Long-term multidisciplinary treatment including proton therapy for a recurrent low-grade endometrial stromal sarcoma and pathologically prominent epithelial differentiation: an autopsy case report.

BACKGROUND: Long-term follow-up reports of low-grade endometrial stromal sarcoma (LGESS) including its clinical course and pathological data are rare. We previously reported the case of a Japanese woman diagnosed with LGESS, who was treated with multidisciplinary therapy. She had been suffering from uterine cervical tumor diagnosed as cervical polyps, or fibroid in statu nascendi, since 24 years old. The patient had survived for 25 years with the disease. This report presents her progress and pathological change since the previous report. CASE PRESENTATION: At age 45, the patient experienced a relapse of the remnant LGESS tumor between the right diaphragm and liver. Although chemotherapy was not effective, the tumor was eliminated by proton therapy. At age 46 years, the remnant tumors outside the irradiated field were resected. The disease was originally diagnosed as "neuroendocrine carcinoma (NEC)" using the surgical specimen. Therefore, cisplatin and irinotecan combination chemotherapy were administered to treat the remnant dissemination. After 4 cycles of chemotherapy, the liver metastases had enlarged and were resected surgically. Consequently, no remnant tumor was visible in the abdominal cavity at the end of the surgery. To determine the origin of NEC, we examined the previously resected specimens obtained from her ileum at age 40 years. A boundary between the LGESS and neuroendocrine tumor grade 2 (NET G2)-like lesion was found in the tumor, indicating that the origin of these tumors was LGESS. After less than 2 years of chemotherapy and undergoing surgery, a relapse of the tumor in the liver induced biliary duct obstruction with jaundice, which was treated with endoscopic retrograde biliary drainage. Although pazopanib prolonged her life for 10 months, she died from sepsis at age 49 years, which was caused by the infection that spread to the liver metastatic tumor via the stented biliary ducts. Autopsy revealed adenocarcinoma-like differentiation of the tumor. CONCLUSION: This LGESS patient has survived for a long time owing to multidisciplinary treatment including proton therapy. The LGESS tumor differentiated to NET G2-like tissue and then further to adenocarcinoma-like tissue during the long-term follow-up.

[Thoracic Aortic Injury after Spinal Fusion;Report of a Case].

A 32-year-old female visited our hospital with back pain. She had undergone spinal fusion for scoliosis at another hospital. She visited our hospital because she was informed that the metal rod had broken. Computed tomography(CT) revealed a suspected penetration of one of the screws into the descending aorta, and aortography showed the aortic wall dented by the screw. The aorta was exposed via thoracotomy, and the top of the screw was found penetrating into the adventitia of the aorta. After carefully detaching the region from the screw, the defect of the adventitia was repaired by continuous suture. The screw was cut to avoid further injury. Several days later, re-operation was successfully performed for the scoliosis.

[Sternal Nonunion after Open Heart Surgery through a Partial Sternotomy;Report of a Case].

Sternal nonunion is an uncommon complication after a sternal wound and some cases have been reported as a complication of open heart surgery. A 28-year-old male suffered from persistent sternal pain. Six years ago, open heart surgery was performed through a partial sternotomy with transverse sternotomy. The sternal nonunion was repaired using 3 sternal wires and 1 absorbable sternal pin. Three years later, the patient experienced recurrence of persistent sternal pain with clicking, because all of the sternal wires had ruptured and the sternal healing was incomplete. The patient was treated with adequate debridement and a technique using metal plates and autogenous bone grafts, which provided excellent pain relief. A transverse sternotomy potentially exposes the patient to the risk of sternal nonunion, which should be treated using autogenous bone grafting.

[Primary Malignant Pericardial Mesothelioma;Report of a Case].

A 69-year-old male was referred to our hospital after being diagnosed as having pericarditis with pericardial effusion. The symptoms of tamponade disappeared after the effusion was drained;although the cause of pericarditis remained unidentified. About 4 months later, the tamponade symptoms recurred due to the thickened nodular pericardium. Partial pericardiectomy was performed, however the patient died on the 52nd day after surgery. Immunohistological examination with calretinin led to the diagnosis of primary malignant pericardial mesothelioma, which was an extremely rare pathology. Because the hyaluronic acid content of the effusion has been reported as a diagnostic aid for malignant mesothelioma, routine examination of the hyaluronic acid content for pericarditis with pericardial effusion may be necessary for early diagnosis and to improve prognosis.

[Off-pump surgery for coronary-pulmonary artery fistula with giant coronary aneurysm; report of a case].

A 60-year-old male was referred to our hospital due to suspected chronic heart failure. He also had a symptom of ischemic heart disease. The multi-detector row computed tomography (MDCT) demonstrated the coronary-pulmonary artery fistula with a giant coronary aneurysm, which was partly thrombosed. Two abnormal arteries to the aneurysm and the outflow to the pulmonary artery were clearly detected. Intraoperative epicardial echocardiography revealed the complete interruption of blood flow and the thrombus formation in the coronary aneurysm, that was achieved just by ligation of 2 arteries under off-pump surgery. Generally, congenital coronary-pulmonary artery fistula has complex morphological variations. In this case, MDCT and epicardial echocardiography were useful for the surgical decision-making to avoid the residual and/or the recurrent fistula.

Diaphragmatic hernia post coronary artery bypass with gastroepiploic artery.

An 86-year-old man, with a medical history of coronary artery bypass grafting with the right gastroepiploic artery 20 years prior, was admitted to our hospital for right-sided heart failure. Computed tomography findings revealed an intrapericardial diaphragmatic hernia of the transverse colon compressing the right ventricle. The hernia was successfully repaired, and the patient recovered without any complications. Diaphragmatic hernia is rare but may be a lethal complication following coronary artery bypass grafting. We should be aware of the possibility of this condition.

[Coronary artery bypass grafting after total esophagectomy].

A 69-year-old male with a history of total esophagectomy and substernal placement of the gastric tube for esophageal carcinoma was admitted due to an unstable angina. Cardiac catheterization revealed a severe stenosis just proximal to the left anterior descending coronary artery and a 75% stenosis of the right coronary artery. Intraaortic balloon pumping was started in the catheter laboratory. Off-pump coronary artery bypass grafting was performed through left thoracotomy. The left internal mammary artery could not be utilized as a bypass graft to the left anterior descending artery due to severe substernal adhesion. Percutaneous coronary intervention was selected for the revascularization of the right coronary artery lesion.