Outcomes and failure patterns in childhood craniopharyngiomas

Past studies of craniopharyngiomas in children have shown overall survival (OS) up to 95% at 5 years and 80% progression-free survival (PFS) at 5 years, although many of these series predate modern neuroimaging and current therapeutic management. Moreover, little mention has been made of failure patterns for craniopharyngioma in children. To obtain a contemporary assessment of outcome among pediatric craniopharyngioma patients, and also to determine the failure patterns for this tumor, we completed a retrospective study of a consecutive cohort of all children with craniopharyngioma diagnosed at the Johns Hopkins Hospital from 1980 to 1996. Resection was performed in 30 children, in 8 of whom gross total resection (GTR) was achieved. Initial treatment took the form of GTR followed by observation for 8, subtotal resection (STR) plus observation in 11, and STR followed immediately by radiotherapy in 8. The timing of radiotherapy following STR was unclear for 3. OS was 95.2% (SE= 4.7%) at 5 years, with only 2 children dying after 4 years from diagnosis. Five-year PFS was 59.4% (SE=10.2%). Before surgery, 19 children had visual loss and 15, endocrine deficits; after surgery, 21 children had visual loss and 29, endocrine deficits. Median time to relapse was 0.98 years (SD=2.5 years). Radiographic (n=4) and clinical (n=7) relapses did not differ in time to progression (P=0.32), but radiographic relapses were significantly associated with age at diagnosis less than 5 years (P=0.02). Degree of resection was not significantly associated with PFS (P=0.32) or with postoperative visual or endocrine deficits. Absence of calcification on diagnostic neuroimaging (n=8) was significantly associated with improved PFS [5-year PFS 100% vs. 42.9% (SE=14.7%), P=0.02], even when adjusted for extent of resection (P=0.03). Preoperative visual loss was predictive of postoperative visual loss (P=0.03). Survival for children diagnosed with craniopharyngioma in the current era is outstanding, even with relapse, although postoperative visual and endocrinological morbidities are high. Failures occurred both radiographically and clinically, typically in the first 3-4 years after surgery, suggesting a need for close surveillance initially with neuroimaging, particularly in younger children, and also clinical examination. The short times to relapse observed here may stem from a tendency to delay radiotherapy until recurrence. Lack of calcification at diagnosis is associated with a tendency to remain free of relapse.     

Relationship between CA 15-3 serum levels and disease extent in predicting overall survival of breast cancer patients with newly diagnosed metastatic disease

Soft tissue sarcomas (STS) of the hand are rare in children and adolescents. From 1965 through 1995, 18 children with STS of the hand were treated at our institution. Rhabdomyosarcoma (RMS) was diagnosed in 11 patients; alveolar histological results predominated (7 of 11 cases). Seven patients presented with metastatic disease and died 4 to 23 months (median, 9 months) from diagnosis; their surgical treatment comprised above-elbow amputation (n = 1), local excision (n = 1), and biopsy (n = 5). For the four patients who presented with localized RMS, surgery consisted of wide local excision (n = 1), local excision (n = 2), or ray amputation (n = 1). With an average follow-up of 5.5 years (range, 4 months to 18 years), 3 of the 11 patients diagnosed with RMS still survive (27%). The remaining seven patients presented with nonrhabdomyosarcomatous soft tissue sarcoma (NRSTS); the most common histological variants were epithelioid and malignant fibrous histiocytoma (two cases each). Surgical treatment for these patients comprised ray amputation (n = 3), wide local excision (n = 3), excisional biopsy (n = 1), and regional lymph node dissection (n = 3). One patient received adjuvant multiagent chemotherapy; three patients received supplemental radiotherapy. Six of the seven (85%) patients are alive with no evidence of disease at an average follow-up of 4.7 years (range, 6 months to 12 years).     

Supratentorial malignant gliomas in childhood: a single institution perspective

BACKGROUND: A retrospective study evaluated the clinical characteristics, prognostic factors, and outcome of patients with newly diagnosed supratentorial malignant gliomas treated with preirradiation chemotherapy. METHODS: Of 41 patients with supratentorial malignant gliomas accrued between 1984-1994, all had neuroimaging documentation of the extent of resection and 37 had complete neuraxis staging prior to treatment; 80% were treated with one of a variety of neoadjuvant chemotherapy regimens. RESULTS: Thirteen patients had anaplastic astrocytoma (AA), 25 had glioblastoma multiforme (GBM), and 3 had anaplastic oligodendroglioma. Gross total resection (GTR) was performed in 10 patients, subtotal resection (STR) in 22 patients, and biopsy (Bx) alone in 9 patients. For the entire group the 3-year overall and progression free survivals were 35 +/- 8% and 18 +/- 6%, respectively. Tumor recurrence was dominantly local. However, 9 patients with initially local disease failed at a distant neuraxis site, giving a 26 +/- 7% actuarial risk of dissemination at 3 years. The only significant prognostic factor was extent of tumor resection: patients who underwent GTR survived longer than those who underwent STR or Bx (P = 0.004). Histology (GBM vs. AA), age, and the use of enhanced local dose radiation therapy (brachytherapy or stereotactic irradiation) did not affect survival. CONCLUSIONS: Neoadjuvant chemotherapy was not associated with a survival rate significantly different from that observed in adjuvant chemotherapy studies. Systematic neuraxis staging at diagnosis and recurrence revealed a rate of neuraxis dissemination as a component of recurrence that was higher than previously reported; the utility of craniospinal irradiation in preventing isolated dissemination remains uncertain.     

Vagal paraganglioma: a review of 46 patients treated during a 20-year period

BACKGROUND: Vagal paragangliomas (VPs) arise from paraganglia associated with the vagus nerve. Approximately 200 cases have been reported in the medical literature. Because of their rarity, most information regarding these tumors has arisen from case reports and small clinical series. OBJECTIVE: To detail the clinicopathologic features of 46 patients with VP with an emphasis on the role of a multidisciplinary skull base team in both the successful extirpation and rehabilitation. DESIGN: Retrospective review of 46 patients with VP managed by a single skull base team. SETTING: An academic tertiary medical center. RESULTS: Forty-six patients were treated over a 20-year period (1978-1998). Ten (22%) demonstrated intracranial extension. There was a history of familial paragangliomas in 9 (20%) of the patients. The incidence of multicentric paragangliomas was 78% in patients with familial paragangliomas vs 23% in patients with nonfamilial paragangliomas. Management of this group of 46 patients consisted of surgery (n = 40), radiation therapy (n = 4), and observation (n = 2). The operative approach consisted of a transcervical excision often combined with a transtemporal or lateral skull base approach as dictated by the tumor extent. Postoperative cranial nerve deficits were common, and, as such, aggressive rehabilitation was a vital component in the management of these tumors. CONCLUSIONS: The management of VP and its associated cranial nerve deficits remains a difficult clinical problem. Options for treatment include surgical resection, radiation therapy, and, in selected cases, observation. Surgical extirpation requires a multidisciplinary skull base team to achieve complete tumor resection. Radiation therapy is reserved for elderly patients and patients at risk for bilateral cranial nerve deficits. Rehabilitation of cranial nerve deficits is an integral part of the management of VP.     

Combination of systemic acetazolamide and topical dorzolamide in reducing intraocular pressure and aqueous humor formation

BACKGROUND: Neuroendocrine tumors commonly metastasize to the liver. Although surgical resection is considered a treatment option for patients with localized metastases confined to the liver, the longterm survival benefit of liver resection has not been clearly demonstrated. We examined the survival of patients undergoing liver resection for this disease. STUDY DESIGN: Between 1984 and 1995, we evaluated 38 patients with liver-only metastases from neuroendocrine tumors, including 21 carcinoid, 13 islet cell, and 4 atypical neuroendocrine neoplasms. Data from a combined prospective and retrospective database and a tumor registry were analyzed. Of these patients, 15 underwent complete resection of all known disease. The remaining 23 patients, who also had disease confined to the liver, had comparable tumor burden but were believed to be unresectable. The longterm survival rates of these two groups were compared. RESULTS: Patients who underwent liver resection did not differ from those who were unresectable with regard to age, pathology, primary tumor site, serum alkaline phosphatase levels, or percentage of the liver involved. All resections were complete, leaving no residual disease, and consisted of lobectomy (n = 3), segmentectomy (n = 1), and wedge resections (n = 11). There were no operative deaths. Patients who underwent hepatic resection had a significantly longer survival than unresected patients. Although median survival had not been reached in resected patients, the median survival in the unresectable group was 27 months. Patients who underwent liver resection had a higher 5-year actuarial survival (73% versus 29%). CONCLUSIONS: Hepatic resection in selected patients with isolated liver metastases from neuroendocrine tumors may prolong survival. This conclusion was reached by comparing our resected group with an unresectable group with similar tumor burden.     

Hereditary persistence of alpha-fetoprotein. Case report and review of the literature

BACKGROUND: The issue of performing simultaneous pulmonary resection and cardiac surgery in patients with coexisting lung carcinoma and ischaemic heart disease remains controversial. We report our experience and review the literature. METHODS: Thirteen patients (male ten, female three; mean age 65 years) underwent simultaneous cardiac surgery and pulmonary resection. Lung pathology consisted of primary lung carcinoma (n = 10), benign disease (n = 2) and carcinoid (n = 1). Lung resections included pneumonectomy (n = 3), lobectomy (n = 4), segmentectomy (n = 1) and local excision (n = 5). Cardiac procedures consisted of coronary artery bypass grafting (CABG) in 11, aortic valve replacement in one and mitral valve repair with CABG in one patient. In all but one case the lung resection was performed prior to heparinization and cardiopulmonary bypass (CPB). In two patients, with suitable coronary anatomy, myocardial revascularization without CPB was performed to reduce morbidity. RESULTS: There was no hospital mortality. Postoperative blood loss and ventilation requirements were reduced in the patients who were operated on without CPB. Prolonged ventilatory support was required in two cases. All patients with benign pathology are alive. In the lung cancer group there have been five late deaths: disseminated metastatic disease (n = 3), anticoagulant related haemorrhage (n = 1) and broncho-pleural fistula (n = 1). Of the remaining five patients four are alive and disease free 7-23 months post-operatively; one patient has recurrent disease 40 months post-operatively. CONCLUSIONS: Simultaneous pulmonary resection and cardiac surgery is associated with acceptable operative morbidity and mortality. In patients with lung carcinoma long-term survival was determined by tumour stage. The avoidance of CPB may be advantageous by decreasing blood loss and ventilation requirements.     

"Salvage" transjugular intrahepatic portosystemic shunts: gastric fundal compared with esophageal variceal bleeding

PURPOSE: To determine the efficacy of definitive surgery and radiation in patients aged 70 years and older with supratentorial glioblastoma multiforme. METHODS AND MATERIALS: We selected elderly patients (> or = 70 years) who had primary treatment for glioblastoma multiforme at our tertiary care institution from 1977 through 1996. The study group (n = 102) included 58 patients treated with definitive radiation, 19 treated with palliative radiation, and 25 who received no radiation. To compare our results with published findings, we grouped our patients according to the applicable prognostic categories developed by the Radiation Therapy Oncology Group (RTOG): RTOG group IV (n = 6), V (n = 70), and VI (n = 26). Patients were retrospectively assigned to prognostic group IV, V, or VI based on age, performance status, extent of surgery, mental status, neurologic function, and radiation dose. Treatment included surgical resection and radiation (n = 49), biopsy alone (n = 25), and biopsy followed by radiation (n = 28). Patients were also stratified according to whether they were optimally treated (gross total or subtotal resection with postoperative definitive radiation) or suboptimally treated (biopsy, biopsy + radiation, surgery alone, or surgery + palliative radiation). Patients were considered to have a favorable prognosis (n = 39) if they were optimally treated and had a Karnofsky Performance Status (KPS) score of at least 70. RESULTS: The median survival for patients according to RTOG groups IV, V, and VI was 9.2, 6.6, and 3.1 months, respectively (log-rank, p < 0.0004). The median overall survival was 5.3 months. The definitive radiation group (n = 58) had a median survival of 7.3 months compared to 4.5 months in the palliative radiation group (n = 19) and 1.2 months in the biopsy-alone group (p < 0.0001). Optimally treated patients had a median survival of 7.4 months compared to 2.4 months in those suboptimally treated (p < 0.0001). The favorable prognosis group had an 8.4-month median survival compared to 2.4 months in the unfavorable group (p < 0.0001). On multivariate analysis, the KPS, RTOG group, favorable/unfavorable prognosis, and optimal treatment/suboptimal treatment were significant predictors of survival. CONCLUSION: Elderly patients with good performance status (> or = 70 KPS) when treated aggressively with maximal resection and definitive radiation had longer survival than those treated with palliative radiation and biopsy. Aggressive treatment in such patients should be considered.     

The predictive value of intraoperative somatosensory evoked potential monitoring: review of 244 procedures

INTRODUCTION: There is some controversy regarding the value of intraoperative neurophysiological monitoring in predicting postoperative neurological deficits. We discuss our experience with the use of intraoperative somatosensory evoked potentials (SSEPs) during surgery of cranial base tumors. METHODS: We retrospectively reviewed all of the procedures that had been performed for the resection of cranial base tumors from July 29, 1993, through March 16, 1995. One hundred ninety-three consecutive patients had undergone a total of 244 procedures. SSEP waveforms were classified as follows: Type I, no change; Type II, change that reverts to baseline; Type III, change that does not revert to baseline; and Type IV, complete flattening of the SSEP waveform without improvement. Two patients had no waveforms from the beginning of the case (Type V) and were excluded from further analysis. New immediate postoperative neurological deficits were recorded. RESULTS: There were 64 male and 129 female patients, with a mean age of 46.6 years. One hundred seventy-seven patients had Type I SSEP waveforms, 13 of whom had postoperative deficits (7%). Fifty-six patients had Type II SSEPs, and nine (16%) of them had postoperative neurological deficits. Six patients had Type III SSEPs, and three had Type IV SSEPs, all of whom (100%) had postoperative deficits. There was a correlation between SSEP type and the results of the postoperative neurological examinations. The positive predictive value is 100%, and the negative predictive value is 90%. Although a change in the waveform that did not revert to baseline (Types III and IV) always predicted a postoperative deficit, a normal waveform did not always rule out postoperative deficits. Pathological abnormality, vessel encasement, vessel narrowing, degree of cavernous sinus involvement, brain stem edema, middle fossa location, final amount of resection, age, and tumor size correlated with a high predictive value of SSEP monitoring on univariate analysis (P < 0.05). None of these variables correlated significantly on multivariate analysis (P > 0.05), although brain stem edema was close (P = 0.0571). CONCLUSION: Intraoperative SSEPs have a high positive predictive value during surgery for cranial base tumors, but they do not detect all postoperative deficits.     

Pancreatic resection for pancreatic carcinoma combined with neo- and adjuvant locoregional targeting immuno-chemotherapy--a prospective randomized study

BACKGROUND/AIMS: The effects on early and late results of combined pancreatic resection with neo- and adjuvant immuno-chemotherapy for patients undergoing pancreatic resection are prospectively evaluated. METHODOLOGY: A group of 26 patients was divided into two groups, which were matched in terms of age-sex ratio, stage of disease, histological diagnosis and mode of pancreatic resection. Group A patients received a multimodality therapy, combining pancreatic resection with neo- and adjuvant locoregional targeting immunochemotherapy. Group B received pancreatic surgery only. RESULTS: For Group A patients (n = 14pts), a complete response was seen in 11 patients with a time interval ranging from 9 to 29 months. In the remaining 3 patients liver secondaries developed 12 months after pancreatic resection in 2 patients and the other patient developed pulmonary metastases 22 months after pancreatic resection. All patients (n = 3pts) are alive, but continue to have the disease. For Group B patients (n = 12pts), a complete response was seen in 3 patients with a survival of 9, 10 and 20 months following pancreatic resection. Six patients died due to locoregional recurrence of the disease, with the survival rate ranging from 7 to 18 months (mean 10 months). Locoregional recurrence was complicated with liver secondaries (n = 3) and with peritoneal dissemination of the disease in a further 3 patients. The remaining 3 patients are alive, but continue to have the disease due to locoregional recurrence. CONCLUSIONS: Patients in whom neo- and adjuvant locoregional immunochemotherapy was carried out in conjunction with pancreatic resection, demonstrated a significantly higher response rate to treatment. Therefore the data collected offers clear evidence, regarding the usefulness, applicability and safety of combined neo- and adjuvant therapy with pancreatic resection. A Multi-modality approach is therefore highly recommended.     

Early operative intervention after thrombolytic therapy for primary subclavian vein thrombosis: an effective treatment approach

PURPOSE: Effective treatment of primary subclavian vein thrombosis focuses on restoration of venous patency, relief of intrinsic stenosis, and decompression of the thoracic inlet. The appropriate method and timing for surgery, however, have not been not well defined. We conducted a study to determine an acceptable treatment approach. METHODS: A retrospective review evaluated 11 patients seen at our institution in an 8-year period. Seven patients were male and four were female, with an average age of 30 years (range 15 to 54 years). Two patients who had symptomatic stenosis without occlusion were omitted from the study. All patients with occlusion received urokinase therapy and underwent surgical decompression within 5 days of thrombolytic therapy. Five percutaneous transluminal angioplasties were attempted before operative intervention. Eleven decompressions were performed, including nine first-rib resections and two scalenectomies. Five operative venous procedures, consisting of thrombectomy with patch closure (n = 3) and bypass (n = 2), and seven venolysis procedures were performed. All patients received coumadin for 3 to 6 months after the operation. RESULTS: Urokinase therapy established wide venous patency in nine of the 11 extremities treated, with the remaining two requiring thrombectomy for residual thrombus at the time of operation. One patient who underwent transluminal angioplasty before the operation had rethrombosis, and the remaining four showed no improvement in venous stenosis after the intervention. Eight of nine extremities treated by first-rib resection and one of two treated by scalenectomy were free of residual symptoms at follow-up. CONCLUSIONS: Preoperative use of percutaneous balloon angioplasty is ineffective and should be avoided in this setting. Surgical intervention within days of thrombolysis obviates the need for interim oral anticoagulation and enables patients to return to normal activity sooner.     

Long-term stability of the mucogingival complex following guided tissue regeneration in gingival recession defects

The purpose of the present study was to evaluate the stability of soft tissue conditions in gingival recession defects treated with guided tissue regeneration (GTR). The study population was selected among those patients who had been treated with GTR procedures for Miller's class I or II, deep (> or =3 mm), buccal gingival recession defects. Defects were included only when they had revealed recession depth reduction > or =2 mm and root coverage > or =60% at 6 months following GTR treatment. These defects were regarded as successfully treated and scheduled for further monitoring. 20 patients, 11 male and 9 female, aged 23 to 57 years (mean age: 33.2 years), each contributing 1 defect, were selected. 9 patients were smokers (> or =10 cigarette per day). Recession depth (RD), probing depth (PD), clinical attachment level (CAL), and width of keratinized gingiva (KG) were assessed immediately before surgery, at 6 months post-surgery (baseline examination), and at 4 years post-surgery (4-year examination). At baseline examination, RD reduction was 3.6+/-0.9 mm (mean root coverage: 80%). CAL gain amounted to 4.2+/-1.3 mm, 60% of the defects showing CAL gain > or =4 mm. KG increased from 1.9+/-1.2 mm at presurgery examination to 3.1+/-0.9 mm at baseline examination. At 4-year examination, no significant changes from baseline RD, CAL and KG recordings were observed. Differences in baseline-4 year changes between smokers and non-smokers were not statistically significant. The results of the present study demonstrate that clinical outcome achieved following GTR procedure in gingival recession defects can be maintained over periods up to 4 years.     

Giant cranial base tumours

Thirty-three patients with giant (diameter > or = 4.5 cm) cranial base tumours who underwent surgery at the Hadassah Hospital over the last ten years are described. Twenty-three of the patients had meningiomas, 4 neurinomas, one giant cell tumour, one haemangiopericytoma, and 4 had malignant meningiomas. Four tumours were at the cerebellopontine angle, 9 within the anterior cranial fossa, 8 petroclival, 8 on middle fossa floor, and 4 along the sphenoid ridge. The average pre-operative symptom duration was 31 months, range 3-180 months. Nineteen patients had a radical tumour resection, 10 subtotal, and 4 a partial resection on an average 1.7 operations per patient. The mean follow-up period from the first operation was 39 months (range 2-120). There was no mortality peri-operatively or during the follow-up period. The mean pre-operative Karnofsky score was 68 and at the last follow-up 76. There was no correlation between histology and degree of resection, complications, or status at last follow-up. The best resections (92% radical) and outcome (mean Karnofsky 92) with the least number of operations (mean 1.4) were in the anterior fossa and along the sphenoid wing. The patients requiring the most operations (mean 2.1), having the smallest percentage of radical resections (25%) and the least favourable outcomes (mean Karnofsky 52) were those with petroclival tumours. Patients with giant cranial base tumours have a good overall long-term prognosis, but especially those with petroclival tumours challenge us to improve our techniques.     

Surgical management of non-small-cell lung cancer with ipsilateral mediastinal node metastasis (N2 disease)

Between 1979 and 1989, 876 patients with non-small-cell lung carcinoma were referred to our unit for surgical treatment. One hundred forty-six patients were judged not suitable for surgical treatment on clinical, radiologic, or bronchoscopic findings. Cervical mediastinoscopy or anterior mediastinotomy (or both) showed that 151 patients had mediastinal involvement by invasion or metastases into the ipsilateral (N2 disease) or contralateral (N3 disease) superior mediastinal lymph nodes and were therefore deemed inoperable. Except for one patient who had involvement of a single nodal station at mediastinoscopy, all other patients (n = 578) undergoing thoracotomy were thought, on the basis of computed tomographic scan or mediastinal exploration (or both) not to have N2 disease. Despite our efforts to avoid surgery on patients with N2 disease, at thoracotomy routine mediastinal node dissection disclosed that 149 patients had unsuspected N2 disease. Resection was possible in 130 (87.3%) by pneumonectomy (n = 72), bilobectomy (n = 7), lobectomy (n = 49), or lesser resection (n = 2). In three patients the resection was incomplete (2.3%), but in 127 a complete resection was performed (85%). Histologic examination in these 149 patients showed that 72 tumors were squamous cell carcinoma, 54 adenocarcinoma, 14 large-cell carcinoma, and 9 of mixed type. Eight patients died in the hospital after thoracotomy. Adjuvant therapy was not used after complete resection. Complete follow-up was obtained in 134 patients and the mean follow-up period was 27.25 months (1 to 116 months). The actuarial 5-year survival for those having complete resection was 20.1%. There was a statistically significant difference favoring long-term survival in those patients with squamous cell carcinoma (p < 0.01) and those in whom only one nodal station was involved (p < 0.05). Neither the extent of resection nor the involvement of any specific nodal station influenced long-term survival. Despite rigorous preoperative investigations, routine mediastinal node dissection demonstrated mediastinal node metastasis for the first time at thoracotomy in 26% of our patients. We believe resection is justified in these patients, who have already necessarily incurred the morbidity and mortality of thoracotomy, so long as complete resection is possible.     

Efficacy of combination therapy (hepatectomy and prophylactic arterial chemoinfusion) for liver metastases of colorectal cancer

From 1979 to 1997, 146 patients had hepatectomy for metastases of colorectal cancer (curative B: 122; curative C: 24). We categorized the severity of liver metastases as follows, H1: one lobe; H2: bilateral but less than five, and H3: bilateral with five or more lesions. In H1 and H2 patients, we compared the survival rate after resection alone (including repeat hepatectomy) with that after combination therapy (resection and prophylactic arterial chemoinfusion of 12-24 g of 5-FU). In H1 patients, the 3-year survival rate of the resected group (n = 74) and combination group (n = 6) was 47.2 and 53.3, respectively. In H2 patients, the resected group (n = 16) and combination group (n = 7) had survival rates of 34.5 and 100%, respectively. In H1 cases, the 3-year recurrence rate in the remnant liver was 63.4 versus 16.7% and in H2 cases it was 58.0 versus 0%. H3 patients received one week of continuous prophylactic arterial chemoinfusion [total dose of 5-FU = 6 g]. All four patients in the H3 combination group are alive at 20, 13, 13, and 12 months after resection, while the median survival of the resection only group (n = 4) was 12.5 months. We suggest that our combination therapy may be applicable to all patients with liver metastases of colorectal cancer.     

Microwave coagulation therapy for hepatocellular carcinoma

BACKGROUND & AIMS: Surgical resection is not always feasible in patients with hepatocellular carcinoma. Microwave coagulation therapy has been used as an alternative to resection, and its efficacy has been evaluated. METHODS: Nineteen patients with unresectable hepatocellular carcinoma underwent microwave coagulation therapy through laparotomy (n = 12), laparoscopy (n = 5), or thoracotomy (n = 2) because of advanced liver cirrhosis and/or intrahepatic metastases. One nodule was treated in 13 patients, tumor size ranged from 5 to 90 mm. Patient outcomes were studied. RESULTS: Microwave coagulation therapy created a reproducible regional necrosis. Fourteen patients underwent potentially curative treatment; the remaining 5 patients underwent palliative treatment (n = 4) or incomplete tumor coagulation (n = 1). Of the 31 nodules treated, 28 underwent complete tumor ablation. Only 2 patients undergoing laparoscopic microwave coagulation therapy developed local recurrence. The coagulated area subsequently shrank. Patients showed rapid recovery without hepatic dysfunction. Thirteen patients, including 2 long-term survivors, are alive either without tumor (n = 10; 14-64 months) or with tumor (n = 3; 17-22 months). Six patients died of hepatocellular carcinoma (n = 4) or liver insufficiency (n = 2). CONCLUSIONS: This preliminary study suggests the efficacy of microwave coagulation therapy, including safety and potential curability, in patients with hepatocellular carcinoma with advanced liver cirrhosis and multifocal or central tumors.     

Unruptured aneurysms presenting with mass effect symptoms: response to endosaccular treatment with Guglielmi detachable coils. Part I. Symptoms of cranial nerve dysfunction

OBJECT: Embolization of intracranial aneurysms by using Guglielmi detachable coils (GDCs) is proving to be a safe method of protecting aneurysms from rupture. Occasionally, patients with unruptured intracranial aneurysms present with symptoms related to the aneurysm's mass effect on either the brain parenchyma or cranial nerves. In the present study, the authors conducted a retrospective review to evaluate the response to GDC embolization in a series of 19 patients presenting with cranial nerve dysfunction due to mass effect. METHODS: Aneurysms were classified by size, shape, wall calcification, and amount of intraluminal thrombus. Patients were classified by duration of symptoms prior to GDC treatment (range < 1 month to > 10 years). Clinical assessment was performed within days of the GDC procedure and at later follow-up appointments (range 1-70 months, mean 24 months). In the immediate post-GDC period, four patients experienced worsening of cranial nerve deficits. Two of the four patients had transient worsening of visual acuity, which later improved to better than baseline status. Another patient who had presented with headache and seventh and eighth cranial nerve deficits from a vertebrobasilar junction aneurysm had improvement in these symptoms, but developed a new diplopia. The fourth patient had worsening of her visual acuity, which had not resolved at the 1-month follow-up examination; this patient later underwent surgical decompression. CONCLUSIONS: On late follow-up review, the response was classified as complete resolution of symptoms in six patients (32%), improvement in eight patients (42%), no significant change in four patients (21%), and symptom worsening in one patient (5%). Patients with smaller aneurysms and those with shorter pretreatment duration of symptoms were more likely to experience an improvement in their symptoms following GDC treatment, although statistical significance was not reached in this series (p=0.603 and p=0.111, respectively). The presence of aneurysmal wall calcification (six patients) or intraluminal thrombus (12 patients) showed no correlation with the response of mass effect symptoms in these patients.     

Influence of bacterial overgrowth and intestinal inflammation on duration of parenteral nutrition in children with short bowel syndrome

OBJECTIVES: Massive intestinal resection results in short bowel syndrome and necessitates prolonged parenteral feeding. The purpose of this work was to assess the impact of late complications of short bowel syndrome, including intestinal bacterial overgrowth and enterocolitis, on the duration of parenteral nutrition (PN) in comparison with factors evident in the neonatal period. METHODS: Retrospective chart review. RESULTS: Of 49 children, 42 were weaned from parenteral nutrition after a treatment course of 17 +/- 14 months. In these 42, postresection small intestinal length equaled 81 +/- 65 cm; 45% had an ileocecal valve. Small intestinal length in the seven children who were PN dependent was 31 +/- 30 cm (p < 0.05); none had an ileocecal valve (p < 0.05). Bacterial overgrowth occurred in all seven PN-dependent children and in 23 of 42 children eventually weaned from PN (p < 0.05). When bacterial overgrowth was identified before weaning (n = 12), the duration pf PN was 28 +/- 17 months, but when bacterial overgrowth was first identified only after weaning (n = 11), the duration of PN was 16 +/- 13 months (p < 0.05). Small intestinal inflammation correlated with bacterial overgrowth (r = 0.69). Those children with severe enteritis identified before weaning remained on the PN regimen for 36 +/- 15 months, in comparison with 21 +/- 14 months in those with mild enteritis and 13 +/- 11 months in those without inflammation (p < 0.02). CONCLUSIONS: Although the length of small intestine remaining after resection is the best immediate predictor of final success in terminating PN in children with short bowel syndrome, PN is prolonged by bacterial overgrowth and associated enteritis in those who will ultimately be weaned.     

Synchronous lingual granular cell tumor and squamous carcinoma. A case report and review of the literature

PURPOSE: The aim of this article is to determine the outcome of the pelvic pouch after the occurrence of a fistula. MATERIALS AND METHODS: From 1983 to 1995, 1,040 pelvic pouch surgeries were done at our institution. We reviewed the records of all patients with pouch-related fistulas. Data were collected from chart reviews and our pouch registry. RESULTS: Among 59 patients (22 males) with fistulas, mean age was 33 (range, 19-57) years. Preoperative diagnosis was mucosal ulcerative colitis (n = 52), indeterminate colitis (n = 6), and familial polyposis (n = 1). Site of fistulas included pouch/vaginal (n = 24), pouch/ cutaneous (n = 11), pouch/perineal (n = 16), and pouch/ presacral (n = 8). Postoperative diagnosis was mucosal ulcerative colitis (n = 40), Crohn's disease (n = 14), indeterminate colitis (n = 4), and familial polyposis (n = 1). One hundred eleven (range, 1-7) surgeries for treatment were performed. At a mean follow-up of 26 (range, 1-121) months, 19 pouches (32 percent) had been excised, 34 patients had functioning pouches and no fistula, 5 patients had a closed fistula but refused ileostomy closure, and 1-patient had died of unrelated causes (but the fistula was closed). Pouch type and preoperative diagnosis did not statistically affect pouch failure rates (P = 0.43 and 0.10. respectively). CONCLUSION: Successful treatment of fistula from a pelvic pouch can be achieved in more than 60 percent of patients. However, multiple procedures may be needed for a successful outcome. Ultimately, 32 percent had their pouches excised.     

Modifications of erythropoiesis in myelodysplastic syndromes treated with recombinant erythropoietin as evaluated by soluble transferrin receptor, high fluorescence reticulocytes and hypochromic erythrocytes

BACKGROUND: The aim of this study was to evaluate changes in erythropoiesis induced in vivo by recombinant erythropoietin (r-EPO) treatment in myelodysplastic syndromes (MDS), by means of some new, non invasive laboratory parameters. PATIENTS AND METHODS: Serum levels of soluble transferrin receptor (STR), a marker of total marrow erythroid activity, and automated detection of high fluorescence reticulocytes (HFR) and hypochromic erythrocytes (HE) (respectively, indexes of effective erythropoiesis and functional iron deficiency) were longitudinally measured in 25 MDS patients treated with r-EPO, and then correlated with conventional clinical and laboratory features. RESULTS: Stimulation of erythropoiesis was documented in 8 patients, whose serum STR levels showed a significant, early (within 16 days) increase during treatment with r-EPO. However, only 3 of these patients demonstrated a concomitant rise in HFR, and these were the only subjects who experienced a significant clinical response. Two of these patients also developed a functional iron deficiency while on treatment, as documented by an increase in HE, despite normal serum iron, transferrin saturation and even very high levels of ferritin. They needed iron supplementation to maintain the response to r-EPO. No variation in STR, HFR or HE occurred in the remaining 17 unresponsive patients during at least two months of treatment. Serum levels of thymidine kinase, as aspecific marker of cellular proliferative activity, paralleled those of STR. No correlation was found between STR, HFR or HE and serum levels of endogenous EPO, hemoglobin or transfusion requirements in MDS patients. CONCLUSIONS: These findings suggest that there is a heterogeneous and complex pattern of erythroid response in MDS patients treated with r-EPO. In addition, our results indicate that STR, HFR and HE may provide useful information for the clinical management of these patients.     

Long-term results of thoracoscopic resection of lung cysts

The treatment modality of spontaneous pneumothorax is extended by the introduction of an endoscopically applicable linear stapler. During the last 2 years 35 resections of cysts in 33 patients (24 men, 9 women, age median mean = 34 years) were started thoracoscopically in our hospital. Indications were: First pneumothorax (n = 15), recurrent pneumothorax (n = 16) and prophylactic resections (n = 4). A switch to open thoracotomy was necessary in 4 cases (11%) because of interpleural adhesions or large bullae. The median operation time was mean = 90 min. (range 60-240), the postoperative hospital stay mean = 8 days (range 4-25). Early complications occurred in 2 cases: One hematothorax, which was treated thoracoscopically, and one recurrent pneumothorax at the ninth postoperative day, which was treated by a chest drain. The follow up investigation 2 to 24 months (median mean = 6) after therapy was complete in 28 cases. It revealed that only 2 late recurrences (7%) occurred after 4 and 6 months. One was treated thoracoscopically again, the other one by thoracotomy. The endoscopically treated patients had less complaints than patients after thoracotomy. Only 4 patients complained of sensitivity in the scars due to weather changes. In conclusion the minimal-invasive resection of lung parenchyma represents an effective alternative to open thoracotomy with a much better quality of life, a low rate of complications and a comparable rate of recurrences.