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C Langner

Publications and source records attributed to C Langner.

14 recordsLinked to original sources

[Histopathological diagnosis of Barrett's mucosa and associated neoplasias. Results of a consensus conference of the Working Group for "Gastroenterological Pathology of the German Society for Pathology" on 22 September 2001].

There are a number of difficulties regarding the diagnosis of Barrett's mucosa and the varying grades of neoplasia that may be associated with it. It was therefore the aim of a consensus conference of the "Working Group for Gastroenterological Pathology within the German Society of Pathology" to achieve standardization regarding the following issues: definition and diagnostic criteria for Barrett's mucosa and its discrimination from intestinal metaplasia of the cardia, diagnostic criteria for intraepithelial neoplasia, number of biopsies necessary to establish the diagnosis, significance of additional immunohistochemical and/or molecular biological methods as well as importance of a second opinion in the diagnosis of intraepithelial neoplasia.

Barrett Esophagus↗

[Hemangiosarcoma of the liver with unusual expression of neuron-specific enolase (NSE)].

A 68-year-old male patient presented with unspecific decline of general health, multiple tumours in the liver discovered by ultrasound examination and a focus suggestive of tumour on chest x-ray. He was hospitalised because of suspected metastatic lung carcinoma. Laboratory analysis revealed a distinct elevation of serum neuron-specific enolase (NSE, 26,1 micro g/l). A CT scan of the abdomen confirmed the presence of multiple tumour foci in both hepatic lobes, while the lung, however, was found to be free of tumour on CT-examination. Bronchoscopy, gastroduodenoscopy and colonoscopy were unremarkable. Subsequently, an ultrasound guided fine-needle biopsy of the liver was performed. Histological examination disclosed hemangiosarcoma with unusual, though unequivocal expression of NSE in tumour cells. As even follow-up examinations were unable to trace out any extrahepatic primary tumour, the hemangiosarcoma was concluded to be of hepatic origin. The patient succumbed to his disease 6 months later, an autopsy was not performed. This case report of a NSE-producing primary hemangiosarcoma of the liver clearly outlines that a histological confirmation of an allegedly clear clinical diagnosis should always be performed because malignant tumours are capable of (co-)expressing so called tumour markers independently of their histogenesis.

Aged↗

[Pigmented pheochromocytoma. Case report with immunohistochemical and electron microscopic characterization].

We present a case of pigmented adrenal paraganglioma in a 39-year-old female patient with associated neurofibromatosis type 1 (NF1). Histology showed features typical for phaeochromocytomas except for varying amounts of brown pigment within the cytoplasm of tumour cells, which proved to be melanin by histochemical and ultrastructural analysis. The occurrence of melanin is believed to reflect the origin of this neoplasm from multipotent cells of the neural crest. Pigmented phaeochromocytoma has to be taken in consideration in the differential diagnosis of pigmented neoplasms, especially in the adrenal gland, where it has to be discriminated from pigmented cortical adenoma (so-called black adenoma) and primary malignant melanoma.

Adrenal Gland Neoplasms↗

[Acute segmental hemorrhagic antibiotic-associated colitis].

Three cases of acute segmental hemorrhagic antibiotic-associated colitis are described occurring in three male adults between 30 and 33 years of age after treatment with oral ampicillin or amoxicillin because of upper respiratory tract infection, tonsillitis, or HLO eradication therapy, respectively. All presented with cramping abdominal pain and bloody diarrhoea of acute onset, however, microbial analysis of fecal samples was negative. Endoscopy showed right-sided segmental hemorrhagic colitis. Histopathological examination demonstrated edema, patchy superficial hemorrhage and a scattered predominantly mononuclear infiltrate of the lamina propria. The surface epithelium was partly desquamated and displayed foci of grouped intraepithelial red blood cells. All patients spontaneously recovered after discontinuation of antimicrobial therapy. The value and limitations of diagnostic features are discussed with respect of the literature.

Abdominal Pain↗

[Chronic cholangitis].

The term chronic cholangitis comprises a heterogeneous group of intra- and/or extrahepatic diseases that may either be bacterially caused or immunologically mediated. In this review, we will focus on the latter and present the clinicopathological and pathogenetic aspects of primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC). The histopathological criteria are defined and differential diagnosis is discussed. Particular emphasis is given to overlap syndromes of PBC and PSC with autoimmune hepatitis. Finally, a diagnostic algorithm is presented.

Bile Ducts, Intrahepatic↗

[Concomitant occurrence of angiomyolipoma, focal nodular hyperplasia, bile duct adenoma, and cavernous hemangioma in the liver].

The simultaneous occurrence of different hepatic tumors is rare. We present for the first time a concomitant manifestation of an angiomyolipoma, a focal nodular hyperplasia, a bile duct adenoma, and a cavernous hemangioma in a 63-year-old female patient. The largest of the tumors, preoperatively suspected to be hepatocellular carcinoma, was an angiomyolipoma with monotypic epithelioid histology and positive immunoreactivity for HMB-45, actin, desmin, and pancytokeratin. The significance of immunohistochemistry for the differential diagnosis of hepatic neoplasms is emphasized. Finally, a review of the literature with special regard to etiology and pathogenesis of neoplastic liver disease is given, leading to the assumption that the association of four different benign intrahepatic tumors is rather more fortuitous than pathogenetically related, despite the putative similar pathogenesis of focal nodular hyperplasia and hemangioma.

Adenoma, Bile Duct↗

[Diffuse hemangiomatosis of the liver and spleen in an adult].

Diffuse hemangiomatoses are extremely rare in adults. The etiology and natural history of the disease are not well understood. A case of diffuse hemangiomatosis of the liver and spleen associated with progressive liver failure, thrombocytopenia, and disturbance of blood coagulation (comparable to Kasabach-Merritt syndrome) is presented in a 62-year-old male. We describe the histopathological and immunohistochemical findings and illustrate the morphological aspects of differential diagnosis, distinguishing the disease from other vascular proliferations.

Biopsy↗

Piffalls in diagnostic molecular pathology--significance of sampling error.

Today, molecular diagnostic tests are widely used in clinical medicine with polymerase chain reaction (PCR)-based techniques being of particular interest. In tissue specimens, however, false-positive and false-negative results can be obtained if pathomorphological and processing aspects are not considered. We therefore studied the impact of tissue sampling in three widely used diagnostic tests: (1) assessment of clonality in B-cell non-Hodgkin's lymphoma, (2) analysis of microsatellite instability (MSI) in colorectal neoplasia, and (3) demonstration of mycobacterium tuberculosis. Tissue sections of routinely formalin-fixed and paraffin-embedded diagnostic specimens were taken, and the significance of sampling was systematically investigated using laser microdissection or processing of the entire section. PCR analyses were done according to standard protocols. False-positive pseudo-monoclonality was obtained in small gastrointestinal biopsies and in laser microdissected lymph follicles of non-neoplastic tonsils. False negativity (pseudo-stability) could be demonstrated in a case with hereditary rectal adenoma if whole tissue sections were taken without microdissection of the most dysplastic subareas. Demonstration of mycobacteria failed in tissue sections of a formalin-fixed lymph node that was positive after complete digestion or in fresh frozen material of the same patient. In diagnostic molecular pathology, sampling error is an important source of false-positive and false-negative results, particularly if disease- and tissue-specific morphological features, such as sample size, type of fixation, and intralesional heterogeneity, are ignored.

Clone Cells↗

Linear atrophoderma of Moulin.

We report a typical case of linear atrophoderma of Moulin that represents a distinct clinical entity. A 17-year-old woman presented with hyperpigmented and atrophic band-like skin lesions measuring 3-5 cm in breadth on the right side of her trunk and on the right buttock, in an arrangement following the system of Blaschko's lines. The skin lesions had a normal texture and showed no signs of inflammation, lilac ring, erythema, induration, sclerosis or depigmentation. Routine laboratory data were normal. Antinuclear antibodies and anti-Scl70-antibodies were negative. Histopathologically, a moderate diffuse hyperpigmentation within the lower epidermis, a focal vacuolar degeneration of the basal layer, and a few dermal perivascular lymphocytes accompanied by signs of pigment incontinence were noted. Intravenous penicillin G was administered for 14 days in a dosage of 10 x 10(6) IU twice daily. This treatment was repeated after 3 and 9 months. No effect was noted. Linear atrophoderma of Moulin takes a chronic course without progression or regression. The disease leads to significant cosmetic impairment and this may cause emotional stress. Due to its relationship to idiopathic atrophoderma of Pierini-Pasini and linear scleroderma, treatment with intravenous penicillin was tried. In our case it had no effect.

Adolescent↗

[Squamous cell carcinoma developing from epidermodysplasia verruciformis].

We report on a 50-year-old man who presented with squamous cell carcinoma of the forehead skin developing from epidermodysplasia verruciformis (EV). EV is an uncommon disease characterized by flat warts or scaling macules resembling pityriasis vericolor on sun-exposed skin. Histogenetic predisposition and human papilloma virus infection play a part. As shown in our case there is, depending on HPV subtype and additional exposure to ultraviolet light, a high risk of malignant transformation. These patients require close monitoring.

Carcinoma, Squamous Cell↗

[Restrictive dermopathy].

Restrictive dermopathy is a rare, fatal, autosomal recessive, congenital skin disease. Rigidity of translucent thin skin, which is thus highly vulnerable and tears, spontaneously causes intra-uterine fetal akinesia or hypokinesia deformation sequence (FADS), characteristic dysmorphic facies with fixed open mouth in O position, and generalized joint contractures (arthrogryposis). Polyhydramnios and pulmonary hypoplasia are distinctive manifestations, leading to respiratory insufficiency and premature delivery at about 31 weeks of gestation. We report on a case of a prematurely born infant who presented with the typical morphological features and describe the light- and electron-microscopical findings as described in the literature.

Abnormalities, Multiple↗

[Correlation between histological and molecular mechanisms of carcinogenesis in stomach cancer].

Since gastric cancer is an exceptional heterogeneous tumor conflicting results have been obtained about the relationship between genotype and phenotype. From the molecular point of view gastric carcinoma diffuse type forms a distinct entity which is microsatellite stable, has almost no p53 mutations and exhibits in at least half of the cases mutations in the E-cadherin gene. In contrast, all other gastric carcinomas comprise a heterogeneous group of which about one third exhibits microsatellite instability (MSI) but no p53 protein stabilization or gene mutations. These tumors are either of pure intestinal (glandular) type or show large solid (medullary) tumor cell clusters. Thereby, in sporadic gastric cancer MSI is caused by loss of hMLH1 expression due to hypermethylation of the promotor region rather than by mutation of the gene itself. Tumors that are microsatellite stable (MSS) and show p53 alterations are either intestinal (about 70%) or a mixed-type encompassing at least 5% glandular and poorly differentiated diffuse components (about 30%). Whereas pure diffuse type gastric cancer is unlikely to develop from intestinal type carcinoma, this may, however, be the case in some advanced mixed-type gastric cancers.

Base Pair Mismatch↗