DDIT3-amplified or low-polysomic pleomorphic sarcomas without MDM2 amplification: Clinicopathological review and immunohistochemical profile of nine cases.

Several high-grade pleomorphic sarcoma cases that cannot be classified into any existing established categories have been reported. These cases were provisionally classified into undifferentiated pleomorphic sarcoma (UPS). Some dedifferentiated liposarcoma (DDLS) cases may also have been classified into the UPS category due to the absence of MDM2 amplification or an atypical lipomatous tumor/well-differentiated liposarcoma component. We retrieved and reviewed 77 high-grade pleomorphic sarcoma cases, initially diagnosed as UPS in 66 cases and DDLS in 11 cases. Fluorescence in situ hybridization (FISH) analyses of DDIT3 and MDM2 were performed for available cases. Of the cases successfully subjected to DDIT3 FISH (n = 56), nine (7 UPS and 2 DDLS) showed DDIT3 amplification but no MDM2 amplification. Two UPS cases showed both telomeric (5') and centromeric (3') amplification of DDIT3 or low polysomy of chromosome 12, whereas 5 UPS and 2 DDLS cases showed 5'-predominant DDIT3 amplification. Histopathologically, all cases showed UPS-like proliferation of atypical pleomorphic tumor cells. Immunohistochemically, only one case showed focal nuclear positivity for DDIT3, supporting the previous finding that DDIT3 expression was not correlated with DDIT3 amplification. All three cases with focal MDM2 expression involved 5'-predominant amplification, two of which showed DDLS-like histological features. The majority of cases (7/9) showed decreased expression in p53 staining, suggesting that DDIT3 amplification regulates the expression of TP53 like MDM2. From a clinicopathological perspective, we hypothesize that DDIT3-amplified sarcoma, especially with 5'-predominant amplification, can be reclassified out of the UPS category.

A case of inflammatory myofibroblastic tumor harboring EML4-ALK fusion with a brain metastasis responding to alectinib.

Metastatic inflammatory myofibroblastic tumor (IMT) is very rare and detailed reports on diagnosis and treatment are limited. Here, we report a case of metastatic IMT with ALK rearrangement. A 73-year-old woman was diagnosed with IMT involving a brain metastasis. Next generation sequencing (NGS) panel testing with Oncomine dx target test revealed that her tumor was positive for EML4-ALK. Treatment with alectinib was initiated, resulting in remarkable shrinkage of both the primary tumor and the brain metastasis. This report is the first to identify ALK rearrangement in IMT using a commercially available NGS panel testing, followed by treatment with alectinib. This case suggests that NGS panel testing may be useful in the diagnosis and treatment of patients with metastatic IMT.

Conversion surgery for initially unresectable locally advanced pancreatic ductal adenocarcinoma after chemotherapy followed by carbon-ion radiotherapy: a case report.

BACKGROUND: Recent advances in chemotherapy and chemoradiotherapy have enabled conversion surgery (CS) to be performed for selected patients with initially unresectable locally advanced (LA) pancreatic ductal adenocarcinoma (PDAC). Many studies indicate CS might extend the survival of patients with initially unresectable LA PDAC. However, several clinical questions concerning CS remain, such as the optimal preoperative treatment. Carbon-ion radiotherapy (CIRT) is a unique radiotherapy that offers higher biological effectiveness than conventional radiotherapy. Here, we report a long-term survival case with initially unresectable LA PDAC who underwent CS after chemotherapy followed by CIRT. CASE PRESENTATION: The patient was a 72-year-old Japanese woman with unresectable LA pancreatic head cancer with tumor contact to the superior mesenteric artery (SMA). She underwent four courses of chemotherapy (gemcitabine plus nanoparticle albumin-bound paclitaxel). However, the lesion did not shrink and tumor contact with the SMA did not improve after chemotherapy. Because the probability of achieving curative resection was judged to be low, she underwent radical dose CIRT, and chemotherapy was continued. She complained of vomiting 2 months after CIRT. Although imaging studies showed no tumor growth or metastasis, a duodenal obstruction which was speculated to be an adverse effect of CIRT was observed. She could not eat solid food and a trans-nasal feeding tube was inserted. Therapeutic intervention was required to enable enteral nutrition. We proposed several treatment options. She chose resection with the expectation of an anti-tumor effect of chemotherapy and CIRT rather than course observation with tube feeding or bypass surgery. Therefore, subtotal-stomach-preserving pancreatoduodenectomy with portal vein resection was performed as CS. Pathological examination of the resected specimen revealed an R0 resection with a histological response of Evans grade IIA. Postoperatively, she recovered uneventfully. Adjuvant chemotherapy with tegafur/gimeracil/oteracil (S1) was administrated. At the time of this report, 5 years have passed since the initial consultation and she has experienced no tumor recurrence. CONCLUSIONS: The present case suggests that multidisciplinary treatment consisting of a combination of recent chemotherapy and CIRT may be beneficial for unresectable LA PDAC. However, further studies are required to assess the true efficacy of this treatment strategy.

Multiple angiosarcomas of both breasts: a case report.

BACKGROUND: Primary angiosarcomas of the breast are rare and highly aggressive. We herein report a rare case of multiple angiosarcomas detected concurrently in both breasts. CASE PRESENTATION: A 49-year-old woman visited a doctor after noticing a lump in her right breast. At that time, mammography and ultrasonography revealed no abnormal findings in either breast. She was referred to our hospital 5 months later, because screening mammography had revealed a focal asymmetric density in her right breast. Ultrasonography showed ill-defined hyper- and hypo-echoic lesions in both breasts. Magnetic resonance imaging disclosed five heterogeneously enhanced masses (5.8 cm in maximum diameter) in the right breast and six enhanced masses (approximately 1-3 cm in diameter) in the left breast. Histological examination of core needle biopsies revealed proliferation of irregularly shaped vascular channels lined by atypical endothelial cells throughout the adipose tissue and lobules of the breasts, leading to a diagnosis of well-differentiated angiosarcoma. The lesions were assumed to be primary angiosarcomas, because she had neither a history of breast surgery nor of radiation therapy. She underwent bilateral mastectomies and postoperative chest wall irradiation. Computed tomography 11 weeks after the surgery revealed multiple, small, subcutaneous nodules in the chest wall that were suspected of being angiosarcoma metastases. We started chemotherapy (weekly paclitaxel 80 mg/m2), which achieved shrinkage of these nodules within 2 months. CONCLUSIONS: Early diagnosis, immediate initiation of local and systemic therapies, and intensive follow-up are important in improving the prognosis of angiosarcomas.

Anaplastic lymphoma kinase-positive inflammatory myofibroblastic tumor of the breast: a case report and review of the literature.

BACKGROUND: Few reports of inflammatory myofibroblastic tumor (IMT) of the breast have been published worldwide. Furthermore, primary anaplastic lymphoma kinase (ALK)-positive IMT of the breast is extremely rare. To date, only six patients with ALK-positive IMT have been reported in the literature. CASE PRESENTATION: A 52-year-old woman underwent a medical examination, and a left breast mass was detected. She did not feel a mass in her chest. Mammography showed a focal asymmetric density at the lower outer portion of the left breast. Breast ultrasonography showed a 1.2-cm hypoechoic lesion with relatively clear boundaries and poor blood flow. Magnetic resonance imaging and computed tomography revealed a solitary heterogeneous mass in the left breast. Pathologic examination revealed a fibrosing lesion with proliferation of fibroblastic cells arranged in a storiform pattern and admixed inflammatory cells. Immunohistochemical examination showed that the tumor cells were positive for ALK. Under the preoperative diagnosis of IMT, we performed partial mastectomy with adequate margins. The postoperative diagnosis was pathologically confirmed as IMT. Immunohistochemical staining also showed overexpression of ALK-1 in the tumor. The patient had a good clinical course for 24 months postoperatively, without recurrence or metastasis. CONCLUSIONS: IMT of the breast shows nonspecific imaging findings, making preoperative diagnosis difficult. Nevertheless, IMT has the characteristics of low-grade neoplasms with recurrence, invasion, and metastatic potential. Our report emphasizes the importance of determining a treatment plan as soon as possible based on an accurate diagnosis to improve the prognosis of this disease.

Mixed neuroendocrine-non-neuroendocrine neoplasm of the ampulla of Vater: a case report.

BACKGROUND: Mixed neuroendocrine-non-neuroendocrine neoplasms of the ampulla of Vater are rare and heterogenous, making it difficult to achieve a definitive preoperative diagnosis. Herein, we describe a patient in whom a provisional diagnosis of mixed neuroendocrine-non-neuroendocrine neoplasm of the ampulla of Vater was made preoperatively. CASE PRESENTATION: Computed tomography revealed an enhancing periampullary tumor in a 69-year-old man with obstructive jaundice. Subsequent duodenoscopy revealed an ulcerated lesion in the swollen ampulla of Vater, from which six biopsies were collected. Pathological examination revealed adenocarcinoma in five of them. The remaining one was a neuroendocrine neoplasm according to immunohistochemical analysis. With a provisional diagnosis of mixed neuroendocrine-non-neuroendocrine neoplasm of the ampulla of Vater, the patient underwent subtotal stomach-preserving pancreaticoduodenectomy with modified Child's reconstruction and was discharged without complications. Pathological examination revealed both adenocarcinoma and neuroendocrine carcinomas, each accounting for ≥ 30% of the tumor, resulting in a definitive diagnosis of mixed neuroendocrine-non-neuroendocrine neoplasm of the ampulla of Vater. Lymph node metastases with neuroendocrine components were also observed. Adjuvant chemotherapy was not administered because of the patient's renal dysfunction. Liver and lymph node metastases were detected 2 months after surgery, the neuroendocrine component being considered responsible for that relapse. The patient underwent platinum-based chemotherapy at 50% dosage, which initially resulted in significant tumor shrinkage; however, he died 6 months after surgery. CONCLUSIONS: While these tumors' heterogeneity make definitive preoperative diagnosis of mixed neuroendocrine-non-neuroendocrine neoplasm of the ampulla of Vater difficult, the possibility of this disease can be considered by careful examination. Further study is needed to establish the optimal diagnostic criteria and treatment strategy.

Relationship between prognostic impact of N3 lymph node metastasis at the root of the feeding artery and location of colon cancer.

PURPOSE: To determine whether N3 nodal involvement predicts outcomes and whether its prognostic implications vary with tumor location in patients with Stage III colon cancer (CC). METHODS: We defined N3 as lymph node metastases near the bases of the major feeding arteries. We retrospectively examined recurrence rates and patterns by tumor location and sites of lymph node metastases in 57 patients with N3 CC who had undergone curative resections between January 2000 and March 2019. Survival analysis was performed to compare the prognoses of patients with and without N3 lymph node metastasis. RESULTS: Most N3 patients had large tumors (T ≥ 3); five had T2 disease. Recurrence occurred quickly in one patient with T2N3M0 disease. Multivariate survival analysis demonstrated that N3 lymph node metastasis is an independent predictor of poor prognosis in Stage III CC patients (P < 0.001). Categorizing N3 patients according to UICC-TNM staging system does not stratify risk of recurrence (P = 0.970). To investigate the impact of tumor location on recurrence risk, we classified N3 CC into two subtypes according to tumor location: metastasis at the base of the superior mesenteric artery in right-sided CC and inferior mesenteric artery in left-sided CC. The former was found to have a statistically significant poorer prognosis than the latter (P = 0.091). CONCLUSION: N3 is a robust prognostic marker in CC patients. Recurrence risk varies by tumor location. N3 right-sided CCs with lymph node metastasis at the base of the superior mesenteric artery have poorer prognoses than do N3 left-sided CCs.

Cross-sectional area of psoas muscle as a predictive marker of anastomotic failure in male rectal cancer patients: Japanese single institutional retrospective observational study.

PURPOSE: Preoperative sarcopenia worsens postoperative outcomes in various cancer types including colorectal cancer. However, we often experienced postoperative anastomotic leakage in muscular male patients such as Judo players, especially in rectal cancer surgery with lower anastomosis. It is controversial whether the whole skeletal muscle mass impacts the potential for anastomotic failure in male rectal cancer patients. Thus, the purpose of this study was to clarify whether skeletal muscle mass impacts anastomotic leakage in rectal cancer in men. METHODS: We reviewed the medical charts of male patients suffering from rectal cancer who underwent colo-procto anastomosis below the peritoneal reflection without a protective diverting stoma. We measured the psoas muscle area and calculated the psoas muscle index. RESULTS: One hundred ninety-seven male rectal cancer patients were enrolled in this study. The psoas muscle index was significantly higher in patients with anastomotic leakage (P<0.001). Receiver operating characteristic curve determined the optimal cut-off value of the psoas muscle index for predicting anastomotic leakage as 812.67 cm2/m2 (sensitivity of 60% and specificity of 74.3%). Multivariate analysis revealed that high psoas muscle index (risk ratio [RR], 3.933; P<0.001; 95% confidence interval [CI], 1.917-8.070) and super low anastomosis (RR, 2.792; P=0.015; 95% CI, 1.221-6.384) were independent predictive factors of anastomotic leakage. CONCLUSION: This study showed that male rectal cancer patients with a large psoas muscle mass who underwent lower anastomosis had a higher rate of postoperative anastomotic leakage.

Histological background of dedifferentiated solitary fibrous tumour.

AIMS: Dedifferentiation is a histological phenomenon characterised by abrupt transition of histology to a sarcomatous component with high-grade malignant potential in solitary fibrous tumour (SFT). The authors histologically reviewed SFT cases to reveal the histological background of dedifferentiated SFTs. METHODS: Clinicopathological and histopathological findings of 145 SFT cases were reviewed. Immunohistochemical staining and genetic analysis were also performed. RESULTS: The non-dedifferentiated components showed a cellular component in 45 of 145 (31%), high mitotic rate (≥4/10 high-powered field) in 12 of 145 (8.2%) tumours, necrosis in 7 of 145 (4.8%) tumours, multinodular growth pattern in 39 of 132 (29.5%) available tumours and intratumoural fibrous septa in 37 of 131 (28.2%). Immunohistochemically, the non-dedifferentiated components were positive for CD34 in 128 of 141 (90.7%), bcl-2 in 101 of 133 (75.9%), nuclear pattern of ß-catenin in 64 of 127 (50.3%) and p16 in 22 of 140 (15.7%). Loss of Rb protein expression was detected in 17 of 110 (15.4%) cases. Statistically, cellular component, multinodular structure, p16 overexpression and Rb protein loss were significantly associated with dedifferentiation. Moreover, cellular component and multinodular structure were significantly associated with p16 overexpression and Rb protein loss. All the non-deddifferentiated components showed wild type of p53 expression. The dedifferentiated components of all 10 dedifferentiated tumours presented positivity for p16 in 9 of 10 (90%) and mutational type of p53 in 5 of 10 (50%). Loss of Rb protein expression was detected in 6 of 10 (60%). CONCLUSIONS: The authors propose that cellular or multinodular transformation may be associated with dedifferentiation. They also suggest that cellular and multinodular transformation may be associated with p16 overexpression and Rb downregulation.

Gallbladder Ciliated Foregut Cyst Suspected of Malignancy Preoperatively.

BACKGROUND: Gallbladder ciliated foregut cysts (CFCs) of the lower diaphragm are extremely rare. Furthermore, they are rarely suspected of malignancy preoperatively. Case Presentation. A 50-year-old woman was referred to our hospital for further examination and treatment of a gallbladder tumor that was detected using abdominal ultrasonography (US). After a close inspection, she was diagnosed with a gallbladder tumor that was possibly malignant. Accordingly, open whole layer cholecystectomy was performed because intraoperative US revealed a tumor located on the intraperitoneal side of the gallbladder, and a rapid intraoperative pathological diagnosis identified no malignancy. A postoperative pathological examination revealed a cystic lesion with thin walls covered with ciliated epithelium, which laid on a connective tissue with smooth muscle fibers. Based on the above results, the final pathological diagnosis was CFC of the gallbladder without malignancy. CONCLUSIONS: Cases of gallbladder CFC can be considered as cysts requiring treatment owing to CFCs' potential for malignant transformation and high-frequency symptoms.

Tumor microenvironment in giant cell tumor of bone: evaluation of PD-L1 expression and SIRPα infiltration after denosumab treatment.

Giant cell tumor of bone (GCTB) is an intermediate malignant bone tumor that is locally aggressive and rarely metastasizes. Denosumab, which is a receptor activator of nuclear factor kappa B ligand (RANKL) inhibitor, can be used to treat GCTB. We focused on potential immunotherapy for GCTB and investigated the tumor microenvironment of GCTB. Programmed death-ligand 1 (PD-L1) and indoleamine 2,3-dioxygenase 1 (IDO1) expression and signal-regulatory protein alpha (SIRPα), forkhead box P3 (FOXP3), and cluster of differentiation 8 (CD8) infiltration were assessed by immunohistochemical studies of 137 tumor tissues from 96 patients. Of the naive primary specimens, 28% exhibited PD-L1 expression and 39% exhibited IDO1 expression. There was significantly more SIRPα+, FOXP3+, and CD8+ cell infiltration in PD-L1- and IDO1-positive tumors than in PD-L1- and IDO1-negative tumors. The frequency of PD-L1 expression and SIRPα+ cell infiltration in recurrent lesions treated with denosumab was significantly higher than in primary lesions and recurrent lesions not treated with denosumab. PD-L1 expression and higher SIRPα+ cell infiltration were significantly correlated with shorter recurrence-free survival. PD-L1 and SIRPα immune checkpoint inhibitors may provide clinical benefit in GCTB patients with recurrent lesions after denosumab therapy.

Predictive factors associated with relapse of stage II/III colon cancer treated with peroral anti-cancer agents in the adjuvant setting.

Postoperative adjuvant chemotherapy for patients with stage III colon cancer (CC) is regarded as the standard treatment worldwide for outcome improvement and relapse prevention. Similarly, high-risk stage II CC requires adjuvant chemotherapy because of its high recurrence rate. Previous randomized controlled trials showed that oxaliplatin (OX), in addition to fluorinated pyrimidine-based therapy for patients with stage II/III CC, significantly improves cancer survival but it remains controversial as to which patient groups should receive OX-containing regimens. Among 1,150 consecutive patients who underwent curative resection for stage II/III CC between 2009 and 2016 at two tertiary hospitals, 349 patients treated with only peroral (PO) fluorinated pyrimidine-based chemotherapy and 149 patients who received fluorinated pyrimidine-based chemotherapy with OX as adjuvant chemotherapy were retrospectively reviewed. The primary outcome was recurrence-free survival (RFS). Clinicopathological factors were more advanced in patients treated with OX than in patients treated only with PO fluorinated pyrimidine agents. Multivariate analysis for 5-year RFS showed that T4 [hazard ratio (HR), 2.947; P=0.0001], N2 (HR, 2.704; P=0.0075), vessel or lymphatic invasion (HR, 1.675; P=0.0437) and high cancer antigen (CA)19-9 (HR 3.367, P=0.0002) levels were independent risk factors of cancer relapse. Propensity score matching analysis was performed to match clinicopathological differences between the PO and OX groups. After matching, subgroup analysis of the patients showed that greater effects of OX on cancer survival were observed in patients in the OX group with high CA19-9 levels and tended to be associated with T4 and N2 compared with the PO group. Thus, OX-containing regimens should be recommended for patients with CC with these factors in an adjuvant setting.

Nuclear grade and comedo necrosis of ductal carcinoma in situ as histopathological eligible criteria for the Japan Clinical Oncology Group 1505 trial: an interobserver agreement study.

OBJECTIVE: The Japan Clinical Oncology Group 1505 trial is a single-arm multicentre prospective study that examined the possibility of non-surgical follow-up with endocrine therapy for patients with low-grade ductal carcinoma in situ. In that study, the eligible criteria included histopathological findings comprising low to intermediate nuclear grade and absence of comedo necrosis, and cases were entered according to the local histopathological diagnosis. Nuclear grade is largely based on the Consensus Conference criteria (1997), whereas comedo necrosis is judged according to the Rosen's criteria (2017). The purpose of this study was to standardize and examine the interobserver agreement levels of these histopathological criteria amongst the participating pathologists. METHODS: We held slide conferences, where photomicrographs of haematoxylin-eosin-stained slides from 68 patients with ductal carcinoma in situ were presented using PowerPoint. The nuclear grade and comedo necrosis statuses individually judged by the pathologists were analysed using κ statistics. RESULTS: In the first and second sessions, where 22 cases each were presented, the interobserver agreement levels of nuclear grade whether low/intermediate grade or high grade were moderate amongst 29 and 24 participating pathologists, respectively (κ = 0.595 and 0.519, respectively). In the third session where 24 cases were presented, interobserver agreement levels of comedo necrosis or non-comedo necrosis were substantial amongst 25 participating pathologists (κ = 0.753). CONCLUSION: Although the concordance rates in nuclear grade or comedo necrosis were not high in a few of the cases, we believe that these results could provide a rationale for employing the present criteria of nuclear grade and comedo necrosis in the clinical study of ductal carcinoma in situ.

Correction to: Long-term survival after hepatectomy for metachronous liver metastasis of pancreatic ductal adenocarcinoma: a case report.

An amendment to this paper has been published and can be accessed via the original article.

Development of mesothelioma in situ and its progression to invasive disease observed in a patient with uncontrolled pleural effusions for 15 years.

Mesothelioma in situ (MIS) has recently been investigated as a distinct phase of mesothelioma carcinogenesis. The diagnostic criteria proposed for MIS include a loss of BRCA1-associated protein 1 (BAP1) expression detected by immunohistochemistry (IHC). However, the length of time that MIS typically remains an in situ lesion before progression to invasive disease is still unclear. Herein, we report a case of a Japanese woman in her early seventies who had suffered from recurrent pleural effusions for 15 years, during which MIS developed and progressed to invasive mesothelioma. Retrospective diagnosis of partial MIS, fully developed MIS, and invasive disease was made using BAP1 IHC on three biopsy specimens and via clinical observations with radiological images. MIS and invasive lesions revealed BAP1 loss. The interval from partial or full MIS to invasive disease was 14 and 7 years, respectively. These results support a diagnostic strategy combining histomorphology with genomic-based assays including BAP1 IHC in biopsy tissues from a patient with recurrent pleural effusions.

Synchronous solid pseudopapillary neoplasm and invasive ductal carcinoma of the pancreas: a case report.

BACKGROUND: Solid pseudopapillary neoplasm (SPN) of the pancreas is an extremely rare neoplasm with a favorable prognosis. On the other hand, pancreatic invasive ductal carcinoma (IDC) is known to be an aggressive malignancy. To the best of our knowledge, there is no report of SPN combined with IDC of the pancreas. CASE PRESENTATION: A 66-year-old woman presented with abnormal genital bleeding and was diagnosed with inoperable cervical cancer. During computed tomography for cancer staging, the patient was incidentally diagnosed with pancreatic cancer. After radiation therapy for the cervical cancer, distal pancreatectomy with D2 lymph node dissection was performed. A postoperative pathological examination revealed SPN with ossification and well-differentiated IDC in the pancreatic body. On immunohistochemical staining, SPN tumor cells showed positive ß-catenin and CD10 staining, whereas IDC cells were negative for both. The tumor boundaries were clear. Accordingly, the final pathological diagnosis was synchronous SPN and IDC of the pancreas. Moreover, pathological findings such as the ossification and small number of SPN cells suggested that SPN may have existed long before IDC initiation. CONCLUSIONS: Here, we report the first case of SPN combined with IDC of the pancreas. They may occur independently, and the long-term presence of SPN may lead to the development of IDC.

Long-term survival after hepatectomy for metachronous liver metastasis of pancreatic ductal adenocarcinoma: a case report.

BACKGROUND: Pancreatic ductal adenocarcinoma (PDAC) is among the most aggressive malignancies. The prognosis for recurrence after surgery is extremely unfavorable, and liver metastasis of PDAC confers poor prognosis despite resection. CASE PRESENTATION: A 69-year-old man was admitted to our hospital for further examination and treatment, including surgery for a pancreatic tumor. On close inspection, he was suspected to have pancreatic head cancer without enlarged lymph nodes or distant metastasis, and pancreatoduodenectomy with D2 lymph node dissection was performed. A postoperative pathological examination revealed well-differentiated invasive ductal adenocarcinoma with lymph node metastasis (stage IIB; pT2N1M0). Postoperatively, he received adjuvant chemotherapy containing gemcitabine for 1 year. Eight years after the radical surgery, his serum carbohydrate antigen 19-9 level was elevated, and computed tomography (CT) and magnetic resonance imaging revealed a well-circumscribed 10-mm mass in liver segment 5. Positron emission tomography/CT also revealed high fluorine-18-fluorodeoxyglucose uptake only in this hepatic tumor. Accordingly, the patient was diagnosed with a solitary liver metastasis of PDAC. As the liver metastasis was isolated and identified long after the initial surgery, we decided to resect it using laparoscopic partial hepatectomy of segment 5. Histopathological examination confirmed liver metastasis of PDAC and the patient received adjuvant chemotherapy containing S-1. No evidence of recurrence has been seen for 11 years since the pancreatoduodenectomy and 3 years since the hepatic resection. CONCLUSIONS: Cases of metachronous liver metastasis of PDAC after radical surgery, in which patients exhibit long-term survival without recurrence after hepatectomy, are extremely rare. Hepatectomy may confer long-term survival, and the time to postoperative recurrence and the number of liver metastases may be useful criteria for deciding whether to perform hepatic resection.

Intramural metastasis to the appendix from ascending colon cancer: a case report.

BACKGROUND: Intramural metastasis is rare in colorectal cancer, especially metastasis of ascending colon cancer to the appendix. CASE PRESENTATION: A 64-year-old man was admitted to our hospital for surgery for ascending colon cancer detected by medical examination. Colonoscopy identified a type-2 tumor in the ascending colon, which was diagnosed as adenocarcinoma. Abdominal computed tomography revealed focal thickening of the ascending colon and middle of the appendix and swelling of the lymph nodes around the ileocolic artery. The patient underwent laparoscopic right hemi-colectomy with D3 lymph node dissection. Histopathological findings revealed that the ascending colon cancer was moderately differentiated adenocarcinoma with lymphatic and vascular invasion (stage IIIB; pT3N2M0). Additionally, moderately differentiated adenocarcinoma was observed mainly in the submucosa and muscularis propria of the appendix, which was approximately 10 cm proximal to the ascending colon cancer. These findings indicated intramural metastasis to the appendix from the ascending colon cancer. The patient experienced recurrence with lung metastasis 2.5 years after the first surgery. CONCLUSIONS: Intramural metastasis of ascending colon cancer to the appendix is extremely rare. Because the risk of recurrence and the prognosis for intramural metastasis has not been clarified, careful follow-up is recommended.

Correction to: Intramural metastasis to the appendix from ascending colon cancer: a case report.

In the original publication of this article [1], an author's name should be changed from Shin Takasue to Shin Takesue.

A Case of Repeated TAFRO Syndrome-Like Symptoms and Retroperitoneal Hemorrhage in a Patient With Sjögren Syndrome.

A 50-year-old Japanese man complaining of dry mouth and eyes, pale skin with cold irritation, and worsening epigastric pain was admitted to the hospital, whereupon he developed fever and anasarca. A computed tomography (CT) scan showed ascites, hepatosplenomegaly, and mildly enlarged multiple lymph nodes, and blood examination revealed renal impairment, thrombocytopenia, and high levels of C-reactive protein (CRP). He was diagnosed with Sjögren syndrome and concurrently manifested symptoms resembling TAFRO syndrome (i.e., thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O)). Although the TAFRO syndrome-like symptoms progressed, he gradually recovered with immunosuppressive agents. Seven years and five months after the admission, the TAFRO syndrome-like symptoms recurred. Bone marrow biopsy specimens revealed reticulin fibrosis. Inguinal and mediastinal lymph nodes biopsy specimens revealed Castleman disease-like features. Although the symptoms indicated TAFRO syndrome, a diagnosis was not possible owing to the presence of hypergammaglobulinemia and Sjögren syndrome, which required exclusion. Corticosteroid treatment was initiated; however, it was complicated by retroperitoneal hemorrhage, probably due to microangiopathy. After additional treatment with tocilizumab and rituximab, the TAFRO syndrome-like symptoms improved and the hemorrhage progression stopped. In conclusion, TAFRO syndrome-like symptoms may recur with vascular complications and can be successfully treated with tocilizumab and rituximab during Sjögren syndrome. The etiology of TAFRO syndrome could potentially involve Sjögren syndrome, and these syndromes may co-exist.