Xanthogranulomatous appendicitis presenting asymptomatically 3 years after surgery for hilar cholangiocarcinoma: A case report.

INTRODUCTION: Xanthogranulomatous appendicitis (XGA) is a rare condition involving chronic inflammation of the appendix that is often difficult to distinguish from malignancy using imaging because of the formation of a heterogeneous mass with indistinct borders. Herein, we present a case of XGA with unusual clinical manifestations. PRESENTATION OF CASE: A 78-year-old female patient underwent radical resection of hilar cholangiocarcinoma with extended right hepatic lobectomy and biliary reconstruction. Three years postoperatively, she presented with an irregular mass in the right lateral pelvis, which was observed on computed tomography. The patient had not experienced recent clinical symptoms and did not present with abdominal tenderness. Routine blood tests did not indicate an increased inflammatory response; however, carcinoembryonic antigen levels continued to increase. Although disseminated recurrence of hilar cholangiocarcinoma and appendiceal carcinoma were suspected, XGA was diagnosed via laparoscopic appendectomy. DISCUSSION: XGA generally presents with symptoms of acute or chronic appendicitis, and is diagnosed incidentally during surgery. Hilar cholangiocarcinoma has a high recurrence rate, even after radical resection, and disseminated recurrence usually requires chemotherapy. In the present case, XGA was not suspected preoperatively because of the lack of physical symptoms and increased levels of tumor markers during follow-up for hilar cholangiocarcinoma. There have been no reports of XGA with such a confusing clinical course, thus confirming the difficulty in preoperatively diagnosing XGA. CONCLUSION: The preoperative diagnosis of XGA is difficult to differentiate from malignancy because of its clinical and imaging findings. We diagnosed the patient with XGA using laparoscopic surgery.

Minimally Invasive Conversion Surgery for Unresectable Gastric Cancer with Splenic Metastasis and Splenic Vein Tumor Thrombus: A Case Report.

While the importance of conversion surgery has increased with the development of systemic chemotherapy for gastric cancer (GC), reports of conversion surgery for patients with GC with distant metastasis and tumor thrombus are extremely scarce, and a definitive surgical strategy has yet to be established. Herein, we report a 67-year-old man with left abdominal pain referred to our hospital following a diagnosis of unresectable GC. Esophagogastroduodenoscopy and contrast-enhanced abdominal computed tomography (CT) revealed advanced GC with splenic metastasis. A splenic vein tumor thrombus (SVTT) and a continuous thrombus to the main trunk of the portal vein were detected. The patient was treated with anticoagulation therapy and systemic chemotherapy comprising S-1 and oxaliplatin. One year following chemotherapy initiation, a CT scan revealed progressive disease (PD); therefore, the chemotherapy regimen was switched to ramucirumab with paclitaxel. After 10 courses of chemotherapy resulting in primary tumor and SVTT shrinkage, the patient underwent laparoscopic total gastrectomy (LTG) and distal pancreaticosplenectomy (DPS). He was discharged without complications and remained alive 6 months postoperatively without recurrence. In summary, the wait-and-see approach was effective in a patient with GC with splenic metastasis and SVTT, ultimately leading to an R0 resection performed via LTG and DPS.

Wake-like skin patterning and neural activity during octopus sleep.

While sleeping, many vertebrate groups alternate between at least two sleep stages: rapid eye movement and slow wave sleep1-4, in part characterized by wake-like and synchronous brain activity, respectively. Here we delineate neural and behavioural correlates of two stages of sleep in octopuses, marine invertebrates that evolutionarily diverged from vertebrates roughly 550 million years ago (ref. 5) and have independently evolved large brains and behavioural sophistication. 'Quiet' sleep in octopuses is rhythmically interrupted by approximately 60-s bouts of pronounced body movements and rapid changes in skin patterning and texture6. We show that these bouts are homeostatically regulated, rapidly reversible and come with increased arousal threshold, representing a distinct 'active' sleep stage. Computational analysis of active sleep skin patterning reveals diverse dynamics through a set of patterns conserved across octopuses and strongly resembling those seen while awake. High-density electrophysiological recordings from the central brain reveal that the local field potential (LFP) activity during active sleep resembles that of waking. LFP activity differs across brain regions, with the strongest activity during active sleep seen in the superior frontal and vertical lobes, anatomically connected regions associated with learning and memory function7-10. During quiet sleep, these regions are relatively silent but generate LFP oscillations resembling mammalian sleep spindles11,12 in frequency and duration. The range of similarities with vertebrates indicates that aspects of two-stage sleep in octopuses may represent convergent features of complex cognition.

Oncocytic variant, a novel subtype of chromophobe renal cell carcinoma: a report of two cases and a literature review.

A novel variant of chromophobe renal cell carcinoma showing an oncocytic phenotype is proposed. Two new cases of this rare entity are presented and discussed along with six previous cases from our colleagues. A 76-year-old man and a 78-year-old man had a 3.4-cm and a 3.2-cm-diameter renal mass, respectively. On histopathological examination of surgical specimens, uniform eosinophilic cuboidal cells without a perinuclear halo growing in a tubular pattern were seen, and differential diagnosis from oncocytoma was necessary. Immunohistochemical staining for cytokeratin 7 and E-cadherin showed diffusely positive patterns in both, as in the previous reports. Although monosomy of chromosomes 7, 10, 13, and 17 was commonly observed in the previous reports, gains of chromosome 19 were observed in the two present cases. Immunohistochemical and cytogenetic approaches lead to exclusion of oncocytoma and the diagnosis of an oncocytic variant of chromophobe renal cell carcinoma.

Pneumothorax during lenvatinib treatment for hepatocellular carcinoma with lung metastasis.

Lenvatinib is an inhibitor of tyrosine kinases, such as vascular endothelial growth factor receptor and fibroblast growth factor receptor, and was first approved for use in thyroid cancer in 2015 in Japan. Additional approval was given in March 2018 for its use as a first-line treatment for advanced or unresectable hepatocellular carcinoma. Herein, we report a case of pneumothorax during lenvatinib treatment for multiple lung metastases of hepatocellular carcinoma in a 71-year-old man. Although the development of pneumothorax during treatment with anticancer agents for lung metastases is well-known, this is the first report of pneumothorax induced by lenvatinib during treatment for lung metastases of hepatocellular carcinoma.

Bilateral Xp11.2 translocation renal cell carcinoma: a case report.

BACKGROUND: Xp11.2 translocation renal cell carcinoma (RCC) is a rare variety of a kidney neoplasm. We report a case of bilateral Xp11.2 translocation RCC occurring metachronously and discuss this very rare entity with reference to the literature. CASE PRESENTATION: The patient was a 56-year-old woman who presented with a right renal tumor. The patient had undergone left radical nephrectomy 7 years previously, which resulted in a histopathological diagnosis of clear cell RCC. Open right partial nephrectomy was performed under the presumptive diagnosis of recurrence of clear cell RCC. The present right renal tumor was pathologically diagnosed Xp11.2 translocation RCC. More than 70% of the tumor cells in the present right tumor were strongly positive for transcription factor E3 (TFE3) expression by immunohistochemical analysis with an anti-TFE3 antibody. A break-apart of the TFE3 genes in the bilateral tumors was identified by fluorescence in situ hybridization analysis. Real time-polymerase chain reaction analysis for the alveolar soft part sarcoma locus-TFE3 fusion gene was performed, which gave a positive result in the bilateral tumors. Pathological comparison of each of the tumors might lead to a final diagnosis of Xp11.2 translocation RCC occurring metachronously. CONCLUSIONS: We present the bilateral Xp11.2 translocation RCC. A combination of immunohistochemical, cytogenetic and molecular biological approaches allowed the final diagnosis of such a rare RCC.

Multiple coronary and cerebral aneurysms in a patient with chronic thromboangiitis.

A 73-year-old man had multiple coronary aneurysms that resulted in acute myocardial infarction on the day before surgery for cerebral aneurysms. Emergent coronary angiography revealed that the lesion that caused the myocardial infarction was a distal left circumflex artery, and two huge coronary aneurysms were also found in the left circumflex artery. A two-stage treatment strategy was planned, including coronary aneurysm surgery, followed by cerebral aneurysm surgery. He underwent coronary artery aneurysmorrhaphy with closure of the ostia of the afferent and efferent arteries, and coronary artery bypass grafting with a saphenous vein graft applied to the left circumflex artery. The pathological findings suggested chronic thromboangiitis, as the inflammatory cells were observed to have infiltrated the coronary artery wall. The tissue remodeling of the aneurysmal wall indicated a positive response to tenascin C. We report a case of multiple coronary aneurysms, focusing on the pathological findings. .

A Recurrent Cervical Neurenteric Cyst Treated Anteriorly: Safe, Gross-Total Excision Facilitated by Prophylactic Unilateral Vertebral Artery Exposure, Microdissection, and Spinal Cord Monitoring-A Case Report and Technical Note.

This study reports on a 67-year-old woman with partial Brown-Séquard syndrome due to a recurrent cervical neurenteric cyst at C3 to C4. The myelopathic symptoms reappeared 22 years after a previous shunting operation performed posteriorly with a silicone tube connecting the intradural cervical cyst cavity to the subarachnoid space. We have now succeeded in removing the cyst nearly completely with the anterior approach. The surgical procedure consisted of right vertebral artery exposure at C3 and C4 and a subtotal corpectomy of C3 followed by microdissection of the cyst, duraplasty, and iliac strut graft fusion. Spinal cord monitoring with motor-evoked potential studies helped us safely dissect the cyst wall tightly adhering to the spinal cord. Duraplasty with Gore-Tex patch-grafting in conjunction with postoperative lumbar subarachnoid drainage worked well in preventing a spinal fluid fistula. At two years after surgery, the patient showed a nearly complete return of function without any recurrence of the cyst.

Two Parallel Pathways Assign Opposing Odor Valences during Drosophila Memory Formation.

During olfactory associative learning in Drosophila, odors activate specific subsets of intrinsic mushroom body (MB) neurons. Coincident exposure to either rewards or punishments is thought to activate extrinsic dopaminergic neurons, which modulate synaptic connections between odor-encoding MB neurons and MB output neurons to alter behaviors. However, here we identify two classes of intrinsic MB γ neurons based on cAMP response element (CRE)-dependent expression, γCRE-p and γCRE-n, which encode aversive and appetitive valences. γCRE-p and γCRE-n neurons act antagonistically to maintain neutral valences for neutral odors. Activation or inhibition of either cell type upsets this balance, toggling odor preferences to either positive or negative values. The mushroom body output neurons, MBON-γ5ß'2a/ß'2mp and MBON-γ2α'1, mediate the actions of γCRE-p and γCRE-n neurons. Our data indicate that MB neurons encode valence information, as well as odor information, and this information is integrated through a process involving MBONs to regulate learning and memory.

Lymph node retrieval after dissolution of surrounding adipose tissue for pathological examination of colorectal cancer.

Examination of >12 lymph nodes (LNs) is important for the diagnostic accuracy of nodal status following resection of colorectal cancer. In the present study, the efficacy of a fat dissolution technique for LN retrieval was evaluated using resected colon and rectum mesentery. First, the resected mesentery was searched for LNs by inspection and palpation immediately after surgery. Subsequently, fat dissolution liquid was applied to the remnant fat and the LN search was repeated. The primary endpoint was whether the second assessment would increase the number of evaluated LNs. Recruitment of 20 patients was planned. The study was conducted after institutional review board approval and written informed consent was obtained. Among 20 participants, 1 patient was excluded because LN dissection was not performed. The median number of LNs identified at the first and second assessments was 13 and 6, respectively, producing a significant increase in total LNs evaluated (13 vs. 20, respectively; P<0.01; paired t-test). One positive node was identified among the additionally identified LNs (0.9%, 1/107). The second assessment increased the number of LNs assessed to >12 in 4 patients, and although staging was not changed, the treatment was potentially altered in 2 stage II patients. The maximum diameter of the additionally obtained LNs was significantly smaller compared with those from the first assessment (4 vs. 7.7 mm, respectively; P<0.01; Wilcoxon signed-rank test). After the fat dissolution technique, the tumor cells were satisfactorily stained by carcinoembryonic antigen and cytokeratin-20. In conclusion, applying fat dissolution liquid to the remnant adipose tissue of the mesentery of the colon and rectum identified additional LNs. This method should be considered when insufficient LNs are identified after conventional LN retrieval.

Loeys-Dietz syndrome with aortic wall necrosis secondary to external wrapping.

External aortic wrapping may overcompress the aortic wall. An 18-year-old man underwent valve-sparing aortic root replacement for annuloaortic ectasia caused by Loeys-Dietz syndrome. External Dacron graft wrapping was performed to protect the ascending aorta. A year later, he underwent mitral valve repair. Intraoperatively, after unwrapping the external graft, the wrapped ascending aortic wall appeared necrotic. Histopathology showed damaged smooth muscle cells in the adventitia and outer media with unaffected inner layers and an intact intimal surface. Ischaemic necrosis of the aortic wall was associated with arterial insufficiency secondary to overcompression of the aortic wall that was already weakened by Loeys-Dietz syndrome. We describe a patient with Loeys-Dietz syndrome in whom pathognomonic necrosis in the wrapped aortic wall was observed histopathologically.

Intramurally spreading aortic intimal sarcoma masquerading as ruptured aortic dissection.

Preoperative diagnosis of aortic sarcomas is often difficult. Herein, we describe a case of a 68-year-old man who had an aortic intimal sarcoma with aortic wall hypertrophy that masqueraded as a thrombosed aortic dissection or mural thrombus on computed tomography. Because of the sudden appearance and rapid growth of the tumour surrounding the left subclavian artery with precordial pain and haemosputum, left subclavian arterial rupture caused by aortic dissection was suspected, requiring emergent total arch replacement. Surgery revealed an intimal sarcoma, not an aortic dissection, with the thickening of the fragile intima spreading into the aorta. Thus, an intramurally spreading aortic intimal sarcoma should be suspected when computed tomography reveals an irregular thickening of the aortic intima.

Metachronous solitary splenic metastasis arising from early gastric cancer: a case report and literature review.

BACKGROUND: The metastasis of malignant tumors to the spleen is rare, and only a small percentage of cases can be treated surgically, as splenic metastases generally occur in the context of multivisceral metastatic cancer at a terminal stage. We report a rare case of metachronous solitary splenic metastasis arising from early gastric cancer. CASE PRESENTATION: A 75-year-old man was initially referred to our hospital for examination of gastric cancer, diagnosed at a medical check-up. Esophagogastroduodenoscopy showed a slightly elevated lesion with a central irregular depression in the upper-third of the stomach. Biopsy specimens of the lesion showed a moderately-differentiated adenocarcinoma, and abdominal computed tomography showed no evidence of distant metastases. Endoscopic submucosal dissection was performed, with histological confirmation of a moderately-differentiated adenocarcinoma invading the submucosal layer. The patient subsequently underwent laparoscopic total gastrectomy with regional lymph node dissection, resulting in no residual carcinoma and no lymph node metastasis. Computed tomography, 28 months later, showed a well-defined mass measuring 4.2 cm in diameter in the spleen, and the patient underwent a splenectomy, since there was no evidence of further metastatic lesions in any other organs. Histological examination confirmed the diagnosis of a poorly-differentiated adenocarcinoma originating from the previous gastric cancer. The patient was alive 2 months after surgical resection of the splenic metastasis without any recurrence. CONCLUSION: To the best of our knowledge, this is only the second case of a solitary splenic metastasis from early gastric cancer to be reported in the English literature. The present case suggests surgical resection may be the preferred treatment of choice for patients with a solitary splenic metastasis from gastric cancer.

Suppression of a single pair of mushroom body output neurons in Drosophila triggers aversive associations.

Memory includes the processes of acquisition, consolidation and retrieval. In the study of aversive olfactory memory in Drosophila melanogaster, flies are first exposed to an odor (conditioned stimulus, CS+) that is associated with an electric shock (unconditioned stimulus, US), then to another odor (CS-) without the US, before allowing the flies to choose to avoid one of the two odors. The center for memory formation is the mushroom body which consists of Kenyon cells (KCs), dopaminergic neurons (DANs) and mushroom body output neurons (MBONs). However, the roles of individual neurons are not fully understood. We focused on the role of a single pair of GABAergic neurons (MBON-γ1pedc) and found that it could inhibit the effects of DANs, resulting in the suppression of aversive memory acquisition during the CS- odor presentation, but not during the CS+ odor presentation. We propose that MBON-γ1pedc suppresses the DAN-dependent effect that can convey the aversive US during the CS- odor presentation, and thereby prevents an insignificant stimulus from becoming an aversive US.

DISCO Interacting Protein 2 regulates axonal bifurcation and guidance of Drosophila mushroom body neurons.

Axonal branching is one of the key processes within the enormous complexity of the nervous system to enable a single neuron to send information to multiple targets. However, the molecular mechanisms that control branch formation are poorly understood. In particular, previous studies have rarely addressed the mechanisms underlying axonal bifurcation, in which axons form new branches via splitting of the growth cone. We demonstrate that DISCO Interacting Protein 2 (DIP2) is required for precise axonal bifurcation in Drosophila mushroom body (MB) neurons by suppressing ectopic bifurcation and regulating the guidance of sister axons. We also found that DIP2 localize to the plasma membrane. Domain function analysis revealed that the AMP-synthetase domains of DIP2 are essential for its function, which may involve exerting a catalytic activity that modifies fatty acids. Genetic analysis and subsequent biochemical analysis suggested that DIP2 is involved in the fatty acid metabolization of acyl-CoA. Taken together, our results reveal a function of DIP2 in the developing nervous system and provide a potential functional relationship between fatty acid metabolism and axon morphogenesis.

Suppression of Dopamine Neurons Mediates Reward.

Massive activation of dopamine neurons is critical for natural reward and drug abuse. In contrast, the significance of their spontaneous activity remains elusive. In Drosophila melanogaster, depolarization of the protocerebral anterior medial (PAM) cluster dopamine neurons en masse signals reward to the mushroom body (MB) and drives appetitive memory. Focusing on the functional heterogeneity of PAM cluster neurons, we identified that a single class of PAM neurons, PAM-γ3, mediates sugar reward by suppressing their own activity. PAM-γ3 is selectively required for appetitive olfactory learning, while activation of these neurons in turn induces aversive memory. Ongoing activity of PAM-γ3 gets suppressed upon sugar ingestion. Strikingly, transient inactivation of basal PAM-γ3 activity can substitute for reward and induces appetitive memory. Furthermore, we identified the satiety-signaling neuropeptide Allatostatin A (AstA) as a key mediator that conveys inhibitory input onto PAM-γ3. Our results suggest the significance of basal dopamine release in reward signaling and reveal a circuit mechanism for negative regulation.

[Undifferentiated Prostate Cancer Treated with Radiation Therapy].

We describe a 75-year-old man with undifferentiated prostate cancer that was treated with radiation therapy. He presented at a nearby general hospital with dysuria and pain upon micturition. He was diagnosed with undifferentiated prostate cancer by a needle biopsy and referred to our hospital for further examination and treatment. Enhanced computed tomography and magnetic resonance images showed prostate cancer and right obturator lymph node metastasis measuring 2.5 cm. Cystoscopy and colonoscopy revealed direct invasion of the urinary bladder and rectum. We constructed a vesical fistula and an artificial anus, and then treated the primary tumor and lymph node metastasis with radiation. Undifferentiated prostate cancer is extremely rare and to our knowledge only a few cases have been reported. We suggest that radiation might be effective for treating undifferentiated prostate cancer with or without local invasion and/or metastasis along with total body control.

Young adult onset systemic Epstein-Barr virus-positive T-cell lymphoproliferative disorders of childhood.

A 20-year-old woman had a fever, pancytopenia, and liver failure, and was suspected to be suffering from chronic active Epstein-Barr virus (EBV) infection, based on the detection of high EBV-DNA and EBV antibody titers at another hospital. At our institution one month later, clinical manifestations had diminished, and antibody titers had decreased but remained elevated relative to normal levels. Four days later, the patient required hospitalization due to fever, liver damage, and cervical lymphadenopathy. Bone marrow examination and lymph node biopsy results showed EBV-positive cytotoxic T-cells that were predominantly CD4-positive. The disease followed a fulminant course and the patient died of multiple organ failure on hospitalization day 11. Because complicated chromosomal aberrations and T-cell receptor gene rearrangements were identified, we diagnosed her as having systemic EBV-positive T-cell lymphoproliferative disorder of childhood. This disease type includes a lymphoproliferative disorder that is associated with chronic active EBV infection. However, it is clinically different from the type following acute EBV infection. We consider distinguishing between these two types to be important for selecting an early diagnostic procedure and the optimal therapy.

[A case of serous cystic neoplasm of the pancreas with stenosis of the main pancreatic duct].

A 64-year-old woman was accidentally detected to have multiple cystic tumors measuring 30 mm in diameter in the pancreatic head in 2009. The probable diagnosis was a serous cystic neoplasm of the pancreas. However, the tumor had grown to 52 mm in diameter in 4 years, and endoscopic retrograde pancreatography (ERCP) showed stenosis of the main pancreatic duct. We performed subtotal stomach-preserving pancreaticoduodenectomy, and histopathological diagnosis was serous cystadenoma.