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. The tumors have a similar appearance to ewing sarcomaanother type of bone cancer. Histologically, the tumor may be difficult to distinguish from other small round cell malignancies, especially ewings sarcoma, malignant lymphoma, and mesenchymal chondrosarcoma. Differential diagnosis includes small round blue cell tumors like ewing sarcomaprimitive neuroectodermal tumor. One study suggests similar eventfree survival rates for mfh. 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. The patients were young 631 years of age and presented with symptoms of pain andor swelling of 110 months duration. Small cell osteosarcoma successfully treated by highdose. 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. Treatment measures for small cell osteosarcoma include the following.
Osteosarcoma childhood and adolescence symptoms and signs. 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. Common subtypes include osteoblastic, condroblastic, fibroblastic, smallcell and epithelioid. Synchronous multifocal osteosarcoma with small cell. Smallcell osteosarcoma, a subtype of osteogenic sarcoma, consists of sheets of round cells that produce an osteoid matrix.
A 10yearold child presented with small cell osteosarcoma of the proximal end of the tibia. 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 sco is a rare but distinct variant of osteosarcoma. Pdf establishment and characterization of a human small cell osteosarcoma cancer stem cell line. Extraskeletal osteosarcoma is a rare malignant mesenchymal neoplasm and its small cell variant is one among the rarest variant. Low grade central osteosarcoma memperlihatkan lesi litik destruktif ekspansil, disrupsi korteks, massa jaringan lunak dan reaksi periosteal. It may be confused with ewing sarcoma if the osteoid matrix is not included in the biopsy. Small round cell tumors of bone archives of pathology. Conventional central osteosarcoma, such as osteoblastic, chondroblastic, fibroblastic, and mixed types.
Small cell osteosarcoma is a rare intramedullary subtype, comprising. Clinicopathologic, immunohistochemical, and molecular analysis of 36 cases small round cell osteosarcoma is a. Small cell variant of osteosarcoma on histopathology was another interesting and unusual finding found in the present case, which makes the case even rarer. Pdf osteosarcoma is a malignant tumor that primarily affects the long bones but can also involve other bones in the body. Small cell osteosarcoma of bone is a rare form of osteosarcoma, with an incidence rate of 1. Small cell osteosarcoma, once thought to be a variant of another tumor type, now appears to be a recognized variant of osteosarcoma.
Osteosarcoma has an incidence rate of about 1 in 1 million. Pdf a smallcell osteosarcoma with multiple skeletal. The symptoms of osteosarcoma depend on the bone in which the tumor developed. Small cell osteosarcoma, a rare histological subtype, has very infrequently been reported in the mandible. When examined under a microscope, small cell osteosarcoma is made up of small, round blue cells. Or, the cause of a symptom may be a different medical condition that is not cancer. 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. Multidrugresistant varieties of osteosarcoma are a case in point. Small cell osteosarcoma may present a challenging primary diagnosis on cytologic assessment owing to its rarity and its. Whether you or someone you love has cancer, knowing what to expect can help you cope. Histologically, the tumors were composed of small, round cells that produced. Small cell osteosarcoma of bone nakajima 1997 cancer. The anatomic distribution of small cell osteosarcoma is similar to that. Small cell osteosarcoma is a rare tumour that histologically mimics ewing sarcoma, mesenchymal chondrosarcoma and lymphoma, the presence of osteoid being diagnostic.
To differentiate between the two cancers, your doctor will look for the production of osteoid, a characteristic of osteosarcomas. 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. Small cell osteosarcoma is an exceedingly rare tumor, estimated to account for less than 1% of all cases of osteosarcoma. B osteoid produced by tumor cells was found after thorough sampling of the specimen, confirming the diagnosis of small cell osteosarcoma. As noted previously, osteosarcoma is an uncommon tumor. These osteosarcomas develop on the outer surface of the bones or the. Mall cell osteosarcoma sco was first reported by sim et al. Seventynine cases of small round cell tumors involving bone were studied in an attempt to learn whether the immunohistochemical features of the lesions might. 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. One of the rare histological variant is small cell osteosarcoma which consists of sheets of. Any combination of chemotherapy, radiation therapy, and invasive procedures, maybe used to treat the tumor. Small cell osteosarcoma, a variant of osteosarcoma, was first reported by sim et al. The cancer malignant cells make immature bone cells osteoid. Small cell osteosarcoma of bone, first defined in 1979, 1 is a rare form of osteosarcoma that poses unique diagnostic and treatment considerations.
The malignant cells produce immature woven bone, or osteoid, which is why the tumor is named osteosarcomait is a boneproducing sarcoma. Histological examination revealed a small cell osteosarcoma of the proximal phalanx. 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. Osteosarcomas are rare, highly malignant, bone tumors defined by the presence of malignant mesenchymal cells producing osteoid or immature bone. Unfortunately, the patient died of rapid progression of the osteosarcoma within a week of admission to the hospital. Small cell osteosarcoma is a variant of osteosarcoma composed of small cells and varying amount of osteoid. There are mostly solid areas of small round or spindle cells that may resemble other small cell sarcomas, such as ewing sarcoma.
Sometimes, children and teens with osteosarcoma do not have any of these changes. An accurate diagnosis, usually facilitated by chemotherapy ct, mri and biopsy, is required in order to define the. The aim of this article is to highlight such an entity at an unusual sitethe. The lesions demonstrated a wide variation in radiographic appearance. 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. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. This tumor must be differentiated from other small cell malignancies because of treatment considerations, particularly patient response to chemotherapy. Small round cell osteosarcoma is a very rare type of osteosarcoma, histologically mimicking other small round cell malignancies of bone, most notably ewing sarcoma.
We present a case of a 28yearold female who had classic signs, symptoms, and radiographic features of the lesion. Pdf a small cell osteosarcoma with multiple skeletal. Small cell osteosarcoma is composed of small cells with variable degrees of. Small cell osteosarcoma is an exceedingly rare tumor, estimated to account for pdf. Osteosarcoma is rare, but it is the most common type of bone cancer in children and teens.
Supplementary information s2 commonly used osteosarcoma cell lines pdf 154 kb. An osteosarcoma os or osteogenic sarcoma ogs or simply bone cancer is a cancerous tumor in a bone. Small cell osteosarcoma of bone nakajima 1997 cancer wiley. Wide surgical excision of small cell osteosarcoma and removal of the entire lesion is the standard treatment mode. According to the world health organization, os of bone is classified into eight subtypes with distinct biologic behaviors and clinical outcomes.
Mfh of bone is treated according to osteosarcoma treatment protocols. Osteosarcomas of the jaws are extremely rare, representing about 7% of all osteosarcomas and 1% of all head and neck malignancies. Early identification of such patients perhaps at initial biopsy would allow a tailored approach. On microscopic analysis, small cell osteosarcoma shows scanty osteoid formation. Intraosseous welldifferentiated, or lowgrade, osteosarcoma. 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. 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 differentiation. However, peroperatively the lesion had a malignant aspect. Pulmonary metastases appeared after a gap of 9 months of ablative surgery, as did late multiple extrapulmonary skeletal metastases, including the hand and. Osteosarcoma os is a common primary malignant tumor of bone that produces osteoid matrix.
The defining histologic feature of small cell osteosarcoma is the presence of osteoid, which has been described in each and every case reported. 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. Endobronchial metastasis as an uncommon pattern of. Osteosarcoma causes, risk factors, and prevention 1. Small cell osteosarcoma memperlihatkan lesi litik permeatif, destruksi korteks, massa jaringan lunak, reaksi periosteal, serta kalsifikasi matriks osteoid. Pdf establishment and characterization of a human small. Osteosarcoma cell an overview sciencedirect topics. In this study, for the first time, we have isolated, established, and characterized a cell line of cancer stem cells cscs from.
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