Summary

Starting from human musculoskeletal sarcomas, we isolated a subset of cells that display cancer stem cell properties. The control of culture conditions is crucial to enhance the isolation of this cell population.

Purpose: Aim of this study was to analyse our Institution experience with osteosarcoma in Paget’s disease (PD).

Methods: Twenty-six patients treated between 1961 and 2006 were retrospectively analysed. Information focusing on treatment, imaging and oncologic outcome were obtained from clinical charts.

Results Fifteen patients had previous diagnosis (mean time 9 yrs) of monostotic (80%) or polyostotic (20%) PD; in 11 sarcoma and PD were contemporarily diagnosed. The most frequents osteosarcoma histotypes observed were osteoblastic in 18 cases (69%), fibroblastic in 5 cases (19%), teleangectasic in 2 patients and chondroblastic in one case. In 6 patients surgery only was performed: 3 amputations and 3 resections. In 3 pts surgery (amputation), adjuvant chemotherapy and radio-therapy were given. In 1 pt surgery (amputation) and radiotherapy. In 12 patients surgery and chemotherapy: adjuvant in 10 cases (8 amputations and 2 resections) and neoadjuvant in 2 (both amputations). Two patients had only radiotherapy and 2 had only chemotherapy. Oncologic outcome showed 4 pts with no evidence sisease at a mean follow up of 139 months (min.42.6, max.257.4) and 22 died with disease at a mean time of 20.15 months (min.1, max. 84). One patient only of 6 (11%) treated with surgery only is NED at 10 years, while the other 5 died from disease at a mean time of 30 mos. Three of 12 patients (25%) treated with surgery and chemotherapy are NED at a mean follow up of 12 years, while 9 died of disease at a mean time of 24 months. All patients treated without surgery died at of 7.5 mos on average (min.1 – max.13.7).

Conclusions: Sarcomas in PD have a poor prognosis in pts treated without surgery or with surgery only. Surgery with chemotherapy – when feasible – improves prognosis.

Solitary fibrous tumour (SFT) is a relatively uncommon mesenchymal neoplasm that most frequently arises in the pleura, but is also known to affect extrathoracic sites. About 15 % of SFT’s behave in an aggressive way, giving rise to local recurrence and/or distant metastasis. However, the behaviour of SFT remains unpredictable and due to the rarity of this tumour, it is difficult to define prognostic factors. The purpose of this study was to describe our experience with SFT, trying to define the pathologic features of this rare entity and better understand its clinical behaviour.

We performed a clinicopathologic review of all cases treated for a SFT at the Istituto Ortopedico Rizzoli in Bologna, between 1996 and 2008. We included 24 patients, nine males and fifteen females, ranging in age from 22 to 82 years (median 43.5 years). The anatomical sites involved were: the thigh (12 cases), shoulder region (four cases), gluteus (three cases), foot (two cases), extrapleural thoracic wall (two cases), and the lower leg (one case).

The tumour was > 5 cm in 15 cases, ranging in diameter from 2.5 cm to 18 cm (median 7.5 cm). Pain and swelling were the most frequently reported symptoms at presentation, with a mean duration of symptoms of 10 months. All patients were treated by excisional surgery (wide margins in 11, marginal margins in 13). Three patients had undergone pre-operative radiotherapy (44Gy) and one of these had also adjuvant radiotherapy after marginal excision of the tumour. Six tumours showed at least one atypical histologic feature (moderate to marked cytological atypia, extensive tumor necrosis, ≥ four mitoses per ten high-power fields, or infiltrative margins). On immunohistochemistry, 21 cases were positive for CD-34, 10 for CD-99, 17 for vimentin, three for CD-31, four for actin and one for S-100. Subsequent follow-up (average 33 months, range 5 to 112 months) revealed tumour relapse in only one case: a bone metastasis after 36 months of follow-up. The initial lesion was considered a large, deep, malignant SFT of the thigh, treated with wide surgical excision.

In the current review, including 24 extrathoracic solitary fibrous tumours, all lesions but one had a benign course. Nevertheless, this entity has a potential to recur or metastasize, and therefore careful long-term follow-up is necessary for all patients, even after wide excisional surgery. Although specific prognostic factors are yet to be defined, a high degree of suspicion for malignant behaviour is warranted for those cases in which atypical histologic features are present, particularly in the context of a deep tumor > 5cm in diameter.

Forty-six hemangioendotheliomas (HE) of bone treated at Rizzoli from 1985 to 2004 were studied with minimum follow up of 4 years: 19 females and 27 males, mean age 37 years, mean follow-up 9 years, 35 cases unifocal at diagnosis (10 spine – 1 with lung metastasis also- 11 lower limb, 8 upper limb, 6 pelvis) and 11 with multifocal involvement. In 10 patients intralesional surgery was previously performed elsewhere. In 27 patients primarily treated at Rizzoli with unifocal localization, surgery was used in 15 cases, surgery and radiotherapy in 7, surgery with radio/chemotherapy in 1 and no surgery in 4 (2 radiotherapy, 1 radio/chemotherapy and 1 embolization). Eight unifocal patients already treated elsewhere had surgery in 3 cases, surgery and radiotherapy in 3, surgery with radio/chemotherapy in 1 and surgery plus chemotherapy in 1. Three of the unifocal cases had further bone involvement subsequently. Nine multifocal patients primarily treated at Rizzoli had surgery in 4 cases, surgery and radiotherapy in 4, surgery with radio/chemotherapy in 1. The 2 previously treated multifocal HE had 1 surgery and 1 radiotherapy.

Six patients died: 3 of disease, 1 of radio-induced osteosarcoma, 2 of different disease. Two patients are AWD. Of remaining 40 patients, 26 are NED (mean follow up 9 years), 11 NED after treatment of recurrence, 1 NED after treatment of radio-induced sarcoma. No lung metastases were diagnosed after treatment. All 10 cases previously treated intralesionally had recurrence. Two of 15 unifocal cases treated with surgery recurred (13%). None of 9 resected unifocal cases previously untreated recurred. Two of 21 pts. with radiotherapy (9.5%) had radio-induced sarcoma.

Surgery is recommended, resection when feasible. Radiotherapy, implying risk of induced sarcoma, should be reserved to multifocal or unresectable cases. Adverse prognostic factor was previous intralesional surgery.