A familial case of multiple recurrent cardiac myxomas.

The familial variant of cardiac myxomas is an autosomally dominant disease. Here, we report a case of recurrent multiple cardiac myxomas wherein the patient, a 36-year-old woman who was referred for palpitations and nocturnal dyspnea, had a relevant familial history. Her mother had undergone extirpation of cardiac myxoma when she was about 50 years old. Echocardiography showed the presence of multiple cardiac myxomas. One of the cardiac myxomas nearly obstructed the left ventricular inflow; therefore, we extirpated the myxomas under cardiopulmonary bypass and cardioplegic arrest. The operation was uneventful and the postoperative clinical course was good. Pathological examination confirmed that the extirpated mass was a myxoma. Approximately 3 years after the first cardiac operation, we found a recurrent cardiac myxoma in the same patient. She then underwent a second operation and was discharged without any complications. Pathological examination also confirmed that the cardiac mass was a myxoma and that there was no malignancy. .

[A case of esophageal endocrine cell carcinoma with multiple liver metastases that had a complete response to multidisciplinary therapy].

A 71-year-old man was admitted to our hospital because of dysphagia, and primary endocrine cell carcinoma of the esophagus with multiple liver metastases was diagnosed. After 6 courses of CPT-11+CDDP combination chemotherapy, the liver metastases disappeared, although the esophageal squamous cell carcinoma component remained. Radiation therapy was added to treat the residual esophageal tumor, and a complete response was obtained. This case seems to suggest that multidisciplinary therapy, including chemotherapy, may be effective for treating esophageal endocrine cell carcinoma with other types of organ metastasis.

Extensive portal and mesenteric vein thrombosis in a young man with Klinefelter's syndrome.

Klinefelter's syndrome (KS) is a unique physical condition characterized by tall stature, eunuchoid body proportions, gynecomastia, and azoospermia, in addition to an extra X chromosome. In contrast to the original description, symptoms or physical findings can be extremely varied. KS is the most common chromosomal disorder, with an incidence of 1 in 500 males and is also the most commonly undiagnosed chromosomal disorder. Here, we present the case of a 26-year-old man with KS, who visited our hospital with complaints of abdominal pain and fever. On a routine physical examination, he did not differ from a normal karyotype male. Computed tomography showed extensive portal and mesenteric vein thrombosis (PMVT). It is well known that KS is frequently associated with venous thrombosis, but KS with PMVT has rarely been reported. Approximately one-third of PMVT is idiopathic, but this case suggests the possibility that undiagnosed KS is one of the causes of PMVT, as some individuals with KS are not easily distinguishable from those with the normal karyotype.

[Effectiveness and safety of chemoradiation in a community-based population of patients with esophageal cancer].

BACKGROUND: In Japan, esophagectomy with three-field lymphadenectomy is the standard therapy for resectable esophageal cancer. However, its outcome is considered unsatisfactory because the 5-year survival rate is less than 50%. Chemoradiotherapy (CRT) is the standard therapy for unresectable esophageal cancer and could also be considered as an option for resectable esophageal cancer. We retrospectively determined the efficacy and safety of CRT for patients with esophageal cancer. METHODS: The study population comprised patients with esophageal cancer who had been treated with CRT between April 2004 and October 2009 in our institute. Acute and late toxicity was assessed with NCI-CTC and RTOG/EORTC late radiation morbidity scoring scheme, respectively. Survival time was calculated using Kaplan-Meier methods. RESULTS: We enrolled 29 consecutive patients and classified them on the basis of clinical staging: stage I, 4 patients; stage II/III, 11 patients; and stage IV, 14 patients. Complete response was achieved in 37.9% and 45.5% of the total study population and the stage II/III group, respectively. The median survival time in these groups was 12.1 months and 15 months, respectively. Grade 3/4 acute toxicities were observed in 62.1% of the patients. Grade 3/4 late toxicities were observed in 12% of the patients. The first failure after CRT was almost locoregional. CONCLUSION: CRT appears to be an effective therapy for esophageal cancer; however, its outcome is not satisfactory. Therefore, it is necessary to evaluate the role of salvage surgery after CRT and new chemotherapeutic agents.

[Primary malignant melanoma of the esophagus--detection of circulating tumor cells].

Primary malignant melanoma of esophagus (PMME) is a rare tumor; therefore, the prognostic factors, predictive factors, and difference in biological behaviors of cutaneous melanoma and primary esophageal squamous cell carcinoma remain uncertain. Although we did not adopt a standard therapeutic strategy, we performed surgical resection, chemotherapy, immunotherapy, and radiotherapy either alone or in combination; all procedures resulted in poor outcomes. A 67-year-old woman presented with a swallowing disorder. An esophagogastroduodenoscopy was performed, leading to diagnosis of PMME. According to the Japanese Classification of Esophageal Cancer, the pathological stage was T1b, ly0, v0, N0, M0, stage I . KIT immunostaining was focally positive. After subtotal esophagectomy, adjuvant chemotherapy was performed, but the malignant melanoma relapsed in the mediastinum and the patient died 10 months after diagnosis. We serially monitored the patient using several new modalities, including PET/CT, metabolites of melanin: 5-S-CD, and circulating tumor cells (CTCs) by reverse transcription-polymerase chain reaction to identify the melanoma-specific gene. To our knowledge, this is the first report of a case in which CTCs in PMME were detected.

Effects of intracanal carbon dioxide laser irradiation on cultured human fibroblasts.

OBJECTIVE: The purpose of this study was to evaluate, under various conditions, the damage that might occur to viable cells after CO2 laser irradiation of the root canal. STUDY DESIGN: A laser tip was placed within the root canal of extracted human teeth that were positioned above cultured fibroblasts (NB cells), and then irradiation was applied. The irradiation modes used were either pulse (40 pps) or super-pulse (151 pps). The laser energy was set at 32 J in both modes, and the tip of the laser beam was positioned to a point 2 mm from the fibroblasts. RESULTS: When the pulse mode irradiation was applied, there was significant difference (P < .05) in the percentage of viable cells between teeth with closed apical foramen (1-mm wall between root canal and root apex) and the #15 group (No. 15 K-file used), #30 group (No. 30 K-file), or #50 group (No. 50 K-file). The difference between the #30 group and the #50 group (P < .05) was also significant. The larger the diameter of the apical foramen became, the lower was the percentage of viable cells. CONCLUSION: If a CO2 laser irradiates a root canal system enlarged to within 1 mm of the root canal length, little damage to the periapical tissues would be expected to occur.