Intermediate grade osteosarcoma and chondrosarcoma arising in an osteochondroma. A case report of a patient with hereditary multiple exostoses

Bovée, Judith V.M.G.; Sakkers, R. J. B.; Geirnaerdt, Maartje J. A.; Taminiau, A. H. M.; Hogendoorn, Pancras C.W.

doi:10.1136/jcp.55.3.226

Cited by 42 publications

(25 citation statements)

References 16 publications

(19 reference statements)

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“…3,[7][8][9][10][11][12][13][14][15][16][17][18][19][20] It is sometimes difficult to rule out the possibility that another second malignancy is found in an osteochondroma. [36][37][38][39][40] In our series of 16 dedifferentiated peripheral chondrosarcomas, we show that the anaplastic component most often displays myo(fibro)blastic differentiation in its so-called dedifferentiated component, as could be determined from the musclespecific actin and smooth muscle actin staining. It is important to realize that the anaplastic component can also show desmin positivity, as we found in 2 of 16 cases, to avoid confusion with leiomyosarcoma in the diagnostic practice.…”

Section: Discussionmentioning

confidence: 98%

“…42,43 We studied the expression of growth plate signaling molecules in the PTHLH-BCL-2, FGF, and TFG-b signaling pathways, which have been extensively studied in other cartilaginous tumors. [34][35][36][44][45][46][47] In addition, the expression of matrix molecules was studied and the expression in the two components was compared. These signaling molecules have an important role in the growth and differentiation of the normal growth plate and could therefore be of importance in the cartilaginous tumor development and tumor progression.…”

Section: Discussionmentioning

confidence: 99%

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Dedifferentiated peripheral chondrosarcomas: regulation of EXT-downstream molecules and differentiation-related genes

et al. 2009

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Dedifferentiated peripheral chondrosarcoma is a rare subtype of chondrosarcoma arising superimposed on the cartilage cap of a preexisting osteochondroma. It consists of two clearly defined components, a low-grade malignant, well-differentiated cartilage component and a high-grade non-cartilaginous sarcoma. Signaling pathways having a role in normal cartilage development were analyzed in these tumors, and compared with available data of other cartilaginous tumors. Sixteen well-characterized dedifferentiated peripheral chondrosarcomas were immunohistochemically analyzed for parathyroid hormone-like hormone (PTHLH)-BCL-2, fibroblastic growth factor (FGF), and transforming growth factor-b signaling molecules, as well as matrix molecules and p53, comparing the chondrogenic component of dedifferentiated peripheral chondrosarcomas with the anaplastic component and with previously published data obtained from conventional grade I and II secondary peripheral chondrosarcomas. Results were correlated with clinical outcome. In the anaplastic component, various lines of differentiation could be found (collagen I (6/16), CD31 (1/16), smooth muscle actin (12/16), muscle-specific actin (12/16) and desmin (2/9)). Compared with the anaplastic component, the chondrogenic component of dedifferentiated peripheral chondrosarcomas shows more often expression of cyclin D1 (P ¼ 0.05), p53 (P ¼ 0.008), plasminogen activator inhibitor 1 (PAI-1) (P ¼ 0.005), and CD44 (P ¼ 0.030). Compared with secondary peripheral chondrosarcomas, more samples were positive in the chondrogenic component of dedifferentiated peripheral chondrosarcomas for FGF signaling (FGF receptor 3 P ¼ 0.000; bFGF P ¼ 0.003) and CD44 (P ¼ 0.000). Lower expression of BCL-2 (P ¼ 0.025) and absence of CD44v3 (P ¼ 0.000), a splice variant of CD44, was observed in the chondrogenic component of dedifferentiated peripheral chondrosarcomas compared with secondary peripheral chondrosarcomas. With regard to clinical data, PAI-1 expression in the chondrogenic component of dedifferentiated peripheral chondrosarcomas correlated with better survival (P ¼ 0.019). In conclusion, in the chondrogenic component of dedifferentiated peripheral chondrosarcomas, FGF signaling pathway is active, whereas PTHLH signaling seems to be low/downregulated. Interestingly, although the chondrogenic component of dedifferentiated peripheral chondrosarcoma is CD44 þ / CD44v3À, secondary peripheral chondrosarcomas is CD44À/CD44v3 þ , which suggest different splicing (preference). The prognostic value of PAI-1 in dedifferentiated peripheral chondrosarcomas might also be of interest for the more common dedifferentiated central chondrosarcomas.

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Section: Discussionmentioning

confidence: 98%

Section: Discussionmentioning

confidence: 99%

Dedifferentiated peripheral chondrosarcomas: regulation of EXT-downstream molecules and differentiation-related genes

et al. 2009

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“…In general, transformation leads to chondrosarcomas in 94% of cases, affecting a population younger than that of primary chondrosarcomas (Lamovec et al, 1999;Bovée et al, 2002). Malignant transformation is suspected mainly if there is recent onset of pain without any adequate alternative explanation or continued growth of an OC after skeletal maturity.…”

Section: Introductionmentioning

confidence: 99%

Case Report Osteosarcoma arising from osteochondroma of the tibia: case report and cytogenetic findings

Engel¹,

Nogueira-Barbosa²,

Brassesco³

et al. 2012

Genet. Mol. Res.

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ABSTRACT. Osteochondroma is a cartilage capped benign tumor developing mainly at the juxta-epiphyseal region of long bones. The rate of malignant transformation, mainly into chondrosarcoma, is estimated to be less than 1-3%. Transformation into osteosarcoma is very rare and has been reported only thirteen times. There is little information on 449 ©FUNPEC-RP www.funpecrp.com.br Genetics and Molecular Research 11 (1): 448-454 (2012) Osteosarcoma arising from osteochondroma treatment and outcome. We report the case of a secondary osteosarcoma arising in the left tibia of a 23-year-old male, 10 years after the initial diagnosis of osteochondroma and after two partial resections. Malignant transformation occurred at the stalk and not at the cartilage cap, as would normally be expected. Chromosome banding analysis revealed the karyotype: 46,XY, t(3;13)(q21;q34) [2]/46,XY [18]. Records from additional cases will help determine the parameters that define these rare secondary bone lesions.

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“…Other cases develop from the spongy bone of the stalk and have no association with the cartilaginous cap. In this type of secondary osteosarcoma, no thickening of the cap is observed and a neoplastic cartilaginous component is not present (22,26,27).…”

Section: A B C D Discussionmentioning

confidence: 99%

Secondary osteosarcoma arising from osteochondroma following autologous stem cell transplantation with total-body irradiation for neuroblastoma: A case report

et al. 2015

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Abstract. The present study reports the first case of malignant transformation to osteosarcoma arising from osteochondroma following childhood total-body irradiation (TBI). The association between TBI and later development of osteochondroma is well-known; however, malignant degeneration arising from radiation-induced osteochondroma is rare. The current study describes the case of a 17-year-old boy with osteosarcoma arising from osteochondroma of the left distal humerus, which developed following TBI. TBI was administered as part of a conditioning regimen received prior to autologous peripheral hematopoietic stem cell transplantation (HSCT) at the age of 6 years, following an initial diagnosis of neuroblastoma at the age of 5 years. The patient subsequently underwent preoperative chemotherapy followed by wide local excision and reconstruction with an extracorporeally irradiated autograft. Postoperative chemotherapy was administered, and the patient demonstrated no clinical or radiographic evidence of recurrence after 40 months of follow-up. To the best of our knowledge, this is only the second reported case of malignant degeneration of osteochondroma following childhood TBI, and the first reported case of transformation to osteosarcoma. The current case highlights the importance of close observation for secondary malignancies in this patient population.

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Intermediate grade osteosarcoma and chondrosarcoma arising in an osteochondroma. A case report of a patient with hereditary multiple exostoses

Cited by 42 publications

References 16 publications

Dedifferentiated peripheral chondrosarcomas: regulation of EXT-downstream molecules and differentiation-related genes

Dedifferentiated peripheral chondrosarcomas: regulation of EXT-downstream molecules and differentiation-related genes

Case Report Osteosarcoma arising from osteochondroma of the tibia: case report and cytogenetic findings

Secondary osteosarcoma arising from osteochondroma following autologous stem cell transplantation with total-body irradiation for neuroblastoma: A case report

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