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Paraganglioma is a rare tumor arising from paraganglia. Few reports have described paragangliomas in the superior mediastinum. We report a case of superior mediastinal paraganglioma treated in our department. A 28-year-old woman visited our department because of suspected mediastinal tumor during a medical checkup. Contrast-enhanced CT showed a 39 × 35 × 65-mm tumor with a well-defined border extending from the lower pole of the left thyroid gland to the superior mediastinum. Laboratory tests showed no evidence of catecholamine overproduction. Mediastinal thyroid goiter was the most suspected preoperative diagnosis. We decided to perform a transcervical excision for both diagnosis and treatment. The tumor was easily detached from the lower pole of the left thyroid gland and was not continuous. The tumor capsule was brittle and bled easily. The operating time was 3 h and 11 min, and the amount of bleeding was 571 mL. The pathological diagnosis was paraganglioma. Paragangliomas are characterized by abundant blood flow and are likely to result in a high intraoperative bleeding volume. In addition, if the tumor is functional, circulatory abnormalities can occur during the perioperative period. Accurate preoperative diagnoses are important, and the possibility that paragangliomas can develop in the superior mediastinum should be considered.

Objective ndoscopic laryngopharyngeal surgery (ELPS) is a useful surgery for superficial cancers of the head and neck region, but it has not yet been well evaluated for synchronous multiple primary cancers (multiple primaries). The purpose of this study was to clarify the safety and usefulness of ELPS for patients with multiple superficial primary cancers in the head and neck region. Methods rom December 2009 to December 2016, 145patients with superficial head and neck cancers underwent ELPS. The patients were divided into two groups; a group consisting of patients with a single primary cancer (single primary) and another group consisting of patients with synchronous multiple primaries, and the incidences of postoperative complications and lymph node metastasis were retrospectively compared between the two groups. Results f the 145 patients, 107 had a single primary cancer and 38 had multiple primaries. There was no significant difference in the age, sex, or rate of intraepithelial cancer between the two groups. Postoperative complications included dysphagia in 6 (5.6%) patients with a single primary and 2 (5.3%) patients with multiple primaries. One patient with multiple primaries required gastrostomy because of aspiration pneumonia. In addition, the following complications were also observed. Laryngeal paralysis occurred in 2 (1.9%) patients with a single primary, and 1 (2.6%) patient with multiple primaries; tracheostomy because of postoperative bleeding in 1 (0.9%) patient with a single primary; infection occurred in 2 (5.3%) patients with multiple primaries. Postoperative lymph node metastasis was found in 7 (6.5%) patients with a single primary and 6 (15.8%) patients with multiple primaries. Lymphatic invasion of the primary cancer was noted in 3 (2.8%) patients with a single primary and 5 (13.2%) patients with multiple primaries, being significantly higher in the latter group. Conclusion ELPS is also a safe surgery for patients with multiple primaries. However, the incidence of lymphatic invasion of the primary cancer was significantly higher in patients with multiple primaries.

Comprehensive genomic profiling (CGP) provides information regarding cancer-related genetic aberrations. However, its clinical utility in recurrent/metastatic head and neck cancer (R/M HNC) remains unknown. Additionally, predictive biomarkers for immune checkpoint inhibitors (ICIs) should be fully elucidated because of their low response rate. Here, we analyzed the clinical utility of CGP and identified predictive biomarkers that respond to ICIs in R/M HNC. We evaluated over 1100 cases of HNC using the nationwide genetic clinical database established by the Center for Cancer Genomics and Advanced Therapeutics (C-CAT) and 54 cases in an institution-based study. The C-CAT database revealed that 23% of the cases were candidates for clinical trials, and 5% received biomarker-matched therapy, including NTRK fusion. Our institution-based study showed that 9% of SCC cases and 25% of salivary gland cancer cases received targeted agents. In SCC cases, the tumor mutational burden (TMB) high (≥10 Mut/Mb) group showed long-term survival (>2 years) in response to ICI therapy, whereas the PD-L1 combined positive score showed no significant difference in progression-free survival. In multivariate analysis, CCND1 amplification was associated with a lower response to ICIs. Our results indicate that CGP may be useful in identifying prognostic biomarkers for immunotherapy in patients with HNC.

Background: Biomarkers predicting therapeutic response to immunotherapy have been widely explored via monitoring the liquid and tissue-derived components. Increasing treatment options for recurrent/metastatic head and neck squamous cell carcinoma (R/M HNSCC) mandates prediction of the therapeutic response of anti-PD-1 antibody alone as well as optimization of the treatment sequence. In view of improving biomarkers predicting the efficacy of immunotherapy for R/M HNSCC, we hypothesized that biomarkers can be personalized depending on clinicopathological backgrounds and treatment sequence. Methods: In this study, we retrospectively included formalin-fixed paraffin-embedded (FFPE) samples, peripheral blood cell counts at treatment, clinicopathological information, and outcome data for patients with R/M HNSCC receiving nivolumab across 22 institutions in Japan (N = 100). FFPE samples were subjected to 14-marker multiplex immunohistochemistry (IHC) and image cytometry analysis (Tsujikawa T et al. Cell Reports, 2017) to quantitatively evaluate CD8+ T cells, helper T cells, regulatory T cells, B cells, natural killer (NK) cells, macrophages, dendritic cells, CD66b+ granulocytes, mast cells, programmed death ligand 1 (PD-L1) and PD-1 expression in a single slide. Intratumoral and circulating immune cell frequencies were comparatively analyzed between responders (CR, n = 14; PR, n = 39) and non-responders (SD, n = 2; PD, n = 45). Results: Of 100 patients included, responders had significantly lower smoking and alcohol index, higher incidence of immune related adverse events, and higher PD-L1 expression in immune cells as well as PD-L1 combined positive score (CPS) than non-responders. Next, focusing on the history of prior therapy, stratified analysis revealed that the frequency of NK cells was associated with nivolumab response in patients with prior cetuximab use, but not in cetuximab-naïve status. Furthermore, stratified analysis by patient age revealed that nivolumab response was significantly associated with high CPS and lymphoid-inflamed profiles based on cell densities of nine immune cell lineages in the group aged 65 years or older, but not in the group under 65 years of age. On the contrary, the neutrophil/lymphocyte ratios (NLR) in peripheral blood counts at nivolumab treatment were significantly lower in responders (mean 4.96) than those in non-responders (mean 10.46) in the group under 65 years of age, but not in those over 65 years of age (7.41 versus 8.47). Conclusions: Using peripheral blood data and tumor tissue profiling stratified by patient age and prior treatment might provide better predictive biomarkers in nivolumab response to HNSCC. Further preclinical and clinical studies elucidating immune mechanisms in different patient backgrounds will be warranted. Citation Format: Takahiro Tsujikawa, Kazuchika Ohno, Sumiyo Saburi, Junichi Mitsuda, Kanako Yoshimura, Alisa Kimura, Hiroki Morimoto, Gaku Ohmura, Akihito Arai, Hiroshi Ogi, Saya Shibata, Yosuke Ariizumi, Akihisa Tasaki, Ryosuke Takahashi, Yumiko Tateishi, Hiroaki Kawabe, Sadakatsu Ikeda, Kei-ichi Morita, Tatsuhiko Tsunoda, Takumi Akashi, Morito Kurata, Issei Imoto, Yasushi Shimizu, Akihito Watanabe, Yukinori Asada, Ryuichi Hayashi, Yuki Saito, Hiroyuki Ozawa, Kiyoaki Tsukahara, Nobuhiko Oridate, Arata Horii, Takashi Maruo, Nobuhiro Hanai, Hidenori Inohara, Hiroshi Iwai, Takashi Fujii, Ken-ichi Nibu, Shigemichi Iwae, Tsutomu Ueda, Ryuji Yasumatsu, Hirohito Umeno, Muneyuki Masuda, Kyoko Itoh, Shigeru Hirano, Takahiro Asakage. Tumor immune characterization identifies age-stratified biomarkers for nivolumab in patients with head and neck squamous cell carcinoma: A nationwide collaborative study in Japan [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2022; 2022 Apr 8-13. Philadelphia (PA): AACR; Cancer Res 2022;82(12_Suppl):Abstract nr 5210.

To clarify the direction and characteristics of nystagmus at the onset of a vertiginous attack in Ménière's disease.Two patients with Ménière's disease, whose nystagmus at the onset of a vertiginous attack was recorded using electronystagmography.Diagnostic.Electronystagmographic recordings of nystagmus.In both patients, nystagmus was directed toward the affected side over the entire course of the vertiginous attack. One patient experienced a severe sensation of vertigo and exhibited strong nystagmus from the onset of the attack. The other patient reported a mild sensation of vertigo, which was accompanied by intermittent nystagmus.Vertiginous attacks in Ménière's disease are accompanied by irritative nystagmus. The intensity and characteristics (e.g., continuous or intermittent expression) of the nystagmus may be associated with pathophysiological severity.

Objective Pigmented villonodular synovitis occurring in the region of the temporomandibular joint is a rare disease, requiring a review of the treatment method, follow-up period. Method Refer to the past literature, along with a retrospective search. Results An excision, including the skull base bone, was performed in all cases; however, recurrence was found in one case on which fractional excision was performed. Past reports have also indicated that en bloc resection was considered desirable. Conclusion It is necessary to perform en bloc resection on patients with pigmented villonodular synovitis occurring in the region of the temporomandibular joint. Furthermore, due to reported cases of recurrence after a long period of time, follow-up observations of about 10 years are considered necessary.

Mumps infection is anecdotally believed to occur only once over a lifetime. However, in recent years, it has gradually come to be recognized among pediatricians that mumps reinfection is not a rare condition, and some criteria for the mumps reinfection have been proposed. One of the widely accepted criteria is levels higher than 25.8 IU/dl of serum IgG antibodies against the mumps virus and lower than 2.0 IU/dl of serum IgM antibodies. From July 2010 to June 2011, 45 patients with acute swelling of the major salivary gland(s) were enrolled into our survey of mumps reinfection in Tsuchiura Kyodo General hospital. Serum IgG and IgM antibodies against the mumps virus were measured at the initial visit. Ten cases were diagnosed as having primary infection with the mumps virus, while the other 10 cases were diagnosed as having reinfection with the mumps virus according to the criteria. The present study suggests that mumps reinfection is a common condition in patients with acute swelling of the major salivary glands in adulthood.

We report on the case of a 51-year-old woman who presented with refractory pharyngitis caused by toxigenic Corynebacteriumn ulcerans (C. ulcerans). Thick pseudomembrane formations and yellowish pus were observed in her nasopharynx. Based on her clinical course and history of breeding cats, we considered C. ulcerans infection as the possible diagnosis. She was treated with macrolide administration and her symptoms immediately improved. C. ulcerans was identified in pus from the patient's pharynx as well as in discharge material from her cat's eyes, and C. ulcerans was thought to have caused her pharyngitis. C. ulcerans is one of the infecting bacteria which can cause a zoonotic infection. In Japan, some cases with C. ulcerans infection from cats have been reported. It is important that we should consider C. ulcerans infection as a differential diagnosis of refractory pharyngitis.