Secondary osteosarcoma: is there a predilection for the chondroblastic subtype?

Osteosarcoma is the most common form of primary bone cancer in the adolescent and young adult patient population. Outcomes in patients with secondary osteosarcoma are inferior compared with outcomes in patients with primary osteosarcoma. The goal of this study was to investigate whether there is a predilection for the chondroblastic histologic subtype in secondary osteosarcoma. A retrospective chart review was performed to identify cases of secondary osteosarcoma treated at 1 institution from 1991 to 2012. Histologic subtypes were evaluated by a pathologist, and a review of the literature was also performed to identify the histologic subclassification of additional series of secondary osteosarcomas. Of a total of 131 cases of osteosarcoma, 9 (6.9%) were identified as a secondary malignancy. Only 2 cases (22%) were identified as chondroblastic variants, although 6 (67%) showed some degree of chondroid differentiation. Of the 3 cases meeting the criteria for postradiation osteosarcoma, 2 (67%) were identified as chondroblastic variants and all 3 showed some degree of chondroid differentiation. Five other studies evaluating histologic subtypes in postradiation osteosarcoma showed a cumulative frequency of 20% for the chondroblastic variant. Although the study results did not support the hypothesis of an association between secondary osteosarcoma and the chondroblastic subtype, the high proportion of cases of postradiation osteosarcoma with the chondroblastic subtype and the even higher proportion showing some degree of chondroid differentiation are noteworthy features of this disease.

Management of craniofacial chondroid tumors.

BACKGROUND: Craniofacial chondroid tumors (CFCTs) constitute less than 1% of all intracranial mass lesions. No protocol for evaluation and management of CFCTs is developed at the moment. MATERIALS AND METHODS: We analyzed 51 patients with CFCTs operated on in Burdenko Neurosurgical Institute from 1980 until 2012, which included chondroma (15), chondroblastoma (3), chondromyxoid fibroma (11), and chondrosarcoma (22). Age varied from 2 to 76 years (mean, 40 y); the series included 23 women and 28 men. All tumors were divided into 4 groups: midline unilateral (8),midline bilateral (21), anterolateral (19), and lateral (3). This division was based on differences in surgical approaches (P = 0.009). RESULTS: All patients underwent surgical treatment. Complete removal was achieved in 20; subtotal, in 21; and partial, in 10. Two patients died, and early complications were observed in 10 cases. Early outcomes correlated with the benign nature of the tumors (P = 0.002). Follow-up data were available in 22 patients. Fifteen of 51 patients were reoperated on because of recurrence (a total of 43 reoperations were performed). The mean recurrence-free period was 45 months. In 3 patients, the tumor metastasized, and malignant transformation was observed in 3 cases. Sixteen patients received postoperative radiation therapy. Delayed sequelae occurred in 5 observations, and 5 patients died during long-term follow-up. Three-year survival in benign and malignant tumors was 87.5% and 55.6%, respectively, and 5-year survival was 83.3% and 40.0%, respectively. CONCLUSION: Surgical resection is the mainstay in treatment of both benign and malignant craniofacial tumors, and adjuvant radiation therapy is mandatory in malignant lesions; however, it should be avoided in benign lesions.

A fatal case of pure metaphyseal chondroblastoma.

The chondroblastoma (CB) is a rare cartilaginous tumour; it represents less than 1% of all bone tumours. It is mostly localised at the level of the epiphysis of long bones. We report a fatal case of pure metaphyseal CB of the tibia in a 9-year-old boy whose pulmonary metastases developed soon after operative therapy of the primary tumour.

Extraskeletal osteosarcoma of the breast in an adolescent girl.

Extraskeletal osteosarcoma (ESOS) is a rare malignancy, usually arising in older adults. We were unable to find reports of children or adolescents affected by an ESOS of the breast. Here, we present the case of a high-grade osteosarcoma arising in the breast of a 16-year-old girl. The tumor was treated with breast-conserving resections and adjuvant multiagent chemotherapy, based on a regimen of doxorubicin, high-dose methotrexate, cisplatin, and ifosfamide. At last follow-up, the patient was in first complete remission, 29 months after initial diagnosis.

Chondroblastoma with pulmonary metastasis in a patient presenting with spontaneous bilateral pneumothorax: Report of a case.

Chondroblastoma is a benign bone tumor with a relatively high incidence in older children and adolescents. Although it is generally regarded as a benign neoplasm, it sometimes grows aggressively or recurs but rarely metastasizes to the lung. We herein present a very rare case of a bilateral pneumothorax due to a pulmonary metastasis from a chondroblastoma. A 21-year-old man developed a bilateral pneumothorax 20 months after an operation for a chondroblastoma of the right ischium. A pertinent literature review revealed similar cases of chondroblastoma with pulmonary metastasis, but revealed no reports of a pneumothorax caused by a metastatic chondroblastoma.

Aggressive scapular chondroblastoma with secondary metastasis--a case report and review of literature.

Chondroblastoma is a benign bone tumor, accounting for approximately one percent of all benign bone tumors. It mostly occurs in typical locations such as long bones. Malignant transformation including metastasis has been described in only a few cases. Therefore, we report a unique case of chondroblastoma with tumor manifestation in the 7th decade of life, location of the tumor in the scapula and occurrence of metastasis in the soft tissue of the mandible branch. Due to aggravation of the clinical course, a scapula en bloc resection was performed. The differential diagnosis is discussed and the current literature concerning malignant transformation of chondroblastoma is reviewed.

Giant chondroblastoma of the scapula with pulmonary metastases.

A 53-year-old man presented with a 12-year history of a progressively growing solid mass at his left shoulder. A 39x30x18-cm and 14.440-kg mass including the scapula was resected. Pathologic features were specific for chondroblastoma. During the 36-month follow-up, he had multiple inoperable metastatic lesions in his lungs. Histology of the transthoracic needle biopsy showed the metastatic nodules had features specific for chondroblastoma; however, the microscopic features additionally had hyperchromasia and increased mitotic activity in some areas. In the English literature, there are a few cases of chondroblastoma located in the scapula. It is exceptional to see this lesion in the sixth decade of life and with pulmonary metastases.

Treatment and prognosis of chondroblastoma.

UNLABELLED: Chondroblastoma is an aggressive tumor of bone with the capacity for recurrence and metastasis. We sought to determine the prognostic factors that affect survival and local recurrence with particular emphasis on surgical technique and the anatomic constraints of the open physis. It was hypothesized that an open growth plate would impact the local recurrence rate negatively and be a primary determinant of treatment outcome. We retrospectively reviewed 82 consecutive patients treated at one institution. Intralesional treatment with meticulous curettage and bone graft resulted in local control in the majority of patients. Four local recurrences developed between 5 and 51 months. An open growth plate was not found to correlate with local recurrence. In most cases, the open physis did not considerably impact surgical technique. Although the median age of the patients was 16 years, the majority of patients had a closed or closing physis. Few patients had substantial growth remaining. A physeal-sparing operation was done in six patients, and no local recurrences were observed in this group. The factors that seemed to affect local recurrence included inadequate surgery and biologic aggressiveness of the tumor. Inadequate surgery was likely to be the cause of local recurrence in patients who presented after previous treatment elsewhere. Three patients who developed local recurrence manifested increased biologic aggressiveness of disease. These patients subsequently developed metastatic disease and malignant transformation of disease. All three patients died from their disease. Pelvic tumors tend to be biologically more aggressive and more apt to recur locally and metastasize to distant locations. LEVEL OF EVIDENCE: Therapeutic Study, Level IV (case series). See the Guidelines for Authors for a complete description of levels of evidence.

Chondroblastoma of bone--not a " benign disease": clinico-pathologic observations on sixty cases.

Chondroblastomas (CBL) account for less than 1% of all bone tumors. A 46 year review (1955-2000) of bone tumors yielded 60 cases of CBL. This paper reviews their histology, radiology, clinical presentation and emphasizes that this seemingly benign lesion is capable of aggressive behaviour. Histopathology slides and radiographs of sixty patients were studied and clinical details and follow-up were obtained. There were 44 males and 16 females, aged between 10 to 30 years. Long bones were involved in 81.4% of the cases, tibia being the commonest site. Symptoms comprised pain (60%), local swelling (25%) and joint symptoms (5%) or a combination of these. Radiologically, an oval sharply demarcated lytic lesion predominantly in the epiphysis was noted in 50% of the cases. On microscopy all lesions revealed chondroblasts having irregular grooved nuclei and osteoclasts with 80% of the tumors showing chondroid differentiation and calcification. Treatment comprised curetting (34 cases), wide excision (21 cases), radical excision (1 case) and was unknown in 4 cases. Follow-up ranging from 2 to 26 years was available in 83.3% of the patients; six (10%) had local recurrence (at 6 months, 8 months, 1 year, 2 years, 3 years and 5 years), two (3.3%) developed lung metastasis (synchronous in one and at 18 months in the other) and spontaneous malignant transformation occurred in one (1.4%) patient after 9 years; subsequent to three local recurrences. CBL, though seemingly benign, can behave in an aggressive manner. Since there are no reliable histological features to predict biologic behaviour, regular long term follow-up is recommended.

Metastatic chondroblastoma with elevated creatine kinase and paraneoplastic neurologic autoimmunity.

A 17-year-old girl presented with chondroblastoma of the fibula and pulmonary metastases. Serum creatine kinase (CK) was elevated for a decade preoperatively and fell to near normal after tumor excision. An episode of aseptic meningitis 3 months later led to serologic detection of three antibody markers of paraneoplastic neurologic autoimmunity that in adult patients are characteristic of thymoma and lung carcinoma: muscle-type acetylcholine receptor (AChR) autoantibody (Ab), type 2 Purkinje cell cytoplasmic Ab (PCA-2), and collapsin response-mediator protein-5-IgG (CRMP-5-IgG). Locally recurrent chondroblastoma was excised 1 year later. Serum CK was elevated, CRMP-5-IgG was positive, PCA-2 was lower, and AChR Ab was undetectable. Three years after diagnosis she has stable pulmonary metastases, with elevated CK, higher PCA-2, positive CRMP-5-IgG, and undetectable AChR Ab. The association of metastatic chondroblastoma, elevated CK, and an evolving profile of paraneoplastic autoantibodies reveals a previously unrecognized immunobiologic dimension of chondroblastoma.

[Comparative study of histopathological patterns of primary tumour and pulmonary metastases of malignant tumours in children and adolescents after complex therapy]. / Badanie porównawcze cech histopatologicznych ogniska pierwotnego i przerzutów plucnych w nowotworach zlosliwych u dzieci i mlodziezy poddanych skojarzonemu leczeniu przeciwnowotworowemu.

The histopathological features of the primary site and 102 pulmonal metastases were compared in the material from 40 malignancies in children and adolescents. All patients were treated by chemotherapy. Among the malignancies 30 were osteogenic sarcomas. A great histological variability in the multiple metastases removed at one surgical procedure and also in the metastases removed in the subsequent surgeries, were found. Summing up, the whole investigation; in 61.8% of metastatic focusses no difference was found in the histopathological patterns seen at the primary site and pulmonary metastases, in 17.6% total regression was observed, in 13.8% dedifferentiation and in 6.8% maturation of the tumours tissue were observed.

Benign metastasizing chondroblastoma: a case report.

BACKGROUND: Metastasis of histologically benign chondroblastoma is a rare event. The authors report a new case, 12th in the literature, wherein multiple lung metastases appeared almost simultaneously with the primary lesion in the right talus bone. METHODS: A histologic evaluation of the primary lesion in the talus and the pulmonary metastasis was performed, and an ultrastructural study of the latter was done. Published literature on metastasizing chondroblastoma was reviewed to identify any consistency in the pattern and the outcome. RESULTS: Metastasis of chondroblastoma is uncommon but well known. Although radiologic and histologic aggressive features have been sought, they do not necessarily correlate with the outcome. CONCLUSIONS: Metastasis in chondroblastoma has been insufficiently stressed in the literature, unlike metastasis in giant cell tumors. The purpose of this case report is not only to document this uncommon event (the 12th case of lung metastasis) but also to emphasize that patients with chondroblastoma may have metastasis at presentation. Hence, all patients need to be evaluated regularly from the onset for possible lung metastasis so that deposits can be detected early for total resection.

Chondroblastoma with multiple distant soft tissue metastases.

A case of metachronous distant soft tissue metastases from a primary rib chondroblastoma, occurring in a 60-year-old man 17 years following resection of the initial lesion, is described. Metastatic chondroblastomas are very rare and almost always involve the lungs. Discussed herein are the clinical, radiologic, and pathologic findings of this unusual case with comparison of our findings to other reports of metastasizing chondroblastomas.

Metastatic malignant chondroblastoma.

A case of malignant chondroblastoma with metastases is reported. The patient initially presented with a lytic lesion in his left pubic ramus. He was treated with curettage, but the lesion recurred 3 years later. After repeated curettage, radiation therapy, and the late development of multiple bone and soft-tissue metastases, he succumbed to his disease 13 years after diagnosis. The surgical pathology from each of his several procedures was reviewed. Although no definite malignant transformation was apparent, a metastatic deposit curetted 3 months prior to death showed some increase in mitotic activity. Flow cytometry of specimens from the patient's first local recurrence and a late distant metastasis was performed and revealed the interval development of a minor aneuploid peak between the two samples. This fatal chondroblastoma is the only one in our series of 80 patients treated over the past 25 years.

Chondromyxoid fibroma-like osteosarcoma: a distinct variant of low-grade osteosarcoma.

Chondromyxoid fibroma-like osteosarcoma is a recently described, extremely rare subtype of low-grade osteosarcoma. Two such cases were encountered among 102 cases of osteosarcoma seen in the Prince of Wales Hospital, Hong Kong, between 1984 and 1994. The first patient, a 39-year-old woman, presented with a mass in her right maxilla which was resected and mistaken as a myxoma. The tumour recurred locally four years later and she now has extensive local recurrent disease six years after initial presentation and is amenable to support treatment only. The second patient, a 28-year-old man, had a pelvic tumour which recurred in the form of a polypoid left atrial tumour and pulmonary nodules six years after operation. The left atrial tumour recurred one year after operation, and led to sudden death of the patient seven years after initial presentation. Radiologically, the tumours in both cases appeared as expansile osteolytic lesions with erosion of adjacent bone and infiltration into soft tissue. Histologically, they consisted of lobules of spindle, stellate or polygonal tumour cells showing mitotic activity and with moderate nuclear pleomorphism and hyperchromatism, set in a highly myxoid stroma superficially mimicking chondromyxoid fibroma. The histological hallmark was the direct production of osteoid by tumour cells. Chondromyxoid fibroma-like osteosarcoma merits recognition as a distinct variant of low-grade osteosarcoma for which early appropriate surgery is indicated.