Clinical Significance of Adjuvant Surgical Resection for Initially Unresectable Pancreatic Cancer Responsive to Arterial Infusion Chemotherapy.

BACKGROUND/AIMS: We have reported a clinically meaningful local-control effect and a hepatic metastatic tumor-regression effect of transcatheter peripancreatic arterial embolization-hepatic and splenic arterial infusion chemotherapy (TPPAE-HSAIC) for unresectable advanced pancreatic cancer. The aim of this study was to evaluate the clinical significance, of adjuvant surgical resection after TPPAE-HSAIC. METHODOLOGY: We assessed histopathological findings and outcomes of 6 patients who underwent surgical resection of tumors judged to be radically resectable after attaining tumor down-staging or long-term tumor control following TPPAE-HSAIC for pancreatic cancer initially diagnosed as unresectable. RESULTS: Clinical stage at the initial diagnosis was T4N0M0 Stage III in 4 patients and T4N0M1 Stage IV in 2 patients. The durations of TPPAE-HSAIC ranged from 5 to 46 months with a median of 19 months. An R0 resection was performed in 5 of the 6 patients (83%) and pathological down-staging, from the viewpoint of clinical stage, was observed in 4 patients. Of the 5 patients with R0 resection, one died from a postoperative complication at 7 months and another from pulmonary metastasis at 30 months post-operatively, while the other 3 patients have survived for 45 to 83 months to date. CONCLUSIONS: If surgical resection of pancreatic cancer initially diagnosed as unresectable can be carried out in patients responding favorably to TPPAE-HSAIC, the likelihood of long-term survival might be increased.

[Primary diffuse large B-cell lymphoma of the uterine cervix successfully treated with rituximabplus cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy-a case report].

Primary malignant lymphoma of the uterine cervix is a rare disease, and the therapeutic strategy has not been clearly established. A 45-year old woman presented with vaginal bleeding and hypermenorrhea in January 2012. Physical examination revealed a mass in the pelvic cavity approximately the size of a neonate's head. Pelvic magnetic resonance imaging(MRI) showed a solid mass 11 cm in size in the uterine cervix with homogeneous low intensity on T1-weighted images, iso-high intensity on T2-weighted images, and heterogeneous iso-high intensity on gadolinium-diethylenetriaminepentaacetate(Gd- DTPA)-enhanced images. Multiple lymphadenopathy were also detected in the pelvis. The Papanicolaou smear indicated class 5 cervical cytology, and a subsequent histological examination by a punch biopsy of the cervix showed diffuse infiltration of medium- to large-sized mononuclear cells that stained positive for CD20 and CD79a and negative for CD3, CD5, and EBER. Bone marrow biopsy revealed no abnormality. Positron emission tomography-computed tomography(PET-CT)showed strong fluorodeoxyglucose(FDG)accumulation in the uterine cervix mass, and in the pelvic and right inguinal lymphadenopathy. The patient was diagnosed with diffuse large B-cell lymphoma of the uterine cervix, Ann Arbor stage II AE. She was successfully treated with 8 courses of rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone(R-CHOP) chemotherapy, and maintains a complete remission.

[Successful rituximab treatment for acquired amegakaryocytic thrombocytopenic purpura complicated with Coombs-negative autoimmune hemolytic anemia].

Acquired amegakaryocytic thrombocytopenic purpura (AATP) is a rare disorder characterized by severe thrombocytopenia associated with total absence or a selective decrease in bone marrow megakaryocytes. A 67-year-old male presented with a 2-month bleeding tendency. He was referred to our hospital because of severe thrombocytopenia. Bone marrow biopsy showed complete absence of megakaryocytes without dysplasia in cells of the myeloid and erythroid lineages. AATP was diagnosed. In addition, mild normocytic normochromic anemia and reticulocytosis were also observed and haptoglobin was below the detectable level. Coombs-negative autoimmune hemolytic anemia (AIHA) was diagnosed based on the high titer of RBC-bound IgG and negative direct and indirect coombs test results. He was first treated with cyclosporine 200 mg per day and subsequently with prednisolone but only slight temporary improvement was achieved. Administration of eight doses of rituximab 375 mg/m(2) per week ameliorated both thrombocytopenia and anemia. AATP should be considered in the differential diagnosis of thrombocytopenia, and immunosuppressive therapy is a potential first-line treatment. This is the first case report of AATP accompanied by AIHA successfully treated with rituximab.

[Loss of CD23 expression after bortezomib plus dexamethasone therapy in CCND1/IGH-positive multiple myeloma].

A 69-year-old male was referred to our hospital because of anemia, renal insufficiency, and a positive urine test for Bence-Jones protein. A bone marrow examination showed 73.7% of myeloma cells with lymphoplasmacytic morphology, the strong expressions of CD20 and CD23 by flow cytometry, and the chromosomal aberration of CCND1/IGH by FISH analysis. He was diagnosed with multiple myeloma, IgG-λ type. The initial treatment with bortezomib plus dexamethasone (BD) provided a rapid decrease in the level of IgG; however, he developed bortezomib-induced recurrent paralytic ileus accompanied by aspiration pneumonia during the second course. Interestingly, CD23 expression on myeloma cells decreased from 87.7% to 2.2% after 2 courses of BD. Negative CD23 expression was maintained following lenalidomide plus dexamethasone therapy. There are extremely few reports on CD23 expression on myeloma cells, and this is the first case report of multiple myeloma in which CD23 expression was lost after BD therapy.

[A case of anal variceal bleeding successfully treated with endoscopic injection sclerotherapy].

We report a case of anal variceal bleeding successfully treated with endoscopic injection sclerotherapy (EIS). A 64-year-old man with alcoholic liver cirrhosis was hospitalized because of repeated anal bleeding. Colonoscopy revealed external anal varices connecting with rectal varices. Three days after admission, external anal variceal bleeding was observed. Angiography revealed that the anorectal varices formed by hepatofugal inferior mesenteric vein drained into the internal iliac vein. On angiography, the variceal blood flow rate was extremely low, therefore we performed EIS. Seven days after therapy, thrombosis of anorectal varices was observed.

AICA syndrome with facial palsy following vertigo and acute sensorineural hearing loss.

We report a case of infarction of the anterior inferior cerebellar artery (AICA) with peripheral facial palsy following vertigo and acute sensorineural hearing loss. A 39-year-old female presented with vertigo and sudden hearing loss, tinnitus, and aural fullness of the right ear. An audiogram revealed a severe hearing loss at all tested frequencies in the right ear. Spontaneous nystagmus toward the left side was also observed. Otoneurological examinations showed sensorineural hearing loss of the right ear and horizontal and rotatory gaze nystagmus toward the left side, and a caloric reflex test demonstrated canal paresis. Initially, we diagnosed the patient for sudden deafness with vertigo. However, right peripheral facial palsy appeared 2 days later. An eye tracking test (ETT) and optokinetic pattern test (OKP) showed centralis abnormality. The patient's brain was examined by magnetic resonance imaging (MRI) and magnetic resonance angioglaphy (MRA) and showed an infarction localized in the pons and cerebellum. MRI and MRA revealed infarction of the right cerebellar hemisphere indicating occlusion of the AICA. Consequently, the patient was diagnosed with AICA syndrome but demonstrated regression following steroid and edaravone treatment. We suggest that performing MRI and MRA in the early stage of AICA syndrome is important for distinguishing cerebellar infarction resulting from vestibular disease.

Clinical features of functional hearing loss with inattention problem in Japanese children.

OBJECTIVE: Functional hearing loss is a condition in which hearing is lost without actual pathology. In children, inattention during pure tone audiometry may be due in part to functional hearing loss. This study examined the issue of inattention as a psychological trait by analyzing the clinical features of functional hearing loss children in Japan. METHODS: Using the ADHD-Rating Scale IV, 97 functional hearing loss children were screened for inattention (27 boys, 70 girls; mean age 9 years 5 months, range 5-17 years). Those with high levels of inattention (Inattention group) were compared with others (Attention group) for clinical features statistically. Furthermore observed psychological clinical features were described. RESULTS: 36.1% were categorized in the Inattention group, which had more boys, lower in age, and had more physical, developmental, and environmental problems than the Attention group. Two groups had very different psychological feature. CONCLUSIONS: In children's functional hearing loss, there exists a group with psychological trait of inattention. Three younger children 5-6 years old with attention problems showed no psychological problems, their FHL was considered to be caused by generalized maturation and development. Nearly all of the rest children showed psychological problems, supporting the notion that FHL in children is psychogenic in nature. Because clinical features in Inattention group children were different from the Attention group significantly, it was concluded that distinguishing the Inattention group as a subtype of functional hearing loss in children would be effective for both diagnosis and treatment. Larger scale studies with many angles needed for the inattention problem in FHL children.

Pulsatile tinnitus as a first symptom of essential thrombocythemia.

Tinnitus is the sensation of sound inside the head and is a common symptom encountered daily by otorhinolaryngologists. Pulsatile tinnitus sufferers hear rhythmical noise at the same rate as a heartbeat and can present a diagnostic challenge. In this report, we present a 32-year-old patient with pulsatile tinnitus that led to the diagnosis of essential thrombocythemia. The symptom of pulsatile tinnitus allowed an early diagnosis of essential thrombocythemia and a more favorable prognosis. The case demonstrates the importance of blood tests for all patients who present with pulsatile tinnitus of unknown origin.

[A case of early esophageal adenocarcinoma arising from esophageal cardiac glands].

A 54-year-old man was referred to our hospital with an esophageal lesion. On esophagogastroduodenoscopy a pedunculated elevated lesion about 15mm in diameter was identified in the abdominal esophagus. The surface of the lesion had irregular nodules with white coating. We found no Barrett's mucosa around the lesion. A biopsy specimen of this tumor showed well-differentiated adenocarcinoma. A lower esophagus-proximal gastrectomy was performed because this tumor was suspected to invade the submucosal layer. Eventually, the histological diagnosis after resection was esophageal adenocarcinoma arising from cardiac glands, limited to the mucosal layer (M2). This origin of the esophageal adenocarcinoma is rare. Furthermore, this form of the tumor is especially unusual, and there have been few reports of similar cases.

Adenocarcinoma of Meckel's diverticulum diagnosed by capsule endoscopy and single-balloon enteroscopy.

A case of adenocarcinoma arising in Meckel's diverticulum in a 58-year-old man is reported. Abdominal computed tomography and ultrasonography showed a solid tumor in the middle of abdomen. Capsule endoscopy (CE) showed tumorous lesion in the distal ileum. Single-balloon enteroscopy (SBE) subsequently showed the tumor in Meckel's diverticulum. Furthermore, biopsy specimen obtained from the lesion revealed it as adenocarcinoma. At laparotomy, we found the tumor at the blind end of the diverticulum and enlarged lymph node about 7 cm in diameter in the small intestinal mesentery. Segmental resection of the ileum, including the tumor-bearing diverticulum, was performed along with regional lymph node dissection. Histologically, origin of the tumor was assumed to be ectopic gastric mucosa. Although neoplasm in Meckel's diverticulum is difficult to diagnose preoperatively, the combination of CE and SBE was useful. Based on our search, this is thought to be the first case of neoplasm in Meckel's diverticulum diagnosed endoscopically.

[The effects of GABAergic neurotransmitters and GABAA receptors on the auditory afferent pathway in the brainstem analyzed by optical recording].

AIM: To explore the influence of GABAergic neurotransmitters and GABAA receptors on the auditory afferent impulses recorded in the brainstem evoked by electro-stimulation. METHODS: Brainstem slices were prepared using ddy/ddy mice of postnatal 0-5th days. The brainstem slices were stained with a voltage-sensitive dye(NK3041). The cut end of the vestibulocochlear nerve (nVIIIth) connected with slices was stimulated by a tungsten electrode, a 16 x 16 pixels silicon photodiode array apparatus was used to record the optical mapping from auditory brainstem slices. The data were analyzed by ARGUS-50/PDA software. RESULTS: The spatial-temporal patterns of the excitatory propagation from the vestibulocochlear nerve (nVIIIth) to cochlear nucleus and vestibular nucleus were displayed with multiple-sites optical recording. The optical signal coming from one pixel consisted of a fast spike-like response and a following slow response. Inhibitory neurotransmitter GABA decreased the fast spike-like response and following slow response of evoked optical signals, while an antagonist BMI against GABAA receptors increased the both responses. CONCLUSION: A 16 x 16 pixel silicon photodiode array apparatus can be used to record multiple-sites optical mapping evoked by electro-stimulation to the cut end of the vestibulocochlear nerve. The every optical signal consists of both presynaptic and postsynaptic elements. Inhibitory neurotransmitter GABA and an antagonist BMI of GABAA receptors can modulate the excitatory propagation of evoked optical signals.

Hearing results for ossicular reconstruction using a cartilage-connecting hydroxyapatite prosthesis with a spearhead.

OBJECTIVE: Assessment of the efficacy of ossicular reconstruction using a cartilage-connecting hydroxyapatite prosthesis designed with a spearhead to reduce extrusion and dislocation of the implant. PATIENTS: All patients undergoing ossicular reconstruction after chronic ear surgery, connecting the cartilage to the prosthesis, with a minimum of 1 year of postoperative follow-up. MAIN OUTCOME MEASURES: Postoperative change in pure-tone averages. Air-bone gap closures, and implant extrusion rates. RESULTS: Overall mean pure-tone averages improved by 12.2 dB (ranged between -40 and 60 dB). In total, 68.4% of the patients achieved an air-bone gap less than 20 dB. Gains in the mean air conduction thresholds were 9.5 dB in cases of partial ossicular reconstruction and 14.9 dB in cases with total ossicular reconstruction (p < 0.05). The overall extrusion rate was 4.21%. CONCLUSION: The cartilage-connecting hydroxyapatite prosthesis with a spearhead was found to restore hearing to a satisfactory level. The extrusion rate was relatively low. The cartilage-connecting hydroxyapatite prosthesis with a spearhead is an effective ossicular implant and offers an attractive alternative for ossicular reconstruction, particularly for total ossicular reconstructions.

[A case of unresectable advanced pancreatic cancer with peritoneal dissemination responding to continuous dose S-1 monotherapy after chemo-radiation therapy].

A 57-year-old woman with a pancreatic body tumor was admitted to our hospital. She was diagnosed with unresectable advanced pancreatic cancer (stage IVb) due to portal vein invasion, arterial invasion, retro peritoneal invasion and lymphnode metastases, so radiation therapy (50 Gy/25 Fr) with concurrent arterial infusion of gemcitabine (GEM) was carried out. After the chemo-radiation therapy, her arterial infusion treatment of GEM was continued in our outpatient clinic for 3 months until she was complicated with ascites due to peritoneal dissemination. Peritonitis carcinomatosa was controlled by S-1 oral administration with intraperitoneal infusion of MMC and CDDP. For 18 months after discharge, she has maintained good quality of life without any adverse effects by a continuous dose of S-1 at our outpatient clinic.

[Transcatheter embolization of an inferior mesenteric arteriovenous fistula with frequent mucous diarrhea].

We report a case of inferior mesenteric arteriovenous fistula without portal hypertension or mesenteric ischemia. A 64-year-old man had developed frequent mucous diarrhea during the previous month. Colonoscopy showed highly edematous mucosa of the rectum. Barium enema demonstrated localized stricture of the same part but no evidence of malignancy. Finally we established a diagnosis by 3D-CT and selective abdominal angiography. Transcatheter arterial embolization was successfully performed. After that, his symptoms gradually improved as all abnormal findings on colonoscopy, barium enema and abdominal CT disappeared.