Functional vagal paraganglioma developing 15 years after resection of a retroperitoneal paraganglioma.

The patient, a 40-year-old woman, was diagnosed as having a functional right vagal paraganglioma (PGL) 15 years after undergoing resection for a retroperitoneal PGL. 123I-MIBG scintigraphy showed no accumulation, but as the blood noradrenaline and urinary normetanephrine concentrations were elevated, the tumor was judged as being functional, and surgery was scheduled. The patient was started on doxazosin infusion and embolization of the tumor feeding vessel was performed before the surgery. Intraoperative examination showed that the tumor was contiguous with the vagal nerve, necessitating combined resection of the vagal nerve with the tumor. Postoperatively, the catecholamine levels returned to normal range. Histopathologically, the tumor was diagnosed as a moderately differentiated, intermediate-malignant-grade PGL, with a GAPP score of 4 to 6. No non-chromaffin tissue was observed in the tumor background, so that the functional vagal PGL was considered as a sporadic metachronous tumor rather than as a metastasis from the retroperitoneal PGL. More than half of head and neck paragangliomas (HNPGLs) are reported to arise in the carotid body, and about 5% from the vagal nerve. In addition, HNPGLs rarely produce catecholamines. Herein, we consider the relationship with the previously resected retroperitoneal PGL based on a review of the literature.

[18F]FDG PET/CT Findings of Mass-Forming Actinomycosis in an Uncontrolled Diabetic Patient.

We report a case of cervicofacial actinomycosis mimicking malignant sarcoma in a 78-year-old man with diabetes. High lesion uptake and decreased cerebral uptake on [18F]FDG PET/CT provide a potentially important diagnostic clue suggesting infectious disease in a poorly controlled diabetic patient.

Comprehensive Genomic Profiling Reveals Clinical Associations in Response to Immune Therapy in Head and Neck Cancer.

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.

Superior mediastinal paraganglioma initially suspected of being a mediastinal thyroid goiter.

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.

Evaluation of synchronous multiple primary superficial laryngo-pharyngeal cancers that were treated by endoscopic laryngo-pharyngeal surgery.

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.

Pigmented villonodular synovitis occurring in the temporomandibular joint.

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.

Nystagmus at the Onset of Vertiginous Attack in Ménière's Disease.

OBJECTIVE: To clarify the direction and characteristics of nystagmus at the onset of a vertiginous attack in Ménière's disease. PATIENTS: Two patients with Ménière's disease, whose nystagmus at the onset of a vertiginous attack was recorded using electronystagmography. INTERVENTIONS: Diagnostic. MAIN OUTCOME MEASURES: Electronystagmographic recordings of nystagmus. RESULTS: 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. CONCLUSIONS: 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.

[Rate and clinical characteristics of mumps reinfection].

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.

[Case of acute pharyngitis caused by Corynebacterium ulcerans in Ibaraki Prefecture].

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.