Originally, it was considered restricted to bone, but that is no. People who have chondrosarcoma have a tumor growth starting from the medullary canal of a long and flat bone. Mesenchymal chondrosarcoma is a rare tumor of soft tissues and bone characterized by a bimorphic appearance on histopathology. Mesenchymal chondrosarcoma an overview sciencedirect. Although occurring at any age, it most commonly affects teenagers and young adults with a peak incidence in the third decade of life. Mesenchymal chondrosarcoma is a vicious but rare form of bone cancer. Dowling subsequently reported this entity in the soft tissue in. Approximately two thirds of cases of mesenchymal chondrosarcoma occur in bone, while the rest occur in soft tissue muscle or fat. Extraskeletal mesenchymal chondrosarcoma komal arora, md. Dedifferentiated chondrosarcoma, although typically arising from. Mesenchymal chondrosarcoma is a malignant soft tissue tumor of uncertain lineage characterized at the genetic level by the hey1ncoa2 gene fusion. Report of a unique case with clinical, histopathologic, and immunohistochemical findings, and a.
Unlike most chondrosarcomas, mesenchymal chondrosarcoma grows rapidly, tends to spread, and occurs more often in children and young adults than in older adults. The tumour frequently originates from the endocardium, grows into the atrial or ventricular cavity and then, infiltrates into myocardial wall and extends to mediastinal structures 7. Introduction mesenchymal chondrosarcoma mcs is a rare tumour characterized by undifferentiated mesenchymal cells with islands of mature hyaline cartilage. One third to one half of mcs are extraosseous in origin. Grade and stage are independent predictors of survival. It is malignant by nature and is more quick to develop and spread compared to the conventional forms of chondrosarcomas. The results reveal strong similarities between mesenchymal chondrosarcoma and normal chondrogenesis. Gk, mesos, middle, enchyma, infusion, chondros, cartilage, sarx, flesh, oma, tumor a rare malignant tumor of. Classically, mesenchymal chondrosarcoma shows stereotypic morphology, with primitive round to spindled cells, a prominent branching hemangiopericytomalike vasculature, islands of relatively. Extraskeletal mesenchymal chondrosarcoma archives of pathology.
Cytopathology of mesenchymal chondrosarcomas trembath. Pathology they tend to be of higher grade than runofthemill conventional intramedullary chondrosarcomas, with the majority being of the myxoid most common or mesenchymal varieties 3 location. Comprised primarily of noncartilaginous small, round, oval, or spindle. Even after treatment, mesenchymal chondrosarcoma can relapse years later. Conventional chondrosarcoma is notorious for its locally aggressive behaviour as well as for its resistance to chemotherapy and radiotherapy. In children and adolescents, mesenchymal chondrosarcoma accounts for up to 25% of all chondrosarcomas. It usually presents during the second to third decades of life, and has a slight predominance in females. Histopathologically, it is a biphasic tumor comprising of islands of hyaline. Extraskeletal mesenchymal chondrosarcoma is a rare form of sarcoma that is not connected to bone, but typically involves the muscles or central nervous system. Elmofty, in diagnostic surgical pathology of the head and neck second edition, 2009. It represents only 2% to 10% of all chondrosarcomas27 and has a frequency of 0.
Mesenchymal chondrosarcoma nord national organization for. An analysis of patients treated at a single institution. These tumors are generally painless, contributing to the frequent delays in their diagnosis. The histologic subtypes of chondrosarcoma are uncommon.
Linkpage citation mesenchymal chondrosarcoma is a rare, highgrade malignancy of bone or soft tissue first described by lichtenstein and bernstein 1 in 1959. However, the phenotypic attributes of the smallcell population in this neoplasm have not been well characterized, and its relationship to precartilage mesenchyme remains unclear. Pdf clinicopathological and histological behavior of. Chemotherapy improved prognosis of mesenchymal chondrosarcoma. Mesenchymal chondrosarcoma of the sinonasal tract is a rare, malignant tumor of extraskeletal origin. Mesenchymal chondrosarcoma is an extremely rare, often aggressive form of cancer. Conventional chondrosarcoma is a form of bone cancer that arises from cartilage cells. Two years after its original description, dahlin and henderson 2 reported 9 cases from the files of the mayo clinic. Mesenchymal chondrosarcoma mcs, is an unusual tumor first described by lichtenstein and bernstein. Mesenchymal chondrosarcoma is a rare malignant neoplasm first described by lichtenstein and bernstein in 1959.
Dec 04, 2018 chondrosarcoma is a tumor of mesenchymal origin that predominantly is made of cartilage. Cartilage is the specialized tissue that serves as a buffer or cushion at joints. Mesenchymal chondrosarcoma mcs is a rare highgrade of variant of chondrosarcoma first described in 1959. Isolated cases have been reported in the english literature, with no large series evaluating. Mesenchymal chondrosarcomamcs is a rare highgrade of variant of chondrosarcoma first described in 1959. Extraskeletal mesenchymal chondrosarcoma emcs is a rare malignant soft tissue tumor of chondroprogenitor cell origin. Mesenchymal chondrosarcoma of bone and soft tissue. Dedifferentiated chondrosarcoma, although typically arising from conventional chondrosarcoma, is. The histopathological sections showed tumor tissue composed of numerous round primitive mesenchymal cells arranged in lobules, having. During the past 10 years, substantial new insights have been gained about. Primary chondrosarcoma of the heart is presumed to arise from multipotent mesenchymal stem cells that undergo malignant cartilaginous differentiation. The tumour may mestastasize to the lungs in some cases. Chondrosarcoma is a malignant cancer that results in abnormal bone and cartilage growth. Primary chondrosarcomas arise from a small collection of cartilage cells.
Mesenchymal chondrosarcoma mcs was first described by lichtenstein and bernstein 1 in 1959. Mesenchymal chondrosarcomas showing immunohistochemical. Comprised primarily of noncartilaginous small, round, oval, or spindle shaped cells with islands of malignant cartilage dispersed throughout noncartilaginous component of tumor. Primary chondrosarcoma of the heart european journal of. Unlike other types of malignant chondrosarcoma, which have a tendency to grow more slowly and rarely spread, mesenchymal chondrosarcoma is a fast. Mesenchymal chondrosarcoma is a rare, high grade malignancy of bone or soft tissue with a unique, biphasic histology and poor prognosis.
This is why roughly 60% of cases of mesenchymal chondrosarcoma affect the patients bones. Subtypes vary substantially chondrosarcoma nos and myxoid chondrosarcoma have a five year survival of 70%, but mesenchymal chondrosarcoma only 50%, and dedifferentiated chondrosarcoma an abysmal 0%. Most cases of mesenchymal chondrosarcoma develop within the bones of the body, while the remaining third of cases are able to grow outside of the bone. Mesenchymal chondrosarcoma comprises 210% of all chondrosarcomas 15. Chondrosarcoma symptoms, prognosis, treatment, survival rate. Mesenchymal chondrosarcoma is a form of malignant chondrosarcoma. Insm1 expression and its diagnostic significance in. Pattern formation in chondroid development is compared with growth models exhibited by mesenchymal and myxoid chondrosarcomas. It occurs in children and young adults considerably more often than in older people, though it is a very rare type of cancer overall. Font et al 12 correlated computed tomography appearance with histopathology of orbital mesenchymal chondrosarcoma. Extraskeletal mesenchymal chondrosarcoma rd of the cases that involve the soft tissues of the body. Webpathology is a free educational resource with 10308 high quality pathology images of benign and malignant neoplasms and related entities. Lichtenstein and bernstein first described bony mcs in 1959.
Mesenchymal chondrosarcoma is an uncommon smallcell neoplasm of bone and soft tissue, the chondrogenic nature of which has been generally accepted. This study included five patients with histologically confirmed mcs of the orbit who had undergone. Chondrosarcoma is a tumor of mesenchymal origin that predominantly is made of cartilage. We report a 39yearold man with extraskeletal mesenchymal chondrosarcoma of the buttock, who had metastases to the pancreas, bones, and lung. The cause of tumor formation is reportedly due to genetic mutations and chromosomal aberrations.
Extraskeletal myxoid chondrosarcoma is a rare subtype of sarcoma that affects the soft tissue and bones in middleaged and elderly adults. Extraskeletal mesenchymal chondrosarcoma is a rare soft tissue sarcoma arising from soft tissues, mainly of the lower extremities, meninges. Pdf mesenchymal chondrosarcoma mc is a rare variety of. Mesenchymal chondrosarcoma treatment, support and research. Lightenstein and bernstein first described mesenchymal chondrosarcomas in 1959, and dowling reported the first case of an extraskeletal mesenchymal chondrosarcoma in 1964. Jan 01, 2012 in an attempt to improve diagnostic specificity, wehrli et al 30 compared immunohistochemistry profiles of 12 mesenchymal chondrosarcoma samples with several other small, round, bluecell tumors and demonstrated expression of sox9 in both cartilaginous and mesenchymal regions in 21 of 22 mesenchymal chondrosarcoma tumors 95. Mc mostly affects children and young adults between the ages of 15. These tumours, which principally presented in middle. Histologic variants of chondrosarcoma diagnostic histopathology.
Apr 07, 2015 mesenchymal chondrosarcomamcs is a rare highgrade of variant of chondrosarcoma first described in 1959. It is commonly thought that a history of trauma could be a. Mesenchymal chondrosarcoma is a type of chondrosarcoma, which itself is a type of bone cancer that has its origins in cartilage cells. Mesenchymal chondrosarcoma of the left coronoid process. Request pdf mesenchymal chondrosarcoma of the left coronoid process. The fine vascularization of the tumor lobules of myxoid chondrosarcoma is discussed in relation to chondroid differentiation. It accounts for less than 3% of all chondrosarcomas. Craniofacial bones, the ribs, vertebrae and pelvis are the. Despite their rarity, they present with characteristic clinical and histopathologic features distinguishing them from conventional type of chondrosarcoma. Mesenchymal chondrosarcoma is a rare, highgrade malignancy of bone or soft tissue first described by lichtenstein and bernstein1 in 1959. Type ii collagen can help distinguish it from other tumors. Extraskeletal mesenchymal chondrosarcoma is rare and metastasis to the pancreas is extremely rare, with only four cases reported in the literature.
Extraskeletal mesenchymal chondrosarcoma of the thigh. Figure 6 metastatic mesenchymal chondrosarcoma to adrenal gland. Extraskeletal mesenchymal chondrosarcoma archives of. A rare case of extraskeletal mesenchymal chondrosarcoma with. Figure 7 mesenchymal chondrosarcoma with small round blue cell component right merging into differentiated chondrocytic component left 400. Extraskeletal chondrosarcoma radiology reference article. Mesenchymal chondrosarcoma mcs of the orbit is a rare and aggressive form of chondrosarcoma. Mesenchymal chondrosarcoma mc was first described as an occurrence in the bone by lichtenstein and bernstein in 1954. Chondrosarcoma, rare malignant tumour of bone formed from cartilage. Nov 20, 2018 extraskeletal mesenchymal chondrosarcoma rd of the cases that involve the soft tissues of the body. They occur at all ages but typically around the age of 50 3. The term chondrosarcoma is used to define an heterogeneous group of lesions with diverse features and clinical behavior. Extra skeletal myxoid chondrosarcomas typically occur in the extremities, with the thigh being most common.
Mesenchymal chondrosarcoma an overview sciencedirect topics. Mesenchymal chondrosarcoma is a malignant cancer of the cartilage, that tends to grow and spread more quickly than other similar tumors. Teens and young adults are most prone to mesenchymal chondrosarcoma. Its diagnosis can be challenging, with the differential.
The purpose of this study was to retrospectively identify the imaging features of mesenchymal chondrosarcoma of the orbit. Emerging pathways in the development of chondrosarcoma of. Desmin and myogenin reactivity in mesenchymal chondrosarcoma. Among seven patients with extraskeletal mesenchymal chondrosarcoma emc, three children aged 36 years developed emc in a central location and four adults aged 3854 years developed emc in both central and peripheral sites. The risk factors for mesenchymal chondrosarcoma remain presently unidentified. Riddle, md extraskeletal mesenchymal chondrosarcoma is a rare soft tissue sarcoma arising from soft tissues, mainly of the lower extremities, meninges, and orbits. Ct scanning, mri, and plain radiographs may identify these lesions.
Low grade chondrosarcomas do not often have an associated soft tissue mass and are most difficult. Chondrosarcoma is a collective term that encompasses a group of heterogeneous lesions with diverse morphologic features and clinical behaviors. It only accounts for 1% to 10% of all chondrosarcomas24. Mesenchymal chondrosarcoma mc accounts for 3% to 5% of all chondrosarcomas. Mesenchymal chondrosarcoma definition of mesenchymal. Mcs differs from typical chondrosarcomas in the following respects. It occurs in both young and old people and is more aggressive on initial presentation than other types of cartilaginous tumors. This histological subtype occurs in both osseous and extraosseous tissues and has a tendency for late local and disseminated recurrence 1, 2, 4, 7. Mesenchymal chondrosarcoma international journal of particle. Chondrosarcoma is a malignant cartilageforming tumour of bone, of which distinct clinicopathological subtypes are known. It is a subtype of chondrosarcoma and is assumed to arise from remnants of the embryonic cartilage or metaplasia of meningeal fibroblasts 1. Most cases of mesenchymal chondrosarcoma develop within the bones of the body, while the remaining third of cases are able to.
University institutes of pathology of lausanne and geneva, switzerland. The rest of the time, the cancer actually develops in other areas of the. Usually a good prognosis 75% five year survival in one large data set. Malignant cartilage tumors chondrosarcoma james wittig, m. Histologically, mcs has a typical biphasic pattern consisting of both small cells and islands of atypical cartilage.
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