Periosteal osteosarcoma: a single-institution experience

BACKGROUND:

Periosteal osteosarcoma is a rare variant of osteosarcoma. Wide surgical removal is the mainstay of treatment, but controversy remains about the role of chemotherapy. The objective of this study was to review and analyze the clinical and treatment‐related factors that influence the survival of patients with periosteal osteosarcoma who received treatment in a single institution.

METHODS:

Thirty‐three patients with periosteal osteosarcoma (19 males and 14 females) with a median age of 16 years (range, ages 6‐32 years) underwent surgery (32 patients) and received radiotherapy (1 patient). Chemotherapy was received according to different regimens for high‐grade osteosarcoma by 14 patients who had grade 3 tumors.

RESULTS:

The 10‐year overall survival rate was 84%. The only patient who did not undergo surgery died of disease after 9 months; for the remaining 32 patients the 10‐year disease‐free survival rate was 65%. Survival was not influenced by the receipt of chemotherapy. The patients who received chemotherapy had a 10‐year overall survival rate of 86%, and those who received only local treatment had an overall survival rate of 83% (P = .73).

CONCLUSIONS:

The authors' experience indicated that the treatment of periosteal osteosarcoma requires only wide surgical removal of the tumor and that adjuvant chemotherapy does not improve survival. Cancer 2011. © 2010 American Cancer Society.     

Periosteal Ewing's sarcoma

Ewing's sarcoma is a small cell malignant tumor that usually arises in the medullary cavity of bone. Less frequently, it originates in soft tissue and may secondarily invade underlying bone. The origin of Ewing's sarcoma in a periosteal location without extension into either the bone or adjacent soft tissue has not been clearly documented. Other malignant tumors of bone (e.g., osteosarcoma) appear to have a somewhat better prognosis when confined between periosteum and bone. The case of a patient with a periosteal Ewing's sarcoma who received a radical excision and postoperative chemotherapy and who is without evidence of disease with over 2 years follow-up is reported.     

Periosteal osteogenic sarcoma.

A study of 102 osteogenic sarcomas of the bone surface revealed that 79 were parosteal osteogenic sarcomas. Roentgenographically, these 79 were dense, lobulated lesions attached by a broad base to underlying bone, usually the lower femoral shaft. Histologically, they were low-grade osteosarcomas. The other 23 lesions (periosteal osteogenic sarcoma) usually involved the upper tibial shaft and presented as small radiolucent lesions on the surface, with formation of spicules of bone perpendicular to the bone shaft. Histologically, these 23 were relatively high-grade, predominantly chondroblastic osteogenic sarcomas. Thirteen of the 23 patients were males, and most were in the second decade of life. Of five patients who had excision of the tumor, two had recurrence. Seven of 13 patients who underwent amputation initially were alive without disease at last follow-up. Only 4 of the 23 patients have dies of metastatic disease.     

Periosteal margin in soft-tissue sarcoma

BACKGROUND: Soft-tissue sarcomas frequently rest in contact with bone. The purpose of the study was to evaluate the risk of local recurrence for sarcomas adjacent to bone and to determine whether the periosteum provides an adequate margin of resection. METHODS: Fifty patients with soft-tissue sarcomas abutting bone were treated at a single institution between 1990 and 2004. All patients had high-grade, T2 (>5 cm), nonmetastatic disease in the lower extremity. Bone contact was verified by preoperative magnetic resonance imaging (MRI) and/or computed tomography (CT) scans. Forty-three of 50 patients received preoperative radiation with a mean dose of 50 Gy. In 11 cases a composite resection of bone and soft tissue was performed. In 39 cases the excision involved only soft tissue. RESULTS: True bone invasion was verified by histopathologic examination in 3 of 50 cases (6%). Local recurrence in the soft tissues developed in 8 of 50 (16%) patients. In no case did the recurrence involve destruction of cortical bone or erosion into bone. The recurrent tumor resided against the region of previous bone contact in 1 of 8 cases. There was no statistically significant difference in local recurrence between patients who had composite bone resection and patients who had soft-tissue resection only (P = .87). CONCLUSIONS: Relatively few sarcomas are able to penetrate cortical bone. Composite bone and soft-tissue resections are indicated primarily for frank bone invasion. In the absence of this, the periosteum is an adequate surgical margin for sarcomas treated with wide excision and radiation.     

Periosteal osteosarcoma--a European review of outcome

Periosteal osteosarcoma is a rare primary malignant bone tumour. Treatment is by surgical excision, but controversy remains about the value of chemotherapy. The members of the European Musculo Skeletal Oncology Society (EMSOS) collaborated to produce a dataset of 119 patients. The predominant site for the tumour was the femur, followed by the tibia. All but 2 patients underwent surgery, with 9 requiring amputation and the others having limb salvage. A total of 81 patients had chemotherapy, of whom 50 had neoadjuvant chemotherapy. There was no standard chemotherapy regime, but all patients receiving chemotherapy were given doxorubicin combined with at least one other agent. The overall survival was 89% at 5 years and 83% at 10 years. Eight patients developed local recurrence, of whom 5 died. Survival was related to appearance of local recurrence (P < 0.0001) but no other single factor. The use of chemotherapy was not shown to be a prognostic factor, but was used in two-thirds of the patients in this study.     

Extraskeletal osteosarcoma

A retrospective study of 88 cases of extraskeletal osteosarcoma revealed that this tumor affects adults almost exclusively, with a high incidence in patients older than 50 years, and is slightly more common in males (58%) than in female patients. The tumor occurred principally as a soft tissue mass in an extremity, with a predilection for the thighs (lower extremity, 46.6%; upper extremity, 20.5%) and the retroperitoneum (17%). Most were deep-seated and were firmly attached to the fascia, but occasionally they were freely movable and confined to the subcutis or dermis. Nearly all presented as an insidiously growing mass rarely causing pain or tenderness. The preoperative duration of symptoms ranged from 2 weeks to 25 years (median, 6 months). In 17 cases, it exceeded 2 years. A history of prior trauma to the site of the tumor was stated in 11 of the 88 cases (12.5%) and of radiation in five cases (5.7%). Microscopically, the tumors contained varying amounts of neoplastic osteoid and bone, sometimes together with islands of malignant-appearing cartilage. Like osteosarcoma of bone, extraskeletal osteosarcoma showed a striking variation in histologic appearance and focally resembled malignant fibrous histiocytoma, fibrosarcoma, and malignant schwannoma. Follow-up information was available for 65 patients. Eight (12.3%) patients were alive with no evidence of recurrence; 12 (18.5%) patients were alive with one or more recurrences; and five (7.6%) were alive with metastases. Twenty-eight of the tumors (43%) recurred and 39 (63%) metastasized. Forty (61.5%) of the patients with follow-up information had died, 36 from the tumor and four from miscellaneous causes. The prevailing sites of metastases were the lung, the regional lymph nodes, and bone.     

Metastatic osteosarcoma

BACKGROUND: The outcome of patients with metastatic osteosarcoma treated in two consecutive trials from 1986 to 1997 was analyzed to evaluate the efficacy of carboplatin-based multiagent chemotherapy and to identify prognostic factors. The initial study (OS-86) used ifosfamide, cisplatin, doxorubicin, and high-dose methotrexate, and the subsequent study (OS-91) used the same agents at similar doses, but carboplatin was substituted for cisplatin. METHODS: Twelve patients (median age, 15.1 yrs) were treated in OS-86 for osteosarcoma metastatic to the lung only (11 patients) or bone only (1 patient), and 17 patients (median age, 15.1 yrs) were treated in OS-91 for osteosarcoma metastatic to the lung only (12 patients), bone only (2 patients), lung and bone (2 patients), or other site (1 patient). RESULTS: Patients with metastatic disease enrolled in OS-86 and those with metastatic disease enrolled in OS-91 did not differ in terms of demographic features, histologic subtype, site of primary tumor, or site of metastases. There was a difference in survival according to treatment protocol (P = 0.054). All survivors (four of whom were enrolled in OS-86 and one of whom was enrolled in OS-91) had lung metastases only. Five-year survival estimates for patients with lung metastases only were 45.5 +/- 13.7% (OS-86) and 8.3 +/- 5.6% (OS-91) (P = 0.084). Unilateral lung metastases (P = 0.006), no more than three lung nodules (P = 0.014), and surgical remission (P = 0.001) were associated with improved survival probability. CONCLUSIONS: The poor outcome of patients with metastatic osteosarcoma treated in OS-91 justifies the use of cisplatin with its associated toxicity in patients with high-risk disease.     

Craniofacial osteosarcoma Experience of the cooperative German-Austrian-Swiss osteosarcoma study group

The aim of this retrospective analysis was to evaluate patient and tumor characteristics and treatment results and prognostic factors for patients with craniofacial osteosarcoma (CFOS). The COSS database was searched for patients with previously untreated, histologically confirmed craniofacial osteosarcoma with at least one follow-up examination. In a 28-year period extending from 1977 to 2004, 49 eligible patients were identified and their charts retrospectively analysed. The median age at diagnosis was 19.7 years (range: 4.6-57.2) with no gender predilection. Thirteen CFOS were second or even third primary malignancies. The jaws were the primary site in 27 patients (55% - mandible 15 (31%), maxilla 12 (24%)), while extragnathic bones were involved in 22 (45%). All 49 patients underwent surgery; in 37 (76%) combined with chemotherapy, in seven (14%) with chemotherapy and radiotherapy. Twenty-one patients (43%) received preoperative chemotherapy and the other 28 (57%) had primary surgery. A complete surgical remission was achieved in 32 patients, of whom 24 remained in local control. Actuarial five-year overall and event-free survival rates were 74% and 44%, respectively. Extragnathic site (p<.001) and documented postsurgical rest of the primary tumor (p<.001) were associated with inferior overall survival probabilities. All 24 patients who achieved and maintained local surgical control survived disease-free. Multidisciplinary treatment of CFOS within a multicenter setting resulted in long-term survival in well over two thirds of affected patients. Extragnathic sites and failure to achieve and maintain local surgical control emerged as strong negative prognostic factors.     

Management of osteosarcoma

Opinion statement Improving cure rates for osteosarcoma continues to be a major challenge. The clinical management of individual patients is exacting and requires a skilled, experienced team including a surgeon, pathologist, oncologist, and radiologist, with support from specialist nurses and rehabilitation teams. Outcomes from treatment have improved little in 20 years and remain disappointing. Chemotherapy for osteosarcoma is among the most grueling of any given for solid tumors, and treatment of the primary tumor is associated with permanent disability of some degree in a significant proportion of patients. New systemic treatments remain beyond the horizon. In recognition of these difficulties, an international cooperation has begun with the opening of a randomized trial, European and American Osteosarcoma (EURAMOS) 1, in Europe and the United States. This study heralds a new era of clinical investigation into osteosarcoma, with the promise of valuable biologic insights and rapid evaluation of investigational strategies. Osteosarcoma should always be treated under the guidance of a specialist team, and we recommend that whenever possible, patients be offered entry into EURAMOS 1 or other well-designed clinical trials.     

Primary cardiac osteosarcoma

Primary cardiac neoplasms are an infrequent disease, as most of the tumors arising in the heart are metastatic. Between the malignant tumors, sarcomas are the most frequent ones, accounting for at least 95% of them. We report the case of a 70-year old woman, diagnosed of primary cardiac osteosarcoma arising in the left atrium. Although complete excision of the tumor was performed, the disease relapsed and subsequently developed metastatic disease. In this paper, we also review briefly the management of this rare disease and the therapeutic options.     

去分化皮质旁骨肉瘤

目的 探讨去分化皮质旁骨肉瘤(Dedifferentiated parosteal osteosarcoma,dd-POS)的临床特点及其治疗。方法 回顾5例dd-POS的临床、影像和组织学资料,总结出dd-POS的临床病理学特点、治疗及其预后。结果 5例患者中3例分别于术后21个月、25个月和31个月死于肺转移,2例无瘤存活50个月和72个月,其中1例在术后1年局部复发,经再次手术得以控制。结论 虽然dd-POS的诊断最终有赖于组织学检查,但影像资料是早期鉴别高度恶性变区最有效的方法。     

Low-grade intraosseous osteosarcoma

A study of 80 well-differentiated osteosarcomas, 16 from the Mayo Clinic files and 64 from our consultation files, revealed that males and females were about equally affected, most of the patients were young adults, and skeletal distribution was similar to the distribution seen in conventional osteosarcoma. Radiographs generally showed poor margination, suggestive of malignancy. Histologically, the tumors were composed of spindle cells with only minimal atypia. Some tumors had bone spicules similar to those seen in fibrous dysplasia. An invasive growth pattern was the most helpful feature in differentiating low-grade osteosarcoma from fibrous dysplasia. Initial treatment was highly variable. Local excision was almost always associated with recurrence. Wide excision was almost never followed by recurrence. The recurrent tumor was a high-grade, conventional osteosarcoma in 15% of the patients, and this was associated with a poor prognosis. Wide excision seems to be the treatment of choice for this very rare variant of osteosarcoma.     

Periosteal Ewing's sarcoma treated by photodynamic therapy with acridine orange

We recently encountered a very rare case of periosteal Ewing's sarcoma (PES), which was treated by surgery followed by photodynamic therapy using acridine orange with radiodynamic therapy. No more than 15 cases of PES have been reported previously in literature. In our case, MRI revealed the tumor to be localized on the cortical surface of the proximal humerus. Histopathological examination of biopsy specimens revealed a small round cell sarcoma suggestive of Ewing's sarcoma or PNET, and immunohistochemistry showed positive staining for MIC2. Although fusion genes EWS-FLI1 and EWS-ERG were undetectable, PES was still considered to be the most likely diagnosis. Therefore, we administered preoperative chemotherapy, as a result of which the tumor shrank to 48% of its original volume. With a view to preserve excellent shoulder and upper limb function, we attempted intralesional tumor resection supported by photodynamic therapy using acridine orange with radiodynamic therapy. After surgery followed by postoperative chemotherapy, the patient has, until the time of writing, had no local tumor recurrence and no evidence of metastatic disease, and can move his shoulder fully and throw a ball well. Since it has been reported that PES has a better prognosis and responsiveness to chemotherapy than intramedullary Ewing's sarcoma, we believe that such reduction surgery with photodynamic therapy might be the strategy of choice to obtain satisfactory limb function in cases of PES.     

Enhanced osteosarcoma growth produced in rats by osteosarcoma allografts.

Transplanted syngeneic osteosarcomas (induced by 32P in DA rats) grew significantly larger in DA rats receiving a simultaneous transplant of allogeneic osteosarcoma than in rats receiving syngeneic tumour only (P less than 0-01). Two other malignant allogeneic tumours, and allogeneic spleen cells, did not alter the growth of the transplanted syngeneic osteosarcomas. When the allogeneic osteosarcoma was given 7 days before the syngeneic tumour, the reverse effect (retardation) occurred. When given 7 days after the syngeneic tumour cells, the effect on both syngeneic and allogeneic tumour growth was variable. Some possible reasons for these findings are discussed, and the argument is presented that immunological phenonema are involved in the reaction.     

骨肉瘤的化疗

骨肉瘤是最常见的原发骨恶性肿瘤.自从化疗引入骨肉瘤的综合治疗,患者的生存率获得了显著的改善,大约2/3的患者可以获得长期生存.本文就骨肉瘤的化疗历史和当前的现状进行述评.骨肉瘤患者应当在专业的骨肿瘤诊治中心接受治疗,我国进行骨肉瘤多中心协作研究己迫在眉睫.