Giant cell arteritis without cranial manifestations caused mesenteric involvement: a case report.

BACKGROUND: Giant cell arteritis (GCA) is a granulomatous vasculitis and targets large vessels with predominance for the aortic arch and the cranial branches. GCA with cranial symptoms shows headache, jaw claudication, and ophthalmologic symptoms and thus was previously called temporal arteritis. Recently, cases of GCA without cranial manifestations and extracranial GCA have been reported. CASE PRESENTATION: A 76-year-old woman was referred to our hospital complaining of sudden abdominal pain and high fever. Her present history of illness did not show any cranial symptoms such as headache, visual disturbance, or stroke. CT images showed severe thickening of the small intestinal mesentery and massive ascites. She was diagnosed to have acute abdomen probably with gastrointestinal perforation and underwent the emergent laparotomy. Excisions of a 60-cm length of the jejunum including the thickening mesenteric lesion were carried out. Marked hypertrophy of the vascular intima and mild stenosis of the arterial lumen were displayed with infiltration of lymphocytes, neutrophils, and eosinophils. Scattered multinucleated giant cells on the endothelium, in the intima, media, and adventitia were demonstrated. Elastica van Gieson stain showed focal loss and fragmentation of the internal elastic lamina. Histopathological examinations showed typical GCA. Her postoperative process was uneventful without any symptoms, and she was followed as an out-patient prescribed with daily doses of 40 mg of prednisolone. CONCLUSIONS: We hereby report a rare case of mesenteric involvement in GCA without cranial manifestations and elucidate the histopathological features of extracranial GCA in arteries as well as veins and jejunum.

[A Resected Case of Castleman's Disease That Was Difficult to Diagnose Preoperatively].

A 70-year-old man was referred to our hospital and admitted after abdominal computed tomography(CT)examination revealed a tumor in the retroperitoneum. The specific blood test findings were poor, and surveillance CT demonstrated a wellcircumscribed, enhanced mass measuring 40mm with calcification behind the horizontal portion of the duodenum. The tumor presented with a slightly high signal in low signal, T2 in T1, and it showed a diffusion decrease in DWI; the simple abdominal MRIrevealed heterogeneous accumulation of SUVmax 3.0 only for the lesion in FDG/PET-CT. Specific findings did not lead to a diagnosis, although EUS-FNA lower aspiration biopsy cytology was performed. Laparoscopic tumorectomy was performed to help determine the treatment strategy. Histopathological examination indicated that the tumor was composed of multiple lymph follicles with concentric layers of mantle zone cells, showing an onion skin-like lesion and atrophic germinal centers. The germinal centers had penetrating arterioles with hyalinized vessel walls, and Castleman's disease(hyaline vascular type)was therefore diagnosed. In this case, it was difficult to diagnose Castleman's disease before surgery. Surgical excision is a diagnostic as well as a curative method for management of this disease.

[Pathological Complete Response in a Case of Advanced Esophageal Cancer with Widespread Lymph Node Metastases Treated Using Preoperative Chemotherapy with Docetaxel, Cisplatin, and 5-FU(DCF therapy)].

The patient was a 66-year-old woman, who was diagnosed with cT3N3M0, cStage III esophageal cancer with widespread lymph node metastases in the mediastinum and abdomen. She was treated with 2 courses of chemotherapy with docetaxel/ cisplatin/5-FU(DCF therapy). CT and FDG PET-CT showed a significant reduction in both the primary tumor and the metastatic lymph nodes following treatment. We performed subtotal esophagectomy and gastric tube reconstruction with lymphadenectomy. The histopathological findings showed no residual viable tumor cells or foreign body-type giant cells with necrosis. The pathological effect of chemotherapy was defined as Grade 3(pCR). Our case suggested that DCF chemotherapy is potentially a very effective treatment for advanced esophageal cancer with widespread lymph node metastases.

[Two Cases of Systemic Steroid Therapy for Hyperbilirubinemia after Right Hepatic Lobectomy of Liver Metastasis from Colorectal Cancer].

We report 2 cases of systemic steroid therapy for hyperbilirubinemia after right hepatic lobectomy for liver metastases from colorectal cancer.One of the patients was a 65-year-old man, diagnosed with transverse colon cancer with multiple liver metastases.After surgery for the primary lesion and chemotherapy(FOLFOX plus bevacizumab, FOLFIRI plus panitumumab), he underwent right hepatic lobectomy for the liver metastasis.His preoperative ICG 15 min was 8%.Conjugated hyperbilirubinemia without biliary stenosis occurred after the hepatectomy.Two courses of pulse steroid therapy were effective for only a limited period.After initiation of oral prednisolone therapy, his hyperbilirubinemia decreased.The other patient was a 66- year-old man with rectal cancer who underwent a low anterior resection.Postoperatively, a liver metastasis appeared.After CapeOX therapy, he underwent right hepatic lobectomy.His preoperative ICG 15 min was 5%.Conjugated hyperbilirubinemia occurred after hepatectomy.Pulse steroid therapy was effective for only a limited period.After initiation of prednisolone therapy, his hyperbilirubinemia decreased.In both cases, fatty changes in the liver were detected.Chemotherapy -associated hepatotoxicity may induce hyperbilirubinemia after hepatectomy.The effectiveness of systemic steroid therapy for hyperbilirubinemia is demonstrated by these cases.

[Surgical Resection for Advanced Gastric Cancer with Portal Vein Tumor Embolus - Two Cases].

Gastric cancer with portal tumor embolus is rare and there is no definite strategy for its surgical resection. We report 2 cases ofgastric cancer with portal vein tumor embolus treated using gastrectomy and thrombectomy. Case 1: The patient was a 56- year-old man. We performed total gastrectomy, distal pancreatectomy, splenectomy, and thrombectomy. The patient was treated with 4 courses ofS -1 plus CDDP chemotherapy followed by S-1 administration. Eight months after surgery, CT revealed metastasis in the left adrenal gland and he died 2 years after surgery. Case 2: The patient was a 57-year-old man. We performed total gastrectomy, distal pancreatectomy, splenectomy, partial resection of the transverse colon, and thrombectomy. The patient was treated using adjuvant S-1 chemotherapy followed by UFT administration for 3 years. The patient has been alive with no tumor recurrence for the past 10 years. If there is no other therapeutic option for portal vein embolus, gastrectomy with thrombectomy could increase the possibility oflong -term survival.

Efficacy of preoperative dexamethasone for postoperative nausea and vomiting after laparoscopic cholecystectomy: a large-scale, multicenter, randomized, double-blind, placebo-controlled trial in Japan.

BACKGROUND: To assess the efficacy of preoperative dexamethasone for postoperative nausea and vomiting (PONV) after laparoscopic cholecystectomy (LC) in Japan. METHODS: A total of 270 patients at eight hospitals were randomized to receive dexamethasone 8 mg (n = 136) or placebo (n = 134) intravenously before LC. The primary endpoint was the degree of PONV and antiemetic requirements within 24 h after LC. Secondary endpoints were postoperative complications, postoperative hospital stay, and cost of hospital stay. This study was registered: UMIN-CTR (UMIN000003841). RESULTS: Within 6 h after LC, 17% (23/136) of patients in the dexamethasone group versus 24% (32/134) in the placebo group reported nausea (P = 0.3), and 5% (7/136) versus 7% (10/134) reported vomiting (P = 0.2). Metoclopramide 10 mg was used 0.09 ± 0.31 versus 0.14 ± 0.35 times (P = 0.2). From 6 to 24 h, 10% (14/136) versus 13% (17/134) reported nausea (P = 0.5), and 5% (7/136) versus 5% (7/134) reported vomiting (P = 0.8). Metoclopramide was used 0.04 ± 0.19 versus 0.03 ± 0.17 times (P = 0.8). Postoperative complications and postoperative hospital stay did not differ significantly between the two groups, but the cost of hospital stay was slightly higher in the dexamethasone group (P < 0.05). CONCLUSIONS: Routine use of preoperative dexamethasone for PONV after elective LC in Japan was not shown to have a clinical advantage.

CT and MRI findings for Brunner's gland hamartoma: report of three cases.

Brunner's gland hamartoma (BGH) is an uncommon, benign, tumor-like lesion of the duodenum. Endoscopic diagnosis of larger BGH is sometimes difficult. We describe computed tomography (CT) and magnetic resonance imaging (MRI) findings for three patients with BGH. In all three cases, CT and MRI revealed internal cystic changes within the mass and a stalk originating in the duodenal bulb. These findings may be useful for diagnosis of BGH.

Predicting prolonged hospital stay after laparoscopic cholecystectomy.

INTRODUCTION: Widespread application of laparoscopic cholecystectomy (LC) has resulted in a high complication rate and leads to prolonged hospital stays. This study aimed to investigate the preoperative and intraoperative clinical factors that relate to prolongation of hospital stay. METHODS: We studied 370 patients who underwent LC for gallbladder disease between 2008 and 2012. Clinical risk factors were retrospectively collected. The clinical pathway for LC was indicated for all patients, and they were divided into two groups according to postoperative length of stay (LOS): the normal duration group (LOS ≤5 days) and the long duration (LD) group (LOS ≥6 days). Multiple regression analysis was used to predict risk factors that identified hospital prolongation to create a LOS prediction score. RESULTS: The normal duration group was 236 patients and the LD group was 134. Seventeen patients (4.6%) required conversion from laparoscopic to open surgery. LOS was 4.82 days in the normal duration group and 12.08 days in the LD group. In the LD group, 18.7% of the patients stayed more than 14 days, but no patients were readmitted. Thirteen clinical factors were statistically different between the two groups. ASA score and LC difficulty were the most predictive risk factors for LOS prolongation. LOS prediction score consisted of eight variables selected from 13 factors; it helped determine the likelihood of whether a patients' hospital stay was prolonged (sensitivity, 82.1%; specificity, 75.0%). CONCLUSION: Thirteen factors closely related to hospital stay duration and LOS prediction score could predict the prolongation of a patient's hospital stay.

A case of retroperitoneal liposarcoma after delivery with expression of estrogen receptor: Report of a case.

INTRODUCTION: Liposarcoma is one of the most common soft tissue sarcomas; however, early diagnosis is rare as the tumor remains difficult and unpalpable for a prolonged period of time. PRESENTATION OF CASE: Here we report the first case of retroperitoneal liposarcoma associated with pregnancy and expression of estrogen receptor. A 34-year-old woman experienced persistent abdominal distension after her first delivery. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a large (40cm×35cm), solid, palpable abdominal mass with fat attenuation displacing the ascending colon and the right kidney to the left. Laparotomy and an en-bloc resection of the tumor were performed; further, right nephrectomy and adrenalectomy were required. Histopathology showed a well-differentiated liposarcoma; approximately 10-20% of the tumor cells were ER-positive. DISCUSSION: Retroperitoneal liposarcoma associated with pregnancy is an extremely rare occurrence. Surgical resection is unquestionably the first choice of treatment, but complete resection is sometimes impossible due to the volume and depth of invasion of the tumor. In such cases, additional therapy for liposarcoma is important to improve prognosis. Thus, this report highlights the need for further research into hormone therapy. CONCLUSION: Retroperitoneal liposarcoma has a high local recurrence rate due to the difficulty in complete surgical resection; therefore, additional hormone therapy is important for improving the prognosis.

Immunodeficiency overview.

Primary immunodeficiencies are challenging in primary care settings, where clinicians often encounter patients with a history of recurrent infection. With advances in diagnostics and therapeutics, these disorders have been better understood and more successfully treated, yet their prognosis depends on early recognition of the disorder and initiation of the appropriate management. Because the primary care physician is most often the first physician encountered by a patient with immunodeficiency, primary care practitioners should be familiar with these rare but important disorders. This article provides an overview of the diagnosis and treatment of primary immunodeficiencies and two of the most common primary immunodeficiencies: common variable immunodeficiency and selective IgA deficiency.

[A case of unresectable gastric cancer complicated by serious pyloric stenosis in which S-1 administration after gastrojejunostomy proved effective].

We report a patient with unresectable gastric cancer who was effectively treated with S-1 after gastrojejunostomy. A 64-year-old man was referred to our hospital for anorexia and epigastric palpable mass. Upper gastrointestinal endoscopy revealed an ulcerous tumor in the antrum of the stomach, and gastric roentgenography showed pyloric stenosis. CT showed simultaneous multiple liver metastases. We performed gastrojejunostomy to allow oral intake. He was treated with daily oral administration of 120-80 mg S-1 for two weeks followed by one week rest as one course. The treatment was repeated for 19 courses until remission was observed. Weekly paclitaxel therapy (80 mg/m(2)/week) was then chosen as second-line chemotherapy. Administration was continued for three weeks with one-week rest. The treatment course was repeated for 6 courses. Bi-weekly administration of CPT-11 (80 mg) and CDDP (30 mg) was chosen as third-line chemotherapy. He died of progressive disease 2 years and 2 months after surgery. During chemotherapy, he maintained a performance status of 0 to 1, and maintained quality of life. This case suggested that the gastrojejunostomy was a useful method for treating unresectable gastric cancer, allowing the possibility of oral intake, and the use of S-1.

Surgical strategy for advanced gastric cancer with a concomitant thoracoabdominal aortic aneurysm requiring arterial reconstruction of the visceral branches.

A 70-year-old man who presented with hematemesis was found to have advanced gastric cancer concomitant with a thoracoabdominal aortic aneurysm (TAAA), which involved all branches of the visceral arteries. The patient underwent the following staged operations: first, radical resection of the advanced gastric cancer with simultaneous reconstruction of the visceral branches, followed 1 month later by endovascular aortic replacement of the TAAA. He recovered uneventfully and was discharged without any paralytic complications or sign of graft infection.

Pulmonary infection with Mycobacterium neoaurum identified by 16S ribosomal DNA sequence.

Mycobacterium neoaurum infection has rarely been found in humans, and only a limited number of cases have been reported. We describe the first case of pulmonary infection with M. neoaurum. We speculate that unrecognized aspiration and long-term corticosteroid therapy predisposed our patient to this rare mycobacterial infection.

Possible role of human herpesvirus 8 in the lymphoproliferative disorders in common variable immunodeficiency.

Patients who have common variable immunodeficiency (CVID) and granulomatous/lymphocytic interstitial lung disease (GLILD) are at high risk for early mortality and B cell lymphomas. Infection with human herpes virus type 8 (HHV8), a B cell lymphotrophic virus, is linked to lymphoproliferative disorders in people who have secondary immunodeficiencies. Therefore, we determined the prevalence of HHV8 infection in CVID patients with GLILD. Genomic DNA isolated from peripheral blood mononuclear cells was screened by nested- and real time-quantitative PCR (QRT-PCR) for the presence of HHV8 genome. It was positive in 6/9 CVID patients with GLILD (CVID-GLILD), 1/21 CVID patients without GLILD (CVID-control), and no patients receiving intravenous gamma globulin (n = 13) or normal blood donors (n = 20). Immunohistochemistry (IHC) demonstrated expression of the latency-associated nuclear antigen-1 (LANA-1) in the biopsies of the lung, liver, and bone marrow of four patients with CVID-GLILD. One CVID-GLILD patient developed a B cell lymphoma during the course of the study. QRT-PCR demonstrated high copy number of HHV8 genome and IHC showed diffuse staining for LANA-1 in the malignant lymph node. HHV8 infection may be an important factor in the pathogenesis of the interstitial lung disease and lymphoproliferative disorders in patients with CVID.

Granulomatous disease in common variable immunodeficiency.

Common variable immunodeficiency (CVID) is a primary immunodeficiency of unknown etiology characterized by low levels of immunoglobulin (Ig)G, failure to make specific antibodies in response to infection or immunization, and variable T-cell abnormalities. Multisystemic granulomatous disease is a well-documented complication of CVID, and its presence is associated with significant morbidity and early mortality. Although the lung is the most common organ system affected, granulomas are also found frequently in other organs, including skin, liver, spleen, and the gastrointestinal tract. Autoimmune disorders are common in these patients, and there appears to be an increased propensity to develop lymphoproliferative disorders. Common physical, radiographic, and laboratory abnormalities in patients with CVID and granulomatous disease include splenomegaly, hilar and mediastinal lymphadenopathy with ground glass or nodular opacities in the lung parenchyma, and reduced T-cell numbers and function. The etiology of granulomatous disease in patients with CVID is unknown, and optimal treatment of granulomatous disease in CVID remains to be established. Further studies are needed to elucidate the underlying etiology of granulomatous lymphoproliferative interstitial lung disease and to delineate appropriate treatments for this disease.