[A Case Report of an Appendiceal Goblet Cell Carcinoid].

A 56-year-old woman complaining of right lower abdominal pain was admitted to our hospital. An abdominal computed tomography showed the enlarged appendix tip and a high density area around the appendix to retroperitoneum. The patient was diagnosis with acute appendicitis and underwent emergency laparoscopic appendectomy. Histopathological findings of the resected specimens revealed a component with signet ring cell carcinoma morphology that was positive for neuroendocrine markers by immunohistochemical staining, which led to the diagnosis of goblet cell carcinoid(GCC)of appendix. GCC cells were found to infiltrate the surrounding serosa and Ly positive. An additional laparoscopic ileocecal resection with D3 dissection was performed. In the appendix GCC, additional resection is considered because the lymph node metastasis rate increases(SS/13%)as the depth of wall progresses. Appendiceal tumors including GCC may develop acute appendicitis and may be followed by additional resection. Therefore, it is important to consider how to deal with the first surgery.

Clinical silence of pulmonary lymphoepithelioma-like carcinoma with subcutaneous metastasis: a case report.

BACKGROUND: Dissemination of lung cancer to cutaneous sites usually results in a poor prognosis. Pulmonary lymphoepithelioma-like carcinoma (PLELC) is a rare tumor, and no therapeutic strategy for it has yet been established. We present herein an extremely rare case of a long-term surviving patient with PLELC showing subcutaneous metastasis. CASE PRESENTATION: A 76-year-old woman was diagnosed unexpectedly as having PLELC based on a nodule on her back. After surgical resection of the primary and metastatic lesions, she has remained alive with no recurrence for over 5 years without any additional therapy. CONCLUSION: Even in the case of PLELC with subcutaneous metastasis, surgical management may afford a prognosis of long-term survival.

Clinicopathological features of neuroblastic tumors with opsoclonus-myoclonus-ataxia syndrome: Follicular structure predicts a better neurological outcome.

Neuroblastic tumors (NT) with opsoclonus-myoclonus syndrome (OMS) display characteristic histological features, such as lymphocytic infiltration with lymphoid follicles, indicating an underlying immune response. We retrospectively assessed NT patients from 2001 to 2016. Five cases of NT with OMS and 76 cases of NT without OMS were histopathologically reviewed in this study. The grade of lymphocytic infiltration was evaluated. The number of follicles was counted and the presence or absence of lymphoid follicles was recorded for each case. We also confirmed the presence or absence of follicular dendritic cells (FDCs). We investigated the relationship between the histopathological and clinical findings of NT with OMS. Lymphocytic infiltration was observed in all cases; however, the precise follicular structure was occasionally unclear. Patients with clear follicular structures displayed germinal centers including tingible body macrophages and FDCs. All patients without neurological sequelae demonstrated a clear follicular structure with a FDC meshwork pattern. The interval between OMS onset and the detection and initial treatment of NT was typically longer in patients with neurological sequelae compared to those without neurological sequelae. Early detection and treatment of NT with OMS at the phase of a clear follicular formation with multiple FDC may provide favorable neurological outcomes.

Sarcoid reaction in the spleen after sigmoid colon cancer resection: a case report.

BACKGROUND: A sarcoid reaction is a phenomenon characterized by histologically proven granulomatous lesions without evidence of sarcoidosis. This pathology is a benign tumor itself, but several reports have described sarcoid reactions accompanying malignant tumors. Sarcoid reactions occur in various cancers, such as skin, lung, ovary, stomach, and breast cancers. However, only a few published reports have described sarcoid reactions in patients with colorectal cancer. CASE PRESENTATION: A 76-year-old woman underwent laparoscopic sigmoidectomy for sigmoid colon cancer. The postoperative follow-up computed tomography and 18-fluorodeoxyglucose positron emission tomography-computed tomography findings were suspicious for splenic metastasis of the sigmoid colon cancer. The patient then underwent laparoscopic splenectomy. Histopathological examination of the resected lymph nodes and spleen showed a non-caseating epithelioid cell granuloma. The patient was diagnosed with a sarcoid reaction. CONCLUSIONS: To our knowledge, this is the first report of a sarcoid reaction in the spleen and regional lymph nodes after colon cancer resection. The effect of a sarcoid reaction on the prognosis in patients with colorectal cancer has not been fully determined because of the small number of such cases. Further analyses involving a larger number of cases are necessary to evaluate the relationship between sarcoid reactions and prognosis in patients with colorectal cancer. We herein present an extremely rare case of a sarcoid reaction in the spleen and regional lymph nodes.

[Familial Hemophagocytic Lymphohistiocytosis, That was Considered as Progressive Encephalitis before Sibling was Diagnosed].

Familial hemophagocytic lymphohistiocytosis (FHL) is an autosomal recessive disorder that is clinically characterized by fever, hepatosplenomegaly, cytopenia and sometimes vague or dramatic central nervous system (CNS) dysfunction. FHL affecting the CNS imitates several neurologic disorders and may be misdiagnosed, in particular when family history is unknown. We report an autopsy case of FHL that was firstly considered as progressive encephalitis. FHL was suspected after sibling had been affected by hemophagocytosis and the same CNS symptoms. Histopathologically, lymphocytes and macrophages infiltrated into the meninges, perivascular space, and parenchyma of the brain. Those lymphocytes were positive for CD3, CD8, GranzymeB, and negative for CD4, perforin. FHL must be included in the differential diagnostic considerations in children with progressive encephalitis.

[Case of primary malignant melanoma of the female urethra at age 94 : a case report].

A 94-year-old woman visited a local urological clinic in January 2011 complaining of dysuria and vulvar discomfort that lasted for a month. Clinical examination showed a hematoma-like tumor at the external urethral meatus. She refused an operation. After 2 months, the tumor became hemorrhagic and she was referred to our hospital for operation. A black tumor of fingertip-size was seen at the external urethral meatus. Urinary sediment examination and urine cytology revealed malignant melanoma cells. The tumor showed T1WI high intensity, and T2WI low intensity by magnetic resonance imaging. Collectively, the patient was diagnosed with malignant melanoma. The tumor was extirpated. Pathological diagnosis showed malignant nodular melanoma. However, additional therapy was not performed due to her age and thereafter, the patient was followed up carefully. After 3 months, right inguinal LN swelled, and tumor recurred at the external urethral meatus. She died from multiple metastases 11 months from the initial consultation. We herein report this case of primary malignant melanoma of the female urethra in an elderly patient.

[A case of sarcomatoid carcinoma (undifferentiated carcinoma, sarcomatoid type) of the duodenum].

A 98-year-old woman with aspiration pneumonia caused by vomiting was admitted to our hospital. Abdominal computed tomography and ultrasonography showed common bile duct dilation and occlusion of the gastrointestinal tract due to a large tumor in the duodenum. Aspiration pneumonia and hepatic failure led to the patient's death. Pathological autopsy showed an 11 × 6 × 2.5-cm polypoid tumor originating from the descending part of the duodenum and involving the common bile duct. Histological examination of the tumor showed mainly spindle cells but also some adenocarcinoma cells. Tumor cells, including spindle cells, were strongly positive for cytokeratin 7; therefore, sarcomatoid carcinoma originating from the duodenum was diagnosed. To the best of our knowledge, only 5 cases of sarcomatoid carcinoma originating from the duodenum have been reported, and they often show polypoid growth. The possibility of sarcomatoid carcinoma should be considered if such findings are noted.

A case series of children with high-risk metastatic neuroblastoma treated with a novel treatment strategy consisting of postponed primary surgery until the end of systemic chemotherapy including high-dose chemotherapy.

The aim of this study was to clarify the feasibility of a novel treatment strategy consisting of postponed primary surgery till the end of systemic chemotherapy including HDC without interruption by local therapy for neuroblastoma patients at a high risk for relapse. After induction chemotherapy, patients received double conditioning HDC consisting of thiotepa and melphalan. Radical surgery was applied to local lesions. Irradiation was not applied to any lesions. Eleven consecutive pediatric neuroblastoma patients were treated according to this strategy. Seven of 11 patients remained in complete remission for 21-171 months. This treatment strategy seems feasible and a further study is warranted.

Expression pattern of keratin subclasses in pancreatoblastoma with special emphasis on squamoid corpuscles.

Expression patterns of keratins (K), both simple epithelia-type (K7, K8, K18, K19) and complex/stratified epithelia-type (K1, K4, K5/6, K10, K13, K14, K15, K16, K17), and epithelial membrane antigen (EMA) were immunohistochemically studied in six pancreatoblastomas (PBL). In all six tumors, areas with overt acinar differentiation (AA), solid areas without any specific differentiation (SO), and squamoid corpuscles (SC) were diffusely positive for K8, K18, and K19. The AA and SO in all the tumors were diffusely positive for K7, but the SC were negative or displayed only scattered reactivity for K7. In three tumors, the AA and the SC showed scattered reactivity for K5/6. No reactivity for other complex/stratified epithelia-type K was found in any of the examined tumor. All tumors were reactive for EMA with consistent predominancy in the SC. Ultrastructurally, well-developed desmosome-tonofilament complexes were only partially observed in tumor cells comprising the SC. These results implied that (i) the SC usually lack a character of complete squamous metaplasia; and (ii) the SC have a characteristic phenotype (K8/K18/K19/EMA-positive, K7-negative or scatteredly positive) that can potentially be useful to delineate the SC in PBL.

Epigenetic differences between Wilms' tumours in white and east-Asian children.

BACKGROUND: Variations in the international incidence of Wilms' tumour might be due to genetic factors. The maternal insulin-like growth factor 2 gene (IGF2) is imprinted in normal tissues, whereas in some Wilms' tumours and other tumour types the imprint is lost, leading to biallelic transcription of IGF2. We investigated whether the difference in incidence of Wilms' tumour between children of east-Asian descent and white children is due to variations in proportion of tumours with loss of IGF2 imprinting (IGF2 LOI). METHODS: We assessed IGF2 LOI by use of an ApaI polymorphism in IGF2 exon 9 or quantitative PCR measuring DNA methylation of the H19 and KvDMR1 alleles. The frequencies of perilobar nephrogenic rests associated with Wilms' tumour were assessed histologically in Japanese children and children of white and east-Asian descent. FINDINGS: IGF2 LOI was present in Wilms' tumours from predominantly white children from New Zealand (13 of 41 tumours) but absent in tumours from Japanese children (0 of 21 tumours; difference in proportions 0.32, 95% CI 0.07-0.52). Frequency of perilobar nephrogenic rests accompanying tumours from white American children (1192 of 5002, 24%) was significantly higher than in Japanese (one of 56, 1%, difference in proportions 0.22, 95% CI 0.14-0.25) and east-Asian American children (seven of 92, 8%, 0.16, 0.09-0.21). INTERPRETATION: Wilms' tumours in the east-Asian population rarely arise from the IGF2 LOI mechanism frequently noted in white patients. Our findings that IGF2 LOI and perilobar nephrogenic rests associated with this mechanism arise at low frequency in Japanese and east-Asian American children lend support to this conclusion. Variation in frequency of this epigenetic mechanism provides one explanation for the difference in incidence of Wilms' tumour between populations.

Apoptosis and microglial activation in influenza encephalopathy.

During influenza epidemics in Japan, the number of children with acute encephalopathies and encephalitis has recently increased. Although the pathophysiologies remain unclear, there is usually brain edema with evidence of damage to the blood-brain-barrier (BBB). We investigated the glial reaction and apoptosis in brains of eight such cases comprising two of acute necrotizing encephalopathy and six of influenza encephalopathy, and compared the results with those in five control brains. Apoptosis, evidenced by chromatin condensation and fragmentation in hematoxylin sections, in situ end labeling of fragmented DNA (TUNEL) and DNA laddering, was observed in neurons and glial cells in four brains with influenza encephalopathy. In the TUNEL-positive brains, the increase in microglia was greater than in the TUNEL-negative brains. Immunoreactivity for active-caspase 3, demonstrated by immunohistochemistry, and the overexpression of a caspase-cleaved fragment of poly(ADP-ribose) polymerase, demonstrated by Western blotting, indicated that activation of caspase 3 is involved in the apoptotic pathway in the brains of influenza encephalopathy cases. Apoptosis or specific pathological processes that cause apoptosis may give rise to aggravated encephalopathy.

Significance of aberrant (cytoplasmic/nuclear) expression of beta-catenin in pancreatoblastoma.

This study concerns the significance of aberrant (nuclear/cytoplasmic) expression of beta-catenin in pancreatoblastoma (PBL). On immunohistochemistry, all seven PBLs examined showed nuclear/cytoplasmic expression of beta-catenin, predominantly in the squamoid corpuscles (SCs). In areas with acinar/ductular differentiation, few tumour cells displayed nuclear/cytoplasmic expression of beta-catenin and more than half of the tumour cells showed membranous expression. Two out of five (40%) tumours examined showed missense mutations in codons 33 and 37 of exon 3 of the beta-catenin gene. No mutation of the adenomatous polyposis coli (APC) gene was detected in two of the remaining three tumours. Amplifiable DNA for APC analysis was not obtained from the one other tumour. Immunoreactivity for cyclin D1, one of the nuclear targets of beta-catenin, was found predominantly in the SCs of the seven tumours. In contrast, the Ki-67 labelling index was 2-4% (median 3%) in the SCs and 8-18% (median 12%) in the other areas, indicating a negative correlation with nuclear cyclin D1 reactivity. These results imply that in PBLs, nuclear/cytoplasmic accumulation of beta-catenin and overexpression of its target gene cyclin D1 are not associated with the induction of tumour cell proliferation. Nuclear/cytoplasmic accumulation of beta-catenin may be related to the morphogenesis of the SCs that are considered most characteristic for PBL.