Case report myxoid chondrosarcoma of maxilla in a pediatric. Sep 05, 2011 lobulation pattern and fibrous tissue formation around the tumor can be an effective and helpful indicator for histopathologic differentiation between enchondroma and welldifferentiated chondrosarcoma. The condition in children differs to that in adults and to skeletal myxoid chondrosarcoma. Myxoid chondrosarcoma chordoid sarcoma of bone kilpatrick. Histopathology, immunohistochemistry, and molecular pathology testing revealed intraosseous myxoid chondrosarcoma. Chondrosarcoma is a malignant cartilaginous group of tumors with highly diverse features and behavior patterns that characteristically produce cartilage matrix from neoplastic tissue devoid of osteoid in which ossification, calcification, and myxoid changes can occur. Oct 11, 2012 extraskeletal myxoid chondrosarcoma emcs is a rare soft tissue sarcoma of uncertain differentiation, characterized in most cases by a translocation that results in the fusion protein ewsr1chn the latter even called nr4a3 or tec. Cytopathology of extraskeletal myxoid chondrosarcoma. Usually a good prognosis 75% five year survival in one large data set. Mri is the modality of choice for characterization of myxoid softtis.
Unusual extraskeletal myxoid chondrosarcoma in a child. Despite their rarity, they present with characteristic clinical and histopathologic features distinguishing them from conventional type of chondrosarcoma. Lobulation pattern and fibrous tissue formation around the tumor can be an effective and helpful indicator for histopathologic differentiation between enchondroma and welldifferentiated chondrosarcoma. Cytology and histopathology of an unusual neck mass. Extraskeletal myxoid chondrosarcoma emcs is a rare soft tissue sarcoma of uncertain differentiation, characterized in most cases by a translocation that results in the fusion protein ewsr1chn the latter even called nr4a3 or tec.
A case of highly aggressive extraskeletal myxoid chondrosarcoma. Its diagnosis can be challenging, with the differential. Extraskeletal myxoid chondrosarcoma with neuroendocrine. Extraskeletal myxoid chondrosarcoma emc is aggressive in children. Dedifferentiated chondrosarcoma, although typically arising from conventional chondrosarcoma, is.
Myxoid variant of chondrosarcoma is an uncommon potentially lethal malignant tumor which is even rare in pediatric age group. Histology alone is inadequate for the diagnosis of emc. 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. It involves the deep soft tissues of the proximal limbs, especially the thigh and popliteal fossa. Age this tumour usually occurs in adults, between 30 and 60 years siteusually located on the hands and feet gross firm, well demarcated, lobulated lesion, not more than 3cm in maximum diameter microscopic features well circumscribed, multinodular tumour. Article pdf available in biomed research international 20184. Chondrosarcoma is a rare type of cancer that usually begins in the bones, but can sometimes occur in the soft tissue near bones. It is generally resistant to conventional chemotherapy, and, to the best of. Males are affected about twice as often as females. A diagnosis of a myxoid sarcoma favoring an emc was made in this patient. A rare case report pranalinimonkar, 1 nitinbhola, 1 anenddjadhav, 1 anujjain, 1 rajivborle, 1 rajulranka, 2 andminalchaudhary 2 department of oral and maxillofacial surgery, sharad. Finally, socalled extraskeletal myxoid chondrosarcoma, which may rarely arise primary in bone, is also discussed despite its uncertain histogenesis. Chondrosarcoma symptoms, prognosis, treatment, survival rate. It is generally resistant to conventional chemotherapy, and, to the best of our.
Chondrosarcoma mesenchymal and extraskeletal myxoid symptoms, treatment, prognosis, survival rate. Myxoid chondrosarcoma an overview sciencedirect topics. Myxoid chondrosarcoma is a type of chondrosarcoma it has been associated with a t9. To the authors knowledge, there are limited data examining chemotherapy outcomes as a guide to therapeutic decisions for unresectable disease. Extraskeletal myxoid chondrosarcoma of the thigh in a. These tumors can be ditinguished by using tissue patterns derived from biologic nature. Extraskeletal myxoid chondrosarcoma extraskeletal myxoid chondrosarcoma. Extraskeletal myxoid chondrosarcoma is a rare subtype of sarcoma that typically affects the deep soft tissue of adults mainly in the 4th to 7th decade of life, with an approximately 2.
We report a case of ppms with molecular confirmation using fluorescence in situ hybridization fish and dna sequencing in a 45yearold female patient. Case report myxoid chondrosarcoma of maxilla in a pediatric patient. The most common locations for chondrosarcoma tumors are in the pelvis, hip and shoulder. Histopathology salivary glandpleomorphic adenoma mixed tu duration. Histopathologic differentiation between enchondroma and well. Histopathology images of extraskeletal myxoid chondrosarcoma. Two years after its original description, dahlin and henderson 2 reported 9 cases from the files of the mayo clinic.
The histologic subtypes of chondrosarcoma are uncommon. Extraskeletal myxoid chondrosarcoma emcs, initially recognized by stout and verner in 1953, is a rare soft tissue malignancy. Pdf extraskeletal myxoid chondrosarcoma is a rare soft tissue tumor of the extremities. Pathology of extraskeletal chondroosseous tumours dr. Department of laboratory medicine and pathology amo, tjs, tag, agn and the department of. We describe herein, a 51yearold asian female initially manifested with signs of severe anemia who presented with. It is found in both bone and soft tissues see extraskeletal chondrosarcoma. Dedifferentiated chondrosarcoma, although typically arising from conventional. My approach to myxoid soft tissue tumours site size level architecture vessels cellularity atypia immunohistochemistry andor molecular pathology van roggen j, hogendoorn p, fletcher c. Extraskeletal myxoid chondrosarcoma emc is a rare soft. Abstract background skeletal myxoid chondrosarcoma smc is considered to be. Grade and stage are independent predictors of survival.
Aug 17, 2011 a case of highly aggressive extraskeletal myxoid chondrosarcoma article pdf available in case reports in oncology 42. The fourth edition of the world health organization who classification of tumors of soft tissue and bone published in 20 updated the older 2002 classification, introduced several changes in softtissue tumor classification, and described new genetic and molecular data for the tumors 5, 6. These tumours, which principally presented in middle. Webpathology is a free educational resource with 10255 high quality pathology images of benign and malignant neoplasms and related entities. With the exception of a few small series, the cytology literature of emc is largely limited to single. Pdf extraskeletal myxoid chondrosarcoma emc is a rare soft tissue. Extraskeletal myxoid chondrosarcoma esmc is a rare soft tissue sarcoma, of which its histopathologic and cytogenetic features have been. Five months later, a small mass was noted at the right lower lobe of the lung. Myxoid liposarcoma lipoblasts and vessels with branching pattern present, less pleomorphic. Read unusual extraskeletal myxoid chondrosarcoma in a child, histopathology on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at. Skeletal and extraskeletal myxoid chondrosarcoma antonescu. Myxoid chondrosarcoma has a median age at diagnosis of 50 years and is more commonly diagnosed in males than females at a ratio of approximately 2.
Extraskeletal myxoid chondrosarcoma, neuroendocrine differentiation, histochemistry, immunohistochemistry, ultrastructure, t9. Insm1 expression and its diagnostic significance in. Mean ages reported in various series range from 46 to 57 years. Extraskeletal mesenchymal chondrosarcoma emcs is a rare malignant soft tissue tumor of chondroprogenitor cell origin. Extraskeletal mesenchymal chondrosarcoma archives of. Extraskeletal myxoid chondrosarcoma emc is a genetically distinct sarcoma with a propensity for local recurrence and metastasis despite an indolent course. Extraskeletal myxoid chondrosarcoma chordoid sarcoma. Myxoid leiomyosarcomamyxoid variants of nerve sheath tumour and smooth muscle tumour immunohistochemistry plays an important role in establishing the final diagnosis. We report on a 9yearold girl with emc in her left thigh. Chondrosarcoma is a form of cancer that results from the abnormal cellular.
The defining characteristic of a chondrosarcoma is that its cells produce cartilage. Extraskeletal myxoid chondrosarcoma of the thigh in a child. Sep 11, 2018 extraskeletal myxoid chondrosarcoma emc is an infrequent, lowgrade tumor of the soft tissues, forming around bones. In the present paper, we report one such case of intermediate grade myxoid chondrosarcoma of left side of maxilla in a 12yearold girl. Extraskeletal myxoid chondrosarcoma is a rare tumor, encompassing 2. Case report primary intraosseous myxoid chondrosarcoma arising. Extraskeletal myxoid chondrosarcoma emc is a rare soft tissue sarcoma that was recognized as a distinct pathologic entity by stout and verner in 1953. Morphologically distinct soft tissue chordoid sarcoma cs, or extraskeletal myxoid chondrosarcoma, is a relatively rare tumor that has generally been documented in extraosseous soft tissues. Histologic variants of chondrosarcoma diagnostic histopathology. Molecular analysis of the fusion of ews to an orphan nuclear receptor gene in extraskeletal myxoid chondrosarcoma. Myxoid leiomyosarcoma myxoid variants of nerve sheath tumour and smooth muscle tumour immunohistochemistry plays an important role in establishing the final diagnosis. A rare case report pranalinimonkar, 1 nitinbhola, 1 anenddjadhav, 1 anujjain, 1 rajivborle, 1 rajulranka, 2 andminalchaudhary 2 department of oral and maxillofacial surgery, sharad pawar dental college and hospital, datta meghe institute of medical sciences, sawangi, wardha. Subsequent excision of the mass for histopathological examination confirmed this. Primary pulmonary myxoid sarcoma with ewsr1creb1 fusion.
Rare, first described in 1972 50 65% males, median age 50 52 years, range 6 89 years 80% in proximal extremities or limb girdles, 20% in trunk. A case of highly aggressive extraskeletal myxoid chondrosarcoma article pdf available in case reports in oncology 42. The present case had a firm, painless, and lobulated growth in premolarmolar region which was associated with bicortical expansion. 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. Extraskeletal myxoid chondrosarcoma, sarcoma, soft tissue, histopathology, tumor grade introduction extraskeletal myxoid chondrosarcoma emc is a rare malignancy with distinctive morphologic, histologic, and immunohistochemical features. It was defined as a distinct clinicopathologic entity by enzinger and shiraki in 1972 and was called chordoid sarcoma because it resembles chordoma histologically. Abstract background chondrosarcoma of bone is a well.
The purpose of this study was to achieve better understanding of extraskeletal myxoid chondrosarcoma emc. Chondrosarcoma of bone is a well recognized, relatively common clinicopathologic entity. Among six patients who underwent preoperative ct examinations, six cases of lesions exhibited hypodensity on unenhanced image, three cases of tumor. Initially, the pain occurred only with weightbearing, but is now beginning to bother him while sitting and has awoken him from sleep on a few occasions. Pdf a case of highly aggressive extraskeletal myxoid. Extraskeletal myxoid chondrosarcoma with neuroendocrine differentiation. Epidemiology typically affects patients in their 30s to 60. All patients underwent preoperative ct orand mri examinations.
Subsequent excision of the mass for histopathological examination. Traditionally regarded a lowgrade indolent malignancy with only late local recurrences, 40% to 50% of cases are now acknowledged to metastasize after 10 or more years. Aug 28, 2012 extraskeletal myxoid chondrosarcoma emc is a rare softtissue sarcoma, which primarily occurs deep in the extremities, especially in skeletal muscle, or tendon. This is an uncommon soft tissue sarcoma that has morphologic and immunohistochemical resemblance to skeletal myxoid chondrosarcomas. 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. Dec 15, 2008 extraskeletal myxoid chondrosarcoma emc is a genetically distinct sarcoma with a propensity for local recurrence and metastasis despite an indolent course. Extraskeletal myxoid chondrosarcoma emc is a slowly growing, painless tumor of adults over 35 years of age. Fine needle aspiration cytology of primary extraskeletal myxoid chondrosarcoma. Jan 12, 2018 extraskeletal myxoid chondrosarcoma is a rare subtype of sarcoma that affects the soft tissue and bones in middleaged and elderly adults. Emcs is marked by 40% incidence of metastases in spite of its indolent behaviour. Middleaged to elderly individuals are most prone to this tumor formation. Apr 21, 2014 the histologic subtypes of chondrosarcoma are uncommon. Emc of the pleura has been described, however, no case of primary emc arising from lung has been previously reported.
There is an uncertain differentiation there is no evidence yet showing that emc exhibits the feature of cartilaginous. Arch pathol lab medvol 141, august 2017 soft tissue tumor immunohistochemistry updatewei et al 1073. Histopathologic differentiation between enchondroma and. Mesenchymal chondrosarcoma mcs was first described by lichtenstein and bernstein 1 in 1959. Mar 07, 2011 extraskeletal myxoid chondrosarcoma mghpict. Primary pulmonary myxoid sarcoma ppms is an extremely rare lung sarcoma that is characterized in most cases by recurrent balanced chromosomal translocation t2. Myxoid chondrosarcomas are rare, intermediate grade subtype of chondrosarcoma see chondrosarcoma grading. There is an uncertain differentiation there is no evidence yet showing that emc exhibits the feature of cartilaginous differentiation and neuroendocrine differentiation is even.
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