Mesenchymal chondrosarcoma radiology reference article. The most common locations are the jaw, pelvis, spine, and scapula. Mesenchymal chondrosarcomas arise both in bone 70% and in the soft tissues 30%. Chondrosarcoma is a malignant cancer that results in abnormal bone and cartilage growth.
Health, general cancer chemotherapy stem cells tumors prognosis. Distant metastasesfree survival rates were 37% at 5 years and. Mesenchymal chondrosarcoma is a form of malignant chondrosarcoma. The following is important information for anyone who has received a diagnosis. 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. It represents only 2% to 10% of all chondrosarcomas27 and has a frequency of 0. Mesenchymal chondrosarcoma mc is an aggressive small, round, blue cell tumor with chondrogenic differentiation that typically arises in bony sites. A free powerpoint ppt presentation displayed as a flash slide show on id. Chondrosarcoma a malignant tumor of cartilage tissue, sometimes accompanied by mucous degeneration. Intracranial mesenchymal chondrosarcoma is very rare, only 14 cases being reported in europe and in the united states of america.
No targets for therapy have been reported for dedifferentiated chondrosarcoma. It is an extremely rare malignant tumor of the kidney, and we report what we believe is only the fifth case of primary renal chondrosarcoma published in the english literature, in which the tumor size is the smallest reported, and it is also the first case of a 6. Although the oncologic outcome for patients with pelvic and chest wall chondrosarcoma has been reported, 57 site. 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. The term chondrosarcoma is used to define an heterogeneous group of lesions with diverse features and clinical behavior. Histology mesenchymal chondrosarcomas are characterized by bimorphic histology. Extraskeletal mesenchymal chondrosarcoma of the orbit. Mesenchymal chondrosarcoma was first described in the medical literature in 1959. Survival in mesenchymal chondrosarcoma varies based on age. Mesenchymal chondrosarcoma treatment, support and research. We describe a case of mesenchymal chondrosarcoma arising in the right maxilla extending to the basisphenoid. Goldberg, md and holcombe grier, md also available in italian. Recently we experienced a case in which the followup indicating. Mesenchymal chondrosarcoma definition of mesenchymal.
Find out about the symptoms, causes, and treatment of chondrosarcoma, a type of bone cancer most common in your pelvis, thighbone, or upper arm. Study on trabectedin in advanced rearranged mesenchymal chondrosarcoma isgmcs the safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Mesenchymal chondrosarcoma mc is a rare tumour, with a predilection for the head and neck region. The aim of the present study was to report a case of chondrosarcoma in the scapula. Definition of extraskeletal mesenchymal chondrosarcoma nci.
It is interesting to note that most of the metastatic tumors four of seven were considered high. Mesenchymal chondrosarcoma of bone and soft tissue. They develop mostly on the inferior metaphysis of the femur, the pelvic bones, and the ribs. Their typical histological appearance includes a biphasic pattern with areas of round, primitive mesenchymal cells and interspersed islands of well differentiated hyaline cartilage. The defining characteristic of a chondrosarcoma is that its cells produce cartilage. Few reports of the mr findings of mesenchymal chondrosarcoma exist in the literature.
Mesenchymal chondrosarcoma nord national organization. Mesenchymal chondrosarcoma is a malignant cancerous type of chondrosarcoma, or cancer of cartilage connective tissue. Mesenchymal chondrosarcoma introduction mesenchymal chondrosarcoma, first discirbed by lichtenstein and bernstein cancer 1959. 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. Very little data were available on the presence of dural invasion in skull base chondrosarcoma. Mesenchymal chondrosarcomas are a rare chondrosarcoma subtype. Mesenchymal chondrosarcoma of the soft tissues represents a very rare tumor, even rarer than its skeleton. Mesenchymal chondrosarcoma is a malignant cancer of the cartilage, that tends to grow and spread more quickly than other similar tumors. Mesenchymal chondrosarcoma comprises about 2% of all chondrosarcomas. Aggressive surgical resection of mesenchymal chondrosarcoma with. The most common locations for chondrosarcoma tumors are in the pelvis, hip and shoulder. Pathology they tend to be of higher grade than runofthemill conventional intramedullary chondrosarcomas, with the majority being of t. 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.
Histologically, mcs has a typical biphasic pattern consisting of both small cells and islands of atypical cartilage. Pathology they are found in both bone and soft tissues see. Apr 07, 2015 mesenchymal chondrosarcomamcs is a rare highgrade of variant of chondrosarcoma first described in 1959. Mesenchymal chondrosarcoma oxford university hospitals. Mesenchymal chondrosarcoma of the sinonasal tract is an aggressive tumor with a predilection for young women. Mesenchymal chondrosarcoma of the sinonasal tract lester dr. Mesenchymal chondrosarcoma can have distinct areas of these two appearances or the areas can be relatively mixed up. Listing a study does not mean it has been evaluated by the u. In patients without metastatic disease, mean survival was 37 months.
Its computed tomography ct and magnetic resonance imaging mri and histopathological features and the management are presented. Sites femur, ribs, jaw, skull, and spine are most common, though it may occur in any bone. As the incidence of mesenchymal chondrosarcoma is extremely low, the limited number of patients discussed in most of. Mesenchymal chondrosarcoma in children and young adults. Mesenchymal chondrosarcoma is a malignant type of chondrosarcoma, or cancer of cartilage. It is malignant by nature and is more quick to develop and spread compared to the conventional forms of chondrosarcomas. Rare variant of chondrosarcoma 210% of primary chondrosarcomas. Chondrosarcomas mesenchymal chondrosarcoma is a type of chondrosarcoma, which itself is a type of bone cancer that has its origins in cartilage cells. Mesenchymal chondrosarcoma is a rare, highgrade malignancy of bone or soft tissue first described by lichtenstein and bernstein1 in 1959. Imaging features of extraskeletal mesenchymal chondrosarcoma of. Pdf intracranial mesenchymal chondrosarcoma mahendra. Because of its rarity and variable length of diseasefree survival, the natural.
The grade of the tumor is extremely important in terms of overall and disease free survival. Chondrosarcoma symptoms, prognosis, treatment, survival rate. Mesenchymal chondrosarcoma is a highly malignant lesion that can occur in bone and soft tissue of relatively young patients and is characterized by varying amounts of differentiated cartilage admixed with undifferentiated small round cells. Pdf extraskeletal mesenchymal chondrosarcoma of the.
At one year followup the patient is free of disease. It exhibits a high propensity for hematogenous dissemination and is associated with significant morbidity and mortality. Mesenchymal chondrosarcoma international journal of particle. Approximately two thirds of cases of mesenchymal chondrosarcoma occur in bone while the rest occur in places outside of the bonei. Extraskeletal mesenchymal chondrosarcoma archives of. Mesenchymal chondrosarcoma mcs was first described by lichtenstein and bernstein 1 in 1959. Two years after its original description, dahlin and henderson 2 reported 9 cases from the files of the mayo clinic. Lifraumeni syndrome lfs is a rare cancer predisposition syndrome with a wide tumour spectrum, associated with tp53 germline mutations. Chondrosarcoma definition in the cambridge english. Studies suggest that mesenchymal chondrosarcoma is associated with a poorer prognosis and a. Alternative treatments for mesenchymal chondrosarcoma. Mesenchymal chondrosarcoma an overview sciencedirect topics. Study on trabectedin in advanced rearranged mesenchymal.
Extraskeletal chondrosarcomas make up only 2% of softtissue sarcomas and only 1% of all chondrosarcomas. The mcs are wellcircumscribed, lobulated masses, with focal calcification. It is most frequently found in adults in their third decade of life. Chondrosarcoma is a rare type of cancer that usually begins in the bones, but can sometimes occur in the soft tissue near bones.
Mesenchymal chondrosarcoma is one of the most unusual neoplasms involving bone and soft tissue. However, certain subtypes of chondrosarcoma may be responsive to chemotherapy. Mesenchymal chondrosarcoma, sinonasal tract, nasal cavity, prognosis. Primary spinal intradural mesenchymal chondrosarcoma with. Mesenchymal chondrosarcoma of the maxilla the journal of. Mesenchymal chondrosarcoma of the jaw bones vencio 1998. Synonyms for mesenchymal chondrosarcoma in free thesaurus. Mesenchymal chondrosarcoma mesenchymal chondrosarcoma is a rare form of cancer, but one that can spread throughout its victims quickly. Introduction mesenchymal chondrosarcoma comprises 210% of all chondrosarcomas 15. For nonmetastatic mesenchymal chondrosarcoma, chemotherapy as well as negative surgical margins have been shown to be associated with improved overall and event free survival in some trials frezza, eur j cancer.
Rationale chondrosarcoma is a malignant mesenchymal tumor originating from cartilage. Prognosis for patients with mesenchymal chondrosarcoma is fair, with a 5year survival after wide resection of. Most commonly intraosseous but can occur in extraskeletal sites especially the central nervous system from the meninges. Several variants of cs have been proposed including clear cell, dedifferentiated, myxoid and mesenchymal. Mesenchymal chondrosarcoma mc is a rare, aggressive small round blue cell malignancy that may arise in bone or soft tissue 110. Comprised primarily of noncartilaginous small, round, oval, or spindle shaped cells with islands of malignant cartilage dispersed throughout noncartilaginous component of tumor. Approximately, a third of these tumors develop in extraskeletal sites such as the meninges, and somatic soft tissue. The clinical approach towards chondrosarcoma gelderblom. The recommended treatment is resection with wide margins. People who have chondrosarcoma have a tumor growth starting from the medullary canal of a long and flat bone. Mesenchymal chondrosarcomamcs is a rare highgrade of variant of chondrosarcoma first described in 1959. Unlike most chondrosarcomas, mesenchymal chondrosarcoma grows rapidly, tends to spread, and occurs more often in children and young adults than in older adults.
Chondrosarcoma frequently involves the flat bones, including the pelvis, ribs, sternum, and scapula. Extraskeletal mesenchymal chondrosarcoma emcs is a rare malignant soft tissue tumor of chondroprogenitor cell origin. Mesenchymal chondrosarcomamcs is a rare highgrade variant of. The patient is currently free of symptoms at two years following the. Mesenchymal chondrosarcoma mc is a rare variant of cs that accounts for up to 39% of all cs and has high predilection for the head and neck region. This tumor is most common in children and young adults aged 1030, but can affect individuals of any age including young children and older adults. Background mesenchymal chondrosarcoma mcs is a rare tumor with a strong tendency toward late recurrences leading to reported 10year survival rates below 50%. Extraskeletal mesenchymal chondrosarcoma of the kidney. It characteristically contains undifferentiated mesenchymal cells admixed with mature chondrocytes.
There is a significant difference in 10year survival rates between patients with lowgrade lesions and patients with highgrade lesions. Extraskeletal mesenchymal chondrosarcoma tends to arise in the head and neck region, and the lower extremities. Chidamearn and sanville 4 described a chondrosarcoma of the right maxilla that on contrastenhanced t2weighted imaging appears heterogeneous but has a predominantly isointense signal relative to. Jun 17, 2005 the capacity for metastasis most likely was intrinsic to the genetic constitution of the tumors. New hope unlimited offers alternative mesenchymal chondrosarcoma treatments that restore the bodys disease free condition.
An analysis of patients treated at a single institution. Mesenchymal chondrosarcoma mcs is a rare highgrade of variant of chondrosarcoma first described in 1959. Dec 04, 2018 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 grade i chondrosarcoma had no clear age preference. Ppt mesenchymal chondrosarcoma powerpoint presentation. Mesenchymal chondrosarcoma mcs is a rare histological variant of chondrosarcoma, with aggressive behaviour. In children and adolescents, mesenchymal chondrosarcoma accounts for up to 25% of all. Mesenchymal chondrosarcoma nord national organization for.
Mesenchymal chondrosarcoma comprises 210% of all chondrosarcomas 15. This histological subtype occurs in both osseous and extraosseous tissues and has a tendency for late local and disseminated recurrence 1, 2, 4, 7. They are two to three times more common in bone than in soft tissue and are mostly found in the. Mesenchymal chondrosarcoma international journal of. Mesenchymal chondrosarcoma article about mesenchymal. The more malignant mesenchymal chondrosarcoma had a tendency towards a malign growth pattern which is illustrated by the relatively high frequency of extension into dural and cerebral tissue. Most cases of mesenchymal chondrosarcoma develop within the bones of the body, while the remaining third of cases are able to. Mesenchymal chondrosarcoma is a vicious but rare form of bone cancer. Chondrosarcoma of the scapula pant 2005 cancer wiley. The disease recently was reported to have a median event free survival of 57 months 25. It occurs in children and young adults considerably more often than in older people, though it is a very rare type of cancer overall. Approximately one third are extraskeletal and affect the soft tissues of. The pelvis, ribs, femur, and humerus are the most frequently affected sites, and scapula involvement is relatively rare.
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. Due to the unique nature of this disease, management strategies are not well established. The pattern of growth and scarcity of cartilaginous matrix result in frequent. In children and adolescents, mesenchymal chondrosarcoma accounts for up to 25% of all chondrosarcomas. An analysis of patients treated at a single institution article pdf available in tumori 935. Spinal mesenchymal chondrosarcomas are even rarer and, therefore, few. In our case, the mesenchymal chondrosarcoma, which is a rare malignant tumor and accounts for less than 3% of primary chondrosarcomas, is characterized by relatively common originating from soft tissue, different from conventional chondrosarcoma. Extraskeletal chondrosarcoma radiology reference article. Mesenchymal chondrosarcoma has a bimorphic histological appearance. It only accounts for 1% to 10% of all chondrosarcomas24. Mesenchymal chondrosarcomas are rare tumors that account for 210% of primary chondrosarcomas. What hiring strategies have worked for you in this covid19 pandemic era. This histological subtype occurs in both osseous and extraosseous tissues 6 and has a tendency for late local and disseminated recurrence 1, 2, 4, 7. The mean survival for cases with metastatic disease was 14.
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