[An endoscopic transnasal transsphenoidal biopsy for pituitary gland Langerhans cell histiocytosis in a child: a case report].

Endoscopic transnasal transsphenoidal surgery is gaining wide acceptance as the first-line treatment of pituitary tumors in adults, as this procedure is minimally invasive and associated with good outcomes. On the other hand, conventional transcranial and sublabial approaches are chosen for infants as their small anatomical structures pose some difficulty. This study reports on the case of a 5-year-old boy who had a Langerhans cell histiocytosis appearing in the sella. The biopsy was performed using the endoscopic transnasal transsphenoidal approach, and the patient has not exhibited any post-operative complications. Although the patient was a fairly young child, the endoscopic transnasal transsphenoidal approach resulted in fewer complications as compared to the transcranial and translabial approaches. Otolaryngologists, therefore, should view this approach more positively. The indication for pediatric skull base diseases can be expected to expand to include endoscopic transnasal surgery.

[A case of a large neonatal pharyngeal teratoma].

The congenital teratoma is a rare malformation. We present herein on the case of a large teratoma that arose from the epipharynx in a neonate. The obstructive mass caused maternal polyhydramnios and was identified prenatally by ultrasonography at another hospital. The mother was transferred to our hospital for delivery. We explained about the ex utero intrapartum treatment (EXIT) because of the risk of airway obstruction at birth, but EXIT was refused by the parents. Delivery was accomplished by cesarean section at the 35 th week. Since the newborn had a severe respiratory disorder due to a large intraoral tumor and intratracheal intubation was very difficult, a tracheostomy was done under local anesthesia for 7 min. The tumor was removed successfully on the 9th day and 44th day of age.

[A case report on cervical neuroblastoma with ataxia].

We present a case of pediatric primary cervical neuroblastoma (NB), which is extremely rare. A 3-year-old girl with ataxia but no nasal obstruction, dysphagia, or stridor was diagnosed with cervical NB. Diagnostic results including clinical chemistry, full blood count, and serology, were normal. Ataxia worsened within a few days after onset and was not cured by steroids or intravenous immunoglobulin, delaying a definite diagnosis until a tumor was detected. Opsoclonus-myoclonus syndrome is typified by opsoclonus with myoclonus and ataxia, primarily associated with neuroblastoma. Brain stem cell and cerebellum dysfunction is thought to be due to an autoimmune mechanism. Following chemotherapy and selective neck dissection, the girl has had no recurrence or adverse sequelae. Ataxia disappeared during chemotherapy. We suggest that neuroblastoma should be considered in any child with unexpected by prolonged ataxia.

[A successful case of advanced nasal adenocarcinoma treated with tumor dormancy therapy with S -1 alone].

We report a 65-year-old female case of low-grade non-intestinal type adenocarcinoma of the nasal cavity with bilateral metastases to the cervical lymph nodes(T3N2cM0: Stage IV A). Chemoradiotherapy administered as first-line therapy yielded only a partial response in the primary tumor and metastatic lesions, and subsequent chemotherapy with S-1(at a dose of 80 mg per day)alone was applied as tumor dormancy therapy(TDT)on an outpatient basis. Adverse events during S-1 medication were limited to a decrease in leukocyte count and hemoglobin level, both of which were grade 1. The tumor has not enlarged, and the patient has survived 29 months since the beginning of chemotherapy with S-1 alone without any decrease in quality of life. Although the effectiveness of S-1 for adenocarcinoma of the head and neck has not been fully demonstrated, S-1 might be useful in patients with advanced head and neck adenocarcinoma for the purpose of TDT.

Spindle cell carcinoma as a locally recurrent malignancy after surgery for early lingual squamous cell carcinoma.

Spindle cell carcinoma (SpCC) of the tongue is a relatively rare malignancy. We treated a patient with metachronous early squamous cell carcinoma (SCC) in both margins of the tongue (right side first followed by left side). Eight years after treatment for the first SCC by surgery and radiotherapy, the second SCC occurred in the contralateral margin of the tongue. Surgical resection was performed for the second SCC, with local recurrence 8 months later associated with pathological change from SCC to SpCC. The SpCC was completely resected with wide surgical margins in combination with bilateral neck dissection. However, the patient developed pulmonary metastasis and died of respiratory failure 4 months after the final surgery. Postoperative inflammatory reaction followed by scar formation in the previously irradiated surgical site might have caused the transition from SCC to SpCC during the process of recurrence.