Incomplete Tumor Resection Research Articles

Characterization of hemorrhagic complications after surgery for temporal lobe epilepsy.

To assess the significance of symptomatic hemorrhagic complications occurring after different temporal resections for temporal lobe epilepsy (TLE) and to compare this data to findings with postoperative hematomas after temporal surgery for mostly glial or metastatic tumors. Patient charts were retrospectively reviewed for 442 patients who underwent surgery for refractory TLE between 1995 and 2000. Procedures were 247 transsylvian amygdalohippocampectomies (AH), 40 transcortical AH, 57 anterior temporal lobectomies (ATL), 23 lesionectomies plus AH, and 75 lesionectomies without AH. All patients with delayed awakening or new neurological deficits due to hemorrhages were included in the study. An identical procedure was performed to detect symptomatic hemorrhages after 208 procedures for temporal tumor resection during the same time period. Symptomatic postoperative hemorrhages were found in 17 patients (3.8 %) undergoing epilepsy surgery, while the incidence was 3.0 % in a group with space-occupying temporal tumors (six patients). Hemorrhages showed a characteristic distribution after epilepsy surgery: in eight patients they were located remote from the site of surgery in the upper cerebellar vermis and foliae. Five typical hemorrhages associated with dysphasia were found in the left frontal operculum, only three patients had hematomas in the resection cavity, and one was located epidurally. Two patients had more than one location of hemorrhage. Transsylvian AH and ATL had a similar risk for postoperative hemorrhage, whereas none was found after lateral lesionectomies or transcortical AH. Intraoperative manipulations were associated with opercular hemorrhages; the only predisposing factor for resection site hematomas was older age, whereas cerebellar hemorrhages were associated with cerebrospinal fluid (CSF) loss during AH and ATL. There was no mortality in the TLE group, and 0.75 % permanent mild deficits. Seizure outcome did not differ from the rest of the group (82.5 % satisfactory seizure control). In contrast, all intraaxial hematomas after tumor surgery (N = 4, incidence 1.9 %) were located in or adjacent to the resection cavity. Prognosis was much worse with parenchymal hemorrhages after tumor surgery: three of four patients died, one survived with a severe hemiparesis, only two patients with extraaxial hematomas (incidence 1 %) had a complete recovery. The 3 % incidence of symptomatic hemorrhages was only insignificantly lower compared to the TLE group, patients with tumor surgery were older than TLE patients (49 versus 33 years), and in five of six patients only incomplete tumor resection was achieved. Although associated with a low permanent morbidity, features of postoperative hemorrhages after TLE surgery are characteristically different to complications after surgery for other indications, which has to be kept in mind for patient counseling and obtaining informed consent.

Surveillance neuroimaging of intracranial medulloblastoma in children: how effective, how often, and for how long?

The goal of this paper was to review brain and spine images obtained in children with medulloblastomas to determine the risk factors for tumor recurrence and to assess the impact of surveillance imaging on patient outcomes among patients who remain alive 1 month postsurgery. Imaging studies and clinical data obtained in children with medulloblastomas, who presented between January 1987 and August 1998, were retrospectively reviewed. Images were termed surveillance if they were follow-up studies and symptom prompted if they were obtained to investigate new symptoms. One hundred seven patients (mean age 6 years and 3 months, range 2 months-15 years and 6 months) were entered into the study. Fifty-three children experienced tumor recurrence; 41 had one recurrence, nine had two, and three had three recurrences. Surveillance imaging revealed 10 of the first 53 recurrences and 15 of all 68 recurrences. When the first recurrence was identified by the emergence of symptoms (42 patients), the children tended to survive for a shorter time (hazard ratio 3.72, 95% confidence interval 1.42-9.76, p = 0.008) than children in whom the first recurrence was detected before symptoms occurred (10 patients). The median survival time following symptomatic tumor recurrence was 4 months and that after surveillance-detected tumor recurrence was 17 months. The median increased survival time among patients whose recurrence was asymptomatic and identified by imaging studies was 13 months, more than half the mean time between surveillance imaging sessions. Incomplete tumor resection was associated with a significantly reduced time to recurrence (p = 0.048) and to death (p = 0.002). The number of recurrences that were experienced was associated with a reduced time to death (p < 0.001). Surveillance imaging is associated with an increase in survival in children with medulloblastomas. More frequent surveillance imaging in children with incomplete tumor excision and recurrent disease may further improve the length of survival.

Surgical Management of Advanced Malignant Adrenal Tumours

Background: Advanced adrenal carcinomas tend to infiltrate the adrenal vein and to grow intraluminally into the inferior vena cava, thereby diminishing or occluding the venous flow. Less often, tumour thrombi extend into the right atrium. Radical surgery requires caval or cavo-atrial thrombectomy while maintaining vascular control, complemented by multivisceral resections, if applicable. Methods: A review of the literature — undertaken in orcer to describe surgical techniques as well as postoperative results in the treatment of organ-exceeding adrenal cancer — and own experiences are the basis of management recommendations. Results: The extent of vascular involvement of adrenal neoplasms is classified into four different types, which require increasingly challenging surgical procedures. Low perioperative mortality and long tumour-free survival rates are reported in malignant phaeochromocytomas, adrenocortical malignancies, and other primary adrenal neoplasms such as leiomyosarcoma. Conclusions: Nowadays, advanced disease does not preclude the patient from radical surgical therapy in adrenal malignancies. Advances in vascular surgery and extracorporeal bypass techniques have led to the development of safe techniques for the removal of tumour thrombi, even of the retrohepatic and subdiaphragmatic vena cava. The poor prognosis of patients without resection or with incomplete tumour resections justifies an aggressive surgical approach.

Prognostic factors influencing surgical management and outcome of gastrointestinal stromal tumours

The purpose of this study was to review surgical experience with gastrointestinal stromal tumours (GISTs) at a single tertiary university hospital, and to identify morphological and genetic prognostic markers of tumour progression. Forty-eight GISTs from 39 patients were reviewed retrospectively. The prognostic significance of DNA copy number changes, measured by comparative genomic hybridization (CGH), and morphological markers in low-risk and high-risk tumours were investigated. Significantly more patients died from disease after incomplete tumour resection than after complete primary resection (P = 0.020). Tumour size of 5 cm or greater, mitotic count of 2 or more, and proliferative activity greater than 10 per cent were significantly associated with a shorter recurrence-free survival (P = 0.020, P = 0.001 and P = 0.002 respectively). Patients with low-risk tumours had a significantly better outcome than those with high-risk GISTs, both in terms of overall and recurrence-free survival (P < or = 0.001). CGH performed on 16 tumours revealed fewer DNA sequence copy number changes in low-risk than in high-risk GISTs. Non-progressive GISTs contained significantly fewer genetic alterations than recurrent or metastatic tumours (P < 0.001). Only tumours with more than five changes showed disease progression. Complete surgical resection is the most important means of cure for GISTs. DNA copy number changes are related to the behaviour of these tumours and may serve as additional prognostic markers.

Thoracolumbopelvic stabilization for the treatment of instability caused by recurrent myxopapillary ependymoma.

The authors report a patient with recurrent lumbosacral myxopapillary ependymoma, followed for more than 20 years, who presented with severe axial pain resulting from osteolytic destruction at the lumbosacral junction. Because the patient had a long history of paraplegia despite three previous incomplete tumor resections, we chose not to attempt a fourth resection. Moreover, because viable fixation points were not present within the sacrum and most of the lumbar spine, instrumented fusion was extended from T7 to the ilia using a modified Galveston L-rod technique, which we believe to be unique in its application to this problem. This case demonstrates the long-term potential for instability from locally destructive myxopapillary ependymoma that is incompletely excised. We are not aware of any previous reports of lumbopelvic instability in association with myxopapillary ependymomas.

Clinical outcome of endoscopic aspiration mucosectomy for early stage gastric cancer

Background: Endoscopic mucosal resection is an established treatment option for early stage gastric cancer. However, several problems with endoscopic mucosal resection remain to be solved, such as appropriate treatment for recurrence and incomplete tumor resection. The outcome for patients undergoing endoscopic aspiration mucosectomy (endoscopic mucosal resection) by a modification of the cap-fitted technique was evaluated retrospectively to determine factors associated with complete resection and tumor recurrence. Methods: Endoscopic mucosal resection was performed in 106 patients with early stage gastric cancers up to 20 mm in diameter that were well or moderately differentiated adenocarcinoma. All were superficial lesions without ulceration, distinct signs of submucosal invasion, or a poorly demarcated border. En bloc (tumors <10 mm in diameter) or piecemeal (tumors 10-20 mm in diameter) resection was performed. Follow-up endoscopy was performed at 2, 6, 12, 18, and 24 months and thereafter once per year. Outcome and factors associated with complete resection and tumor recurrence were assessed retrospectively. Results: Sixty-eight patients (64%) underwent en bloc resection and 38 (36%) piecemeal resection. The mean longest dimension (SD) of the resected lesions was significantly greater after piecemeal resection (12.3 [4.0] mm) than after en bloc resection (7.6 [4.0] mm; p < 0.01). In patients with tumors completely resected, there was no recurrence after either en bloc or piecemeal resection. Six of 8 patients found to have submucosal invasion after endoscopic mucosal resection underwent surgery. Patients with incompletely resected intramucosal lesions underwent additional endoscopic treatment. Cancer recurred in 3 patients (2.8%), all of whom had lesions measuring more than 15 mm in diameter. Conclusions: Endoscopic mucosal resection is safe and useful for the management of early stage gastric cancer. Further improvement in outcome requires more accurate preoperative diagnosis and postoperative histopathologic evaluation. Patients with incompletely resected lesions should undergo aggressive additional treatment. (Gastrointest Endosc 2002;56:708-13.)

The influence of preoperative chemotherapy and surgical technique in the treatment of hepatoblastoma--a report from the German Cooperative Liver Tumour Studies HB 89 and HB 94.

Despite the success of chemotherapy in the treatment of hepatoblastoma (HB), the complete surgical resection of the primary liver tumour and metastases is the most important factor for survival. From 1989 to 1998, 141 children with HB were treated in the German Cooperative Paediatric Liver Tumour Study HB 89 and HB 94. The study determines the results of surgical strategy, which adapts the procedure at the initial operation to the tumour extension in the liver and the occurrence of metastases. The median follow-up of the survivors was 72 months (range 24 - 132 months). 98/141 (78 %) patients were alive and 31/141 (22 %) died. 12/141 (8.5 %) children had no surgical treatment. A complete resection of the primary tumour was achieved in 107/129 (83 %) cases. Forty-eight children were primary resected and eighty-one children were operated on after initial chemotherapy. In 36 cases, an atypical tumour resection, in 90 cases an anatomical liver resection, was performed. Three children were transplanted. There was no perioperative death. 14/48 (30 %) children with primary tumour resection had microscopical or macroscopical residual tumour in the liver. Despite the larger number of advanced HB in the primary chemotherapy group, an incomplete tumour resection was performed in only 15/78 (19 %) cases after liver resection (p < 0.044). 14/36 (38 %) cases with atypical tumour resection and only 16/90 (18 %) cases with anatomical liver resection had residual tumours in the liver (p < 0.019). These results underline the necessity for preoperative chemotherapy in all HB, which was accepted in the following study HB 99. Atypical tumour resection should be avoided because of the higher rate of incomplete tumour resections and local relapse compared to the group with anatomical tumour resection.

How accurate is diagnostic imaging in determination of size and multifocality of renal cell carcinoma as a prerequisite for nephron-sparing surgery?

Background: The indication for elective nephron-sparing surgery (NSS) in renal cell carcinoma (RCC) is under discussion in the urological literature. The main problem of NSS is the multifocality of RCC. The presented study was preformed to assess the accuracy of pre-and intraoperative ultrasound (US), and computerized tomography (CT) in determination of tumor size and detection of multifocal lesions. Materials and methods: Tumor size was measured by preoperative US and CT and compared with the tumor diameters in gross sections of the neoplastic kidneys. Multifocality was determined by 3-mm step sectioning of the nephrectomy specimen, and the results were correlated with preoperative US and CT on the one hand, and the ex situ sonography of the nephrectomized kidney on the other hand. Results: US and CT show similar results in the determination of the tumor size. In only 22.9%, preoperative US and CT were able to detect multifocal tumors. Ex situ sonography had a sensitivity of 40.0% and a specificity of 87.2% in this regard. Conclusions: In preparation for nephron-sparing surgery of renal cell carcinoma, neither preoperative routine imaging, nor intraoperative ultrasound can safely predict multifocal lesions of renal cell carcinoma. Commentary Local tumor recurrence following nephron-sparing surgery (NSS) for renal cell carcinoma (RCC) may be due to incomplete resection of the primary tumor, occult multicentric disease or the development of a new primary or metastatic focus of RCC in the renal remnant. The risk of multicentric disease in RCC has been evaluated and debated extensively in the literature. RCC generally occurs as a discrete focal lesion rather than an infiltrative process which is seen in carcinoma of the prostate. At issue is whether the molecular events that give rise to malignant transformation affect a discrete segment of the kidney or a broader segment of the renal tubular epithelium. A high incidence of multicentric RCC has been reported in patients with germ line mutations such as those that exist in von Hippel Lindau disease and other forms of hereditary RCC suggesting a global predisposition to malignant degeneration throughout the entire renal parenchyma. The incidence of multicentricity in sporadic RCC has been less clear. Emerging cytogenetic and molecular data suggest that satellite lesions may occasionally arise from the same malignant clone as their corresponding primary lesion and may therefore represent biologically significant intra-renal metastasises. A recent review of published studies comprising over 1100 cases of sporadic RCC indicated an aggregate incidence of 15.2% of tumor multifocality (range 6.5%–28%)[1]. It is important to remember that these studies represent a diverse group of patients and that RCC is in fact a heterogenous group of tumors. The risk of multicentricity is not equal in all patients and appears to be related to other prognostic variables such as tumor histology, stage and grade. For example, papillary RCC is known to be associated with a higher incidence of multifocality than the more common clear cell variant. The risk of multifocal disease also increases with larger tumors, particularly those that extend beyond the renal capsule (pT3+). Finally, some microfocal tumors are of unknown biological significance such as the finding of satellite adenomas. Of importance when considering relative indications for elective NSS is the incidence of multifocality when the primary or index tumor is ⩽ 4 cm. A recent review of the literature indicated that the incidence of multifocality in this setting is approximately 5%. The most worrisome implication of multifocal RCC is that this will predispose to an increased risk of local tumor recurrence following NSS. Although this potential risk must be considered, the relationship between multifocality and local recurrence is neither linear or predictable as suggested by the low overall local recurrence rates reported following NSS in several large series. In nearly 1800 cases of NSS reported in the literature to date, the risk of local tumor recurrence has ranged from 0–10% and is clearly lowest among patients undergoing elective NSS for small (⩽ 4 cm) low stage lesions [1]. The true biologic significance of multicentric renal tumors and their implication for NSS therefore remain to be fully elucidated. [1] Uzzo RG, Novick AC. Nephron-sparing surgery for renal tumors: indications, techniques and outcomes. J Urol 2001;166:6–18. Andrew C. Novick, M.D.

Analysis of experimental liver metastasis from tumors inoculated in the mouse stomach cavity.

This study was conducted to develop a patient-like hematogenous metastatic model of gastric-cancer in order to gain an understanding of the tumor biology and to search for new methods of treatment. We established a natural and easily reproducible liver metastasis model by orthotopic gastric inoculation in Balb/c mice, using the syngeneic tumor, colon 26. This model allowed us to evaluate the effect of partial gastric resection with excision of tumor nodules on the formation of experimental liver metastases from the stomach cavity. Mice given partial gastrectomy showed less metastatic ability than control mice. In these experimental groups, liver metastasis was observed in the only group of mice that died of local tumor regrowth due to incomplete resection of the primary tumor. It is suggested that a period of at least 10 days is required for the formation of liver metastases after tumor inoculation into the stomach cavity. There was no significant increase in the number of liver metastases following splenectomy, or after the administration of anti-asialo GM1 antibody or silica. This experimental model of liver metastases will provide a useful means of understanding tumor biology and the regulation of liver metastases by host immunocompetent cells, and for assessing new therapeutic agents.

Malignant minor pleural effusion detected on thoracotomy for patients with non–small cell lung cancer: is tumor resection beneficial for prognosis?

Background. This study attempts to clarify the benefit of surgery for non–small cell lung cancer (NSCLC) with malignant minor pleural effusion that is detected at thoracotomy. Methods. Records of surgical patients with NSCLC were reviewed, with a definition of minor pleural effusion as less than 300 mL. The patients were divided into three groups as follows: (1) group C consisted of patients who underwent grossly complete resection; group I, patients with incomplete tumor resection; and group E, patients who underwent exploratory thoracotomy only. Results. There were 196 patients who had minor pleural effusion; of these, 96 (46%) underwent an examination to define the malignancy status of pleural effusion after surgery. In 43 patients (45%), the effusion was found to be malignant. The median survival time and 5-year survival rate, respectively, were 13 months and 9% for group C (n = 11); 34 months and 10% for group I (n = 14; p = 0.3); and 17 months and 0% for group E (n = 18; p = 0.8). Conclusions. Tumor resection is not beneficial for the survival of patients with NSCLC who have a minor malignant pleural effusion.

Intracranial ganglioglioma: preoperative characteristics and oncologic outcome after surgery.

In order to investigate the clinical characteristics, neuroimaging findings, pathological features, treatment, and outcomes, and to provide valuable guidance for the diagnosis and management of intracranial gangliogliomas, 34 cases of intracranial gangliogliomas were retrospectively analyzed. This study included 23 males and 11 females. Age at operation ranged from 17 days to 50 years. All patients had preoperative magnetic resonance imaging (MRI). Computed tomography (CT), magnetic resonance spectroscopy (MRS), 18F-FDG-PET (positron emission tomography), and 99Tc-HMPAO-SPECT (single photon emission computed tomography) were also performed in some cases. All pathological specimens and all available neuroimages were re-evaluated. The follow-up period varied from 12 to 89 months (mean 36 months). Seizure was the most common presenting symptom (85%). Tumor calcification was detected by CT scan in six of 11 cases. Seventeen gangliogliomas (50%) showed cystic components and 18 tumors (53%) were enhanced on MRI. All temporal lobe gangliogliomas were located intracortically and most of them had poor demarcation on MRI. In comparison with the contralateral normal area, the gangliogliomas showed a reduced Cho/Cr and NAA/Cr ratio, and an increased Cho/NAA ratio. On 18F-FDG-PET, gangliogliomas were usually hypometabolic. HMPAO-SPECT indicated tumor hypoperfusion or isoperfusion. A gross total resection was achieved in 25 patients. Tumor progression was observed in three patients who underwent an incomplete tumor resection. Two of them underwent a malignant transformation to a glioblastoma. Twenty-seven patients could carry on their normal life activity with the Karnofsky Performance Scale (KPS) of more than 80. Even though ganglioglioma is a slowly growing benign tumor, which could be demonstrated by magnetic resonance spectroscopy (MRS), PET, and SPECT, there is a chance of malignant transformation, especially in cases of incomplete tumor resection. Gangliogliomas should be resected gross totally, if feasible, to achieve the best long-term outcomes.

Surgical treatment of pituitary tumors in the elderly: clinical outcome and long-term follow-up.

Pituitary adenomas in the elderly represent an increasing proportion of pituitary tumors, making the surgical management of these patients of special importance. We therefore decided to review our experience with transsphenoidal surgery (TSS) in this age group, in order to better evaluate its efficacy, safety and outcome. A retrospective study was performed on 39 patients aged more than 70 years at the time of surgery (mean age 74.1 +/- 2.9 years) and with a minimum follow-up duration of 4 years at the time of the study. Thirty-one had a nonfunctioning pituitary adenoma, 5 had a GH-secreting adenoma and 3 a macroprolactinoma, respectively. The commonest presenting symptom was visual deterioration (60%), whereas hypopituitarism were present in 41% of patients. All patients underwent TSS without any major peri- and post-operative complications. Visual fields improved in 74% of patients. Post-operative radiotherapy was performed in 17 patients with partial surgical resection and/or persistent hormonal hypersecretion, whereas 22 patients with subtotal or total surgical resection were treated by surgery only. Long-term follow-up (mean 9.1 +/- 2.7 years) showed evidence for tumor regrowth in one irradiated (5.9%) and 3 non-irradiated patients (13.6%), respectively. A noticeable complication of radiotherapy was progressive hypopituitarism, which worsened or developed in 65% of patients. We conclude that TSS is safe and well tolerated even in elderly patients, with a low incidence of minor complications. The indications for post-operative radiotherapy in patients with incomplete tumor resection should be better defined.

Results for microsurgical removal of tentorial meningiomas.

Growth patterns of tentorial meningiomas are related to the deep cerebral venous system and to cranial nerves IV-XI. Localization and surgical aggressiveness are decisive for the outcome to be expected. n = 25 patients (22 f, 3 m), aged from 26-77 (mean: 56.4) years underwent microsurgical removal of their tentorial meningioma. Tumor size was as follows: n = 11 < 3 cm, n = 6 3-5 cm, n = 8 > 5 cm. The median of the preoperative Karnofsky scores was 90. The operative approaches chosen were suboccipital in n = 14, subtemporal in n = 6, occasionally a combined supra- and infratentorial approach was chosen. Data regarding surgery, histology and postoperative course were available through the patient's charts and through outpatient clinic. n = 20 (80%) of the tumors were rated WHO grade I, n = 5 (20%) WHO grade II. Tumor removal according to Simpson was degrees I in n = 9 (36%), degrees II in n = 14 (56%), degrees III in n = 2 (8%). Mortality was 0%. In n = 6 patients (24%) neurological worsening, mainly due to transient cranial nerve deficits was noted. Surgical complications (CSF fistula, wound healing problems) occurred in n = 5 patients (20%). The median of the postoperative Karnofsky scores on last follow up was 90 after a median of 41.9 months. Two patients (8%), one of whom underwent reoperation developed tumor recurrency during follow up. Neurological deficits following microsurgical removal of tentorial meningiomas are transient in the majority of patients. The apparently high rate of incomplete tumor resection (app. 60% Simpson grades II and III) is due to the close topographical relationship of these tumors with important neurovascular structures. Thus, the operative strategy should not be excessively aggressive, but rather take into account the option to observe residual tumor or to apply additional stereotactic convergent beam radiation in selected cases.

Treatment of recurrent malignant sacrococcygeal germ cell tumors: analysis of 22 patients registered in the German protocols MAKEI 83/86, 89, and 96.

To evaluate therapeutic options for recurrent malignant sacrococcygeal germ cell tumors (GCT) following three-agent, cisplatinum-based, first-line chemotherapy and tumor resection. Twenty-two patients were evaluated in 22 first-, 14 second-, five third-, and two fourth-relapse situations. One patient, who relapsed with pure teratoma, was excluded from the analysis of adjuvant treatment. Seventeen patients presented with an isolated local recurrence, two patients showed a distant relapse, and three patients suffered from a combined local and distant recurrence. Twelve patients achieved complete remission (CR) after surgery (n = 12) and adjuvant platinum chemotherapy (n = 10). Seven of these patients remain in continuous CR, and five patients relapsed. All patients who achieved only a partial remission developed a second relapse. Three of 14 patients could be cured after a second (or further) relapse. Altogether, 10 patients survived disease free, and 12 patients died as a result of tumor progression (n = 11) or therapy-related complications (n = 1). The completeness of salvage surgery and clinical remission status after first salvage treatment were the most important prognostic parameters. In addition, patients in first or second relapse with locally advanced or poorly responding tumors benefited from preoperative chemotherapy in combination with regional hyperthermia (RHT). In some patients after microscopically incomplete resection, irradiation at doses > 45 Gy contributed to a favorable outcome. The complete resection of the local recurrence represents the cornerstone of salvage treatment. Preoperative platinum-based chemotherapy, combined with RHT in some patients, facilitates complete tumor resection. Radiotherapy should be reserved for those patients with microscopically incomplete tumor resection. As the chance of cure decreases with further relapses, it is important to establish a stringent therapeutic strategy to avoid significant treatment delays and, most importantly, insufficient local therapy.

Lymphatic vessel invasion is an independent prognostic factor in patients with a primary resected tumor with esophageal squamous cell carcinoma.

Little data exist about the prognostic role of a lymphatic vessel invasion (LVI) in patients with esophageal carcinoma. The objective of this study was to clarify the presence and prognostic impact of LVI in a large group of patients resected for esophageal squamous cell carcinoma (SCC) at one surgical center. Three hundred sixty-six patients, who had a primary resection for SCC, were analyzed by univariate and multivariate analysis. Follow-up was complete for 93.7% patients with a median follow-up of 8.3 years. The total rate of LVI was 39.1% (n = 143). Univariate analysis revealed a significant relation between LVI and different T classifications (P = 0.001), N classifications (P < 0.0001), M classifications (P < 0.0001), International Union Against Cancer (UICC) stages (P < 0.0001), and residual tumor (P < 0.0001). Multivariate analysis of the patients with R0-resected tumors proved LVI as an independent prognostic factor. The 2-, 5- and 10-year survival rates in patients with LVI were 28.5%, 11.1%, and 9.2% compared with 63.4%, 46.6%, and 27%, respectively, without LVI (P < 0.0001). Patients with LVI had a median survival time of 11.4 months compared with 28.6 months without LVI (P < 0.0001). Patients with R0-resected tumors without LVI had a median survival time of 54.1 months compared with 12.1 months in patients with LVI (P < 0.0001) and compared with 11.3 months in patients with R1-resected tumors P < 0.0001). These data clearly show that LVI is an independent prognostic factor in patients with SCC and confirm the importance of a systematic pathohistologic workup. The prognosis of patients with R0-resected tumors with LVI is equal to patients with an incomplete tumor resection. This supports the inclusion of LVI in the UICC classification system for esophageal carcinoma.

Hemangiopericytoma in pediatric ages

Hemangiopericytoma (HPC) is very uncommon in childhood and comprises two different clinical entities, the adult type and the infantile type, occurring in the first year of age. We report on a series of 27 pediatric patients treated from 1978 to 1999 by the Italian and German Soft Tissue Sarcoma Cooperative Group. Seven patients had infantile HPC; complete resection was achieved in the tumors of five patients and chemotherapy was given to four patients. Twenty children had adult type HPC; nine received complete tumor resection (four patients at diagnosis and five at delayed surgery). Post-operative radiotherapy was administered to 15 patients, chemotherapy to 19. Six of seven patients with infantile HPC were alive in first remission; one patient died of disease. Chemotherapy achieved an objective response in four of four patients. Among the adult type HPC cases, 5-year event free survival was 64% (median follow-up 125 months); 12 patients were alive in first remission, eight patients relapsed and died of disease. Seven of 10 evaluable patients showed good response to chemotherapy. Statistically significant differences in outcome were observed in relation to Intergroup Rhabdomyosarcoma Study grouping, size, local invasiveness, and gender. Infantile HPC is a unique entity probably related to infantile myofibroblastic lesions and characterized by a high response to chemotherapy, which is required in case of unresectable, life-threatening tumors. In children over 1 year of age, HPC behaves like its adult counterpart; complete surgical resection remains the mainstay of treatment, but chemotherapy and radiotherapy seem effective and are recommended in all patients with incomplete tumor resection and/or locally invasive, large tumors.

Radiotherapy of esthesioneuroblastoma

Purpose: Only 3% of all malignant intranasal tumors are esthesioneuroblastomas (ENB). As the tumor is very rare, the number of ENB treated in individual departments is small. In order to evaluate the efficacy of radiotherapy (RT), patients’ data of 2 centres were analysed with reference to new reports in literature. Methods and Materials: From 1981 to 1998, 17 patients with ENB, 8 men and 9 women aged between 6 and 81 years, were treated in the departments of radiotherapy of the universities of Cologne and Muenster. The tumors were Kadish Stage B in 4/17 patients and Stage C in 13/17 patients. Treatment included incomplete surgery and irradiation in 2/17 patients, adjuvant RT postoperatively in 6/17 patients, definitive RT in 7/17 patients and RT after incomplete surgery of recurrent tumors in 2/17 patients. Postoperatively, the median target dose of EBRT was 56 (range 50–60) Gy; for definitive RT it was 58 (range 40–70) Gy. Results: After a median follow-up period of 86 (range 2–208) months 10/17 patients showed no evidence of disease (NED). There were 6 patients treated with radical complete surgery plus postoperative irradiation and 5 of them were NED. There were 7 patients treated with only irradiation and 3 of those patients were NED. Of 2 patients with incomplete surgery and irradiation there was one patient NED. Of 2 patients with incomplete resection of recurrent tumor who received irradiation, there was one patient NED. 2 of the patients with NED died after 22 and 94 months respectively. 4/17 patients died as a result of local recurrence and 2/17 patients as a result of distant metastases (liver, brain). One patient with a recurrent tumor is alive. Median survival of all 17 patients was 94 months. Progressive disease after definitive RT occurred after a median of 11 months. Conclusions: Esthesioneuroblastomas are radiocurable tumors. In correlation to literature a primarily complete tumor resection followed by adjuvant RT (50–60 Gy) offers the best disease free survival.

Benign meningioma: Partially resected, biopsied, and recurrent intracranial tumors treated with combined proton and photon radiotherapy

Purpose/Objective: To evaluate the recurrence-free survival and complications of combined proton and photon radiotherapy of patients with incompletely resected or recurrent histologically-confirmed benign meningioma. Methods and Materials: Between May 1981 and November 1996, 46 patients with partially resected, biopsied, or recurrent meningiomas (median age of 50 years; range 11–74 years) were treated with combined photon and 160-MeV proton beam therapy at the Massachusetts General Hospital (MGH) and the Harvard Cyclotron Laboratory, using computed tomography-based conformal 3D treatment planning. Nine patients were treated after incomplete tumor resection, 8 patients after tumor biopsy only, and 29 patients after tumor recurrence following gross total (10/29 patients) or progression after subtotal (19/29 patients) resection. All patients were classified as benign meningioma on review slides at MGH. The median dose to the macroscopic gross tumor volume was 59.0 CGE (range 53.1–74.1 CGE, CGE = proton Gy × 1.1 RBE). The median follow-up was 53 months (range 12–207). Results: Overall survival at 5 and 10 years was 93 and 77%, respectively, and the recurrence-free rate at 5 and 10 years was 100% and 88%, respectively. Survival without severe toxicity was 80% at 5 and 10 years. Three patients presented with local tumor recurrence at 61, 95, and 125 months. One patient developed distant intradural metastasis at 21 and 88 months. No patient died from recurrent meningioma; however, 4 patients died of other causes. A fifth patient died from a brainstem necrosis after 22 months. Eight patients developed severe long-term toxicity from radiotherapy, including ophthalmologic (4 patients), neurologic (4 patients), and otologic (2 patients) complications. All patients with ophthalmologic toxicity received doses higher than those allowed for the optic nerve structures in the current protocol. Conclusion: Combined proton and photon radiotherapy is an effective treatment for patients with recurrent or incompletely resected benign intracranial menigiomas. Observed toxicity appears to be dose-related; with currently employed dose constraints, toxicity should not exceed that seen in patients treated with conformal fractionated supervoltage photon radiotherapy.

Adrenocortical tumors in children.

Childhood adrenocortical tumors (ACT) are rare. In the USA, only about 25 new cases occur each year. In Southern Brazil, however, approximately 10 times that many cases are diagnosed each year. Most cases occur in the contiguous states of São Paulo and Paraná. The cause of this higher rate has not been identified. Familial genetic predisposition to cancer (p53 mutations) and selected genetic syndromes (Beckwith-Wiedemann syndrome) have been associated with childhood ACT in general but not with the Brazilian counterpart. Most of the affected children are young girls with classic endocrine syndromes (virilizing and/or Cushing). Levels of urinary 17-ketosteroids and plasma dehydroepiandrosterone sulfate (DHEA-S), which are abnormal in approximately 90% of the cases, provide the pivotal clue to a diagnosis of ACT. Typical imaging findings of pediatric ACT consist of a large, well-defined suprarenal tumor containing calcifications with a thin capsule and central necrosis or hemorrhage. The pathologic classification of pediatric ACT is troublesome. Even an experienced pathologist can find it difficult to differentiate carcinoma from adenoma. Surgery is the single most important procedure in the successful treatment of ACT. The role of chemotherapy in the management of childhood ACT has not been established although occasional tumors are responsive to mitotane or cisplatin-containing regimens. Because of the heterogeneity and rarity of the disease, prognostic factors have been difficult to establish in pediatric ACT. Patients with incomplete tumor resection or with metastatic disease at diagnosis have a dismal prognosis. In patients with localized and completely resected tumors, the size of the tumor has predictive value. Patients with large tumors have a much higher relapse rate than those with small tumors.

Pelvic neuroblastoma: Low mortality and high morbidity

Background/Purpose: Cervical, thoracic, and pelvic neuroblastomas are regarded as having a better outcome than abdominal primaries. The aim of the study was to analyze the results of treatment of pelvic neuroblastomas in our institution. Methods: The authors reviewed the records of 284 patients with neuroblastoma treated in our hospital during the period 1983 through 1998 and identified 17 (6%) with pelvic tumors. The revised International Neuroblastoma Staging System was used. Results: There were 6 patients with stage 1 disease, 8 with stage 2, 2 with stage 3, and 1 with stage 4 disease. Intraspinal extension of the tumor was present in 7 patients (41%). Except for one child with stage 4 disease, all patients underwent an attempt of tumor excision, and 6 had a complete resection. All 7 patients with intraspinal tumor survived. Permanent postoperative neurological complications occurred in 6 patients (35%). These included sciatic nerve palsy, urinary and fecal incontinence, neuropathic bladder, and leg weakness or nerve root injury L4-S1. Three of 17 patients died, but 1 fatality was unrelated to the tumor. The overall survival rate was 82% and was not influenced by the completeness of tumor resection. Conclusions: The survival of nonmetastatic pelvic neuroblastoma in our institution is good despite incomplete tumor resection. Intraspinal extension is not a negative prognostic factor. Considering the high incidence of permanent neurological damage after surgery and the generally favorable biological characteristics of these tumors, surgical treatment should not be overaggressive. J Pediatr Surg 35:724-728. Copyright © 2000 by W.B. Saunders Company.