Giant cell tumor expanded into the thoracic cavity with spinal involvement

This article describes a case of a giant cell tumor that expanded into the thoracic cavity and through the spinal canal into the vertebrae. A 36-year-old man presented with a 6-month history of back pain and dyspnea. Plain chest radiographs showed a huge mass accompanied by right pleural effusion. The mass involved the 12th thoracic spine, and the spinal cord was severely compressed. The tumor was resected with a 2-stage procedure. As a first stage to separate the tumor from the anterior vital structures under direct vision, thoracic surgeons performed a right thoracotomy with chest wall reconstruction from the 8th to 11th ribs. The right lung and inferior vena cava were gently retracted, and the tumor was carefully detached from these structures. We were not able to separate the tumor from the right diaphragm due to severe invasion; therefore, we performed partial resection of the right diaphragm with the tumor. After excision of the anterior part of the tumor, the thoracic wall was reconstructed with the right eighth rib and Marlex mesh. When the patient's general condition improved 2 weeks later, spondylectomy by posterior approach was performed. We achieved excision of a giant cell tumor that had expanded into the thoracic cavity and through the spinal canal into the vertebrae. The patient had achieved full rehabilitation with no neurological or respiratory abnormalities at 7 years postoperatively.     

Preoperative embolization significantly decreases intraoperative blood loss during palliative surgery for spinal metastasis

Several studies have evaluated the efficacy of preoperative embolization in devascularizing tumors. However, no study has measured intraoperative blood loss in a single palliative surgery compared with a control group without preoperative embolization. The purpose of this retrospective study was to evaluate the efficacy of preoperative embolization on intraoperative blood loss in palliative decompression and instrumented surgery using a posterior approach for spinal metastasis.Between 2000 and 2010, forty-six patients underwent palliative decompression and instrumented surgery using a posterior approach for spinal metastasis in the thoracic and lumbar spine. Preoperative embolization was performed in 23 patients (embolization group), and surgery was performed within 3 days after embolization. The embolic materials used were polyvinyl alcohol particles, gelatin sponge, and metallic coils. Twenty-three patients did not undergo embolization (no embolization group). Pain and neurologic symptoms in all 46 patients were relieved postoperatively. Average intraoperative blood loss was 520 mL (range, 140-1380 mL) in the embolization group and 1128 mL (range, 100-3260 mL) in the no embolization group (P<.05). In the embolization group, intraoperative blood loss was not correlated with the degree of tumor vascularization, completeness of embolization, or time between embolization and surgery.Intraoperative blood loss after preoperative embolization was less than half that after no preoperative embolization.     

A case of complex (type IV) paraesophageal hiatal hernia with herniation of the small intestine

A 78-year-old woman was admitted to our hospital because of abdominal pain. She was diagnosed with complex (type IV) paraesophageal hiatal hernia, for which an operation was performed. At laparotomy, a 50 cm long ileum was found to have herniated into the thoracic cavity through the esophageal hiatus along with the sliding hiatal hernia of the stomach. Both the organs were reduced to the abdominal cavity. The hiatal hernia defect (diameter, 7cm) was repaired with a direct suture, and the gastric fundus was sutured to the diaphragm. Presently, 6 years have passed since the operation, there is no sign of recurrence.     

Estimating the Need for Neck Lymphadenectomy in Submucosal Esophageal Cancer Using Superparamagnetic Iron Oxide-Enhanced Magnetic Resonance Imaging: Clinical Validation Study

Background  

In cases of thoracic esophageal cancer, multidirectional lymphatic flow from the tumor means that lymph node metastasis can occur in an area extending from the neck to the abdomen. To validate a method for limiting the performance of three-field lymphadenectomy only to patients who need it, we carried out a prospective study in which superparamagnetic iron oxide (SPIO)-enhanced lymphatic mapping was used to determine whether to perform neck lymph node dissection in patients with submucosal thoracic esophageal cancer.     

Effects of Emptying Function of Remaining Stomach on QOL in Postgastrectomy Patients

Background  

Attention has recently focused on decreased quality of life (QOL) that occurs in postgastrectomy patients. We verified how gastric emptying function affected QOL.     

Difficulty of pinching behind the back: an atypical symptom of carpal tunnel syndrome related to a specific wrist position. Two case reports

Carpal tunnel syndrome (CTS) may be overlooked in the absence of typical sensory symptoms. Two patients with CTS lacked the attendant sensory symptoms but experienced difficulties performing a pinching action behind the back (manipulation of the buckle of a baby sling or the hook of a brassiere), a mode of pinching that required wrist flexion. The causative mechanism was probably exacerbation of a latent weakness of the intrinsic muscles of the thumb by wrist flexion, in which the strength of the extrinsic flexors of the thumb and index finger were decreased due to loosening of the tendons. Such symptoms, induced by a specific wrist position, may be help to diagnose a latent weakness in the intrinsic muscles of the thumb, present in patients with CTS.     

Deep sequencing reveals stepwise mutation acquisition in paroxysmal nocturnal hemoglobinuria

Paroxysmal nocturnal hemoglobinuria (PNH) is a nonmalignant clonal disease of hematopoietic stem cells that is associated with hemolysis, marrow failure, and thrombophilia. PNH has been considered a monogenic disease that results from somatic mutations in the gene encoding PIGA, which is required for biosynthesis of glycosylphosphatidylinisotol-anchored (GPI-anchored) proteins. The loss of certain GPI-anchored proteins is hypothesized to provide the mutant clone with an extrinsic growth advantage, but some features of PNH argue that there are intrinsic drivers of clonal expansion. Here, we performed whole-exome sequencing of paired PNH⁺ and PNH^– fractions on samples taken from 12 patients as well as targeted deep sequencing of an additional 36 PNH patients. We identified additional somatic mutations that resulted in a complex hierarchical clonal architecture, similar to that observed in myeloid neoplasms. In addition to mutations in PIGA, mutations were found in genes known to be involved in myeloid neoplasm pathogenesis, including TET2, SUZ12, U2AF1, and JAK2. Clonal analysis indicated that these additional mutations arose either as a subclone within the PIGA-mutant population, or prior to PIGA mutation. Together, our data indicate that in addition to PIGA mutations, accessory genetic events are frequent in PNH, suggesting a stepwise clonal evolution derived from a singular stem cell clone.