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.

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.

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.

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 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.

Coexistence of amyotrophic lateral sclerosis with neuro-Behçet's disease presenting as a longitudinally extensive spinal cord lesion: clinicopathologic features of an autopsied patient.

We report the clinical and autopsy features of a 65-year-old Japanese man who clinically exhibited overlap of both neuro-Behçet's disease (NBD) and amyotrophic lateral sclerosis (ALS). The patient had a HLA-B51 serotype, a recent history of uveitis and had suffered paraparesis, sensory and autonomic disturbance, frontal signs and tremor. A brain and spine MRI study revealed a longitudinally extensive thoracic cord (Th) lesion, but no apparent intracranial abnormalities. The lesion extended ventrally from Th4 to Th9, exhibiting low intensity on T1-weighted images, high intensity on T2-weighted and fluid-attenuated inversion recovery images and gadolinium enhancement. The patient's upper and lower motor neuron signs and sensory disturbance worsened and he died 16 months after admission. At autopsy, the spinal cord and brain exhibited characteristic histopathological features of both NBD and ALS, including chronic destruction of the ventral thoracic white and gray matter, perivascular lymphocytic infiltration, binucleated neurons, lower and upper motor neuron degeneration, Bunina bodies and skein-like inclusions. Although incidental coexistence of these rare disorders could occur in an individual, this case raises the possibility of a pathomechanistic association between NBD and ALS.

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.

Primary spinal cord gliomas: Pathologic features associated with prognosis.

Primary spinal cord gliomas are rare and are associated with high mortality. Unlike brain tumors, the clinicopathological features of spinal cord gliomas are not well defined. We analyzed clinical, histopathology, and immunohistochemical features and overall survival (OS) of 25 patients with primary spinal cord gliomas treated between 1994 and 2023 at 4 institutions. IDH1 R132H, H3K27M, and p53 were assessed by immunohistochemistry (IHC). Four (16%), 5 (20%), 2 (8%), and 13 (52%) patients were diagnosed as having grades 1, 2, 3, and 4 gliomas according to the World Health Organization (WHO) 2021 classification, respectively. One case (4%), with a circumscribed diffuse midline glioma, H3K27-altered, had a rare molecular profile and could not be graded. IHC demonstrated H3K27M positivity, indicative of H3F3A K27M or HIST1H3B K27M mutation, in 9 (36%) patients. H3K27me3-loss was evident in 13 (52%) patients. In one patient with a grade 1 tumor that showed negative staining for H3K27M and H3K27me3 loss, numbers of EZHIP-positive cells were increased, suggesting diffuse midline glioma, H3K27-altered (WHO grade 4). H3K27me3 loss, frequency of p53 positive cells (≥10%), MIB-1 index (≥10%), and high histopathological grades significantly correlated with poor OS. These results indicate the pathological and immunohistochemical characteristics of primary spinal cord gliomas that impact prognosis.

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.

Real-time detection of hepatic micrometastases from pancreatic cancer by intraoperative fluorescence imaging: preliminary results of a prospective study.

BACKGROUND: Recently, a highly sensitive fluorescent imaging technique was developed for the real-time identification of hepatic tumors. The authors applied this procedure for the intraoperative detection of radiographically occult hepatic micrometastases from pancreatic cancer. METHODS: Forty-nine consecutive patients with pancreatic cancer who underwent surgical intervention were examined. Preoperative clinical images had not revealed any hepatic metastases. On the day before surgery, indocyanine green was injected intravenously. During the operation, the liver was observed with a near-infrared camera system, and abnormal fluorescent foci were examined by frozen-section histology. The patients with hepatic micrometastases were judged to have unresectable disease and underwent only palliative surgery followed by systemic chemotherapy using gemcitabine. RESULTS: Abnormal hepatic fluorescence at least 1.5 mm in greatest dimension without any apparent tumor was observed in 13 patients. Among them, histologic examination confirmed micrometastases in 8 of 49 patients (16%). All patients with hepatic micrometastases had clinical T3 or T4 disease and high serum CA19-9 levels (P = .042). On follow-up computed tomography images that were obtained within 6 months after surgery, the patients with hepatic micrometastases manifested hepatic overt metastases (7 of 8 patients; 88%) more frequently than the patients without hepatic micrometastases (4 of 41 patients; 10%; P < .001). Regardless of histologic confirmation, the positive predictive value of abnormal fluorescence for the manifestation of hepatic relapse within 6 months was 77% (10 of 13 patients), and the negative predictive value was 97% (35 of 36 patients). CONCLUSIONS: Indocyanine green-fluorescent imaging can detect hepatic micrometastases of pancreatic cancer during surgery. The hepatic micrometastases seem to have an adverse clinical impact identical to that of evident distant metastases.

The effectiveness of postmortem multidetector computed tomography in the detection of fatal findings related to cause of non-traumatic death in the emergency department.

OBJECTIVES: To investigate the diagnostic performance of postmortem multidetector computed tomography (PMMDCT) for the detection of fatal findings related to causes of non-traumatic death in the emergency department (ED). METHODS: 494 consecutive cases of clinically diagnosed non-traumatic death in ED involving PMMDCT were enrolled. The fatal findings were detected on PMMDCT and classified as definite or possible findings. These findings were confirmed by autopsy in 20 cases. RESULTS: The fatal findings were detected in 188 subjects (38.1%) including 122 with definite (24.7%) and 66 with possible finding (13.4%). Definite findings included 21 cases of intracranial vascular lesions, 84 with intra-thoracic haemorrhage, 13 with retroperitoneal haemorrhage and one with oesophagogastric haemorrhage. In three patients who had initially been diagnosed with non-traumatic death, PMMDCT revealed fatal traumatic findings. Two definite findings (two haemopericardiums) and seven possible findings (two intestinal obstructions, one each of multiple liver tumours central pulmonary artery dilatation, pulmonary congestion, peritoneal haematoma, and brain oedema) were confirmed by autopsy. The causes of death were not determined in cases with possible findings without autopsy. CONCLUSIONS: PMMDCT is a feasible tool for detecting morphological fatal findings in non-traumatic death in ED. It is important to know the ability and limitation of PMMDCT.

Kikuchi-Fujimoto disease following vaccination against human papilloma virus infection and Japanese encephalitis.

Kikuchi-Fujimoto disease (KFD) is a benign and self-limited syndrome, characterized by regional lymphadenopathy and fever. Although the pathogenesis of KFD remains unclear, infectious agents, autoimmune causes, and physicochemical factors have been suggested as triggers. However, KFD following vaccination has never been reported. We present a 14-year-old girl who suffered from fever and cervical lymph node swelling following simultaneous administration of human papilloma virus vaccine and Japanese encephalitis virus vaccine. The patient was diagnosed with KFD based on the histopathologic findings of a lymph node biopsy, and her fever and swelling resolved with oral corticosteroid therapy. Although the exact pathogenesis of the development of KFD following immunization remains unknown, this should be added to the list of potential triggers or factors associated with the development of KFD.