[Development of Cancer of the Remnant Colorectal Segment after Ileal-Pouch Anal Anastomosis/Ileorectal Anastomosis in Patients with Familial Adenomatous Polyposis].

PURPOSE: This retrospective study was performed to investigate the recent trend of occurrence of cancer of the remnant colorectal segment(RCRS)after ileal-pouch anal anastomosis(IPAA)/ileorectal anastomosis(IRA)and to consider the optimal surveillance methods in patients with familial adenomatous polyposis(FAP)undergoing(procto)colectomy. PATIENTS AND METHODS: The subject was a total of patients with FAP undergoing IPAA or IRA between 2005 and 2022. Clinicopathological data were extracted from medical charts and analyzed. Cumulative incidence of cancer in the RCRS and overall survival after treatment of such tumors were calculated by the Kaplan-Meier method. RESULTS: There were 45 male and 56 female. IPAA was performed in 49 patients(hand-sewn; n=33, stapled; n=16)and IRA was performed in 52 patients. The median age at initial colorectal surgery was 32 years old(range, 13-66 years old). Median postoperative follow-up was 11 years(range, 1-48 years). Eighty-one patients were confirmed to have pathogenic variant of APC by genetic test. The cumulative incidence of cancer of the RCRS did not differ between patients undergoing IPAA and those undergoing IRA(p= 0.73, 4.1% versus 1.9% at 10 years). The cumulative 5-year overall survival rate after additional surgery for the tumor of RCRS was 82%. CONCLUSION: This study has several limitations due to single institutional retrospective study with small cases and non-standardized postoperative endoscopic surveillance. However, our results seem to show satisfactory oncological outcomes of patients with FAP in terms of the control of cancer of the RCRS under postoperative periodic surveillance, regardless of the type of colorectal resection.

[Complete Response to Immune Checkpoint Inhibitors in Unresectable Gastric Cancer-A Report of Five Cases].

Immune checkpoint inhibitors(ICIs)are widely used for the treatment of unresectable gastric cancer. We treated approximately 70 patients with ICIs. ICI treatment with pembrolizumab was administered for MSI-high cases and nivolumab for MSS cases in the second- or third-line chemotherapy. We observed 5 cases of complete response. Among these, 2 patients presented with liver metastases, 2 with peritoneal disseminations, and 1 with pulmonary metastasis. In 1 patient, the primary tumor invaded the diaphragm and descending aorta; whereas, in another patient the primary tumor invaded the pancreas and liver. All patients had progressive disease after first-line chemotherapy.

[A Rare Case of Cronkhite-Canada Syndrome Associated with Gastric Cancer and Gastric Outlet Obstruction].

Cronkhite-Canada syndrome(CCS)is a rare non-inherited disease characterized by gastrointestinal polyposis and ectodermal abnormalities. We report a rare case of CCS associated with gastric cancer and gastric outlet obstruction with a review of the literature. A 75-year-old man was admitted because of frequent vomiting and hypoproteinemia. He was diagnosed with CCS due to typical clinical and laboratory findings including alopecia, nail atrophy, hypoproteinemia, and typical gastrointestinal polyposis. Upper endoscopic examination also pointed out a large gastric cancer mainly located in the antrum and the reversible pyloric obstruction caused by the gastric tumor. Biopsy of the tumor revealed tubular adenocarcinoma. Computed tomography demonstrated the dilated duodenum caused by packing of the gastric tumor. 1.5 months after prednisolone therapy, he underwent total gastrectomy with complete resection of the dilated duodenal bulb. Histological examination revealed gastric cancer(pap>tub1)classified into Stage ⅢC. Postoperative course was uneventful and he moved to another hospital. To our knowledge, including the present case, there were 20 reported cases of CCS associated with gastric cancer from Japan(1979-2022). Also, 7 cases of CCS associated with gastric outlet obstruction was reported.

AI analysis and modified type classification for endocytoscopic observation of esophageal lesions.

Endocytoscopy (EC) facilitates real-time histological diagnosis of esophageal lesions in vivo. We developed a deep-learning artificial intelligence (AI) system for analysis of EC images and compared its diagnostic ability with that of an expert pathologist and nonexpert endoscopists. Our new AI was based on a vision transformer model (DeiT) and trained using 7983 EC images of the esophagus (2368 malignant and 5615 nonmalignant). The AI evaluated 114 randomly arranged EC pictures (33 ESCC and 81 nonmalignant lesions) from 38 consecutive cases. An expert pathologist and two nonexpert endoscopists also analyzed the same image set according to the modified type classification (adding four EC features of nonmalignant lesions to our previous classification). The area under the curve calculated from the receiver-operating characteristic curve for the AI analysis was 0.92. In per-image analysis, the overall accuracy of the AI, pathologist, and two endoscopists was 91.2%, 91.2%, 85.9%, and 83.3%, respectively. The kappa value between the pathologist and the AI, and between the two endoscopists and the AI showed moderate concordance; that between the pathologist and the two endoscopists showed poor concordance. In per-patient analysis, the overall accuracy of the AI, pathologist, and two endoscopists was 94.7%, 92.1%, 86.8%, and 89.5%, respectively. The modified type classification aided high overall diagnostic accuracy by the pathologist and nonexpert endoscopists. The diagnostic ability of the AI was equal or superior to that of the experienced pathologist. AI is expected to support endoscopists in diagnosing esophageal lesions based on EC images.

[A Case of Curative Resection of a Retroperitoneal Liposarcoma].

A 55-year-old woman had been admitted to a hospital with abdominal bloating. Retroperitoneal liposarcoma was suspected and diagnosed as not resectable. She was then referred to our hospital with dyspnea and difficulties with movement due to the huge mass. An abdominal CT revealed a large mass originating in the left retroperitoneum. The tumor occupied most of the abdominal cavity, resulting in the displacement of her organs. However, there was no evidence of infiltration of the tumor into the aorta and inferior vena cava. Under a provisional diagnosis of retroperitoneal liposarcoma, a surgical resection was undertaken. The resected specimen had a maximum diameter of 48 cm and weighed 14 kg. Histopathological examination revealed a differentiated liposarcoma. The patient remains alive 6 months after the operation, without recurrence.

[A Case of Malignant Peripheral Nerve Sheath Tumor of the Anus Resected by Robot Assisted-APR].

An 81-year-old female visited a local hospital with complaints of anal pain. A tumor was found on the right side of her anus, and the histopathological diagnosis was a non-epithelial malignant tumor. Therefore, the patient was referred to our hospital. Result of imaging inspection revealed that the tumor had invaded the lower rectum, but had not distantly metastasized. Based on the findings of another biopsy, the patient was diagnosed with a malignant peripheral nerve sheath tumor (MPNST). Robot-assisted abdominoperineal resection(D1)was performed, and the lesion was resected without any pathological remnants. During the postoperative period, the patient developed perineal wound infection. Subsequently, the patient was discharged from the hospital on postoperative day 10. At the 6-month postoperative follow-up, no recurrence was noted. Most MPNSTs occur in the limbs, trunk, and neck. MPNST in the primary gastrointestinal tract or in the vicinity of the gastrointestinal tract is relatively rare, and in principle, combined resection of the intestinal tract is required for surgical treatment. Here, we report a case of MPNST that occurred near the anus and infiltrated to the lower rectum and was completely resected by robot-assisted abdominoperineal resection.

[Conversion Surgery following Second-Line Chemotherapy for Unresectable Gastric Cancer].

We retrospectively analyzed clinicopathological and survival data of 8 patients with unresectable gastric cancer who underwent conversion surgery(CS)following second-line chemotherapy from April 2013 to December 2020. There were 7 males and 1 female patients with a median age of 69(64-76)years old. Five patients had 1 unresectable factor, 2 had 2 unresectable factors, and 1 had 3 unresectable factors. All patients achieved R0 resection. The median survival time(MST) of patients with CS following second-line chemotherapy was significantly longer than that without CS(73.4 vs 12.3 months, respectively). The MST of patients with CS following first-line chemotherapy was significantly longer than that without CS (22.9 vs 12.3 months, respectively). This study suggested that CS following first- or second-line chemotherapy may improve survival duration for unresectable gastric cancer.

[Two Cases of Esophageal Neuroendocrine Carcinoma That Were Successfully Controlled by Multi-Modality Therapy].

Esophageal neuroendocrine carcinoma is extremely rare, and its treatment strategy has not been established. We report 2 cases esophageal neuroendocrine carcinoma. Case 1: A 74-year-old man was diagnosed as having esophageal neuroendocrine carcinoma(clinical T3N4M0, Stage Ⅳa). He received 60 Gy of radiation therapy with etoposide(100 mg/m2)plus cisplatin(80 mg/m2). No recurrence has been detected 1 year after treatment. Case 2: A 78-year-old man was diagnosed as esophageal neuroendocrine carcinoma(clinical T3N0M0, Stage Ⅱ). He underwent esophagectomy with 3 field lymph nodes dissection. Adjuvant chemotherapy was administered with irinotecan(60 mg/m2)plus cisplatin(60 mg/m2). After chemotherapy, he survived 1 year without recurrence.

[Three Cases of Conversion Surgery for Unresectable Gastric Cancer with Multiple Liver Metastases].

We herein report 3 cases of advanced gastric cancer with multiple liver metastases who was successfully treated with systemic chemotherapy and underwent conversion surgery with liver resection. There were 2 males and 1 female patients with a range 68 to 74 years of age. Two patients received S-1 plus oxaliplatin therapy and 1 received S-1 plus cisplatin therapy. All patients survived after 5-49 months postoperatively.

[A Case of Advanced Recurrent Colorectal Cancer with Complete Response to Pembrolizumab Chemotherapy].

The efficacy of pembrolizumab has been demonstrated for all solid tumors showing high frequency microsatellite instability- high(MSI-High). It is a possible treatment option even in cases which do not respond to other forms of chemotherapy. We report a case of a 69-year-old man with MSI-High recurrent colorectal cancer with complete response(CR)after pembrolizumab therapy. Sigmoidectomy, extensive lymph node dissection, and partial bladder resection were performed for sigmoid colon cancer at another hospital. Histopathological examination revealed a T4a, N0, M0, Stage Ⅱb tumor. Six months after the operation, Hartmann's operation and partial resection of the small intestine were performed for local recurrence. However, the tumor invading the retroperitoneum was unresectable. Postoperative SOX therapy was performed, but it was discontinued due to Grade 3 diarrhea during the first course. The laboratory test showed MSI-High during the first course. Pembrolizumab chemotherapy was introduced as second-line therapy. Computed tomography examination after 2 courses (6 weeks)revealed reduction in the major axis of the tumor by 30% or more. After 4 courses(12 weeks), the tumor was further reduced, and a partial response(PR)was diagnosed. The tumor completely disappeared after 6 courses, and a complete response was achieved after 8 courses. The CR has been maintained for about 7 months.

[A Case of Low-Grade Appendiceal Mucinous Neoplasm Complicated by Pseudomyxoma Peritoneri Successfully Treated with Cytoreductive Surgery and Intraperitoneal Chemotherapy].

We experienced a case of low-grade appendiceal mucinous neoplasm complicated by pseudomyxoma peritonei that was successfully treated with cytoreductive surgery and early postoperative intraperitoneal chemotherapy. The patient was a 26- year-old man with massive ascites and a swollen appendix on the computed tomography(CT). The appendix was a cystic mass of 5 cm in size. The entire parietal peritoneum, omentum, stomach, spleen, gall bladder, and entire colon were covered with numerous mucous nodules. Total colectomy, total gastrectomy, splenectomy, cholecystectomy, total omentectomy, parietal peritonectomy, ileostomy, and intraperitoneal irrigation were performed. The pathological diagnosis was low-grade appendiceal mucinous neoplasm. Postoperative intraperitoneal chemotherapy with cisplatin and mitomycin C was performed. A residual tumor was found on the dorsal side of the hepatoduodenal ligament 3 months postoperation on the CT. The residual tumor was successfully excised via a concomitant resection of the hepatic caudate lobe. Postoperative intraperitoneal chemotherapy was then performed. No recurrence was found at 8 months postoperation. The addition of early postoperative intraperitoneal chemotherapy improved the patient's quality of life in a short period. This could be one of the treatment options.

Anorexia nervosa-associated pancytopenia mimicking idiopathic aplastic anemia: a case report.

BACKGROUND: Patients with anorexia nervosa (AN) often present with pancytopenia. In most cases described in the literature, AN with pancytopenia demonstrates gelatinous marrow transformation (GMT), which is a typical bone marrow feature of malnutrition. Differentiation of AN-associated pancytopenia from other types of pancytopenia, especially idiopathic aplastic anemia (IAA), has not been studied. We encountered a case of pancytopenia in a patient with AN and relatively poor nutritional status, whose hematological findings mimicked those of IAA, specifically fatty bone marrow and absence of GMT. CASE PRESENTATION: The patient was a 32-year-old woman with poorly controlled AN. At 31 years of age, her body mass index (BMI) had fallen from 17.0 kg/m2 to below 13.8 kg/m2. The patient presented with ongoing fatigue and thus was examined by a hematologist. Hematological findings were consistent with IAA: peripheral blood tests revealed pancytopenia, whereas the bone marrow displayed fatty replacement without GMT. Despite the absence of bone marrow features typically seen in malnutrition, the patient's hematological abnormalities had manifested after a decrease in body weight. Thus, although the bone marrow findings indicated IAA, we considered that the nutritional etiology of pancytopenia could not be thoroughly ruled out. Using nutritional therapy alone, the hematological abnormalities improved as BMI increased to 16.5 kg/m2. The final diagnosis was pancytopenia secondary to malnutrition because pancytopenia and fatty bone marrow improved after implementation of nutritional therapy alone. CONCLUSIONS: The present case is the first documented case of AN with pancytopenia for which bone marrow examination confirmed fatty marrow without any evidence of GMT. IAA and pancytopenia secondary to malnutrition can present the same clinical findings. This case is significant because it suggests a need to differentiate between malnutrition and IAA.

[Specified expectant mother, Child Welfare Act, and community cooperation: the role of psychiatrists].

In 2009, the amended provisions of Article 6 of the Child Welfare Act defined "Specified Expectant Mother" (Tokutei-ninpu) as an expectant mother with the need for potential support in nurturing her baby before its delivery. This term was subsequently supplemented with relevant notices and manuals of the Ministry of Health, Labor, and Welfare; it then became a new target of the community network built for the prevention of child abuse. The two notices issued on November 30, 2012 are of great significance. One notice indicates that pregnant females should be screened for psychiatric disorders as well as some other risk factors by officers of their municipalities as a precaution against child abuse. The other states that in the context of Article 5 of the Child Abuse Prevention Law, medical service workers are immunized from sanctions if they divulge privileged information about a "Specified Expectant Mother" with the potential risk for child abuse to municipalities without patient approval. As a result of the latter notice, medical staff must judge whether the privacy of a parent or health of an unborn child with potential risk is more worthy of protection. However, the official criteria for judging the potential for child abuse and those who fall into the category of "Specified Expectant Mother" have yet to be specified. Fetal abuse, as defined by Condon in 1986, serves as a suitable surrogate marker for possible harmful actions that would justify public community cooperation without patient approval. Parenting capacity has rarely been discussed in the field of child abuse and neglect in Japan. The capacity is a legal requirement for custody. Considering the capacity of a "Specified Expectant Mother" to prevent child abuse and neglect, health care workers supporting pregnant women with psychiatric disorders or intellectual disabilities should deliberate, first, their capacities to appreciate their situations and, then, their parenting capacities. A custody substitution system for children of incompetent parents is clearly indicated for minors in the Civil Code (Article 833 and 867); however, it ignores psychiatric disorders and intellectual disabilities. The rights of children, which are lost in this omission, must be guaranteed by competent persons, usually by child guardians. Contributions of psychiatrists are essential for applying legal protections. Without these contributions, their rights are voided of all substance. Advocacy for such pregnant females requires wide and precise knowledge of relevant laws and ethics.

Improved functional status by comprehensive physical and psychosocial approach through right insula activation in poststroke vascular dementia.

The aim is to investigate the effect of a comprehensive physical and psychosocial approach on functional outcome and cerebral glucose metabolism in poststroke vascular dementia (PSVaD). Ten PSVaD patients participated in the study. They were diagnosed according to the National Institute of Neurological Disorders and Stroke and Association Internationale pour la Recherché et l'Enseignement en Neurosciences (NINDS-AIREN) criteria and needed physical assistance in sit-to-stand transfer activities. Six were enrolled in a comprehensive program consisted of an individualized task-specific exercise regimen of transfer training and a psychosocial intervention program. The other 4 patients participated in the control group. The programs were undertaken over a period of 2 months. Outcomes were the scores on the Mini-Mental State Examination and the Functional Independence Measure (FIM), and on cerebral glucose metabolism determined by (18)F-fluorodeoxyglucose positron emission tomography performed before and at the end of the program. The score on the transfer mobility subscale of the FIM increased at the end of the program in all patients who received the comprehensive program. Regional glucose metabolism was increased in the right insular cortex at the end of the combined program. Control patients showed no change in FIM score or regional cerebral metabolism. A combined approach may be associated with an increase in glucose metabolism of the right insula cortex in PSVaD patients.

Greater responsiveness to donepezil in Alzheimer patients with higher levels of acetylcholinesterase based on attention task scores and a donepezil PET study.

The aim of the study was to predict donepezil responders among patients with Alzheimer disease (AD) based on cognitive tests and positron emission tomography. The Mini-Mental State Examination, Digit Symbol subtest (DigSm) of Wechsler Adult Intelligence Scale Revised, and Trail-Making Test A were administered for 80 patients with AD to assess global function, attention, and executive function, respectively. The same tests and the Clinical Global Impression (CGI) scale were conducted after treatment with oral donepezil (5 mg/d) for 6 months (study 1). [C]-Donepezil positron emission tomography examinations were conducted before and after treatment for 30 randomly selected patients. The distribution volume (DV), which indicates the density of donepezil-binding sites, was calculated using Logan graphical analysis (study 2). In study 1, 35 patients were identified as responders based on the CGI and Mini-Mental State Examination changes. These patients had higher baseline DigSm scores compared with nonresponders. In study 2, 15 patients were responders. DigSm correlated with DV at baseline. DV at baseline and %DV change in responders were higher than in nonresponders, and these variables correlated with ΔDigSm and CGI scores. Higher baseline attention may predict responsiveness to donepezil in patients with AD, and higher acetylcholinesterase levels result in a greater clinical effect.

Silent infarction or white matter hyperintensity and impaired attention task scores in a nondemented population: the Osaki-Tajiri Project.

Whether silent infarction can be completely asymptomatic remains unclear. Although the central cholinergic system affects cognition, little attention has been given to infarction. We hypothesized that specific damage to the cholinergic pathways due to infarction or white matter hyperintensity (WMH) would deteriorate cognition, especially attention. A total of 502 representative elderly participants enrolled in the Osaki-Tajiri Project in 1998 were studied. Participants with focal neurologic signs or previous history of stroke or transient ischemic attack were excluded from the analysis. MRIs were available for all participants, and the Cholinergic Pathways Hyperintensities scale (CHIPS) was used to assess vascular damage in the cholinergic pathways. The Mini-Mental State Examination (MMSE), word fluency test, Digit Symbol test, and digit span test were used to assess global cognitive function and several aspects of attention. Participants were divided into 3 groups according to the comorbidity of cerebrovascular disease (CVD) and cholinergic involvement: non-CVD, CVD with cholinergic involvement [CVD-Ch(+)], and CVD without cholinergic involvement [CVD-Ch(-)]. Cognitive scores were compared among the 3 groups. In the non-CVD group, the correlations between cognitive function and the CHIPS score were examined. The CVD-Ch(+) group exhibited significantly lower scores for the Digit Symbol test compared with the other two groups, regardless of the MMSE score. In the non-CVD group, the CHIPS score of white matter changes was irreversibly correlated (ie, biologically meaningful) with the Digit Symbol score in participants age >80 years. Our findings suggest that silent infarction or WMH may deteriorate attention regardless of global cognitive function by interrupting the central cholinergic pathway.

Prevalence of dementia and dementing diseases in the old-old population in Japan: the Kurihara Project. Implications for Long-Term Care Insurance data.

BACKGROUND: There have been no reports on the prevalence of dementia among the old-old people in Japan. METHODS: We studied the old-old population in Kurihara, northern Japan. Analysis 1 of Participants 1 (n=590) was performed to evaluate the prevalence of dementia and dementing diseases by intensive evaluation including MRI. Analysis 2 aimed to determine a good indicator for detecting 'suspected dementia condition' based on the Long-Term Care Insurance index. Analysis 3 of Participants 2 (n=3915) aimed to estimate the prevalence of 'suspected dementia condition'. RESULTS: In Analysis 1, 73 people (12.4%) were diagnosed with dementia. The most common cause was Alzheimer's disease with cerebrovascular disease. In Analysis 2, level I of the Impairment Level of Dementia was found to be a good indicator of 'suspected dementia condition'. In Analysis 3, the overall estimated prevalence of 'suspected dementia condition' was 23.6%. In men, the ratio increased gradually from 75 to 87 years old to about 20%, increased to 40% at the age of 88 and became stable thereafter. In contrast, in women, the ratio increased from 75 to 95+ years old, reaching about 70%. CONCLUSIONS: The prevalence was higher than that reported previously. There was a difference between the sexes: an 'age-related' increase occurred in men and an 'ageing-related' increase in women. Alzheimer's disease with cerebrovascular disease was the most common cause, which coincided with the previous findings of individuals aged 65 years and older; however, the ratio of mixed dementia was greater.

Pregabalin withdrawal in patients without psychiatric disorders taking a regular dose of pregabalin: A case series and literature review.

Pregabalin is a drug that can cause psychiatric symptoms via pregabalin withdrawal. Prior reports on pregabalin withdrawal have mainly focused on cases with pregabalin dependence or abuse, and little attention has been paid to patients who are prescribed regular doses of pregabalin. Herein, we report three cases of pregabalin withdrawal in patients without psychiatric disorders, taking regular doses of pregabalin, who developed psychiatric symptoms such as insomnia and anxiety after abrupt discontinuation of pregabalin. In addition, we conducted a systematic review of six case reports (previous studies) of pregabalin withdrawal under regular doses of pregabalin. Among the six cases, three patients had no comorbid mental or substance use disorders, the dose of pregabalin ranged from 150 to 600 mg/d, and the duration of pregabalin use ranged from a few weeks to many years. Of these six cases of pregabalin withdrawal, five had psychopathological symptoms, three had vegetative symptoms, and three had neurologic and physical complications. We concluded that since pregabalin withdrawal can occur even with regular doses and short-term use, clinicians must carefully reduce pregabalin doses when reducing or discontinuing treatment, paying close attention to withdrawal symptoms. Our case series sheds light on the scant evidence from previous research on physical dependence in patients who are taking regular doses of pregabalin. Furthermore, our cases were also valuable in demonstrating that pregabalin withdrawal can occur even after a relatively short period of 2 months.