Diagnosis, Treatment and Prognosis of Low Grade Central Osteosarcoma

Won Seok Song; Wan Hyeong Cho; Kwang-Youl Lee; Chang-Bae Kong; Jae-Soo Koh; Dae-Geun Jeon; Soo-Yong Lee

doi:10.5292/jkbjts.2014.20.2.47

Journal List > J Korean Bone Joint Tumor Soc > v.20(2) > 1052048

Go to TopGo to Top Go to BottomGo to Bottom

TOOLS

Song, Cho, Lee, Kong, Koh, Jeon, and Lee: Diagnosis, Treatment and Prognosis of Low Grade Central Osteosarcoma

Original Article

J Korean Bone Joint Tumor Soc 2014;20(2):47-53.

Published online: 10 January 2014

DOI: https://doi.org/10.5292/jkbjts.2014.20.2.47

Diagnosis, Treatment and Prognosis of Low Grade Central Osteosarcoma

Won Seok Song, Wan Hyeong Cho, Kwang-Youl Lee, Chang-Bae Kong, Jae-Soo Koh^*, Dae-Geun Jeon, Soo-Yong Lee

^*Pathology, Korea Cancer Center Hospital, Seoul, Korea

Departments of Orthopedic Surgery, Korea Cancer Center Hospital, Seoul, Korea

Correspondence to: Dae-Geun Jeon Department of Orthopedic Surgery, Korea Cancer Center Hospital, 215-4, Gongneung-dong, Nowon-gu, Seoul 139-706, Korea TEL: +82-2-970-1242 FAX: +82-2-970-2427 E-mail: dgjeon@kcch.re.kr

Received 16 September 2014 Revised 26 November 2014 Accepted 28 November 2014

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Purpose

We analyzed the diagnosis and the treatment outcomes of patients with central low grade osteosarcoma.

Materials and Methods

We retrospectively reviewed 16 patients with central low grade osteosarcoma were treated at out institution between 1994 and 2011.

Results

There were 4 men and 12 women with mean age of 26 years. Eleven patients were correctly diagnosed but 5 patients were misdiagnosed as osteoid osteoma, non ossifying fibroma, aneurysmal bone cyst, desmoplastic fibroma. 15 patients finally received wide margin en bloc excision and one of them treated under neoadjuvant chemotherapy. Final survival status was continuous disease free in 14 and 1 patient died of renal cell cancer. Remaining 1 with multifocal lesions is alive with disease for 7 years only treated radiation therapy on residual tumors. Nine (56%) of 16 tumors showed extraosseous extension of tumor (56%) and 1 of them showed extra-compartmental tumors.

Conclusion

The diagnosis of central low grade osteosarcoma is challenging, however, considering of the clinical suspicion, the typical findings of radiologic and pathologic features, proper diagnosis is needed. This tumor should be treated with wide excision, even after an intralesional excision, to avoid local recurrence or transformation to higher histologic grade.

Go to :

Keywords: central low grade osteosarcoma, diagnosis, treatment

References

1. Schwab JH, Antonescu CR, Athanasian EA, Boland PJ, Healey JH, Morris CD. A comparison of intramedullary and juxtacortical low-grade osteogenic sarcoma. Clin Orthop Relat Res. 2008; 466:1318–22.

2. Kurt AM, Unni KK, McLeod RA, Pritchard DJ. Low-grade intraosseous osteosarcoma. Cancer. 1990; 65:1418–28.

3. Choong PF, Pritchard DJ, Rock MG, Sim FH, McLeod RA, Unni KK. Low grade central osteogenic sarcoma. A longterm followup of 20 patients. Clin Orthop Relat Res. 1996; 322:198–206.

4. Franceschina MJ, Hankin RC, Irwin RB. Low-grade central osteosarcoma resembling fibrous dysplasia. A report of two cases. Am J Orthop (Belle Mead NJ). 1997; 26:432–40.

5. Kenan S, Ginat DT, Steiner GC. Dedifferentiated high-grade osteosarcoma originating from low-grade central osteosarcoma of the fibula. Skeletal Radiol. 2007; 36:347–51.

6. Muramatsu K, Hashimoto T, Seto S, Gondo T, Ihara K, Taguchi T. Low-grade central osteosarcoma mimicking fibrous dysplasia: a report of two cases. Arch Orthop Trauma Surg. 2008; 128:11–5.

7. Andresen KJ, Sundaram M, Unni KK, Sim FH. Imaging features of low-grade central osteosarcoma of the long bones and pelvis. Skeletal Radiol. 2004; 33:373–9.

8. Bertoni F, Bacchini P, Fabbri N, et al. Osteosarcoma. Low-grade intraosseous-type osteosarcoma, histologically resembling parosteal osteosarcoma, fibrous dysplasia, and desmoplastic fibroma. Cancer. 1993; 71:338–45.

9. Kumar A, Varshney MK, Khan SA, Rastogi S, Safaya R. Low grade central osteosarcoma–a diagnostic dilemma. Joint Bone Spine. 2008; 75:613–5.

10. Hayashi K, Tsuchiya H, Yamamoto N, et al. Diagnosis and treatment of low-grade osteosarcoma: experience with nine cases. Int J Clin Oncol. 2014; 19:731–8.

11. Malhas AM, Sumathi VP, James SL, et al. Low-grade central osteosarcoma: a difficult condition to diagnose. Sarcoma. 2012; 2012:764796.

12. Yoshida A, Ushiku T, Motoi T, et al. Immunohistochemical analysis of MDM2 and CDK4 distinguishes low-grade osteosarcoma from benign mimics. Mod Pathol. 2010; 23:1279–88.

13. Antonescu CR, Huvos AG. Low-grade osteogenic sarcoma arising in medullary and surface osseous locations. Am J Clin Pathol. 2000; 114(Suppl):S90–103.

14. Gisselsson D, Pålsson E, Höglund M, et al. Differentially amplified chromosome 12 sequences in low- and high-grade osteosarcoma. Genes Chromosomes Cancer. 2002; 33:133–40.

15. Tarkkanen M, Böhling T, Gamberi G, et al. Comparative genomic hybridization of low-grade central osteosarcoma. Mod Pathol. 1998; 11:421–6.

16. Song WS, Jeon DG, Kong CB, Cho WH, Lee SY. Outcome of re-excision for intralesionally treated parosteal osteosarcoma. J Surg Oncol. 2011; 103:264–8.

Go to :

Figure 1.

Plain radiograph of a 21-year-old man showing expansile mixed osteolytic and sclerotic lesion in the distal meta-diaphysis of femur. Radiologic differential diagnosis included osteosarcoma, fibrosarcoma, adamantinoma, and giant osteoblastoma.

undefined

Figure 3.

Plain radiograph of a 20-year-old man showing predominantly lytic lesion in the distal femur. Radiologic differential diagnosis included osteosarcoma, fibrosarcoma, chondrosarcoma, and giant cell tumor with aneurismal bone cystic change.

undefined

Figure 2.

(A) Plain radiograph of a 19-year-old girl showing mixed osteolytic and sclerotic lesion in the proximal humerus. (B) The typical histologic appearance of low-grade osteosarcoma, permeation of osseous trabeculae by spindle cell stroma (hematoxylin-eosin, ×100).

undefined

Figure 4.

(A) Plain radiograph of a 54-year-old woman showing sclerotic lesion in the distal femur. (B) Mature bone trabeculae is admixed with permeating spindle cell stroma (×100).

undefined

Table 1.

Patient Characteristics and Radiologic Feature

Case number	Age/ Gender	Previous diagnosis	Previous treatment	Location	Radiologic Pattern	Tumor size (cm)/ Volume (cc) E	xtra-osseous extension
1	7/F	–	–	Proximal femur	Lytic, coarse trabeculation	3×2.1×1.6/5.3	–
2	11/F	–	–	Proximal humerus	Densely sclerotic	3.2×1.7×1.6/4.6	–
3	19/F	–	–	Proximal humerus	Mixed lytic and sclerotic	6.4×4.2×4/56.3	+
4	19/F	–	–	Distal radius	Densely sclerotic	7.5×2.9×2.5/28.5	+
5	20/F	–	–	Pelvis, pubis	Lytic, coarse trabeculation	8×5×3/62.8	+
6	20/M	–	–	Distal femur	Predominantly lytic	8.6×5.6×3.9/98.3	+
7	21/M	–	–	Distal femur	Mixed lytic and sclerotic	9.5×6.8×5.9/199.6	+
8	24/F	–	–	Proximal humerus	Densely sclerotic	8.7×6×4.9/140	+
9	32/M	–	–	Proximal tibia	Mixed lytic and sclerotic	7×6×5/110	–
10	36/M	–	–	Pelvis, ilium	Densely sclerotic	4.6×4.5×2.4/26	–
11	38/F	–	–	Scapular	Densely sclerotic	7.8×6.5×4.5/119.5	+
12	39/F	–	–	Distal fibular	Mixed lytic and sclerotic	17×3.5×2.5/77.9	+
13	54/F	–	–	Distal femur	Densely sclerotic	6.5×4×2.8/38.1	–
14	16/F	Low-grade sarcoma	Radiofrequency Ablation	Distal femur	Lytic, coarse trabeculation	10.3×3.6×3.2/62.1	–
15	20/F	NOF	Curettage	Calcaneus	Mixed lytic and sclerotic	6.3×4.5×3.4/50.5	+
16	38/F	ABC on biopsy*	Curettage	Proximal tibia	Predominantly lytic	7×6×5/110	–

ABC, aneurismal bone cyst; *Hemangioendothelioma on curettage specimen; NOF, nonossifying fibroma.

Table 2.

Surgical Procedure, Immune-Histochemical Analysis, and Clinical Course

Case number	Chemotherapy	Definitive treatment	Surgical margin	Local recurrence	Metastasis	Followup (months)	Final statu
1	–	Intercalary resection, overlapping allograft	Wide	–	–	56	CDF
2	–	Intercalary resection, overlapping allograft	Wide	–	–	57	CDF
3	–	Allograft prosthesis composite	Wide	–	–	35	CDF
4	–	Excision, fibula graft	Wide	–	–	171	CDF
5	–	Internal pelvectomy	Marginal	–	–	141	CDF
6	–	Tumor prosthesis	Wide	–	–	74	CDF
7	+	Tumor prosthesis	Wide	–	–	44	CDF
8	–	Tumor prosthesis	Wide	–	–	45	CDF
9	–	Tumor prosthesis	Wide	–	–	107	CDF
10	–	Internal pelvectomy	Marginal	–	–	21	DOC
11	–	Scapulectomy	Wide	–	–	122	CDF
12	–	Resection, overlapping allograft	Wide	–	–	46	CDF
13	–	Tumor prosthesis	Wide	–	–	36	CDF
14	–	Radiotherapy	–	+	–	142	AWD
15	–	Cement spacer	Marginal	–	–	46	CDF
16	–	Pasteurized bone-prosthesis composite	Wide	–	–	162	CDF

CDF, continous disease free; AWD, alive with disease; DOC, died of other cause (renal cell carcinoma).

Table 3.

Comparison with Previous Studies

Author	Number of patients	Definitive treatment	Intralesional operation* (%)	Chemotherpy	LR^†	Metastasis	Final status
Current study	16	13/3	3/16 (19%)	1/16 (6%)	0%	0%	NED (15), DOC (1), AWD (1)
Choong et al⁸⁾	20	12/8	12/20 (60%)	–	14%	20%	NED (16), DOD (4)
Malhas et al¹²⁾	18	11/7	10/18 (56%)	7/18 (39%)	11%	11%	NED (16), AWD (1), DOD (1)
Yoshida et al¹⁴⁾	9	3/6	3/9 (33%)	5/9 (56%)	14%	43%	NED (4), AWD (2), DOD (1)
Dujardin et al¹⁸⁾	6	NA	NA	NA	NA	NA	NA
Hayashi et al¹³⁾	5	NA	2/5 (40%)	NA	0%	0%	NED (5)

^* Either at primary or referral center

^† LR, local recurrence after definitive treatment; NED, no evidence of disease; DOC, died of other cause; AWD alive with disease; DOD, died of disease; NA, data not available.

TOOLS

Similar articles