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Background and purpose -- Soft-tissue sarcoma (STS) is rare, with challenging individualized treatment, so diagnostics and treatment should be centralized. Historical controls are sometimes used for investigation of whether new diagnostic or therapeutic tools affect patient outcome. However, as yet unknown factors may affect the outcome. We investigated prognostic factors and prognosis in 2 nationwide cohorts of patients diagnosed with a local STS during the periods 1998-2001 and 2005-2010, with special interest in finding factors lying behind possible improvement of prognosis. Patients and methods -- 2 cohorts of patients with STS of the extremities or trunk diagnosed during the periods 1998-2001 and 2005-2010 were retrieved from the nationwide Finnish Cancer Registry. Detailed information was gathered from patient files. Results -- Compared to first cohort, a larger proportion of patients with inadequate surgery in the second cohort received radiation therapy, and both the local control rate and the sarcoma-specific survival rate improved in the second cohort. For sarcoma-specific survival, cohort (HR = 0.6, 95% CI: 0.5-0.9), age, depth, grade, and margin were significant factors in multivariate analysis. For local control, cohort (HR = 0.6, 95% CI: 0.5- 0.9), age, and margin were significant in multivariate analysis. Interpretation -- Known prognostic factors including type of treatment did not entirely explain the secular trend of continuous improvement in prognosis in STS. This illustrates the danger of using historical controls for investigation of whether new diagnostic or therapeutic tools have an effect on patient outcome. [ABSTRACT FROM AUTHOR]

Desmoid tumours are uncommon non-malignant tumours that show a locally aggressive growth pattern and a high local recurrence rate after surgery. Approximately 10% of the desmoid tumours are associated with familial adenomatous polyposis (FAP). Variable natural history of the disease challenges treatment decision-making in the absence of prospective, randomised data. Association of this rare tumour to GIST is speculated and the tumorigenesis may share common steps. This study reviews given treatment and reports prognostic factors for local control and concurrent neoplasms in patients evaluated by a single soft tissue tumour group. Patients referred to the soft tissue tumour group at Helsinki University Central Hospital (HUCH) for a desmoid tumour (primary or recurred) during 1987-2007 and receiving surgical treatment with or without adjuvant treatment were included in this retrospective review. All locations and also patients with a FAP-associated tumour were included. Extra-abdominal location showed lower local control despite the fact that 27% of patients also received radiation therapy. One amputation was performed. Female sex and location in the rectus abdominis muscle predicted improved local control in multivariate analysis. In this review, the occurrence (14%) of concurrent neoplasms was higher than expected with unusual tumour types noted including two GISTs. In those patients in whom surgical treatment is chosen, adjuvant radiation therapy should also be considered in order to decrease morbidity from aggressive surgery aiming at R0 resection. Further studies are suggested to illuminate the biological association between the desmoid tumour and other neoplasms. [ABSTRACT FROM AUTHOR]

Background. Most local recurrences have developed in the clinical target volume in previously published series after combined modality treatment for soft tissue sarcoma. However, marginal misses were seen in almost 20% of the patients. The aim of the present study was to determine the location of the recurrence and the total dose at the centre point of the local recurrence for future radiation therapy planning. Material and methods. We included only patients with images in digital form, during 1999-2006 (n = 17), treated for soft tissue sarcoma with combined surgical therapy and radiotherapy at Helsinki University Central Hospital. Image fusion was used to determine the location of the recurrence in relation to radiation therapy target. Results. In the present study utilising digital image fusion, in patients with 3D CT-based radiation treatment planning the risk of marginal miss was low as only one patient of 17 relapsed outside the target. Estimated mean radiation dose at the site of local recurrence was 49.1 Gy in patients with positive margins and 48.1 Gy in patients with negative margins. Conclusion. The risk of marginal miss in soft tissue sarcoma is low after modern 3D planned radiation treatment combined with surgery. More generous use of boost might improve in-target local control. [ABSTRACT FROM AUTHOR]

Background. Ewing's sarcoma family of tumors (ESFTs) are rare bone and soft tissue tumors characterized by specific genetic alterations. Our aim was to carry out a nationwide analysis of ESFT, to survey the treatments used and to report the five-year disease specific and event-free survival rates (EFS and DSS). Material and methods. The study data was gathered from the Finnish National Cancer Registry and all five University Hospitals and consisted of 76 bone and soft tissue ESFT patients diagnosed during 1990-2009. Their medical records were reviewed and data on their disease, treatments, complications and outcome were analyzed. Results. The five-year EFS and DSS of patients with localized disease at diagnosis (n = 57) were 70% and 60%, respectively. Factors contributing to DSS and EFS were the axial vs. peripheral site of primary tumor and adequate surgical resection of the primary tumor. DSS was also affected by patient's age at diagnosis and the treatment employed. The five-year DSS of patients with metastatic disease at diagnosis (n = 19) was 33% and both preoperative and high dose chemotherapy were associated with improved survival. Conclusion. Population-based studies including both bone and soft tissue ESFTs are few. In this nationwide, population-based study on Finnish bone and soft tissue ESFT patients, we find their treatment successful and results comparable to those previously published. Absence of metastases, young age at diagnosis and a peripheral primary tumor site were associated with a better prognosis. It seems that surgical resection of the primary tumor should be performed whenever adequate resection margins can be achieved. The role of high dose chemotherapy merits further studies in this setting. [ABSTRACT FROM AUTHOR]

Merkel cell carcinoma (MCC) is a rare cutaneous neuroendocrine carcinoma. The aims of this study were to investigate the expression of the transcription factors B-lymphoma Moloney murine leukaemia virus insertion (BMI1), myelocytomatosis viral oncogene homologue (c-Myc) and Snail in MCC tumour specimens and to examine the relationship of these markers to Merkel cell polyoma virus (MCV). The study comprised of 133 patients with primary MCC. The expression of BMI1, Snail and c-Myc protein was assessed by immunohistochemistry and compared with clinical parameters, MCV status and patient survival. The presence of MCV was inversely correlated with the expression of BMI1 protein. Tumours expressing BMI1 protein more often presented with lymph node metastases. Snail protein expression was decreased in cases with metastatic dissemination. This study identified two subgroups of MCC: tumours expressing BMI1 but negative for MCV DNA and tumours negative for BMI1 expression but positive for MCV. Importantly, BMI1-positive cases often presented with lymph node metastases. Combined, these results suggest that subtypes of this malignancy exist. [ABSTRACT FROM AUTHOR]

Abstract: Background: Merkel cell carcinoma (MCC) is a rare malignancy of the skin, and its incidence is reported to be rising. The purpose of this study was to calculate its incidence and survival ratios, and to describe the clinical characteristics of Merkel cell carcinoma patients in Finland. Methods: We calculated the incidence of MCC based on data from the Finnish Cancer Registry. In addition, patient files from hospitals and primary health care centres were reviewed for detailed data on the treatment and disease recurrence of 181 patients diagnosed with MCC in Finland during 1983–2004, and relative survival ratios were calculated for them. Results: The incidence (per 100,000) of MCC in Finland in 1989–2008 was 0.11 for men and 0.12 for women, adjusted for age to the world standard population. The mean age at diagnosis was 76years (range 27–100), and 69% of the patients were women. The most common site of the primary tumour was the head and neck (53%). No extra benefit was gained from a wide surgical margin (⩾2cm) compared to a margin of 0.1–0.19cm, but an intralesional excision was more often associated with local recurrence. None of the patients with Stage I–II disease who had received postoperative radiotherapy to the tumour bed had a local recurrence. The 5-year relative survival ratio amongst men was 36% (95% confidence interval 20–54%), and amongst women 69% (56 to −82%). Conclusions: MCC is a rare disease in Finland, with incidence rates similar to those in the other Nordic countries. Our results support the view that complete excision with clear margins and post operative radiotherapy decrease local recurrences. [ABSTRACT FROM AUTHOR]

Koljonen V, Tukiainen E, Haglund C, Böhling T. Expression of cyclin A in Merkel cell carcinoma. APMIS 2004;112:39–44. Merkel cell carcinoma (MCC) is a rare and aggressive primary neuroendocrine carcinoma of the skin. Large tumours, in particular, show rapid progression and metastatic dissemination, while smaller tumours show less aggressive behaviour. Cyclin A is considered to play a central role in cell cycle control. Its overexpression has been found to correlate with prognosis in many types of carcinomas and sarcomas. Twenty-six MCC patients with complete clinical data were selected. Formalin-fixed paraffin-embedded primary tumour samples were immunohistochemically stained for cyclin A. Correlation between expression of cyclin A and primary tumour size, invasion of subcutaneous tissue, local recurrence, metastasis and survival was statistically analysed. Twenty-five samples expressed cyclin A, and the mean value of positive cells was 25%. Our findings demonstrate that even small and superficial MCC show overexpression of cyclin A. There was no statistical correlation between cyclin A expression and the above-mentioned variables. Cyclin A does not seem to be useful as a prognostic tool. Consequently, size of the primary tumour (≥2 cm) remains the primary prognostic tool. As a result every tumour, even those small in size, should be considered aggressive and treated radically and rapidly. [ABSTRACT FROM AUTHOR]