In Japan, sinonasal cancer is estimated to account for about 8% of all cases of head and neck cancer. While sinonasal adenocarcinoma accounts for about 10%–50% of all cases of sinonasal carcinoma in western countries, this percentage is lower in Japan. According to the WHO classification of 2017, sinonasal adenocarcinoma is categorized into intestinal and non-intestinal types. Intestinal-type adenocarcinoma (ITAC) is associated with wood and leather dust exposure, whereas non-intestinal type adenocarcinoma (non-ITAC) is not known to be associated with any environmental factors. Non-ITAC is further divided into low-grade (LG) non-ITAC and high-grade (HG) non-ITAC.

Surgery followed by radiotherapy is considered to represent the gold standard for the management of sinonasal adenocarcinoma. In order to improve the local control and preserve organ function, some centers have explored the addition of chemotherapy to the aforementioned standard treatment strategy. Recently, the usefulness of concurrent chemoradiotherapy for squamous cell carcinoma and malignant melanoma of the sinonasal region was demonstrated; however, little is known yet about the usefulness of this strategy for cases of sinonasal non-ITAC. Herein, we describe the case of a patient with primary, locally advanced sinonasal non-ITAC who was successfully treated by intra-arterial cisplatin (CDDP) with concurrent radiotherapy.

Diseases caused by varicella zoster virus (VZV) infection, such as Ramsay Hunt syndrome and pharyngolaryngeal herpes zoster, are commonly encountered in clinical practice.

They are usually diagnosed by their characteristic physical findings, such as formation of blisters in a localized area on one side of the body, and immediately treated with anti-herpes virus agents. Results of conventional serological tests for VZV antigen and antibodies require several days to obtain results and therefore, usually arrive only after the start of treatment. In recent years, a VZV antigen detection kit has been developed that has made diagnosis possible within a shorter time. The new detection kit is expected to be helpful for differentiating VZV infection from herpes simplex virus infection in difficult cases, and also to confirm the diagnosis in patients with an atypical presentation. We report our experience with the use of this kit in 4 cases of Ramsay Hunt syndrome (including 1 case with pharyngeal herpes zoster and 1 case with pharyngolaryngeal herpes zoster) and 1 case of oral herpes simplex infection at our department.

Of the 4 cases of Ramsay Hunt syndrome, the test results with the kit were positive in 2 cases in which the samples were obtained from the skin of the auricle. In one case, the test specimen was collected from a pharyngeal erosion and the test result with the antigen kit was positive. In another case, a specimen obtained from a pharyngeal erosion tested negative with the antigen kit, but tested positive by the fluorescent antibody (FA) method at a later date.

As previously reported, the detection sensitivity of the kit for samples obtained from erosions and ulcers may be lower than that for samples obtained from blisters and pustules.

Absence of cross-antigenicity with herpes simplex infection was confirmed.

In the case of mucosal lesions, the antigen kit may yield false-negative results in a relatively high percentage of cases. It is considered desirable to collect a sufficient amount of test sample and to use an antigen qualitative test, such as the FA method, in addition, as necessary.

Patient with Ramsay Hunt syndrome (Hunt’s syndrome) caused by varicella-zoster virus (VZV) infection often present with other cranial nerve palsies, besides palsy of cranial nerves VII and VIII. We report a case of Hunt’s syndrome which was complicated by palsy of the VI, IX, and X cranial nerves.

A 77-year-old man was diagnosed as having Hunt’s syndrome, with a herpetic rash, mainly over the right auricle, facial nerve palsy, hearing loss, and dizziness, and was treated with steroids and valacyclovir. The patient developed aspiration pneumonia due to palsy of the vagal and glossopharyngeal nerves during the course of this disease, and also had abducens nerve palsy.

The abducens nerve palsy improved within about 2 months, while the vagus nerve paralysis persisted for a long time, and the patient had recurrent aspiration pneumonia. In addition, the hospitalization was prolonged due to aspiration pneumonia.

We think it is necessary to consider the possibility of multiple cranial nerve palsies in patients with Hunt’s syndrome and to pay attention to aspiration.

Schwannoma is a benign tumor of Schwann cell origin. It arises in the soft tissues, such as the subcutaneous tissue and muscle, in most cases, and is rarely found in the nasal cavity. We report a case of nasal schwannoma in a pediatric patient. A 14-year-old male patient have presented with a history of epistaxis and nasal mucus discharge ever since he was a little child. Physical examination showed a tumor in the right nasal cavity. Computed tomography demonstrated a tumor in the right nasal cavity compressing the nasal septum, with no bone destruction. Biopsy indicated the diagnosis of hemangioma and we decided to remove the entire tumor. While most of the tumor was removed by endoscopic surgery under general anesthesia, small amounts of colorless fluid flowed out in a pulsatile manner after the mucous membrane around the base was incised, and we could not remove the base of the tumor completely, because the possibility that the leaking fluid represented cerebrospinal fluid leakage could not be excluded. The histopathological diagnosis was Antoni A type schwannoma. The postoperative course was uneventful, with the patient showing no signs of recurrence or nervous symptoms until the last follow-up at 18 months after the surgery. Schwannomas do not have characteristic macroscopic or imagining findings, and the possibility of schwannoma should be considered in the differential diagnosis in patients presenting with a nasal tumor.

Introduction: Solitary fibrous tumor (SFT) is a rare neoplastic entity which typically originates from the pleura and other serosal tissues, and infrequently in the head and neck region. Case: A 46-year-old male patient complaining of nasal obstruction was referred to us for a tumor in the left nasal cavity. Contrast-enhanced computed tomography showed a heterogeneously enhancing mass. The histopathological findings of this hemorrhagic tumor on the first biopsy were compatible with a nasal polyp; however, a rebiopsy revealed a spindle cell neoplasm which was immunohistochemically positive for CD34 and negative for S-100 protein, which led to the diagnosis of SFT. The tumor was removed by endoscopic medial maxillectomy (EMM), and histopathology of the resected specimen confirmed the preoperative diagnosis. The patient showed no evidence of recurrence until at least five years after the operation. Conclusion: Diagnosis of SFT is confirmed by immunohistochemical study. EMM may be considered as the treatment of choice for maxillary SFT.

Postcricoid vascular abnormalities are rare causes of chronic dysphagia and dyspnea in infants and children. A postcricoid cushion is a mass that becomes obvious when children cry because of venous flux dilation in the postcricoid lesion. The condition can present as a sucking or respiratory disorder, attributable to laryngeal stenosis caused by venous pressure imparted by the mass. The epidemiology and pathophysiology of a postcricoid cushion are poorly known; many cases are asymptomatic and many are often diagnosed as hemangiomas or vascular malformations. Herein, we report a case of an infant in whom postcricoid cushion was diagnosed by laryngoscopy under sedation, considering the unique pressure-dependent characteristics of the lesion.

A 9-month-old female presented with intermittent stridor associated only with crying. Flexible laryngoscopy revealed a spherical bluish mass in the postcricoid area. Magnetic resonance imaging did not reveal the mass, so that no definitive diagnosis could be made. As we suspected the presence of a pressure-dependent vascular mass (a postcricoid cushion), we performed flexible laryngoscopy with the child under sedation. The lesion was difficult to visualize. We made the diagnosis of postcricoid cushion and followed the patient for 2 years by only serial laryngoscopies. The mass became smaller over time.

Postcricoid cushion in an infant was diagnosed by laryngoscopy under sedation. It is important to be aware of this unique condition when examining the upper aerodigestive tract. No treatment for postcricoid cushion is needed in the majority of cases, as most patients can be safely observed.

The disease termed von Willebrand’s disease (vWD) is an autosomal-dominant hereditary bleeding disorder caused by dysfunction or deficiency of the clotting factor called von Willebrand factor (vWF); vWF is produced by the vascular endothelial cells and bone marrow megakaryocytes, and plays a major role in platelet adhesion and aggregation in the processes of primary hemostasis.

We reported the case of 3-year-old girl with vWD, who presented us with refractory tonsillar hemorrhage. Because of the family history of refractory bleeding, the concentration and activity of vWF in the blood were measured. Accordingly, the child was diagnosed as having vWD as she met the following diagnostic criteria: 1) abnormal bleeding episode; 2) vWF antigen titer or vWF activity less than 30% or equivalent to the mean -2SD. After additional laboratory examination, we diagnosed her as having type2A vWD. Moreover, her mother, grandmother and 2 second cousins were also diagnosed having type2A vWD. Following the diagnosis, the refractory pharyngeal bleeding was controlled by intravenous factor VIII/vWF supplementation.

Because refractory epistaxis and oral mucosal bleeding are common symptoms in patients with vWD, it is important for otolaryngologists to promptly diagnose this disease by type and advise patients on the appropriate course of treatment.

Subglottic cyst is a rare disorder and predominantly occur in premature infants with a history of endotracheal intubation. Formation of these cysts could be induced by blockage of the subglottic ducts resulting from erosion and subsequent scarring of the subglottis following endotracheal intubation.

A male infant, weighing 3240 grams, was admitted to our hospital with respiratory distress at the age of 4 months. He had been born at 23 weeks and 1 day of gestation. He had needed tracheal intubation on the day of birth, and received mechanical ventilation for 39 days. Subsequently, he developed breathing difficulties that worsened gradually until respiratory distress and biphasic stridor became apparent on day 143. Both flexible fiberoptic laryngoscopy and computed tomography revealed cyst-like masses just below the vocal cords, causing airway narrowing. Emergent subtotal cystectomy was planned on the admission day. Under general anesthesia, an endotracheal tube was introduced into the space posterior to the cystic lesions. A Miller laryngoscope and flexible laryngoscope for adults offered an optimal surgical view, the cyst-like masses were resected with wire-guided forceps. Extubation was performed with no trouble, 9 days after the surgery. Histopathological examination revealed that the cyst walls were lined with respiratory epithelial cells. A second surgery was performed 5 months after the first surgery because recurrence of the cysts. No recurrence has been observed for 5 months after the second operation.

Subglottic cysts should be considered in the differential diagnosis in premature infants with a history of endotracheal intubation presenting with symptoms of upper airway obstruction. When the patient is too small to allow insertion of a rigid laryngoscope, a Miller laryngoscope, flexible laryngoscope, and wire-guided forceps are useful alternatives for diagnosis and treatment.

A saccular cyst of the larynx, a lesion originating from obstruction of the orifice between the saccule and the laryngeal ventricles, can cause airway obstruction. Herein, we report a rare case of a saccular cyst extending into the extralaryngeal space. A 19-year-old male patient presented to us with complaints of hoarseness of the voice and sore throat. He gave a history of having received treatment for a laryngeal cyst at the age of 1 year 7 months. Flexible fiberoptic laryngoscopic examination showed a large submucosal mass in the left laryngeal ventricle extending into the subglottis and the false vocal cord. Computed tomography revealed a 3-cm cystic lesion in the left lateral part of the larynx, extending into the extralaryngeal space through the cricothyroid membrane. The lesion was completely removed by an external approach under a vertical midline thyrotomy after tracheostomy. Based on the clinical, imaging and histopathological findings, we made a final diagnosis of a lateral saccular cyst. There has been no obvious recurrence for a year since the surgical resection. A vertical midline thyrotomy approach was effective to remove the cyst with a good prognosis.

An extracranial internal carotid artery aneurysm is rarely found manifesting as a mass in the parapharyngeal space. We report a case of internal carotid artery aneurysm in the parapharyngeal space that was treated by endoscopic-assisted transoral surgery. A 74-year-old woman had undergone coil embolization for a left internal carotid artery aneurysm at another hospital 20 years earlier. She presented with the complaint of pharyngeal discomfort, and was referred to our hospital with suspected internal carotid artery aneurysm. A hard mass was palpable in the left soft palate, and CT and MRI examinations revealed two large internal carotid artery aneurysms in the left parapharyngeal space. Since no blood flow to the aneurysm was observed on angiography, we decided to remove only the interior aneurysm by endoscopic-assisted surgery with a Dingman mouth opener. The aneurysm was successfully removed, with no significant bleeding, as it was not adherent to the surrounding tissues. The patient could resume oral intake from the day after the surgery, and the preoperative symptoms promptly resolved. Most cases of extracranial internal carotid artery aneurysms are treated by endovascular therapy or surgery by the neurosurgeon and cardiovascular surgeon, while there are no reports in the literature, to the best of our knowledge, of cases treated by endoscopic-assisted transoral surgery. Since our case had undergone endovascular treatment and the aneurysm had organized, we could treat her by minimally invasive surgery. Endoscopic-assisted transoral surgery may be a useful treatment modality for selected cases of tumor or internal carotid artery aneurysms in the parapharyngeal space.

Childhood thyroid carcinomas that occur under the age of 15 years are relatively rare, and most are papillary carcinomas. Papillary carcinomas account for more than 90% of all pediatric cases of thyroid tumor, with follicular carcinoma and medullary carcinoma accounting for about 3% and 5% of all cases, respectively.

A 10-year-old girl presented with an anterior cervical mass. Fine needle aspiration cytology (FNAC) showed small clusters of follicular epithelial cells that were poor in colloid content, suggesting a possible diagnosis of thyroid follicular tumor. A left thyroidectomy was performed. Histopathological analysis of the tumor confirmed follicular carcinoma. Until the last follow-up at three months after the surgery, there were no recurrences or metastases.

In a child presenting with the chief complaint of an anterior cervical mass, it is important to keep in mind the possibility of thyroid carcinoma in the differential diagnosis.