A case of acute liver failure caused by Budd-Chiari syndrome salvaged by brain-dead donor liver transplantation.

A 24-year-old man was admitted to our hospital with abdominal distension. He was found to have acute liver failure and diagnosed with Budd-Chiari syndrome based on angiography and liver biopsy. Liver transplantation was deemed necessary when angiography showed extensive thrombotic occlusion of the hepatic veins and liver biopsy revealed submassive hepatic necrosis. The patient was found to have the JAK2V617F mutation, indicating a myeloproliferative neoplasm as the background disease. He developed hepatic encephalopathy but remained conscious on on-line hemodiafiltration. Brain-dead donor liver transplantation was performed on hospital day 30. Since then, the patient has remained well.

Successful treatment of immune-related cystitis with bladder hydrodistension.

Introduction: Although immune checkpoint inhibitors offer significant therapeutic benefits to patients with advanced cancer, they can also cause a variety of immune-related adverse events. As immune checkpoint inhibitors are being widely used, rare immune-related adverse events are being reported. Case presentation: A 70-year-old man with advanced salivary duct carcinoma was treated with pembrolizumab following radiotherapy. After receiving two doses of pembrolizumab, the patient experienced symptoms such as micturition pain and hematuria. Immune-related cystitis was suspected, and the patient underwent a bladder biopsy and bladder hydrodistension. Histological analysis revealed non-neoplastic bladder mucosa with CD8-positive lymphocyte-dominant inflammatory cell infiltration, consistent with immune-related cystitis. The patient's bladder symptoms improved postoperatively without steroid administration. Conclusion: Although steroids are commonly administered to treat immune-related adverse events, bladder hydrodistension may be a promising treatment option for immune-related cystitis to avoid administration of steroids, which may impair the therapeutic effect of immune checkpoint inhibitors.

Hemophagocytic Lymphohistiocytosis and Anti-neutrophil Cytoplasmic Antibody-associated Vasculitis Possibly Caused by Enterococcus faecalis Infective Endocarditis.

Infection can induce hemophagocytic lymphohistiocytosis (HLH) and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We herein report a 52-year-old man who had HLH and AAV simultaneously, possibly caused by Enterococcus faecalis infective endocarditis. The HLH diagnosis was based on the HLH-2004 criteria. AAV was diagnosed based on a positive result for proteinase-3 ANCA and necrotizing vasculitis of the small vessels on a skin biopsy. He eventually died and was sent for autopsy after combination treatment of valve replacement, antibiotics, and immunosuppressants, including corticosteroids. This case involved a challenging diagnosis and treatment of HLH with various complications in an adult.

Sarcomatoid urothelial carcinoma of the renal pelvis treated with immunotherapy.

BACKGROUND: Sarcomatoid carcinoma is a rare, high-grade malignancy with epithelial and mesenchymal components. It may be a good candidate for immunotherapy because it is associated with overexpression of programmed cell death ligand 1. Sarcomatoid urothelial carcinoma (UC) of the upper urinary tract is extremely rare. Here we report the first case of sarcomatoid UC of the renal pelvis that responded to immunotherapy. CASE PRESENTATION: A 79-year-old man was referred to our hospital complaining of various symptoms, including anorexia and abdominal pain. A computed tomography scan revealed a right atrial tumor, a 9 cm left renal mass with a renal vein tumor thrombus, para-aortic lymphadenopathy, and multiple small lung nodules. The patient underwent resection of the right atrial tumor. Pathological analysis of the tumor did not lead to an accurate diagnosis even after several rounds of immunohistochemistry. He underwent a needle biopsy of the left kidney and was initially diagnosed with collecting duct carcinoma, a rare subtype of renal cell carcinoma (RCC). Following the initial diagnosis, immunotherapy with nivolumab and ipilimumab commenced. Thereafter, almost all lesions, including the left renal tumor, were reduced in size. However, he underwent a left nephrectomy approximately a year after beginning immunotherapy due to repeated left renal bleeding. Histological examination of the nephrectomy specimen revealed two forms of cancer-sarcomatoid UC and conventional high-grade UC. Two months after surgery, the patient was found to have new lung metastases. He underwent chemotherapy with gemcitabine and cisplatin, followed by immunotherapy with pembrolizumab. However, both treatments were ineffective. The patient died of cancer 19 months after his first admission. CONCLUSIONS: The presented case of sarcomatoid UC of the renal pelvis that partially responded to immunotherapy suggests that immunotherapy can be a promising treatment for sarcomatoid UC.

[Initial Recurrence around the Hilar of the Liver Which Was Difficult to Differentiate from Perihilar Cholangiocarcinoma with Portal Vein Invasion after Resection for Breast Cancer-A Case Report].

A 63-year-old woman underwent mastectomy and axillary dissection for right breast cancer(cT4bN1M0, Stage ⅢB, scirrhous carcinoma, moderately positive for ER, PgR negative, and HER2 negative)following neoadjuvant chemotherapy. She received no adjuvant therapy. A follow-up computed tomography 3 years later showed a soft tissue mass around the hilar bile ducts and mass in segment 6 of the liver. Based on these imaging findings, a diagnosis of perihilar cholangiocarcinoma with liver metastasis was made. She received chemotherapy with gemcitabine plus cisplatin, followed by S-1 monotherapy. Two years after the initiation of chemotherapy, an increase in the size of the liver mass and duodenal stenosis due to peritoneal dissemination were detected. Gastro-jejunal bypass was performed and a biopsy of the disseminated peritoneal mass supported a histologic diagnosis of breast cancer. The patient then received chemotherapy for breast cancer for 1 year. However, she eventually died due to the progression of the peritoneal dissemination. Although initial recurrence around the hilar of the liver is extremely rare after resection for breast cancer, when a new lesion is detected after breast cancer surgery the diagnosis and initial treatment should be made with the possibility of breast cancer recurrence in mind, based on the clinicopathological findings and the risk of recurrence.

Treatment for Appendicitis With Appendicolith by the Stone Size and Serum C-Reactive Protein Level.

INTRODUCTION: Appendicolith causes acute appendicitis. However, surgical indications for appendicolith-related acute appendicitis have not been established. We aimed to clarify the clinical features of appendicolith-associated appendicitis and determine an appropriate treatment strategy based on the initial presentation. MATERIALS AND METHODS: We retrospectively reviewed the records of 479 consecutive patients with acute appendicitis and verified the therapeutic strategy as per the appendicolith and clinical status. RESULTS: Appendicoliths were identified in 214 of 479 patients (44.6%) using computed tomography. Surgery was more frequently required in patients with appendicolith than in patients without appendicolith (82.7 versus 64.9%; P < 0.001). The stones were smaller and serum C-reactive protein (CRP) concentration was lower among patients with appendicoliths treated with medication alone than among those surgically treated (both P < 0.001). An appendicolith measuring ≤5 mm in diameter and CRP concentration ≤5.36 mg/dL were predictive of completion of nonsurgical therapy. CRP concentration >10 mg/dL and stone diameter of 10 mm were significantly associated with appendiceal perforation. CONCLUSIONS: Nonsurgical therapy could be considered for patients with appendicoliths measuring ≤5 mm in diameter and in cases where the serum CRP concentration is ≤5 mg/dL. An appendicolith measuring >10 mm in diameter or CRP concentration >10 mg/dL is an indication for surgery.

A 40-year-old man with a rapidly growing intrascrotal tumor in the fibroma-thecoma group.

Introduction: Tumors in the fibroma-thecoma group are benign tumors, typically found in the ovaries of postmenopausal women and occasionally develop in the testes. These tumors are mostly treated with radical orchiectomy because preoperative diagnosis confirming the benign nature is difficult. Case presentation: A 40-year-old man was incidentally pointed out to have a right intrascrotal mass, measuring approximately 10 cm on computed tomography. Malignant testicular tumor was suspected based on the location and size of the tumor. The patient underwent right radical orchiectomy. Histologically, the tumor had no evidence of malignancy, and the diagnosis of tumors in the fibroma-thecoma group was made. The patient had no recurrence 8 months after surgery. Conclusion: Intrascrotal tumors in the fibroma-thecoma group are rare benign tumors and mostly treated with radical orchiectomy due to concerns about malignancies. Further investigation is needed for accurate preoperative diagnosis, and we should be aware of these rare tumors.

Unresectable primary hepatic adenosquamous carcinoma successfully treated with systemic and transcatheter hepatic arterial injection chemotherapies followed by conversion surgery: a case report and literature review.

BACKGROUND: Primary hepatic adenosquamous carcinoma (ASC) is a type of tumor that has the features of both adenocarcinoma and squamous cell carcinoma (SCC). The prognosis for patients with ASC is poor, as the chemotherapy has been ineffective so far. CASE PRESENTATION: Here, we report a case of a 62-year-old male patient who presented with high fever. The tumor marker levels were high, and abdominal dynamic computed tomography showed a liver tumor and distant lymph node metastases. Upon further investigation, needle biopsy of the liver tumor showed a primary hepatic SCC. Because the SCC was unresectable, the patient was treated with tegafur/gimeracil/oteracil (S-1) and transcatheter hepatic arterial injection (TAI) of cisplatin. After chemotherapy, a surgical resection performed on the remaining liver tumor, made the patient cancer-free. After the operation, the liver tumor was confirmed as primary hepatic ASC. Subsequently, the patient was administered postoperative adjuvant chemotherapy, which prevented its recurrence. CONCLUSIONS: Due to the lack of an effective treatment for primary hepatic ASC, its prognosis is poor. Here, we suggest that a chemotherapy combination of 5-fluorouracil (S-1) and cisplatin along with conversion surgery might be an effective way for treating primary hepatic ASC. Our experience from this case shall be valuable to clinicians around the world involved in the treatment of primary hepatic ASC.

Surgical technique for the successful curative resection of locally advanced caecal cancer invading the external iliac artery: A case report.

INTRODUCTION: Curative resection generally has a good prognosis if the tumor is a locally advanced colorectal tumor. However, resection of a primary tumor that has invaded the aortoiliac artery is controversial. Herein, we report a case of successful resection of advanced cecal cancer invading the external iliac artery. CASE REPORT: A 29-year-old male patient had advanced cecal cancer invading the right external iliac artery and vein, right ureter, iliopsoas muscle, and sigmoid colon. We collected the patient's pre-/intra-/postoperative, clinical, and histological data. We reviewed the factors that may have contributed to curative resection without complications. We performed a palliative terminal ileum-sigmoid anastomosis for the prevention of intestinal obstruction. The patient received neoadjuvant chemotherapy, and the tumor patently regressed. After arterial reconstruction was performed with a femoral-femoral bypass, we performed radical resection: right hemicolectomy; partial sigmoidectomy; and partial resection of the right ureter, iliopsoas muscle, right testicular, and external iliac vessels. Pathologically, 99% of the tumor cells disappeared after chemotherapy. The patient was discharged on postoperative day 9. No recurrence has been noted 24 months after surgical resection, and the patient is receiving adjuvant chemotherapy. CONCLUSIONS: Thus, we successfully resected advanced cecal cancer without complications. Reconstruction with femoral-femoral arterial bypass and neoadjuvant chemotherapy are useful methods for curative resection without complications.

Possible Coronary Sequelae of Kawasaki Disease in an Elderly Man.

Kawasaki disease (KD) is an acute self-limited syndrome that predominantly affects children. Coronary sequelae have been identified to be responsible for a small, but significant percentage of young adults who present with myocardial ischemia. In this study, we present a case of an elderly patient with possible coronary sequelae of KD. A 76-year-old man was referred to our outpatient department for silent myocardial ischemia. Axial images of coronary computed tomography showed multiple lumens in the proximal left anterior descending (LAD) artery. Coronary angiography demonstrated braid-like appearance in the proximal and distal segment of the LAD. Coronary intervention was successfully performed for the proximal LAD lesion using directional atherectomy (DCA) catheter. Microscopic examination of the DCA specimens showed the following histological features: tissues in densely hyalinized fibrosis with occasional microcalcification, or those containing a number of smooth muscle cells (SMCs) with myxoid extracellular matrix. There was paucity of cholesterin crystals and aggregation of foamy cells. In addition, scarcely any inflammatory cell filtration was identified. In the section of SMC-containing samples, formation of multiple re-canalized vessels embracing endothelial cells was confirmed. These histopathologic findings indicated that the present coronary artery lesion has a high possibility of very late cardiovascular sequelae caused by arteritis due to KD, rather than arteriosclerosis. This is the oldest adult case with coronary artery disease possibly resulting from KD sequelae. This case highlights that KD sequelae must be considered as a cause of coronary artery lesion even in older patients.

A rare case of nivolumab-related myasthenia gravis and myocarditis in a patient with metastatic gastric cancer.

BACKGROUND: Although rare, several immune-related adverse effects can be life-threatening. Here, we describe a metastatic gastric cancer patient presenting with nivolumab-related myasthenia gravis and myocarditis, a previously unreported adverse effect of gastric cancer treatment. CASE PRESENTATION: A 66-year-old man with metastatic gastric cancer visited the emergency department because of dizziness after the first dose of nivolumab. Diagnoses of nivolumab-related myasthenia gravis and myocarditis were established. Myocardial biopsy results and anti-acetylcholine receptor antibody positivity confirmed the diagnoses. Despite plasma exchange and intravenous methylprednisolone and immunoglobulin administration, the patient's general condition gradually worsened, and he died. CONCLUSIONS: Strict monitoring for cardiac and neuromuscular symptoms after nivolumab administration is necessary to rapidly treat these adverse effects.

A rare case of neonatal colonic obstruction caused by a solitary intestinal tumor.

BACKGROUND: Intestinal obstruction caused by a tumor is very rare in newborns, and the preoperative diagnosis is difficult. We herein report a rare case of neonatal colonic obstruction due to solitary intestinal myofibroma with characteristic findings on gastrografin enema and the surgical strategy. CASE PRESENTATION: A 4-day-old female infant presented to our neonatal intensive-care unit with abdominal distention and bilious vomiting after feeding. A gastrografin enema showed that the transverse colon near the hepatic flexure was not delineated at the oral side. When pressure was applied, a small amount of contrast material moved into the mouth in the form of threads. Microcolon was not observed, and stenosis of the transverse colon was found 9 cm from the Bauhin valve. Partial resection and end-to-end anastomosis were performed. A pathological examination of the resected specimen suggested gastrointestinal stromal tumor (GIST). After obtaining a second opinion, the histology and immunohistological markers were deemed characteristic of infantile myofibroma. CONCLUSION: If string sign and a napkin ring appearance are found in a case of neonatal intestinal obstruction, surgery should be performed with a tumor in mind. In cases of neonatal intestinal obstruction caused by a tumor, the lesion should be resected with a sufficient surgical margin before the pathological examination.

Gastrointestinal stromal tumor of the stomach with lymph node metastasis treated by laparoscopic and endoscopic cooperative surgery with lymph node pick-up resection: A case report and literature review.

INTRODUCTION: Gastrointestinal stromal tumor of the stomach with lymph node metastasis is a rare condition, and few cases have been treated by gastrectomy and systematic lymph node dissection. PRESENTATION OF CASE: We report the case of a patient who was treated by local resection with laparoscopic and endoscopic cooperative surgery and lymph node pick-up resection. A 73-year-old woman presented with gastric submucosal tumor and swollen lymph node. The submucosal tumor was 30 mm in size. After surgery, the tumor was confirmed to be a gastrointestinal stromal tumor by immunostaining. No recurrence was noted 36 months following the surgery. DISCUSSION: Laparoscopic and endoscopic cooperative surgery allows minimal resection because the exact resection margin can be identified. Therefore, endoscopy is a useful aid in the treatment of gastric gastrointestinal stromal tumor. The MIB-1 index of metastatic lymph node was lower than that of a primary lesion; hence, the prognosis might not depend on lymph node metastasis. CONCLUSION: Laparoscopic and endoscopic cooperative surgery and lymph node pick-up resection were useful and minimally invasive. Systematic lymph node dissection is not a viable option.

Malignant adenomyoepithelioma of the breast.

BACKGROUND: Adenomyoepithelioma (AME) of the breast is a very rare tumor and is generally considered to be benign. However, some show malignant transformation, which results in local recurrences or distant metastases. The morphological features of AME that might predict malignant potential have not been elucidated. Moreover, there is also no established multidisciplinary treatment for malignant AME aside from complete excision at an early stage. CASE PRESENTATION: A 64-year-old female diagnosed with AME of the left breast underwent lumpectomy. The surgical margins were negative. Six months after the operation, however, malignant AME recurred locally in the left breast. MRI showed multiple masses, which invaded the skin. A left mastectomy with axillary lymph node dissection was performed. Additional areas of AME were found in about one third of the entire breast. Eight months after the mastectomy, lung metastases were detected. She underwent chemotherapy with fluorouracil, epirubicin, and cyclophosphamide (FEC) for 9 cycles with little response. Lung metastasectomy was performed. Nine months after lung metastasectomy, the metastases were widespread to the brain, heart, and kidney; she subsequently died 2 months later. CONCLUSIONS: Malignant AME has various morphological features, and in this report, we characterize new findings from both imaging and pathology/autopsy. Malignant potency is related to the tumor size, tumor appearance, and mitoses, even if only a few. Given that ductal spread is one of the morphological features of malignant AME, it is of paramount importance to assess the surgical margins.

[A Case of 11-Year Patient Survival after the Resection of a Metastatic Pancreatic Tumor of Sigmoid Colon Cancer].

A 44-year-old woman underwent a sigmoidectomy for sigmoid colon cancer in March 2002. At 5 years after the primary surgery, she underwent 2 hepatectomies, 2 anastomotic resections, 1 lung resection, and 1 radiofrequency ablation. Computed tomography revealed a mass in the pancreatic tail in January 2008. We performed distal pancreatectomy. The specimen showed metastases ofthe sigmoid colon cancer. Computed tomography revealed metastases to the left iliac lymph node in September 2010. She underwent chemotherapy with IRIS for the lymph node metastases. After 16 chemotherapy sessions, the lymph node metastases disappeared. The patient is alive 11 years after the pancreatectomy. Resectable pancreatic metastasis from colorectal cancer is rare. Multimodal treatment that includes radical surgery may achieve long-term survival in some cases.

[Case of Ductal Carcinoma In Situ of the Nipple in a 93-Year-Old Man].

We report the case of an elderly male patient with ductal carcinoma in situ(DCIS) of the nipple. A 93-year-old man visited the hospital because of pain and bleeding in and swelling of the right nipple. A benign tumor was suspected, but a definite diagnosis could not be made before surgery based on echo and cytology findings; thus, a malignant tumor could not be ruled out. He underwent partial mastectomy combined with the areola and nipple for diagnosis and treatment. Histologic examination confirmed the diagnosis of DCIS of the nipple. The surgical margin was negative. At 6 months after the surgery, he was doing well with no evidence of disease in the absence of postoperative adjuvant therapy. Thus, clinicians should consider breast carcinoma of the nipple as a differential diagnosis when an elderly man presents with swelling of the nipple.

A rare case of peritoneal deposits with carbon pigmentation after preoperative endoscopic tattooing for sigmoid colon cancer.

PURPOSE: We report a case in which pigmented peritoneal deposits were found during laparoscopic surgery following preoperative endoscopic tattooing for sigmoid colon cancer. METHODS: The patient's clinical, endoscopic, and histological data from the Niigata City General Hospital were reviewed, as well as the literature on laparoscopic surgery involving the preoperative endoscopic tattoo, with a focus on the relevance of peritoneal deposits and tattooing ink. RESULTS: A 71-year-old man presented to our hospital complaining of vomiting and abdominal distention. Abdominal computed tomography revealed obstructive sigmoid colon cancer. An emergency endoscopic colon stenting procedure and injection of 0.2 ml India ink to the submucosal layer of the tumor's anal side were performed. Laparoscopic-assisted sigmoid colectomy was done 14 days after stenting. At surgery, seven small peritoneal deposits were seen in the rectovesical pouch and at the site adjacent to the tumor. All peritoneal deposits were stained by the ink. Gross leakage of the ink into extraintestinal sites was seen. The seven peritoneal deposits were resected under laparoscope. Histological findings revealed that the seven peritoneal deposits were composed of adenocarcinoma and carbon pigments. Immunohistochemical staining for cluster of differentiation 163 showed that the carbon pigments in the peritoneal deposits were within macrophages. CONCLUSIONS: The possibility of the tattooing procedure causing peritoneal dissemination cannot be completely denied, but it can be hypothesized that the carbon pigmentation was transferred to peritoneal deposits by macrophages. In the future, we hope that this phenomenon becomes a keystone for diagnoses and treatments for peritoneal dissemination.

Histopathologic features of an autopsied patient with cerebral small vessel disease and a heterozygous HTRA1 mutation.

Cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CARASIL) is a hereditary cerebral small vessel disease (CSVD) caused by homozygous or compound heterozygous mutations of the high temperature requirement A serine peptidase 1 gene (HTRA1). Affected patients suffer from cognitive impairment, recurrent strokes, lumbago and alopecia. Recently, clinical studies have indicated that some patients with heterozygous mutations in HTRA1 may also suffer CSVD. Here, we report the histopathologic features of an autopsied 55-year-old male patient who had shown cognitive impairment and multiple cerebral infarcts, and was found to have a heterozygous missense mutation (p.R302Q) in the HTRA1 gene. Histologically, small vessels in the brain and spinal cord showed intimal proliferation, splitting of the internal elastic lamina, and degeneration of smooth muscle cells in the tunica media. Thus, although less severe, the features were quite similar to those of patients with CARASIL, indicating that patients with heterozygous mutations develop CSVD through underlying pathomechanisms similar to those of CARASIL.

[Brain Metastasis of Triple Negative Breast Cancer after Pathological Complete Response to Neoadjuvant Chemotherapy - A Case Report].

We report the case of a patient with triple negative breast cancer(TNBC)who showed isolated brain metastasis relatively soon after pathological complete response(pCR)to neoadjuvant chemotherapy. A 45-year-old woman with a diagnosis of TNBC(T2N1M0, Stage II B)received neoadjuvant chemotherapy with 5-FU/epirubicin/cyclophosphamide(FEC), followed by docetaxel. After the neoadjuvant chemotherapy, she underwent mastectomy and axillary lymph node dissection. Histological examination of the resected specimens revealed pCR. Brain metastasis, however, developed 7 months after the resection. She underwent total removal of the brain tumor and 50 Gy irradiation to the right frontal lobe. Histological examination confirmed a diagnosis of metastasis from TNBC. She is doing well with no evidence of disease 81 months after resection of the brain metastasis. This case and a review of the literature suggest that the clinician should be aware that brain metastasis from breast cancer may develop even after achieving pCR to neoadjuvant chemotherapy. Surgical resection followed by radiotherapy may provide a survival benefit for selected patients with isolated brain metastasis from breast cancer.

[A Case of Squamous Cell Carcinoma with Negative Conversion of ER Status after Endocrine Therapy in an Elderly Woman].

We report the case ofan elderly patient with squamous cell carcinoma(SCC)ofthe breast, which showed negative conversion after endocrine therapy. An 82-year-old woman with a diagnosis ofER -positive SCC(cT1N0M0, StageⅠ)received primary endocrine therapy with 5 hormonal medicines. Following the endocrine therapy, she underwent mastectomy and axillary node resection. Histological examination confirmed a diagnosis of ER-negative SCC. At 10 months after the operation, she was doing well with no evidence ofdisease without postoperative adjuvant therapy. Thus, clinicians should be aware that the ER status may change after primary endocrine therapy. For elderly patients with breast cancer, it is important to be aware that primary endocrine therapy can become ineffective due to ER-negative conversion and aging due to prolonged treatment.