycoproteins to extricate immunohistochemistry profiles of expression associated to the studied clinical and histological forms. However the expression of Ki-67 in more than 50% of the cells involving the full epithelium thickness would seem to suggest a particular cellular behavior Whereas the expression of the protein remains generally confined to the basal and suprabasal layers, a more significant population of Ki-67+ cells disseminated in the epithelium, would signify a tendency of the epithelium to escape the regulation mechanisms. This cellular behavior could constitute a prognostic histological marker but it would require a broader study to be confirmed.

[Multiple verruciform xanthomas of the oral mucosa associated with graft versus host disease]. / Xanthomes verruciformes multiples buccaux au cours d'une maladie du greffon contre l'hôte.

BACKGROUND: Verruciform xanthoma is an uncommon lesion that occurs primarily on the mucous membranes and more rarely on the skin. A few authors have reported the extremely rare occurrence of multiple lesions. Triggering or enhancing factors have been occasionally described such as an underlying immunosuppression or associated inflammatory mucous or skin diseases. We report, to the best of our knowledge, the first case report of multiple verruciform xanthomas of the oral mucosa in a patient with graft-versus-host disease with specific oral lesions. CASE REPORT: A 57 year-old man presented with an 8-year history of chronic myeloid leukemia. He was considered in complete remission for leukemia after allogenic bone marrow transplantation. Nevertheless, he was still treated with immunosuppressive drugs for oral and cutaneous lesions of chronic graft-versus-host disease. In this context, the patient presented two symmetric lesions of the gingiva. These lesions had progressed over several months. The clinical presentation was similar, with a yellowish and verrucous aspect and a sessile base. Histologic and immunohistochemical analysis led to the diagnosis of multiple verruciform xanthomas. DISCUSSION: The occurrence of multiple lesions of this rare tumour in our patient was probably not fortuitous. Immunosuppression associated with oral chronic inflammatory lesions are certainly involved in the pathogenesis of these two verruciform xanthomas, for example following degeneration of epithelial cells after local chronic irritation and/or reduction of Langerhans cells. Systematic research of enhancing or triggering factors seems essential in verruciform xanthoma.

[Lymphoma with initial renal involvement: four cases]. / Lymphomes à manifestations initiales rénales: à propos de quatre observations.

PURPOSE: To present a comprehensive description of the clinical features of patients with renal manifestations during lymphoma. METHODS: Retrospective review of medical records from all patients diagnosed with lymphoma associated with kidney involvement in our hospital between 1996 to 2004. Four cases were identified and analysed. RESULTS: Four patients presented a non-Hodgkin's lymphoma. One patient showed intravascular large B-cell lymphoma, revealed by proteinuria. Another patient had a nephrotic syndrome, and two had a renal mass. Renal histology allowed diagnosis of lymphoma in 3 cases. CONCLUSION: The diagnosis of lymphoma associated with renal involvement is rather difficult, and more specifically in case of intravascular large B-cell lymphoma, or even primary renal lymphoma. We present here a comprehensive review of the literature and we discuss pathogenesis of these conditions.

An unusual endocarditis-induced crescentic glomerulonephritis treated by plasmapheresis.

A 58-year-old man presented with fever and a rapidly progressive glomerulonephritis. An infective endocarditis due to Streptococcus parasanguis was diagnosed. A renal biopsy revealed type III pauci-immune crescentic glomerulonephritis. As first-line therapy, antibiotics were administered alone. Faced to the unsuccessful anti-infective approach, corticosteroid therapy was added as a second-line therapy. Finally, plasmapheresis introduced as the third-line therapy, significantly improved renal function. This case is an original type III rapidly progressive glomerulonephritis, since ANCA were repeatedly found negative. In very few cases, plasmapheresis was successfully used for the treatment of infective endocarditis-induced crescentic glomerulonephritis. The pathophysiology and the potential efficiency of plasmapheresis are discussed.

[Glomangioma or "glomus tumor" of the nasal cavity: apropos of a new case and review of the literature]. / Glomangiome ou "tumeur glomique" des cavités nasales: à propos d'un nouveau cas et revue de la littérature.

Glomangioma, or "glomic tumour" is a benign soft tissue tumour appearing most commonly at the distal extremities, in the nailbed and subcutaneous tissue. This is a vascular tumour, rare for the dermatologist, and exceptional in an ENT site. We describe here what is, as far as we can tell from the literature, the eighteenth case. The problem is the diagnosis of a vascular tumour, arising usually from the septal mucosa, which has to be distinguished histopathologically from a haemangiopericytoma. Radiological investigation will define the extent of the tumour and guide the treatment plan, which is purely surgical and consists of wide excision to avoid the possibility of a local recurrence.

Long-term (> or =20 yr) status of 14 cadaveric kidney-transplant recipients.

The aim of this study was to analyze the status of patients with a successful long-term (> or =20 yr) kidney graft. Nineteen (8.1%) of the 234 recipients who received a cadaveric kidney transplant between 1968 and 1978 in our center are still alive 21.7+/-1.6 yr (mean+/-standard error of the mean) later with a functioning allograft. Function, including measurement of the renal functional reserve (RFR), histological status, and morbidity were evaluated. Fourteen patients agreed to participate in this study. Their current immunosuppressive regimens combined prednisone (P)+azathioprine (AZA) (n=9), P+AZA+cyclosporine (CsA) (n=3) or P+CsA (n=2). Although they described their quality of life as good, 10 patients had mild hypertension, 5 developed 10 malignancies (9 cutaneous), 5 had replicative hepatitis, 8 had osteopenia, and 6 had cataracts, but none had diabetes mellitus. Proteinuria was detected in 6 patients, but was always less than 1 g/d. Mean serum creatinine was 1.28+/-0.28 mg/dL and glomerular filtration rate was 54.5+/-5.3 mL/min/1.73 m2. RFR was present for 4 patients with a mean value of +14.8+/-1.9 mL/min. Their functional status was not correlated with the histological lesions observed in concomitant transplant biopsies. Kidney grafts are able to function well even more than 20 yr post-transplantation, with some having a RFR whose significance remains unknown. Morbidity is of minor clinical severity, but could be further reduced with optimized management. Moreover, transplantation is much less costly than hemodialysis.

Nephrotoxic nephritis and obstructive nephropathy: evaluation with MR imaging enhanced with ultrasmall superparamagnetic iron oxide-preliminary findings in a rat model.

PURPOSE: To evaluate the role of magnetic resonance (MR) imaging enhanced with ultrasmall superparamagnetic iron oxide (USPIO) in the evaluation and differentiation of different types of nephropathies. MATERIALS AND METHODS: Two experimental rat models of nephropathies were studied: a model of nephrotoxic nephritis induced by means of intravenous injection of sheep anti-rat glomerular basement membrane serum (n = 43) and a model of obstructive nephropathy (n = 6). Imaging sessions were performed with a spectrometer operating at 4.7 T with fast low-angle shot, or FLASH, sequences. Signal intensity was measured in each kidney compartment before and 24 hours after intravenous injection of USPIO (90 micromol of iron per kilogram of body weight). MR findings were compared with histologic data and urine protein levels. RESULTS: In the nephrotoxic nephritis model 24 hours after injection of USPIO, a significant signal intensity decrease (P: <.05) was present only in the cortex where the glomerular lesions were located. In the obstructive nephropathy model, the signal intensity decrease (P: <.05) was located in all kidney compartments in response to diffuse interstitial lesions. The decrease in signal intensity was due to iron uptake by either macrophages or mesangial cells gaining endocytic activity and was correlated, in the nephrotoxic nephritis model, to the degree of proteinuria. CONCLUSION: Twenty-four-hour delayed USPIO-enhanced MR imaging may help identify and differentiate various types of nephropathies.

[Multiple medullary and extramedullary plasmacytomas in an HIV infected female patient]. / Plasmocytomes multiples médullaires et extramédullaires chez une patiente infectée par le VIH.

INTRODUCTION: Before the HIV infection era, plasmocyte tumor rarely occurred in patients younger than 40 years of age. Less frequent than lymphomas, the incidence of these blood diseases has however substantially increased in HIV-infected patients. In these patients, in addition to onset at earlier age, their clinical presentation is quite different and extramedullary plasmocytomas in unexpected locations are more common. EXEGESIS: We report the case of a 29-year-old HIV-infected female patient in whom were diagnosed occipital, parotidal, sphenoidal, epidural, and uterine plasmocytomas for which chemotherapy and subsequent radiotherapy were successful. The increase in the incidence of plasmocyte tumors in HIV-infected patients might be facilitated by Epstein Barr Virus (EBV) co-infection, HIV-related chronic antigenic stimulation, and secretion of interleukin 6 by infected lymphocytes. CONCLUSION: Plasmocyte tumors belong to neoplasia whose incidence is increased in HIV infection. Their currently poor diagnosis should be improved by highly active antiretroviral therapies allowing enhanced chemotherapy with possibility of autograft.