Xanthogranulomatous cholecystitis: Diagnosis and management.

Xanthogranulomatous cholecystitis (XGC) is a rare form of cholecystitis, characterized by the presence of xanthogranuloma, prominent yellow structures within the gallbladder wall that is very often lithiasic. When XGC presents in its pseudo-tumoral form with occasional adjacent organ involvement, it can mimic gallbladder carcinoma (GBC). The etiopathogenesis of XGC is inflammatory destruction of Rokitansky-Aschoff sinuses containing biliary and cholesterol pigments within the gallbladder wall; this leads to a florid granulomatous histiocytic inflammatory reaction. The prevalence ranges from 1.3% to 8.8% of all cholecystectomies and varies from country to country; XGC occurs predominantly in patients over 50 years of age, and is equally distributed between males and females. Its association with GBC remains a topic of debate in the literature (between 0 and 20%). Symptoms are non-specific and generally similar to those of acute or chronic cholecystitis. XGC, when associated with altered health status, leads to the suspicion of GBC. XGC can also come to light due to an acute complication of cholecystolithiasis, in particular, gallstone migration. Imaging by sonography and CT scan is suggestive, but magnetic resonance imaging is more specific. In difficult cases, biopsy may be necessary to eliminate the diagnosis of tumor. In case of pre- or intra-operative diagnostic doubt, the opinion of a hepatobiliary specialty center can be of help. When diagnosis of GBC has been eliminated, laparoscopic cholecystectomy is recommended, although with a high risk of conversion to laparotomy and complications.

Low-Phospholipid Associated Cholelithiasis (LPAC) syndrome: A synthetic review.

Low-Phospholipid Associated Cholelithiasis (LPAC) is a genetic disease responsible for the development of intrahepatic lithiasis. It is associated with a mutation of the ABCB4 gene which codes for protein MDR3, a biliary carrier. As a nosological entity, it is defined by presence of two of the three following criteria: age less than 40 years at onset of biliary symptoms, recurrence of biliary symptoms after cholecystectomy, and intrahepatic hyperechogenic foci detected by ultrasound. While the majority of clinical forms are simple, there also exist complicated forms, involving extended intrahepatic lithiasis and its consequences: lithiasis migration, acute cholangitis, intrahepatic abscess. Chronic evolution can lead to secondary sclerosing cholangitis or secondary biliary cirrhosis. In unusual cases, degeneration into cholangiocarcinoma may occur. Treatment is built around ursodeoxycholic acid, which yields dissolution of biliary calculi. Complicated forms may call for interventional, radiological, endoscopic or surgical treatment. This synthetic review illustrates and summarizes the different aspects of this entity, from simple gallbladder lithiasis to cholangiocarcinoma, as well as secondary biliary cirrhosis requiring liver transplant, on the basis of clinical cases and the iconography of patients treated in our ward.

Acute fatal pulmonary embolism during cyanoacrylate injection in gastric varices.

We report a case of massive pulmonary embolism during cyanoacrylate glue endoscopic injection in a patient with gastric varices from portal hypertension. A review of the literature and results in an animal model show the physiopathology and risk factors associated with this endoscopic procedure.

Large hepatocellular carcinoma: Does fibrosis really impact prognosis after resection?

BACKGROUND: Hepatectomy remains the standard treatment for large hepatocellular carcinoma (LHCC) ≥5cm. Fibrosis may constitute a contraindication for resection because of high risk of post-hepatectomy liver failure, but its impact on patient outcome and cancer recurrence remains ill defined. Our aim was to compare predictors of survival in patients with and without cirrhosis following hepatectomy for LHCC. METHODS: The data on consecutive patients undergoing hepatectomy for LHCC in two tertiary centres between 2012 and 2016 were reviewed. The outcomes of cirrhotic (F4) and non-cirrhotic (F0-F3) patients were compared. Patients with perioperative medical (sorafenib) or radiological (transarterial chemoembolization, radiofrequency) treatments were excluded. RESULTS: Sixty patients were included. Preoperative and intraoperative features were identical between both groups. Cirrhotics (n=15) presented more satellite nodules on specimens (73% vs. 44%; P=0.073) but better differentiated lesions than non-cirrhotics (P=0.041). The median overall survival of cirrhotics was 34 vs. 29months for non-cirrhotics (P=0.8), and their disease-free survival was 14 versus 18 months (P=0.9). Fibrosis stage did not impact overall (P=0.2) nor disease-free survivals (P=0.6). CONCLUSION: Hepatectomy for LHCC in cirrhotics can achieve acceptable oncological results when compared to non-cirrhotic patients. Curative resection of LHCC should be attempted if liver function is acceptable, whatever the fibrosis stage.

[Capillary hemangioma of the spleen on conventional and contrast-enhanced US]. / Hémangiome capillaire splénique en échographie conventionnelle et avec produit de contraste.

We report a case of splenic vascular neoplasm in a 75 year old asymptomatic woman. CT and enhanced sonography were not consistent with a typical hemangioma. Splenectomy was finally realised and pathologic exam showed a capillary hemangioma with thrombosis. The imaging appearance of splenic hemangiomas may be complex because of splenic topography, size and complicating features. The differentiation of these lesions from malignant disease may not be possible.

Mutagenesis of ras proto-oncogenes in rat liver tumors induced by vinyl chloride.

Vinyl chloride is a DNA-damaging carcinogen which induces liver angiosarcomas in humans and animals. Activation of the Ki-ras 2 gene by a GC-->AT transition at the second base of codon 13 in human liver angiosarcomas associated with occupational exposure to vinyl chloride has been reported recently. In order to compare the molecular pathways of carcinogenesis in humans and animals, Sprague-Dawley rats were exposed to vinyl chloride and hepatic tumors, including two hepatocellular carcinomas and five liver angiosarcomas, were investigated for mutations at codons 12, 13 and 61 of the Ha-ras, Ki-ras and N-ras genes. High molecular weight DNA was amplified by the polymerase chain reaction and point mutations were analyzed by allele specific oligonucleotide hybridization, direct sequencing of polymerase chain reaction products and sequencing after cloning. None of the tumors exhibited a mutation in codons 12, 13 and 61 of the Ki-ras gene, nor in codons 12 of the Ha-ras gene or 61 of the N-ras gene. However, an activating AT-->TA transversion at base 2 of codon 61 of the Ha-ras gene was detected in the two hepatocellular carcinomas. Mutations involving codon 13 (GGC-->GAC) and codon 36 (ATA-->CTA) of the N-ras A gene were detected in two liver angiosarcomas, suggesting that the nature of the ras gene affected by a given carcinogen depends on host factors specific to cell types. Several additional base pair substitutions were found in exon 1 of the N-ras B and C sequences. NIH 3T3 transfection assays and Southern blot analysis of DNA from transformed NIH 3T3 cells confirmed the presence of a dominant activated N-ras gene. These results emphasize the differences in the molecular pathways leading to tumors in humans and rats and within a given species between different cell types.

Inducible nitric oxide synthase, anti-oxidant enzymes and Helicobacter pylori infection in gastritis and gastric precancerous lesions in humans.

Chronic inflammation induced by Helicobacter pylori infection has been associated with an increased risk of stomach cancer. We have analysed 167 stomach biopsies from 99 patients for H. pylori infection and immunohistochemically for the expression of inducible nitric oxide synthase (iNOS), catalase and superoxide dismutases (SODs) as markers of oxidative stress. Biopsies were graded as follows on the basis of histology: normal, superficial gastritis, variable severity of atrophic gastritis with or without intestinal metaplasia, and dysplasia. iNOS was detected in inflammatory cells in all types of gastritis with or without H. pylori infection and independently of its severity. In foveolar cells, iNOS was observed in approximately 25% of all biopsies showing any type of gastritis, but in a markedly higher proportion of dysplastic samples. Catalase and Mn-type SOD in inflammatory cells and catalase in foveolar cells were more frequently observed in marked atrophic gastritis biopsies than in less severe gastritis. Individual differences were found in the expression of these enzymes within groups with the same severity of gastritis. Prolonged oxidative stress in severe gastritis and dysplasia may play an important role in gastric carcinogenesis, through increased damage of DNA and tissue by reactive oxygen and nitrogen species.

P.O.E.M.S. syndrome with complete recovery after treatment of a solitary plasmocytoma.

The P.O.E.M.S. syndrome is a rare clinical entity that has been described mainly in Japan. It is characterized by a progressive polyneuropathy with raised CSF protein content, organomegaly, endocrinopathy, skin changes and plasma cell dyscrasia. We report a new documented case associated with a solitary plasmocytoma of the 12th thoracic vertebra. Immunopathological and ultrastructural studies of the peripheral nerve did not disclose any immune-specific changes. Surgery and radiation therapy of the plasmocytoma allowed a complete recovery, with a 5-year follow-up. This case report illustrates the need for serial full skeletal survey, including scintigraphy, in middle-aged patients with progressive polyneuropathy of obscure origin.

[Intra-spinal ganglioglioma. A case and review of the literature]. / Le gangliogliome intra-médullaire. Une observation, revue de la littérature.

A case of intra medullary ganglioglioma is reported in a 25 year old woman, who had presented an acute poliomyelitis when she was 2 years old. Since 7 years, new neurological signs occurred with spastic paraplegia, sensitive and sphincter disturbances. Cervical and lumbar myelography showed an intra medullary tumor, extending from C2-C3 to T7-T8. Macroscopically, the lesion was well circumscribed, except on the cervico-dorsal junction where it engulfed the anterior spinal artery. Histologically, the tumor was a ganglioglioma, grade I. Two years after surgery, the patient remains paraplegic. This case is compared with the 13 other cases of the literature.

[Hepatic epithelioid hemangioendothelioma. A case with liver transplantation. Review of the literature]. / Hémangioendothéliome épithélioïde hépatique. Un cas avec transplantation hépatique. Revue de la littérature.

Epithelioid hemangioendothelioma (HEE) is an unusual malignant neoplasm of vasculo-endothelial origin, arising in soft tissues, lung and liver. One case of hepatic HEE is reported in a 32 year-old man wit an isolated multinodular hepatomegaly. The diagnosis was made by the histologic examination of biopsy specimens. At the time of diagnosis, there was evidence of pulmonary metastasis. However, an orthotopic liver transplantation was performed. The tumor cells were immunoreactive with factor VIII-related antigen, BNH9 and vimentin. There was no expression of cytokeratin and epithelial membrane antigen. The patient is alive and well 24 months after. The thoracic X-ray are unchanged. This case is compared with the 84 others cases of the literature. Fourteen experienced a favorable outcome after transplantation. Extra-hepatic dissemination doesn't contra-indicate the graft. But hilar vascular involvement may be correlated with worse prognosis.

[Lumbar osteomyelitis of actinomycotic or atypical mycobacterial origin?]. / Ostéomyélite lombaire actinomycosique ou mycobactérienne atypique?

A case of lumbar epiduro-vertebral actinomycosis is related, in a 45 years old man, who was immuno depressed by an evolutive pulmonary sarcoidosis. 72 similar cases are reported in the literature. 50 of them are autopsied++. Clinically our patient looked like a Pott disease. The infection began probably in the kidney. The smear's diagnosis was actinomycosis, confirmed by the routine examination. An atypical mycobacteria, Mycobacterium xenopi was bacteriologically isolated twice in the pus. It was considered as the pathogen agent for 14 months. Actinomycosis was never identified bacteriologically. The patient responded to surgery and penicillin therapy. In view of the pathologic constatations and the therapeutic response, the final diagnostic was epiduro-vertebral actinomycosis. Mycobacterium xenopi acted probably as an "accompaning " germ in this case.

[Cystic lymphangioma of the mediastinum. 2 personal cases and a review of the literature]. / Lymphangiomes kystiques du médiastin. 2 cas personnels, revue de la littérature.

Two mediastinal lymphangiomas (KLM) are described, in 36 and 41 year-old women. They presented as an incidental finding on chest radiograph. The mediastinal mass was anterior or posterior, compressing the adjacent vital structures, and cystic on computed tomography. The complete surgical removal was possible, but complicated by the size (12 x 7 x 4 cm and 10 x 7 x 5 cm), the hypervascularization and the infiltrative tendency of the lesions. The patients are well 36 and 30 months after removal. We compare these cases with 109 previously reported. They account for 1 p. 100 of the whole lymphangiomas and for 0.5 to 4.5% of all mediastinal tumours. The congenital or infantile cervico-mediastinal lymphangiomas are more frequent (10%). The general signs, symptoms, pathological findings, treatment and pathogenetic theories are discussed.

[Immunohistochemistry of thyroglobulin in the diagnosis of clear cell carcinomas of the cervical region]. / Immunohistochimie de la thyroglobuline dans le diagnostic des cancers à cellules claires de la région cervicale.

6 thyroid gland and 3 cervical lymph node clear-cells carcinomas are investigated by means of indirect immunofluorescence detection of thyroglobulin. Immunofluorescence was positive only in 1 case, which was a primitive thyroid carcinoma. In 7 other cases, its negativity pointed at the metastatic nature of the lesion, from a clear cell carcinoma of kidney (in 4 cases, nephrectomy was very remote, and in 3 cases, the renal lesion was discovered after our examination of the thyroid tumour). One observation showed negative staining reaction, and was a propagation of a parathyroid carcinoma to the thyroid gland. It is concluded that thyroglobulin can be used as an immunohistochemical marker to establish whether a clear cell cervical tumour originates from thyroid gland, or is a metastatic neoplasm.

[A case of aggressive juvenile fibromatosis of the mandible. Review of the literature]. / Un cas de fibromatose juvénile agressive de la mandibule. Revue de la littérature.

We report a new case of aggressive juvenile fibromatosis (A.J.F.) in a 20-month-old girl. The lesion affected the inferior border of the left mandible and the adjacent soft tissues. The child presented with a painless mass, which had grown over a period of 2 months. Radiographs and computed tomographic scan showed a multilacunar bone defect with subcutaneous and gingival involvement. A biopsy was performed, followed by a partial hemimandibulectomy and a costal graft. The surgical specimen measured 4 X 3 X 3 cm. The patient did well 1 year after surgery. 16 cases of A.J.F. have been reported; with ours, 14 are mandibular. A.J.F. is a locally aggressive lesion, which doesn't metastasize. It occurs chiefly in childhood and adolescence from 1 1/2 to 18 years (median 6.5 year-old). Duration of symptoms prior to presentation varies from a few weeks to months. Clinically, it is a firm nodule. Radiographs are non-specific, but 9 cases have poorly defined destruction of the mandibular inferior border. Following a block resection of the tumor (13 cases), there is no recurrence. In this review, we discuss the clinico-pathologic diagnosis of this impressive tumor which is compared with other mandibular fibrous tumors in children.

[Histogenesis of subependymal glioma in Bourneville's tuberous sclerosis]. / Contribution à l'étude de l'histogenèse des gliomes sous-épendymaires de la sclérose tubéreuse de Bourneville.

The phenotypic characteristics of 7 subependymal giant cell astrocytomas (GSECG) (6 of these being associated with tuberous sclerosis) are studied using morphological and immunohistochemical methods with antiserums against vimentine, glial fibrillary acid protein (GFA), S100 protein, and neurofilaments. The glycoproteic secretion of the tumor cells was also analyzed after exposure to Concanavalin A (CON A) by a direct fluorescent method. Our results suggest that some GSECG originate from specialized ependyma (circum-ventricular organs). They have the same location (foramen of Monro), present some common ultrastructural features (cytoplasm with lumen containing cilia), are positive with certain immunohistochemical markers (staining with S100 protein in 4 cases, with vimentin in 3 cases) and show a strong glycoproteic secretion (positive with CON A). Therefore, some GSECG might be considered hamartomas of specialized ependyma, with a reduced evolutivity potential.

[Immunohistochemistry of thyroglobulin by indirect immunofluorescence in thyroid cancers]. / Immunohistochimie de la thyroglobuline par immunofluorescence indirecte appliquée aux cancers thyroïdiens.

The authors have developed an indirect immunofluorescence technique for histochemical detection of thyroglobulin and have tested it on 66 tissue sections. Fluorescence reflecting the presence of thyroglobulin was elicited in 57 samples of differentiated thyroid tissue, including healthy or hyperfunctional tissue and primary or metastatic papillary or follicular carcinomas. Thyroglobulin was found to be distributed heterogeneously between different areas and different cells. It may occupy the whole cell, the apex, the colloid substance and sometimes extracellular spaces. No fluorescence was present in non-thyroid tissues. This technique could be applied mainly to the diagnosis of thyroid carcinomas, where 25 metastases have been examined by this method, and to clear cell carcinomas. In these two cases, thyroglobulin appeared to be a good marker of tumoral tissue.

Grading of small hepatocellular carcinomas (≤2 cm): correlation between histology, T2 and diffusion-weighted imaging.

OBJECTIVE: To evaluate the capacity of diffusion-weighted imaging (DWI) to determine the histological grade of small-sized hepatocellular carcinomas (HCCs) in liver cirrhosis in comparison with T2 weighted imaging. METHODS: 51 cirrhotic patients with 63 histologically proven HCCs ≤2 cm underwent abdominal MRI, including DWI (b-values 50, 400 and 800 s mm(-2)) and T2 weighted sequences. HCCs were classified into well-differentiated HCCs (n = 37) and moderately differentiated HCCs (n = 26). Relative contrast ratios (RCRs) between the lesions and the surrounding liver were performed and compared between the two groups for T2 weighted images, each b-value and apparent diffusion coefficients (ADCs). A receiver operating characteristic (ROC) analysis was performed to compare RCRs in T2 and diffusion-weighted images. RESULTS: We found significant differences in RCRs between well-differentiated vs moderately differentiated HCCs for b = 50, 400 and 800 s mm(-2) and T2 weighted images (1.35 ± 0.36 vs 1.86 ± 0.62; 1.35 ± 0.38 vs 1.82 ± 0.60; 1.27 ± 0.30 vs 1.74 ± 0.53; 1.14 ± 0.18 vs 1.43 ± 0.28, respectively; p < 0.001), whereas no significant differences were observed in ADC and ADC RCR (1.05 ± 0.19 vs 0.99 ± 0.15 and 1.1 ± 0.22 vs 1.09 ± 0.23; p = 0.16 and p = 0.82, respectively). No significant difference was found in the areas under the ROC curve for RCRs of T2 weighted images and every DWI b-value (p = 0.18). CONCLUSION: The RCR measurement performed in DWI 50, 400 and 800 b-values and T2 demonstrated a significant difference between well-differentiated and moderately differentiated small-sized HCCs. Furthermore, no difference was shown by using either ADC or ADC RCR. ADVANCES IN KNOWLEDGE: DWI with RCR measurement may be a valuable tool for non-invasively predicting the histological grade of small HCCs.

[Microscopic extensions of head and neck squamous cell carcinomas: impact for clinical target volume definition]. / Approche anatomopathologique de l'extension microscopique des carcinomes épidermoïdes ORL : implications pour la définition du volume cible anatomoclinique.

PURPOSE: To assess microscopic extensions of head and neck squamous cell carcinomas aiming at a proposal for target volumes of radiation therapy. MATERIALS AND METHODS: Surgical specimens were prospectively analysed macroscopically and microscopically. Tumour borders were identified per macroscopic visual examination and inked on stained slides. Then microscopic implants (perineural or lymphatic involvement, or in situ carcinomas) were looked for with an optic microscope in the macroscopic healthy tissue surrounding the tumour. The maximal length from tumour border was correlated with the maximal length of macroscopically healthy tissues assessable. RESULTS: Twenty-one specimens were analysed and 12 were locally advanced tumours. Mean and median maximal microscopic extensions were 2.9 and 1.0mm (0-15mm), respectively. The 90th and 95th percentiles were 5 and 11mm, respectively. The ratio between healthy tissue length and maximal microscopic tumour extension was 10%. No correlation was found with tumour grade or volume. CONCLUSION: The presence of microscopic tumour was unlikely after 5mm from macroscopic tumour (≤5% of patients in this series) but should be assessed along with other histoclinical factors and particularities of tumour behaviour by anatomic site. A rigorous terminology should authorize a relevant appreciation of local risk of recurrence, particularly in adjuvant setting or for clinical target volume definition. Larger and more homogenous confirmatory series are needed.