Objectiveshypothesis mesenchymal chondrosarcoma of the sinonasal tract is a rare, malignant tumor of extraskeletal origin. Primary mesenchymal chondrosarcoma of the lung the annals of. Few reports of the mr findings of mesenchymal chondrosarcoma exist in the literature. Also assident infiltrating urothelial carcinoma of the ureter is an extremely rare cancer. A challenging case of mesenchymal chondrosarcoma involving. The estimated overall incidence of chss is 1 in 200,000 per year, and it is the third most frequent malignant bone tumor after multiple myeloma and osteosarcoma. Mesenchymal chondrosarcomas showing immunohistochemical. As the orbit is a rare site of mesenchymal chondrosarcoma, here we report 3 pediatric cases with primary orbital. Pdf extraskeletal mesenchymal chondrosarcoma in an elderly. Approximately, a third of these tumors develop in extraskeletal sites such as the meninges, and somatic soft tissue. Mesenchymal chondrosarcoma is a malignant soft tissue tumor of uncertain lineage characterized at the genetic level by the hey1ncoa2 gene fusion. Commonly, mcs originates in the bone, but it can also arise in extraskeletal sites, such as the brain and the intraspinal area. 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.
Emerging pathways in the development of chondrosarcoma of. The pattern of growth and scarcity of cartilaginous. Extra skeletal myxoid chondrosarcomas typically occur in the extremities, with the thigh being most common. Conventional chondrosarcoma is notorious for its locally aggressive behaviour as well as for its resistance to chemotherapy and radiotherapy. It is a rare subtype of chondrosarcoma characterized by the presence of islands of chondroid or by less osteoid tissue enmeshed within dense sheets of primitive small blue mesenchymal cells with hemangiopericytomalike vessels, or by both. Mesenchymal chondrosarcomas are a rare chondrosarcoma subtype.
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. There is an uncertain differentiation there is no evidence yet showing that emc exhibits the feature of cartilaginous differentiation and. Conclusions mesenchymal chondrosarcoma of the sinonasal tract is an aggressive tumor with a predilection for young women. Pathology they are found in both bone and soft tissues see. Management of renal extraskeletal mesenchymal chondrosarcoma. An 18yearold woman presented with a tumor on her left fourth rib. Mesenchymal chondrosarcoma mc is a very rare malignant mesenchymal neoplasm of the bone and soft tissue. It is composed of round to slightly spindled primitive mesenchymal cells arrayed about a prominent hemangiopericytomalike vasculature, with production of relatively welldifferentiated cartilage. Extraskeletal mesenchymal chondrosarcoma is rare and metastasis to the pancreas is extremely rare, with only four cases reported in the literature. Extraskeletal chondrosarcoma radiology reference article. Both primary and metastatic mesenchymal chondrosarcoma found in the thyroid gland are extremely rare. Primary spinal intradural mesenchymal chondrosarcoma with.
It is malignant by nature and is more quick to develop and spread compared to the conventional forms of chondrosarcomas. 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 brain with diffuse enhancement. Mesenchymal chondrosarcomamcs is a rare highgrade of variant of chondrosarcoma first described in 1959. 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. The esmopaedcaneuracan guidelines are in agreement with nccn regarding chemotherapy regimens for mesenchymal chondrosarcoma, and they also note the following 22. Epiphyseal gaint cell tumor chondroblastoma clear cell chondrosarcoma 35. Histology mesenchymal chondrosarcomas are characterized by bimorphic histology. Mesenchymal chondrosarcoma is an aggressive, highgrade chondrosarcoma subtype with a reported 10year survival rate between 27 and 67%. Lifraumeni syndrome lfs is a rare cancer predisposition syndrome with a wide tumour spectrum, associated with tp53 germline mutations. Mesenchymal chondrosarcoma mc is an aggressive small, round, blue cell tumor with chondrogenic differentiation that typically arises in bony sites. Mesenchymal chondrosarcoma radiology reference article. Approximately two thirds of cases of mesenchymal chondrosarcoma occur in bone while the rest occur in places outside of the bonei.
These lesions are referred to as extraskeletal chondrosarcoma, or softparts chondrosarcoma. Mesenchymal chondrosarcoma mcs is an aggressive variant of chondrosarcoma and is a rare tumor, particularly within the pediatric population. Classically, mesenchymal chondrosarcoma shows stereotypic morphology, with primitive round to spindled cells, a prominent branching hemangiopericytomalike vasculature, islands of relatively. The observation of similar chromosomal abnormalities in both skeletal and extraskeletal mesenchymal chondrosarcoma supports a genetic as well as his. Mesenchymal chondrosarcoma high grade small round blue cells with islands of benign appearing cartilage hemangiopericytomatous growth with staghorn like vessels aggressive radiographic findings 36. The following is important information for anyone who has received a diagnosis. Due to the unique nature of this disease, management strategies are not well established. Teens and young adults are most prone to mesenchymal chondrosarcoma. Mesenchymal chondrosarcoma of bone and soft tissue. Mesenchymal chondrosarcoma mcs is a rare histological variant of chondrosarcoma, with aggressive behaviour. We report a case of primary mesenchymal chondrosarcoma occurring in the left kidney with an ipsilateral and. Primary mesenchymal chondrosarcoma of the kidney with.
Mesenchymal chondrosarcoma is a rare malignant cartilaginous tumor arising within the bone or soft tissue. Extraskeletal mesenchymal chondrosarcoma rd of the cases that involve the soft tissues of the body. For dedifferentiated chondrosarcoma, if wide margins cannot be achieved with limb salvage, consideration of amputation is recommended. Mesenchymal chondrosarcoma nord national organization. Mesenchymal chondrosarcoma, a recently described malignant neoplasm, usually occurs in bone. A diagnosis of extraskeletal mesenchymal chondrosarcoma was made based on the histological pattern characterized by the coexistence of cartilaginous islands and undifferentiated mesenchymal cells, results of alcian blue staining at various ph, immunohistochemical reactivity against vimentin and s100, and the absence of skeletal involvement.
Edge s, byrd dr, compton cc, fritz ag, greene fl, trotti a, eds. Spinal mesenchymal chondrosarcomas are even rarer and, therefore, few. Lifraumeni syndrome lfs is a rare cancer predisposition syndrome with a wide tumour spectrum, associated with tp53 germline. Mesenchymal chondrosarcoma is a malignant cancer of the cartilage, that tends to grow and spread more quickly than other similar tumors. The disease primarily affects young adults with a predilection for. Mesenchymal chondrosarcoma is an aggressive, uncommon histologic entity arising in bone and soft tissues. Radical resection with negative margins is the mainstay of treatment. Mesenchymal chondrosarcoma mcs was first described by lichtenstein and bernstein 1 in 1959.
We performed a wide resection of the tumor and administered three. Chondrosarcoma symptoms, prognosis, treatment, survival rate. Herein, we present a case with biopsyproven primary pulmonary chondrosarcoma after exclusion of primary origin. Primary mesenchymal chondrosarcoma of the lung the. Unlimited viewing of the articlechapter pdf and any associated supplements and figures. Type ii collagen can help distinguish it from other tumors. Mesenchymal chondrosarcoma was first described in the medical literature in 1959. Goldberg, md and holcombe grier, md also available in italian. Mesenchymal chondrosarcoma is a form of malignant chondrosarcoma. Approximately one third are extraskeletal and affect the soft tissues of the orbit, cranial and spinal meninges, and lower limbs. Unlike most chondrosarcomas, mesenchymal chondrosarcoma grows rapidly, tends to spread, and occurs more often in children and young adults than in older adults. Mesenchymal chondrosarcoma is a vicious but rare form of bone cancer.
We report a 39yearold man with extraskeletal mesenchymal chondrosarcoma of the buttock, who had metastases to the pancreas, bones, and lung. The risk factors for mesenchymal chondrosarcoma remain presently unidentified. Gk, mesos, middle, enchyma, infusion, chondros, cartilage, sarx, flesh, oma, tumor a rare malignant tumor of. It has characteristic radiological features and pathognomic histological biphasic pattern. Chondrosarcoma is a malignant cancer that results in abnormal bone and cartilage growth.
The mcs are wellcircumscribed, lobulated masses, with focal calcification. Chondrosarcomas are tumors consisting of osseous or cartilaginous stroma. An analysis of patients treated at a single institution. It is estimated that chss account for approximately 3. 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 has been well documented in the somatic soft tissue and bone. It occurs in children and young adults considerably more often than in older people, though it is a very rare type of cancer overall. They develop mostly on the inferior metaphysis of the femur, the pelvic bones, and the ribs. Primary mesenchymal chondrosarcoma of the cerebrum in. Histologically, mcs has a typical biphasic pattern consisting of both small cells and islands of atypical cartilage. Extraskeletal mesenchymal chondrosarcoma archives of. Isolated cases have been reported in the english literature, with no large series evaluating the clinicopathological aspects of these tumors. Chondrosarcomas mesenchymal chondrosarcoma is a type of chondrosarcoma, which itself is a type of bone cancer that has its origins in cartilage cells.
To our best knowledge, only 9 such cases have been reported so far. Chondrosarcoma article about chondrosarcoma by the free. Mesenchymal chondrosarcoma mcs is an unusual tumor mainly found in the skeleton. Mesenchymal chondrosarcoma mesenchymal chondrosarcoma is a rare form of cancer, but one that can spread throughout its victims quickly. Conditional survival is greater than overall survival at diagnosis in patients. Mesenchymal chondrosarcoma is a malignant type of chondrosarcoma, or cancer of cartilage. Purposemesenchymal chondrosarcoma mcs is an aggressive variant of. Two years after its original description, dahlin and henderson 2 reported 9 cases from the files of the mayo clinic. Mesenchymal chondrosarcoma is a rare tumour with orbital involvement being an exceptional occurrence. The therapeutic effectiveness of chemotherapy remains uncertain.
Immunohistochemical analysis of nuclear and cytoplasmic survivin in dedifferentiated, clear cell and mesenchymal chondrosarcoma. It is a rare subtype of chondrosarcoma characterized by the. Targeting survivin as a potential new treatment for. Mesenchymal chondrosarcoma in children and young adults. Mesenchymal chondrosarcoma of the sinonasal tract is an aggressive tumor with a predilection for young women. The pattern of growth and scarcity of cartilaginous matrix result in frequent. Chondrosarcoma a malignant tumor of cartilage tissue, sometimes accompanied by mucous degeneration. Pdf mesenchymal chondrosarcoma mcs is a rare high grade malignant tumor having both skeletal and. In this case, we report a rare presentation of mesenchymal chondrosarcoma of the thyroid gland. Mesenchymal chondrosarcoma of bone and soft tissue plos. Primary mesenchymal chondrosarcoma of the kidney is an extremely rare malignant tumor. The term chondrosarcoma is used to define an heterogeneous group of lesions with diverse features and clinical behavior. Comprised primarily of noncartilaginous small, round, oval, or spindle shaped cells with islands of malignant cartilage dispersed throughout noncartilaginous component of tumor.
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. Mesenchymal chondrosarcoma mcs is a rare highgrade of variant of chondrosarcoma first described in 1959. Patients with mesenchymal chondrosarcoma who received. Mesenchymal chondrosarcoma definition of mesenchymal.
Mesenchymal chondrosarcoma tends to appear more commonly in young people. Inhibition of bcl2 family members sensitizes mesenchymal. Reported 10year survival rates range from 10 to 54% 5,6,7,8,9,10. Prognosis for patients with mesenchymal chondrosarcoma is fair, with a 5year survival after wide resection of 60% and a 10year survival of 25%. Chondrosarcoma is a malignant cartilageforming tumour of bone, of which distinct clinicopathological subtypes are known. Survival in mesenchymal chondrosarcoma varies based on age. 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. Mesenchymal chondrosarcoma mcs is a distinct, very rare sarcoma with little evidence supporting treatment recommendations. Mesenchymal chondrosarcoma mcs accounts for 310% of all. Mesenchymal chondrosarcoma treatment, support and research. Mesenchymal chondrosarcoma comprises about 2% of all.
It only accounts for 1% to 10% of all chondrosarcomas24. People who have chondrosarcoma have a tumor growth starting from the medullary canal of a long and flat bone. Cutaneous extraskeletal mesenchymal chondrosarcoma in a. They occur at all ages but typically around the age of 50 3. Patients with dedifferentiated chondrosarcoma show a 5year overall survival between 7 and 24%. Pdf download for intraspinal mesenchymal chondrosarcoma. Mesenchymal chondrosarcoma an overview sciencedirect. Extraskeletal mesenchymal chondrosarcoma emcs is a rare malignant soft tissue tumor of chondroprogenitor cell origin. Primary mesenchymal chondrosarcoma of the kidney is rare, and it shows distinct undifferentiated tumor cells and well differentiated cartilagenous components. Extraskeletal orbital mesenchymal chondrosarcoma mc is an extremely rare and highly aggressive tumour. In the current paper, we present the case of a 67 yearold patient with recurrent left lumbar pain, increased fatigability and intermittent macroscopic hematuria. The cause of tumor formation is reportedly due to genetic mutations and chromosomal aberrations. Twenty percent of these tumors have metastatic disease at diagnosis.
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