Health-related quality of life after oncological resection and reconstruction of the chest wall.

OBJECTIVES: There is limited information of the health-related quality of life (HRQoL) after surgical treatment of chest wall tumors. This cross-sectional study aimed to assess long-term HRQoL after chest wall reconstruction following oncological resection. METHODS: Seventy-eight patients having undergone chest wall tumor resection and reconstruction during 1997-2015 were invited to complete the 15D and QLQ-C30 HRQoL instruments. RESULTS: Altogether, 55 patients (17 men and 38 women), with a mean (SD) age of 68 (14) years, completed the questionnaires (response rate 71%). Patients had been operated due to soft tissue sarcoma (n = 16), advanced breast cancer (n = 15), osteo- or chondrosarcoma (n = 14), or other tumor (n = 10). Median time after primary surgery was 66 (IQR 38, 141) months. The resection was full thickness in 29/55 cases and partial thickness in 26/55 cases. Chest wall reconstruction was required for 47/55 cases (85%). Reconstruction was performed using soft-tissue flap in eight cases, skeletal stabilizations with mesh or mesh-cement-mesh (sandwich method) in 15 cases, and skeletal stabilizations and soft-tissue flap in 24 cases. Patients' mean 15D score (0.878, SD 0.111) was comparable to that of the age- and gender-standardized general population (0.891, SD 0.041). Limitations in breathing and usual activities were noted. The QLQ-C30 cancer-specific HRQoL was 72 points (maximum 100). Scores in the QLQ-C30 Functional scales ranged from 78 (Physical) to 91 (Social). CONCLUSIONS: Long-term HRQoL in patients after chest wall reconstruction following oncological resection is fair and comparable to that of the general population. Limitations in breathing and usual activities can occur.

Functional Outcome After Lower Extremity Soft Tissue Sarcoma Treatment: A Pilot Study Based on Translated and Culturally Adapted Measures.

BACKGROUND AND AIMS: The present standard of care in treating lower extremity soft tissue sarcomas is function-sparing, limb-preserving resection and reconstruction with or without oncological therapy. The aim of this pilot study was to test the suitability and adequacy of the Finnish translations of two functional outcome questionnaires (Toronto Extremity Salvage Score and Musculoskeletal Tumor Society score) and to perform a preliminary investigation of functional outcomes of Finnish lower-limb soft tissue sarcoma patients after operative treatment. MATERIALS AND METHODS: Between June 2015 and December 2015, consecutive surgically treated outpatients were asked to participate in the study. Demographic, clinical, surgical, and oncological outcome data were collected. Two functional outcome questionnaires were used (Toronto Extremity Salvage Score and Musculoskeletal Tumor Society scores). A comparative analysis is presented. RESULTS: A total of 19 lower-limb soft tissue sarcoma patients with a mean follow-up time of 2 years and 10 months were included. All (n = 19) invited patients participated in the study. Mean age was 62.3 years. In total, 13 had high-grade sarcomas. Eight wounds were closed directly, four used skin grafts, and five required flap reconstructions. One patient required a tumor prosthesis, and one required a rotationplasty. A total of 14 patients received oncological therapy. No problems or difficulties were reported in using and completing the Finnish versions of the Toronto Extremity Salvage Score or Musculoskeletal Tumor Society questionnaires. The overall Toronto Extremity Salvage Score and Musculoskeletal Tumor Society scores were 88 and 76, respectively. CONCLUSION: This pilot study suggests that the Finnish versions of the Toronto Extremity Salvage Score and Musculoskeletal Tumor Society questionnaires are suitable for measuring functional outcome after lower extremity soft tissue sarcomas treatment. Functional outcomes vary from moderate to excellent.

Mechanical analysis of cranial distractor attachment with three different resorbable fixation systems.

Distraction osteogenesis (DO) has become increasingly popular to correct craniosynostosis. Disadvantages of DO include the secondary operation needed for device removal and titanium screw related dura injury. To reduce invasiveness of the secondary device removal operation and to overcome titanium-related problems, fixation of the cranial distractor with resorbable materials is a potential alternative. New resorbable fixation methods, such as ultrasound-activated pins (UAPs) or heat-activated pins (HAPs), allow faster attachment on thinner bone than conventional resorbable screws (CRSs) since tapping is not required. However, resorbable materials are designed to be attached with a resorbable plate, not with a titanium distractor. We evaluated the suitability of CRSs, HAPs and UAPs for the cranial distractor fixation in a laboratory setting with a mechanical testing machine. Fracture tests were conducted in two directions with respect to the longitudinal axis; vertical i.e. axial pull-out strength, and horizontal i.e. shear strength. Mean maximum pull-out strength for CRS, HAP and UAP was 48.9 N, 32.5 N and 14.7 N, respectively. Mean maximum shear strength for CRS, HAP and UAP was 40.8 N, 77.9 N and 38.9 N, respectively. According to our in vitro tests, the cranial distractor attachment with four CRSs or six HAPs per footplate would provide sufficient fixation stability.

Force measurements during posterior calvarial vault osteodistraction: A novel measurement method.

Posterior calvarial vault osteodistraction (PCVO) has become increasingly popular in the correction of craniosynostosis. When compared to cranioplasty, PCVO offers a shorter, less invasive operation, greater intracranial volume advancement and a lower rate of relapse. In general, distraction protocols are based primarily on clinical observations rather than systematic research. Faster distraction protocols may reduce complications. However, distraction protocols producing higher forces can increase complications. Thus, we need to understand these forces in order to improve distraction protocols and devices. We developed a force measurement method that can be used on PCVO devices. Here, we present preliminary data about the forces developed during PCVO. We measured the forces in four bicoronal craniosynostosis patients during PCVO. We observed a linear-like trend between the force increase and the distraction distance within distraction sessions. We also observed a step-wise force increase between distraction sessions and found that the distraction force relaxed rapidly shortly after the distraction session. The mean maximum pre-distraction force for one distracter was 20.4 N, while the mean maximum end-distraction force for one distracter was 57.6 N. Our data suggests that current treatment protocols might be re-evaluated favouring shorter distraction distances and more frequent distraction sessions.

The Use of Blood Products in Adult Patients with Burns.

INTRODUCTION: Burn anemia represents a common complication following a burn injury. Burn anemia etiology carries distinct features occurring at each stage of the post-injury and treatment periods resulting from different causes. We aimed to analyze the use of blood components in Finnish burn victims and to identify patient- and injury-related factors influencing their use. METHODS: To study the use of blood products in burn patients, we used data collected from the Optimal Use of Blood registry, developed through co-operation between 10 major hospital districts and the Finnish Red Cross Blood Service. Burn patients ⩾18 years treated at the Helsinki University Hospital between 2005 and 2011 with an in-hospital stay ⩾1 day who received at least one transfusion during their hospital stay were included in this study. RESULTS: Among all 558 burn patients, 192 (34%) received blood products during their hospital stay. The transfused cohort comprised 192 burn patients. The study cohort received a total of 6087 units of blood components, 2422 units of leukoreduced red blood cells, 1728 units of leukoreduced platelets, and 420 units of single-donor fresh frozen plasma or, after 2007, 1517 units of Octaplas(®) frozen plasma. All three types of blood components were administered to 29% of patients, whereas 45% received only red blood cells and 6% received only Octaplas. Transfused patients were significantly older (p < 0.001), experienced fire-/flame-related accidents and burns to multiple locations (p < 0.001), and their in-hospital mortality exceeded that for non-transfused burn patients fivefold (p < 0.05). DISCUSSION: We show that Finnish adult burn patients received ample transfusions. The number of blood components transfused varied according to the anatomical location of the injury and patient survival. Whether the additional mortality is related directly to transfusions or is merely a manifestation of the more severe burn injury remains unknown.

Treatment of compound tibia fracture with microvascular latissimus dorsi flap and the Ilizarov technique: A cross-sectional study of long-term outcomes.

BACKGROUND: Extensive compound tibial fractures present reconstructive challenges. The present study aimed to assess the outcomes of microvascular latissimus dorsi (LD) flap combined with the Ilizarov technique for extensive compound tibial fractures with bone loss and bone healing complications. METHODS: Patient records were reviewed retrospectively. The Lower Extremity Functional Scale (LEFS), the Disabilities of the Arm, Hand and Shoulder (DASH), and the 15D health-related quality of life (HRQoL) instrument were applied. RESULTS: Between 1989 and 2014, 16 patients underwent reconstruction with a microvascular LD flap and bone transport (11/16) or late bone lengthening (5/16). The mean clinical follow-up time was 6.6 (standard deviation (SD): 6.5) years. Three patients had minor complications requiring reoperation. Partial necrosis of one flap required late flap reconstruction in one case. Late bone grafting was used to enhance union in eight of 16 cases. The mean new bone gain was 3.8 cm (SD: 2.5). Overall, 11 patients completed the questionnaires in a mean of 22.3 years (SD: 2.4) after surgery. The main findings revealed a relatively good function of the reconstructed limb and good shoulder function. The mean HRQoL was comparable to that of an age-standardized sample of the general population. CONCLUSION: Segmental tibia transport and lengthening to correct limb length discrepancy do not compromise the microvascular muscle flap. Combined microvascular LD flap reconstruction and the Ilizarov technique can be used in treating acute compound tibial defects, pseudoarthrosis, and osteitis, all associated with significant amputation risk. Fair long-term functional outcomes and HRQoL are achieved when these combined techniques are used.

Strategies for free flap transfer and revascularisation with long-term outcome in the treatment of large diabetic foot lesions.

OBJECTIVE/BACKGROUND: To analyse the impact of ischaemia and revascularisation strategies on the long-term outcome of patients undergoing free flap transfer (FFT) for large diabetic foot lesions penetrating to the tendon, bone, or joint. METHODS: Foot lesions of 63 patients with diabetes (median age 56 years; 70% male) were covered with a FTT in 1991-2003. Three groups were formed and followed until 2009: patients with a native in line artery to the ulcer area (n = 19; group A), patients with correctable ischaemia requiring vascular bypass (n = 32; group B), and patients with uncorrectable ischaemia lacking a recipient vessel in the ulcer area (n = 12; group C). RESULTS: The respective 1, 5, and 10 year amputation free survival rates were 90%, 79%, and 63% in group A; 66%, 25%, and 18% in group B; and 50%, 42%, and 17%, in group C. The respective 1, 5, and 10 year leg salvage rates were 94%, 94%, and 87% in group A; 71%, 65%, and 65% in group B; and 50%, 50%, and 50% in group C. In 1 year, 43%, 45%, and 18% of the patients in groups A, B, and C, respectively, achieved stable epithelisation for at least 6 months. The overall amputation rate was associated with smoking (relative risk [RR] 3.09, 95% confidence interval [CI] 1.8-5.3), heel ulceration (RR 2.25, 95% CI 1.1-4.7), nephropathy (RR 2.24, 95% CI 1.04-4.82), and an ulcer diameter of >10 cm (RR 2.08, 95% CI 1.03-4.48). CONCLUSION: Despite diabetic comorbidities, complicated foot defects may be covered by means of an FFT with excellent long-term amputation free survival, provided that a patent native artery feeds the ulcer area. Ischaemic limbs may also be salvaged with combined FFT and vascular reconstruction in non-smokers and in the absence of very extensive heel ulcers. Occasionally, amputation is avoidable with FFT, even without the possibility of direct revascularisation.

Reduction mammaplasty as an outpatient procedure: a retrospective analysis of outcome and success rate.

BACKGROUND AND AIMS: Reduction mammaplasty is an increasingly common plastic surgical procedure. In the United States, majority of breast reductions are performed as outpatient surgery. In European public health care, outpatient breast reductions have still been rare. Our aim was to retrospectively determine clinical outcome and the success rate of outpatient reduction mammaplasty. MATERIAL AND METHODS: A total of 110 consecutive patients underwent bilateral reduction mammaplasty with a minimum resection of 200 g per breast in an outpatient unit between 2006 and 2009. A comparison group consisted of 28 inpatients. Demographic data and pre-, intra-, and postoperative events as well as complications were recorded. RESULTS: A total of 83 outpatients (75%) were successfully discharged on the day of operation. Reasons for unexpected overnight admission were lack of adult company for the first postoperative night (13 patients, 12%), surgeon's wish (4 patients, 4%), hematoma requiring evacuation (5 patients, 5%), nausea (3 patients, 3%), and pain (2 patients, 2%). Minor complications, especially delayed healing, were common (45 patients, 41%), but major complications were rare (18 patients, 16%). Complication rate was not increased in the outpatient group. Increased duration of operation correlated with increasing complications. CONCLUSION: Reduction mammaplasty can be successfully and safely performed as an outpatient procedure in European public health care.

Chest wall reconstruction after oncological resections.

Most chest wall defects requiring reconstruction result from tumor resection. Bone and soft tissue sarcomas and recurrent mammary cancer are the most common tumors. Careful preoperative evaluation, meticulous surgical technique and active postoperative treatment are important. The selection of reconstruction is based on the nature, size and location of the defect as well as on the general health and prognosis of the patient. The goals of the reconstruction are adequate stability, water- and airtight closure of the chest cavity, and acceptable cosmetic appearance. The pedicled muscular or musculocutaneous flaps are usually the first choice for tis-sue coverage. These include flaps such as latissimus dorsi, vertical or transverse rectus abdominis and pectoralis. In certain cases also the breast flap or omental flap can be used. In selected cases, a free flap reconstruction is indicated if the local options for reconstruction have been used, or if they are unreliable due to earlier scars or radiotherapy. The free flaps to be used for chest wall can be harvested from the thigh (tensor fascia latae flap, anterolateral thigh flap), from the abdomen (transverse rectus abdominis flaps, deep epigastric perforator flaps) or from the chest wall (latissimus dorsi flap and other flaps based on the subscapular artery). Sometimes a fillet forearm can be used as a flap to cover a defect after extended forequarter amputation. Artificial meshes are commonly used to give stability in the defect and to give a platform for the flap. Methylmethacrylate embedded between the two layers of a mesh, or one or two rib grafts fixed to the mesh, can be used to give additional stability in extensive defects to prevent paradoxical movement.

Reconstruction of complex abdominal wall defects.

Complex abdominal wall defects refer to situations where simple ventral hernia repair is not feasible because the defect is very large, there is a concomitant infection or failed previous re-pair attempt, or if there is not enough original skin to cover the repair. Usually a complex abdominal wall repair is preceded by a period of temporary abdominal closure where the short-term aims include closure of the catabolic drain, protection of the viscera and preventing fistula formation, preventing bowel adherence to the abdominal wall, and enabling future fascial and skin closure. Currently the best way to achieve these goals is the vacuum- and mesh-mediated fascial traction method achieving close to 90% fascial closure rates. The long-term aims of an abdominal closure following a planned hernia strategy include intact skin cover, fascial closure at midline (if possible), good functional outcome with innervated abdominal musculature, no pain and good cosmetic result. The main methods of abdominal wall reconstruction include the use of prosthetic (mesh) or autologous material (tissue flaps). In patients with original skin cover over the fascial defect (simple ventral hernia), the most commonly used method is hernia repair with an artificial mesh. For more complex defects, our first choice of reconstruction is the component separation technique, sometimes combined with a mesh. In contaminated fields where component separation alone is not feasible, a combination with a biological mesh can be used. In large defects with grafted skin, a free TFL flap is the best option, sometimes reinforced with a mesh and enhanced with components separation.

Pelvic, perineal and genital reconstructions.

Perineogenital and pelvic surgery is challenging due to the complex anatomy and physiology, multi-organ involvement and microbial environment of this region. In reconstructive surgery local and pedicled flaps are usually applicable. Microvascular flaps are rarely needed. Positioning of the scars, tension in the wound edges and pressure conditions must be taken into account, because failed correction may create more functional and aesthetic problems as the defect itself. This brief review focuses on the reconstructive methods of perineum, genitals and pelvic floor, site by site, with special emphasis on functional details.

Late results following flap reconstruction for chest wall recurrent breast cancer.

INTRODUCTION: Locally extensive recurrent breast cancer usually portends a poor prognosis but certain cases can be treated surgically by wide soft-tissue resection as well as full-thickness chest wall resection (FTCWR). The resulting defect usually necessitates immediate flap coverage. The aim of this study was to assess local control, morbidity, choice of flap reconstruction, patient selection and overall long-term survival following surgical salvage of patients with chest wall recurrent breast cancer. MATERIALS AND METHODS: Forty patients were treated with wide soft-tissue resection and immediate flap reconstruction from 1984 to 2011 in a single institution. Demographic, treatment and mortality data were obtained from patients' files. RESULTS: Mean age at surgery was 54 years. FTCWR was performed in 19 cases including three extended forequarter amputations. Chest wall stabilisation involved a synthetic mesh in 12 patients, fascia lata in two patients, free rib grafts in one patient and synthetic mesh and free rib graft in one patient. Soft-tissue reconstruction consisted of microvascular free flaps in seven patients and pedicled flaps in 33 patients. In-hospital mortality was 0%, 30-day mortality was 5%; there were two re-operations, six minor wound complications and one pulmonary embolism. There were no flap losses. In patients operated on with curative intent (n=31) median disease-free interval was 31 months and median survival was 52 months. CONCLUSIONS: In selected cases wide resections for extensive chest wall recurrent breast cancer can result in reasonable local control and survival. Several flap options exist for soft-tissue reconstruction.

A web-based prognostic tool for extremity and trunk wall soft tissue sarcomas and its external validation.

BACKGROUND: We developed a web-based, prognostic tool for extremity and trunk wall soft tissue sarcoma to predict 10-year sarcoma-specific survival. External validation was performed. METHODS: Patients referred during 1987-2002 to Helsinki University Central Hospital are included. External validation was obtained from the Lund University Hospital register. Cox proportional hazards models were fitted with the Helsinki data. The previously described model (SIN) includes size, necrosis, and vascular invasion. The extended model (SAM) includes the SIN factors and in addition depth, location, grade, and size on a continuous scale. Models were statistically compared according to accuracy (area under the ROC curve=AUC) of 10-year sarcoma-specific survival prediction. RESULTS: The AUC of the SAM model in 10-year survival prediction in the Helsinki patient series was 0.81 as compared with 0.74 for the SIN model (P=0.0007). The corresponding AUCs in the external validation series were 0.77 for the SAM model and 0.73 for the SIN model (P=0.03). A web-based calculator for the SAM model is available at http://www.prognomics.org/sam. CONCLUSION: Addition of grade, depth, and location as well as tumour size on a continuous scale significantly improved the accuracy of the prognostic model when compared with a model that includes only size, necrosis, and vascular invasion.

Complement activation during liver transplantation-special emphasis on patients with atypical hemolytic uremic syndrome.

Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy often caused by mutations in complement factor H (CFH), the main regulator of alternative complement pathway. Because CFH is produced mainly by the liver, combined liver-kidney transplantation is a reasonable option in treatment of patients with severe aHUS. We studied complement activation by monitoring activation markers during liver transplantation in two aHUS patients treated extensively with plasma exchange and nine other liver transplantation patients. After the reperfusion, a clear increase in all the activation markers except C4d was observed indicating that the activation occurs mainly through the alternative pathway. Concentration of SC5b-9 was higher in the hepatic than the portal vein indicating complement activation in the graft. Preoperatively and early during the operation, the aHUS patients showed highest C3d concentrations but otherwise their activation markers were similar to the other patients. In the other patients, correlation was found between perioperative SC5b-9 concentration and postoperative alanine aminotransferase and histological changes. This study explains why supply of normal CFH by extensive plasma exchange is beneficial before combined liver-kidney transplantation of aHUS patients. Also the results suggest that perioperative inhibition of the terminal complement cascade might be beneficial if enhanced complement activation is expected.

Reconstruction of extensive abdominal wall defects with microvascular tensor fasciae latae flap.

BACKGROUND: Most abdominal wall defects can be repaired with a mesh, components separation technique or pedicle flaps, but a free flap reconstruction might be the only option for large epigastric or non-midline defects. This retrospective study reviewed the results of consecutive patients who had extensive full-thickness abdominal wall defects reconstructed with a large, microvascular tensor fasciae latae (TFL) flap. METHODS: A 30-35 × 15-20-cm TFL flap was harvested and microvascular anastomoses were performed using a saphenous arteriovenous loop. RESULTS: From 1995 to 2009, 20 patients were operated on with a TFL flap. The repair was combined with a mesh in nine patients, components separation in one patient, and both techniques were used in one patient. The median follow-up was 2 (range 0·5-13) years. There were no perioperative deaths, or intra-abdominal or deep surgical-site infections. The flap failed in one patient, two patients had minor distal tip necrosis of the flap and one developed a recurrent hernia 3 months after TFL repair. CONCLUSION: A microvascular TFL flap is a feasible option for reconstruction of exceptionally large abdominal wall defects if other means of reconstruction have already been used or are insufficient. It can also be combined with other methods of reconstruction. A close collaboration between plastic and abdominal surgeons is important. Copyright © 2011 British Journal of Surgery Society Ltd. Published by John Wiley & Sons, Ltd.

Surgical management of radiation-associated cutaneous breast angiosarcoma.

The purpose of this study was to investigate the surgical management of radiation-associated cutaneous breast angiosarcoma with an emphasis on surgical margins and choice of reconstruction. Nine cases of angiosarcoma were identified in patients earlier treated with radiotherapy for breast cancer. Breast angiosarcoma was diagnosed a median of 5.25 years following radiotherapy. Median age at diagnosis was 60 years. Surgical treatment consisted of radical mastectomy (four cases), simple mastectomy (two cases) and wide local excision (three cases). Defect reconstruction involved three latissimus dorsi flap reconstructions and four skin grafts. Clear histological margins were achieved in all cases. Median follow-up was 81 months. Six patients were alive and disease-free at the end of the study period. Aggressive surgical resection with wide margins is essential to reduce local recurrence and improve survival.

Cost-utility of breast reduction surgery--a prospective study.

OBJECTIVE: To assess the cost-utility of routine breast-reduction surgery in the setting of a large university hospital. METHODS: In the framework of a large trial exploring the feasibility of routine health-related quality of life (HRQoL) assessment, 80 patients (mean age: 45 years) entering the Department of Plastic Surgery for operative breast reduction filled in the 15-dimension (15D) HRQoL survey before and 6 months after surgery. Data on hospital costs were obtained from the hospital records. RESULTS: Mean (+/-SD) HRQoL score (on a 0-1 scale) increased as a result of surgery from the preoperative 0.916+/-0.075 to 0.939+/-0.076 (p<0.001) 6 months after surgery, corresponding to a mean (+/-SD) gain of 0.930+/-2.117 quality-adjusted life years (QALYs). Of the 15 health dimensions, discomfort and symptoms showed the greatest improvement (p<0.001). A statistically significant improvement was also seen on the dimensions of breathing, sleeping and distress. Mean hospital cost of treatment was 3383 euro+/-1744, and the cost per QALY was 3638 euro. Using 5% discounting for QALYs, the cost per QALY increased to 8973 euro. CONCLUSION: Breast-reduction surgery improved HRQoL in a statistically significant manner and at a reasonable cost, as the cost per QALY was in the same range as that observed in our material, for example, for hip-replacement surgery.

Microvascular reconstructions after extensive soft tissue sarcoma resections in the upper limb.

AIMS: Limb-sparing surgery combined with radiotherapy (RT) is the basis of extremity soft tissue sarcoma (STS) treatment. The aim of this study was to evaluate the results of microvascular reconstruction after extensive tumour resections in the upper extremity. METHODS: Twenty patients with STS of the upper extremity were treated with excision and microvascular reconstruction. RT was administered if surgical margins were less than 25 mm. Results were evaluated retrospectively. RESULTS: Twenty free flaps were performed and no flaps were lost. There was no operative mortality, and wound complication rate was 15%. Median follow-up length was 74 months. Five-year local recurrence-free survival was 57%, metastasis-free survival 67%, disease-free survival 45% and disease-specific overall survival 80%. Ten patients had no or only mild impairment of upper extremity function, seven had impaired function affecting daily life, and three patients underwent amputation. For patients treated with curative intent, limb salvage rate was 94%. CONCLUSIONS: Free flaps are useful and reliable in the treatment of patients with STS of the upper extremity. Without microvascular reconstruction limb salvage would have been impossible in these patients. Oncological outcome is comparable to other extremity STS patients and upper extremity function is acceptable.

Chronic lymphocytic leukaemia patients have a high risk of Merkel-cell polyomavirus DNA-positive Merkel-cell carcinoma.

BACKGROUND: Immunosuppression and Merkel-cell polyomavirus (MCPyV) infection may have a role in the pathogenesis of Merkel-cell carcinoma (MCC), a rare neuroendocrine carcinoma of the skin. METHODS: We studied incidence of chronic lymphocytic leukaemia (CLL) and MCC from the files of the Finnish Cancer Registry and the largest hospital of Finland, Helsinki University Central Hospital, from 1979 to 2006. Presence of MCPyV DNA in MCCs was investigated by quantitative PCR. RESULTS: We identified 4164 patients diagnosed with CLL and 172 diagnosed with MCC. Six patients diagnosed with both diseases were found; CLL was the first diagnosis in four cases and MCC in two. The standardised incidence ratio (SIR) for CLL after the diagnosis of MCC was highly elevated, 17.9 (95% confidence interval (CI), 2.2-64.6; P<0.001), and the SIR for MCC after the diagnosis of CLL was also elevated, 15.7 (3.2-46.0, P<0.01). Merkel-cell polyomavirus DNA was present in all five MCCs with tumour tissue available for analysis. CONCLUSIONS: We conclude that patients diagnosed with CLL have a substantially increased risk for MCC, and vice versa. Merkel-cell polyomavirus DNA is frequently present in MCCs that occur in CLL patients. Immunosuppression related with CLL and viral infection might explain the association between CLL and MCC.