Background

Angiosarcoma is a rare complication of breast cancer treatment. In order to define predictors, clinical presentation, and outcome, we characterized a population-based 50-year cohort of angiosarcomas after breast cancer.

Retroperitoneal sarcomas (RS) are rare tumours that may reach considerable size at diagnosis and should optimally be treated by a specialized multidisciplinary team. In Sweden, we have since 10 years enforced referral of RS to sarcoma centers, but have experienced a considerable delay, which provides the basis for this study on delays in RS diagnosis and treatment.

We identified 33 patients treated for RS at the Southern Sweden Sarcoma Center (covering a population of 1.5 million), Lund University Hospital between 2003-2009. Data, including onset of symptoms, time to diagnosis and time to treatment were recorded from clinical files. Patient's delay was defined as the time from onset of symptoms to the first visit to a doctor, which could be a general practitioner or a specialist. Doctor's delay was defined as the time from the first visit to a doctor to the start of treatment, which was in most cases surgery.

In total, 30 patients were referred to the sarcoma centre for treatment. Complete data are available from 25 patients (13 men) with a median age of 62 (20-86) years. Median patient's delay was 15 days (0-9 months) and median doctor's delay was 97 days (0-40 months). Median doctor's delay was indeed somewhat longer (52 days) at the sarcoma centre than at the local hospitals (38 days). Some of the longest delays were caused by primary erroneous diagnosis (16 and 40 months) and comorbidity (4, 8 and 19 months) that required other medical procedures before surgery.

Though almost all patients with RS in Southern Sweden are referred to a sarcoma centre for treatment, delays are considerable for many patients with doctor's delays outnumbering patient's delays. Our findings demonstrate that centralisation per se is not sufficient to treat RS, but that optimized diagnostics and clinical management is needed also at sarcoma centers.

Deep-seated lipomas are often atypical histologically and are considered by some to have a high risk of recurrence after excision. We reviewed 215 deep-seated lipomas of the extremities and trunk wall with reference to histology, cytogenetics, clinical features and local recurrence. We classified tumours with atypical features and/or ring chromosomes as atypical lipomas. These were more common in men, larger than ordinary lipomas and more often located in the upper leg. The annual incidence was estimated as ten per million inhabitants and the ratio of atypical to ordinary lipomas was 1:3. In total, six tumours (3%), recurred locally after a median of eight years (1 to 16); of these, four were classified as atypical.

The low recurrence rate of deep-seated lipomas of the extremity or trunk wall, irrespective of histological subtype, implies that if surgery is indicated, the tumour may be shelled out, that atypical lipomas in these locations do not deserve the designation well-differentiated liposarcoma, and that routine review after surgery is not required.

We reviewed nine patients at a mean period of 11 years (6 to 16) after curettage and cementing of a giant-cell tumour around the knee to determine if there were any long-term adverse effects on the cartilage. Plain radiography, MRI, delayed gadolinium-enhanced MRI of the cartilage and measurement of the serum level of cartilage oligomeric matrix protein were carried out. The functional outcome was evaluated using the Lysholm knee score.

Each patient was physically active and had returned to their previous occupation. Most participated in recreational sports or exercise.

The mean Lysholm knee score was 92 (83 to 100). Only one patient was found to have cartilage damage adjacent to the cement. This patient had a history of intra-articular fracture and local recurrence, leading to degenerative changes.

Interpretation of the data obtained from delayed gadolinium-enhanced MRI of the cartilage was difficult, with variation in the T1 values which did not correlate with the clinical or radiological findings. We did not find it helpful in the early diagnosis of degeneration of cartilage. We also found no obvious correlation between the serum cartilage oligomeric matrix protein level and the radiological and MR findings, function, time after surgery and the age of the patient.

In summary, we found no evidence that the long-term presence of cement close to the knee joint was associated with the development of degenerative osteoarthritis.

We retrospectively studied local recurrence of giant cell tumour in long bones following treatment with curettage and cementing in 137 patients. The median follow-up time was 60 months (3 to 166). A total of 19 patients (14%) had at least one local recurrence, the first was diagnosed at a median of 17 months (3 to 29) after treatment of the primary tumour. There were 13 patients with a total of 15 local recurrences who were successfully treated by further curettage and cementing. Two patients with a second local recurrence were consequently treated twice. At the last follow-up, at a median of 53 months (3 to 128) after the most recent operation, all patients were free from disease and had good function.

We concluded that local recurrence of giant cell tumour after curettage and cementing in long bones can generally be successfully treated with further curettage and cementing, with only a minor risk of increased morbidity. This suggests that more extensive surgery for the primary tumour in an attempt to obtain wide margins is not the method of choice, since it leaves the patient with higher morbidity with no significant gain with respect to cure of the disease.