Development of the external ear and middle ear as the organs of hearing is a complex series of events involving many tissues of different embryonic origins. The pharyngeal arches give rise to six auricular hillocks that form the major anatomic structures of the external ear. It is well known that the first branchial arch is the origin of the head of the malleus, body of the incus, and the mandible, and the derivatives of the second arch include the stapes bone, the long process of the incus, and the manubrium of the malleus. There is a spectrum of development, and the more hypoplastic the mandible, the more hypoplastic the middle ear, because of the earlier arrest in development. While dysfunction of the chorda tympani nerve was not correlated significantly with the anatomic structures of middle ear anomalies, presence of facial nerve paralysis was significantly correlated with the development of the middle ear structures. In congenital microtia, surgical correction for hearing improvement is sometimes difficult and challenging. Therefore, not only surgery, but also hearing acquisition with the aid of a device should be considered. Recently introduced devices have been shown to improve the hearing outcomes and quality of life in patients with microtia who might not otherwise benefit from traditional hearing aids. Thorough knowledge of the embryology and anatomy of the ear is essential for establishing accurate diagnosis and providing appropriate treatment.

The subject was a 63-year-old female patient who visited the neurosurgery outpatient clinic with the chief complaint of speech impairment. As she also had a fever and earache, she was referred to the otorhinolaryngology clinic. Examination at that department revealed several cotton-like foreign bodies from the left external auditory meatus to the tympanic cavity, and when they were removed, the patient exhibited malodorous otorrhea. CT of the temporal bone showed soft-tissue densities from the left external auditory meatus to the middle ear and a defect in the tegmen of the temporal bone from the left tympanic cavity to the mastoid antrum. Head MRI showed high-intensity signals in the mastoid air cells of the left middle ear to the temporal lobe and in the ventricles, and signal abnormalities with low values of the apparent diffusion coefficient (ADC) on diffusion-weighted imaging (DWI). On contrast-enhanced MR imaging, mass shadow containing a region with a low signal intensity in the central area with peripheral enhancement was observed in the left temporal lobe. The patient was diagnosed as having otitis media and cerebral abscess caused by foreign bodies in the external auditory meatus. Drainage of the brain abscess was performed and antibiotic therapy was initiated. The speech impairment gradually improved. Several bone defects in the tegmen of the left temporal bone were observed during the left tympanoplasty. No exacerbation has been observed during the three years since the surgery.

A solitary fibrous tumor is a rare neoplasm that typically arises from the pleura, but recently, there have been reports of the tumor arising from other areas as well. A 33-year-old female patient presented to us with the chief complaint of nasal obstruction and was found to have a nasal tumor. MRI showed a low-intensity mass on T1-weighted images and a high-intensity mass on T2-weighted images. The tumor cells were immunohistochemically positive for STAT6 and CD34; especially, STAT6 has a high specificity for SFT and a positive result for this maker was useful for preoperative diagnosis. We considered resection of the tumor by the endoscopic transnasal approach under general anesthesia and evaluated the blood flow in the tumor by CT angiography. After the operation, the patient has shown no evidence of recurrence until now, 24 months since the surgery.

While in most cases, viral parotitis is caused by mumps virus, other viruses have also been known to cause the disease. Over 100 viruses have been documented as causing viral parotitis, including parainfluenza, coxsackie and Epstein-Barr virus. Dozens of cases of acute parotitis with laryngeal edema have been reported domestically.

We report a case of parotitis and submandibular inflammation associated with significant laryngeal edema that was caused by a non-mumps virus.

The patient was a 34-year-old female who presented with a 3-day history of bilateral swelling of the parotid and submandibular regions. The swelling had become steadily worse and she complained of difficulty in swallowing. Physical examination revealed bilateral swelling of the parotid and submandibular regions. Laryngeal endoscopic examination showed significant edema of the epiglottis, valleculae and arytenoid cartilages of both sides. The patient was begun on intravenous steroid treatment, and the laryngeal edema immediately improved. Mumps was ruled out as the cause by the negative serum test for mumps virus antibodies, as also other viruses such as parainfluenza, coxsackie and EB viruses, by the negative serological tests for the respective viruses. We suspected some other virus as the cause.

Some cases of parotitis with laryngeal edema have been reported previously in Japan. It is presumed that parotitis with laryngeal edema is caused by circulatory disturbance because of bilateral swelling of the submandibular glands. Some reported cases of parotitis with laryngeal edema required tracheostomy.

In order to avoid unexpected or critical airway obstruction, laryngeal endoscopic examination and careful observation are important and essential.

Kimura disease is characterized by painless subcutaneous masses, associated with an elevated blood eosinophil count and elevated serum IgE levels. These masses are often found in the head and neck region, especially in the parotid glands and submandibular glands. We report the rare case of Kimura disease arising from the palatine tonsil.

The patient was a 23-year-old male, who presented with an abnormal sensation in his left tonsil. Blood examination revealed an elevated peripheral blood eosinophil count and an elevated serum IgE level. We decided to perform complete resection of his left tonsil, because the tumor was localized in the left tonsil. Histopathological examination revealed that the surgical margin was negative. Until now, 14 months since the surgery, the patient has shown no evidence of recurrence.

Treatment of Kimura disease is not established, but we suggested that total resection of Kimura disease is one of the effective treatments in local tumor such as this case.

Total thyroidectomy and radioiodine ablation are the standard treatment modalities for differentiated thyroid carcinomas (DTC). Disease recurrence after these treatments can easily be detected by monitoring the serum thyroglobulin (Tg) concentrations/anti-thyroglobulin antibody (TgAb) titers and/or by radioiodine whole body scan (WBS). The aim of this study is to estimate the usefulness of FDG/PET-CT (PET) in patients with DTC after first-line treatment. We classified 265 patients with DTC treated during the last 21 years according to the serum Tg/TgAb levels, results of WBS, and presence/absence of a poorly differentiated component in the tumor, and examined the rate of performance and usefulness of PET in each group.

A total of 134 patients who were Tg-negative, TgAb-negative and WBS-negative were classified into the Biochemical Complete Remission (BCR) group, 23 patients who were Tg-negative, WBS-negative and TgAb-positive were classified into the BCR+Ab group, 66 patients who were Tg-positive and WBS-negative were classified into the Biochemical Persistent Disease (BPD) group, 16 patients in whom the tumor contained poorly differentiated components were classified into poorly differentiated group, and 15 patients with known metastatic lesions were classified into the M1 group.

The rates of performance of PET were 3%, 13%, 21%, 50% and 27% in the BCR, BCR+Ab, BPD, poorly differentiated and M1 groups, respectively. The rates of detection of recurrence in the five groups were 0%, 33%, 57%, 38% and 50%, respectively.

In the BPD group, the Tg values were significantly higher in the patients who were determined as being recurrence-positive by PET (median Tg value is 19.4 ng/ml) than in those who were determined as being recurrence-negative by PET (median Tg value is 1.35 ng/ml).

However, there were no significant differences in the Tg values between cases in the poorly differentiated group who were determined by PET as being recurrence-positive (median 0.4 ng/ml) or recurrence-negative (median 0 ng/ml).

We concluded that PET is useful for detecting recurrence in patients of the BPD, poorly differentiated, M1 and BCR+Ab groups with elevated serum TgAb titers.

The incidence of thyroid cancer has been increasing in Japan. The major histological type of thyroid cancer is papillary thyroid carcinoma (PTC) which is derived from follicular cells, whereas medullary thyroid carcinoma (MTC) is rarer and derived from the para-follicular C-cells. Concurrent occurrence of these two different types of thyroid cancer is extremely rare and a precise diagnosis is difficult in such cases. Herein, we report a rare patient with co-occurrence of PTC and MTC.

A 55-year-old Japanese woman with no significant family medical history was referred to our hospital with a thyroid nodule that had been detected incidentally. She had a history of hypertension and type 2 diabetes as comorbidities. Two nodules could be detected by CT; one nodule was diagnosed as an adenomatous goiter and the other as a PTC by fine needle aspiration cytology. No other tumor could be detected before treatment. We performed total thyroidectomy, because the PTC was located in the thyroid isthmus. In the final pathological diagnosis, the two tumors were confirmed as adenomatous goiter and PTC. In addition, small MTC tumors were also detected in both the right and left lobes of the thyroid gland, that had not been detected before the surgery. There was no evidence of either pheochromocytoma or hyperparathyroidism, and the patient refused to provide consent for RET gene mutation analysis. Therefore the MTC was diagnosed as being sporadic.

In this case, preoperative diagnosis of the MTC tumors by imaging could not be made, because the tumors were too small to detect by imaging. Measurement of the serum calcitonin and serum carcinoembryonic antigen levels may have been useful, however, no guidelines recommend measurement of these parameters for patients diagnosed as having PTC. To avoid inadequate treatment because of missed diagnosis of MTC, as in this case, a more effective diagnostic approach is expected.

Head and neck malignant lymphoma accounts for less than 10% of case of head and neck malignancies. Because patients with head and neck malignant lymphoma often present with neck swellings, they are often examined by otolaryngologists. Fine-needle aspiration cytology (FNAC) may yield useful clue to the ­diagnosis, although tissue biopsy is required for reliable diagnosis and classification of lymphoma, so that appropriate treatment can be administered. Although open biopsy of lymph nodes has been regarded as the standard method of tissue sampling, this method has drawbacks, including the risks associated with general anesthesia and surgical invasion, as well as the high cost and relatively long time to diagnosis. Core needle biopsy (CNB) provides a possible alternative to traditional FNAC and open biopsy. In this study, we assessed the usefulness of CNB in determining the histology of head and neck malignant lymphomas.

Seven patients with suspected head and neck malignant lymphoma were examined by CNB between June 2017 and November 2018. Indications for CNB included a lesion diameter of ≥1.5 cm, lesion location at a distance from important blood vessels as judged by prior imaging examination, and the absence of bleeding tendency. US-guided CNB was performed using a Bard^® Monopty^® 14 G×90 mm, with a stroke length of 11 mm, or a Temno Evolution^® 14 G×60 mm, with a stroke length of 10/20 mm.

Of the seven lesions, four were in the neck, one was in the thyroid, and one was in the parotid gland. The mean lesion size was 4.3 cm (range, 2.5–6.8 cm). In all patients, the US-guided CNB was performed under local anesthesia. Histopathologically, four patients had diffuse large cell lymphoma, one had follicular lymphoma, one had MALT lymphoma, and one had ALK-negative anaplastic large cell lymphoma. The ­histopathological diagnosis could be made in all the patients by examination of the US-guided CNB, with none of the patients requiring further open biopsy. The shortest time from the first visit to biopsy was 0 day, with the CNB samples taken at the first outpatient consultation. None of the patients developed compli­cations such as hemorrhage, hematoma, infection, or facial nerve palsy.

Lesions suspected as being malignant lymphoma not requiring surgical excision may be diagnosed by US-guided CNB, reducing the burden of invasion on the patients.

Cases of foreign body ingestion in children are often encountered by otorhinolaryngologists in clinical practice. We report the case of foreign body in the left hypopharynx in an 19-month-old boy. The foreign body was a food pick, which is often used in “character lunch boxes.” He was suspected as having accidentally ingested the food pick and was urgently transported to our hospital. It was difficult to identify the foreign body on a plain X-ray or CT. Nasopharyngeal endoscopy revealed a white foreign body lodged in the hypopharynx. It was removed by direct laryngoscopy under general anesthesia. For 3 days post-surgery, mild edema was noted in the arytenoid region, which necessitated administration of a steroid by intravenous drip infusion and of epinephrine by inhalation. Parental education is important to prevent accidental ingestion of foreign bodies by children. Special attention should be paid to preventing toddlers and infants from accidentally ingesting items such as food picks that are commonly and repetitively taken to the mouth.

Background

Nivolumab, approved in Japan for platinum-refractory recurrent/metastatic squamous cell carcinoma of the head and neck (RM-SCCHN) in 2017, is of uncertain cost-effectiveness.

Patients and Methods

We reviewed the data of 16 patients with platinum-refractory RM-SCCHN treated with nivolumab monotherapy, 3 mg/kg every 2 weeks, between April 2017 and February 2018.

Results

All 16 patients were male. The number of previous treatments was 1, 2, and 3 in 1, 5 and 10 patients, respectively. All patients had been previously treated with regimens that included platinum, and 15 patients had previously received cetuximab. The best response rate was a partial response in two patients. Stable disease occurred in 11 patients and disease progression occurred in 2 patients. The disease control rate was 81.2%. The median follow-up time was approximately 8.7 months, and the median progression-free survival (PFS) was 2.1 months. Adverse events (AE) ≤Grade 3 included pneumonitis and rash in 38%, pruritus in 31%, fatigue and kidney dysfunction in 25% and endocrine disorder in 12% each. AEs >Grade 3 included pruritus in 12%, and pneumonitis in 6%. Drug discontinuation was requested by patients’ clinicians for disease progression in seven patients and Grade 3 AEs in three. Following nivolumab treatment, seven patients received salvage treatment.

Conclusion

Nivolumab showed some efficacy in disease control, but the PFS was low. The AE rate was acceptable, with no Grade 4 or 5 AEs. If patient selection can be fine-tuned, treatment with this agent may become cost-effective.