Successful management of 30 kg Gigantic para-testicular liposarcoma.

We report the successful management of a paratesticular liposarcoma, which, to the best of our knowledge, is the largest known of its type. A 62-year-old male presented with a painless, gradually progressive left testicular "giant" mass measuring 60 × 40 cm, weighing 30 kg and growing over a period of three 3 years. Additionally, a 5 × 5 cm trophic ulcer could be seen at the bottom of the scrotum. The ultrasound of the left testis revealed the testis having been completely replaced with a cystic and solid tumour. Preoperative serum testicular tumour markers (STM) were within normal limits. The markers included Alpha Feto Protein, Beta Human Chorionic Gonadotropin and Lactose Dehydrogenase. A left sided high inguino-scrotal approach with a huge skin resection including the trophic ulcer with complete removal of the tumour and a primary complex closure of the wound was performed. The post-operative period was uneventful, and histopathology revealed a dedifferentiated liposarcoma. We believe social taboo and fear of disfigurement impart a sense of shame in patients which led to the delayed presentation in a hospital in the index patient. The absence of metastases even with a protracted course is surprising.

Detection of the Lateral Thermal Spread during Bipolar Vessel Sealing in an Ex Vivo Model-Preliminary Results.

Background: As an unwanted side effect, lateral thermal expansion in bipolar tissue sealing may lead to collateral tissue damage. Materials and Methods: Our investigations were carried out on an ex vivo model of porcine carotid arteries. Lateral thermal expansion was measured and a calculated index, based on thermographic recording and histologic examination, was designed to describe the risk of tissue damage. Results: For instrument 1, the mean extent of the critical zone > 50 °C was 2315 ± 509.2 µm above and 1700 ± 331.3 µm below the branches. The width of the necrosis zone was 412.5 ± 79.0 µm above and 426.7 ± 100.7µm below the branches. For instrument 2, the mean extent of the zone > 50 °C was 2032 ± 592.4 µm above and 1182 ± 386.9 µm below the branches. The width of the necrosis zone was 642.6 ± 158.2 µm above and 645.3 ± 111.9 µm below the branches. Our risk index indicated a low risk of damage for instrument 1 and a moderate to high risk for instrument 2. Conclusion: Thermography is a suitable method to estimate lateral heat propagation, and a validated risk index may lead to improved surgical handling.

Long Non-Coding RNA H19 Expression Correlates with Autophagy Process in Adrenocortical Carcinoma.

Adrenocortical carcinoma (ACC) is characterized by poor prognosis and high mortality. The suppression of the long-non-coding RNA H19, counterbalanced by IGF2 over-expression, leads to down-regulation of the autophagy markers, high proliferation rate and metastatic potential in patients affected by ACC. The administration of the deacetylase inhibitors (DACi) panobinostat, trichostatin A (TSA) and SAHA affected the cell viability of H295R monolayer and spheroids and induced the over-expression of H19 and autophagy transcripts. H19 knock down in H295R cells was not able to modulate the expression level of autophagy transcripts. Instead, H19 knock down was able to impede the ability of DACi to modulate the protein level of the autophagy markers. Furthermore, the administration of higher concentration of DACi was able to down-regulate the protein level of Beclin1 and p62 and to induce the conversion of LC3B-I into the active LC3B-II form, thus confirming an active autophagic process. Neither the active protein level nor the activity of caspases 8 and 3 was prompted by the DACi, thus excluding the involvement of the executioners of apoptosis in H295R decay. The DACi restore H19, the autophagy markers and trigger cell death in ACC cells. The re-activation of autophagy would represent a novel strategy for the treatment of patients affected by this severe malignancy.

Endoscopic ultrasound-fine needle biopsies of pancreatic lesions: Prospective study of histology quality using Franseen needle.

BACKGROUND: The introduction of fine needle biopsies (FNB) to clinical practice presents a changing trend towards histology in the endoscopic ultrasound-guided tissue acquisition (EUS-TA). AIM: To evaluate the clinical performance of a new FNB needle, the 22-gauge (22G) Franseen needle, when sampling pancreatic solid lesions. METHODS: Consecutive patients with an indication for EUS-TA for the assessment of pancreatic solid lesions were included in this prospective, single-center, single-arm trial. Each patient underwent a puncture of the lesion two times using the 22G Franseen needle and the obtained samples were directly placed into formalin for histological analysis. The primary study endpoint was the rate of high-quality obtained specimen. Secondary endpoints included the length and diameter of the core specimen, the diagnostic accuracy and the complication rate. RESULTS: From June 2017 to December 2018, forty patients with pancreatic solid lesions (22 females; mean age 67.2 years) were enrolled. Tissue acquisition was achieved in all cases. High-quality histology, rated with Payne score 3, was obtained in 37/40 cases (92.5%) after two needle passes. The mean size of the acquired histological core tissue was 1.54 mm × 0.39 mm. The diagnostic accuracy for the correct diagnosis was 85% (34/40). Only one adverse event was occurred, consisting of a self-limiting bleeding in the puncture site. CONCLUSION: The 22G Franseen needle achieved according to our standardized protocol a high rate of histological core procurement, and a high diagnostic accuracy, with one minor adverse event reported.

Burkitt's lymphoma of the prostate presenting as acute urinary retention: a case report.

BACKGROUND: Non-Hodgkin lymphomas, which include Burkitt's lymphoma, affect the prostate in only 0.1% of cases. They most commonly present as painless lymphadenopathy elsewhere in the body and can cause abdominal or thoracic pain and systemic symptoms such as fever, weight loss and night sweats. Here we report a rare case of sporadic Burkitt's lymphoma of the prostate whose initial clinical presentation was acute urinary retention. CASE PRESENTATION: A 28-year-old Caucasian male presented repeatedly with urinary retention. First, he was misdiagnosed with alcohol-induced urinary retention and later with benign prostatic hyperplasia. After the appearance of new symptoms, including hematuria and hydronephrosis, endoscopic and radiographic evaluation was performed. Transurethral biopsy of the prostate secured the diagnosis of Burkitt's lymphoma. The symptoms receded under chemotherapy and complete remission of the disease was established. CONCLUSION: This case report brings lymphomas into focus as a differential diagnosis for urinary retention in young males. Early use of extensive diagnostic measures is advised in patients with urinary retention for uncertain reasons to make prompt diagnosis and start appropriate treatment early.

Rhombencephalitis associated with isolated Zic4-antibodies in Paraneoplastic cerebellar degeneration: a case report.

BACKGROUND: Cerebellar degeneration as a consequence of a malignancy is a rare condition most commonly related to the presence of anti-Yo, anti-Hu, and anti-Tr/DNER antibodies. In recent years, several reports have indicated Zinc-finger protein 4 (Zic4) antibodies being associated with paraneoplastic cerebellar degeneration (PCD) in patients with small cell lung carcinoma. However, the prevalence and the significance of Zic4-antibodies may be underestimated due to their co-occurrence with more frequent antibodies such as anti-Hu. A literature review of isolated Zic4 mediated paraneoplastic syndromes yielded 14 cases reporting mainly benign clinical courses when treated early. CASE PRESENTATION: We present the case of a 67-year-old woman with progressive Zic4 antibody mediated PCD and rhombencephalitis. Immunomodulatory treatment, including intravenous methylprednisolone, plasmaphereses, and intravenous immunoglobulin (IVIG) was administered. Small cell lung cancer (SCLC) was detected, lobectomy performed and cyclophosphamide started. Despite this considerable therapeutic effort, rhombencephalitis led to defiant dysautonomia. CONCLUSION: Paraneoplastic syndromes related to isolated Zic4 antibodies are rare and typically show a benign clinical course. Here, we present the first case of a rapidly progressive isolated Zic4 associated PCD and rhombencephalitis. Despite considerable therapeutic efforts, the patient passed away on autonomic dysfunction, highlighting the significance of Zic4 associated disease.

Suturing of the laser resection area is recommended over a depth of 2 cm in an experimental porcine lung model.

BACKGROUND: Lung metastases can be removed by an Nd:YAG laser to save lung parenchyma. At these sites, a coagulated lung surface remains. Airtightness was investigated in relation to the depth of resection on an ex vivo porcine lung model. METHODS: Freshly slaughtered porcine double lung preparations were connected to a ventilator via a tube. Non-anatomical laser resections were performed with an 800 µm laser fiber and the Nd:YAG laser LIMAX® 120 (power: 40 and 60 watts). The following resection depths (each n=12) from the lung surface were examined: 0.5, 1, 1.5 and 2.0 cm. After resection the lungs were submerged under water and ventilated (frequency 10/min, Pinsp =25 mbar, PEEP =5 mbar). Airtightness of resection surfaces was determined by a leakage score, as well as the measurement of the leakage volume (in mL) per respiration (Group 1). Afterwards, the resection areas were coagulated for 5 seconds with a laser power of 60 watts at a distance of approximately 1 cm from the surface. This was followed by a re-evaluation for airtightness (Group 2). Finally, the resection surface was closed by a suture (PDS USP 4-0) and re-tested for airtightness (Group 3). The individual groups were compared for their significance (P<0.05) using a nonparametric test. RESULTS: Up to a resection depth of 1.5 cm, the ventilated resection surfaces were completely airtight regardless of the laser power. From a depth of resection of 1.5 cm, a mean air volume loss of 28.9±5.3 mL/respiratory cycle at 40 watts and of 26.4±5.8 mL at 60 watts was found. Additional surface coagulation did not significantly reduce the leakage rate. In contrast, suturing significantly reduced (P<0.0001) to 7.2±3.7 mL/ventilation (40 watts) and 6.0±3.4 mL/ventilation. At a resection depth of 2 cm, the leakage volume was 42.9±3.3 mL/respiratory cycle (40 watt) and 46.3±6.4 mL/respiratory cycle (60 watt). Additional surface coagulation failed to significantly reduce leakage volume, but suture closure provided airtightness. CONCLUSIONS: In non-ventilated porcine lungs, Nd:YAG laser resection surfaces up to a resection depth of 1.5 cm are airtight after ventilation onset. From a depth of 1.5 cm, closure of resection surfaces by an additional suture is needed. Airtightness of resection surfaces was not increased by additional coagulation.

Suture or Stapling Are Superior in Comparison to Bipolar Sealing for Closing the Bronchi.

Background Every anatomical lung resection requires the airtight closure of at least one bronchus. In current clinical practice, these bronchi are sealed with sutures or staplers. This study investigated in an ex vivo pig model whether a new bipolar sealing device MARSEAL (KLS Martin, Tuttlingen, Germany) could be an alternative for this purpose. Methods Complete bronchial trees were prepared free from pig heart-lung blocks that were removed at a slaughterhouse. These preparations were taken to the laboratory, and main, lobar, and segmental bronchi were closed using sutures, staples, or bipolar sealing. Each trachea was then intubated and connected to a ventilating device. The initial airtight closure of the bronchus was first tested with lung-protective ventilation. After 15 minutes of ventilation, the inspiratory ventilation pressure was slowly increased and the burst pressure (in mbar) was recorded. Each group included 12 bronchus closures. Group mean burst pressures were compared using a nonparametric test (Mann-Whitney U test). The significance level was p < 0.05. Results The main bronchi closed both stapler magazines or sutures were all initially airtight during ventilation. The mean burst pressure was 60 ± 0 mbar for staplers and 57.92 ± 5.8 mbar for sutures. In contrast, 50% of main bronchi sealed with MARSEAL devices (5 or 10 mm) leaked air from the beginning. This was also noted in all lobar bronchi sealed with the MARSEAL 5-mm device and 80% of those sealed with the MARSEAL 10-mm device. The mean burst pressure of initially airtight lobar bronchi was 12.7 ± 7.25 mbar. In contrast, all segmental bronchi (mean width: 1.6 cm) were airtight when ventilated. Mean burst pressure was 14.64 ± 9.1 mbar with the MARSEAL 5-mm device and 29.64 ± 21.3 mbar with the MARSEAL 10-mm device. Histological investigation of the preparations (with hematoxylin and eosin staining) showed intact cartilaginous structures that were largely unaffected by bipolar coagulation. The airtight sealing of the segmental bronchi resulted from fusion of the peribronchial tissue and not the cartilage. Conclusion Bipolar sealing is an inappropriate tool for the closure of the bronchi in comparison to suture or stapling.

Double Bipolar Sealing of the Pulmonary Artery Improves the Bursting Pressures.

Background In every anatomic lung resection, branches of the pulmonary artery have to be divided. In open surgery, this can be done with ligatures or staplers. In endoscopic surgery, only an endostapler can be used. By routing we ligate the vessels double. Bipolar sealing had yielded promising results, so we wanted to know if we can improve the bursting pressures especially in case of larger vessels by double sealing. Methods Experiments were performed on preparations of the left pulmonary artery extracted at the slaughterhouse. A pressure sensor was implanted at the central end to provide digital measurement of the pneumatic load on the vessel seal and thus establish bursting pressure in each case. Vessels were sealed with MARSEAL 5 (Gebrüder Martin GmbH & Co KG, Tuttlingen, Germany) and SealSafe G3 electric current. The vessels investigated were separated into three sizes: 1 to 6 mm, 7 to 12 mm, and >12 mm. The groups (n = 12 in each) were investigated for each vessel size-Group 1: ligature; Group 2: single seal; Group 3: double seals separated by gap of 0.5 cm; and Group 4: double seals separated by gap of 1.0 cm. Mean bursting pressure (mbar) was calculated for each group. Differences between groups were calculated with Mann-Whitney U test; differences with p < 0.05 were considered significant. Results The ligated vessels in the 1 to 6 mm group showed the highest bursting pressures (mean 515.7 ± 39.6 mbar). Mean bursting pressure in the single seal group was 231.6 ± 47.5 mbar. This was not significantly different from the group with double seals placed 0.5 cm apart. However, bursting pressures were significantly higher in the group with double seals placed 1 cm apart (p < 0.001). Mean value in this case was 308.5 ± 44.5 mbar. In the 7 to 12 mm vessels, mean bursting pressure was highest with ligation at 361 ± 67.1 mbar but was significantly higher in both groups with double bipolar seals (180.3 ± 52.1 mbar with 0.5-cm separation and 277.0 ± 64.5 with 1-cm separation) than in the single seal group (102.7 ± 16.1 mbar). In large vessels (>12 mm), mean bursting pressures were low (66.3 ± 12.7 mbar) with single seals but were significantly higher with double seals (162.3 ± 35.8 mbar [0.5-cm separation] and 137.3 ± 22.9 mbar [1-cm separation]). Conclusions In the ex vivo model of the pulmonary artery, double seals revealed significantly higher bursting pressures than single seals. If there is enough vessel length, the two seals should be placed 1 cm apart.

An unusual phenotype of MEN1 syndrome with a SI-NEN associated with a deletion of the MEN1 gene.

UNLABELLED: We report about a young female who developed an unusual and an aggressive phenotype of the MEN1 syndrome characterized by the development of a pHPT, malignant non-functioning pancreatic and duodenal neuroendocrine neoplasias, a pituitary adenoma, a non-functioning adrenal adenoma and also a malignant jejunal NET at the age of 37 years. Initial Sanger sequencing could not detect a germline mutation of the MEN1 gene, but next generation sequencing and MPLA revealed a deletion of the MEN1 gene ranging between 7.6 and 25.9 kb. Small intestine neuroendocrine neoplasias (SI-NENs) are currently not considered to be a part of the phenotype of the MEN1-syndrome. In our patient the SI-NENs were detected during follow-up imaging on Ga68-Dotatoc PET/CT and could be completely resected. Although SI-NENs are extremely rare, these tumors should also be considered in MEN1 patients. Whether an aggressive phenotype or the occurrence of SI-NENs in MEN1 are more likely associated with large deletions of the gene warrants further investigation. LEARNING POINTS: Our patient presents an extraordinary course of disease.Although SI-NENs are extremely rare, these tumors should also be considered in MEN1 patients, besides the typical MEN1 associated tumors.This case reports indicate that in some cases conventional mutation analysis of MEN1 patients should be supplemented by the search for larger gene deletions with modern techniques, if no germline mutation could be identified by Sanger sequencing.

More compression improves sealing effect on larger pulmonary arteries.

BACKGROUND: Small arteries and veins up to 7 mm can be sealed safe and divided with a bipolar sealing instrument. The results for the safe sealing of larger vessels were unsatisfactory in the past. Using an ex vivo pulmonary artery model, we aimed to investigate, if a higher compression force and duration will improve the bursting pressures in case of vessels >7 mm. MATERIAL AND METHODS: Heart-lung preparations (from 90 kg pigs) were removed en bloc at a slaughterhouse. The whole pulmonary artery was exposed from the pulmonary valve up to the periphery of the left lung. In the laboratory, a digital pressure sensor was implanted in the central end of the blood vessel to measure the bursting pressure (in mbar). The vessels examined were divided into three groups by diameter: 1-6 mm, 7-12 mm and >12 mm. After bipolar sealing, bursting pressures were determined by pneumatic testing. Seals were made using three equal MARSEAL instruments (Gebrüder Martin GmbH & CoKG, Tuttlingen, Germany) with a SealSafe G3 electric current and different jaw compression forces of each 35 N, 45 N, and 55 N. Bursting pressures were also measured for different compression durations (0 s, 5 s, 10 s, and 20 s) with 35 N compression. Mean bursting pressures were calculated for each group (n = 15). Groups were compared using a nonparametric test (Mann-Whitney U test). The significance level was P < 0.05. RESULTS: Mean bursting pressures in the 1-6 mm blood vessels were 290.5 ± 77.1 mbar (35 N), 323.0 ± 76.0 mbar (45 N) and 301.6 ± 69.9 mbar (55 N). The groups did not differ significantly. Mean bursting pressures in the 7-12 mm vessels were 108.1 ± 19.1 mbar (35 N), 154.3 ± 28.5 mbar (45 N), and 212.4 ± 45.3 mbar (55 N). In blood vessels >12 mm in diameter, we found mean bursting pressures of 77.7 ± 11.7 mbar (35 N), 117.6 ± 27.1 mbar (45 N), and 166.3 ± 56.6 mbar (55 N). The results for the groups with 55 N compression were significantly higher than for the other groups. A compression duration of 5 s led to significantly higher mean bursting pressures than a duration of 0 s but a duration of >5 s did not bring a further significant increase in mean bursting pressure. Histologic staining of the seal zone and microscopic examination did not reveal any differences relating to compression force. CONCLUSIONS: With a higher compression force, we reached satisfactory bursting pressures in case of pulmonary arteries >7 mm. An additional 5 s of compression before starting coagulation brings a further significant increase in bursting pressure. However, there is no advantage in a longer compression.

Bipolar impedance-controlled sealing of the pulmonary artery with SealSafe G3 electric current: determination of bursting pressures in an ex vivo model.

BACKGROUND: In every anatomic lung resection operation, the pulmonary artery itself or its branches must be sealed. This involves either stapling or ligating the vessels. Based on the positive results with the bipolar vessel sealing ≤7 mm in abdominal surgery the present study aimed to evaluate burst pressures of the pulmonary artery after sealing with the sealing instrument SealSafe G3 (Gebrüder Martin & CoKG, Tuttlingen, Germany). MATERIAL AND METHODS: The whole pulmonary artery above the pulmonary valve was exposed up to the periphery of the left lung in freshly removed pig heart-lung blocks. A pressure-measuring cylinder was then implanted in the prepared vessel on the side at the main trunk of the pulmonary artery to determine the pressure in the vessel. After either ligation or bipolar sealing of the pulmonary artery, the pneumatic burst pressure (millimeters of mercury) was determined in a water bath. Three groups (n = 12 for each seal type) with different vessel diameters were examined: group 1: 0-6 mm, group 2: 7-12 mm, and group 3: >12 mm. In all cases, vessel sealing was performed with a MARSEAL 5 instrument (Gebrüder Martin & Co KG, Tuttlingen, Germany) and the SealSafe G3 current. The mean burst pressures of the individual groups (ligature and bipolar sealing) were compared using two-tailed, nonparametric Mann-Whitney U test. Significance was defined as P < 0.05. RESULTS: The mean burst pressures in group 1 were measured by 340 ± 13.4 mm Hg with ligature and 205 ± 44.4 mm Hg with bipolar sealing (P < 0.001). In group 2, the mean values obtained were 270 ± 28.2 mm Hg for ligature and 162 ± 36.0 mm Hg for bipolar sealing (P < 0.001). In group 3, the mean burst pressures for bipolar sealing were only 52.1 ± 15.1 mm Hg, whereas those for ligated vessels were 253 ± 46.9 mm Hg (P < 0.001). For this size of vessel the burst pressure was also determined after stapling. The mean value in this case was 230 ± 21.8 mm Hg. CONCLUSIONS: In all groups, the mean burst pressures after bipolar sealing were significantly lower than those achieved with ligation, but they were sufficient for a save closure of the pulmonary artery with diameters up to 12 mm.

Laser resection of lung tissue: heat accumulation from adjacent laser application and how to cool it down.

BACKGROUND: Heat accumulation might induce thermal damage of the surrounding lung tissue, especially when multiple lesions are resected in one session. The present study aimed to investigate whether heat accumulates in the immediate vicinity of the resection surface and leads to thermal damage of the lung parenchyma, and what is the most effective cooling strategy in this situation. MATERIALS AND METHODS: In normothermic perfused paracardial swine lobes (n = 6), four punctiform laser lesions forming a square were created. Each lesion was lasered at a power of 100 W for 5 seconds. Two test conditions with square sides of either 1.0 or 0.5 cm were compared. Temperatures were recorded immediately after completing the laser procedure in the square center and in the corners using a thermal camera and continued during the cooling process at 10-second intervals until normothermia (37°C). We examined two cooling methods: rinsing with ice-cold (4°C) Ringer solution during the laser procedure (group B, n = 6) or submerging the lung in ice-cold water for 5 seconds immediately after laser application (group C, n = 6). In the control group A (n = 6), there was no cooling. RESULTS: In the 0.5 cm squares, mean temperature in the center immediately after laser application was 103.17 ± 8.56°C, significantly higher than in the corners (76.39 ± 2.87°C, p < 0.05). Normothermia in the quadrant corners was reached after 81 ± 14 and after 108 ± 29 seconds in the centers. Tissue in the square center revealed histological signs of thermic cell damage. In the 1.0 cm squares, mean temperature in the center was 64 ± 5°C, and in the corners was 77 ± 3.1°C (p < 0.05). Normothermia was regained after 93 ± 22 seconds in the center and 120 ± 21 seconds in the corners. Histological examination in the 1.0-quadrant centers revealed no signs of thermic cell damage. Submerging the lobe into ice-cold water lowered the temperature rapidly to under 40°C, and normothermia was regained after 75 ± 1.3 seconds. CONCLUSION: Laser application to the lung parenchyma causes considerable heat accumulation in closely related lesions. To prevent such cell damage, a distance of at least 1.0 cm between laser targets should be maintained. If no topical cooling method applied, sufficient time for spontaneous tissue cooling before additional laser application should be provided. The most effective cooling strategy against heat accumulation is submerging in ice-cold water for at least 5 seconds.

Giant intrathoracic left-sided vagal schwannoma.

Extensive intrathoracic tumors are rarely diagnosed radiologically without pre-existing symptoms. If located in the posterior mediastinum, it is most probably a neurogenic tumor. Schwannoma is the most frequent neurogenic neoplasia in this location, and most schwannomas are benign. To specify the diagnosis, a thoracic computed tomography must be done; if the growth is close to the medullary canal, a magnetic resonance tomography of the spinal column is necessary to detect neuroforamen infiltration. Our surgical goal was complete excision of the tumor, although many authors favor a minimally invasive approach. In our patient we performed open, en bloc removal of the tumor; removal of parts of the intraforamen was also necessary, which necessitated revision of the affected neuroforamen. Histologically this was a very rare case of vagal schwannoma (which has an incidence of less than 6% of all neurogenic tumors). This patient has a very promising prognosis following complete tumor resection.