An update on recent molecular genetic advances shi wei, md, phd and gene p. Incidence of sarcoma increases with age, some sarcoma types occur frequently in specific age groups. The animals were assessed in terms of age, sex, breed, topographic tumor distribution, and presence of. It may be confused with ewing sarcoma if the osteoid matrix is not included in the biopsy. Among the extraskeletal osteosarcomas, the small cell type is extremely rare. They ranged from 6 to 28 years of age with a median of 14 years. Desmoplastic small round cell tumor dsrct is a rare type of soft tissue cancer sarcoma that usually begins in the abdomen. 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. Cancer happens when cells start to divide uncontrollably and spread to other tissues. 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. Learn about the risk factors for osteosarcoma and if there are things that might help lower risk. Mall cell osteosarcoma sco was first reported by sim et al.
Osteosarcoma is a type of bone cancer that most often affects children and teens. Small cell osteosarcoma of bone is a rare form of osteosarcoma, with an incidence rate of 1. Pdf small cell osteosarcoma of the soft tissue gene. Histopathology and molecular pathology of bone and. Abstract background small cell osteosarcoma of bone is a rare form of osteosarcoma, with an incidence rate of 1. 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. This article describes a 31yearold man who had small cell extraskeletal osteosarcoma arising from the small cell. Small cell osteosarcoma sco is a rare but distinct variant of osteosarcoma. The distinctive radiographic features of an osteoblastic tumor and a pattern. Desmoplastic small round cell tumor genetic and rare. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis. It primarily affects children and young adults and is more common in males. Learn more about the subtypes, causes, symptoms, diagnosis, treatment, and prognosis for osteosarcoma. Prognosis is unaffected by the cytological features or cell size.
Pediatric sarcomas memorial sloan kettering cancer center. The absence of the latter would have made it difficult to distinguish from other small round cell tumors of bone, especially ewings sarcoma. The ability of osteosarcoma cells to metastasise by such a pathway relies on complex cell cell and cell matrix interactions. Clinicopathologic, immunohistochemical, and molecular analysis of 36 cases small round cell osteosarcoma is a. Establishment and characterization of a human small cell. Carcinomaadenocarcinoma sarcoma lung non small cell osteosarcoma lung small cell. According to the predominant cell pattern, tumours are classified to round cell type or short spindle cell type. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. We care for about 200 children, adolescents, and young adults with this disease each year. Small cell osteosarcoma is distinguished by the presence of a mineralised matrix. The nccn guidelines panel for cervical cancer screening endorses the following guidelines for the prevention and early detection of cervical cancer. This man presented with pain, swelling and redness of the left third toe. Small cell osteosarcoma consists of small, round blue cells, which makes it hard to be differentiated from ewings sarcoma many clinical conditions may have similar signs and symptoms. The classic or socalled conventional osteosarcoma develops in the medullary cavity of the metaphysis of long bones.
It is formed by small, round cancer cells surrounded by scarlike tissue and is often found in the tissue peritoneum that lines the inside of the abdomen and pelvis. Compared to nonsmall cell carcinoma, small cell carcinoma has a shorter doubling time, higher growth fraction, and earlier development of metastases. However, fibrous or cartilaginous tissue may coexist or even predominate. Osteosarcoma is the most common type of cancer affecting the bone and involves abnormal osteoblast cells that are responsible for the construction of bone.
Small cell osteosarcoma sco, composed of small round cells, which mimic ewing sarcoma cells 1, is the most rare variant of osteosarcoma, comprising. There are mostly solid areas of small round or spindle cells that may resemble other small cell sarcomas, such as ewing sarcoma. The small amount of osteoid formation makes these tumors a challenge for the pathologist. Summary sarcoma is a rare cancer that can occur anywhere in the body, but more frequently in the extremities, chest and abdomen. Round cell tumors of bone are a divergent group of neoplasms that largely constitute ewing sarcoma primitive neuroectodermal tumor, small cell osteosarcoma, langerhans cell his.
Smallcell carcinoma is a type of highly malignant cancer that most commonly arises within the lung, although it can occasionally arise in other body sites, such as the cervix, prostate, and gastrointestinal tract. Find out how osteosarcoma is tested for, diagnosed. Small cell osteosarcoma american journal of clinical. However, peroperatively the lesion had a malignant aspect. Osteosarcoma os is a malignant spindle cell sarcoma in which the malignant cells produce osteoid or bone in the background of a sarcomatous stroma. It has been difficult to identify the molecular features central to the pathogenesis of osteosarcoma owing to a lack of understanding of the cell or origin, the absence of identifiable precursor lesions.
Osteo boneosteoid tissue sarcoma malignant tumour of connective tissue 020412 dr. Also known as osteogenic sarcoma explanation of smallcell osteosarcoma. An immunohistochemical study with differential diagnostic considerations kenneth devaney, md, tuyethoa n. Most cases of cancer involve either a carcinoma or a sarcoma. Webmd explains the symptoms, causes, and treatment. Small round cell sco sample, obtained by surgical resection of a part of the tumor figure 2 permits the isolation of only one osteosarcoma cell line osa if treated precisely, as described before. 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. Five cases of small cell osteosarcoma were identified in a 19year period 19902009. Small cell osteosarcoma, a subtype of osteogenic sarcoma, consists of sheets of round cells that produce an osteoid matrix. Galectin1 gal1 expression is a useful tool to differentiate between small cell osteosarcoma and ewing sarcoma.
On microscopic analysis, small cell osteosarcoma shows scanty osteoid formation. Small cell osteosarcoma is a rare intramedullary subtype, comprising about 1 % of osteosarcomas 29. Clinicopathologic, immunohistochemical, and molecular analysis of 36 cases article in american journal of surgical pathology 395 february 2015 with 6 reads. Small cell osteosarcoma american journal of clinical pathology. Imaging plays a crucial role in the diagnosis of each subtype of os and.
Small cell osteosarcoma successfully treated by highdose. Three cases were fnas from bony lesions in the classic location for osteosarcoma 2 distal femur and 1 proximal tibia with a size range of 4. There were 12 male patients and 15 female patients. A sarcoma is a rare kind of cancer that grows in connective tissue cells that connect or support other kinds of tissue in your body. Abstract smallcell osteosarcoma, a subtype of osteogenic sarcoma, consists of sheets of round cells that produce an osteoid matrix. The number of cells isolated from the small biopsy is very low figure 3.
This file is intended as a reference file for icdo3 only and is not to be used for casefinding purposes. Of all the varieties of osteosarcoma, the juxtacortical type is rare and comprises only less than 4% of all osteosarcomas. Get an overview of osteosarcoma and the latest key statistics in the us. American cancer society, american society for colposcopy and cervical pathology, and american society for clinical pathology screening guidelines for the prevention and early detection of cervical cancer.
Small cell osteosarcoma of bone nakajima 1997 cancer wiley. The distinctive radiographic features of an osteoblastic tumor and a pattern of permeative destruction will confirm the histologic. Histological examination revealed a small cell osteosarcoma of the proximal phalanx. In this study, for the rst time, we have isolated, established, and characterized a cell line of cancer stem cells cscs from a h uman sco. A malignant tumor principally composed of anaplastic cells of mesenchymal derivation. The ages of the patients ranged from 11 to 37 years. Histologically, sheets of small, round, blue cells observed appearance is similar to that of ewing sarcoma, but differs from ewing in that these cells produce osteoid use of ewing immunohistochemical markers and evaluation for an 11. The icdo3 sitetype validation program was modified to allow only for the sitehistologybehavior combinations listed in this publication. Based on clinical findings and medical history the lesion was diagnosed as an osteomyelitis. We report a study of 27 patients with small cell osteosarcoma sco, 17 from the m. The presence of the glycogen can be demonstrated with positive pas staining and negative pas diastase staining. This report describes the radiological and histological findings of a small cell osteosarcoma of a toe phalanx in a 38 year old man. People with certain noncancerous bone diseases have an increased risk of developing osteosarcoma.
Medical history revealed an osteomyelitis of this toe eight years prior. This disruption can result from uncontrolled cell growth or loss of a cell s ability to undergo apoptosis. There are numerous types of primary osteosarcoma, including intramedullary high grade, telangiectatic, low grade, small cell, osteosarcomatosis, and gnathic, surface intracortical, parosteal, periosteal, and highgrade surface, and extraskeletal. Round to ovoidshaped cells, similar to ewing sarcoma cells, organized in sheets and nests, with osteoid matrix selected bibliography bertoni f, present d, bacchini p, pignatti g, picci p, campanacci m 1989 the istituto rizzoli experience with small cell osteosarcoma. At memorial sloan kettering cancer center, we have extensive experience in diagnosing and treating pediatric sarcomas of all types, even those beyond the most common. Imaging characteristics of primary osteosarcoma rsna. Osteosarcoma may also occur as a secondary lesion in association with underlying benign conditions.
The characteristic immunostain is cd99, which diffusely marks the cell membrane. The metastatic sequence involves the detachment of osteosarcoma cells from the primary tumour, adhesion to the extracellular matrix, local migration and invasion through stromal tissue, intravasation, and extravasation. Small cell osteosarcoma is a rare histological subtype of osteosarcoma. Anderson cancer center mdah and ten from the pediatric oncology group pog. One of the rare histological variant is small cell osteosarcoma which consists of sheets of. Small round cell osteosarcoma is a very rare type of osteosarcoma, histologically mimicking other small round cell malignancies of bone, most notably ewing sarcoma.
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