Short- and mid-term effects of irreversible electroporation on normal renal tissue: an animal model.

PURPOSE: Irreversible electroporation (IRE) is a novel nonthermal tissue ablation technique by high current application leading to apoptosis without affecting extracellular matrix. Previous results of renal IRE shall be supplemented by functional MRI and differentiated histological analysis of renal parenchyma in a chronic treatment setting. METHODS: Three swine were treated with two to three multifocal percutaneous IRE of the right kidney. MRI was performed before, 30 min (immediate-term), 7 days (short-term), and 28 days (mid-term) after IRE. A statistical analysis of the lesion surrounded renal parenchyma intensities was made to analyze functional differences depending on renal part, side and posttreatment time. Histological follow-up of cortex and medulla was performed after 28 days. RESULTS: A total of eight ablations were created. MRI showed no collateral damage of surrounded tissue. The highest visual contrast between lesions and normal parenchyma was obtained by T2-HR-SPIR-TSE-w sequence of DCE-MRI. Ablation zones showed inhomogeneous necroses with small perifocal edema in the short-term and sharp delimitable scars in the mid-term. MRI showed no significant differences between adjoined renal parenchyma around ablations and parenchyma of untreated kidney. Histological analysis demonstrated complete destruction of cortical glomeruli and tubules, while collecting ducts, renal calyxes, and pelvis of medulla were preserved. Adjoined kidney parenchyma around IRE lesions showed no qualitative differences to normal parenchyma of untreated kidney. CONCLUSIONS: This porcine IRE study reveals a multifocal renal ablation, while protecting surrounded renal parenchyma and collecting system over a mid-term period. That offers prevention of renal function ablating centrally located or multifocal renal masses.

Male infertility as a component of Carney complex.

Carney complex (CNC) is a multiple neoplasia syndrome characterised by endocrine tumours, spotty skin pigmentation, cardiac and other myxomas, psamommatous and pigmented schwannomas, large cell calcifying Sertoli cell tumours, and mammary ductal adenomas and other more rare lesions. CNC is inherited in an autosomal-dominant manner and has been mapped to at least two chromosomal loci. Patients who map to the CNC1 locus located on chromosome 17 carry inactivating mutations of the PRKAR1A gene that encodes the cAMP-dependent protein kinase regulatory subunit type 1-alpha (Kirschner et al., 2000). One gene responsible for type 2 (CNC2) is located on chromosome 2p16. Infertility in CNC can be caused by a number of factors; there is evidence that prkar1a deficiency in mice leads directly to infertility (Burton et al., 2006), but patients with CNC also have Sertoli cell tumours and a number of other reasons to affect fertility. We report on an infertile male with CNC and present evidence that male infertility should be considered as part of the phenotype of CNC.

Extended radical lymphadenectomy in patients with urothelial bladder cancer: results of a prospective multicenter study.

PURPOSE: Previous studies demonstrate a positive correlation between postoperative survival and the extent of pelvic lymphadenectomies in patients with bladder cancer. However, the distribution of nodal metastases has not been examined in sufficient detail. Therefore, we conducted a comprehensive prospective analysis of lymph node metastases to obtain precise knowledge about the pattern of lymphatic tumor spread. MATERIALS AND METHODS: Between 1999 and 2002 we performed 290 radical cystectomies and extended lymphadenectomies. Cranial border of the lymphadenectomy was the level of the inferior mesenteric artery, lateral border was the genitofemoral nerve and caudal border was the pelvic floor. We made every effort to excise and examine microscopically all lymph nodes from 12 well-defined anatomical locations. RESULTS: Mean total number and standard deviation of lymph nodes removed was 43.1 +/- 16.1. Nodal metastases were present in 27.9% of patients. The percentage of metastases at different sites ranged from 14.1% (right obturator nodes) to 2.9% (right paracaval nodes above the aortic bifurcation). By studying cases of unilateral primary tumors or with only 1 metastasis we observed a preferred pattern of metastatic spread. However, there were many exceptions to the rule and we did not identify a well-defined sentinel lymph node. CONCLUSIONS: We strongly recommend extended radical lymphadenectomy to all patients undergoing radical cystectomy for bladder cancer to remove all metastatic tumor deposits completely. The operation can be conducted in routine clinical practice and our data may serve as a guideline for future standardization and quality control of the procedure.

Primary sarcoma of the inferior vena cava: review of diagnosis, treatment, and outcomes in a case series.

Leiomyosarcoma of the inferior vena cava is a rare mesenchymal tumor. The diagnostic approach, based on general guidelines of oncologic surgery, seems to be relatively routine; specific aspects of treatment, including vascular reconstruction, depend on tumor stage, grade, and location. In this report, the management of this disease in 5 patients is summarized and the literature is reviewed. A thorough diagnostic assessment includes sonography, computed tomography, angiography or duplex ultrasonography, perioperative pathohistologic examination, and appropriate differential diagnosis. Radical resection is associated with the best outcome and long-term survival. In this series, 4 of 5 patients underwent tumor resection. In 2 patients, the disease was classified as R0. Another patient had R1 status found at resection and underwent postoperative radiation after the tumor bed was marked intraoperatively. She has remained stable since treatment. One patient died of pulmonary metastases 32 months after primary R1 tumor resection. The 5th patient has been stable since diagnosis; resection was not possible because of severe accompanying diseases and because consent for surgical intervention could not be obtained from the patient. There is reasonable hope that leiomyosarcoma of the inferior vena cava can be treated successfully, even in advanced stages, with novel antineoplastic drugs and radiotherapeutic protocols. However, general treatment recommendations have not yet been compiled.

[Multiple metachronous metastases of renal cell carcinoma in the pancreas. Case report and review of the literature]. / Multiple metachrone Metastasen eines Nierenzellcarcinoms im Pankreas. Fallbeschreibung und Literaturübersicht.

Isolated pancreatic metastases from renal-cell carcinomas (RCC) are extremely rare. Only 96 cases of clinically diagnosed renal-cell carcinoma metastatic to the pancreas have been reported in the world literature, and 70 of the patients (including ours) underwent a definitive surgical resection. In many cases the time between the nephrectomy and diagnosis of metachronous metastases is reported to exceed 10 years. Therefore, the initial diagnosis may be overlooked when there is a prolonged disease-free interval. When it does occur simultaneously or metachronously, aggressive surgical resection, when possible, seems to be the most effective treatment for this metastatic lesion. Surgical resection can provide long-term survival in selected cases. We present the case of a 69-year-old woman in whom two pancreatic metastases were treated by a left-sided subtotal pancreatectomy with splenectomy, 12 years after radical nephrectomy for a RCC. The patient simultaneously presented with both a mass in the body of the pancreas and a right-sided colon cancer. Thus, the diagnosis of pancreatic metastasis of colon cancer was suspected initially. Both tumors were radically resected, and histological examination revealed two pancreatic metastases from the previous RCC. In the world literature this report represents the eighth case of multiple pancreatic metastases due to RCC. It illustrates a rare indication of pancreatic resection because of pancreatic metastasis. The need for prolonged follow-up of patients is emphasized. The few reports on surgically treated pancreatic metastases from RCC are reviewed after the presentation of this case.

[Lipoma-like liposarcoma of the tongue]. / Lipomähnliches Liposarkom der Zunge.

BACKGROUND: Liposarcomas of the tongue are rare. To date only 7 cases have been reported. PATIENT: We report a new case of a well differentiated lipoma-like liposarcoma of the body of the tongue in a 71-year old woman. Complete uneventful resection was performed. Histologically the tumor consisted of fat cells with occasional lipoblasts and spindle cell areas. There is no sign of recurrence of the tumor 2 years later. RESULTS AND CONCLUSIONS: Liposarcomas of the tongue are rare neoplasms. All cases reported were histologically well differentiated. Liposarcomas have to be considered in the differential diagnosis of solid masses of the tongue.

Hepatic angiomyolipoma in a 26-year-old Caucasian woman with a history of tibial osteosarcoma.

We report on a 26-year-old Caucasian woman who was referred to the Department of Surgery complaining of general malaise, feeling of fullness with occasional vomiting and intermittent jaundice. The patient had previously suffered from tibial osteosarcoma of the left leg which was resected 13 years ago and subsequently treated with radiation and chemotherapy. During clinical investigations a 12 x 12 x 6.5 cm large mass was found in the left lobe of the liver. This was resected, and subsequently shown to be a sporadic hepatic angiomyolipoma. In order to investigate a possible link between the two tumours, we investigated mutations in the p53-gene, loss of heterozygosity (LOH) at p53, Rb and p16, c-Myc expression, and the telomerase activity of the angiomyolipoma and the osteosarcoma. Whilst the tibial osteosarcoma showed LOH at p16, no genetic alterations or increased telomerase activity were found in the angiomyolipoma. The occurrence of both these tumours in this patient is therefore probably a coincidence.

Alveolar soft part sarcoma of the uterine corpus. Report of two cases and review of the literature.

Alveolar soft-part sarcoma (ASPS) is a rare tumor of uncertain histogenesis, mainly localized in the extremities and less frequently found in the head, neck and trunk. The present report describes two cases of ASPS localized in the uterus. In general, this entity is very rarely encountered in the female genital tract. Including the two cases presented here, 27 patients have been described in the literature. Whereas the prognosis for ASPS in soft tissues is usually poor (most of the patients died of lung metastases), those localized in the female genital tract are associated with a much better survival. This could be explained by the fact that their diameter seldom exceeds 5 cm, the size which is regarded as being the critical prognostic limit. Histologically, both tumors showed the same characteristics as known for ASPS in other localizations: organoid or nest-like arrangement of tumor cells; rounded or polygonal cells with cytoplasm of varying density; thin-walled, sinusoidal vascular spaces between tumor cell nests. Immunohistochemically, we found positivity for desmin and, to a lesser extent, for vimentin. Cytokeratin was negative, which is important in differential diagnosis to other rare uterine neoplasias like clear cell (mesonephroid) adenocarcinomas or metastases.

Bellini duct carcinoma of the kidney--a case report.

Bellini duct carcinoma (BDC), originating from the principal cells of the collecting duct of the kidney, accounts for only 1% of renal carcinomas. This lesion is a histogenetically, morphologically and cytogenetically defined entity. We report an eosinophilic variant of BDC. A 71-year old male presented with a tumor in the right kidney. Nephrectomy was performed. BDC (stage pT3a N0 M0 G2, based on UICC classification) was diagnosed. Electron microscopy revealed an abundance of mitochondria. Intranuclear rodlets were seen. Neuroendocrine differentiation was not demonstrated. After a three-year observation period, there are no signs of recurrence or metastases.

Extra-abdominal aggressive fibromatosis after treatment of a Morbus Hodgkin. A case report.

The proliferations of the connective tissue, which are summed up under the term fibromatoses, assume a special position with regard to their biologic behavior. While superficial fibromatoses are more likely to grow slowly, musculoaponeurotic fibromatoses (Desmoid tumor)--like malignant tumors--show a locally bound aggressive, rapid growth behavior. A metastazation, however, can never be proved. The research on etiology of fibromatoses has yielded only little knowledge. Besides genetic and hormonal factors, physical reasons are under discussion, too. The present study aims to add another description of a case report to the small number of case presentations on radiotherapy-associated fibromatosis. In 1977, a now 67-old-patient was irradiated because of a Morbus Hodgkin lymphoma in the left side of his neck. Prior to the radiation, the patient had undergone several surgeries. 17 years after the radiation, the patient developed an aggressive fibromatosis (prelaryngeal, right) in the formerly irradiated region. As the localization of the fibromatosis does not allow us to establish a connection to his numerous operations preceding the fibromatosis, a radiation-associated Desmoid tumor must be assumed. Immunohistologically, no estrogen receptors were detectable.

[Functional and morphologic results of pedicled and microvascular anastomosis in tissue transfer]. / Funktionelle und morphologische Resultate nach gestieltem und mikrovaskulär anastomosiertem Gewebetransfer.

UNLABELLED: From 1982 to 1992, large intraoral and pharyngeal soft-tissue defects were reconstructed using myocutaneous island flaps in 95 patients, free jejunal interpositions in 10 patients and forearm flaps in 18 patients at the Magdeburg School of Medicine, Department and Clinic of Otorhinolaryngology. Sixty-five patients were followed up after various time periods in order to assess the functional results. Investigation and evaluation of the individual patients' resultant quality of life were based on a specifically prepared examination questionnaire. In particular, the evaluation included: the mouth opening gap, motility of the tongue, sufficient masticatory area, adequate provision to fit artificial teeth, malocclusion, the extent of mandibular deviation, assessment of the swallowing act, articulation disturbances, paraesthesia, function accessory nerve, atrophy of the trapezius muscle, motility of the cervical spine, flexibility in the shoulder girdle, as well as subjective information obtained from the patients (pain, feeling of dryness, increased salivation, occupational rehabilitation, organisation of leisure time). In 45 patients, biopsy material from the transferred tissue was examined at varied time intervals after primary therapy. IN CONCLUSION: The functional results obtained for the myocutaneous island flaps, the forearm flap and the free jejunal interposition were satisfactory, and only minor differences were noted. From the morphological point of view, all the three types of tissue transfer were found to have adapted adequately to the recipient site.

[Value of computer tomography and lymphography for staging of malignant testicular tumors]. / Zur Wertigkeit von Computertomographie und Lymphographie für die Stadieneinteilung maligner Hodentumoren.

Findings of computer tomographic and lymphographic examinations of 74 patients were evaluated concerning their evidence in the pretherapeutic staging compared with the histological results of lymphonodectomy. The security of the computer tomography was about 82.4%, the specifity about 95.12%, the sensitivity about 66.66%. Lymphographically, a security of 85.14%, a specifity of 95.12% and a sensitivity of 66.66% was achieved. The combination of the methods achieved a security of 91.7%, a specifity of 100% and a sensitivity of 71.72% was achieved. Metastases of lymphatic nodes less than 0.5 cm diameter were not recognized by means of the two methods. In 2 cases no metastases, which were not covered by means of computer tomography, could not be recognized lymphographically in slightly enlarged lymphatic nodes. In contrast to this in 11 out of 74 patients (14.8%) in the computer tomography an enlargement of the findings was the result in comparison to lymphography. Since none of the two methods is absolutely reliable for the proof or the exclusion of smaller metastases, in a secure evidence of the computer tomography before a lymphonodectomy the lymphography can be omitted.

[Importance of post-therapeutic computer tomography follow-up studies of patients with malignant testicular tumors]. / Wertigkeit posttherapeutischer computertomographischer Verlaufsuntersuchungen bei Patienten mit malignen Hodentumoren.

120 patients with malignant tumours of the testicles were subjected to follow-up studies for up to 7 years after the completion of therapy. In 27 cases (22%) metastases were found, which appeared almost exclusively within the first 3 years after treatment. There was definite evidence of metastasis in 9 out of 46 cases (19%) of seminoma and with 18 out of 74 nonseminomatous tumours.

[Results of computer tomographic and lymphographic diagnosis of malignant testicular tumors]. / Ergebnisse computertomographischer und lymphographischer Diagnostik bei malignen Hodentumoren.

89.5% accuracy was achieved using the pre-therapeutic combination of CT scanning and lymphography to diagnose the spread of malign testicular tumours in 28 cases. In 14% of the patients CT scanning was able to increase the diagnostic information. CT scanning should follow lymphography. The use of CT scanning to check on progress proved useful in 42 cases, both after lymphonodectomy and after irradiation therapy. In 7 cases (17%) the CT examination revealed metastasis.

[Leukemia of a still-born child (author's transl)]. / Leukose bei einem Totgeborenen

A case of a stemcell-leukemia of a mature male macerated still-born child is described. The mother, a 20-year-old healthy bipara, a milker-maid by profession, had a normal hemogram. There was no evidence of blood group incompatibility. The autopsy of the fetus showed an excessive tumorous enlargement of the pancreas caused by the leukemic infiltration (Fig. 1). The microscopic examination of the placenta showed leukemic cells in the fetal blood vessels and the villous stroma (Fig. 2). In 1966 Werner compiled from literature 28 cases of true connatal leukemia and added a case of his own. From the newer accessible literature we collected further 19 reports (Table). In 7 of the 49 cases the connatal leukemia appeared together with Down's syndrom.