Telangiectatic Osteosarcoma Research Articles

Telangiectatic Osteosarcoma of the Patella

Although osteosarcoma is the most common primary malignancy of bone, it has only been reported to arise from the patella in a handful of cases. Telangiectatic osteosarcoma accounts for <5% of all osteosarcomas, and it is distinguished histologically by spaces, often blood filled, separated by septa containing highly malignant cells and radiographically by a predominately lytic and/or expansile component. Telangiectatic osteosarcoma can be radiologically confused with aneurysmal bone cyst or giant cell tumor. A 22-year-old otherwise healthy man presented with increasing pain, swelling, and limited flexion of the right knee after failing physical therapy for anterior knee pain. Standard anteroposterior and lateral radiographs demonstrated a diffuse destructive process involving the majority of the patella (including loss of the inferior patellar cortex) and a lytic lesion of the proximal tibia. Apparent osteoid matrix was visible in the soft tissue extension along the inferior pole of the patella. A computed tomography scan of the chest showed 2 pulmonary nodules consistent with metastatic disease. Evaluation of core needle biopsy showed osteosarcoma with telangiectatic features. Given that the majority of the tumor involved the patella/extensor mechanism, it was clear that the tumor originated in the patella. This case presents the first published report of a telangiectatic osteosarcoma arising from the patella.

Telangiectatic osteosarcoma of the spine: a case report

Telangiectatic osteosarcoma (TOS) of the spine is rare accounting for only 0.08% of all primary osteosarcomas. Though a well described radio-pathological entity it is not often thought of as a cause of paraplegia. We describe the clinical, radiological and pathological features and discuss the treatment options of telangiectatic osteosarcoma of the dorsal spine presenting in a young man. The diagnostic pitfalls are discussed emphasising the fact that the diagnosis of TOS of the spine requires not only a multi modal approach of appropriate radiological and pathological tests but also an awareness of this condition.

Telangiectatic osteosarcoma: The St. Jude Children's Research Hospital's experience

Telangiectatic osteosarcoma (TOS) is a rare subtype of osteosarcoma (OS). The authors reviewed their experience with TOS to characterize its histologic, radiologic, and clinical features. The authors reviewed records, pathology material, and imaging studies from all patients with TOS who were treated between 1978 and 2005 and compared their outcomes with the outcomes of patients with all other subtypes of high-grade osteosarcoma (OS). Among 323 patients with OS, 22 patients (6.8%) had TOS. Two additional patients who were treated in Chile on a recent OS trial were included. The median age at diagnosis of the 24 patients was 15.7 years. Four patients (17%) had metastatic disease, and 9 of 21 patients (43%) had pathologic fractures. Only 5 patients (who were treated after 1994) underwent limb-salvage surgery. Estimates of 5-year event-free survival (58.3% +/- 11.9%) and overall survival (66.8% +/- 11.6%) were similar to those for patients with other OS subtypes (P > or = .85). The absence of local disease progression and chemotherapy with > or =3 agents that were active against OS were correlated with improved outcome (P < or = .005). The presence of a pathologic fracture was not associated with surgery type or patient outcome. TOS was associated with a high rate of pathologic fracture. With multimodality therapy, the outcome of patients with TOS was similar to that of patients with other high-grade OS subtypes. The absence of local disease progression and chemotherapy with > or =3 active agents were associated with a favorable outcome.

Sarcomatoid Basal Cell Carcinoma—Predilection for Osteosarcomatous Differentiation

Primary cutaneous carcinomas rarely show heterologous malignant mesenchymal differentiation. We report 11 cases of sarcomatoid basal cell carcinoma (BCC) with osteosarcomatous differentiation. The patients (7 men and 4 women) ranged in age from 61 to 92 years (median 75 y). The tumors presented as exophytic nodules (0.3 to 7 cm) on the head (n=6), upper limb (n=3), and lower limb (n=2). All lesions were completely excised. Seven patients were alive without evidence of disease (follow-up interval 5 to 24 mo) and 1 patient died of unrelated causes at 7 months without evidence of disease. On histology, the tumors were dermal in location with 2 cases showing focal subcutaneous involvement. Ten tumors were well-circumscribed and 1 tumor showed focally infiltrative edges. Ten tumors revealed conventional BCC associated with varying proportions of osteosarcomatous and undifferentiated sarcomatous stroma. Transition from neoplastic epithelial to mesenchymal cells was seen in 8 cases. One case showed a purely osteoclastic giant cell rich malignant mesenchyme, interpreted as representing early stages of osteosarcomatous transformation. Previously unreported in sarcomatoid BCC, the mesenchymal component of another two cases displayed predominant malignant giant cell tumor like areas and 1 further case disclosed areas reminiscent of telangiectatic osteosarcoma. Pancytokeratins (AE1/3 and MNF116) and smooth muscle actin stained occasional undifferentiated sarcomatous cells in 2 and 3 tumors, respectively. MNF116 and EMA were focally positive in osteosarcomatous tumor cells of 1 case. Although the follow-up interval is short, our data suggest an excellent prognosis for polypoid and exophytic sarcomatoid BCC after complete surgical resection.

Prevalence, extension and characteristics of fluid-fluid levels in bone and soft tissue tumors

The purpose of this study was to determine the prevalence, extension and signal characteristics of fluid-fluid levels in a large series of 700 bone and 700 soft tissue tumors. Out of a multi-institutional database, MRI of 700 consecutive patients with a bone tumor and MRI of 700 consecutive patients with a soft tissue neoplasm were retrospectively reviewed for the presence of fluid-fluid levels. Extension (single, multiple and proportion of the lesion occupied by fluid-fluid levels) and signal characteristics on magnetic resonance imaging of fluid-fluid levels were determined. In all patients, pathologic correlation was available. Of 700 patients with a bone tumor, 19 (10 male and 9 female; mean age, 29 years) presented with a fluid-fluid level (prevalence 2.7%). Multiple fluid-fluid levels occupying at least one half of the total volume of the lesion were found in the majority of patients. Diagnoses included aneurysmal bone cyst (ten cases), fibrous dysplasia (two cases), osteoblastoma (one case), simple bone cyst (one case), telangiectatic osteosarcoma (one case), "brown tumor" (one case), chondroblastoma (one case) and giant cell tumor (two cases). Of 700 patients with a soft tissue tumor, 20 (9 males and 11 females; mean age, 34 years) presented with a fluid-fluid level (prevalence 2.9%). Multiple fluid-fluid levels occupying at least one half of the total volume of the lesion were found in the majority of patients. Diagnoses included cavernous hemangioma (12 cases), synovial sarcoma (3 cases), angiosarcoma (1 case), aneurysmal bone cyst of soft tissue (1 case), myxofibrosarcoma (1 case) and high-grade sarcoma "not otherwise specified" (2 cases). In our series, the largest reported in the literature to the best of our knowledge, the presence of fluid-fluid levels is a rare finding with a prevalence of 2.7 and 2.9% in bone and soft tissue tumors, respectively. Fluid-fluid levels remain a non-specific finding and can occur in a wide range of bone and soft tissue tumors, both benign and malignant. Therefore, they cannot be considered diagnostic of any particular type of tumor, and the diagnosis should be made on the basis of other radiological and clinical findings.

Osteossarcoma e leucemia mielóide aguda: dois casos em crianças

A ocorrência de segunda neoplasia após quimioterapia é mais comum em pacientes com doença de Hodgkin e tumores cerebrais.1-4 Na literatura há poucos relatos de ocorrência de leucemia mielóide aguda (LMA) após tratamento para osteossarcoma. Os autores relatam dois casos de osteossarcoma que evoluíram com LMA após tratamento com múltiplos agentes quimioterápicos. O primeiro caso teve diagnóstico de osteossarcoma teleangectásico, foi tratado com carboplatina, ifosfamida, epirrubicina e metotrexato e desenvolveu LMA trinta e oito meses após o início do tratamento. O segundo caso teve diagnóstico de osteossarcoma condroblástico, foi tratado com cisplatina, doxorrubicina e ifosfamida e desenvolveu LMA seis meses após o início do tratamento. Os autores discutem a provável relação entre o uso de agentes alquilantes, inibidores da topoisomerase II e compostos da platina e o desenvolvimento de LMA.

Complete Segmental Resection of the Spine, Including the Spinal Cord, for Telangiectatic Osteosarcoma

Two case reports of telangiectatic osteosarcoma treated with complete segmental resection of the spine, including the spinal cord. To report the en bloc tumor excision, including the spinal cord, for telangiectatic osteosarcoma, and discuss the indication of cord transection and influence after cutting the spinal cord. To our knowledge, there are no previous reports describing telangiectatic osteosarcoma of the spine and the subsequent en bloc excision of the spine, including the spinal cord. The clinical and radiographic presentations of 2 cases with telangiectatic osteosarcoma are presented. Because these 2 cases already had complete paralysis for at least 1 month, it was suspected that there was no possibility of recovering spinal cord function. Complete segmental spinal resection (total en bloc spondylectomy) was performed. At that level, the spinal cord was also cut and resected. En bloc excision of the tumor with a wide margin was achieved in both cases. In the resected specimen, the nerve cells in the spinal cord had lapsed into degenerative necrosis. The pathologic findings showed that there was no hope for recovery of spinal cord function. En bloc spinal resection, including the spinal cord, is an operation allowed when there is no hope for recovery of spinal cord function. This surgery should be accepted as an option in spine tumor surgeries.

Neglected Telangiectatic Osteosarcoma of the Clavicle in a Child

We present a case of telengectietic osteosarcoma of the clavicle in a ten-year-old female child. The tumor was neglected for more than six months and no treatment was taken. The case report highlights the neglect of bone tumors in developing countries to a stage where they become untreatable. The malignant nature of the lesion with limited treatment modalities at hand makes the management of these tumors extremely difficult with poor outcome.

CASE REPORTS: Malignant Transformation of Aneurysmal Bone Cysts

An aneurysmal bone cyst is an uncommon benign primary bone tumor. Careful intralesional curettage through a wide cortical window in addition to cauterization with or without adjuvant therapy (phenol or hydrogen peroxide) and bone grafting or cementation is the preferred surgical treatment. Adjuvant or primary radiation of an aneurysmal bone cyst rarely is used because of its association with malignant transformation of the lesion. Several cases of late malignant transformation of primary aneurysmal bone cysts without adjuvant radiation have been reported. We provide additional documentation of two primary aneurysmal bone cysts treated surgically with careful intralesional curettage through a wide cortical window and allograft bone grafting without adjuvant radiation. At 5.5 years and 12 years after treatment, a telangiectatic osteosarcoma and a fibroblastic osteosarcoma, respectively, were identified in the site of the original lesions. Not only should aneurysmal bone cysts be evaluated carefully through histologic examination at presentation, patients also should be counseled regarding possible recurrence and the need for routine followups, especially if symptoms change.

Grade of chemotherapy-induced necrosis as a predictor of local and systemic control in 881 patients with non-metastatic osteosarcoma of the extremities treated with neoadjuvant chemotherapy in a single institution

To determine whether necrosis induced by pre-operative chemotherapy correlates with the rate of systemic and local relapse, may change the pattern of relapse and/or may modify the chance of success of post-relapse treatments, we evaluated 881 patients with non-metastatic osteosarcoma of the extremities treated with five different protocols of neoadjuvant chemotherapy and surgery at the same institution between 1983 and 1999. The 5-year disease-free survival (DFS) and overall survival (OS) correlated significantly with the histological response to chemotherapy. Five-year DFS and OS in good and poor responders were 67.9% versus 51.3% ( P < 0.0001) and 78.4% versus 63.7% ( P < 0.0001), respectively. The prognostic value of the histological response was valid only for osteoblastic and telangiectatic osteosarcoma subtypes. Nonetheless, since they represent more than 70% of all osteosarcomas, we conclude that chemotherapy-induced necrosis has a significant prognostic value, regardless of the type of chemotherapy performed after surgery.

Ostéosarcome télangiectasique de l’omoplate: À propos d’un cas

Telangiectatic osteosarcoma is a rare entity and rarely involves flat bones. The authors report the case of a 4 year old child, with a four month history of progressive left shoulder swelling. Radiographs and CT of the left shoulder revealed a large lytic lesion with cystic areas of the scapula, simulating the appearance of an aneurysmal bone cyst. Surgical biopsy was performed and showed telangiectatic osteosarcoma.

Osteosarcoma at the Site of a Previously Treated Aneurysmal Bone Cyst

Hsu, Chia-Chen MD1; Wang, Jun-Wen MD1; Huang, Cheng-Hua MD1; Chen, Wei-Jen MD1 Author Information

Segmental cement extraction system (SEG-CES) and the Ilizarov method in limb salvage procedure after total knee cemented prosthesis removal in a former osteosarcoma patient

Survival of osteosarcoma has greatly improved in the past few decades. Knee prosthesis is a well-recognized limb salvage procedure for osteosarcoma of the distal end of the femur. One drawback is that prostheses have a limited life and prosthetic failure with the inherent high rate of reoperations remains a serious long-term problem for former osteosarcoma patients. The segmental cement extraction system (SEG-CES) is a technique to remove cement in arthroplasty revision, based on a cement-bone interface with a lower strength compared to the old cement-new cement interface. We report the case of a 32-year-old former osteosarcoma patient in whom the SEG-CES was applied to remove a long-stemmed total knee cemented prosthesis. The prosthesis was placed 17 years before for a recurrent telangiectatic osteosarcoma of the left femur. Thirteen years after the prosthesis implantation, the patient complained of knee instability, pain, and complete failure of the extensor apparatus. The extraction of the prosthesis was performed using cylindrical batters of diameter corresponding to the diameter of the axle, two hammer extractors clamping the prosthesis components between two jaws. Extraction of the periprosthetic cement in the femoral and tibial components was done using the SEG-CES technique. The successful prosthesis removal performed in this patient allowed us to perform an external fixation with bone lengthening and reconstruction by the Ilizarov method.

FNAC of osteosarcomas - A report of 31 cases

A total of 296 tumour and tumour - like lesions of the bone were studied between 1990 and 2003 (14 years) in Goa medical college and we report the utility of FNAC in the diagnosis of osteosarcomas. Osteosarcomas comprised 31 cases out of 296 and they were the most common malignant primary bone tumour. Of these, 27 were conventional osteosarcomas, 2 cases were small cell osteosarcomas, one case was telangiectatic type of osteosarcoma and one was multicentric or multifocal osteosarcoma. In this paper the utility of FNAC in these lesions along with the clinical and radiological correlation is also discussed. For the diagnosis of osteosarcoma great importance is to be given to the age of the patient, site in the bone, type of bone affected, the radiologic and the cytologic picture. The cytology smears are hypercellular or moderately cellular with hyperchromatic, pleomorphic, bizarre cells, mononuclear as well as multinucleated tumour giant cells with osteoid. The cytology must be evaluated in a total clinical - radiological and pathological scenario. Small cell osteosarcomas can be confused with Ewing's sarcoma and malignant lymphoma. Telangiectatic osteosarcoma yields mainly blood on aspiration. FNAC is also invaluable in the diagnosis of multicentric or multifocal osteosarcoma.

Total en bloc spondylectomy including resection of part of the spinal cord for telangiectatic osteosarcoma: A report of 2 cases

Total en bloc spondylectomy including resection of part of the spinal cord for telangiectatic osteosarcoma: A report of 2 cases

Aneurysmal bone cyst of the acetabulum: a case report and long term follow-up

Aneurysmal bone cysts (ABCs) are benign, non-neoplastic, expansile, osteolytic lesions of unknown etiology. ABCs are most commonly located in the metaphysis of long bones. Differential diagnoses include unicameral bone cyst, chondromyxoid fibroma, giant cell tumour, osteoblastoma and telangiectatic osteosarcoma. We describe a case of a 14-year-old girl who presented to the accident and emergency department with an 18-h history of right-sided medial thigh pain. Plain radiology of the pelvis demonstrated a well-circumscribed lytic lesion with sclerotic margins in the medial part of the right acetabulum. Curettage and primary autogenous bone grafting was performed. The diagnosis of this condition requires a high index of suspicion. This report demonstrates that with adequate surgical treatment, a long-term, excellent outcome is achievable.

Five-year evolution of a telangiectatic osteosarcoma initially managed as an aneurysmal bone cyst

We present the clinical, radiographic, and pathologic features of a telangiectatic osteosarcoma (TOS) of the right femoral neck in a 20-year-old man which was initially diagnosed and managed as an aneurysmal bone cyst (ABC). The lesion recurred twice. At the second recurrence TOS was diagnosed. The first local recurrence was recognized 4 years 8 months after the first operation. The clinical diagnosis for the recurrent lesion was recurrent ABC, and curettage and bone graft with internal fixation were performed. The second local recurrence was observed 8 months after the second surgery. The right lesser trochanter appeared destroyed on the radiograph, and a large medial soft tissue mass was demonstrated by computed tomography. The patient underwent wide resection of the tumor with prosthetic replacement of the right proximal femur. The histologic section for this lesion showed a blood-filled cystic lesion, and its wall contained sarcomatous cells with atypical mitoses and tumor osteoid. The histologic diagnosis for the second recurrent lesion was high-grade TOS. The retrospective review of the histologic section for the primary lesion showed similar features to ABC except for a few bizarre cells without mitosis in the tissue of cystic wall. The patient demonstrates no evidence of disease 13 months after the last surgery without adjuvant therapy (he declined chemotherapy).

Soft tissue telangiectatic osteosarcoma in a young patient: imaging and immunostains

Telangiectatic osteosarcoma of the soft tissue is rare and generally affects adults older than 50 years of age. We present the case of a 17-year-old male who developed a mass in the right thigh. The CT and MRI findings suggested a malignant hemorrhagic tumor without discernable osteoid matrix. Differential diagnosis included various hemorrhagic mesenchymal tumors. Histology revealed large multiple cysts lined with septae and filled with numerous giant cells, pleomorphic spindle cells, and occasionally an osteoid-like substance. Immunostaining for osteocalcin was positive in the cytoplasm of both stromal mononucleate and multinucleate giant cells. Surgery and adjuvant chemotherapy were applied and the patient survives to the time of writing after 30 months of follow-up without recurrence or metastasis.

Multifocal intrafollicular granulosa cell tumor of the ovary associated with an unusual germline p53 mutation

A 23-year-old woman presented with a 7 cm right multicystic mass in the ovary, which corresponded microscopically to an unusual lesion consisting of a multifocal granulosa cell tumor with intrafollicular (‘in situ‘) growth involving two-thirds of mature follicles. Stromal invasion was found in only one area where neoplastic follicles coalesced. Granulosa cells had atypical, bizarre TP53 positive nuclei with hyperchromatism, abundant mitoses and numerous hyaline globules. The contralateral ovary was normal. From the age of 10 years, the patient had a complex medical history of multiple tumors, including telangiectatic osteosarcoma, typical and malignant phyllodes tumor, reticulohistiocytoma of skin, carcinomas of the breast and lipo- and leiomyosarcoma. The female genital tract also harbored myometrial leiomyomas and an early endometrial carcinoma. Retrospective histologic study of all mesenchymal neoplasms in this patient showed, the conspicuous presence of similar bizarre TP53 positive cells with hyaline globules in all the mesenchymal neoplasms. In the genetic study, a germline p53 gene mutation was detected in exon 10, codon 336, generating a stop codon in the oligomerization domain of the protein (E336X). A further p53 mutation was found in exon 7 in the granulosa cell tumor. Mutation occurred de novo since there was no history of tumors in any family members, all of whom had a wild-type p53. Although this patient shows a typical tumor phenotype of Li Fraumeni syndrome, the germline mutation corresponded to a highly unusual mutated domain, which is similar to the one found in childhood malignant adrenocortical tumor; also a rare neoplasm that originates in adrenocortical cells; which are closely related, both functionally and embryologically, to granulosa cells.

Telangiectatic osteosarcoma: radiologic-pathologic comparison.

To describe the imaging characteristics of a large series of telangiectatic osteosarcomas with pathologic findings for comparison. The authors retrospectively reviewed 40 pathologically confirmed telangiectatic osteosarcomas. Patient demographics and images from radiography (n = 36), bone scintigraphy (n = 17), angiography (n = 4), computed tomography (CT) (n = 25), and magnetic resonance (MR) imaging (n = 27) were evaluated by three authors in consensus for lesion location, size, and intrinsic characteristics. There were 27 men (68%) and 13 women (32%) in the study, with an age range of 4-83 years (mean age, 24 years). Lesions frequently affected the femur, tibia, and humerus. Radiographs showed geographic bone lysis, a wide zone of transition, and matrix mineralization. CT demonstrated low attenuation, MR demonstrated high signal intensity on T2-weighted images, and both demonstrated hemorrhage, which simulated the appearance of aneurysmal bone cyst. Viable sarcomatous tissue surrounding hemorrhagic and/or necrotic regions was best seen at contrast material-enhanced CT and MR imaging, with thick peripheral, septal, and nodular enhancement in all cases. Subtle matrix mineralization in this viable tissue was best seen at CT. An associated soft-tissue mass was also seen in 19 of 25 cases (76%) at CT and in 24 of 27 cases (89%) at MR imaging. CT and MR imaging findings of telangiectatic osteosarcoma often include thick nodular tissue (and matrix mineralization at CT) in a largely hemorrhagic and/or necrotic osseous lesion with an associated soft-tissue mass, which allows distinction from aneurysmal bone cyst.