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Discusses the diagnosis and treatment of soft tissue tumors in the Netherlands. Definition and classification of soft tissue tumors; Preparation of pathology specimens; Reporting of diagnostic results; Therapeutic possibilities of surgery, radiotherapy and chemotherapy.

Desmoid tumor, also known as aggressive fibromatosis or desmoid-type fibromatosis, is a rare monoclonal, fibroblastic proliferation arising in musculoaponeurotic structures. Although histologically benign, desmoids are often locally invasive and associated with a high local recurrence rate after resection. Since it is a heterogeneous disease, in particular regarding clinical presentation, anatomic location and biological behavior, treatment should be individualized to reduce local tumor control failure with concurrently acceptable morbidity and preservation of quality of life. Many issues regarding optimal treatment of desmoids remain controversial. However, wide surgical excision remains the treatment of choice, except when surgery is mutilating and is associated with considerable function loss or major morbidity. Involvement of surgical margins is probably associated with an increased risk of local recurrence. Postoperative radiotherapy results in a significant reduction of the local recurrence rate, but only in the case of involved surgical margins. Radiotherapy for gross disease is considerably effective, but is associated with a relatively high rate of complications, which are usually mild or moderate and radiation dose dependent. Radiotherapy should only be applied where anatomic constraints preclude complete resection and radiotherapy is not too toxic. Risk factors for local tumor control failure include young age, large size, presentation as recurrent disease, limb/girdle or intra-abdominal location, involved surgical margins, omission of radiotherapy, radiation dose less than 50 Gy and insufficient radiation field size. Increased comprehension of the pathogenesis and biological behavior of desmoids resulted in the emerging applicability of systemic therapies and a wait-and-see policy. Systemic treatment may be indicated in patients that have anatomic barriers to effective surgery or radiotherapy. Considering the significant morbidity of surgery and/or radiotherapy for certain locations, especially mutilation and loss of function, and the tumor's natural history, which is often characterized by prolonged periods of stability or even regression, a period of watchful waiting may compose the most appropriate management in selected asymptomatic patients. Attempts to complete eradication of the disease may be worse than the disease itself.

With a total incidence of ca. 800 per year, oral cavity or oropharyngeal carcinoma represents only a small fraction of the total number of cases of cancer in The Netherlands. In order to achieve as much nationwide uniformity as possible in its diagnosis, treatment, after-care and follow-up, a practice guideline has now been developed. The diagnosis of the primary tumour and the neck, careful clinical examination is complemented by echography, often combined with a biopsy, MRI and CT, while technetium scintigraphy in combination with an orthopantomogram is used to detect invasion of the mandible. The smaller oropharyngeal carcinomas (T1 and T2), surgery and radiotherapy (external therapy or brachytherapy) are equally useful, but for the larger carcinomas (T3 and T4) in patients in good general condition, surgery in combination with radiotherapy is preferred. When radiotherapy is chosen, then brachytherapy is preferred for the smaller tumours of the tongue and the floor of the mouth. In patients without demonstrable metastases to the lymph nodes, just as in the case oforopharyngeal carcinomas, the decision whether or not to treat the neck depends on the chances that cervical lymph node metastases are present: when the risk is smaller than 20% (as with smaller tumours), then a wait-and-see policy is justified, but if the risk is greater than 20% (as with larger tumours or very thick rumours), then the neck should be treated, either surgically or by radiotherapy. The practice guideline also contains information regarding decision making, information and communication, palliative therapy and symptomatic treatment, psychosocial assistance, prosthetic reconstruction, physiotherapy, oral hygiene, nutrition and dietetics, swallowing and speech rehabilitation, and the policy with regard to smoking and alcohol consumption.

Merkel cell carcinoma is a rare, highly malignant skin tumor with a predisposition for local, nodal and distant recurrence which should be managed in a multimodal approach. Here, we performed a retrospective analysis of 36 patients, treated from 1984 to 1994. In patients with stage I disease, locoregional recurrence after surgery alone occurred in 65% compared to 43% after excision and adjuvant radiotherapy. Although the difference in disease-specific survival was not significant (67% versus 52% at 5 years), combination therapy conferred a benefit for locoregional control and relapse-free survival (88 versus 58 months). The majority of failures (54%) occurred within the draining lymph nodes that were not treated in the initial approach and locoregional recurrence was strongly associated with a later systemic relapse (50% versus 0%). Salvage after localized recurrence was achieved in 67% of resectable cases. The high rate of nodal recurrence after surgery alone warrants the use of elective nodal treatment in patients with stage I disease. Surgical resection of the primary tumor should be followed by postoperative radiotherapy to the primary site and draining lymph nodes. Even after locoregional recurrence, a substantial proportion of patients is able to achieve long-term disease-free survival, particularly after complete resection and adjuvant irradiation.

Aims: To investigate the results of our treatment policy, we present our institutional experience in the management of regional neck node metastases of cutaneous head and neck squamous cell carcinoma (CHNSCC)., Methods: Between 1977 and 1997, 343 patients presented with CHNSCC, of whom 41 were treated for regional metastases, with parotidectomy and/or (selective) neck dissection. Histological analysis was performed on all operation specimens. Patients with multiple nodes and/or extra capsular rupture, at risk for recurrence, received adjuvant radiotherapy., Results: Seventy-six percent of the regional metastases occurred within the first 2 years, but a delay of more than 5 years was also observed. Parotid gland (56%), neck levels II (39%) and V (22%) were most frequently involved. Twenty-four percent of patients treated with curative intent failed at the regional site. Five years overall survival was 46%, with a median survival of 49 months. No survival differences emerged between patients treated by surgery alone and patients receiving adjuvant radiotherapy (P=0.14). Five patients died of disease, of whom three with distant metastases., Conclusion: Regionally metastasized CHNSCC is a serious disease with a high risk of regional recurrence, calling for combined surgery and adjuvant radiotherapy. A small subset of patients with a long interval between completion of primary treatment and presence of regional nodes justifies a long follow-up.

Objective: Evaluation of diagnostic procedures and treatment results in patients with lymph node metastases of squamous cell carcinoma in the head and neck region with an unknown primary., Design: Retrospective, descriptive., Patients and Methods: In 1979-98, 39 patients (30 male, 9 female; mean age 59 years; range: 39-88) were treated for cervical lymph node metastases of squamous cell carcinoma with an unknown primary. According to protocol, all patients underwent ENT examination, radiodiagnostic procedures and, if possible, examination under general anaesthesia of the upper respiratory and digestive tracts. Most patients were treated by neck dissection followed by bilateral radiotherapy to the neck and to mucosal sites of suspected origin. The data were collected from medical records. The number of patients who had had no local or regional recurrence and were still alive after 5 years were calculated by the Kaplan-Meier method., Results: There was a median interval of three months (3-100 weeks) between the first symptoms and presentation in our clinic. During this period an extensive search for a primary tumour was performed. Most lymph nodes metastases (37/44; 84%) were found at level II and III. The N-stage was as follows: N1 (6), N2 (14), N3 (19). In two patients a primary tumour was diagnosed 20 and 37 months after treatment, respectively, both at mucosal sites which were not irradiated. A five-year locoregional recurrence-free interval was seen in 66%. The five-year overall survival rate was 52% and was significantly related to the extent of the cervical lymph node metastases., Conclusion: Early treatment of patients with cervical lymph node metastases from an unknown primary tumour improves survival rates. Radiation therapy of the laryngo-pharyngeal axis seems effective in preventing outgrowth of an occult primary.

Purpose: To assess consequences of voice impairment in daily life for patients following radiotherapy for early glottic cancer, by means of a multidimensional analysis protocol including voice quality, vocal function, and vocal performance measures., Methods and Materials: A total of 60 men treated with radiotherapy (66 Gy/33 fractions, 60 Gy/30 fractions, 60 Gy/25 fractions) for early T1 glottic cancer and 20 matched control speakers filled in questionnaires on vocal performance. Furthermore, perceptual analyses of voice quality and stroboscopic measures of vocal function were performed. There was a longitudinal group of 10 patients from whom data were collected before, as well as 6 months and 2 years after, radiation. Furthermore, data were collected on 5 separate groups of 10 patients each: before, 6 months after, 2 years after, 3-7 years after, and 7-10 years after radiation., Results: High correlations were found between self-ratings of vocal performance and several voice measures. Patients before radiotherapy experienced poor voice characteristics that improved 6 months to 10 years after treatment, and became comparable to vocal performance of control speakers in 50% of the patients. Following radiotherapy, deviant voice characteristics and consequences in daily life occurred significantly more often for patients in whom initial diagnosis consisted of stripping the vocal cord instead of biopsies and for patients who continued smoking after treatment., Conclusion: Voice characteristics of patients diagnosed with early glottic cancer improved after radiotherapy, and became normal in half of our patients. Stripping the vocal cord for initial diagnosis and continued smoking after treatment decreased deviant voice characteristics.

Background: Understanding of prognostic factors in parotid carcinoma has grown considerably. In particular, clinical tumor staging and histologic classification have been found to be prognostically important. Univariate and multivariate analyses have indicated that other variables, such as age, pain, skin invasion, and facial nerve impairment, are important predictors as well. In an actual patient, some of these factors are present and others are absent. However, a clinical tool incorporating this information, resulting in an individualized prognosis based on the combined effects of present adverse prognostic factors, has never been devised., Methods: Of a cohort of 168 patients, 151 were evaluated to assess the prognostic value of clinical and pathologic factors in a multivariate proportional hazards analysis. Follow-up ranged from 1 to 278 months (median, 37 months). The end point was tumor recurrence. Identified prognostic factors and their hazard ratios were combined into prognostic scores., Results: Clinical T classification, clinical N classification, pain, age at diagnosis, skin invasion, facial nerve dysfunction, perineural growth, and positive surgical margins acted as major factors predicting recurrence. A prognostic score (PS), generated by the weighted combination of the factors present in the individual patient, placed the patient in one of four subgroups with markedly different prognoses. In the subgroups based on the preoperative prognostic score, 5-year recurrence free percentages ranged from 92% (in the group PS1=1) to 23% (in PS1=4). In the subgroups based on the postoperative prognostic score, which took into account the histologic details of the resected specimen, 5-year recurrence free percentages ranged from 95% (in the group PS2=1) to 42% (in PS2=4)., Conclusions: The proposed subgrouping, which is based on the combined effects of key prognostic preoperative and postoperative factors, provides a practical prognostic grouping system for the clinician treating patients with parotid carcinoma.