Kenjiro Higashi

Glomangiopericytoma of the nasal cavity

Glomangiopericytoma is a rare tumor arising from the pericytes surrounding capillaries, and accounts for less than 0.5% of all sinonasal tumors. A 60-year-old male patient presented with a glomangiopericytoma of the nasal cavity manifesting as nasal obstruction and epistaxis, which was treated successfully with endoscopic excision. Histological examination showed multiplication of spindle-shaped to oval cells which reacted strongly to immunostaining for α-smooth muscle actin. Glomangiopericytoma is categorized as a borderline low malignancy tumor, which tends to recurrence. Strict follow-up is required, especially if complete resection is not achieved.

A case of myoepithelioma of the nasal cavity

Myoepithelioma is a rare tumor, most frequently located in the salivary gland. Case reports of extra-salivary myoepithelioma are sporadic, with only one case in the nasal cavity. A 68-year-old male patient presented with a myoepithelioma of the nasal cavity manifesting as nasal obstruction and epistaxis, which was treated successfully with endoscopic excision. Histological examination revealed myoepithelial cells and myxoid stroma, which confirmed the diagnosis of myoepithelioma. The behavioral pattern is similar to if not identical with that of the mixed tumor, pleomorphic adenoma, which is benign, but the potential for recurrence is always present, especially if complete resection is not achieved.

Central-part laryngectomy is a useful and less invasive surgical procedure for resolution of intractable aspiration

A novel narrow-field laryngectomy procedure known as central-part laryngectomy (CPL) for less invasive laryngeal diversion in patients with intractable aspiration is introduced. We conducted retrospective case reviews of 15 patients who underwent CPL. In this procedure, an area of the glottis including the mid-part of the thyroid cartilage and cricoid cartilage is removed to separate the digestive tract from the air way. The lateral part of the thyroid cartilage, the entire hypopharyngeal mucosa and epiglottis are preserved. The superior laryngeal vessels and nerve are not invaded. All fifteen patients were relieved of aspiration without major complications. In good accordance with cutting of the cricopharyngeal muscles and removal of the cricoid cartilage, postoperative videofluoroscopy demonstrated smooth passages of barium. Ten of 12 patients who had hoped to resume oral food intake became able to do so after CPL and two others also achieved partial oral deglutition. CPL is a useful procedure for treatment of intractable aspiration and offers considerable advantages over other laryngotracheal diversion procedures from the view point of oral food intake.

Clinicopathological features of melanotic neuroectodermal tumor of infancy: Report of two cases.

Melanotic neuroectodermal tumor of infancy (MNTI) is an extremely rare, pigmented neoplastic entity of neural crest origin. Histological and immunohistochemical profiles indicate the presence of two components, small rounded neuroblast-like cellular areas and areas with large melanin-containing cells which consist of combination of neural, melanocytic, and epithelial cell types. Here we present two interesting cases of infants with MNTI which have different clinicopathological features. The first case is a 3-month-old female with rapidly growing MNTI involving the lacrimal sac and inferior wall of the orbital cavity, treated with total maxillectomy without orbital exenteration followed by chemotherapy. The second case is a 7-month-old male with slow-growing maxillary MNTI treated with complete surgical excision. In the female patient, histological findings revealed a predominance of neuroblast-like cellular areas and a high Ki67 index indicating rapid cellular proliferation. In the male patient however, large melanin-containing cells were dominant in this slow-growing tumor. These findings support the presence of two different types of MNTI, rapid-growing and slow-growing types, determined by the component of neuroblast-like cellular areas.

Simple laryngeal suspension procedure by suturing the digastric muscle to the periosteum of the mandible in neck dissection for tongue cancer

PURPOSE In this article, a simple, new laryngeal suspension procedure is described. The effect of hyoid bone suspension by suturing the digastric muscle to the periosteum of the mandible is analyzed. MATERIALS AND METHODS To elucidate the effect of hyoid bone suspension, CT scans of 26 patients who underwent ipsilateral neck dissection with primary resection of tongue cancer were retrospectively reviewed, and the distance between the hyoid bone and the mandible was measured on the operated and unoperated sides of the neck. A total of 14 patients who underwent suturing of the digastric muscle to the mandible (digastric muscle-sutured group) and the 12 patients who did not (control group) were compared. RESULTS In the digastric muscle-sutured group, the average distance between the hyoid bone and the mandible was significantly smaller on the operated side (17.8 ± 0.57 mm) than on the unoperated side (19.8 ± 0.93 mm; p < 0.05). In the control group, there was no significant difference between the operated side (21.0 ± 1.42 mm) and the unoperated side (19.7 ± 1.39 mm). The difference in the distance between the operated and unoperated sides was significantly larger in the digastric muscle-sutured group (1.97 ± 0.79 mm) than in the control group (-1.32 ± 0.61; p < 0.05). CONCLUSIONS It was shown for the first time that suturing of the digastric muscle to the periosteum of the mandible in neck dissection with primary resection of tongue cancer resulted in hyoid bone suspension. This simple procedure can be useful for laryngeal suspension.

Nasal cavity epithelioid hemangioendothelioma invading the anterior skull base

Background: Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor that frequently occurs in soft tissues. Patients suffer from local recurrence and remote metastasis because of its malignant potential. Here, we present a rare case of EHE that originated from nasal cavity and invaded intracranially through the anterior skull base. Case Description: This is a 27-year-old woman who presented a local physician with intermittent epistaxis and a facial pain around her nose. Preoperative studies demonstrated that the tumor invaded into anterior skull base and the dura matter. Therefore, we performed combined skull base and transnasal surgery, which achieved complete resection of the tumor. Postoperative course of the patient was uneventful. No recurrence or distant metastasis was observed in the patient for 2 years following the radical resection. Conclusions: To date, four cases of EHE in the nasal cavity were reported. This is the first case in which EHE demonstrated invasive potentials with intracranial extension. Radical surgical resection plays an important role for better management of invasive paranasal EHE.

A Novel SDHB IVS2-2A>C Mutation Is Responsible for Hereditary Pheochromocytoma/Paraganglioma Syndrome

Pheochromocytomas and paragangliomas are neuroendocrine tumors which arise from adrenal medulla, and sympathetic or parasympathetic nerves, respectively. Hereditary cases afflicted by both or either pheochromocytomas and paragangliomas have been reported: these are called hereditary pheochromocytoma/paraganglioma syndromes (HPPS). Many cases of HPPS are caused by mutations of one of the succinate dehydrogenase (SDH) genes; mainly SDHB and SDHD that encode subunits for the mitochondrial respiratory chain complex II. In this study, we investigated mutations of SDH genes in six HPPS patients from four Japanese pedigrees using peripheral blood lymphocytes (from one patient with pheochromocytoma and five patients with neck paraganglioma) and tumor tissues (from two patients with paraganglioma). Results showed that all of these pedigrees harbor germline mutations in one of the SDH genes. In two pedigrees, a novel IVS2-2A>C mutation in SDHB, at the acceptor-site in intron 2, was found, and the tumor RNA of the patient clearly showed frameshift caused by exon skipping. Each of the remaining two pedigrees harbors a reported missense mutation, R242H in SDHB or G106D in SDHD. Importantly, all these mutations are heterozygous in constitutional DNAs, and two-hit mutations were evident in tumor DNAs. We thus conclude that the newly identified IVS2-2A>C mutation in SDHB is responsible for HPPS. The novel mutation revealed by our study may contribute to improvement of clinical management for patients with HPPS.

Long-Term Outcomes of Patients with Squamous Cell Carcinoma of the Temporal Bone after Concomitant Chemoradiotherapy

Objectives This article aims to clarify the long‐term outcomes of patients with squamous cell carcinoma of the temporal bone who underwent concomitant chemoradiotherapy (CCRT). Design and Setting The study design was a retrospective chart review. Patients and Methods From December 2001 to June 2014, 23 patients with cancer of the temporal bone who were treated by CCRT at the Tohoku University Hospital and the Iwate Medical University Hospital were enrolled in this study. For advanced cancer of the temporal bone, a modified docetaxel, cisplatin, and 5‐fluorouracil (TPF) regimen was used for CCRT. The long‐term outcomes, including prognoses and late complications, were analyzed after CCRT of patients with cancers of the temporal bone. Results The main long‐term complications were stenosis of the external auditory canal and conductive hearing loss. No harmful late complications were observed in these patients. Disease‐specific survival rates were 84.9% for all patients, 100% for patients of stage I, II, and III (n = 10), and 75.5% for patients of stage IV (n = 13) at 5 years. Conclusions Our study showed that CCRT is an effective treatment choice for squamous cell carcinoma of the temporal bone. Furthermore, CCRT using the TPF regimen is a safe and effective initial treatment for patients with advanced cancers of the temporal bone.