[Dedifferentiated Liposarcoma Originated from Small Bowel Mesentery Resected under Laparoscopic Surgery-A Case Report].

A 74-year-old woman with severe anemia was presented to our hospital to investigate the cause of the disease. Under investigation, submucosal tumor in the small intestine was suspected. We performed the laparoscopic surgery for resection. The pathological diagnosis was dedifferentiated liposarcoma originated from the small bowel mesentery.

[A Case of Radiation Recall Dermatitis That Was Difficult to Distinguish from Inflammatory Breast Recurrence].

The case involved a 51-year-old woman who was diagnosed with Stage Ⅰ right breast cancer(cT1, N0, M0). Partial resection of the right breast and sentinel lymph node biopsy were performed. The histological type was found to be Stage Ⅰ triple-negative medullary carcinoma with pT1c, pN0(sn), and M0. A pituitary tumor was diagnosed after discharge. After removal of the pituitary tumor, whole-breast irradiation was performed. Subsequently, chemotherapy was started. Approximately 5 months after surgery, redness and swelling of the right breast were observed. Inflammatory breast cancer recurrence could not be ruled out by imaging, and skin biopsy was performed. No malignant findings were observed, and the symptoms were considered to indicate radiation recall dermatitis caused by chemotherapy. When chemotherapy was discontinued, the redness of the right breast improved.

[A Case of Localized Amyloidosis of the Right Ureter Treated Effectively by Occulusive Dressing Technique Therapy Using Dimethyl Sulfoxide : Long-Term Follow-Up of Eight Years].

The patient was a 56-year-old female. She was referred to our department for further examination of right hydronephrosis in 2010. Computed tomography (CT) showed right hydronephrosis, and retrograde pyelography (RP) revealed stenosis of the right lower ureter. Urine cytology was negative. Transurethral biopsy of the right ureter was performed using ureteroscopic cup forceps and the histopathlogical diagnosis was ureteral amyloidosis. A whole-body search was performed, including rectal biopsy, but no evidence of amyloidosis was obtained. She was diagnosed with localized amyloidosis of the right ureter. A ureteral stent was indwelled and the patient was given occulusive dressing technique (ODT) therapy using dimethyl sulfoxide (DMSO) for 1 year. After ODT therapy, right hydronephrosis improved. After a 2-year followup, it worsened. ODT therapy was restarted and continued for 2 years. She consulted our department because of fever and right lumbago in April 2017 after a 4-month interruption of ODT therapy. CT revealed progression of the right hydronephrosis. A ureteral stent was indwelled and ODT therapy was restarted. The right hydronephrosis improved after 1 year. ODT therapy using DMSO was effective for localized ureteral amyloidosis, but periodic follow-up was necessary and ODT therapy was also effective when it recurred after the interruption of treatment.

[Autoimmune pancreatitis with bowel obstruction caused by proximal jejunal stricture:a case report].

We herein report a rare case of autoimmune pancreatitis with small intestinal obstruction. A 72-year-old male was admitted to our hospital with abdominal fullness and vomiting and diagnosed with autoimmune pancreatitis by imaging and laboratory tests. Imaging studies also revealed narrowing of the proximal jejunum with dilated bowels and intramural cystic lesion adjacent to the pancreatic body. Small bowel resection was performed to alleviate stenosis. Pathological evaluation demonstrated invasion of IgG4-positive cells and fibrosis.

[A Case of Metastasis of Cholangiocarcinoma to the Bladder].

The patient was an 88-year-old male. He was referred to the Department of Internal Medicine because of total body itching and jaundice in July 2011. The serum bilirubin level was elevated, and the serum CA19-9 level was also elevated to 266. 6 U/ml. Computed tomography (CT) and endoscopic retrograde cholangiopancreatography (ERCP) revealed a solid tumor between the hepatic hilus and common bile duct, and choler cytodiagnosis was class V ; adenocarcinoma. The patient was diagnosed with hilar cholangiocarcinoma and received conservative treatment with endoscopic nasobiliary drainage (ENBD) due to his advanced age. The patient was then referred to our department because CT revealed right hydronephrosis and thickening of the right side of the bladder wall, which had not been detected on admission in October 2011. Cystoscopy revealed a broad-based edematous tumor on the right side of the bladder. Transurethral resection of the bladder tumor (TURBT) was performed. The histological diagnosis was moderately differentiated tubular adenocarcinoma. Immunohistostaining using CA19-9 was performed, and cancer cells were positive. The final histology led to a diagnosis of metastasis of cholangiocarcinoma to the bladder. The patient died of liver failure in March 2012.

A CASE OF ADVANCED NON-SEMINOMATOUS TESTICULAR GERM CELL TUMOR ACCOMPANIED PANIC DISORDER.

We report a 33-year-old male with a left advanced non-seminomatous testicular germ cell tumor (NSGCT) accompanied panic disorder. He had experienced palpitation and hyperpnea in crowds in his twenties. He was admitted to the Department of Otorhinolaryngology with the chief complaint of left neck swelling. 18F-fluorodeoxy glucose positron emission tomography/computed tomography (FDG-PET/CT) demonstrated left neck, left supraclavicular, left axillary, and paraaortic lymph node (LN) swelling and left testicular swelling. He was referred to our department. The left testis had enlarged to the size of a fist. He rejected admission at that time, but next day, he was taken to our hospital by an ambulance because he lost consciousness at home. No abnormalities were found in the brain CT and electrocardiogram. He was admitted and left high orchiectomy was performed. The human chorionic gonadotropin (HCG) level had elevated to 9,717 IU/L and alpha fetoprotein level (AFP) had elevated to 427 ng/ml. The histopathological diagnosis was tumors of more than one histological type, mixed forms: seminoma and embryonal carcinoma.He had palpitation and hyperpnea after admission and was diagnosed with panic disorder by a psychiatrist. Psychotropic drugs (fluvoxamine maleate 50 mg/day, alprazolam 0.8 mg/day) were prescribed and the panic attacks disappeared afterwards. The psychiatric social worker supported his mind side. Bleomycin, etoposide, and cisplatin (BEP) therapy was performed for 4 courses. He put on a blanket to his face and came to avoid a conversation with other people during the chemotherapy. He was diagnosed with depression and psychotropic drugs were increased (fluvoxamine maleate 50→75 mg/day, alprazolam 0.8→1.2 mg/day) in quantity.Lymphadenectomies for LN metastases were performed and their histopathological examination revealed the existence of viable embryonal carcinoma in the supraclavicular LN. Etoposide, ifosfamide, and cisplatin (VIP) therapy was performed for 2 courses.The pateint has remained alive without tumor recurrence. Psychotropic drugs were reduced and the recent drug is fluvoxamine maleate 25 mg/day.

[A case of urachal abscess accompanied by a stone].

A 64-year-old woman presented to our hospital with the chief complaints of abdominal pain and appetite loss, and she was admitted to the internal medicine department. Kidney, ureter and bladder X-ray revealed intrapelvic calcification near the bladder, and so, the patient consulted our department. Computed tomography and magnetic resonance imaging revealed an urachal abscess accompanied by a stone. Open surgery was performed under general anesthesia. The mass adhered tightly to the intestine and bladder. The urachal abscess ruptured during the operation, and pus leaked into the intraabdominal cavity. Partial cystectomy was performed to remove the mass completely. The stone existed in the urachal abscess, and its constituents were CaOxa (51%) and CaP (49%). The pathological diagnosis was urachal abscess without malignancy.

[Case of heterochronous triple urogenital cancer (renal cell carcinoma, bladder cancer, prostatic cancer)].

We report a case of a 73-year-old male with heterochronous triple urogenital cancer. The patient was referred to our hospital because serum PSA was elevated (7.0 ng/ml) in 1998. Prostatic needle biopsy revealed prostatic cancer in the right lobe, and total prostatectomy was performed. The histopathological diagnosis was moderately differentiated adenocarcinoma (TlcNOMO). Non-muscle invasive bladder cancer (NMIBC) was detected during an examination for microhematuria in 2002. Transurethral resection of the bladder tumor (TURBT) procedure was performed, and the histopathological diagnosis was grade 2 urothelial carcinoma (pTa). A right renal mass was detected incidentally on follow-up CT for bladder cancer in 2008. Renal enucleation was performed in 2009. The histopathological diagnosis was grade 2 clear cell renal cell carcinoma (pTlaNXMO). NMIBC was detected on follow-up urethrocystoscopy in 2011. The TURBT procedure was performed, and the histopathological diagnosis was grade 2 urothelial carcinoma (pTa). On follow-up for urogenital cancer patients, it is important to investigate recurrence of the primary cancer and also heterochronous canceration of other urogenital organs.

Lumbar Spinal Canal Stenosis in Acromegaly: A Case Report and Literature Review.

A 60-year-old Japanese man diagnosed with acromegaly at 28 years old had difficulty walking due to worsening back pain. He had been treated with somatostatin analog since 57 years old, but his pain and numbness continued to worsen. Lumbar magnetic resonance imaging showed disc bulging at L3/4 and L4/5, and he was diagnosed with lumbar spinal canal stenosis due to hypertrophy of the yellow ligament. Patients with acromegaly may complain of osteoarthropathy, so we must pay attention to the symptoms of spinal canal stenosis in collaboration with orthopedic specialists.

Association between IgG4-related disease and progressively transformed germinal centers of lymph nodes.

Progressively transformed germinal centers is a benign condition of unknown pathogenesis characterized by a distinctive variant form of reactive follicular hyperplasia in lymph nodes. We recently reported Ig G4-related disease in progressively transformed germinal centers. However, no large case series has been reported and clinicopathologic findings remain unclear. Here, we report 40 Japanese patients (28 men, 12 women; median age, 56 years) with progressively transformed germinal centers of the lymph nodes who fulfilled the histological diagnostic criteria for IgG4-related disease (IgG4(+) progressively transformed germinal centers), with asymptomatic localized lymphadenopathy involving the submandibular nodes in 24, submandibular and cervical nodes in 14, cervical nodes only in 1, and cervical and supraclavicular nodes in 1. In all, 16 (52%) of 31 examined patients had allergic disease. Histologically, the lymph nodes demonstrated uniform histological findings, namely marked follicular hyperplasia with progressively transformed germinal centers, and localization of the majority of IgG4(+) plasma cells in the germinal centers. Serum IgG4, serum IgE and peripheral blood eosinophils were elevated in 87%, 92% and 53% of examined patients, respectively. Eighteen patients subsequently developed extranodal lesions (including five who developed systemic disease), which on histological examination were consistent with IgG4-related disease. IgG4(+) progressively transformed germinal centers presents with uniform clinicopathological features of asymptomatic localized submandibular lymphadenopathy, which persists and/or relapses, and sometimes progresses to extranodal lesions or systemic disease. Nine patients were administered steroid therapy when the lesions progressed, to which all responded well. We suggest that IgG4(+) progressively transformed germinal centers should be included in the IgG4-related disease spectrum.

Primary Cardiac Lymphoma Arising from Both Sides of the Heart.

Primary cardiac lymphomas (PCLs) are extremely rare tumors with a poor prognosis. They usually involve the pericardium and the right side of the heart. PCLs arising from the left side of the heart are relatively rare, with bilateral cases being even rarer. We herein report a case of PCL arising from both the right and left sides of the heart in a 65-year-old man. Multiple imaging modalities clearly demonstrated the tumors at the initial evaluation. The pathological diagnosis was diffuse large B-cell lymphoma.

Lymphohistiocytoid mesothelioma of the pleura.

Lymphohistiocytoid mesothelioma (LHM), reported to be a rare variant of sarcomatoid mesothelioma, is challenging to differentiate from non-Hodgkin's lymphoma due to marked lymphocytic infiltration. To aid accurate recognition of LHM, we examined immunohistochemical, in situ hybridization (ISH) of Epstein-Barr virus RNA (EBER-1) mRNA, fluorescence ISH (FISH) for homozygous deletion of 9p21, and asbestos analysis in four cases (three men and 1 woman). Three patients died, while Case 4 was still alive 19 months after extrapleural pneumonectomy. Histologically, these cases were characterized by heavy lymphocytic infiltration. All neoplastic cells were positive for calretinin, AE1/AE3, and epithelial membrane antigen, but negative for CEA. EBER1 factor was negative. FISH analysis demonstrated homozygous deletion of the 9p21 locus in three of the four cases. In Case 1: (i) autopsy findings showed mesothelioma primarily located in the right parietal pleura, but metastasized into the left lung and abdominal organs; (ii) the histological findings at autopsy indicated sarcomatoid mesothelioma; and (iii) we found asbestos bodies and fibers in extracts from lung tissue (Cases 1 & 4) using digestion with bleaching fluid. LHM, an infrequent variant of sarcomatoid mesothelioma, displayed homozygous deletion of the 9p21 locus (three of four cases), and has a relatively favorable prognosis for the sarcomatoid type.

[Long-term survival case of small-cell lung cancer with Lambert-Eaton myasthenic syndrome without anticancer therapy].

A 78-year-old man with complaints of appetite loss and weight loss visited our hospital in November 2006. Positron-emission tomography and computed tomography (PET/CT) showed swollen lymph nodes in the abdominal para-aorta, mediastinum and neck, with intense FDG accumulation. The pathological findings of the cervical lymph nodes revealed small-cell cancer. We diagnosed extensive small-cell lung cancer (SCLC), which occurred primarily in the left upper lobe. As subsequent CT revealed spontaneous shrinkage of the pulmonary nodule and swollen lymph nodes, the clinical course was monitored without anticancer therapy. In February 2007, progressive muscle weakness of the lower extremities developed. In July he was admitted with respiratory failure and required mechanical ventilation. Although we did not administer anticancer therapy due to his poor performance status, he survived for 30 months receiving mechanical ventilation, and the tumors continued to grow moderately. We diagnosed Lambert-Eaton myasthenic syndrome (LEMS) based on the clinical symptoms, the presence of anti-VGCC antibodies and waxing phenomenon on electromyography obtained in April 2009. Chemotherapy with amrubicin shrank the tumors, but his muscle weakness did not improve. Previous reports showed that a prognosis of SCLC with LEMS was better than that without LEMS. In this case, the tumors showed spontaneous regression without any anticancer therapy, and then increased moderately. The immune response was considered to have affected tumor growth.

A Case of Strangulation Ileus Due to a Low-Grade Appendiceal Mucinous Neoplasm.

BACKGROUND Strangulation ileus is caused by external obstruction to the small bowel, which results in ischemia and loss of bowel peristalsis. Low-grade appendiceal mucinous neoplasm (LAMN) is a low-grade adenocarcinoma that arises in the appendix. LAMN is usually asymptomatic but can present with appendiceal rupture and pseudomyxoma peritonei (PMP). This report is of a rare presentation of LAMN with strangulation ileus in a 92-year-old man. CASE REPORT A 92-year-old man was admitted to the emergency room with sudden onset of lower abdominal pain and abdominal distension. Laboratory investigations showed a leukocytosis with a white blood cell (WBC) count of 14.6×10³/µL with 85.5% neutrophils, blood urea nitrogen (BUN) of 26.6 mg/dL, and serum creatinine of 2.6 mg/dL, consistent with acute renal failure. Arterial blood gas analysis showed lactic acidosis (pH of 7.11) with a base excess of -20.8 mmol/L and lactate of 13.7 mmol/L. Abdominal computed tomography (CT) showed ascites and a dilated obstructed closed loop of the distal ileum associated with an external mass (3.9×2.8 cm). An initial diagnosis was of strangulation ileus due to Meckel's diverticulum. Emergency ileocecal resection was performed. Histopathology showed a low-grade mucinous tumor arising from the mucosa of the appendix, consistent with LAMN. At a 13-month follow-up, the patient was well with no tumor recurrence. CONCLUSIONS This report is of a rare case of LAMN that presented as a surgical emergency with strangulation ileus.

[THREE CASES OF TRANSURETHRAL RESECTION OF THE BLADDER TUMOR (TURBT), PERFORMED FOR BLADDER TUMOR ON THE ANTERIOR WALL, WITH BLADDER RUPTURE OCCURRING AFTER DISCHARGE].

Three cases are reported of TURBT on the anterior wall, with bladder rupture occurring after discharge. Patient 1 was a 68-year-old man. He had macroscopic hematuria and he strained to void a bloody clot on the 10th day after TURBT. Subsequently, right lower abdominal pain occurred. Computed tomography (CT) revealed the extravasation of contrast medium into the prevesical space. He was diagnosed with extraperitoneal bladder rupture, and a urethral catheter was indwelled. Cancer invasion of muscle was diagnosed by pathological examination and total cystectomy was scheduled one and a half months later, but the prostate could not be resected due to hard tissue around the bladder neck. Patient 2 was an 82-year-old man and had a history of radiation therapy for a muscle invasive bladder tumor. He complained of pollakisuria two weeks after TURBT, and renal failure was detected on a blood test. CT revealed ascites, and a urethral catheter was indwelled. Ascites disappeared, but the urethral catheter deviated into the abdominal cavity based on repeated CT the next day, and he was diagnosed with intraperitoneal bladder rupture. Emergent surgery was performed, and the ruptured part was sutured with omentum covering and a cystostomy was created. Patient 3 was an 83-year-old man undergoing treatment for benign prostatic hypertrophy (BPH). He had received bladder instillation therapy of Bacillus Calmette-Guerin (BCG) ten months previously. When urinating 6 days after TURBT, lower abdominal pain developed. CT demonstrated retroperitoneal bladder rupture, and a urethral catheter was indwelled. The urethral catheter was removed 6 days later, but lower abdominal pain occurred again the next day. Thus, the urethral catheter was re-indwelled for a further two weeks.In TURBT on the anterior wall or dome, for the patients who had previously received radiation therapy to the pelvis, or intravesical instillation therapy of the BCG or accompanied by urinary disturbance, such as BPH, it is necessary to consider bladder rupture after discharge.

Chemical pleurodesis with autologous blood and freeze-dried concentrated human thrombin improved spontaneous pneumothorax and thoracic endometriosis: The first case involving a pregnant woman.

OBJECTIVE: Spontaneous pneumothorax combined with thoracic endometriosis is a rare condition during pregnancy. We present a case of chemical pleurodesis with autologous blood and freeze-dried concentrated human thrombin during pregnancy. CASE REPORT: This report presents a case of spontaneous pneumothorax combined with thoracic endometriosis that arose at 22 weeks' gestation in a 35-year-old female. The initial chest drainage was unsuccessful. At 25 weeks' gestation, video-assisted thoracoscopic surgery was performed and revealed endometriosis in the thoracic cavity. Since the leak persisted, chemical pleurodesis was performed with autologous blood and freeze-dried concentrated human thrombin at 28 weeks' gestation. The leak improved markedly and did not recur. CONCLUSION: This is the first case report about chemical pleurodesis with autologous blood and freeze-dried concentrated human thrombin during pregnancy. This procedure might contribute to the management of pneumothorax in pregnant women.

ZFH4 protein is expressed in many neurons of developing rat brain.

The zinc finger-homeodomain (ZFH) transcription factors contain a zinc finger motif and a homeodomain that might regulate neural and mesenchymal cell differentiation. We have cloned the ZFH4 gene that encodes a protein with structures closely related to ATBF1. In order to study the expression pattern of ZFH4 in the developing rat brain, we raised an antibody against a glutathione-S-transferase (GST) fusion protein of ZFH4. Western blotting with this antibody identified a gene product of 390 kDa in the normal rat brain. Levels of the protein were high in the brainstem at embryonic and neonatal periods and in the midbrain and diencephalon in neonatal rat brain. In addition, the corresponding mRNA of 12.5 kb was detected by Northern blotting. An immunolocalization study showed that postmitotic neurons in the brainstem were the major site of ZFH4 expression, and the levels of expression varied depending on age and anatomical sites. Expression was transient and weak in precursor cells at early neurogenesis. Although ZFH4 levels decreased after birth, ZFH4 continued to be expressed in the mature neurons including DOPA decarboxylase-positive neurons. High levels of expression were also detected in non-neuronal cells of the subcommissural organ, but the expression was almost undetectable throughout precursor cells to mature neurons in the cerebral cortex and hippocampus. The spatial and temporal expression patterns closely resembled those of ATBF1, and we detected neurons that expressed ZFH4, ATBF1, or both. We postulate that ZFH4 participates in the regulation of neural cell maturation or of region-specific differentiation of the brain.

Abundant IgG4-positive plasma cell infiltration characterizes chronic sclerosing sialadenitis (Küttner's tumor).

Chronic sclerosing sialadenitis (CSS) is a cryptogenic tumor-like condition of the salivary gland(s). While immune-mediated processes are suspected in its pathogenesis, and CSS is occasionally reported to be associated with sclerosing pancreatitis, an IgG4-related disease, the exact immunopathologic processes of CSS remain speculative. In this study, we examined the clinicopathologic findings of CSS (12 cases) in comparison with sialolithiasis (8 cases) and Sjogren's syndrome (13 cases), and tried to clarify whether CSS is an IgG4-related disease or not. Submandibular gland(s) were affected in all cases of CSS. CSS cases could be divided into two types: 5 cases were associated with sclerosing lesions in extrasalivary glandular tissue (systemic type), while only salivary gland(s) were affected in the remaining 7 cases (localized type). In the former type, which showed male predominance, bilateral salivary glands were frequently affected, and eosinophilia and elevations of gamma-globulin and IgG in serum were frequently found. Histologically, all cases of CSS showed marked lymphoplasmacytic infiltration admixed with fibrosis and the destruction of glandular lobules. Obliterative phlebitis was found in the affected salivary glands in all cases of CSS. Immunohistochemically, the proportion of IgG4/IgG-positive plasma cells was more than 45% in CSS, while it was less than 5% in controls. The resemblance of the clinicopathologic features of CSS with those of sclerosing pancreatitis suggests the participation of a similar immunopathologic process with IgG4 disturbance in CSS. The abundance of IgG4-positive plasma cells in the lesions would be useful for distinguishing CSS from other forms of sialadenitis.

ATBF1-A protein, but not ATBF1-B, is preferentially expressed in developing rat brain.

The ATBF1 gene encodes transcription factors containing four homeodomains and multiple zinc finger motifs. However, the gene products have yet to be identified and the role remains unknown in vivo. In this study, we raised an antiserum for ATBF1 and found high levels of expression of ATBF1 in developing rat brain. Western and Northern blot analyses detected a 400 kDa protein and 12.5 kb mRNA in developing rat brain, respectively; both corresponding to ATBF1-A but not the B isoform. The protein was highly expressed in the midbrain and diencephalon and mRNA was highly expressed in the brainstem, mostly in embryo and neonatal brain. Immunohistochemistry identified postmitotic neurons in the brainstem as the major site of ATBF1 expression, and the expression levels varied depending on age of and location in the brain. Expression was transient and weak in the precursor cells at early neurogenesis. ATBF1 decreased postnatally, but remained in mature neurons, including those expressing DOPA decarboxylase (DDC). High levels of ATBF1 were expressed in precursor cells in accordance with neurogenesis and were continued to the mature neurons in specific areas such as the inferior colliculus. Expression was not significant from precursor cells to mature neurons in the cerebral cortex and hippocampus. ATBF1 and its Drosophila homolog, Zfh-2, are known to regulate cell differentiation and proliferation via the interaction with either of the basic helix-loop-helix transcription factors, c-myb, or the DDC gene. Together with these reported functions the expression features detected here suggest that ATBF1 may participate in the regulation of neuronal cell maturation or region-specific central nervous system differentiation.

A case of myomatous erythrocytosis syndrome associated with a large uterine leiomyoma.

Several etiologies have been proposed for erythrocytosis associated with uterine leiomyoma. We report a case of erythrocytosis associated with a large uterine leiomyoma, in which specific immunostaining for erythropoietin was positive. A 55-year-old woman, gravida 0, para 0, was referred to our hospital for treatment for a large uterine myoma and erythrocytosis. She had no vaginal bleeding after she reached menopause at 50 years old. She had severe polycythemia: hemoglobin (Hb), 19.9 g/dL; red blood cell count (RBC), 6.65 × 10(6)/mm(3); hematocrit, (Hct) 59.1%. An abdominal simple hysterectomy was performed, and a pathological examination confirmed the diagnosis of leiomyoma of the uterus. In addition, immunostaining demonstrated that the cytoplasm of the leiomyoma cells was strongly positive for erythropoietin. After the operation, the patient's hemoglobin and hematocrit levels normalized, and we diagnosed her condition as myomatous erythrocytosis syndrome.