[Laparoscopic Splenectomy for Metachronous Splenic Metastasis of Fallopian Tube Cancer with Synchronous Rectal Metastasis-A Case Report].

A 59-year-old woman underwent simple abdominal total hysterectomy with bilateral salpingo-oophorectomy, partial omentectomy, and extirpation of intrapelvic disseminated nodules for right fallopian tube cancer with rectal metastasis and peritoneal dissemination as primary debulking surgery(PDS). The histopathological diagnosis was high grade serous carcinoma( HGSC)of the right fallopian tube. After adjuvant chemotherapy with 4 courses of paclitaxel-carboplatin(TC), low anterior resection of the rectum for rectal metastasis and pelvic and para-aortic lymph node dissection were performed as interval debulking surgery(IDS). Histopathologically, lymph node metastasis was detected only in the right obturator lymph node. After adjuvant chemotherapy with 4 courses of TC, bevacizumab maintenance monotherapy was administered. Three years after PDS, laparoscopic splenectomy for splenic metastasis and extirpation of the solitary peritoneal metastases were performed as secondary debulking surgery(SDS). After adjuvant chemotherapy with 4 courses of TC, olaparib maintenance monotherapy was administered. The patient has remained alive without recurrence for 4 years after SDS and for 7 years after PDS. No case of metachronous splenic metastasis from fallopian tube cancer with synchronous rectal metastasis has been reported; however, long-term prognosis may be expected with PDS, IDS and SDS for platinum-sensitive HGSC.

[Neuroendocrine Carcinoma of the Non-Ampullary Duodenum-A Case Report].

A 69-year-old woman was admitted to a territory hospital because of severe right hypochondoralgia after 2 weeks of internal medicine for persistent epigastralgia. Gastroduodenal endoscopy revealed a large tumor with a fistula in the duodenal bulb that expanded to the stomach. Histopathologically, the biopsy specimen indicated a poorly differentiated adenocarcinoma and HER2 negative. Computed tomography revealed that the tumor invaded the left lobe of the liver. The patient was referred to our hospital for cancer treatment. After 1 course of chemotherapy with S-1 and CDDP, laparoscopic gastroenterostomy bypass was performed because of tumor hemorrhage and poor food intake. However, the tumor hemorrhage and poor food intake continued, and the tumor enlarged. Therefore, left hemihepatectomy and distal gastrectomy with resection of the duodenal bulb were performed 1 month after bypass surgery. Histological testing confirmed the diagnosis of duodenal large-cell neuroendocrine carcinoma invading the liver without lymph node metastasis. Adjuvant chemotherapy was not administered, and the patient has been alive without recurrence for 7 years and 3 months. Neuroendocrine carcinoma of the non-ampullary duodenum is very rare; however, a large cell type without lymph node metastasis may be a factor in the long-term prognosis.

Methionine homozygosity for PRNP polymorphism and susceptibility to human prion diseases.

BACKGROUND: No studies have assessed the independent association of methionine homozygosity at codon 129 with the susceptibility to prion diseases, controlling for the effects of the codon 219 polymorphisms and other potential confounders, using a large-scale population-based dataset. METHODS: We conducted a case-control study using a Japanese nationwide surveillance database for prion diseases. The main exposure was methionine homozygosity at codon 129, and the outcome was development of prion diseases. Multivariable logistic regression models were employed for specific disease subtypes (sporadic Creutzfeldt-Jakob disease (CJD), genetic CJD and Gerstmann-Sträussler-Scheinker disease (GSS)). RESULTS: Of 5461 patients registered in the database, 2440 cases and 796 controls remained for the analysis. The cases comprised 1676 patients with sporadic CJD (69%), 649 with genetic CJD (27%) and 115 with GSS (5%). For patients with methionine homozygosity, potential risk for occurring prion diseases: adjusted OR (95% CI) was 2.21 (1.46 to 3.34) in sporadic CJD, 0.47 (0.32 to 0.68) in genetic CJD and 0.3 (0.17 to 0.55) in GSS. Among patients with specific prion protein abnormalities, the potential risk was 0.27 (0.17 to 0.41) in genetic CJD with 180 Val/Ile, 1.66 (0.65 to 5.58) in genetic CJD with 200 Glu/Lys, 3.97 (1.2 to 24.62) in genetic CJD with 232 Met/Arg and 0.71 (0.34 to 1.67) in GSS with 102 Pro/Leu. CONCLUSIONS: Methionine homozygosity at codon 129 was predisposing to sporadic CJD, but protective against genetic CJD and GSS, after adjustment for codon 219 polymorphism effect. However, the impacts differed completely among patients with specific prion protein abnormalities.

Soluble APP-α and APP-ß in cerebrospinal fluid as potential biomarkers for differential diagnosis of mild cognitive impairment.

OBJECTIVES: Concentrations of soluble amyloid precursor proteins-α (sAPPα) and -ß (sAPPß) in cerebrospinal fluid (CSF) may reflect the neuropathology of Alzheimer's disease (AD). We previously reported that the concentrations of both sAPPα and sAPPß were significantly higher in patients with mild cognitive impairment (MCI) due to AD (MCI-AD) than in control subjects without cognitive impairment. The present study analyzed whether these sAPPs are useful in the differential diagnosis of MCI. METHODS: A modified and sensitive method was used to analyze concentrations of sAPPα and sAPPß in CSF of patients with MCI-AD (n = 30) and MCI due to other causes (MCI-others) (n = 24). Phosphorylated tau (p-tau) and amyloid ß-protein 42 (Aß42) were also analyzed using standard methods. RESULTS: CSF concentrations of sAPPα and sAPPß were significantly higher in the MCI-AD than in the MCI-others group (p < 0.001). Furthermore, concentrations of both sAPPα and sAPPß were highly correlated with the concentration of p-tau, consistent with our previous report. CONCLUSIONS: Measurement of both sAPPs in CSF using sensitive methods can be helpful in the precise differential diagnosis of patients with MCI.

[Surgical Resection of Pulmonary Oligometastases of Ampullary Cancer-A Case Report and Review of Seven Cases].

A 62-year-old woman underwent a subtotal stomach-preserving pancreatoduodenectomy for ampullary carcinoma (T3bN0M0, Stage Ⅱb). Histopathologically, the tumor was a tubular adenocarcinoma with mixed features, predominantly the intestinal type, following which adjuvant chemotherapy was not performed. Computed tomography performed 32 months after surgery showed a tumor measuring 6.7 mm in diameter at the apex of the right lung. The tumor had gradually increased in size and measured 10 mm in diameter, 47 months postoperatively. Since other metastatic lesions were absent, partial resection of the right lung under video-assisted thoracic surgery was performed 48 months postoperatively. Histopathological testing confirmed a diagnosis of lung metastasis from the resected specimen of ampullary carcinoma without mediastinal lymph node metastasis. Adjuvant chemotherapy was not performed, and recurrence was not observed even after 53 months following the partial lung resection. Previously, 7 resected cases of solitary lung metastasis from ampullary cancer have been reported. The histopathological sub-type of these 7 cases were intestinal type in 5 and pancreatobiliary type in 2 cases, respectively. No mortality or recurrence was observed for 8-119 months in any of the 7 cases(median, 19 months). In conclusion, owing to the good prognosis, solitary lung metastasis from an ampullary cancer can be classified as an oligometastatic disease, based on the concept proposed by Hellman and Weichselbaum.

[Synchronous Double Primary Non-B, and Non-C Intrahepatic Cholangiocarcinoma and Hepatocellular Carcinoma-A Case Report].

A 78-year-old man without hepatitis virus B or C underwent right hemi-hepatectomy and lymph node dissection for a tumor 5 cm in diameter located in the hepatic hilum of the posterior segment of the liver with portal vein thrombi extending into the main portal trunk and a tumor 1.5 cm in diameter in the peripheral side of segment 5 of the liver. Histopathologically, the former was diagnosed as intrahepatic cholangiocarcinoma and the latter as hepatocellular carcinoma(HCC). Five months after the surgery, intrahepatic and lymph node metastases were diagnosed based on computed tomography(CT); therefore, chemotherapy with S-1 for 3 months and gemcitabine and cisplatin(GC)for 5 months was administered, after which the metastatic lesions were not detected. Nineteen months after the surgery, partial resection of segment 2 of the liver was performed for a tumor 3 cm in diameter, which was diagnosed as HCC histopathologically. Two years after the second surgery, 2 recurrent nodules in the liver in segments 3 and 4 were detected on CT. Platinum-based hepatic arterial infusion chemotherapy(HAIC)and transcatheter arterial chemoembolization(TACE)were performed, and chemotherapy with GC was then administered for 7 months. For a new tumor detected in segment 1 in the liver, TACE was performed 17 months after initial HAIC. Seventy-four months after the initial surgery, 5 new nodules less than 1 cm in diameter were detected, and chemotherapy with sorafenib was administered for 5 months, after which the patient died of coronavirus disease 2019.

Characterization of Sporadic Creutzfeldt-Jakob Disease and History of Neurosurgery to Identify Potential Iatrogenic Cases.

We previously reported a phenotype of Creutzfeldt-Jakob disease (CJD), CJD-MMiK, that could help identify iatrogenic CJD. To find cases mimicking CJD-MMiK, we investigated clinical features and pathology of 1,155 patients with diagnosed sporadic CJD or unclassified CJD with and without history of neurosurgery. Patients with history of neurosurgery more frequently had an absence of periodic sharp-wave complexes on electroencephalogram than patients without a history of neurosurgery. Among 27 patients with history of neurosurgery, 5 had no periodic sharp-wave complexes on electroencephalogram. We confirmed 1 case of CJD-MMiK and suspected another. Both had methionine homozygosity at codon 129 of the prion protein gene and hyperintensity lesions in the thalamus on magnetic resonance images of the brain, which might be a clinical marker of CJD-MMiK. A subgroup with a history of neurosurgery and clinical features mimicking dura mater graft-associated CJD might have been infected during neurosurgery and had symptoms develop after many years.

MM2-type sporadic Creutzfeldt-Jakob disease: new diagnostic criteria for MM2-cortical type.

OBJECTIVE: To clinically diagnose MM2-cortical (MM2C) and MM2-thalamic (MM2T)-type sporadic Creutzfeldt-Jakob disease (sCJD) at early stage with high sensitivity and specificity. METHODS: We reviewed the results of Creutzfeldt-Jakob disease Surveillance Study in Japan between April 1999 and September 2019, which included 254 patients with pathologically confirmed prion diseases, including 9 with MM2C-type sCJD (MM2C-sCJD) and 10 with MM2T-type sCJD (MM2T-sCJD), and 607 with non-prion diseases. RESULTS: According to the conventional criteria of sCJD, 4 of 9 patients with MM2C- and 7 of 10 patients with MM2T-sCJD could not be diagnosed with probable sCJD until their death. Compared with other types of sCJD, patients with MM2C-sCJD showed slower progression of the disease and cortical distribution of hyperintensity lesions on diffusion-weighted images of brain MRI. Patients with MM2T-sCJD also showed relatively slow progression and negative results for most of currently established investigations for diagnosis of sCJD. To clinically diagnose MM2C-sCJD, we propose the new criteria; diagnostic sensitivity and specificity to distinguish 'probable' MM2C-sCJD from other subtypes of sCJD, genetic or acquired prion diseases and non-prion disease controls were 77.8% and 98.5%, respectively. As for MM2T-sCJD, clinical and laboratory features are not characterised enough to develop its diagnostic criteria. CONCLUSIONS: MM2C-sCJD can be diagnosed at earlier stage using the new criteria with high sensitivity and specificity, although it is still difficult to diagnose MM2T-sCJD clinically.

[Descending Colon Cancer with Synchronous Solitary Small Intestinal Metastasis-A Case Report].

A 77-year-old man presented with biliary colic and was diagnosed with descending colon cancer and cholelithiasis.During the surgery, a mass was detected in the upper jejunum, located 5 cm from the Treitz ligament on the anal side.Open left hemicolectomy with partial jejunal resection was performed.The mass in the small intestine was diagnosed as solitary small intestinal metastasis of colon cancer through immunohistopathological examination.Isolated intestinal metastases of colorectal cancers are rare.Furthermore, synchronous metastases are rare among these cases.A previous study reported poor prognosis in patients with small intestinal metastasis; therefore, more studies on the small intestinal metastasis of colorectal cancer are necessary.

[Laparoscopic Liver Resection for Liver Metastasis from Unresectable Pancreatic Ductal Adenocarcinoma Well-Controlled by Chemotherapy-A Case Report].

A 45-year-old man with unresectable locally advanced pancreas head cancer with multiple synchronous liver metastases was treated with gemcitabine plus nab-paclitaxel therapy as a first-line chemotherapy. During 24 months of 30 courses of this therapy, the primary lesion remained stable and liver metastases were completely disappeared on CT. Three months later, however, solitary relapse of liver metastasis occurred in segment 2. Therefore, we changed the chemotherapy regimen to the second-line treatment, FOLFIRINOX. After 3 courses of FOLFIRINOX, the primary lesion was kept well-controlled, but the solitary metastatic liver lesion was enlarged. An interdisciplinary team suggested surgical resection of the liver metastasis to control disease progress. We performed laparoscopic lateral segmentectomy of the liver. The postoperative course was uneventful, and the patient was discharged on postoperative day 8. The patient underwent another round of gemcitabine therapy owing to the good response of the primary pancreatic lesion to this drug. Three years after starting the first-line chemotherapy, the patient is still alive with well-controlled PDAC without distant metastasis. Surgical intervention for liver metastases may be a promising treatment option when unresectable primary PDAC is well controlled by chemotherapy.

[A Case of Disturbance of Consciousness Due to Hyperammonemia during Chemotherapy for Metastasis of Sigmoid Colon Cancer].

A 76-year-old woman had underwent 5-fluorouracil(5-FU), oxaliplatin(L-OHP)combination therapy(mFOLFOX6)as first-line chemotherapy for peritoneal recurrence after resection of sigmoid colon cancer. She showed severe general fatigue and disturbance of consciousness on the second day of the 12th course of chemotherapy. Computed tomography of the head detected no abnormal findings in the central nervous system. The laboratory results revealed a marked hyperammonemia. She was diagnosed as a disturbance of consciousness due to hyperammonemia and treated her with branched- chain amino acid solution. Then the disturbance of consciousness resolved on the following day. After changing the regimen of chemotherapy, the disturbance of consciousness was not found. Recently, it has been reported that high-dose 5-FU regimen such as mFOLFOX6 causes hyperammonemia as a rare adverse event. We should take hyperammonemia into account when disturbance of consciousness occurs during high-dose 5-FU chemotherapy.

[Laparoscopic Resection of Solitary Lymph Node Metastasis of Hepatocellular Carcinoma after Transcatheter Arterial Chemoembolization and Percutaneous Locoregional Therapies].

A 75-year-old woman underwent transcatheter chemoembolization(TACE)for 2 small hepatocellular carcinoma(HCC) lesions associated with severe alcoholic liver cirrhosis that necessitated management for ascites. Over 5 years after the initial TACE, she received multidisciplinary therapies with TACE, transcatheter arterial infusion of anticancer agents, percutaneous ethanol injections, or percutaneous radiofrequency ablation performed on 5 occasions for small recurrent HCC lesions. Computed tomography performed after the last therapy for HCC revealed a solitary lymph node swelling(39 mm in diameter) around the common hepatic artery. Magnetic resonance imaging performed 3 months later revealed that the lymph node had enlarged to 45 mm, without recurrence of the primary HCC, and after 4 months, to 60 mm; she then underwent laparoscopic lymph node resection. Histopathological examination of the resected specimen showed HCC metastasis. A recurrent metastatic lymph node(30 mm in diameter)was detected around the common hepatic artery and was resected laparoscopically 17 months postoperatively. Pancreatic head cancer was diagnosed 22 months after the second surgery; however, the patient refused cancer therapy and died 16 months after this diagnosis. No recurrence of the primary HCC or lymph node metastasis was observed over the 38 months after the second surgery.

[A Case of Gastric Neuroendocrine Carcinoma with Bulky Lymph Node Metastases, Solitary Liver Metastasis, and Left Adrenal Metastasis Curatively Resected after Chemotherapy].

A 68-year-old man presented with generalfatigue due to anemia. Gastrointestinalendoscopy showed a tumor approximately 60mm in diameter in the lesser curvature of the angle of the stomach. Large cell neuroendocrine carcinoma was diagnosed by biopsy of a tumor specimen. Computed tomography revealed bulky regional lymph node metastases, solitary liver metastasis, and left adrenal metastasis. After three courses of combined chemotherapy with S-1 and CDDP, the gastric lesion and lymph node metastases shrunk, but the liver metastasis and left adrenal metastasis enlarged. After three courses of combined chemotherapy with ramucirumab and paclitaxel, the liver metastasis and left adrenal metastasis also shrunk, and no other new metastatic lesions appeared. Accordingly, total gastrectomy with lymph nodes dissection, partial resection of the liver and left adrenalectomy were performed for a curative resection. The histological findings revealed neuroendocrine carcinoma and adenocarcinoma of the stomach. The lesions of the liver and lymph nodes were all burned out, and a tiny metastatic lesion of neuroendocrine carcinoma was left in the left adrenal gland. S-1 was administrated for 3 months after surgery. The patient has been alive for 16months without recurrence after surgery.

[A Case of Rupture of Esophageal Varices during a Course of CAPOX with Bevacizumab Therapy for Sigmoid Colon Cancer with Multiple Liver Metastases].

The patient was a 67-year-old man with multiple liver metastases from sigmoid colon cancer and had received capecitabine, oxaliplatin, and bevacizumab(CAPOX plus Bev)combination chemotherapy. After 11 courses of this therapy, he had a rupture of esophageal varices and was treated with endoscopic variceal ligation(EVL). Esophageal varices are rare during the course of oxaliplatin-based chemotherapy. More studies are necessary for early detection of esophageal varices during this therapy.

[A Resected Case of Retroperitoneal Metastasis and Small Bowel Metastasis from HCC].

A 71-year-old man underwent right hemi-hepatectomyfor a hepatocellular carcinoma(HCC)measuring 18 cm in diameter. The pathological diagnosis was poorlydifferentiated HCC. Ten months after the surgery, computed tomographyrevealed a nodule 12mm in diameter in the right lung as well as 2 nodules measuring 19 and 11mm in diameter in the retroperitoneum at the cranial aspect of the left kidney. Four months later, the nodule in the right lung had enlarged to 44 mm, while the 2 nodules in the retroperitoneum had enlarged to 68mm and 34 mm. These nodules were resected and histopathologicallydiagnosed as metastasis from HCC. Twenty-one months after liver resection, computed tomographyrevealed nodules 16 and 25mm in diameter in the retroperitoneum around the urinarybladder and jejunum, respectively. One month later, intussusception resulted from the jejunal tumor. Laparoscopic surgerywas performed for both tumors, which were diagnosed as metastases from HCC. Twenty-five months after liver resection, metastasis from the HCC appeared in the left adrenal grand, at the site of the jejunal anastomosis, and in the fattytissue around the right scapula. Twenty-nine months after liver resection, the patient died of respiratoryfailure from multiple metastases in the left lung.

[Solitary lymph node metastasis from early-stage hepatocellular carcinoma after transcatheter arterial chemoembolization and radiofrequency ablation: a case report and review of the Japanese literature].

A 77-year-old man with chronic hepatitis C underwent transcatheter arterial chemoembolization (TACE) and radiofrequency ablation (RFA) for early-stage hepatocellular carcinoma (HCC) in segment 8 of the liver. Necrosis was confirmed radiologically. After 19 months, recurrent HCC in segment 6 was treated with TACE and RFA. There was no recurrence. Direct-acting antiviral (DAA) therapy 24 months after the initial procedure led to a sustained virologic response. AFP-L3 markedly increased 11 months after DAA therapy, and MRI 6 months after that showed a solitary lymph node near the common bile duct. Because no intrahepatic recurrence or other lymph nodes were seen, the solitary node was excised. Histopathology showed metastatic HCC. There has been no subsequent recurrence over 13 months of follow-up.

Update: Dura Mater Graft-Associated Creutzfeldt-Jakob Disease - Japan, 1975-2017.

Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disorder that, according to the most well accepted hypothesis (1), is caused by replicating, transmissible, abnormal forms of a host-encoded prion protein (prions). Most CJD cases occur spontaneously (sporadic CJD) or are inherited (genetic CJD). Iatrogenic CJD can occur after exposure to prion-contaminated instruments or products in medical/surgical settings. Cadaveric dura mater graft-associated CJD (dCJD) accounts for a common form of iatrogenic CJD. This report summarizes the epidemiologic features of 154 cases of dCJD identified in Japan during 1975-2017; these cases account for >60% of dCJD cases reported worldwide (1,2). The unusually high prevalence of dCJD in Japan was first reported in 1997 (3). In 2008, a single brand of graft (Lyodura [B. Braun Melsungen AG, Melsungen, Germany]), frequently used as a patch in neurosurgical procedures, was identified as the probable vehicle of transmission (4). No international recall of the implicated Lyodura occurred, the product had a relatively long shelf life, and the grafts were used frequently in Japanese patients with non-life-threatening conditions (4,5). Since 2008, additional cases have been ascertained, reflecting the identification of previously missed cases and the occurrence of new cases with longer latency periods (interval from exposure to symptom onset) for dCJD (up to 30 years), underscoring the importance of maintaining surveillance for dCJD.

Characteristics of facial expression recognition ability in patients with Lewy body disease.

BACKGROUND: The facial expression of medical staff has been known to greatly affect the psychological state of patients, making them feel uneasy or conversely, cheering them up. By clarifying the characteristics of facial expression recognition ability in patients with Lewy body disease, the aim of this study is to examine points to facilitate smooth communication between caregivers and patients with the disease whose cognitive function has deteriorated. METHODS: During the period from March 2016 to July 2017, we examined the characteristics of recognition of the six facial expressions of "happiness," "sadness," "fear," "anger," "surprise," and "disgust" for 107 people aged 60 years or more, both outpatient and inpatient, who hospital specialists had diagnosed with Lewy body diseases of Parkinson's disease, Parkinson's disease with dementia, and dementia with Lewy bodies. Based on facial expression recognition test results, we classified them by cluster analysis and clarified features of each type. RESULTS: In patients with Lewy body disease, happiness was kept unaffected by aging, age of onset, duration of the disease, cognitive function, and apathy; however, recognizing the facial expression of fear was difficult. In addition, due to aging, cognitive decline, and apathy, the facial expression recognition ability for sadness and anger decreased. In particular, cognitive decline reduced recognition of all of the facial expressions except for happiness. The test accuracy rates were classified into three types using the cluster analysis: "stable type," "mixed type," and "reduced type". In the "reduced type", the overall facial recognition ability declined except happiness, and in the mixed type, recognition ability of anger particularly declined. CONCLUSION: There were several facial expressions that the Lewy body disease patients were unable to accurately identify. Caregivers are recommended to make an effort to compensate for such situations with language or body contact, etc., as a way to convey correct feeling to the patients of each type.

[Long-Term Survival after Multidisciplinary Treatment for Resected Pancreatic Adenocarcinoma with Recurrence of Pulmonary Metastases - A Case Report].

Recent studies indicated that isolated pulmonary metastases could define a favorable subgroup in metastatic pancreatic cancer. We report a case of isolated pulmonary metastases after curative resection of pancreas head cancer treated with chemotherapy and pulmonary metastasectomy survived for 79 months after recurrence. A 72-year-old male underwent pancreatoduodenectomy for pancreas head cancer. Adjuvant chemotherapy was done with gemcitabine hydrochloride (GEM)for 6 months and then S-1 for 2 months. Twenty-seven months after surgery, 2 small metastatic nodules in the left lung was detected. Chemotherapy with GEM was performed but the lesions grew larger very slowly. A new metastatic nodule was detected in the right lung 40 months after surgery and pleural effusion was detected 52 months after surgery. Then combination chemotherapy with GEM and S-1 was performed for 3 months followed chemotherapy with S-1 alone. Seventytwo months after surgery, chemotherapy with GEM was performed again because of patient's intolerance to S-1. Ninety months after initial surgery, pulmonary metastasectomy of the right lung was performed because of its resistance to chemotherapy. Chemotherapy with GEM was started again 4 months after pulmonary metastasectomy but serum levels of tumor markers remained increase. Combination chemotherapy with GEM and nab-paclitaxel was started 8 months after pulmonary metastasectomy but the patient died 16 months after pulmonary metastasectomy.

[Acute cholecystitis due to microscopic polyangiitis:a case report].

A 78-year-old man with chief complaints of cough and lower limb numbness was admitted to our hospital. Serum myeloperoxidase-antineutrophil cytoplasmic antibody titer (MPO-ANCA) was elevated (48.8U/ml), and a diagnosis of microscopic polyangiitis (MPA) was made. After admission, the patient developed a fever and right upper quadrant pain. Ultrasonography and computed tomography revealed an acute cholecystitis of unknown cause, and laparoscopic cholecystectomy was performed. Histopathological examination of the resected gallbladder revealed necrotizing vasculitis along with the infiltration of eosinophils, lymphocytes, and plasma cells around the small arteries in the muscular layer of the gallbladder, which are characteristics of MPA.