Successful salvage of torsion testis by means of intraoperative indocyanine green fluorescence imaging.

BACKGROUND: Testicular torsion (TT) is common surgical emergency that requires early diagnosis and immediate intervention within 6 h since its onset to salvage the testis. However, the decision was made only by the surgeon's experience whether it has to be resected or not. Recently, indocyanine green (ICG) has become an excellent tool to identify biliary and vascular anatomy, and assess perfusion abnormalities in tissues. In this case report, we successfully salvaged the twisted testis, since the testicular blood perfusion was confirmed by means of intraoperative ICG (IICG) fluorescence imaging. CASE PRESENTATION: A 14-year-old healthy male patient presented due to acute left testicular pain. The patient was diagnosed with TT and had immediate surgery. Macroscopically, the testis had stagnant blood flow and appeared to be dark colored. After manual detorsion, the testis remained cyanotic and with macroscopically poor blood flow. ICG angiography was performed under near-infrared light by laparoscopic camera to assess the perfusion of the affected testicle. An excellent ICG signal appeared after 45 s in the testis, and decision was made to be preserved. Therefore, left orchidopexy was performed to complete the operation. The patient had a good postoperative course and was discharged the day after surgery. Six months later, the testis did not show any shrinkage, and both sides of the testis showed the same size without any consequences. CONCLUSION: The blood flow in the testis was visually confirmed during the IICG fluorescence method. ICG fluorescence imaging may become an effective alternative to evaluate whether a testis can be preserved following TT.

TERT promotor region rearrangements analyzed in high-risk neuroblastomas by FISH method and whole genome sequencing.

BACKGROUND: Unfavorable neuroblastomas (NBLs) achieve telomere stabilization via telomerase activation through MYCN amplification, TERT promoter region (TERT-PR) rearrangements, or alternative telomere lengthening of telomeres. No well-established methods are available for investigating TERT-PR rearrangements. We examined the relationship between and prognosis by fluorescence in situ hybridization (FISH) upstream and downstream of TERT to establish a simple analysis method. PROCEDURE: TERT-PR rearrangements were analyzed in 3 M MYCN amplified cases and, 11MYCN non-amplified cases (1 MS case, 1 L2 case and 2 M cases less than 18 months, and 1 L2 case and  6 M cases over 18 months old at diagnosis) to determine if MYCN and TERT-PR rearrangement were independent prognostic factors. In total, 14 patients (11 males, 3 females; median age 36.4 months, range 1-122 months) with NBLs were evaluated at Hiroshima University. We identified MYCN amplification, TERT expression, and TERT-PR rearrangements. TERT-PR rearrangement was detected by FISH upstream and downstream of TERT on Chr5.p15.33. For TERT-PR rearranged cases, we characterized the fusion partners by whole genome sequencing. RESULTS: We detected TERT-PR rearrangements in two NBL samples. Both samples were high-risk NBLs and MYCN single NBLs, and their TERT expression levels were extremely higher than in the other samples. Genomic translocation occurred at chromosome 5p15.33 according to whole genome sequencing, agreeing with the FISH results. One case showed translocation of the chr5.p15.33 SLCA6A19 gene to 22q12.3, and another case showed chr5p15.33 to chr5q33.3. CONCLUSIONS: FISH is a useful diagnostic tool for evaluating high-risk NBLs in which TERT-PR rearrangements have occurred.

Inflammatory myofibroblastic tumors of the lung carrying a chimeric A2M-ALK gene: report of 2 infantile cases and review of the differential diagnosis of infantile pulmonary lesions.

We report 2 infantile cases of pulmonary tumor carrying a chimeric A2M-ALK gene. A2M-ALK is a newly identified anaplastic lymphoma kinase (ALK)-related chimeric gene from a tumor diagnosed as fetal lung interstitial tumor (FLIT). FLIT is a recently recognized infantile pulmonary lesion defined as a mass-like lesion that morphologically resembles the fetal lung. Grossly, FLIT characteristically appears as a well-circumscribed spongy mass, whereas the tumors in these patients were solid and firm. Histologically, the tumors showed intrapulmonary lesions composed of densely proliferating polygonal or spindle-shaped mesenchymal cells with diffuse and dense infiltrations of inflammatory cells forming microcystic or micropapillary structures lined by thyroid transcription factor 1-positive pneumocytes, favoring inflammatory myofibroblastic tumor rather than FLIT. The proliferating cells were immunoreactive for ALK, and A2M-ALK was identified in both tumors with reverse-transcription polymerase chain reaction. The dense infiltration of inflammatory cells, immunoreactivity for ALK, and identification of an ALK-related chimeric gene suggested a diagnosis of inflammatory myofibroblastic tumor. Histologically, most reported FLITs show sparse inflammatory infiltrates and a relatively low density of interstitial cells in the septa, although prominent infiltration of inflammatory cells and high cellularity of interstitial cells are seen in some FLITs. The present cases suggest that ALK rearrangements, including the chimeric A2M-ALK gene, may be present in these infantile pulmonary lesions, especially those with inflammatory cell infiltration. We propose that these infantile pulmonary lesions containing a chimeric A2M-ALK gene be categorized as a specific type of inflammatory myofibroblastic tumor that develops exclusively in neonates and infants.

[Retrospective Evaluation of Hypoxia and Silent Regurgitation during Laparoscopic Sleeve Gastrectomy].

BACKGROUND: The proportion of obese people is gradually increasing. In recent years, laparoscopic sleeve gastrectomy has been performed as a weight loss surgery. We reported the extent of respiratory complications and the keys of anesthetic management in this procedure. METHODS: Forty consecutive morbidly obese patients received laparoscopic sleeve gastrectomy. A sample for arterial blood gas analysis was taken after intubation and at the end of the operation. Moreover, to examine the risk of silent aspiration, 16 patients were subjected to measuring the gastric juice volume and pH of the pharynx. RESULTS: Oxygenation index (P/F ratio) after intuba- tion was markedly reduced, but there was no correla- tion with the BMI On the other hand, P/F ratio at the end of surgery improved in patients with low BMI Hypercapnia was rare after extubation, but respiratory rate was increased in the patients with high BMI Gas- tric secretion after induction was increased, but there was no overt sign of silent regurgitation of gastric juice. CONCLUSIONS: Oxygenation was extremely deterio- rated immediately after intubation in the morbidly obese patients. There is a need for aggressive respira- tory management because intraoperative oxygenation was not improved in the patients with high BMI.

Telomere biology including TERT rearrangements in neuroblastoma: a useful indicator for surgical treatments.

PURPOSE: Our telomere biology study of neuroblastomas (NBLs) has revealed that unfavorable NBLs acquired telomere stabilization by telomerase activation or ALT (alternative lengthening of telomeres). Recently, genomic rearrangements in a region proximal to the telomerase reverse transcriptase (TERT) gene have been discovered in NBLs. In this study, TERT rearrangements were examined in NBLs along with their relationship to other aspects of telomere biology. METHODS: In 121 NBLs, including 67 cases detected by mass-screening whose telomere length, telomerase activity, ALT with ATRX/DAXX alterations, and MYCN amplification were already known, TERT rearrangements were examined using GeneChip SNP arrays. RESULTS: The 11 ATRX/DAXX mutated ALT cases and 29 cases with high telomerase activity showed poor prognosis. MYCN amplification and TERT rearrangements were independently detected in 16 and 13 cases, respectively, and these alterations were significantly correlated with high telomerase activity. In 81 infant cases, MYCN amplification, TERT rearrangements and ATRX mutations were detected in 3, 4, and 3 cases, respectively. Among them, 6 cases showed progression or recurrences. CONCLUSIONS: Telomere stabilization in NBLs is acquired by telomerase activation through MYCN amplification, TERT rearrangements or by ALT. Since these tumors usually show progression and recurrence, complete resection should be considered, even in infant cases. LEVEL OF EVIDENCE: Prognosis study, level III.

[A Case of Long-Term Survival in a Patient with Small Intestinal Adenocarcinoma with Peritoneal Dissemination].

Primary small intestinal adenocarcinoma is rare and its outcome is poor. A 46-year-old man admitted for vomiting was found in enhanced abdominal CT to have local jejunum stenosis and dilation at its oral site. A partial jejunectomy was performed and a jejunal tumor with multiple disseminated nodules in the peritoneum was revealed. Histologically, the adenocarcinoma of the jejunum appeared to be a papillary adenocarcinoma, and also, in part, a moderately differentiated tubular adenocarcinoma. After the jejunectomy, the patient was treated with S-1 chemotherapy, but 22 months after the initial diagnosis, a recurrence was detected. The patient underwent a second partial jejunectomy, and a weekly dose of paclitaxel (PTX) plus doxifluridine (5'-DFUR) was selected as the second-line treatment. The patient is still responding to the treatment 55 months after the last operation. Combination chemotherapy with weekly PTX/5'-DFUR may improve the prognosis for S-1-resistant small intestinal adenocarcinoma.

[Localized Malignant Mesothelioma of the Pleura].

A 65-year-old man presented with the chief complaint of cough. Chest computed tomography showed the mediastinal tumor. The tumor was resected under the 4th intercostal thoracotomy and the mediastinal approach. The biphasic type of localized malignant mesothelioma was diagnosed by the pathological findings. The postoperative course was uneventful. After postoperative adjuvant radiotherapy and chemotherapy (cisplatin and pemetrexed sodium hydrate), he is well without relapse 7 months after surgery.

[Pulmonary dirofilariasis].

A 70-year-old man was admitted to our department for pulmonary nodule of 15 mm in diameter in the left lower lobe detected by chest computed tomography (CT). A possibility of malignant tumor could not be ruled out, and lung partial resection was performed. Pathological examination during operation revealed a coagulation necrosis and the lesion was finally diagnosed as pulmonary dirofilariasis.

Multicentric solid pseudopapillary neoplasms of the pancreas diagnosed by endoscopic ultrasound-guided fine needle aspiration: a case report.

Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare tumor. This neoplasm usually arises as a single mass; multicentricity is exceptionally rare. We report the preoperative diagnosis of multicentric SPNs by endoscopic ultrasound-guided fine needle aspiration (EUS-FNA). A 32-year-old woman presented to the hospital with a pancreatic tumor that was detected on abdominal echography. Contrast-enhanced computed tomography (CT) scans revealed a 5-mm low-density mass in the body of the pancreas and a 10-mm mass in the tail of the pancreas. Magnetic resonance imaging (MRI) also revealed two tumors in the body and tail of the pancreas. On endoscopic ultrasonography (EUS), two indistinct and heterogeneous echogenic masses were found, and EUS-FNA was performed for each of these tumors. Cytological analysis revealed that the two masses were highly cellular with papillary groups of small, uniform, oval cells surrounding a fibrovascular core. Immunohistochemistry was positive for α-1 antitrypsin, vimentin, neuron-specific enolase (NSE), CD10, and progesterone receptor. These features confirmed the preoperative diagnosis of multicentric SPNs. The patient underwent laparoscopic distal pancreatectomy with splenectomy. The final pathologic diagnosis was multicentric SPNs. During 2 years of follow-up, she has not developed any recurrence.

A rare diagnostic challenge in acute appendicitis: a case report.

Although rare, acute appendicitis presenting as a remote abscess with cellulitis in the gluteal region, retroperitoneal region, groin, or thigh does occur and may present a diagnostic challenge. We report a case of a 78-year-old woman presenting with an extensive gas-forming abscess in the right gluteal region secondary to perforated appendicitis without significant gastrointestinal symptoms. Computed tomography (CT) demonstrated a retroperitoneal abscess extending along the sacropelvic surface of the ilium to the subcutaneous tissue. Subsequently, laparotomy revealed retrocecal appendicitis perforated at the base of the cecum, and contained in the retroperitoneum without any signs of peritonitis. This case not only represents an unusual manifestation of acute appendicitis, but also alerts us to the importance of anatomical considerations when interpreting disease extent with imaging. In the differential diagnosis of gluteal or upper thigh abscesses, the rare possibility of perforated acute appendicitis should be considered.