In this study, for the first time, we have isolated, established, and characterized a cell line of cancer stem cells cscs from. Pdf establishment and characterization of a human small cell osteosarcoma cancer stem cell line. Small cell osteosarcoma may present a challenging primary diagnosis on cytologic assessment owing to its rarity and its. Small cell osteosarcoma successfully treated by highdose. Osteosarcoma cell an overview sciencedirect topics. Wide surgical excision of small cell osteosarcoma and removal of the entire lesion is the standard treatment mode. Synchronous multifocal osteosarcoma with small cell. Multidrugresistant varieties of osteosarcoma are a case in point. Small cell sarcoma an overview sciencedirect topics.
The small amount of osteoid formation makes these tumors a challenge for the pathologist. Pdf osteosarcoma is a malignant tumor that primarily affects the long bones but can also involve other bones in the body. The aim of this article is to highlight such an entity at an unusual sitethe. Pulmonary metastases appeared after a gap of 9 months of ablative surgery, as did late multiple extrapulmonary skeletal metastases, including the hand and. The malignant cells produce immature woven bone, or osteoid, which is why the tumor is named osteosarcomait is a boneproducing sarcoma. Small cell osteosarcoma is an exceedingly rare tumor, estimated to account for pdf. Differential diagnosis includes small round blue cell tumors like ewing sarcomaprimitive neuroectodermal tumor. This article describes a 60yearold woman presenting with a large, lobulated, painful mass in left thigh with associated history of trauma since 18 months. In contrast to ewing sarcoma, which is extremely rare in older adults, there is a bimodal age distribution of osteosarcoma incidence, with peaks in early adolescence and in adults over the age of 65.
Small cell osteosarcoma of bone, first defined in 1979, 1 is a rare form of osteosarcoma that poses unique diagnostic and treatment considerations. One study suggests similar eventfree survival rates for mfh. Small cell osteosarcoma memperlihatkan lesi litik permeatif, destruksi korteks, massa jaringan lunak, reaksi periosteal, serta kalsifikasi matriks osteoid. The defining histologic feature of small cell osteosarcoma is the presence of osteoid, which has been described in each and every case reported. To distinguish small cell osteosarcoma from other primary small cell malignancies of bone, we evaluated the immunohistochemical ihc expression of cd99 and satb2, a marker of osteoblastic. Children and teens with osteosarcoma may experience the following symptoms or signs. A new possible in vitro model for discovering small cell osteosarcoma biology pdf osteosarcoma. Small round cell tumors of bone archives of pathology. Osteosarcomas of the jaws are extremely rare, representing about 7% of all osteosarcomas and 1% of all head and neck malignancies. A malignant tumor in which the cells synthesize bone the most common primary malignant solid tumor of bone plasma cell myeloma is actually the most common primary bone tumor but its not a solid tumor cancer treat res 2009. Small cell osteosarcoma is a rare tumour that histologically mimics ewing sarcoma, mesenchymal chondrosarcoma and lymphoma, the presence of osteoid being diagnostic. Mfh of bone is treated according to osteosarcoma treatment protocols. An osteosarcoma os or osteogenic sarcoma ogs or simply bone cancer is a cancerous tumor in a bone. Osteosarcoma os is a common primary malignant tumor of bone that produces osteoid matrix.
Small cell osteosarcoma american journal of clinical. Smallcell osteosarcoma, a subtype of osteogenic sarcoma, consists of sheets of round cells that produce an osteoid matrix. To differentiate between the two cancers, your doctor will look for the production of osteoid, a characteristic of osteosarcomas. Early identification of such patients perhaps at initial biopsy would allow a tailored approach. Specifically, it is an aggressive malignant neoplasm that arises from primitive transformed cells of mesenchymal origin and thus a sarcoma and that exhibits osteoblastic differentiation and produces malignant osteoid osteosarcoma is the most common histological form of primary bone cancer. The lesions demonstrated a wide variation in radiographic appearance.
This tumor must be differentiated from other small cell malignancies because of treatment considerations, particularly patient response to chemotherapy. The patients were young 631 years of age and presented with symptoms of pain andor swelling of 110 months duration. Osteosarcoma has an incidence rate of about 1 in 1 million. Small cell osteosarcoma american journal of clinical pathology. One of the rare histological variant is small cell osteosarcoma which consists of sheets of. Unfortunately, the patient died of rapid progression of the osteosarcoma within a week of admission to the hospital. Small cell osteosarcoma, once thought to be a variant of another tumor type, now appears to be a recognized variant of osteosarcoma.
However, peroperatively the lesion had a malignant aspect. Or, the cause of a symptom may be a different medical condition that is not cancer. Small cell osteosarcoma is an exceedingly rare tumor, estimated to account for less than 1% of all cases of osteosarcoma. Small cell osteosarcoma of bone nakajima 1997 cancer wiley. Small cell variant of osteosarcoma on histopathology was another interesting and unusual finding found in the present case, which makes the case even rarer. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. Low grade central osteosarcoma memperlihatkan lesi litik destruktif ekspansil, disrupsi korteks, massa jaringan lunak dan reaksi periosteal. Endobronchial metastasis as an uncommon pattern of. Any combination of chemotherapy, radiation therapy, and invasive procedures, maybe used to treat the tumor.
Juxtacortical osteosarcoma this type is the second most common and accounts for between 10 and 15 percent of all diagnoses. It may be confused with ewing sarcoma if the osteoid matrix is not included in the biopsy. Small cell osteosarcoma is a rare intramedullary subtype, comprising. Supplementary information s2 commonly used osteosarcoma cell lines pdf 154 kb. A 10yearold child presented with small cell osteosarcoma of the proximal end of the tibia. As noted previously, osteosarcoma is an uncommon tumor. These osteosarcomas develop on the outer surface of the bones or the. The cancer malignant cells make immature bone cells osteoid. Histologically, the tumors were composed of small, round cells that produced. The distinctive radiographic features of an osteoblastic tumor and a pattern of permeative destruction will confirm the histologic diagnosis or indicate the true nature if tumor. Seventynine cases of small round cell tumors involving bone were studied in an attempt to learn whether the immunohistochemical features of the lesions might. We present a case of a 28yearold female who had classic signs, symptoms, and radiographic features of the lesion. An osteosarcoma is so called, because it is a cancerous tumor that is derived from a mesenchymal stem cell precursor thus, by definition a sarcoma. Small cell osteosarcoma is composed of small cells with variable degrees of.
Small cell osteosarcoma sco, composed of small round cells, which mimic ewing sarcoma cells 1, is the most rare variant of osteosarcoma, comprising. Extraskeletal osteosarcoma is a rare malignant mesenchymal neoplasm and its small cell variant is one among the rarest variant. Small cell osteosarcoma sco is a rare but distinct variant of osteosarcoma. Small cell osteosarcoma of bone nakajima 1997 cancer. Common subtypes include osteoblastic, condroblastic, fibroblastic, smallcell and epithelioid. There are mostly solid areas of small round or spindle cells that may resemble other small cell sarcomas, such as ewing sarcoma. Based on the clinicopathological findings, the patient was diagnosed as having secondary osteosarcoma occurring as a result of the chemoradiotherapy administered previously for the recurrent nonsmallcell lung cancer. Small round cell osteosarcoma is a very rare type of osteosarcoma, histologically mimicking other small round cell malignancies of bone, most notably ewing sarcoma. Pdf a small cell osteosarcoma with multiple skeletal. Small cell osteosarcoma is a variant of osteosarcoma composed of small cells and varying amount of osteoid. This variant needs different management protocol, being nonradiosensitive and behaving more aggressively than conventional osteosarcoma. Sometimes, children and teens with osteosarcoma do not have any of these changes. Clinicopathologic, immunohistochemical, and molecular analysis of 36 cases small round cell osteosarcoma is a. Mfh should be distinguished from angiomatoid fibrous histiocytoma, a lowgrade tumor that is usually noninvasive, small, and associated with an excellent outcome with surgery alone.
A 10yearold child presented with smallcell osteosarcoma of the proximal end of the tibia. Mall cell osteosarcoma sco was first reported by sim et al. From basic information about cancer and its causes to indepth information on specific cancer types including risk factors, early detection, diagnosis, and treatment options youll find it here. Conventional central osteosarcoma, such as osteoblastic, chondroblastic, fibroblastic, and mixed types. Intraosseous welldifferentiated, or lowgrade, osteosarcoma. When examined under a microscope, small cell osteosarcoma is made up of small, round blue cells. A rare tumor usually occurring in persons age 1030 years patients usually present with pain and swelling of variable duration tumor most frequently located in distal femur and humerus, most are metaphyseal lesion may be sclerotic or mixed osteolyticoste. Osteosarcoma osteogenic sarcoma in children cedarssinai. Mild pleomorphism and nuclear hyperchromasia suggest the diagnosis of small cell osteosarcoma. Small cell osteosarcoma, a rare histological subtype, has very infrequently been reported in the mandible.
The anatomic distribution of small cell osteosarcoma is similar to that. Pdf establishment and characterization of a human small. Histologically, the tumor may be difficult to distinguish from other small round cell malignancies, especially ewings sarcoma, malignant lymphoma, and mesenchymal chondrosarcoma. Whether you or someone you love has cancer, knowing what to expect can help you cope. Treatment measures for small cell osteosarcoma include the following. Osteosarcomas are rare, highly malignant, bone tumors defined by the presence of malignant mesenchymal cells producing osteoid or immature bone. The tumors have a similar appearance to ewing sarcomaanother type of bone cancer.
Small cell osteosarcoma, a variant of osteosarcoma, was first reported by sim et al. These cell lines have been shown to be genetically encoded with a certain membranebound glycoprotein that helps render these cancer cells immune to many chemotherapeutic agents. On microscopic analysis, small cell osteosarcoma shows scanty osteoid formation. Osteosarcoma childhood and adolescence symptoms and signs.
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