Aim

Purpose of this study was to review a single Institution experience and results of management of extraskeletal osteosarcoma (OGS), with emphasis on the role of combined treatment consisting of surgery and adjuvant chemotherapy.

Aim

To report late development of sarcomas on sites of previously curetted and grafted benign tumours. Rare cases of development of sarcomas in sites of previous benign lesions are documented, and the development is generally considered secondary to progression of benign lesions, even without radiotherapy.

Introduction: Extraskeletal osteosarcoma is a rare malignant tumor of the soft tissues. Overall, this malignancy has been associated with worse local control and overall survival rates than its skeletal counterpart despite multimodal approach. Purpose of this study was to review a single Institution experience and analyse results of management to identify factors affecting the outcome.

Methods: Retrospective study of 48 patients observed between 1966 and 2007 was undertaken. Of the total, 36 patients were admitted and managed at our Institution while 12 patients were sent for consultation and therefore not included in this study. Clinico-pathologic features and details of treatment of all 36 patients were reviewed and correlated with outcome. Updated follow-up was available in all patients.

Results: There were 21 males and 15 females, mean age was 53.6+/−19.3 years (range 14–84 yrs); 23 patients (63.9%) presented with localised disease while distant metastases were present in 13 patients (36.1%). Surgery consisted of a limb-salvage procedure in 25 patients (69.4%), amputation in 9 patients (25%) and 2 patients were considered inoperable (5.6%). Postoperative radiation therapy was given to 6 patients (16.7%) and multiagent chemotherapy administered to 19 patients (52.8%). At mean follow-up of 5.8 years, 23 patients had died of disease, expected 5 and 10 year overall survival rates were 41% and 31%. Tumor size and age at presentation were the most important predictors of survival while chemotherapy showed a trend towards improved survival in patients with localised disease.

Discussion and Conclusion: Extraskeletal osteosarcoma was associated with substantially worse prognosis than skeletal osteosarcoma despite multimodal management.

Gorham-Stout disease (GSD) is rare, characterized by proliferation of vascular channels resulting in progressive distruction of bone. In the Rizzoli files we found 15 cases of GSD from 1968 to 2008. Two were excluded for insufficient documentation. For 13 cases clinical data, imaging and histology were analysed. Histopathologic features included benign vascular proliferation, vascular pattern of osteolytic angioma, fibro-connective tissue component and bony destruction. A final diagnosis was established based on clinical, radiological and histopathologic features.

Imaging included X-rays in 11 cases and CT or MRI in 5. All lesions were lytic, with associated sclerosis in two cases. There was one lesion only in 4 cases, multiple lesions in the same bone in 1 and multiple bones involved in 6. Primary sites were proximal femur in 7 cases, pelvis in 2, hip and knee, calcaneus, humerus and cervical spine in 1 case each. Two patients had no treatment, 2 conservative treatment (cast or brace), 5 surgery, 6 medical treatment (byphosphonates, calcitonin, zoledronic acid, interferon, steroids), 1 radiotherapy, 2 selective arterial embolization. Surgery consisted of internal fixation of pathologic fractures in 4 patients and reconstruction of the entire humerus with a double composite allograft in 1. Treatment was surgery only in 2 patients, medical treatment in 4 (1 also embolization), surgery and medical treatment in 2 (1 also embolization), radiotherapy only in 1, conservative treatment in 2. Four patients were lost at follow up. Mean follow up was 17 ys.(min 2, max 30) in 9 patients: 2 dead, 3 healed, 3 with stable disease, 1 alive with disease at 24 ys.

No conclusive treatment recommendations are possible; surgery is indicated in pathologic fractures or reconstruction of massively destroyed bones, medical treatment and selective embolization are helpful. In literature prosthetic reconstruction is preferred due to the risk of allografts resorption.

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.