Mesenchymal chondrosarcoma mcs is a rare highgrade of variant of chondrosarcoma first described in 1959. Mesenchymal chondrosarcoma an overview sciencedirect. Lateral radiograph of the femur showing a grade ii myxoid chondrosarcoma. Mesenchymal chondrosarcoma nord national organization for. Mesenchymal chondrosarcoma is a rare, usually highly malignant chondrogenic neoplasm. Chondrosarcomas are further stratified into conventional, mesenchymal, dedifferentiated, and clear cell subtypes. Extraskeletal myxoid chondrosarcoma emc is a rare lowgrade malignant mesenchymal neoplasm of the soft tissues, that differs from other sarcomas by unique histology and characteristic chromosomal translocation. To our best knowledge, only 9 such cases have been reported so far.
This case of orbital mesenchymal chondrosarcoma in a 38yearold man is believed to be the 19th example reported in the world literature, the seventh affecting soft tissues, and the first of orbital location. Mesenchymal chondrosarcomas of the parotid are extremely rare tumours. Mesenchymal chondrosarcoma of the orbit report of the first. Conventional chondrosarcomas have been extensively studied at the genetic level, but for rare chondrosarcoma subtypes, this is merely restricted to case reports. General information extraskeletal chondrosarcoma is a rare cancerous soft tissue tumor. Spinal epidural metastatic mesenchymal chondrosarcoma in. Radiotherapy and chemotherapy are additional treatment options, but no conclusive results for their efficacy have been shown until date.
The diagnosis often difficult to make because of the low incidence among malignant bone tumours. Primary spinal intradural mesenchymal chondrosarcoma with. Sox9, a master regulator of chondrogenesis, distinguishes. Pdf extraskeletal mesenchymal chondrosarcoma in an elderly. It only accounts for 1% to 10% of all chondrosarcomas24. The diagnosis of mesenchymal chondrosarcoma, a distinctive biphasic malignant neoplasm harboring the hey1ncoa2 gene fusion and consisting of primitive round to spindled cells admixed with foci of relatively mature hyaline cartilage, is usually straightforward by morphologic evaluation alone. No preclinical rationale for igf1r directed therapy in. Mesenchymal chondrosarcoma is a rare aggressive subtype, in which distant metastasis can be identified even after 20 years. Mesenchymal chondrosarcoma of the sinonasal tract is an aggressive tumor with a predilection for young women. It is malignant by nature and is more quick to develop and spread compared to the conventional forms of chondrosarcomas. Chondrosarcomas are tumors consisting of osseous or cartilaginous stroma.
An analysis of patients treated at a single institution. This rate tumour is characterized by slow growth, late metastasis up to 20 years after first presentation and poor prognosis. Jun 11, 20 chondrosarcoma is a type of rare bone cancer affecting the bones of pelvis, legs and shoulders. People who have chondrosarcoma have a tumor growth starting from the medullary canal of a long and flat bone. People who have chondrosarcoma have a single tumor growth which can vary in size and location when first discovered. Patients with dedifferentiated chondrosarcoma show a 5year overall survival between 7 and 24%. A case of mesenchymal chondrosarcoma of the maxilla is reported. May 24, 2016 epiphyseal gaint cell tumor chondroblastoma clear cell chondrosarcoma 35. Unusual benign and malignant chondroid tumors of bone.
Response to pd1 inhibition in conventional chondrosarcoma. Clear cell chondrosarcoma is a rare variant that grows slowly. The therapeutic effectiveness of chemotherapy remains uncertain. Type ii collagen can help distinguish it from other tumors. Dec 15, 2014 primary mesenchymal chondrosarcoma of the kidney is an extremely rare malignant tumor. Unlike most chondrosarcomas, mesenchymal chondrosarcoma grows rapidly, tends to spread, and occurs more often in children and young adults than in older adults. The observation of similar chromosomal abnormalities in both skeletal and extraskeletal mesenchymal chondrosarcoma supports a genetic as well as his. Alternatively, you can download the file locally and open with any standalone pdf reader. Most cases of mesenchymal chondrosarcoma develop within the bones of the body, while the remaining third of cases are able to. Scribd is the worlds largest social reading and publishing site. Type ii collagen as specific marker for mesenchymal.
There is an uncertain differentiation there is no evidence yet showing that emc exhibits the feature of cartilaginous. Gk, mesos, middle, enchyma, infusion, chondros, cartilage, sarx, flesh, oma, tumor a rare malignant tumor of. Mesenchymal chondrosarcoma comprises about 2% of all chondrosarcomas. They may also recur come back many years after treatment. Mesenchymal chondrosarcoma is one of the most unusual neoplasms involving bone and soft tissue.
Mesenchymal chondrosarcoma of bone and soft tissue plos. Prognosis for patients with mesenchymal chondrosarcoma is fair, with a 5year survival after wide resection of 60% and a 10year survival of 25%. Mesenchymal chondrosarcomas showing immunohistochemical. Mesenchymal chondrosarcoma of the maxilla the journal of. Mesenchymal chondrosarcoma is a rare and aggressive variant of chondrosarcoma 4, 11. Chondrosarcoma causes, symptoms, treatment, diagnoses. Cartilage is the specialized tissue that serves as a buffer or cushion at joints. It has a 10 year survival rate between 44 and 54 % 6, 7.
Apr 07, 2015 mesenchymal chondrosarcomamcs is a rare highgrade of variant of chondrosarcoma first described in 1959. The term chondrosarcoma is used to define an heterogeneous group of lesions with diverse features and clinical behavior. Most extraskeletal mesenchymal chondrosarcomas are fastgrowing and may spread to other parts of the body. Chondrosarcomas mesenchymal chondrosarcoma is a type of chondrosarcoma, which itself is a type of bone cancer that has its origins in cartilage cells. Histologically, mcs has a typical biphasic pattern consisting of both small cells and islands of atypical cartilage. Comprised primarily of noncartilaginous small, round, oval, or spindle shaped cells with islands of malignant cartilage dispersed throughout noncartilaginous component of tumor. The pattern of growth and scarcity of cartilaginous matrix result in frequent. Mesenchymal chondrosarcoma definition of mesenchymal. Mesenchymal chondrosarcoma comprises 210% of all chondrosarcomas 15. Chondrosarcoma symptoms, prognosis, treatment, survival rate. Mesenchymal chondrosarcoma is reported to have an incidence of 0. A brief summary of the histopathologic aspects of this entity is presented. A survey of some mesenchymal cartilage tumors and malignant chondroblastic tumors, including a few multicenter ones, as well as many atypical benign chondroblastomas and chondromyxoid fibromas. We report a 39yearold man with extraskeletal mesenchymal chondrosarcoma of the buttock, who had metastases to the pancreas, bones, and lung.
Sox9, a master regulator of chondrogenesis, distinguishes mesenchymal chondrosarcoma from other small blue round cell tumors. It develops from normal cartilage which goes through malignant change or can form within a preexisting benign tumor. Both central and peripheral chondrosarcoma can arise secondary to their benign precursor enchondroma and osteochondroma, respectively and they. Targeting survivin as a potential new treatment for. This communication was submitted for publication on jan 29. Mesenchymal chondrosarcoma is a rare disease with poor prognosis. Definition of extraskeletal mesenchymal chondrosarcoma nci. An analysis of patients treated at a single institution article pdf available in tumori 935. 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. It is a subtype of chondrosarcoma and is assumed to arise from remnants of the embryonic cartilage or metaplasia of meningeal fibroblasts.
Mesenchymal chondrosarcoma is a vicious but rare form of bone cancer. Chondrosarcoma patients with unresectable disease, due to tumour location, tumour size or extensive metastatic disease, have a 5 year survival of only 2 %. Primary mesenchymal chondrosarcoma of the kidney with. It was determined to show biphasic pattern with densely cellular regions of small anaplastic cells and other areas of chondroid differentiation, consistent with diagnosis of mesenchymal chondrosarcoma figures 24. Mesenchymal chondrosarcoma has a bimorphic histological appearance. Mesenchymal chondrosarcoma high grade small round blue cells with islands of benign appearing cartilage hemangiopericytomatous growth with staghorn like vessels aggressive radiographic findings 36. Extraskeletal chondrosarcoma radiology reference article. It rarely spreads to other parts of the body unless it has already come back several times in the original location.
Clear cell, mesenchymal, and dedifferentiated chondrosarcoma are rare, cartilaginous tumors with limited treatment options other than surgery. The following is important information for anyone who has received a diagnosis. Pathology they are found in both bone and soft tissues see. Primary mesenchymal chondrosarcoma of the cerebrum in. Download fulltext pdf download fulltext pdf download fulltext pdf. Chondrosarcomas are a group of malignant bone tumors that produce a cartilaginous matrix, typically in. A survey of some mesenchymal cartilage tumors and malignant chondroblastic tumors including a few multicenter ones, as well as many atypical benign chondroblastomas and chondromyxoid fibromas. Survival in mesenchymal chondrosarcoma varies based on age and tumor location. Mesenchymal chondrosarcoma mesenchymal chondrosarcoma is a rare form of cancer, but one that can spread throughout its victims quickly. Mesenchymal chondrosarcoma radiology reference article. Patients with mesenchymal chondrosarcoma who received.
This cancer begins its infection on the bone and on rare cases it can arise even outside the bone cartilages on the adjacent muscles. Aggressive surgical resection of mesenchymal chondrosarcoma with. Mesenchymal chondrosarcomas are a rare chondrosarcoma subtype. The dedifferentiated subtype is associated with a very low survival rate due to often distant metastatic involvement at time of initial diagnosis. He underwent a surgical resection of the left kidney and left hemicolon. Other rare chondrosarcoma subtypes include dedifferentiated, mesenchymal and clear cell chondrosarcoma. In children and adolescents, mesenchymal chondrosarcoma accounts for up to 25% of all chondrosarcomas. It is a cartilaginous producing spindle cell tumor sarcoma that occurs in the soft tissues as opposed to the more typical site in bone. Mesenchymal chondrosarcoma, although there is lack of. Riddle, md extraskeletal mesenchymal chondrosarcoma is a rare soft tissue sarcoma arising from soft tissues, mainly of the lower extremities, meninges, and orbits. Sequence of surgical interventions in a female patient with metastatic mesenchymal chondrosarcoma of the spleen. Lanzkowskys manual of pediatric hematology and oncology sixth edition, 2016. This rare variant of chondrosarcoma occurs in the deep soft tissues, usually of the limb girdles. Costal mesenchymal chondrosarcoma with diffuse pleural and pericardial explantation in a pygmy goat.
It usually presents during the second to third decades of life, and has a slight predominance in females. Chondrosarcoma is a malignant cancer that results in abnormal bone and cartilage growth. There is an uncertain differentiation there is no evidence yet showing that emc exhibits the feature of cartilaginous differentiation and neuroendocrine differentiation is even. Mesenchymal chondrosarcoma comprises about 2% of all. Genetic characterization of mesenchymal, clear cell, and. Epiphyseal gaint cell tumor chondroblastoma clear cell chondrosarcoma 35. Extraskeletal chondrosarcomas make up only 2% of softtissue sarcomas and only 1% of all chondrosarcomas.
Conventional chondrosarcoma represents around 85% of all chondrosarcomas and is notoriously difficult to treat with chemotherapy. Lifraumeni syndrome lfs is a rare cancer predisposition syndrome with a wide tumour spectrum, associated with tp53 germline mutations. Mesenchymal chondrosarcoma is a rare malignant neoplasm characterized by a bimorphic pattern that is composed of poorly differentiated small round cells and islands of well differentiated hyaline cartilage. Primary extraskeletal mesenchymal chondrosarcoma of the vulva. Primary mesenchymal chondrosarcoma of the kidney is rare, and it shows distinct undifferentiated tumor cells and well differentiated. Mesenchymal chondrosarcoma of the jaw bones vencio. Spinal mesenchymal chondrosarcomas are even rarer and, therefore, few. The diagnosis of mesenchymal chondrosarcoma can be challenging, it nonetheless has important therapeutic and. Conventional chondrosarcoma is a form of bone cancer that arises from cartilage cells. Pdf mesenchymal chondrosarcoma mcs is a rare high grade malignant tumor having both skeletal.
These lesions are referred to as extraskeletal chondrosarcoma, or softparts chondrosarcoma. Extraskeletal chondrosarcoma is rare, making up only 1% of reported chondrosarcoma. Pathology they tend to be of higher grade than runofthemill conventional intramedullary chondrosarcomas, with the majority being of t. Treatment including wide or radical excision is very important. Report of the first case in orbital location, was published. Approximately one third are extraskeletal and affect the soft tissues of.
Mesenchymal chondrosarcoma nord national organization. Mesenchymal chondrosarcoma mc is a rare tumour, with a predilection for the head and neck region. Abstractthe current study presents a case of extraskeletal mesenchymal chondrosarcoma esmc involving the vena. Mesenchymal chondrosarcomamcs is a rare highgrade variant of. Mesenchymal chondrosarcoma mc is an uncommon cartilaginous tumor that primarily occurs in adolescents and young adults, accounting for about 1% to 2% of all chondrosarcomas. Extraskeletal mesenchymal chondrosarcoma komal arora, md. Materials and methods this series consisted of 23 cases from the mayo clinic files and 88 from our consultation files, including 38 of 43 cases reported previously 5 tumors were excluded be cause adequate histologic material was no longer avail able.
Herein, we present a case with biopsyproven primary pulmonary chondrosarcoma after exclusion of primary origin elsewhere. Its computed tomography ct and magnetic resonance imaging mri and histopathological features and the management are presented. They are broadly classified under extra skeletal form of mesenchymal chondrosarcomas and account for less than 1% of all sarcomas. Mesenchymal chondrosarcomas of the head and neck the. Soft tissue chondrosarcomas are typically divided into myxoid, mesenchymal, and welldifferentiated type, which are very rare. Mesenchymal chondrosarcoma treatment, support and research. Chondrosarcoma is one of the most common malignant bone tumors in adults. Other variants of chondrosarcoma dedifferentiated, mesenchymal and clear cell chondrosarcoma are rare.
Mesenchymal chondrosarcoma article about mesenchymal. Our findings confirm that the small cell component of mesenchymal chondrosarcoma, as highlighted by expression of sox9 protein, has features of. We describe a case of mesenchymal chondrosarcoma arising in the right maxilla extending to the basisphenoid. The pathological findings showed chondrosarcoma in 7 cases and mesenchymal chondrosarcoma in 1 case. Mesenchymal chondrosarcoma jama ophthalmology jama network. It occurs in children and young adults considerably more often than in older people, though it is a very rare type of cancer overall. Due to the unique nature of this disease, management strategies are not well established. It is a primary malignant neoplasm of the bone and soft tissue, yet it is microscopically distinct from conventional and dedifferentiating chondrosarcoma. Primary intraspinal dumbbellshaped mesenchymal chondrosarcoma with massive calcifications. Management of renal extraskeletal mesenchymal chondrosarcoma.
Mesenchymal chondrosarcoma mcs is a rare histological variant of chondrosarcoma, with aggressive behaviour. Primary chondrosarcoma of the chest wall a case report. Survival in mesenchymal chondrosarcoma varies based on age. Immunohistochemical analysis of nuclear and cytoplasmic survivin in dedifferentiated, clear cell and mesenchymal chondrosarcoma. 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. Mesenchymal chondrosarcoma is an extremely rare, often aggressive form of cancer. Takahashi, kikuo sato, kenichi kanazawa, harusachi wang, xiaoli and kimura, takayuki 1993.
Chemotherapy improved prognosis of mesenchymal chondrosarcoma. If you do not see its contents the file may be temporarily unavailable at the journal website or you do not have a pdf plugin installed and enabled in your browser. The purpose of this study was to retrospectively identify the imaging features of mesenchymal chondrosarcoma of the orbit. Mesenchymal chondrosarcoma of bone and soft tissue.
This histological subtype occurs in both osseous and extraosseous tissues and has a tendency for late local and disseminated recurrence 1, 2, 4, 7. Extraskeletal mesenchymal chondrosarcoma of shoulder. Under the microscope, it appears to have both highly cellular areas composed mostly of only cancer cells and others composed of welldifferentiated cartilage. Conditional survival is greater than overall survival at diagnosis in patients. This variant of chondrosarcoma tends to occur in older patients and is more aggressive than usual chondrosarcomas. Extraskeletal chondrosarcoma emc is a rare, aggressive neoplasm which has been seen in the soft tissue area. Mesenchymal chondrosarcoma can have distinct areas of these two appearances or the areas can be relatively mixed up. Of the more than 11,000 primary neoplasms of bone recorded at the mayo clinic up to the beginning of 1994, only 34 patients had mesenchymal chondrosarcoma. Mesenchymal chondrosarcoma in children and young adults. Two patients had distant recurrences at 15 and 42 months, respectively.
94 68 1351 1546 967 988 118 765 61 722 610 270 445 165 787 1276 84 1418 876 236 1452 229 751 50 1129 264 964 1370 215 320 1118 358 296 354 662 170 777 1491