[A suspected carcinoma finding in the uterus]. / Karzinomverdächtiger Herdbefund im Uterus.

Epithelioid tumor cells of a uterine perivascular epithelioid cell tumor (PEComa) may mimic carcinoma cells in an endometrial sampling (pitfall). Immunohistochemistry (HMB45 positive, keratin negative) helps in the differential diagnosis.

[Tumor-suspected focal lesion on the renal hilus]. / Tumorverdächtiger Herdbefund am Nierenhilus.

Mucosal structures of a non-neoplastic organ can result in a pitfall diagnosis of adenocarcinoma in the case of a wrong correlation with other organs, in this case caused by an adherent gallbladder to the hilar structures of the right kidney. Clinical and radiological data are absolutely crucial for a correct classification.

[Intravascular papillary endothelial hyperplasia (IPEH) : Masson's pseudoangiosarcoma on the forefoot]. / Intravaskuläre papilläre endotheliale Hyperplasie (IPEH) : Masson-Pseudoangiosarkom am Vorfuß.

Intravascular papillary endothelial hyperplasia (IPEH)/Masson's pseudoangiosarcoma is a rare (orphan) disease of the forefoot that has not yet been described in Europe. The pathoanatomic examination of a young patient with a vague interdigital space-consuming lesion revealed a intravascular papillary endothelial hyperplasia/Masson's pseudoangiosarcoma. The intravascular papillary endothelial hyperplasia can be mainly detected in the upper limbs as well as cervically. Heretofore, the intravascular papillary endothelial hyperplasia has only been described in Japan and the USA. For Europe, our case report is the first description of the clinical course of IPEH.

[Tumor of the peroneal compartment]. / Tumor der Peronäusloge.

Degenerative nuclear atypia in mesenchymal neoplasia, especially in benign nerve sheath tumors, may become a pitfall leading to a wrong diagnosis of a sarcoma. Using a case of degenerative (ancient) schwannoma as an example, the characteristic findings of degenerative atypia are presented and discussed.

[Gastric ulcer suspected of being a carcinoma]. / Karzinomverdächtiges Magenulkus.

Following hepatic penetration by a gastric ulcer a pitfall diagnosis of gastric adenocarcinoma can result from a biopsy of the ulcer base. In cases of suspicious "hepatoid" epithelial cells in a gastric biopsy, the possibility of regular liver tissue from a gastric ulcer penetrating the liver should be kept in mind and verified, e.g. by immunohistochemistry.

[Cystic desmoplastic small round cell tumor: tumor development from cystic-"mesothelioblastic" areas?]. / Zystischer desmoplastischer klein- und rundzelliger Tumor: Tumorentwicklung aus zystisch-"mesothelioblastischen" Arealen?

A 7-cm cystic lesion in the upper left abdomen and additional smaller solid tumor nodules were diagnosed incidentally in a 15-year-old boy without tumor symptoms. The main tumorous cystic lesion showed a flattened single-cell tumor cell component in gradual transition to stratified, papillary and truly "invasive" typical desmoplastic areas of a desmoplastic small round-cell tumor (DSRCT). The Ki-67-proliferation index gradually increased within three histologic tumor patterns up to about 70% in the typical desmoplastic (infiltrating) component. Using microdissection techniques, EWS-WT1-gene fusion transcripts were detected in the cystic (single-cell-layered), the papillary and the solid tumor proliferations (exon 7 of EWS on chromosome 22 with exon 8 of WT1 on chromosome 11). The presented case illustrates a predominant cystic growth pattern of DSRCT, in which a stepwise development in the pathogenesis of DSRCT from cystic (-"mesothelioblastic") towards a more papillary proliferation and finally typical "infiltrative" desmoplastic tumor pattern might be discussed. The cystic pattern could represent an initial stage in the development of the neoplasia. The presence of specific EWS-WT1-gene fusion transcripts in all tumor growth patterns in this respect would indicate an early event in t(11;22)(p13;q12) translocation in the pathogenesis of DSRCT.

[Lipoma and atypical lipomatous tumor within the same neoplasia: Evidence for a continuous transition]. / Lipom und atypischer lipomatöser Tumor innerhalb einer Neoplasie: Hinweis für einen kontinuierlichen Ubergang.

The case of a lipomatous tumor with a predominant lipoma component and transition to an atypical lipomatous tumor is presented. A deep-seated soft tissue tumor of the right thigh with a maximum size of 14 cm was resected in a 70-year-old female patient. Corresponding to a comparable macroscopic aspect, the lesion revealed the histological features of an ordinary lipoma without atypia in about 80% of the specimen. In the remaining portion (approximately 20%) histopathology showed an atypical lipomatous tumor (ALT, lipoma-like subtype). Immunohistochemistry for MDM 2 and CDK4 revealed no immunoreactivity in the lipoma component, but within the ALT component. Interphase dual-color fluorescence in situ hybridization showed no amplification of the MDM 2 gene and rarely CDK4 gene amplification within the lipoma component, but high level amplification of MDM 2/CDK4 gene in the ALT area, further supporting the morphologically based diagnosis of a lipomatous tumor including areas of a true lipoma and ALT. This case underlines the concept of a continuous stepwise development of lipomatous soft tissue tumors from benign to malignant counterparts as a biological continuum.

[Atypical lipomatous tumors of the stomach. Clinical, morphological and molecular findings]. / Atypische lipomatöse Tumoren des Magens. Klinische, morphologische und molekularbiologische Befunde.

Two cases of primary gastric atypical lipomatous tumors (ALT) are presented. In case No.1, a 7x4x3 cm submucosal ALT (lipoma-like subtype) of the antrum/pyloric region in a 60-year-old woman was completely resected. Using interphase dual-color-FISH, MDM2- and CDK4 amplifications could be detected in distinguished amplicons. The patient was continuously free of disease after 56 months. In case No. 2, a 3.5 cm (in diameter) submucosal ALT (lipoma-like subtype) of the gastric body in a 56-year-old woman was completely resected. FISH revealed MDM2 amplification while the CDK4 gene remained in diploid copies. This patient was continuously free of disease after 36 months. The morphologic and molecular biological findings of this rare primary gastric mesenchymal tumor are discussed in comparison with the corresponding soft tissue lesions.

[Identification of genes over-expressed in myxoid/round cell liposarcoma. DNA microarray analysis and immunohistochemical correlation]. / Identifikation überexprimierter Gene in myxoid-rundzelligen Liposarkomen. DNA-Mikroarray-Analyse und immunhistochemische Korrelation.

Myxoid/round cell liposarcoma are characterized by typical chromosomal translocations. This genetic alteration might result in specific gene-expression profiles in this tumor entity. To identify over-expressed genes in myxoid/round cell liposarcoma DNA microarray analysis was performed on four tumors and four samples of adult fat tissue. Genes ret, cdk4, cyclin D2 and c-myc showed over-expression by means of microarray analysis and Northern blotting. Immunohistochemistry demonstrated cytoplasmic localization of associated proteins in 36 different tumors. The localization of ret was seen in endothelial cells of plexiform vasculature in addition to its accumulation in tumor cells (25% of cases). The results show an over-expression of cdk4, cyclin D2, c-myc and ret on both the transcriptional and protein level in myxoid/round cell liposarcoma. For cyclin D2 and ret this finding has not been reported in this tumor type. The increase of ret on transcriptional level might be explained by its expression in endothelium in intratumoral plexiform blood vessels. For the molecular pathogenesis of myxoid/round cell liposarcoma our findings imply the involvement of these four genes in the deregulation of the cell cycle, especially as cdk4 and cyclin D2 are target genes of c-myc.

[Gardner fibroma: case report and discussion of a new soft tissue tumor entity]. / Gardner-Fibrom: Eine neue mesenchymale Tumorentität als Indikatorläsion.

Gardner fibroma represents a rare and recently described soft tissue tumor entity in children and young adults. It consists of haphazardly arranged coarse and hyalinized collagen fibers combined with loosely arranged bland spindle and fibroblastic cells. The case of a 13-year-old male patient with Gardner fibroma and osteoma and multicentric desmoid type fibromatosis in his mother is presented with detection of a (heterozygotic) germline mutation of the APC gene leading to a de novo stop codon (deletion of base pairs 5033-5036). FISH analysis revealed a structural loss of heterozygosity (LOH) in the APC gene on chromosomal locus 5q21 in one out of five analysed desmoids of the mother, no LOH of APC gene in the Gardner fibroma. Gardner fibroma in children and young adults may serve as an indicator lesion for familial adenomatous polyposis (FAP), Gardner syndrome, a familial desmoid type fibromatosis without other manifestations of APC or a new APC gene mutation. For the clinician, this diagnosis should be commented upon accordingly by the surgical pathologist. As the result of a detected APC gene mutation, continuous follow-up for the development of colorectal tumors and desmoid type fibromatosis as well as a familial screening for FAP is recommended.

Lymph node metastases in soft tissue sarcomas: a single center analysis of 1,597 patients.

PURPOSE: The aim of this study was to examine the clinical course of patients with the rare finding of regional lymph node metastasis (RLNM) from soft tissue sarcoma. MATERIALS AND METHODS: Data from 28 out of 1,597 consecutive soft tissue sarcoma patients with RLNM were from the patients' charts and interviewing patients and general practitioners. Survival, including possible influencing factors, was statistically calculated. RESULTS: RLNM was seen in 21.4% for epithelioid sarcoma and 17.6% for clear cell sarcoma. All other entities presented RLNM rates below 10%. At follow-up after an average of 9.6 years, only three patients were alive with no evidence of disease. Survival was independent from surgical resection status of the primary tumor and the RLNM as well as from adjuvant radiation and chemotherapy. Tumor entity as well as the length of the time period from primary to RLNM affect survival. CONCLUSIONS: Surgical treatment as well as radiation and chemotherapy may improve survival in selected cases but probably have their value much more in terms of local disease control and improvement life quality of patients who probably already suffer from an aggressive systemic disease at time of nodal involvement.

Metachronous bilateral soft tissue sarcoma of the extremities.

BACKGROUND AND AIMS: Synchronous and heterochronous multiple soft tissue sarcoma of the extremities is very rare. Out of 1,201 of our patients, 4 patients presented with symmetrical bilateral soft tissue sarcoma of the extremities. The aim of this study was to identify possible reasons for this unusual manifestation of sarcomas. MATERIALS AND METHODS: The patients' data was acquired by review of the patients' charts and follow-up information was gathered by phone calls to the patients or their relatives and their general practitioners. RESULTS: All tumours were located at the extremities and were diagnosed as leiomyosarcoma in two patients, malignant fibrous histiocytoma and clear cell sarcoma in one patient each. No other individual or family history of cumulation of neoplasms was known in the patients. The median interval between the diagnoses was 3 1/2 years (range: 4 months to 9 1/2 years). In two patients a second primary sarcoma of the same entity was considered the most likely diagnosis, whereas in one patient a contralateral lymph node metastasis and in one other patient an atypical soft tissue metastasis had to be taken into account. A positive family history with a father with malignant fibrous histiocytoma may indicate a hereditary predisposition in one patient. Aside from irradiation effects, exposition to other carcinogenic agents or genetic predisposition, the reasons for the clustering of soft tissue sarcoma in one same patient remain still unclear. Only one patient, although suffering from disseminated metastatic disease was living at follow-up time, the other three patients had already died. CONCLUSION: The interpretation of the bilateral manifestation of soft tissue sarcoma remains open, but predicts an unfavourable outcome.

[Hospitals failing minimum volumes in 2004: reasons and consequences]. / Unterschreitungen der Mindestmengen 2004: Begründungen und Konsequenzen.

BACKGROUND: In 2004 Germany introduced annual minimum volumes nationwide on five surgical procedures: kidney, liver, stem cell transplantation, complex oesophageal, and pancreatic interventions. Hospitals that fail to reach the minimum volumes are no longer allowed to perform the respective procedures unless they raise one of eight legally accepted exceptions. The goal of our study was to investigate how many hospitals fell short of the minimum volumes in 2004, whether and how this was justified, and whether hospitals that failed the requirements experienced any consequences. METHOD: We analysed data on meeting the minimum volume requirements in 2004 that all German hospitals were obliged to publish as part of their biannual structured quality reports. We performed telephone interviews: a) with all hospitals not achieving the minimum volumes for complex oesophageal, and pancreatic interventions, and b) with the national umbrella organisations of all German sickness funds. RESULTS: In 2004, one quarter of all German acute care hospitals (N=485) performed 23,128 procedures where minimum volumes applied. 197 hospitals (41%) did not meet at least one of the minimum volumes. These hospitals performed N=715 procedures (3.1%) where the minimum volumes were not met. In 43% of these cases the hospitals raised legally accepted exceptions. In 33% of the cases the hospitals argued using reasons that were not legally acknowledged. 69% of those hospitals that failed to achieve the minimum volumes for complex oesophageal and pancreatic interventions did not experience any consequences from the sickness funds. However, one third of those hospitals reported that the sickness funds addressed the issue and partially announced consequences for the future. The sickness funds' umbrella organisations stated that there were only sparse activities related to the minimum volumes and that neither uniform registrations nor uniform proceedings in case of infringements of the standards had been agreed upon. DISCUSSION: In spite of the high number of hospitals that failed to achieve the minimum volumes in 2004, only few hospitals experienced consequences from the sickness funds. The reluctance of the payers may be explained, amongst others, by the small number of patients affected and the percentage of cases where legally accepted exceptions applied. In view of the partly unclear definitions of the exceptions and difficulties in the interpretation and execution of the minimum volumes in the hospitals and at the sickness fund level, it may be helpful to formulate more concrete instructions for the implementation of the standards.

[Low-dose CT-guided transthoracic lung biopsy for evaluation of non-infectious chronic interstitial lung disease in children]. / Die Niedrigdosis-CT navigierte transthorakale Lungenbiopsie zur Evaluation chronisch-interstitieller, nicht-infektiöser Lungenerkrankungen bei Kindern.

BACKGROUND: Children with interstitial pneumonitis (IP) of unknown origin often have to undergo open lung biopsy to establish a final diagnosis. Open lung biopsy is an invasive procedure with major potential complications. In the meantime, CT-guided transthoracic lung biopsy (TLB) has become a common diagnostic procedure in adults. OBJECTIVE: The aim of this study was to retrospectively evaluate the efficacy and radiation exposure of low-dose CT-guided TLB in children with non-infectious IP of unknown origin. METHODS: Twelve children (7-males, age range: 7 months-15 years) with non-infectious IP of unknown origin and inconclusive clinical tests underwent CT-guided TLB with a 20-gauge biopsy instrument. A low-dose protocol with acquisition of single slices was used on a 16-row CT scanner: 80 kVp, 20 mAs, slice thickness 10 mm. Biopsy specimens were processed by standard histopathological and immunohistochemical techniques and effective doses were individually calculated. RESULTS: All biopsies were performed without major complications. Two children (17 %) developed a small pneumothorax/pulmonary haemorrhage that resolved spontaneously. A final diagnosis could be established in 9/12 patients (75 %) by CT-guided TLB. In 2 patients (17 %) the results of TLB were inconclusive; however, the clinical suspicion could be disproved. Open lung biopsy was performed in 1 patient (8 %), which demonstrated idiopathic pulmonary fibrosis. On average, the effective dose of CT-guided TLB was 0.78 mSv (0.4 - 1.1 mSv). CONCLUSION: Low-dose CT-guided TLB can be a helpful method for investigating children with non-infectious IP of unknown origin thus making open lung biopsy unnecessary. Application of a low-dose protocol leads to a significant reduction of radiation exposure in CT-guided TLB.

Analysis of biodegradation of copolymer dermis substitutes in the dorsal skinfold chamber of balb/c mice.

PEGT/PBT-block-copolymer dermis substitutes were inserted into dorsal skinfold chambers of balb/c mice (n=36). Scaffolding matrices with 3 different pore diameters (pore diameter: <75 micro m, 75-212 micro m and 250-300 micro m) were analyzed on days 7, 14, and 21 post implantation by scanning electron and light microscopy. The quantification of matrix fragmentation was performed using image-analytical software analySIS(R). The fragmentation rate in scaffolding matrices with a pore size of < 75 micro m was observed to be higher than in matrices of larger pore sizes. Image-analytical evaluation over 21 days revealed a reduction of the copolymer matrix by approximately 32% for the <75 micro m matrices, 23% for the 75-212 micro m matrices and 18% for the matrices, where pore size ranged between 250 micro m and 300 micro m. Twenty-one days after implantation, the matrix pores of 75-212 micro m and 250-300 micro m scaffolds were totally filled by vascularized fibrous tissue. Contrarily, an increased formation of foreign-body giant cells was observed in matrices with pore size <75 micro m. The pore size of the scaffolding PEGT/PBT dermis substitutes affects their degradative behaviour in vivo.

[Desmoplastic neurotropic melanoma. Diagnosis and therapeutic procedures for a rare clinical entity]. / Spindelzelliges desmoplastisches Melanom. Ein seltenes Krankheitsbild--Diagnostik und therapeutisches Vorgehen.

Desmoplastic neurotropic melanoma is an exceedingly rare variant of the malignant melanomas. Since the initial description, only about 500 cases have been reported in the literature. These melanomas mostly occur on the head, neck, and face, and a wide local excision of the tumor is probably the most important prognostic factor. Therefore, in most cases the tumor resections result in large tissue defects. To gain a good cosmetic result, tissue movement using flaps or grafts will most likely be required to cover the defect. In this paper, a 79-year-old patient with a basal cell carcinoma and desmoplastic melanoma on the scalp is described, and the therapeutic procedure is discussed.

Malignant Glomus Tumour (Glomangiosarcoma) with Additional Neuroendocrine Differentiation in a Horse.

A 13-year-old Icelandic crossbred horse was presented with headshaking and progressive impairment of chewing. A slowly growing mass was identified in the anterior maxilla. This was associated with lysis of the alveolar bone and the roots of the incisors and there were nodular proliferations affecting the nasal septum and conchae. There was no response to chemotherapy and so the horse was humanely destroyed. Based on morphological, immunohistochemical and ultrastructural findings the mass was classified as a malignant glomus tumour with multifocal vascular spaces and additional neuroendocrine differentiation. An oronasal glomus tumour with neuroendocrine differentiation has not been described previously in an animal.