Current Status of Uterine Leiomyosarcoma in the Tohoku Region: Results of the Tohoku Translational Center Development Network Survey.

BACKGROUND: To prepare for a future clinical trial for improving the long-term prognosis of patients with uterine leiomyosarcoma (ULMS), we conducted a multi-institutional survey in the Tohoku region of Japan. METHODS: We conducted a retrospective cohort study between 2011 and 2014 in member institutions of the Tohoku Translational Research Center Development Network. RESULTS: A total of 53 patients with ULMS were registered in 31 institutions for the present survey. The median patient age was 56 years, 67.9% of the patients were postmenopausal, 88.7% had a performance status of 0 or 1, and only 6 patients (11.3%) showed preoperative evidence of malignancy. Although retroperitoneal lymphadenectomy was performed in only 26.4% of patients, 64.2% patients were identified as having FIGO stage 1 disease; 73.6% were eligible to undergo complete surgery. Among 36 patients who were treated with postoperative chemotherapy, 28 (77.8%) received docetaxel and gemcitabine combination therapy. The most frequent recurrence site was the lungs, and the median progression-free survival of all enrolled patients was 11.7 months. However, the median progression-free survival and the median overall survival in patients with stages III and IV disease were 3.4 and 11.4 months, respectively. CONCLUSION: Although ULMS was associated with a high rate of complete or optimal surgery, the long-term prognosis was poor. Effective postoperative therapy should be developed to improve the long-term prognosis of patients with ULMS.

Pretreatment plasma fibrinogen level correlates with tumor progression and metastasis in patients with squamous cell carcinoma of the esophagus.

BACKGROUND: Hypercoagulation has been reported to be associated with tumor progression and a poor prognosis in various carcinomas. In this study, we examined fibrinogen levels in pretreated patients with esophageal squamous cell carcinoma (ESCC) and assessed its correlation with clinicopathological factors and prognosis in patients with ESCC. METHODS: Pretreatment fibrinogen levels were examined prior to surgery or other treatments (e.g. endoscopic mucosal resection and chemoradiotherapy [CRT]) in 105 patients with primary ESCC. We investigated the association of fibrinogen levels with clinicopathological background factors and the survival of ESCC patients. RESULTS: The plasma fibrinogen concentration (PFC) ranged from 209.4 to 781.6 mg/dL. Pretreatment PFC correlated significantly with the depth of invasion (T factor). There also existed a significant correlation between higher fibrinogen levels and lymph node metastasis (N factor) and distant organ metastasis. Patients with a higher fibrinogen level experienced a significantly worse overall survival (P = 0.006). Fibrinogen levels strongly correlated with platelet counts, white blood cell counts and tumor length. Pretreatment PFC were observed to have a significant correlation with CRT responsiveness in ESCC patients in stages II and III (P = 0.005). CONCLUSION: This study revealed that higher levels of fibrinogen correlated with tumor progression, metastasis and poor responsiveness to CRT in ESCC patients.

[A surgically treated case of ileus caused by small intestinal tuberculosis during treatment for pulmonary tuberculosis].

A 44-year-old man consulted medical clinic, complaining of cough and sputum. Then he was admitted to our hospital, because of positive acid-fast bacilli in his sputum and positive PCR (polymerase chain reaction) for Mycobacterium tuberculosis. Combined use of isoniazid (INH), rifampicin (RFP), ethambutol (EB) and pyrazinamide (PZA) was started. But 4 days after starting treatment, we had to suspend tuberculosis chemotherapy because of hepatopathy. Since then he started to complain epigastralgia and vomiting. Plain abdominal X-ray and abdominal computed tomography (CT) led to a diagnosis of ileus. Inspite of insertion of ileus tube symptoms of ileus did not improve. Small bowl series showed severe stenosis at ileum end, necessitating jejunectomy. Macroscopic study revealed a ring ulcer and multiple epithelioid cell granuloma with Langhans' giant cells was detected histopathologically. PCR for M. tuberculosis of extracts from ileum was positive. Therefore the patient was diagnosed small intestinal tuberculosis. Treatment was continued by the combination of INH, RFP, EB, and the symptoms markedly improved. There have been no sign of recurrence since the end of the 6-month treatment for tuberculosis.

Acute exacerbation of interstitial pneumonia following surgical lung biopsy.

STUDY OBJECTIVES: Surgical lung biopsy (SLB) plays an important role in the diagnosis of interstitial pneumonia, however, the occurrence of acute respiratory failure following SLB remains largely unreported. We evaluated the incidence, clinical features, therapy and prognosis of acute exacerbation of interstitial pneumonia following SLB. DESIGN: Retrospective study of consecutive patients who underwent SLB to establish a diagnosis of diffuse lung disease between May 1989 and April 2000. Patients with an acute exacerbation following lung biopsy were studied, and the HRCT images of the chest before and after surgery were reviewed. MEASUREMENTS AND RESULTS: Among the 236 consecutive patients with interstitial pneumonia who underwent a surgical lung biopsy, five (2.1%) (IPF, 3; NSIP, 1; COP, 1) developed acute exacerbation of the diffuse lung disease in the course of 1-18 days after SLB. The extent of parenchymal involvement on HRCT before surgery was not significantly different between operated and contralateral nonoperated lung. Significantly increased regions of parenchymal involvement on HRCT were seen postoperatively compared with the preoperative CT in both the operated (20.7+/-12.5% versus 38.2+/-10.8%, P = 0.0431) and nonoperated lung (22.7+/-13.8% versus 70.5+/-24.4%, P = 0.0431), but the extent of the parenchymal involvement was significantly greater on the nonoperated side (P = 0.0251). Two of the 3 IPF patients died from the acute exacerbation. CONCLUSIONS: It is important to be aware of the possibility of acute exacerbation of interstitial pneumonia following SLB even after an apparently uneventful immediate postoperative course. The asymmetric image findings suggest that intraoperative respiratory management is a possible etiologic factor.

A case of primary gastric small cell carcinoma with a rare pattern of lymph node metastasis.

We report a very rare case of primary gastric small cell carcinoma (GSCC) that was accompanied with gastric tubular adenocarcinoma. A male in his 60s had an elevated tumor with a central ulceration in the middle stomach. The patient underwent a distal gastrectomy with lymph node dissection. The pathological examination showed two separated lesions of the stomach, which contained the components of primary GSCC and primary gastric tubular adenocarcinoma. Immunohistochemical (IHC) examination demonstrated that the tumor cells in the small cell carcinoma stained positive for synaptophysin, chromogranin A, and neural cell adhesion molecule (NCAM). GSCC cells and adenocarcinoma cells independently metastasized to each regional lymph node. Further studies on the biological behavior of individual tumors may allow the development of new treatment strategies for GSCC.

Familial idiopathic pulmonary fibrosis: serial high-resolution computed tomography findings in 9 patients.

OBJECTIVE: The purpose of this study was to assess the high-resolution computed tomography (CT) findings of familial idiopathic pulmonary fibrosis (IPF). METHODS: High-resolution CT scans obtained in 9 consecutive patients with biopsy-proven familial IPF were reviewed. The presence, distribution, and extent of various CT findings were evaluated. RESULTS: All 9 patients had ground-glass opacities, intralobular reticular opacities, and irregular thickening of the interlobular septa; 7 (78%) had traction bronchiectasis, 7 (78%) had small foci of consolidation; and 3 (33%) had honeycombing. The abnormalities involved mainly the lower lung zones in 6 patients and the upper lung zones in 2 patients and had no zonal predominance in 1 patient. Follow-up CT showed increased extent of disease in 8 patients. CONCLUSIONS: The high-resolution CT findings of familial IPF resemble those of nonfamilial IPF. Familial IPF has a lower prevalence of honeycombing and a lower prevalence of predominant lower lung zone distribution than nonfamilial IPF, however.

Ruptured pancreaticoduodenal artery aneurysm with acute gangrenous cholecystitis: a case report and review of the literature.

We have experienced a very rare case of ruptured pancreaticoduodenal artery aneurysm with acute gangrenous cholecystitis. A 67-year-old male complaining of epigastralgia was admitted to our hospital. Ultrasound sonography demonstrated acute cholecystitis and cholecystolithiasis. Computed tomography scan showed the findings of acute cholecystitis and retroperitoneal mass. Emergency laparotomy revealed an acute gangrenous cholecystitis and a retroperitoneal hematoma around the second portion of the duodenum. Cholecystectomy was performed, however, the bleeding vessel was not identified. The patient bled again from the abdomen on the 6th postoperative day. A postoperative angiography indicated an inferior pancreaticoduodenal artery aneurysm. A resection of the aneurysm was performed following the angiography. Pancreaticoduodenal artery aneurysms are uncommon and ruptured pancreaticoduodenal artery aneurysms result in fatal hemorrhage and high mortality. We reviewed the previously reported cases and discussed the suitable and expeditious diagnosis and management of the pancreaticoduodenal artery aneurysms.