[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.

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.

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.

[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.

[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.

[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.

Polyneuropathy in Waldenström's macroglobulinemia. Deposition of M component on myelin sheaths.

A chronic sensory neuropathy was the initial symptom in a case of Waldenström's macroglobulinemia in a 53-year-old woman. Ultrastructural analysis of a biopsied peripheral nerve revealed a modification of the lamellar structure of the myelin sheath. Immunofluorescent microscopy showed IgM-positive deposits on the myelin sheath. The monoclonal globulin (M component) present in the myelin appears to be a causative factor in the neuropathy.

Waldenström's macroglobulinemia and peripheral neuropathy: deposition of M-component and kappa light chain in the endoneurium.

Some cases of peripheral neuropathy associated with benign IgM monoclonal gammopathy, or Waldenström's macroglobulinemia, are probably of autoimmune origin; in some cases, anti-IgM serum reacts with the myelin sheaths of peripheral nerves. However, mechanisms may differ in other cases. In one case of neuropathy with macroglobulinemia, we found deposits of IgM immunoglobulin in the endoneurium. On ultrastructural examination, the deposits had erased the basement membrane of some nerve fibers that showed damage of both myelin and axon.

Renal angiomyolipoma: a clinicopathologic, immunohistochemical, and follow-up study of 46 cases.

We reviewed 46 cases of renal angiomyolipoma covering the period from 1977 to 1997. Eight cases were associated with tuberous sclerosis and one with lymphangiomyomatosis. Histologically, the lesions were most often classic with the three usual components, i.e., mature adipose tissue, thick-walled blood vessels, and smooth muscle. Seven cases were particularly misleading: three cases were entirely adipose mimicking liposarcoma: two cases had an exclusively smooth-muscle component, one mimicking lymphangiomyomatosis and one with epithelioid cells; another case had a monophasic epithelioid pleomorphic component ("REON": renal epithelioid oxyphilic neoplasm) and proved to be fatal; and another case was associated with collecting duct carcinoma. The immunohistochemical profile showed the coexpression of alpha-smooth-muscle actin and HMB45. Our study is the first to show positivity of estrogen and progesteron receptors or both in more than 25% of cases. Of 35 cases with follow-up information, only one patient died of malignant spread of angiomyolipoma.

Thymoma associated with myasthenia gravis and minimal lesion nephrotic syndrome.

A nephrotic syndrome has been observed rarely in association with thymoma. In most of the reported cases, it occurs when the thymoma is in remission; histological examination generally shows minimal change disease. We report a case of a 43-year-old man presenting with minimal lesion nephrotic syndrome at the time of the diagnosis of thymoma and myasthenia gravis, which persists despite remission of the thymoma. The role of a disorder of T-cell function and of circulating cytokines is discussed.

Use of a biodegradable elastin-fibrin material, Neuroplast, as a dural substitute.

Since the new Neuroplast biomaterial is now commonly used in several domains of restorative and curative surgery, its preliminary evaluation as a dura mater substitute in the rabbit was performed and is described herein. Whereas the importance of prosthesis thickness was implicated and solved (0.2 rather than 1.0 mm), both macroscopic examination and histological studies revealed for the neotissue an effective patency, a good continuity and especially no or almost no adherence at all. As Neuroplast is now available for use everywhere in humans, this material is being developed in several fields of neurosurgery.

New artificial connective matrix made of fibrin monomers, elastin peptides and type I + III collagens: structural study, biocompatibility and use as tympanic membranes in rabbit.

Experiments leading to the conception and development of a new artificial connective matrix based on collagen-elastin-fibrin associations are presented. Preliminary evaluation of this biomaterial as an artificial tympanic membrane is described. Morphological evaluation was performed by histopathological and scanning electron microscopic studies. Biocompatibility was documented by subcutaneous implantation and experimental grafting in rabbits was performed with histopathological studies of the tympanic grafted membranes. The positive results obtained in this experimental study enable future studies in humans to be prepared.

USPIO-enhanced MR imaging of glycerol-induced acute renal failure in the rabbit.

Enhanced-MR imaging in combination with ultrasmall superparamagnetic iron oxide (USPIO) was used in the glycerol-induced model of acute renal failure (ARF) in the rabbit to detect renal perfusion abnormalities. A control group (n = 5) and an ARF group (n = 5) were studied after intramuscular injection of glycerol (10 ml/kg) with T2-weighted spin-echo sequence at 1.5 T and a 27 mumol/kg IV dose of iron. The signal intensity (SI) was quantified in the cortex, the outer medulla (OM), and the inner medulla (IM). In control rabbits, the maximum SI decrease after USPIO injection was in the OM (76% +/- 3.6), as this is the region of maximal vascular density, then in the IM (73.4% +/- 2.9). In the glycerol group, SI loss in the OM (61% +/- 12.6) and the IM (45.2% +/- 16.24) was significant less than in the control group (p < .05). Pathology results showed fibrinous thrombus in the efferent arterioles and congestive aspect of the vasa recta in the medulla. We argue that a reduced medullary concentration of USPIO in the renal failure group is indicative of medullary hypoperfusion.

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.

Guillain-Barré syndrome: an ultrastructural study of peripheral nerve in 65 patients.

65 biopsies of peripheral nerve from patients suffering from Guillain-Barré syndrome were studied by electron microscopy. In 48 cases there was macrophagic invasion of the Schwann cells of certain myelinated fibers, and in 32 of these cases some myelin sheaths were stripped away by an elongated macrophagic process. Vesicular disruption of the myelin sheath was observed in only 8 cases and in less than 1% of the myelinated fibers. Uncompacted myelin lamellae were observed in a few myelinated fibers. These ultrastructural lesions are analysed and commented on with a view to selecting patients who are to undergo plasma exchange.

Vallecular acinic cell carcinoma in a 9-year-old girl: report of an unusual case.

An unusual case of acinic cell tumour of the vallecula is presented. Acinic cell carcinoma occurs usually in the major salivary glands. Minor salivary gland location is unusual and vallecular origin exceptional. This peculiar histologic tumour should now be classified as an low grade carcinoma and adequate treatment has to be initiated. The patient, a 9-year-old girl, had undergone a suprahyoid access for total tumor removal with a bilateral neck exploration. Postsurgical radiotherapy has to be done in case with perineural invasion, invaded margins, node invasion or high grade tumor. The clinical and histopathological findings are discussed in the light of the literature.