It is an extremely rare type of osteosarcoma that mostly occurs around the knees the exact cause of the condition is unknown and presently, it is not possible to prevent small cell osteosarcoma occurrence. Small cell variant of osteosarcoma on histopathology was another interesting and unusual finding found in the present case, which makes the case even rarer. The classic or socalled conventional osteosarcoma develops in the medullary cavity of the metaphysis of long bones. Small cell osteosarcoma american journal of clinical pathology.
Synchronous multifocal osteosarcoma with small cell. Pdf osteosarcoma is the most common primary tumor of bone, yet its absolute. Small cell osteosarcoma 23 rhabdomyosarcoma 21 desmoplastic small round blue cell tumor 16 small cell carcinoma 9 merkel cell carcinoma 9. This report describes the radiological and histological findings of a small cell osteosarcoma of a toe phalanx in a 38 year old man. B osteoid produced by tumor cells was found after thorough sampling of the specimen, confirming the diagnosis of small cell osteosarcoma. One of the most challenging diagnostic categories within tumors of the sinonasal tract is the small round blue cell tumors. The absence of the latter would have made it difficult to distinguish from other. The histopathology of osteosarcoma is highly variable, ranging from the more commonly seen. Jan, 2019 small cell osteosarcoma is a medullary type of osteosarcoma that consists of small, round blue cells. It is most prevalent in children and young adults, as it is associated with the. A staging system is a standard way for the cancer care team to sum up the extent of the cancer.
Small round cell tumors of bone archives of pathology. The stage of an osteosarcoma is based on the results of physical exams, imaging tests, and any biopsies that have been done, which are described in tests for osteosarcoma. Small cell osteosarcoma american journal of clinical. The malignant cells produce immature woven bone, or osteoid, which is why the tumor is named osteosarcoma it is a boneproducing sarcoma. A study of 10 cases, histopathology on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips.
This man presented with pain, swelling and redness of the left third toe. Diffuse growth of small, uniform, roundspindle tumor cells, focally produces malignant osteoid, occasionally mixed with cartilage. Fine needle aspiration cytology showed a small cell lesion with very scant osteoid. According to the predominant cell pattern, tumours are classified to round cell type or short spindle cell type. Osteosarcoma os is a malignant spindle cell sarcoma in which the malignant cells produce osteoid or bone in the background of a sarcomatous stroma. Analysis of clinicopathological features and prognostic factors of desmoplastic small round cell tumor.
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. Osteosarcoma osteosarcoma is the most common primary, malignant tumor of bone. Histopathological characterization of small cell osteosarcoma with immunohistochemistry and molecular genetic support. One of the rare histological variant is small cell osteosarcoma which consists of sheets of round. Icd03 seer site histology validation list june 18, 2019 2 lip c000c006,c008c009 neoplasm 800 80003 neoplasm, malignant 800 tumor cells, malignant 80023 malignant tumor, small cell type 80033 malignant tumor, giant cell type. View enhanced pdf access article on wiley online library html view download pdf for offline viewing. Small cell osteosarcoma is an exceedingly rare tumor, estimated to account for less than 1% of all cases of osteosarcoma. Epithelioid osteosarcoma has the cell tumour poorly differentiated, for this reason being difficult to distinguish if is a sarcoma or a carcinoma. The aim of this article is to highlight such an entity at an unusual sitethe parietal. Small cell osteosarcoma, a variant of osteosarcoma, was first reported by sim et al. Arraybased dnamethylation profiling in sarcomas with small. The malignant cells produce immature woven bone, or osteoid, which is why the tumor is named osteosarcomait is a boneproducing sarcoma.
Small cell osteosarcoma of bone nakajima 1997 cancer wiley. Small round blue cell tumors of the sinonasal tract. The anatomic distribution of small cell osteosarcoma is similar to that. Tumor was excised and histopathological diagnosis was small. Small cell osteosarcoma is a rare tumour that histologically mimics ewing sarcoma, mesenchymal chondrosarcoma and lymphoma, the presence of osteoid being diagnostic.
Histopathology and molecular pathology of bone and. 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, a subtype of osteogenic sarcoma, consists of sheets of round cells that produce an osteoid matrix. Conventional os is a high grade intramedullary neoplasm, often arising in the metaphysis of long tubular bones. Small cell osteosarcoma may present a challenging primary. Mar 23, 2018 undifferentiated solid tumors with small blue round cell histology and expression of cd99 mostly resemble ewing sarcoma. Mar 16, 2020 webpathology is a free educational resource with 10287 high quality pathology images of benign and malignant neoplasms and related entities. Thus, in small biopsies not representative, incorrect diagnosis such as chondrosarcoma or fibrosarcoma can be concluded.
Small cell osteosarcoma is an exceedingly rare tumor, estimated to account for small cell osteosarcoma. Osteosarcoma osteogenic sarcoma atlas of pathology. Osteosarcoma os is a highly malignant bone forming tumor characterized by frankly to subtly anaplastic stromal cells with evidence of direct formation of osteoid andor primitive bone by these. An immunohistochemical study with differential diagnostic considerations kenneth devaney, md, tuyethoa n. Immunohistochemical evaluation of small round cell tumors of. 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. However, fibrous or cartilaginous tissue may coexist or even predominate. This variant needs different management protocol, being nonradiosensitive and behaving more aggressively than conventional osteosarcoma. Histopathological characterization of small cell osteosarcoma. The histopathology of osteosarcoma is highly variable, ranging from the more commonly seen osteoblastic and chondroblastic type to the rare. 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.
Mild pleomorphism and nuclear hyperchromasia suggest the diagnosis of small cell osteosarcoma. Most osteosarcomas can be categorized into fo ur major groups. Biopsies are usually small and limited, resulting in considerable. In the image, note the extensive, lacelike bone laid down by the cancerous. Small cell osteosarcoma represents a rare histological combination of osteosarcoma and ewing sarcoma, until 2% of osteosarcomas. Indian journal of pathology and oncology, octoberdecember 2017. Immunohistochemical and molecular aspects of diagnosis, when available, are stressed, especially in the case of ewing tumour. Small cell osteosarcoma sco is a rare but distinct variant of osteosarcoma. Histopathology and molecular pathology of bone and extraskeletal osteosarcomas.
Sweet, md seventynine cases of small round cell tumors involving bone were studied in an attempt to learn whether the immunohistochemical features of the lesions might allow distinction of small cell osteosarcoma from other. Small cell osteosarcoma of bone is a rare form of osteosarcoma, with an incidence rate of 1. Slightly atypical spindle cells and very few mitotic figures are seen. However, they also may include other tumors such as mesenchymal. The histology showed sheets of small round cells and osteoid. Diagnosis of small round cell tumours of bone remains a diagnostic challenge. Ema dragoescu, colleen jacksoncook, gregory domson, davis massey and william c. There is often areas with chondroblastic or fibroblastic differentiation. Jul 01, 2010 read histopathological characterization of small cell osteosarcoma with immunohistochemistry and molecular genetic support. Imaging strategy for detecting lung metastases at presentation in patients with soft tissue sarcomas. Webpathology is a free educational resource with 10287 high quality pathology images of benign and malignant neoplasms and related entities. Small cell sarcoma an overview sciencedirect topics. Osteosarcomas are associated with mutations in the retinoblastoma gene rb and p53. Tumor cells are very pleomorphic anaplastic, some are giant and present numerous and atypical mitotic figures.
Diagnostic evaluation and biopsy techniques for primary bone tumors, an overview of treatment and outcomes, principles guiding surgical management of bone sarcomas, and chemotherapy in the. Small cell osteosarcoma is an exceedingly rare tumor, estimated to account for osteosarcoma. Small round cell osteosarcoma is a very rare type of osteosarcoma, histologically mimicking other small round cell malignancies of bone, most notably ewing sarcoma. The present study was conducted for the immunohistochemical evaluation of pediatric undifferentiated. Osteosarcoma osteogenic sarcoma is a malignant tumor whose neoplastic cells present osteoblastic differentiation and form tumor bone. Osteosarcoma os is the commonest osteoarticular malignancy of nonhematopoetic origin. Giant cell richosteosarcomas contain osteoclastlike giant cells.
Aug 23, 2018 osteosarcoma is a malignant neoplasm of the bone and is the most common histological form of primary bone cancer. Well differentiated intraosseous osteosarcoma is a spindle cell osteosarcoma. It may be confused with ewing sarcoma if the osteoid matrix is not included in the biopsy. Diagnosis of small round cell tumours of bone current. Foster, small cell osteosarcoma with ewing sarcoma breakpoint region 1 gene rearrangement detected by interphase fluorescence in situ hybridization, annals of diagnostic pathology, 10. American journal of clinical pathology, volume 3, issue 5, may 2010, pages 756761.
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