It has been almost a quarter of a century that this rare, still poorly understood and to date insufficiently studied, cartilage neoplasm was described. Most cases of mesenchymal chondrosarcoma develop within the bones of the body, while the remaining third of cases are able to. Unlike other types of malignant chondrosarcoma, which have a tendency to grow more slowly and rarely spread, mesenchymal chondrosarcoma is a fast. Two years after its original description, dahlin and henderson 2 reported 9 cases from the files of the mayo clinic. The term secondary chondrosarcoma refers to those that develop by malignant transformation of cartilage in an osteochondroma. Mesenchymal chondrosarcoma nord national organization. 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. Nov 20, 2018 extraskeletal mesenchymal chondrosarcoma rd of the cases that involve the soft tissues of the body. In children and adolescents, mesenchymal chondrosarcoma accounts for up to 25% of all chondrosarcomas. As might be expected, imc has a much worse prognosis than classical chondrosarcoma, with a. Mesenchymal chondrosarcoma is a malignant type of chondrosarcoma, or cancer of cartilage. Ct scanning, mri, and plain radiographs may identify these lesions. It develops from normal cartilage which goes through malignant change or can form within a preexisting benign tumor.
In grade ii chondrosarcoma, higher nuclear survivin was seen in p53 overexpressing chondrosarcomas, as well as in grade iii chondrosarcoma p o0. Extraskeletal mesenchymal chondrosarcoma is a rare form of sarcoma that is not connected to bone, but typically involves the muscles or central nervous system. Cardiac chondrosarcoma, mesenchymal chondrosarcoma. Mesenchymal chondrosarcoma mc is an aggressive small, round, blue cell tumor with chondrogenic differentiation that typically arises in bony sites. Masson trichrome stain showed no collagen formation between the spindle cells of the.
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 is rare and metastasis to the pancreas is extremely rare, with only four cases reported in the literature. The disease primarily affects young adults with a predilection for flat bones such as the. The mcs are wellcircumscribed, lobulated masses, with focal calcification.
Extraskeletal mesenchymal chondrosarcoma of the thigh. Cartilage is the specialized tissue that serves as a buffer or cushion at joints. People who have chondrosarcoma have a single tumor growth which can vary in size and location when first discovered. Mesenchymal chondrosarcoma of the right side of the pelvis and thigh. The term chondrosarcoma is used to define an heterogeneous group of lesions with diverse features and clinical behavior. Mesenchymal chondrosarcoma an overview sciencedirect. Comprised primarily of noncartilaginous small, round, oval, or spindle shaped cells with islands of malignant cartilage dispersed throughout noncartilaginous component of tumor. We report a 39yearold man with extraskeletal mesenchymal chondrosarcoma of the buttock, who had metastases to the pancreas, bones, and lung. People who have chondrosarcoma have a tumor growth starting from the medullary canal of a long and flat bone. The majority of the cases are diagnosed in the second and third decade of life and the prognosis is highly variable with published 10year overall survival rates ranging from 21% to 67% 1. The risk factors for mesenchymal chondrosarcoma remain presently unidentified. Mesenchymal chondrosarcoma in the young springerlink. Mesenchymal chondrosarcoma of bone and soft tissue. Mesenchymal chondrosarcoma definition of mesenchymal.
Dec 04, 2018 for dedifferentiated chondrosarcoma, if wide margins cannot be achieved with limb salvage, consideration of amputation is recommended. Mesenchymal chondrosarcoma is a malignant cancer of the cartilage, that tends to grow and spread more quickly than other similar tumors. Extraskeletal mesenchymal chondrosarcoma rd of the cases that involve the soft tissues of the body. Mar 26, 2018 mesenchymal chondrosarcoma is a rare but deadly form of chondrosarcoma that typically affects adolescents and young adults. Additional, chondrosarcoma cells derived from primary tumors and enriched for cd3, which showed potent stem cell potential. And she has the physical bravery equivalent to any soldier fighting a war. Goldberg, md and holcombe grier, md also available in italian. They develop mostly on the inferior metaphysis of the femur, the pelvic bones, and the ribs.
They occur at all ages but typically around the age of 50 3. Chondrosarcoma symptoms, prognosis, treatment, survival rate. Primary chondrosarcoma of the heart european journal of cardio. European clinical trials in rare sarcomas within an. Mesenchymal chondrosarcoma nord national organization for. The role of mesenchymal stemprogenitor cells in sarcoma.
Approximately two thirds of cases of mesenchymal chondrosarcoma occur in bone, while the rest occur in soft tissue muscle or fat. Conventional chondrosarcoma is a form of bone cancer that arises from cartilage cells. Apr 07, 2015 mesenchymal chondrosarcomamcs is a rare highgrade of variant of chondrosarcoma first described in 1959. Clinicopathological study of six cases presenting as extraskeletal soft tissue masses saul suster, md, and cesar a, moran, md cartilage and boneforming tumors of the mediastinum are ex tremely rare neoplasms with very few cases having been reported in the literature.
An analysis of patients treated at a single institution. Mesenchymal chondrosarcoma in children and young adults. Different studies have supported the msc origin of osteosarcoma 20,22. Implication of immune system in chondrosarcoma progression and. It only accounts for 1% to 10% of all chondrosarcomas24. Chondrosarcoma is a collective term for a group of tumors that consist predominantly of cartilage and that range from lowgrade tumors with low metastatic potential to highgrade, aggressive tumors characterized by early metastasis.
The esmopaedcaneuracan guidelines are in agreement with nccn regarding chemotherapy regimens for mesenchymal chondrosarcoma, and they also note the following 22. Carly is a diva, a dancer, a good student, a staunch friend, a loving daughter, a silly sister, and an adored granddaughter. Unlike other types of chondrosarcoma, mesenchymal chondrosarcomas grow fast and often give rise to local recurrences and metastases. The pattern of growth and scarcity of cartilaginous matrix result in frequent. Mesenchymal chondrosarcoma can have distinct areas of these two appearances or the areas can be relatively mixed up. The cause of tumor formation is reportedly due to genetic mutations and chromosomal aberrations. Mesenchymal chondrosarcoma mcs was first described by lichtenstein and bernstein 1 in 1959. Chemotherapy improved prognosis of mesenchymal chondrosarcoma. Mesenchymal chondrosarcoma is an extremely rare, often aggressive form of cancer. Histologically, mcs has a typical biphasic pattern consisting of both small cells and islands of atypical cartilage. Mesenchymal chondrosarcoma is an aggressive, uncommon histologic entity arising in bone and soft tissues.
Extraskeletal chondrosarcoma radiology reference article. 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. Mesenchymal chondrosarcoma is a vicious but rare form of bone cancer. Mesenchymal chondrosarcomamcs is a rare highgrade of variant of chondrosarcoma first described in 1959. Mesenchymal chondrosarcoma mc is a very rare malignant mesenchymal neoplasm of the bone and soft tissue. Under the microscope, it appears to have both highly cellular areas composed mostly of only cancer cells and others composed of welldifferentiated cartilage. It usually presents during the second to third decades of life, and has a slight predominance in females. Extraskeletal mesenchymal chondrosarcoma is a rare soft tissue sarcoma arising from soft tissues, mainly of the. Extra skeletal myxoid chondrosarcomas typically occur in the extremities, with the thigh being most common. Extraskeletal mesenchymal chondrosarcoma archives of. 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. Dang vu nguyen, ahmad sobri muda, and yazmin yaacob.
Mesenchymal chondrosarcoma an overview sciencedirect topics. Based on 19 cases in the young representing 26 percent of chondrosarcoma patients under the age of 21 years, this study found equal sex distribution with the youngest patient being a 6year old boy. These lesions are referred to as extraskeletal chondrosarcoma, or softparts chondrosarcoma. At operation, the tumour was observed to arise from the left sixth rib and was adherent to the underly ing pleura. Approximately, a third of these tumors develop in extraskeletal sites such as the meninges, and somatic soft tissue. It occurs in children and young adults considerably more often than in older people, though it is a very rare type of cancer overall.
Histology mesenchymal chondrosarcomas are characterized by bimorphic histology. The therapeutic effectiveness of chemotherapy remains uncertain. It is malignant by nature and is more quick to develop and spread compared to the conventional forms of chondrosarcomas. Pathology they are found in both bone and soft tissues see. Prognosis for patients with mesenchymal chondrosarcoma is fair, with a 5year survival after wide resection of 60% and a 10year survival of 25%. Extraskeletal mesenchymal chondrosarcoma is a rare soft tissue sarcoma. One third to one half of mcs are extraosseous in origin.
The rest of the time, the cancer actually develops in other areas of the. Type ii collagen can help distinguish it from other tumors. Mesenchymal chondrosarcoma comprises 210% of all chondrosarcomas 15. Tsuchida and colleagues firstly reported that sp cells isolated from the osteosarcoma cell line by exposure of the hos osteosarcoma cell line to cisplatin showed stem celllike properties. Extraskeletal mesenchymal chondrosarcoma komal arora, md. 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. Pdf chondrosarcomas are malignant cartilageforming bone tumors, which are. Mesenchymal chondrosarcoma has a bimorphic histological appearance. Mesenchymal chondrosarcoma radiology reference article. Comprised primarily of noncartilaginous small, round, oval, or spindle. It is composed of round to slightly spindled primitive mesenchymal cells arrayed about a prominent hemangiopericytomalike vasculature, with production of relatively welldifferentiated cartilage. Chondrosarcoma is reported most frequently in the dog, where it accounts for 10% of primary tumors of bones and is second only to osteosarcoma in incidence. The cartilaginous areas were sharply demarcated and merged gradually into surrounding small cell areas. Mesenchymal chondrosarcoma portnotes orthopaedicsone.
Approximately two thirds of cases of mesenchymal chondrosarcoma occur in bone while the rest occur in places outside of the bonei. Mesenchymal chondrosarcoma mc accounts for 3% to 5% of all chondrosarcomas. Chondrosarcoma is a malignant cancer that results in abnormal bone and cartilage growth. Mesenchymal chondrosarcoma is a rare but deadly form of chondrosarcoma that typically affects adolescents and young adults. 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. Mesenchymal chondrosarcomas are a rare chondrosarcoma subtype. Pathology they tend to be of higher grade than runofthemill conventional intramedullary chondrosarcomas, with the majority being of t. Oct 27, 2014 this suggests that chondrosarcoma progression probably parallels deregulated chondrocytes differentiation process of mscs 60,61. Gk, mesos, middle, enchyma, infusion, chondros, cartilage, sarx, flesh, oma, tumor a rare malignant tumor of. Mesenchymal chondrosarcoma mcs is a rare highgrade of variant of chondrosarcoma first described in 1959. Approximately one third are extraskeletal and affect the soft tissues of. Mesenchymal chondrosarcoma treatment, support and research.
The authors describe the ct and plain film appear ance of orbital mesenchymal chondrosarcoma in a 21yearold woman and 28yearold man, ie, large expansile masses with heavy or stippled calcifications and no evident erosion of bone. Primary mesenchymal chondrosarcoma of the cerebrum in. The clinical symptoms associated with intracranial mesenchymal chondrosarcoma are quite. These tumors are generally painless, contributing to the frequent delays in their diagnosis. While curative intent is possib thus, it is imperative to understand the fusiondriven biology of this rare malignant neoplasm so as to lead to the future development of better therapeutics for this disease. Teens and young adults are most prone to mesenchymal chondrosarcoma. Mcs differs from typical chondrosarcomas in the following respects.
Extraskeletal mesenchymal chondrosarcoma emcs is a rare malignant soft tissue tumor of chondroprogenitor cell origin. Unlike most chondrosarcomas, mesenchymal chondrosarcoma grows rapidly, tends to spread, and occurs more often in children and young adults than in older adults. Originally, it was considered restricted to bone, but that is no. At 4 years old, when told that swallowing pills would not taste as bad as liquid medicine. Mesenchymal chondrosarcoma article about mesenchymal. Mesenchymal chondrosarcoma is a form of malignant chondrosarcoma. Mesenchymal chondrosarcoma is a type of chondrosarcoma, which itself is a type of bone cancer that has its origins in cartilage cells. Riddle, md extraskeletal mesenchymal chondrosarcoma is a rare soft tissue sarcoma arising from soft tissues, mainly of the lower extremities, meninges, and orbits. Lightenstein and bernstein first described mesenchymal chondrosarcomas in 1959, and dowling reported the first case of an extraskeletal mesenchymal chondrosarcoma in 1964. 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. It is commonly thought that a history of trauma could be a.
Extraskeletal chondrosarcomas make up only 2% of softtissue sarcomas and only 1% of all chondrosarcomas. Intracranialiintraspinal extraosseous mesenchymal chondrosarcomas. The mesenchymal chondrosarcoma is a rare malignant tumor composed of small. Mesenchymal chondrosarcoma of the sinonasal tract is an aggressive tumor with a predilection for young women. Mesenchymal chondrosarcoma deep femoral vein deep venous thrombosis intravascular tumor. Chondrosarcoma a malignant tumor of cartilage tissue, sometimes accompanied by mucous degeneration.
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