The management of retroperitoneal soft tissue sarcoma: a single institution experience with a review of the literature.

AIM: Ten percent of soft tissue sarcomas (STS) arise in the retroperitoneal tissues. The prognosis for patients with retroperitoneal sarcoma is poor with a 5-year survival rate between 12% and 70%. Stage at presentation, high histological grade, unresectable primary tumour and incomplete resection are associated with a less favourable outcome. METHODS: Complete follow-up data were available on 22 patients who underwent surgery for retroperitoneal STS in our institution between 1990 and 2000. Patient, tumour and treatment variables were analysed including use of adjuvant therapy and survival status. RESULTS: Eighteen patients underwent surgery for primary disease, four patients were treated for recurrent disease or metastases. Ten patients presented with pain, seven with an abdominal mass, other presentation included weight loss and haematuria. Thirteen patients presented with tumours larger than 10 cm. The tumours were seven liposarcomas, six leiomyosarcomas, three malignant fibrous histiocytomas, two rhabdomyosarcomas, two malignant schwannomas and two undifferentiated sarcomas. Six primary tumours were completely excised, five patients received radiotherapy and five received chemotherapy. Local recurrence rate was 45% and recurrence-free interval for 10 patients with recurrence was 11 months. Five patients received radiotherapy and five received chemotherapy. The median survival for patients with primary tumours was 36 months, and 5-year survival was 44%. Adjuvant therapy was not associated with higher survival rates. CONCLUSION: This study re-emphasizes the poor outcome of patients with retroperitoneal STS. Adjuvant radiotherapy and chemotherapy do not appear to be any proven benefit and the single most important prognostic factor is aggressive successful en bloc resection of the primary tumour. Our resection rate and 5-year survival rates are comparable with previous reported UK series although lower than large reports from North American centres. This might partly be explained by difficulty in data collection in a retrospective analysis, but may reflect inadequate subspecialization in UK centres. Copyright Harcourt Publishers Limited.     

The use of MRI in the diagnosis of benign and malignant bone and soft tissue tumours

Two hundred and thirty four suspected primary bone and soft tissue tumours were investigated using plain films and MRI. The MR appearance of 200 of these tumours was assessed with respect to the intensity of the lesion, the homogeneity of the tumour, the presence or absence of a capsule or lobulation, whether the tumour was whorled or not and whether it contained either fluid or blood. Apart from benign lipomas and some malignant myxoid liposarcomas, however, it seemed virtually impossible to tell one tumour from another and in many cases to differentiate a benign lesion from a malignant tumour using MRI alone. We recommend that the workup of a suspected soft tissue tumour should be initially by MR scanning and that the workup of a suspected malignant bone tumour should be plain films followed by an MRI scan.     

Major surgery in elderly head and neck cancer patients: immediate and long-term surgical results and complication rates

Background: Evaluation of immediate and long-term surgical results in major surgery for the head and neck tumours in elderly patients and identification of tumour and patient related factors that affect the incidence and severity of surgical and medical complications.Methods: Retrospective analysis of a series of 24 consecutive patients aged 70 and over with head and neck tumours undergoing extensive surgical resections with reconstruction with/without osseous and/or soft tissue transfer. Patients’ demographics and surgery and tumour related data were extracted from appropriate charts and recorded. Pre-existent comorbid conditions, immediate and long-term surgical and medical complications were analysed. Pre-existing comorbidities were graded and staged using the Comorbidity Data Collection Form. Postoperative surgical and medical complications were scored according to their severity.Results: Overall complication rate in present study was 63% and 54% of patients experienced clinically important surgical and/or medical complications. However, medium admission time remained at 16 days. Presence of advanced comorbidity, longer operative times and advanced stage of disease seemed to influence the development of surgical or medical complications.Conclusions: Major surgery for head and neck tumours is of great value even in elderly patients providing very good surgical results with acceptable complication rates. The choice of treatment modality should be based on all factors affecting the treatment outcomes. In particular, the presence of the co-existent underlying diseases should be assessed meticulously and in cases with clinically important comorbidities the surgical treatment stress should be reduced to minimum.     

Thallium-201 scintigraphy–a predictor of tumour necrosis in soft tissue sarcoma following preoperative radiotherapy?

AIM: Thallium-201 (Tl-201) scintigraphy in patients with malignant soft tissue tumours was evaluated to determine whether the images correlated with histological response to preoperative radiotherapy. METHODS: We studied 54 patients, median age 32 (range 17-84) years, with non-metastatic, malignant soft tissue tumours diagnosed between 1996 and 2001. Thirty-eight patients had unoperated tumours and 16 patients had previous incomplete excisions. All patients received preoperative radiotherapy followed by surgery. No patient received chemotherapy as part of their initial management. Qualitative analyses of early phase (30 min) and late phase (4 h) Tl-201 scintigraphic images before and after preoperative radiotherapy were compared with the degree of tumour necrosis determined histologically. RESULTS: In the previously unoperated group, all 38 patients had increased TL-201 uptake in the late phase of scanning prior to radiotherapy suggesting metabolically active tissue. In the previously excised group 11 patients had increased Tl-201 uptake in the late phase of scanning prior to radiotherapy. Following radiotherapy, patients with Tl-201 retention on late phase scans had a lower rate of necrosis than patients with minimal retention, p<0.0001. Following radiotherapy, 28 of 29 patients with minimal uptake on the late phase had 80% or more necrosis, while 24 of 25 patients with increased uptake on the late phase had less than 80% necrosis (p<0.0001). Patients with previously excised tumours who had thallium retention following radiotherapy demonstrated evidence of residual disease at surgery. All patients with incompletely excised tumours who had no thallium retention on late phase scanning after radiotherapy demonstrated no evidence of residual disease at surgery. CONCLUSION: Thallium scintigraphy is a readily available investigative tool, which when used in conjunction with other imaging modalities in the assessment of primary and incompletely excised malignant soft tissue tumours, may predict histological tumour response to preoperative radiotherapy.     

Salivary gland tumours—Analysis of 162 cases

The clinical records of 162 cases of salivary gland tumours were studied. Parotid gland was the commonest site for origin of tumour (110 cases.) Commonest benign tumour was pleomorphic adenoma (42 cases) and the commonest malignant tumour was mucoepidermoid carcinoma (26 out of 162 cases). The next common tumours were adenoidcystic and undifferentiated carcinoma which constituted 23 and 20 cases respectively. Surgery or surgery with radiotherapy were the main modalities of treatment for the primary tumours. Twenty patients had recurrence within three years after the treatment. These patients were treated with either revision surgery or external radiations. Chemotherapy was given to one patient of soft tissue sarcoma. Twenty nine patients had post-operative complications like facial paralysis, parotid fistula, skin necrosis and Frey’s syndrome.     

Surgical management of soft tissue sarcoma in patients over 80 years

AIMS: To report outcome on patients over 80years of age with soft tissue sarcoma (STS), with respect to surgical treatment, co-morbidity, complications and survival. METHODS: From a prospective database of 3400 patients with STS presenting over a 13-year period, all patients over 80years of age were identified and reviewed, with respect to tumour characteristics morbidity, mortality and outcome. RESULTS: 128 patients over 80years were treated for STS with 63 referred for treatment of primary disease, of whom 50 underwent resectional surgery. The remaining 65 patients were treated for recurrent or incompletely excised disease. Of the 50 patients treated primarily with surgery, 56% of tumours where high grade and 56% were greater than 10cm in diameter. The overall complication rate was 34%, with a 30-day mortality of 4%. Two- and 5-year survival rates were 56% and 46%, with a local recurrence rate of 22% at a mean follow-up of 22months. CONCLUSION: This patient group presented with poor prognosis tumours that were associated with poor outcomes in the medium to long term. Age need not be considered a contra-indication to radical surgery with curative intent.     

Osteosarcoma: What did we learn from the paediatric experience for adolescents and young adults?

The term ‘osteosarcoma’ (OS) defines a primary malignant tumour of bone, characterised by the production of osteoid tissue or immature bone by malignant proliferating sarcomatous cells. Due to the variable biological features of different osteosarcoma, several varieties are included in the osteosarcoma family, with different grades of malignancy (Table 1). Of these variables, high grade primary central os- teosarcoma is the most common form and accounts for more than 80% of cases. About 85% of these patients have tumours located in the extremities, and about 15–20% of patients are metastatic at diagnosis. This tumour represents 0.2% of all malignant tumours with an incidence of three new cases/year per million population, and the majority of cases are in children and adolescents younger than 20 years of age. There- fore, it is a very rare tumour and during the course of their activity, orthopaedic surgeons or medical oncologists see about one patient with this tumour every 5 years. Thus, the interest in osteosarcoma for these specialists is quite limited. From a cultural point of view, however, this is an important tumour: (1) for the oncologist, because most current strategies using adjuvant and neoadjuvant treatments in other more common tumours (for instance breast cancer) have been formulated on the basis of results obtained in osteosarcoma;(2)for the orthopaedic surgeon because some of the new methods of surgical reconstruction devised for osteosarcoma may also be used in other orthopaedic pathologies.The present report is limited to primary high grade osteosarcoma of the extremity and will consider separately different presentations (localised, metastatic at diagnosis, relapsed).     

Curative surgery for gastric cancer in the elderly: treatment decisions, surgical morbidity, mortality, prognosis and quality of life

AIMS AND BACKGROUND: Surgical risk is deemed to be higher in the aged population because there are often comorbidities that may affect the postoperative result. This consideration is important for the treatment decision-making for gastric cancer in the elderly. The aim of this study was to identify factors influencing mortality, morbidity, survival and quality of life after curative surgery for gastric cancer in patients aged 75 years and older, and to plan their appropriate management. METHODS AND STUDY DESIGN: From January 1993 to December 2004, 135 patients underwent surgery at our department because of gastric cancer. Ninety-four of these patients (69.6%) underwent potentially curative gastrectomy. A cross-sectional study of 23 patients aged 75 years and older and 71 younger patients who underwent curative gastrectomy was carried out: patient characteristics, tumor characteristics, management, morbidity, mortality, survival, and quality of life were evaluated. RESULTS: Elderly patients had significantly more comorbidities and a poorer nutritional status than younger patients. The surgical procedures were similar in both groups and the overall morbidity rate was 27.9% and the overall mortality rate 8.5%. Medical mortality was significantly higher in elderly patients, and the presence of comorbidities was the only independent factor affecting mortality. The 5-year survival rate was 56.2% in the older group versus 62.1% in the younger group and tumor stage was the only prognostic factor influencing survival. Quality of life after surgery was similar in both groups. The significantly better postoperative functional outcome after subtotal gastrectomy suggested a better compliance of elderly patients with subtotal than total gastrectomy. CONCLUSIONS: In the elderly, surgical strategies must be modulated on the basis of comorbidities, tumor stage and future quality of life. Since elderly patients have no worse prognosis than younger patients, age is not a contraindication to curative resection for gastric cancer. Subtotal gastrectomy should be the procedure of choice mainly in elderly patients as it offers better quality of life.     

Primary tumours and tumour-like conditions of the mandible

Reports on comprehensive mandibular tumours are very rare. The present article describes a total of 81 primary tumour and tumour-like lesions of the mandible seen at the University Hospital, Banaras Hindu University, during last 16 years. In the mandible the ratio of odonto-genic to nonodontogenic tumors is 1.6:1. Ameloblastomas constitute 56% of the odontogenic tumors. Among the benign mesenchymal tumours of the jaw 58% are neoplastic and 42% are nonneoplastic in which cementoostoid tissue is laid down secondary to inflammatory process. Over all 40 cases are benign 28 potentially malignant (ameloblastoma) and 13 are malignant lesions. Among the malignant mandibular tumours, bone tumours are more common than the soft tissue sarcomas. Microscopically mandibular osteosarcomas are better differentiated than similar tumours in the long bones. Local, segmental, or radical surgery seems to be the treatment of choice in the mandibular tumours.     

Merkel cell carcinoma: a clinicopathological study of 34 patients.

BACKGROUND: Merkel cell carcinoma (MCC) is a rare cutaneous malignant tumour. Its natural course is fast progression of the primary tumour and rapid regional metastasis. METHODS: The pathological archives of Helsinki University Hospital and Vaasa Central Hospital were reviewed from 1987 to 2001. Specimens were re-evaluated by two pathologists and size was measured from primary tumours. Multivariate analysis was performed for age, sex, primary tumour size, and method of initial surgery, development of metastatic disease and development of local recurrence. Overall survival (OS) was calculated by the Kaplan-Meier method. RESULTS: Of 34 patients, 12 male and 22 female patients, are reported. Their median age was 76.4 years. Half of the primaries were located in the head and neck region. All patients were treated surgically. The mean OS was 2.7 years by the Kaplan-Meier method. The 2-year survival rate was 65% and 5-year rate was 50%. The mean OS for tumour size >2 cm was 1.7 years and for tumour size 2 cm) and metastatic dissemination. A favourable prognosis seemed to correlate to more aggressive surgical management as use of primary excision with split skin graft or reconstruction with a local flap. CONCLUSION: MCC affects the elderly patients. In this study, female patients predominated. Primary tumour size >2 cm predicts poorer outcome of the disease. A wide surgical excision is recommended for the primary treatment of MCC.     

The evidence-based use of induction chemotherapy in Breast Cancer

The use of induction chemotherapy, also known as pre-operative or neoadjuvant chemotherapy has been studied in a number of randomised trials. Response rates are generally high, ranging from 70-90%, which allows for the downstaging of primary tumours and breast conservation surgery. Conserving surgery as opposed to mastectomy in this setting appears to be safe, effective and improves quality of life, although there is a suggestion of a small increase in local recurrence rate. Data from the randomised trials presented in this review shows that the use of induction chemotherapy neither prolongs nor decreases overall or disease free survival when compared with the same chemotherapy given in an adjuvant setting. There is a direct correlation between response of a primary breast tumour to induction chemotherapy and patient outcome, probably because this approximates the effect of induction chemotherapy on micrometastases which are present at presentation in many patients with apparently localised primary breast cancers. A pathological complete response after surgery is an excellent predictor of long term outcome, and is more likely to be seen in smaller tumours. Induction chemotherapy can be used as an in vivo measure of tumour response to treatment. This allows a further option of switching cytotoxic drugs in apparently resistant tumours.     

Therapeutic value of scapular and pelvic girdle disarticulations in sarcoma

The authors have reviewed 22 cases of proximal disarticulations with the aim of assessing the therapeutic value, taking into account previous radio- and chemotherapy. The following criteria were especially examined: recurrences, survival, quality of life. There were 13/22 soft tissue sarcomas, 9/22 bone sarcomas. In 10 instances, the tumour was primary and treated for the first time whilst, in 12 cases, it was recurrence. Eighteen patients had been previously treated by non radical surgery, 11 by radiotherapy and 10 by chemotherapy. For upper limb tumours, six patients underwent an inter-scapulo-thoracic disarticulation and three an inter-scapulo-thoracic resection according to Tykhor-Lindberg. For lower limb tumours, seven patients were submitted to inter-ilio-abdominal disarticulation, three to coxo-femoral disarticulation and one to internal hemipelvectomy according to Eilber. Mean disease free interval has been 34.5 months and mean survival 38.5 months. Three out of 20 evaluable patients (15%) recurred locally although most of them benefited from second surgery. Quality of life has been excellent in general despite the fact that only seven patients accepted wearing a prosthesis. Karnofsky index ranged between 60 and 100%. No significant difference was seen, whether or not previous radiotherapy and/or chemotherapy had been administered.     

Parathyroid carcinoma: problems in diagnosis and the need for radical surgery even in recurrent disease.

INTRODUCTION: From 1986 to 1999 we operated on 963 patients with primary hyperparathyroidism (pHPT). METHODS AND RESULTS: Parathyroid carcinoma was diagnosed clinically and histologically in four patients (0.4%). In two of these patients diagnosis of parathyroid cancer was delayed by misinterpretation of the histopathology leading to an autotransplantation of malignant parathyroid tissue in one case. In two patients multivisceral surgery was performed: beside thyroidectomy, neck dissection, tracheal wall resection and resection of the muscular layer of the oesophagus one patient received oesophagectomy and gastric transposition and one patient a lung wedge resection. Both patients had a temporary palliation of tumour-associated symptoms after multivisceral surgery. The first patient died 2 years after oesophagectomy and 12 years after primary diagnosis from local tumour recurrence and cachexia. The second patient is living with tumour recurrence presenting a serum calcium level of 4.2 mmol/l (normal range 2.0 to 2.5 mol/l) and multiple brown tumours 2 years after lung resection and 6 years after the primary diagnosis. CONCLUSIONS: We conclude that parathyroid carcinomas, being difficult to diagnose, warrant radical surgery, including multivisceral resection to prolong survival and reduce tumour and hypercalcaemia associated symptoms. Copyright Harcourt Publishers Limited.     

Clear-cell sarcoma of the soft tissue--a rare diagnosis with a fatal outcome

IPACH I., MITTAG F., KOPP H.‐G., KUNZE B., WOLF P. & KLUBA T. (2012) European Journal of Cancer Care 21 , 412–420 Clear‐cell sarcoma of the soft tissue – a rare diagnosis with a fatal outcome Clear‐cell sarcomas account for less than 1% of all soft tissue tumours. They most often occur in middle‐aged adults as a deeply located lesion with predilection to the tendons and aponeuroses. The aim of the present study was to show possible influencing factors on the outcome after surgical treatment in a detailed case series. We reviewed the medical records of 11 patients with the diagnosis of a clear‐cell sarcoma of the soft tissue. These cases were analysed with regard to age, gender, localisation, tumour size, recurrence free survival and overall survival. A minimum follow up of 12 months was achieved. The mean age at the point of diagnosis was 47.9 years. Metastases occurred after a mean of 19.2 months. In the cases with a tumour diameter >5 cm, metastases occurred earlier. When treated in a specialist centre, metastases occurred later. Patients died a mean of 18.4 months after developing metastatic disease. Patients with tumour size >5 cm at the point of primary diagnosis died earlier than patients with a tumour size <5 cm. It is important to detect clear‐cell sarcomas as soon as possible and the final surgical treatment should be performed in a centre familiar with the treatment of soft tissue tumours not only to prolong overall survival, but also to treat the patient in a multiprofessional team.     

Total femur replacement: Primary procedure for treatment of malignant tumours of the femur

We present our experience of treating patients with tumours involving the whole femur with excision and total femur endoprostheses over the last 30 years (1975–2005). There were 26 consecutive patients (14 men and 12 women). Average age was 40 years (14–82 years) at the time of surgery and 21 of the patients had primary malignant bone tumours with five having the procedure for metastases. 11 patients were still alive of which nine were free of disease at the time of review at a mean follow-up of 57 months (3–348). The overall patient survival at 10 years was 37%. The survival of patients with a primary localised tumour was 50% at 10 years.     

Human epidermal growth receptor-2 overexpressing early operable primary breast cancers in older (≥70 years) women: biology and clinical outcome in comparison with younger (<70 years) patients

IntroductionThere is dearth of literature reporting the prevalence and biological characteristics as well as the long-term clinical outcome of human epidermal growth factor receptor-2 (HER2) overexpressing tumours in older women. Currently, research involving trastuzumab at large focuses on the younger population. This study aimed to analyse their biological characteristics and to compare them with their younger counterparts from a single centre with a long-term clinical follow-up.MethodsOver 37 years (1973–2010), 1758 older (≥70 years) women with early operable (<5 cm) primary breast cancer were managed in a dedicated clinic and have complete clinical information available. Of these, 813 patients underwent primary surgery and 575 had good quality tumour samples available for tissue microarray analysis using indirect immunohistochemistry. Comparison was made with data from a well-characterised younger (<70 years) series (N = 1711) treated between 1986 and 1998 (before adjuvant trastuzumab became standard) in our institution. Forty five (7.6%) and 140 (8.2%) patients from the older and younger series, respectively, had HER2-positive tumours.ResultsHER2 overexpression was seen in 45 (7.6%) older women and 140 (8.2%) in younger patients (P = 0.56). HER2 overexpressing tumours in older women when compared with that in their younger counterparts were associated with low Ki67 and high bcl2 expression (P < 0.05). Only 26% of the younger patients and none of the older patients received adjuvant chemotherapy, and no patients at the time received trastuzumab. However, there was no significant difference in the outcome of the two age groups (5-year breast cancer-specific survival rate: <70 years = 65% versus >70 years = 70%, P = 0.51).ConclusionHER2 overexpressing tumours in older women showed relatively a less aggressive phenotype and did not show any inferior long-term clinical outcome despite not having received chemotherapy when compared with the younger patients. The precise role of different adjuvant systemic therapies in this population needs to be delineated.     

Prognostic Value of the Doubling Time of Serum C-reactive Protein and Alkaline Phosphatase Levels in Primary Bone and Soft Tissue Tumors

We investigated the clinical relevance of doubling time (DT) of serum laboratory data obtained in routine clinical examination of patients with primary bone and soft tissue tumors, in comparison with major clinical and pathological parameters (age at presentation, sex, tumor size, location, clinical stage and histologic grade) by uni- and multivariate analyses. In 64 patients with primary bone and soft tissue tumors (primary bone tumors: 39, primary soft tissue tumors: 25) and 68 cancer patients, the pretreatment DT values of serum C-reactive protein (CRP), alkaline phosphatase (ALP), lactate dehydrogenase (LDH), calcium (Ca), phosphate (P) levels were measured, as well as the erythrocyte sedimentation rate (ESR: mm/60 min); these values were then compared with overall survival, local recurrence-free survival and metastasis-free survival. Only DT of CRP and ALP (CRP-DT, ALP-DT) were found to be correlated with disease outcome in patients with primary bone and soft tissue tumors. In cancer patients, only CRP-DT showed a relation with clinical stage and histologic grade, but the ALP-DT in patients with bone metastasis was significantly shorter than that in patients with metastases at other sites or in those with no metastasis. Among all tumor patients, those with bone metastasis showed the shortest ALP-DT compared with those with lung, liver and brain metastasis. Univariate analysis showed that shorter CRP-DT and ALP-DT are associated with poor overall survival, and the development of local recurrence and metastasis. These findings suggest that pretreatment CRP- and ALP-DT could be additional prognostic parameters for disease outcome in patients with primary malignant bone and soft tissue tumors. However, in multivariate analysis, only ALP-DT, but not CRP-DT, was an independent prognostic parameter for these disease outcomes.     

The outcome of combined-modality therapy for stage III non-small-cell lung cancer in the elderly.

PURPOSE: The North Central Cancer Treatment Group performed a phase III trial to determine whether chemotherapy plus either bid radiation therapy (RT) or daily (qd) RT resulted in a better outcome for patients with stage III non-small-cell lung cancer (NSCLC). No difference in survival was identified between the two arms. This secondary analysis was performed to examine the relationship between patient age and outcome. PATIENTS AND METHODS: Two hundred forty-six patients were randomized to receive etoposide plus cisplatin and either RT qd or split-course RT bid. This retrospective study compared the outcomes of patients aged >/=70 years ("elderly patients") with those of younger individuals. Of the 244 assessable patients, 63 (26%) were elderly, and 181 (74%) were younger individuals. RESULTS: The 2-year and 5-year survival rates were 39% and 18%, respectively, in patients younger than 70 years, compared with 36% and 13%, respectively, in elderly patients (P =.4). Grade 4+ toxicity occurred in 62% of patients younger than 70 years compared with 81% of elderly patients (P =.007). Grade 4+ hematologic toxicity occurred in 56% of patients younger than 70 years, compared with 78% of elderly patients (P =.003). Grade 4+ pneumonitis occurred in 1% of those younger than 70 years, compared with 6% of elderly patients (P =.02). CONCLUSION: Toxicity, especially myelosuppression and pneumonitis, was more pronounced in the elderly patients receiving combined-modality therapy for locally advanced NSCLC. Despite increased toxicity, elderly patients have survival rates equivalent to younger individuals. Therefore, fit, elderly patients with locally advanced NSCLC should be encouraged to receive combined-modality therapy, preferably on clinical trials with cautious, judicious monitoring. Future studies should explore ways to decrease toxicity of therapy in elderly patients.     

Primary Malignant Fibrous Histiocytoma of the Stomach: (A Case Report)

A case of primary malignant fibrous histiocytoma (MFH) of the stomach is reported. The patient was a 51-year-old man who had a laparotomy for a malignant gastric tumour diagnosed by radiological studies and endoscopic examination. An inoperable gastric malignancy was found at operation. Histopathological studies of tissue biopsied at surgery showed characteristic light microscopic findings and strong positivity for alpha-1-antitrypsin by immunoperoxidase technique indicating the diagnosis of malignant fibrous histiocytoma. The absence of any other soft tissue or retroperitoneal tumour strongly indicated the stomach is the primary site.     

Soft tissue sarcoma: the predominant primary malignancy in the retroperitoneum

Purpose. In the clinical work-up of a retroperitoneal mass, the diagnosis of soft tissue sarcoma is often not considered. Incidence rates of various malignant and benign retroperitoneal tumours were studied to determine the incidence of soft tissue sarcoma in comparison with other neoplasms in the retroperitoneal space.Method. Nation-wide data on retroperitoneal tumours, collected prospectively over a 5-year period (1 January 1989- 1 January 1994), were supplied by the Netherlands Cancer Registry and The Dutch Network and National Database for Pathology.Results. Seven hundred and six patients with a primary retroperitoneal neoplasm were identified; 566 patients had a malignant tumour (80%). A soft tissue sarcoma (STS) was the most frequently diagnosed malignant tumour (n = 192), The agestandardised incidence of retroperitoneal STS was 2.5 per million person-years. The male/female ratio for STS was 0.73. In females, STS comprised 41%of all malignant retroperitoneal tumours, carcinoma of unknown primary tumour site (CUP) comprised 31%, and malignant lymphomas (ML) comprised 22%, whereas in males these values were 28% (STS), 30% (CUP), and 32% (ML), respectively.Discussion. Soft tissue sarcomas, albeit rare, are relatively common primary tumours in the retroperitoneum, especially in women.