Intra‐operative perforation is an important predictor of local recurrence and impaired survival after abdominoperineal resection for rectal cancer

Aim Abdominoperineal resection for rectal cancer is associated with higher rates of local recurrence and poorer survival than anterior resection. The aim of this study was to evaluate the outcome of conventional abdominoperineal resection in a large national series. Method The study was based on the Danish National Colorectal Cancer Database and included patients treated with abdominoperineal resection between 1 May 2001 and 31 December 2006. Follow up in the departments was supplemented with vital status in the Civil Registration System. The analysis included actuarial local and distant recurrence, and overall and cancer‐specific survival. Risk factors for local recurrence, distant metastases, overall survival and cancer‐specific survival were identified using multivariate analyses. Results A total of 1125 patients were followed up for a median of 57 (25–93) months. Intra‐operative perforation was reported in 108 (10%) patients. The cumulative 5‐year local recurrence rate was 11% [95% confidence interval (CI), 7–13)], overall survival was 56% (95% CI, 53–60) and cancer‐specific survival was 68% (95% CI, 65–71). Multivariate analysis showed that perforation, tumour stage and nonradical surgery were independent risk factors for local recurrence; tumour fixation to other organs, perforation and tumour stage were independent risk factors for distant metastases; and risk factors for impaired overall survival and cancer‐specific survival were age, tumour perforation, tumour stage, lymph node metastases and nonradical surgery. Conclusion Intra‐operative perforation is a major risk factor for local and distant recurrence and survival and therefore should be avoided.     

EXTREMITY SOFT TISSUE SARCOMA: FACTORS PREDICTIVE OF LOCAL RECURRENCE AND SURVIVAL

Background : To identify risk factors for local recurrence and overall survival in patients with extremity soft tissue sarcoma. Methods : A retrospective study was performed of all patients with extremity soft tissue sarcoma treated at the Combined Surgical Oncology Clinic in the Institute of Oncology at Prince of Wales Hospital between 1972 and 1992. Variables analysed included clinical presentation, patient characteristics, tumour characteristics, treatment factors and outcome. Results : One hundred and nineteen patients were eligible for the study. The most common type of presentation was with a painless mass, usually in the thigh. Local control rates at 5 and 10 years were 75% and 73%. Local control was higher in patients who had more radical surgery and in those who received adjuvant radiotherapy. Tumour size and high grade were independent risk factors for poorer survival. Patients over 50 had poorer survival than younger patients and those who presented with recurrent tumours also tended to have poor survival compared to patients presenting de novo. The respective 5- and 10-year survival rates were 65% and 62%. Conclusion : This study suggests that local control of extremity soft tissue sarcoma is improved by radical surgery and by the addition of radiotherapy when more conservative procedures are used. Overall survival appeared to be largely determined by patient (age, recurrent presentation) and tumour characteristics (grade, size).     

Extremity soft tissue sarcoma: factors predictive of local recurrence and survival.

BACKGROUND: To identify risk factors for local recurrence and overall survival in patients with extremity soft tissue sarcoma. METHODS: A retrospective study was performed of all patients with extremity soft tissue sarcoma treated at the Combined Surgical Oncology Clinic in the Institute of Oncology at Prince of Wales Hospital between 1972 and 1992. Variables analysed included clinical presentation, patient characteristics, tumour characteristics, treatment factors and outcome. RESULTS: One hundred and nineteen patients were eligible for the study. The most common type of presentation was with a painless mass, usually in the thigh. Local control rates at 5 and 10 years were 75% and 73%. Local control was higher in patients who had more radical surgery and in those who received adjuvant radiotherapy. Tumour size and high grade were independent risk factors for poorer survival. Patients over 50 had poorer survival than younger patients and those who presented with recurrent tumours also tended to have poor survival compared to patients presenting de novo. The respective 5- and 10-year survival rates were 65% and 62%. CONCLUSION: This study suggests that local control of extremity soft tissue sarcoma is improved by radical surgery and by the addition of radiotherapy when more conservative procedures are used. Overall survival appeared to be largely determined by patient (age, recurrent presentation) and tumour characteristics (grade, size).     

Surgical procedures and prognostic factors for local recurrence of soft tissue sarcomas

Background

Patients with local recurrence of soft tissue sarcomas are predisposed to future recurrences because treatment is challenging and complicated by prior therapy. This study investigated clinical outcomes following surgical procedures for locally recurrent soft tissue sarcomas and risk factors for re-recurrence and metastasis.

Methods

A retrospective analysis was conducted with 105 patients (52 males, 53 females) who underwent surgical procedures for local recurrence without distant metastasis of soft tissue sarcoma between 1987 and 2009. Patient follow-up ranged from 1 to 12 years (mean 4.9 years).

Results

Overall 5- and 10-year survival rates were 83.4 and 67.7 %, respectively. Twenty-one patients (20.0 %) had additional local recurrences, and 23 (21.9 %) had distant metastases. Amputation rate was 10.5 % at the time of surgical procedures and 17.1 % at final follow-up. Locations deep within muscles in the upper limb or trunk and surgical margins <1 cm wide were risk factors for further local recurrence. Locations deep within muscles, tumor sizes >10 cm, high-grade malignancy, and local recurrence after radical surgery were risk factors for distant metastasis.

Conclusions

Surgical margin and location were independent prognostic factors for local control, and a wider margin was especially important for recurrent tumors located in the trunk and upper extremity. For high-grade sarcomas with local recurrence after radical surgery, new approaches are needed to prevent distant metastases.     

Oncological outcome of T1 rectal cancer undergoing standard resection and local excision

Aim We studied the outcome and prognostic factors for T1 rectal cancer patients undergoing standard resection or transanal excision. Method One hundred and twenty‐four patients with T1 rectal cancer were included in the study, of whom 66 (53.2%) underwent standard resection and 58 (46.8%) underwent transanal excision. Survival analysis was performed to compare the outcome. Results The 5‐year local recurrence rate was 11.0% in the transanal excision group versus 1.6% in the standard resection group (P = 0.031) but the 5‐year disease‐free survival and overall survival rates were not significantly different between the two groups. Multivariate analysis suggested that a high tumour grade and perineural or lymphovascular invasion were independent risk factors for local recurrence and recurrence‐free survival. For high‐risk patients (with at least one of the above risk factors), the 5‐year local recurrence and 10‐year recurrence‐free survival rates were 21.2% and 74.5%, versus 1.2% and 92.0% in low‐risk patients (P = 0.00003 and P = 0.003). In patients undergoing transanal excision, none in the low‐risk group had local recurrence during follow up, while 40% (6 of 15) of patients in the high‐risk group developed local recurrence within 5 years after surgery. The 5‐year local recurrence rate was 45.0%. Conclusion Transanal excision in T1 rectal cancer may result in a high rate of local failure for patients with a high‐grade tumour, or perineural or lymphovascular invasion. Local excision should be avoided as a curative treatment in high‐risk patients.     

Malignant peripheral nerve sheath tumours--report of 8 cases and review of the literature.

BACKGROUND: Though Malignant peripheral nerve sheath tumours (MPNST) are a rare entity accounting for 5-10% of soft-tissue sarcomas they are an important differential diagnosis to benign tumours of the peripheral nervous system regarding treatment and prognosis. METHOD: We present our experience with eight patients who underwent surgery for MPNST at the Department of Neurosurgery between 10/1990 and 9/1999. The median age was 37 years [range: 13-64], the male/female ratio was 1:1. Two patients suffered from Neurofibromatosis type 1. Paraffin embedded tumour specimens were immunohistochemically stained for S-100, p53 and Ki67/MIB-1. FINDINGS: The most frequent initial symptoms were local swelling and pain followed by irritation of cranial nerves and spinal ataxia. Four tumours were localised at the head & neck region, three were found in the extremities and one tumour was located on the trunk. All patients underwent surgery with curative intent, but total resection, defined by negative surgical margins, was achieved in only 3 cases. All of these developed local recurrence with a mean disease free survival time of 10.6 months. In five cases, adjuvant radiation was given. During follow up, three patients developed distant metastases located in the lung, liver and subcutaneous tissue. Five out of eight patients died during follow-up with a mean survival time of 11.6 months after diagnosis. Results of immunohistochemical staining were as followed: S-100 (7/8), p53 (7/8). The Ki67/MIB-1 proliferation index was detectable in all tumour samples, it differed from 10-30%. INTERPRETATION: MPNST is a rare and fatal diagnosis in neurosurgery with high risk of local recurrence and occurence of distant metastases. Though mulitimodal therapy including surgical resection and adjuvant radiotherapy including brachytherapy is available, the prognosis remains dismal. Modern clinical studies and the development of effective chemotherapy is needed in order to gain control of the disease.     

Growth patterns of lung metastases from sarcoma: prognostic and surgical implications from histology

OBJECTIVES Pulmonary metastasectomy is firmly established in the multidisciplinary management of patients with advanced sarcomas. While the number of metastases, completeness of resection, disease-free interval and grading of the primary sarcoma are well established prognostic factors in metastatic surgery, histological parameters are not widely evaluated. The objective of the present study was to evaluate the prognostic impact of intrapulmonary growth patterns of sarcoma metastases. METHODS We retrospectively analysed the clinicopathological characteristics of 52 sarcoma patients who underwent surgical resection of lung metastases at our centre from January 2006 to January 2009. The histological growth characteristics of all 261 metastases have been categorized and published previously. 'Interstitial growth' was defined as a diffuse spread of the sarcoma cells into the alveolar septae at the transition of the metastasis to the normal lung tissue and was found to be prognostic. 'Pleural penetration' was defined as the infiltration and destruction of all visceral pleural layers by the tumour and was found to be a risk factor for local recurrence. RESULTS The median post-metastasectomy overall survival was 50.3 months, and the corresponding 5-year survival rate was 44.7%. Age >55 years at metastasectomy (P?=?0.02), the presence of interstitial growth (P?=?0.008), size of the largest metastasis >35?mm (P?=?0.023) and the presence of tumour recurrence at any site after metastasectomy (P?5?mm were found to be risk factors for local intrapulmonary recurrence. CONCLUSIONS Interstitial tumour growth, which is easily detected by light microscopy, can serve as a strong predictor of survival following pulmonary metastasectomy in sarcoma patients. Obvious pleural infiltration indicates the need for larger margins.     

The effect of an unplanned excision of a soft-tissue sarcoma on prognosis

We investigated whether our policy of routine re-excision of the tumour bed after an unplanned excision of a soft-tissue sarcoma was justified. Between April 1982 and December 2005, 2201 patients were referred to our hospital with the diagnosis of soft-tissue sarcoma, of whom 402 (18%) had undergone an unplanned excision elsewhere. A total of 363 (16.5%) were included in this study. Each patient was routinely restaged and the original histology was reviewed. Re-excision was undertaken in 316 (87%). We analysed the patient, tumour and treatment factors in relation to local control, metastasis and overall survival. Residual tumour was found in 188 patients (59%). There was thus no residual disease in 128 patients of whom 10% (13) went on to develop a local recurrence. In 149 patients (47%), the re-excision specimen contained residual tumour, but it had been widely excised. Local recurrence occurred in 30 of these patients (20%). In 39 patients (12%), residual tumour was present in a marginal resection specimen. Of these, 46% (18) developed a local recurrence. A final positive margin in a high-grade tumour had a 60% risk of local recurrence even with post-operative radiotherapy. Metastases developed in 24% (86). The overall survival was 77% at five years. Survival was related to the grade of the tumour and the finding of residual tumour at the time of re-excision. We concluded that our policy of routine re-excision after unplanned excision of soft-tissue sarcoma was justified in view of the high risk of finding residual tumour.     

Treatment of medulloblastoma in children: Long-term results following surgery,radiotherapy and chemotherapy

Summary Forty-seven children with histologically confirmed medulloblastoma are considered. Forty-five cases were surgically treated by direct approach to the tumour, while two cases were treated only with a shunt. A shunt was inserted preoperatively in 14 cases, postoperatively in 4 cases. Surgical resection was total or subtotal in 52% of cases, partial in 35%, and limited to a biopsy in 13%. Radiation treatment to the entire neuraxis was done in 37 cases: 10 of these cases received additional chemotherapy—mostly with CCNU—as primary treatment for medulloblastoma.Ten patients died within 30 days after surgery. Twenty-two patients died months after treatment, mainly from tumour recurrence (19 cases). One patient was lost to follow-up. Thirteen patients are survivors from 10 months to 20 years after treatment. As a whole, the one year survival rate has been 67%, 3 year survival 43%, and 5 year survival 27%. Complications affecting prognosis have been presented by tumour recurrence and metastases. CSF shunting and lack of prophylactic irradiation to the cerebral hemispheres have been considered responsible for the high incidence of supratentorial metastases in our series.Factors influencing prognosis have been the extent of tumour resection and association of primary chemotherapy with radiotherapy. Within 3 years after surgery survival has been 52% in cases with total resection against 31% in cases with partial resection of tumour. As regards chemotherapy, 3 year survival has been 60% for patients with combined treatment (chemo- and radiotherapy) against 37% in patients with radiotherapy alone. It is concluded that the best results in children with medulloblastoma are achieved by a radical resection, associated with a combined primary treatment of radiotherapy and chemotherapy.     

Local recurrence of soft tissue sarcoma a risk factor for late metastases: 379 patients followed for 0.5-20 years

We performed a retrospective analysis of 379 adult patients treated for soft tissue sarcoma. None had metastasis at the time of diagnosis and all were treated surgically. Patients who developed metastatic disease before the local recurrence were excluded. The 8-year metastasis-free survival rate in the group of 261 patients with local tumor control was 0.72, compared to 0.67 in the 118 patients with local recurrence (P 0.2). Multiple regression analysis showed that high-grade malignancy and large tumor size were risk factors for metastases. Local recurrence was not a risk factor. However, when patients with small and/or low-grade tumors were analyzed separately, local recurrence emerged as a risk factor. In this group of patients, the 8-year survival rate was 0.87 for those with local control and 0.64 for those with local recurrence (P 0.004). Local recurrence appears to be a risk factor for the development of late metastases in patients who otherwise have a low risk of metastases.     

Breast Cancer Local Recurrence: Risk Factors and Prognostic Relevance of Early Time to Recurrence

BACKGROUND: Local recurrence occurs in 10%-20% of patients treated with breast-conserving surgery for stage I-II breast cancer. The aim of the present study was to investigate breast cancer local recurrence, potential risk factors, and prognostic impact. METHODS: A total of 503 patients treated with breast-conserving surgery were included in the study. All patients underwent axillary dissection and postoperative radiotherapy, and all patients had negative margins at pathological examination. Median follow-up was 82 months. Local recurrence was classified as early when it occurred within 2 years from surgery. The risk factors for local recurrence and overall survival were estimated by univariate and multivariate analyses. RESULTS: Forty-six cases (9.1%) of local recurrence were observed, 11 of which occurred within 24 months of surgery; the other 35, sometime later. Statistically significant risk factors for local recurrence were premenopausal status, peritumoral vascular invasion, multifocality, and absence of estrogen receptors. Independent negative prognostic factors for overall survival at 5 and 10 years were N stage, absence of estrogen receptors, and early time to recurrence. Overall survival at 10 years was 10.0% for patients with early recurrence, 87.5% for patients with late recurrence, and 87.9% for patients without recurrence. CONCLUSIONS: None of the studied clinicopathological characteristics alone is a determinant for the choice of surgical treatment. Younger patients treated with breast-conserving surgery should receive aggressive postsurgical treatment and should be followed with an intensive follow-up program when metastatic axillary lymph nodes, negative estrogen receptors, or peritumoral vascular invasion are present.     

Ewing's sarcoma of the femur: Prognosis in 69 patients treated by the CESS group

We reviewed the treatment outcome of 69 patients with Ewing's sarcoma of the femur. The patients received chemotherapy according to the CESS 81 (n 14), CESS 86 (n 43), and CESS 91P (n 12) protocols. The 10-year relapse-free survival rates were 36%, 65%, and 65% (p = 0.01). 68 patients received local treatment. The primary tumor was treated by surgery without radiotherapy in 28 patients; 1 developed a local recurrence and 7 metastases. 10 patients received radiotherapy alone; 4 developed metastases and 4 local recurrences and metastases. 30 cases had a combination of surgery and radiotherapy; 7 developed metastases and 1 a local recurrence and metastasis. The survival of patients after radiotherapy alone was worse than that of patients after surgery with/without radiotherapy (p = 0.005). Pathological fractures (n 16) did not influence the prognosis.     

Predictors of extrahepatic recurrence after resection of colorectal liver metastases

BACKGROUND: It is important to identify patients at high risk of extrahepatic recurrence after surgery for liver metastases, in order to maximize the survival benefit obtained by prophylactic regional chemotherapy. METHODS: Data from 68 patients who underwent resection of colorectal liver metastases but who did not receive hepatic arterial chemotherapy or intravenous systemic chemotherapy were collected. Twenty-two variables were examined by univariate and multivariate analyses to determine which factors were relevant to extrahepatic recurrence. A scoring system was developed that included the most relevant factors. RESULTS: The extrahepatic recurrence rate at 3 years after hepatectomy was 57.8 per cent. Three variables were independently associated with extrahepatic recurrence including raised serum level of carcinoembryonic antigen after hepatectomy (relative risk (RR) 5.4, P < 0.001), venous invasion of the primary tumour (RR 4.0, P = 0.001) and high-grade budding of the primary tumour (RR 3.1, P = 0.006). Patients with none of these risk factors had a 3-year extrahepatic recurrence rate of 7.1 per cent, compared with 61.6 per cent for those with one risk factor and 100 per cent for those with two or three risk factors. CONCLUSION: It was possible to identify patients at high risk of disease relapse at extrahepatic sites. This system might be used on an individual basis to select patients with colorectal liver metastases for regional chemotherapy or systemic chemotherapy after surgical intervention.     

CLINICAL MANAGEMENT AND TREATMENT OUTCOMES OF MERKEL CELL CARCINOMA

Background: Merkel cell carcinoma (MCC), first described in 1972, is an uncommon, highly malignant tumour of skin. Its aetiology is unknown although the tumour occurs most frequently in sun-exposed sites. This skin cancer is characterized by a high incidence of metastases, local and regional recurrence and has a high mortality. Few survival data beyond 3 years are available. Treatment strategies include wide local excision of the primary and lymph node dissection, as indicated together with adjuvant radiotherapy. The tumour has also been shown to be chemosensitive. Definitive treatment is inconclusive due to the relative rarity of this malignancy. Methods: A retrospective study was undertaken between the years 1985 and 1996 of 33 patients with MCC at Concord Repatriation General Hospital (CRGH). Demographic details were noted, together with the site and appearance of the primary lesion and the sites and date of metastases. The response and outcome to various modalities of treatment were documented. Results: There were 27 men and six women with an average age of 80 years (range: 60–86 years). The primary lesion in the present series most often resembled a basal cell carcinoma (BCC), with 53% occurring in the head and neck region. Twenty-seven patients (82%) developed metastastic disease, with an average interval of 13.4 months between diagnosis of the primary lesion and the development of metastases. The incidence of locoregional recurrence in the present series was 42%. Radiation combined with surgery achieved locoregional control in 15 of 19 patients with primary, regional or recurrent locoregional disease. Fourteen patients died of MCC and five others from unrelated causes. The 14 survivors have a mean survival of 54 months with six patients surviving more than 5 years. Conclusions: Local excision together with regional lymph node clearance as indicated, combined with adjuvant radiation treatment was associated with an improved survival. The role of chemotherapy remains unclear.     

Salivary gland carcinoma: independent prognostic factors for locoregional control, distant metastases, and overall survival: results of the Dutch head and neck oncology cooperative group

BACKGROUND: We analyzed the records of patients with malignant salivary gland tumors, as diagnosed in centers of the Dutch Head and Neck Oncology Cooperative Group, in search of independent prognostic factors for locoregional control, distant metastases, and overall survival. METHODS: In 565 patients, we analyzed general results and looked for the potential prognostic variables of age, sex, delay, clinical and pathologic T and N stage, site (332 parotid, 76 submandibular, 129 oral cavity, 28 pharynx/larynx), pain, facial weakness, clinical and pathologic skin involvement, histologic type (WHO 1972 classification), treatment, resection margins, spill, perineural and vascular invasion, number of neck nodes, and extranodal disease. The median follow-up period was 74 months; it was 99 months for patients who were alive on the last follow-up. RESULTS: The rates of local control, regional control, distant metastasis-free and overall survival after 10 years were, respectively, 78%, 87%, 67%, and 50%. In multivariable analysis, local control was predicted by clinical T-stage, bone invasion, site, resection margin, and treatment. Regional control depended on N stage, facial nerve paralysis, and treatment. The relative risk with surgery alone, compared to surgery plus postoperative radiotherapy, was 9.7 for local recurrence and 2.3 for regional recurrence. Distant metastases were independently correlated with T and N stage, sex, perineural invasion, histologic type, and clinical skin involvement. Overall survival depended on age, sex, T and pN stage, site, skin and bone invasion. CONCLUSIONS: Several prognostic factors for locoregional control, distant metastases, and overall survival were found. Postoperative radiotherapy was found to improve locoregional control.     

The TME trial after a median follow-up of 6 years: increased local control but no survival benefit in irradiated patients with resectable rectal carcinoma

OBJECTIVE: To investigate the efficacy of preoperative short-term radiotherapy in patients with mobile rectal cancer undergoing total mesorectal excision (TME) surgery. SUMMARY BACKGROUND DATA: Local recurrence is a major problem in rectal cancer treatment. Preoperative short-term radiotherapy has shown to improve local control and survival in combination with conventional surgery. The TME trial investigated the value of this regimen in combination with total mesorectal excision. Long-term results are reported after a median follow-up of 6 years. METHODS: One thousand eight hundred and sixty-one patients with resectable rectal cancer were randomized between TME preceded by 5 x 5 Gy or TME alone. No chemotherapy was allowed. There was no age limit. Surgery, radiotherapy, and pathologic examination were standardized. Primary endpoint was local control. RESULTS: Median follow-up of surviving patients was 6.1 year. Five-year local recurrence risk of patients undergoing a macroscopically complete local resection was 5.6% in case of preoperative radiotherapy compared with 10.9% in patients undergoing TME alone (P < 0.001). Overall survival at 5 years was 64.2% and 63.5%, respectively (P = 0.902). Subgroup analyses showed significant effect of radiotherapy in reducing local recurrence risk for patients with nodal involvement, for patients with lesions between 5 and 10 cm from the anal verge, and for patients with uninvolved circumferential resection margins. CONCLUSIONS: With increasing follow-up, there is a persisting overall effect of preoperative short-term radiotherapy on local control in patients with clinically resectable rectal cancer. However, there is no effect on overall survival. Since survival is mainly determined by distant metastases, efforts should be directed towards preventing systemic disease.     

Squamous cell carcinoma of the lip: a retrospective review of the Peter MacCallum Cancer Institute experience 1979-88

BACKGROUND: Squamous cell carcinoma (SCC) of the lower lip is a common malignancy in Australia. Surgical excision and/or radiotherapy are used in treatment, and are regarded as equally effective. METHODS: A retrospective review of 323 patients treated at the Peter MacCallum Cancer Institute with either surgical excision and/or radiotherapy, evaluated disease recurrence, cause-specific mortality, and the incidence of metachronous lesions. RESULTS: Recurrence-free survival at 10 years was estimated to be 92.5%, and cause-specific survival at 10 years was estimated to be 98.0%. Equivalent rates of local control were obtained with surgery and radiotherapy. Recurrence was related to tumour stage and differentiation. A high incidence of metachronous lesions was noted, 25 patients had a lesion prior to presentation and 33 patients developed second lip lesions during the study period. CONCLUSIONS: Squamous cell carcinoma of the lower lip is well treated with surgery or radiotherapy. The preferred treatment for most patients with SCC of the lower lip in the Australian population is surgical excision. This study has shown a significant incidence of metachronous lip neoplasia, except in those patients whose whole lip had been resurfaced.     

Base of tongue carcinoma: Patterns of failure and predictors of recurrence after surgery alone

Between January 1971 and December 1986, 55 patients with squamous cell carcinoma of the tongue base underwent complete surgical resection with curative intent. No preoperative or postoperative adjuvant therapy was administered. The study group consisted of 41 men and 14 women (median age 61 years). All patients were followed until death (39 patients) or for a median of 9.4 years. Local control at 5 years was 74%. No predictors of local recurrence were discovered. Control in the dissected neck at 5 years was 68%. Control of cancer above the clavicles at 5 years was 48%. Distant metastases developed in 14% of the patients by 5 years. Cause-specific survival at 5 years was 65%. A Cox multivariate regression analysis revealed that pathologic N stage was the only significant independent predictor of recurrence in the dissected neck, recurrence above the clavicles, and cause-specific survival. The 5-year overall survival was 55%. Surgical mortality was 4%.     

Malignant epithelial parotid tumours

Formal parotidectomy was performed in 120 patients by one surgeon over 7 years. Eighteen patients were referred with malignant salivary tumours (eight recurrent). Low-grade tumours (LGT) were treated by surgery alone; untreated high-grade tumours (HGT) were treated by pre- or post-operative radiotherapy according to clinical findings. The aim of surgery was to obtain tumour clearance, to preserve the facial nerve where possible, and to perform radical neck dissection for HGT when peroperative jugulodigastric lymph node biopsy confirmed metastasis. Five patients (all HGT) with complete facial palsy due to tumour underwent radical parotidectomy; of the remainder, only one suffered further deterioration of facial nerve function after surgery. After 5 years median follow-up from presentation, four patients with HGT have died from metastases; one has developed local recurrence. No patient with LGT has died or developed local recurrence. The survival difference between patients with HGT and LGT is statistically significant (P less than 0.05). A logical management policy for malignant parotid tumours requires knowledge of tumour grade.     

Malignant Peripheral Nerve Sheath Tumours – Report of 8 Cases and Review of the Literature

Summary  Background. Though Malignant peripheral nerve sheath tumours (MPNST) are a rare entity accounting for 5–10% of soft-tissue sarcomas they are an important differential diagnosis to benign tumours of the peripheral nervous system regarding treatment and prognosis.  Method. We present our experience with eight patients who underwent surgery for MPNST at the Department of Neurosurgery between 10/1990 and 9/1999. The median age was 37 years [range: 13–64], the male/female ratio was 1:1. Two patients suffered from Neurofibromatosis type 1. Paraffin embedded tumour specimens were immunohistochemically stained for S-100, p53 and Ki67/MIB-1.  Findings. The most frequent initial symptoms were local swelling and pain followed by irritation of cranial nerves and spinal ataxia. Four tumours were localised at the head & neck region, three were found in the extremities and one tumour was located on the trunk. All patients underwent surgery with curative intent, but total resection, defined by negative surgical margins, was achieved in only 3 cases. All of these developed local recurrence with a mean disease free survival time of 10,6 months. In five cases, adjuvant radiation was given. During follow up, three patients developed distant metastases located in the lung, liver and subcutaneous tissue. Five out of eight patients died during follow-up with a mean survival time of 11,6 months after diagnosis.  Results of immunohistochemical staining were as followed: S-100 (7/8), p53 (7/8). The Ki67/MIB-1 proliferation index was detectable in all tumour samples, it differed from 10–30%.  Interpretation. MPNST is a rare and fatal diagnosis in neurosurgery with high risk of local recurrence and occurence of distant metastases. Though mulitimodal therapy including surgical resection and adjuvant radiotherapy including brachytherapy is available, the prognosis remains dismal. Modern clinical studies and the development of effective chemotherapy is needed in order to gain control of the disease.