TWIST gene mutation in a patient with radial aplasia and craniosynostosis: further evidence for heterogeneity of Baller-Gerold syndrome

BACKGROUND: The optimal management of high-grade dysplasia in patients with Barrett's esophagus is controversial. The aim of this study was to assess the prevalence of unsuspected carcinoma at esophagectomy in patients with Barrett's esophagus with high-grade dysplasia after endoscopic surveillance with jumbo biopsy forceps compared with standard biopsy forceps. METHODS: Twelve patients with high-grade dysplasia in Barrett's esophagus without gross or microscopic evidence of carcinoma underwent esophagectomy after preoperative endoscopy with 4-quadrant jumbo biopsies at 2-cm intervals. The findings in this group were compared with those in a group of patients with Barrett's esophagus who underwent esophagectomy for high-grade dysplasia after biopsies obtained at 2-cm intervals with standard biopsy forceps. RESULTS: Unsuspected cancer was found in 4 of 12 (33%) patients in the jumbo biopsy group compared with 6 of 16 (38%) in the standard biopsy group (p = NS). All 6 cancers in the standard biopsy group were intramucosal, whereas 2 were intramucosal and 2 were submucosal in the jumbo biopsy group. No patients in either group had lymph node metastases. CONCLUSIONS: Unsuspected cancer is found frequently in patients with Barrett's esophagus who are undergoing esophagectomy for high-grade dysplasia despite the use of a rigorous jumbo biopsy protocol. Esophageal resection is still indicated in appropriately selected patients with high-grade dysplasia until better markers of cancer risk are available.     

Anterior spinal artery syndrome after thoraco-abdominal esophagus resection. A rare complication

We report a case of postoperative paraplegia resembling an anterior spinal artery syndrome after curative esophagectomy in a patient with carcinoma of esophagus and clinical stage III (UICC). Neurologic deficit was characterized by loss of sensibility at the level of T12/L1 together with paraparesis of both lower extremities. Furthermore, dissociated sensorimotor depletion at C6/C7 (right-sided) and at T5 (left-sided) was noted. This severe complication was most probably caused by peeling of an arteriosclerotic plaque of the thoracic aorta due to preexisting advanced arteriosclerosis, leading to a partial occlusion of the great radicular artery of Adamkiewicz. Even though anterior spinal artery syndrome is a well-known problem in the operative management of thoracic aortic aneurysms, this complication has not previously been reported after esophagectomy.     

Extrathoracic esophagectomy in the treatment of esophageal cancer

Extrathoracic esophagectomy has the potential of improving the results of resectional therapy for carcinoma of the esophagus by eliminating the need for thoracotomy and decreasing postoperative pulmonary complications. This report compares the operative and functional results of blunt extrathoracic esophagectomy and substernal reversed gastric tube reconstruction in patients with esophageal cancer to results in 10 consecutive nonrandomized control patients treated by standard esophagogastrectomy. Extrathoracic esophagectomy was associated with greater pulmonary dysfunction than standard esophagogastrectomy. While there was no significant difference in survival in the two groups, three patients in the standard esophagogastrectomy group (mean survival 9.0 months) and none in the extrathoracic esophagectomy group (mean survival 7.4 months) developed anastomotic recurrence. Extrathoracic esophagectomy evidently does not afford patients with esophageal carcinoma better palliation than standard esophagogastrectomy.     

Bilateral orbital metastases from breast carcinoma. A case of false pseudotumor

OBJECTIVE: To inform clinicians of a metastatic orbital neoplasm that frequently masquerades as an orbital inflammatory syndrome and to stimulate thought regarding this neoplasm's fundamental pathobiology specific to its orbital involvement. DESIGN: Interventional case report and literature review. PARTICIPANTS: A 47-year-old woman with an acquired, bilateral, diffusely infiltrative orbital process is described. INTERVENTION: The clinical and radiographic features of the patient's orbital process are reported. The review of two prior fine-needle aspiration biopsy specimens failed to reveal a diagnosis. Histopathologic examination of an open surgical biopsy specimen included standard light microscopy of frozen and formaldehyde-fixed, paraffin-embedded tissue. RESULTS: Characteristics light microscopy findings suggested the diagnosis of metastatic lobular carcinoma of the breast. The patient refused all therapeutic intervention and died 9 months after orbitotomy. CONCLUSION: The presentation of breast carcinoma metastatic to the orbit as a bilateral infiltrative process is far more common than appreciated previously. Its rate of bilateral orbital involvement is 20%, whereas this rate for other adult neoplasms is much lower. The authors report an additional case, review the clinical features of previous case reports and series, and discuss aspects of the metastatic process. Breast carcinoma must be included in the differential diagnosis of infiltrative orbital processes encountered in adult female patients. When "orbital pseudotumor" is detected in an adult female patient, a thorough medical history must be obtained and physical examination performed. If a possible primary breast carcinoma is indicated, one must be wary of metastatic disease, and a histologic orbital diagnosis is warranted. The frequency of orbital metastasis and bilateral orbital involvement of this neoplasm reinforces the possibility for organ-specific metastases.     

Esophageal cancer and second primary brain tumor

We report a rare case of a second primary brain tumor following esophageal cancer. A 56-year old Japanese man underwent a transhiatal esophagectomy for esophageal carcinoma. Two years after the operation, signs of increased intracranial hypertension including headache, nausea and left limb ataxia were observed. The tumor was totally removed by a suboccipital craniectomy, and the histological diagnosis was hemangioblastoma. The post-operative course was uneventful and the patient is still alive without any signs of recurrence. It is important to make a precise strategy for the second primary tumor.     

A case of free jejunal graft for anastomotic recurrence after subtotal esophagectomy

A case of anastomotic recurrence after a radical operation for thoracic esophageal carcinoma is presented. A 68-year-old male was treated by subtotal esophagectomy and esophagogastrostomy through retrosternal route. One year after the operation he experienced dysphagia and anastomotic recurrence was detected by an upper gastrointestinal series and fiberscopy. He was admitted to our hospital for radiation therapy. The response was poor and dysphagia wasn't disappeared after radiation therapy at a dose of 10,200 rad. He was consulted to our surgical department for operative therapy. Cervical esophagectomy, partial resection of gastric tube and free jejunal transplantation for the reconstruction were performed. He had been eating anything of food after the operation.     

Biopsy-negative malignant esophageal stricture: diagnosis by endoscopic ultrasound

Endoscopic ultrasound (EUS) of the esophagus has been used primarily in staging biopsy-proven cancers. Its use as a primary diagnostic modality for esophageal malignancy has not been previously described. We report our recent experience in four patients with dysphagia and endoscopic biopsies negative for malignancy, including one patient with clinical and manometric features suggestive of achalasia. In all cases, EUS revealed a large infiltrating tumor invading through the esophageal wall into the surrounding tissues, and in one case into the aorta. Computed tomography suggested the possibility of a tumor in only one of the cases. Two patients underwent esophagectomy and were found to have adenocarcinoma. Two patients underwent repeat biopsy with alternative aggressive biopsy techniques and were found to have squamous cell carcinoma. We conclude that EUS is useful in the diagnosis of esophageal cancer and should be performed in selected patients with esophageal strictures whose biopsies are negative for malignancy; i.e., those with suspicious endoscopic or radiographic appearance, atypical presentation (e.g., profound weight loss, short duration of symptoms, or advanced age), and failure to respond to treatment.     

Synchronous multifocal development of invasive transitional cell carcinoma of the urinary tract in a patient with renal failure receiving long-term hemodialysis: a case report

We report a case of transitional cell carcinoma in a patient with chronic renal failure receiving hemodialysis for 22 years. A 55-year-old man was admitted to our hospital. Under diagnosis of invasive bladder cancer and left renal pelvic tumor, removal of the whole urinary tract, e.g., bilateral nephroureterectomy and total cystourethrectomy was performed. Transitional cell carcinoma was found in bilateral renal pelvis, left ureter, bladder and prostate in the resected specimen. Thirteen months after the operation, multiple lung metastases and pathologic bone fracture of the 4th lumber vertebra were found. Chemotherapy (3 courses of modified CISCA, consisting of cisplatin, adriamycin and cyclophosphamide) was performed, but the died of systemic metastases of cancer and bleeding due to perforation of multiple gastric ulcers.     

Mammographic findings after stereotaxic biopsy of the breast performed with large-core needles

Colorectal cancer generally affects men and women in the later decades of life. Typically patients present with bowel obstruction and/or chronic anemia. The epidemiology, presentation, and prognosis of cecal carcinoma, the third most common colorectal cancer, is similar to other cancers of the large bowel. Cecal and other colorectal cancers rarely present in adolescence. In this case report, we describe a 19-year-old woman presenting with a pelvic mass and elevated tumor markers with the presumed diagnosis of ovarian cancer, who was found to have cecal carcinoma at laparotomy. This case illustrates that colorectal cancer, although rare, should be considered in the differential diagnosis of a pelvic mass in young women who present with anemia, constitutional symptoms, and elevated tumor markers.     

Spontaneous regression of pulmonary metastases from transitional cell carcinoma

Spontaneous regression of metastatic cancers occurs rarely and has been reported only once for pulmonary metastases from transitional cell carcinoma. Two cases of spontaneous complete regression of lung metastases from transitional cell carcinoma are presented. In one case, regression occurred after a course of radiation to the primary bladder cancer, but in the other patient, lung lesions disappeared without treatment to the primary or metastatic cancers. The factors that alter the tumor-host relationship to allow spontaneous regression of cancers are unknown, but observation of these phenomena may help reveal parameters that influence tumor progression in the majority of cancer patients.     

Association of xerophthalmia, flecked retina, and pseudotumor cerebri caused by hypovitaminosis A

PURPOSE: To report an unusual case of hypovitaminosis A with bilateral papilledema and flecked retina that regressed after restoration of vitamin A deficiency. METHOD: Case report. A 27-year-old woman had undergone a biliopancreatic bypass for obesity in 1990. In 1995, she presented with bilateral xerophthalmia, bilateral papilledema, and bilateral flecked retina. RESULTS: Laboratory tests demonstrated low serum levels of vitamin A (0.8 micromol/l) and vitamin E (8.54 micromol/l). Xerophthalmia, papilledema, and flecked retina disappeared after restoration of normal vitamin A blood levels. CONCLUSION: Hypovitaminosis A after biliopancreatic bypass for obesity may be associated with xerophthalmia, pseudotumor cerebri, and flecked retina.     

Abdominal incisional hernia occurring after an esophagectomy reconstructed with a gastric tube through an antesternal route: report of a case

A Japanese man, who had undergone a subtotal esophagectomy reconstructed with a gastric tube through an antesternal route for esophageal carcinoma 16 years previously, was admitted to our hospital because of an abdominal incisional hernia. The abdominal incisional hernia was in his upper abdomen and was difficult to push back into the intraabdominal cavity by hand. The hernia was successfully repaired by operation. We thus conclude that an abdominal incisional hernia is a rare but important late-phase complication occurring after an esophagectomy reconstructed with either an antesternal or retrosternal route, and an operation should be the treatment of choice.     

Combined lung resection for advanced thoracic esophageal carcinoma

We performed lung resection together with esophagectomy in 2 patients with advanced thoracic esophageal cancer. Both patients survived more than 2 years with no evidence of disease. The first case was a 60-year-old man who had a cancer lesion in middle of the intra-thoracic esophagus (Im) and the right lower lobe of the lung was involved. In March 1989, right lower lobectomy of the lung was performed with esophagectomy. Pathologic examination showed well differentiated squamous cell carcinoma invading the lung parenchyma and intrapulmonary lymph node. Postoperatively, 44 Gy of radiation and Peplomycin cancer chemotherapy was performed. The patient survived 51 months after surgery and died of chronic myelogenous leukemia. The second case was a 60-year-old man who underwent thoracic esophagectomy with resection of the involved pericardium and right lung in February 1992. Pathologic examination showed N3 lymph node metastasis. Postoperatively, the patient received 48 Gy of radiation and was free from cancer after 30 months. In conclusion, better surgical results are expected in cases of advanced thoracic esophageal cancer with lung involvement which can be completely resected en bloc with the primary tumor even in a3 cases than in those with aortic or tracheobronchial involvement.     

Multiple bilateral chondromatous hamartomas of the lung. A rare entity mimicking metastatic carcinoma

Multiple chondromatous hamartomas are extremely rare and we have not found any bilateral presentation in the literature. We report the case of a 47-year-old woman presenting with a cough, chest pain and dyspnea. Chest x-ray and computed tomography revealed multiple and bilateral pulmonary nodules. A metastatic carcinoma was considered, but the open lung biopsy revealed a chondromatous hamartoma of the lung. The pathogenesis of hamartomas is discussed and the recent literature is reviewed.     

Copper/zinc superoxide dismutase transgenic brain accumulates hydrogen peroxide after perinatal hypoxia ischemia

OBJECTIVE: This study aims to evaluate the risk of esophagectomy in the elderly compared with younger patients and to determine whether results of esophagectomy in the elderly have improved in recent years. SUMMARY BACKGROUND DATA: An increased life expectancy has led to more elderly patients presenting with carcinoma of the esophagus in recent years. Esophagectomy for carcinoma of the esophagus is associated with significant morbidity and mortality, and advanced age is often considered a relative contraindication to esophagectomy despite advances in modern surgical practice. METHODS: The perioperative outcome and long-term survival of 167 elderly patients (70 years or more) with esophagectomy for carcinoma of the esophagus were compared with findings in 570 younger patients with esophagectomy in the period 1982 to 1996. Changes in perioperative outcome and survival between 1982 to 1989 and 1990 to 1996 were separately analyzed. RESULTS: The resection rate in the elderly was 48% (167/345), lower than the 65% (570/874) resection rate in younger patients (p < 0.001). There were significantly more preoperative risk factors and postoperative medical complications in the elderly, but no significant differences were observed in surgical complications. The 30-day mortality rate was higher in the elderly (7.2%) than in younger patients (3.0%) (p = 0.02), but the hospital mortality rate was not significantly different in the elderly (18.0%) and younger age groups (14.4%) (p = 0.27). The long-term survival after curative resection in elderly patients was worse than younger patients (p = 0.01). However, when deaths from unrelated medical conditions were excluded from analysis, survival was similar between the two age groups (p = 0.23). A comparison of data for the periods 1982 to 1989 and 1990 to 1996 revealed that the resection rate had increased from 44% to 54% in the elderly, with significantly fewer postoperative complications and lower 30-day and hospital mortality rates. Long-term survival has also improved, although this has not reached a statistically significant level. CONCLUSIONS: With current surgical management, esophagectomy for carcinoma of the esophagus can be carried out with acceptable risk in the elderly, but intensive perioperative support is required. The improved results of esophagectomy in the elderly in recent years are attributed to increased experience and better perioperative management. Long-term survival was similar to that of younger patients, excluding deaths caused by unrelated medical conditions.     

Endometrioid carcinoma arising from endometriosis of the sigmoid colon during estrogenic treatment

A case of endometrioid carcinoma arising from endometriosis of the sigmoid colon is reported. The patient had been treated with unopposed continuous estrogen injection for twenty years after bilateral salpingo-oophorectomy because of severe endometriosis. The pathology, pertinent literature and implications of the present case are discussed.     

A sensitive assay for studying dopaminergic activity in cultures of rat pituitary cells

Nine male patients with separate primary cancers of the esophagus and head and neck (pharynx, larynx) presented with a mean age of 56 years (41-69). They included 7 pharyngeal cancer patients and 2 laryngeal ones. Esophageal cancer was discovered synchronously in 6 patients and metachronously in 3 (1, 4, and 11 years later, respectively). The head and neck cancer was stage-I in one patient, stage-II in 4 and stage-IV in 4. The esophageal cancer was cervical in 2, thoracic in 6 and abdominal in 1. It was early cancer (stage-0) in 6 patients and advanced (stage-IV) in 3. The esophageal cancer was more advanced in the metachronous group, while it was early in the synchronous group. Since the head and neck cancer was advanced, all patients underwent a total laryngectomy for their head and neck cancers. As for esophageal surgery, a transhiatal esophagectomy was, in principle, performed for early cancers while a total thoracic esophagectomy was done for advanced cancers. For the reconstruction of the esophagus, a gastric tube was used. Four patients are still alive with a mean survival time of 25 months, whereas five died of cancer recurrence of either type a mean of 19 months after surgery. As compared with the survival rates of the patients with esophageal cancer alone, the 5-year survival rate was 18.2% for patients with double cancers in this series and 27.9% for those with esophageal cancer alone.     

The treatment of excessive somnolence with stimulant drugs

In this paper we report on the long-term results achieved in 75 patients with superficial carcinoma of the thoracic esophagus who have been treated since 1965 with esophagectomy at our institute. In three of these patients, the carcinoma was limited to the epithelium (intra-epithelial carcinoma), while in 13 cases it was confined to the mucosa, and in 59 cases to the submucosa. There was no lymph node metastasis in patients with intra-epithelial or mucosal carcinoma, but lymph node involvement was found in 13 (22.0%) of the 59 cases of submucosal carcinoma. The five-year survival rate of patients with intra-epithelial carcinoma and mucosal carcinoma was 100%, which was significantly better than that for carcinoma invading the submucosa (49%). The five-year survival rate for patients with lymph node involvement was only 46%, which was significantly worse than that for patients without lymph node involvement (60%). No recurrence occurred in patients with intra-epithelial carcinoma or mucosal carcinoma, while recurrent disease occurred in 19 (32.2%) of the 59 patients with submucosal carcinoma. Lymph node recurrence and hematogenic recurrence such as lung and bone metastasis were the most frequent developments in patients with submucosal carcinoma of the esophagus.     

Primary, pure, large-cell neuroendocrine carcinoma of the urinary bladder

We report what to our knowledge is the first case in the English-language literature of a primary, pure, undifferentiated large-cell neuroendocrine carcinoma of the urinary bladder. To date, only one case of a large-cell neuroendocrine carcinoma was reported, and it was associated with an adenocarcinoma most likely of urachal origin. On the other hand, slightly more than 100 cases of undifferentiated small-cell carcinoma of the urinary bladder were reported, approximately one-half of which were associated with poorly differentiated transitional-cell carcinoma of the conventional type. The patient in our case was a 73-year-old man with a history of prostatic cancer treated with radiation therapy. He presented with hematuria, leading to the discovery of a solitary tumor on the dorsal wall of the urinary bladder. A diagnosis of large-cell neuroendocrine carcinoma was made, supported by immunohistochemical reactivity for chromogranin, neuron-specific enolase, and synaptophysin; a variety of other hormonal markers of neuroendocrine tumors were negative. The radical cystoprostatectomy and bilateral pelvic lymphadenectomy specimen showed a transmurally invasive tumor, without regional lymph node metastases. The patient died 2 months after surgery, and the autopsy revealed disseminated metastases histologically identical to the urinary bladder neoplasm. Awareness of the occurrence of large-cell neuroendocrine carcinoma of the urinary bladder seems to be important because of the possible aggressive outcome associated with this tumor and because of differential diagnostic considerations, which include malignant lymphoma and metastasis from another primary, especially in tumors occurring in a pure form.