Background : A recent increase in the incidence of amyotrophic lateral sclerosis (ALS) has been reported in several countries. A similar trend was suspected for Nagano prefecture in Japan. Thus, we retrospectively examined the incidence of ALS and the characteristics of ALS patients at Shinshu University Hospital. Methods : From hospital clinical records,we identified and enrolled 199 patients with ALS diagnosed based on the El Escorial criteria during the period 1985-2014. The 30-year period was divided into six terms of 5 years each. The incidence of ALS, sex ratio, subtype (limb-or bulbar-onset ALS), and age at onset (＜65 or ≥65 years) were investigated separately for each term. Results : The incidence of ALS significantly increased in the two most recent terms (2005-2009 and 2010-2014) overall, and in both men and women. A significant difference in mean age at onset was observed in the two most recent terms as compared with the first term (1984-1989). The increase in age at onset was particularly notable in the women with bulbar-onset ALS. No significant change was found in the proportion of ALS patients who were residents of the Matsumoto medical service area as compared with those from other areas. Conclusion : We confirmed that the incidence of ALS in Nagano prefecture has been recently increasing. Furthermore, the ratio of patients with older-onset ALS has recently increased because of the chronological increase in age at onset of ALS patients.
A 69-year-old man was referred to our hospital because of occasional nosebleeds over a period of 6 months. Physical examinations were unremarkable except for anemia. Laboratory findings on admission indicated a hemoglobin level of 7.8g/dL. Magnetic resonance imaging revealed a mass occupying the right nasal cavity and infiltrating the bones of the paranasal sinus. Histological findings revealed olfactory neuroblastoma (esthesioneuroblastoma) and the disease was diagnosed to be at a locally inoperable advanced stage. Concurrent chemoradiotherapy (cisplatin plus etoposide and 60 Gy in 30 fractions of 2.0 Gy) was performed. Chemoradiotherapy failed to show significant tumor size reduction, but the nosebleeds stopped. The patient remains disease-free over 3 years after treatment. Although olfactory neuroblastoma is an extremely rare nasal tumor, we should be aware of the disease as a potential cause of nasal bleeding and anemia
A 57-year-old man was admitted to our hospital because of right lower abdominal pain. He was diagnosed as having acute appendicitis, and underwent laparoscopic appendectomy. However, the histopathological diagnosis was goblet cell carcinoid of the appendix. Since the surgical margin and lymph vascular invasion were negative, and metastasis was not observed in lymph nodes, additional surgery and postoperative adjuvant chemotherapy were not performed. There have been no signs of recurrence as of 1 year after the operation.
Goblet cell carcinoid of the appendix is rare, and only 118 cases including our case have been reported in the Japanese literature. Goblet cell carcinoid is known to be almost as malignant as adenocarcinoma, and no therapeutic guidelines or clear notion of the prognosis have been established as yet. Herein, we report a case of goblet cell carcinoid of the appendix removed by a laparoscopic appendectomy together with a review of the literature.
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