[Case of Lung Cancer with High-Grade Fetal Lung Adenocarcinoma Components Treated with Chemotherapy].

A woman in her 70s presented to our hospital with complaints of cough and dyspnea. Computed tomography(CT) images showed a large amount of left pleural effusion, pleural tumors, and mediastinal lymphadenopathy. Left thoracic drainage was performed, and high-grade fetal lung adenocarcinoma was suspected upon pleural effusion-cell block immunostaining. Pathological evaluation of the CT-guided biopsy specimen provided a diagnosis of carcinoma with high-grade fetal lung adenocarcinoma. Although the tumor progressed rapidly, chemotherapy with atezolizumab/bevacizumab/carboplatin/ paclitaxel was highly effective. However, subsequent maintenance therapy with atezolizumab/bevacizumab led to disease progression.

[A Case of Stage â £ Triple Negative Breast Cancer(TNBC)in Which S-1 Was Successful against Armor-Like Infiltration of the Chest Wall].

A 51-year-old female patient visited our department with a complaint of pain in the left breast. She was found to have Stage Ⅳ breast cancer with liver metastasis. The biopsy-based historical diagnosis was triple negative breast cancer(TNBC). Epirubicin plus cyclophosphamide therapy(EC therapy)plus weekly paclitaxel therapy(weekly PTX)was started for the unresectable advanced breast cancer, but infiltration of an armor-like tumor was observed in the chest wall. It was judged that drug resistance had occurred; hence, the treatment was switched to S-1. Subsequently, almost all the chest wall tumors disappeared after 2 months. However, we did not control the disease, and the patient died. We report about the positioning of S-1 with regard to TNBC, including a literature review.

Hypervascular retained product of conception: Characteristic magnetic resonance imaging and possible relationship to placental polyp and pseudoaneurysm.

The cases of two patients who developed myometrial vascularization following dilatation and curettage are described. In case 1, pathological diagnosis was possible with the resected specimens. This patient had hypervascular retained products of conception (RPOC). In case 2, the natural course of this pathological condition was observed, confirming a process of regression during repeated withdrawal bleeding. The three principal magnetic resonance imaging (MRI) findings in these cases were: (i) presence of a remnant; (ii) breaking of the junctional zone in contact with the remnant; and (iii) vascularization/flow voids infiltrating into the myometrium from the broken junctional zone. These three MRI findings differed in degree and varied in combination in each case of RPOC. Uterine artery pseudoaneurysms have been reported as intrauterine vascularization after abortion or delivery with subsequent spontaneous regression. These reports may include cases of hypervascular RPOC.

[PRIMARY MALIGNANT LYMPHOMA OF THE BLADDER DIAGNOSED BY TRANSURETHRAL BLADDER TUMOR RESECTION: A CASE REPORT].

A 72-year-old woman underwent computed tomography (CT) to identify the underlying cause of thrombocytosis. The CT showed a bladder tumor. Urine cytology was negative. Cystoscopic examination showed a dome-shaped bulge at the right lateral wall of the bladder, suggesting a submucosal tumor. The bladder tumor was immediately resected transurethrally. The histological diagnosis was malignant lymphoma of mucosa-associated lymphoid tissue. Positron emission tomography-CT showed no lesions other than the bladder tumor. The patient was diagnosed with primary malignant lymphoma of the bladder.The tumor was low-grade, and strict follow-up was subsequently carried out. There was no evidence of recurrence or metastasis at 13 months after treatment.

Thymic stromal lymphopoietin in tonsillar follicular dendritic cells correlates with elevated serum immunoglobulin A titer by promoting tonsillar immunoglobulin A class switching in immunoglobulin A nephropathy.

Immunoglobulin A (IgA) nephropathy (IgAN) is characterized by high serum IgA levels and IgA deposition in the renal mesangium. Previous studies suggest that elevated serum IgA partly originates from the tonsils. Here, we investigated the mechanisms of IgA production in the tonsils of patients with IgAN. Immunohistochemistry revealed that the number and relative percentage of IgA-bearing cells were significantly increased in the tonsils of IgAN patients. Compared with non-IgAN patients, enhanced IgA class switching and overexpression of thymic stromal lymphopoietin (TSLP), TSLP receptor (TSLPR), activation-induced cytidine deaminase (AID), transforming growth factor-ß1 (TGF-ß1), B cell-activating factor of the tumor necrosis factor family (BAFF), and a proliferation-inducing ligand (APRIL) were detected in follicular dendritic cells (FDCs) of tonsillar germinal centers from IgAN patients. Importantly, TSLP correlated with IgA production in isolated FDC-associated clusters. Serum TSLP levels were increased and correlated with IgA overexpression in the tonsils and serum of IgAN patients. These data indicated that TSLP overexpression in tonsillar FDCs may promote IgA class switching in IgAN patients through the cooperative roles of AID, TGF-ß1, BAFF, and APRIL. Therefore, interactions between TSLP in FDCs and IgA production in tonsils may be an important mechanism contributing to the pathogenesis of IgAN.

Immunoglobulin and CD8⁺ T-cell distribution in histologically distinctive tonsils of individuals with tonsillar focal infection.

CONCLUSION: This study demonstrated that the common immunological mechanism, which involves aberration of immunoglobulin and T-cell distribution in histologically distinctive tonsils, may be associated with the pathogenesis of tonsillar focal infection. OBJECTIVES: Tonsillar focal infection comprises a group of relatively common diseases combined with chronic tonsillar infection, is associated with unusual immune responses in tonsils, and may cause lesions in another distant target organ. This study aimed to investigate the distribution of inflammatory T cells and T-cell regulatory elements, such as programmed cell death-1 (PD-1) and Fork head box protein 3 (Foxp3), immunoglobulin production, and histological characteristics in tonsils from patients with tonsillar focal infection. METHODS: Immunohistochemistry and reverse transcription-polymerase chain reaction (PCR) were used to compare the expression of CD8(+) T cells, immunoglobulins, and cytokines associated with immunoglobulin production in the tonsils of patients with IgA nephropathy (IgAN), palmoplantar pustulosis (PPP), rheumatoid arthritis (RA), and chronic tonsillitis. RESULTS: The overexpression of CD8(+) T cells combined with decreased expression of Foxp3 and PD-1 and the aberration of immunoglobulin production, which may be due to the elevated expression of activation-induced deaminase (AID), B-cell-activating factor of the TNF family (BAFF), supporting isotype switching, and B-cell survival in the histologically distinctive tonsils.

Identification of novel ALK rearrangement A2M-ALK in a neonate with fetal lung interstitial tumor.

Fetal lung interstitial tumor (FLIT) is a recently reported type of congenital lung lesion comprising solid and cystic components. The pathological features include unique interstitial mesenchyme-based cell proliferation, and differ from other neoplasms represented by pleuropulmonary blastoma or congenital peribronchial myofibroblastic tumor. FLIT is extremely rare and its gene expression profile has not yet been reported. We provide the first report of a novel chromosomal rearrangement resulting in α-2-macroglobulin (A2M) and anaplastic lymphoma kinase (ALK) gene fusion in a patient with FLIT. The tumor cells contained a t(2;12)(p23;p13) and were mesenchymal in origin (e.g., inflammatory myofibroblastic tumors), suggesting the involvement of ALK in this case of FLIT. Break apart fluorescence in situ hybridization demonstrated chromosomal rearrangement at ALK 2p23. Using 5'-rapid amplification of cDNA ends, we further identified a novel transcript fusing exon 22 of A2M to exon 19 of ALK, which was confirmed by reverse-transcription polymerase chain reaction. The corresponding chimeric gene was subsequently confirmed by sequencing, including the genomic break point between intron 22 and 18 of A2M and ALK, respectively. Discovery of A2M as a novel ALK fusion partner, together with the involvement of ALK, provides new insights into the pathogenesis of FLIT, and suggests the potential for new therapeutic strategies based on ALK inhibitors.

Interdigitating dendritic cell sarcoma and follicular dendritic cell sarcoma: histopathological findings for differential diagnosis.

Both interdigitating cell sarcoma (IDCS) and follicular dendritic cell sarcoma (FDCS) are rare neoplasms derived from dendritic cells in lymphoid organs. IDCS is defined as a neoplastic proliferation of spindle-shaped to ovoid cells with phenotypic features similar to those of IDCs. FDCS is a malignant neoplasm derived from FDCs that possess and present antigens to B cells in the follicular (germinal) centers of lymphoid organs. They often occur in lymph nodes, although they can also arise at extranodal sites. In this review, we have highlighted the morphological and immunohistochemical properties of these neoplasms, which could help in unequivocal and accurate diagnosis.

A case of myxoid adrenocortical neoplasm: computed tomography and magnetic resonance imaging characteristics.

Myxoid adrenocortical neoplasms are rare; to our knowledge, only 56 cases have been reported in the literature. Therefore, distinguishing benign from malignant cases is challenging. Although the histopathological features of myxoid adrenocortical neoplasia have been amply demonstrated, their imaging characteristics are yet to be reported. We describe here these characteristics for such a neoplasm. Our patient, a 70-year-old male, was found to have a 3-cm left adrenal incidentaloma through a non-enhanced computed tomography. Attenuation measurements were 22 Hounsfield units on precontrast imagery, and percentage enhancement washout was 92%. Magnetic resonance imaging showed no loss of signal intensity in T1-weighted out-of-phase images, but high signal intensity on T2-weighted and diffusion-weighted images. Left adrenalectomy was performed and the pathological diagnosis was confirmed as myxoid adrenocortical neoplasm. The imaging characteristics reported here will be beneficial to the differential diagnosis of myxoid adrenocortical neoplasms based upon image analysis and will help distinguish benign from malignant neoplasms.

Acute kidney injury and inflammatory immune reconstitution syndrome in mixed genotype (A/E) hepatitis B virus co-infection in HIV-associated lymphoma.

We report a first case of HIV-associated lymphoma (HAL) presenting with acute kidney injury (AKI) and inflammatory immune reconstitution syndrome (IRIS). A 39-year-old male, treated with nonsteroidal anti-inflammatory drugs (NSAIDs) for one month prior to admission, developed AKI, left testicular tumor, and recurrent swelling of the right parotid gland. A resected testicular tumor exhibited features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma. Renal biopsy showed hydro-degeneration of renal tubules, interstitial inflammatory cells, and a small number of lymphoma cells in the sub-capsule, compatible with acute interstitial nephritis. His renal dysfunction rapidly recovered following chemotherapy and combination antiretroviral therapy (cART). He developed pneumonia concomitantly with a decrease in HIV-RNA level and an increase in CD4+ cells after the first cycle of chemotherapy, which spontaneously resolved after the second cycle of chemotherapy without additional anti-infection drugs; thus, his pneumonia fulfilled the diagnostic criteria for IRIS. We suggest that IRIS may frequently develop during chemotherapy for HAL, but may be overlooked. He was coinfected with hepatitis B virus (HBV), which genotypes known as is associated with liver-related mortality and response to antiviral therapy; recently, an intimate interplay between HIV and HBV in the onset of lymphoma has been reported. Therefore, we addressed the HBV genotype in the patient. The analysis revealed that he exhibited a mixed genotype (A/E) not native to Japan and primarily found in Europe and North America or West Africa. These findings suggest that universal vaccination for juveniles against HBV is warranted in Japan.

Marked improvement in opsoclonus and cerebellar ataxia after the surgical removal of a squamous cell carcinoma of the thymus: a case report.

A 69-year-old man with rapidly evolving vertigo and ataxia was admitted to our hospital. He was presented with a dysarthric speech and chaotic eye movements, identified as opsoclonus. Neurological examination revealed limb and truncal ataxias and an inability to stand unless fully assisted. A chest CT scan revealed a mass at the anterior mediastinum, which suggested paraneoplastic neurological syndrome (PNS). However, an extensive search for anti-neuronal antibodies linked to cerebellar ataxia failed to find any autoantibodies, including cell surface autoantibodies. Subsequently, a total surgical removal of the thymic tumor was performed, leading to marked improvements in his signs and symptoms. The pathological findings by conventional and immunohistochemical examinations confirmed a squamous cell carcinoma of the thymus. Three months after onset his signs and symptoms improved and he was able to walk without support. In contrast to thymomas, PNS is extremely rare in patients with thymic carcinoma. Previous reports have shown that neurological symptoms, similar to opsoclonus or cerebellar ataxia, deteriorated in cases of thymic carcinoma that could not be controlled. The present report indicates that early diagnosis and total removal of the rare neoplasm may increase the possibility of neurological recovery.

Myoepithelial carcinoma of the breast with focal rhabdoid features.

Myoepithelial carcinoma of the breast is extremely rare and only 33 cases have been reported in the English literature. Herein, we report a case of myoepithelial carcinoma of the breast with focal rhabdoid features. The patient was a 67-year-old woman, who presented with a lump of the left breast that rapidly grew to 3 cm in diameter within 3 months. Lumpectomy revealed a solid and whitish colored tumor, which was composed mainly of elongated spindle-shaped cells with mild atypia, focal necrosis, and infiltrative margin. In a small area of the lesion, ovoid tumor cells exhibited eccentric nuclei with centrally located nucleoli and plump cytoplasm including round eosinophilic inclusions, resembling a rhabdoid tumor. Immunohistochemically, both types of tumor cells exhibited a myoepithelial phenotype. MIB-1 index was 30%. The cytoplasmic inclusion of the ovoid cells exhibited immunopositivity for both vimentin and cytokeratin. From these findings, this tumor was diagnosed as a myoepithelial carcinoma with focal rhabdoid features. Although rhabdoid features have been reported in some types of malignant and benign tumors, this is the first report of such features in myoepithelial carcinoma of the breast.

IgA production and tonsillar focal infection in IgA nephropathy.

IgA nephropathy (IgAN), the common primary glomerulonephritis, is a tonsillar focal infection characterized by the qualitative abnormality of IgA in circulation and IgA deposition in the renal mesangium. Mesangial deposition of IgA, which is composed predominantly of poorly galactosylated polymeric IgA1 (pIgA1), seems to be the initiating event in the pathogenesis of IgAN. The origin of poorly galactosylated IgA, however, remains unclear. Recent studies suggest that the mesangial polymeric IgA1 deposition could be derived from mucosally primed plasma cells. B cells may undergo IgA class switching to acquire the expression of IgA via T-cell-dependent or T-cell-independent pathways in mucosa-associated lymphoid tissue and then differentiate to IgA plasma cells or home in on systemic sites. Dendritic cells, including plasmacytoid dendritic cells and another type of antigen-retaining cell, follicular dendritic cells, have an irreplaceable role in IgA class-switch mechanisms by producing IgA-inducing signals. Furthermore, an increased number of pIgA1-secreting plasma cells in the bone marrow and tonsil, as well as increased IgA class switching, have been found in IgAN, providing a link between the mucosal immunity and IgAN. The favorable effect of tonsillectomy on patients with IgAN showed that tonsillar focal infection may be closely related to pIgA1 deposition in glomerular mesangium of patients with IgAN and at least a part of pIgA1 may originate from affected tonsils. Therefore, the indication for tonsillectomy should be considered in patients with IgA nephropathy, especially at a mild or early stage, to prevent future renal deterioration. In this paper, we focus on IgA class switching and the role of tonsils with focal infection in IgAN.

Angioimmunoblastic T-cell lymphoma with dual genotype of TCR and IgH genes.

A 70-year-old man complained of fever and sore throat accompanied by hoarseness of voice. On physical examination, there was no systemic abnormality but a mild lymphadenopathy of cervical lymph nodes. With laryngoscopy, there was a marked outgrowth of the bilateral palatine tonsils proximal to the vocal cord. The histology of the resected tumor was compatible with angioimmunoblastic T cell lymphoma (AITL), revealing the effacement of normal tonsillar architecture and small to medium-sized neoplastic cell proliferation around marked vascular proliferation and atrophic lymphoid follicles. Tumor cells were positive for conventional T-cell antigens as well as for the follicular helper T-cell marker, PD-1, and CXCL13. Large hodgkinoid cells, but no tumor cells, were positive for latent membrane protein-1 and Epstein-Barr virus-encoded small RNA (EBER)-1 (in situ hybridization). Non-neoplastic, double positive cells for EBER-1 and CD20 were also scattered. Southern blot analysis revealed dual TCR-Cß1 and IGH-JH gene rearrangements. Although the swelling of bilateral inguinal and perigastric lymph nodes developed later, the radical resection of tumor and chemotherapy appeared to be effective for the treatment of AITL with clinical stage IIIa. We here report a rare case of AITL involving palatine tonsil as primary site and give a review of the literature.

Oncocytic adrenocortical carcinoma.

Only 17 cases of oncocytic adrenocortical carcinoma have been reported in the English literature. Here, we report an incidental case of oncocytic adrenocortical carcinoma. The patient was a 69-year-old man with the chief complaint of abdominal pain. Abdominal computed tomography revealed a left adrenal tumor. No hormonal symptoms were observed. The excised tumor was whitish, encapsulated, and 75 x 60 x 45 mm in size. Large polygonal tumor cells were arranged in a generally diffuse architecture and exhibited abundant eosinophilic granular cytoplasm. Nuclear atypia with atypical mitotic figures and capsular and sinusoidal invasions were observed. The tumor cells were immunopositive for vimentin, neuron-specific enolase, and synaptophysin but not for alpha-inhibin, melan A, or p53. Diffuse and strong immunopositivity with an antimitochondrial antibody proved that this tumor was truly oncocytic. Upon review of previous cases of oncocytic adrenocortical tumors, we reconsidered the diagnostic findings of the potential for malignancy.

Systematic retinotopic reaching error vectors in unilateral optic ataxia.

The main aim of this study is to determine the reference frame of the pointing errors that characterize patients with unilateral optic ataxia (OA). The reaching errors of seven patients with unilateral OA when pointing on a 2D matrix in peripheral vision were investigated in order to better qualify the reference frame of their deficit. Patients were asked to fixate a central target and then to point at one of 24 visual targets presented in their left or right peripheral visual fields, with their left or right hands. The four left and the three right hemisphere lesion patients with OA exhibited an identical pattern of results. In the contralesional visual field, error vectors were systematically directed toward the fixation point, rather than horizontally toward the side of the lesion. OA results from a deficit in transforming targets' eye-centred coordinates into appropriate motor commands.

Differential involvement of regions of rostral prefrontal cortex (Brodmann area 10) in time- and event-based prospective memory.

Rostral prefrontal cortex (approximating Brodmann area 10) has been shown repeatedly to have a role in the maintenance and realization of delayed intentions that are triggered by event cues (i.e., event-based prospective memory). The cerebral organization of the processes associated with the use of time cues (time-based prospective memory) has however received less attention. In two positron emission tomography (PET) studies we therefore examined brain activity associated with time- and event-based prospective memory tasks. In the time-based condition of the first study, young healthy volunteers were asked to make a prospective response based on their self-estimation of the passage of time while engaged in an attention-demanding ongoing activity. In the time-based condition of the second study, participants had a clock available in the ongoing task display and did not need to estimate the time for the prospective response. In the event-based condition of both studies, participants were asked to make a prospective response when prospective cues were presented in ongoing trials. Both studies showed activation differences in rostral prefrontal cortex according to whether the task was time- or event-based. In study one, an area of left superior frontal gyrus was more active in the time-based condition. In study two, three rostral prefrontal regions were more active in the time-based condition: right superior frontal gyrus, anterior medial frontal lobe and anterior cingulate gyrus. A region in left superior frontal gyrus, different from the area found in the first study, was more active in the event-based condition. These results indicate involvement of multiple brain regions of rostral prefrontal cortex in time- and event-based prospective memory. The results are interpreted as reflecting the differing processing demands made by event- or time-based prospective memory tasks, and the differing demands of time-based tasks according to whether a clock is present as an aid.

Neural correlates of context memory with real-world events.

There has been little evidence for the difference in the retrieval processes of when and where something happened, one of the important factors in understanding episodic memory. We used positron emission tomography (PET) to identify the neural networks associated with temporal and spatial context memory of events experienced under experimental conditions similar to those of everyday life. Before PET, subjects experienced 36 events. The events were divided into four groups of nine each. The subjects experienced the first two groups of events before a 15-min recess and the other two after the recess; they experienced the first and last groups of events in one room, took a recess in another room, and experienced the second and third groups in a different room. During PET, the subjects were scanned under three different retrieval tasks: a time-retrieval task, a place-retrieval task, and a simple recognition task. The results showed that the retrieval of time and space, compared with the simple recognition, was associated with activity in substantially different regions as well as a common region: time retrieval with the posterior part of the right orbitofrontal cortex and left inferior parietal lobule, place retrieval with two regions in right parietal association cortex, right posterior cingulate gyrus, left precentral gyrus, and right cerebellum, and both with the right inferior frontal gyrus. These findings indicate that there are unique areas, in addition to a common area, for retrieving temporal and spatial context.