|
|
| Year : 2009 | Volume
: 52
| Issue : 1 | Page : 80-82 |
|
| Primary osteoliposarcoma of the bone |
|
|
Michelle De Padua1, T.P.S Bhandari2, Jairamchander Pingle3
1 Department of Orthopedics, Apollo Hospitals, Hyderabad, India
2 Department of Pathology, Apollo Hospitals, Hyderabad, India
3 Department of Oncology, Apollo Hospitals, Hyderabad, India
Click here for correspondence address and email
|
|
 |
|
 Abstract | | |
Osteoliposarcoma are rare tumors of the bone. To our knowledge, so far only eight cases have been reported. Some of the reports have referred to these tumors as malignant mesenchymoma. We report a case of a male with osteoliposarcoma of the right femur who presented with pain and restricted movements of the right knee joint. Radiologically, a tumor in the lower end of the right femur was seen extending into the soft tissue. The biopsy was reported as osteosarcoma. The patient received three cycles of neo-adjuvant chemotherapy followed by limb-salvage surgery with provisions for a custom-made prosthesis. A histopathological study of the excision specimen revealed areas of pleomorphic liposarcoma with numerous osteoblasts associated with areas of osteoid surrounded by neoplastic cells. The final diagnosis was osteoliposarcoma. Only 21% tumor necrosis (effects of chemotherapy) was observed. Presently, 26 months following diagnosis, the patient is fine with no evidence of local recurrence or distant metastasis. Keywords: Osteoliposarcoma, malignant, mesenchymoma, bone, neo-adjuvant chemotherapy
How to cite this article:
Padua MD, Bhandari T, Pingle J. Primary osteoliposarcoma of the bone. Indian J Pathol Microbiol 2009;52:80-2 |
| Introduction | |  |
Primary lipomatous tumors of the bone include primary liposarcoma and primary osteoliposarcoma, both of which are rare tumors. Schajowicz, et al. [1] were the first to report a primary osteoliposarcoma of the bone in 1966, which they referred to as a malignant mesenchymoma. Subsequently, to our knowledge, only seven more cases have been reported. [2],[3],[4],[5],[6],[7],[8] We report an additional case of primary osteoliposarcoma of the bone.
| Case Report | | |
A 21-year-old male presented with pain and swelling of the right knee joint. There was restricted movement in the joint and he was unable to flex the joint. Magnetic resonance imaging (MRI) revealed heterogenous signals in the lower end of the right femur extending into both condyles and the posterior aspect of femur, into adjacent soft tissue and popliteal fosa [Figure 1] . The size of the tumor was 10.2x9.3 cms. A bone scan revealed increased uptake in the region. A chest X-ray and an ultrasound of the abdomen were normal. An incisional biopsy was performed, which was reported as osteosarcoma. The patient underwent three cycles of neo-adjuvant chemotherapy comprising of adriamycin (30 mg, Day 1-2), iposphamide (2 gm, Day 1-2) and cisplatin (40 mg, Day 1-3) over a period of 50 days. Seventeen days after the last dose of chemotherapy, he underwent limb salvage surgery- excision of the lower end of the femur with provisions for a custom-made prosthesis followed by 3 cycles of the same adjuvant chemotherapy.
Pathologic findings
The lower end of the femur with the surrounding soft tissue measured 16x9x8 cms. Sectioning revealed an ill-defined tumor with a few yellowish areas measuring 9x7 cms occupying the lower end of the femur [Figure 2]. Distance from the proximal resection margin was 6 cms. The tumor was seen breaking through the cortex and extending into soft tissue. The specimen was grossed as per protocol for handling post neo-adjuvant therapy for osteosarcomas. [9] A thin slice of the whole tumor was taken with the bone and decalcified in 10% formic acid. The entire specimen was then embedded in blocks that were numbered so that the whole specimen could be reconstructed to calculate the percentage of tumor necrosis following neo-adjuvant chemotherapy.
On microscopy, the neoplastic cells were large, polygonal with pleomorphic vesicular nuclei and abundant vacuolated cytoplasm. Numerous typical lipoblasts with scallopped nuclei were present [Figure 3]. Mitotic figures were easily noted. Also seen were foci of osteoid formation by tumor cells with hyperchromatic nuclei and scanty eoisinophilic cytoplasm-osteosarcomatous areas [Figure 4]. An oil red O stain for fat showed strong cytoplasmic positivity [Figure 5]. The effects of chemotherapy in the form of necrosis, granulation tissue and hyalinization was low, about 21%. Presently, 26 months following surgery, the patient is disease free and well with no evidence of local recurrence or distant metastasis.
| Discussion | | |
In 1966, Schajowicz, et al. [1] first described a primary tibial bone tumor in a 17-year-old boy with liposarcomatous and osteosarcomatous components, which he referred to as malignant mesenchymoma. Subsequently Ross, et al. [2] described a similar tumor in a 15-year-old boy, which he referred to as osteoliposarcoma, indicating the lines of differentiation in the tumor. Subsequently seven more cases have been reported, [3],[4],[5],[6],[7],[8] referred to as malignant mesenchymoma or osteoliposarcoma. The nomenclature osteoliposarcoma is more specific and differentiates it from other primary bone mixed tumors of mesechymal origin that have been described, which are also referred to as malignant mesenchymoma that include osteosarcoma and rhabdomyosarcoma, osteosarcoma with rhabdomyosarcoma and chondrosarcoma. [10] Two criteria need to be met for a diagnosis of primary osteoliposarcoma. First, the tumor must arise from within the bone, that it must not be a metastatic deposit. Second, tumor cells with the typical appearance of lipoblasts are present. [2] Our case fulfilled the criteria and showed liposarcomatous areas with lipoblasts and areas with osteoid formation indicating osteosarcomatous areas.
The radiological features were that of an intraosseous tumor involving the entire width of the shaft breaking through the cortex and extending into soft tissue. The diagnosis was arrived at only after an examination of the resection specimen while the biopsy was diagnosed as osteosarcoma. Scwajowicz, et al., [1] Ross, et al., [2] and Riejnierse, et al., [8] also had similar experiences where initial biopsies were diagnosed as liposarcoma due to problems with sampling. All the cases reported so far have been in the lower extremities; two cases were in the pelvis. All were destructive lesions with ill defined margins, breakdown of cortex and extension into soft tissue. The median age of most of the cases reported is 15 years old, except in the report of Cremer, et al. [4] where the ages of the patients were 37 and 58 years.
A unique feature of our case is the therapy given. The patient was treated as a case of osteosarcoma after initial biopsy with neo-adjuvant chemotherapy and limb salvage excision with prosthesis followed by chemotherapy. The effects of chemotherapy amounted to about 21%. None of the earlier cases have been treated this way. The prognosis was universally grim in previously described cases despite operative removal with chemotherapy, radiotherapy, or both. In all patients with follow-up, metastasis occurred within a median of 7 months and death occurred within 9-36 months after diagnosis.
Our patient is well and disease-free after 26 months of follow-up. A longer follow-up is essential before a conclusion about the effectiveness of neo-adjuvant chemotherapy can be made. To conclude, primary osteoliposarcoma of the bone is a rare tumor occurring mainly around the knee. Our case is the first case that has been treated with neo-adjuvant chemotherapy and limb salvage surgery.
| References | | |
| 1. | Schajowicz F, Cuevillas AR, Silberman FS. Primary malignant mesenchymoma of bone: A new tumor entity. Cancer 1966;19:1423-8.  [PUBMED] |
| 2. | Ross CF, Hadfield G. Primary osteo-liposarcoma of bone (malignant mesenchymoma): Report of a case. J Bone Joint Surg Br 1968;50:639-43. [PUBMED] [FULLTEXT] |
| 3. | Bertoni F, Laus M. Primary malignant mesenchymoma of bone (case report). Ital J Orthop Traumatol 1978;4:105-8. [PUBMED] |
| 4. | Cremer H, Koischwitz D, Tismer R. Primary osteoliposarcoma of bone. J Cancer Res Clin Oncol 1981;101:203-11. [PUBMED] |
| 5. | Downey EF Jr, Worsham GF, Brower AC. Liposarcoma of bone with osteosarcomatous foci: Case report and review of the literature. Skeletal Radiol 1982;8:47-50. [PUBMED] |
| 6. | Bosman C, Boldrini R, Guzzanti V. Primary osteoliposarcoma of bone: First observation in the pediatric age group. Appl Pathol 1988;6:56-60. [PUBMED] |
| 7. | Marcial-Seoane RA, Marcial-Seoane MA, Davila-Toro FJ, Marcial-Rojas RA. Bone tumors of rhabdomyosarcoma mixed origin: Osteo-liposarcoma and osteo-rhabdomyosarcoma. Bol Asoc Med P R 1990;82:378-93. |
| 8. | Reijnierse M, Kroon HM, Van Der Heul RO, Mulder JD. Mesenchymoma of bone. J Bone Joint Surg Am 1993;75:112-5. [PUBMED] |
| 9. | Raymond AK, Ayala AG. Specimen management after chemotherapy. Contemp Issues Surg Pathol 1988;11:157-81. |
| 10. | Van Dorpe J, Sciot R, Samson I, De Vos R, Brys P, Van Damme B. Primary osteorhabdomyosarcoma (malignant mesenchymoma) of bone: A case report and review of the literature. Mod Pathol 1997;10:1047-53. [PUBMED] |
Correspondence Address:
Michelle De Padua
Department of Pathology, Apollo Hospitals, Jubilee hills, Hyderabad
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0377-4929.44973
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5] |
|
| This article has been cited by | | 1 |
Parosteal osteosarcoma associated with a low-grade component mimicking well-differentiated liposarcoma: a case report |
|
| P. Sohier, M. Rodrigues, P. Anract, A. Feydy, F. Larousserie | | Skeletal Radiology. 2021; 50(1): 243 | | [Pubmed] | [DOI] | | | 2 |
Composite sarcoma of bone with focal rhabdomyosarcoma and lymph node metastasis in an adolescent |
|
| Maitane Andión Catalán, Susana Buendía López, Natalia Camarena Pavón, Alba Rubio San Simón, Teresa Cañas Maciá, Daniel Azorín Cuadrillero | | Pediatric Blood & Cancer. 2020; 67(11) | | [Pubmed] | [DOI] | | | 3 |
Critical reappraisal of primary osseous composite sarcoma (malignant mesenchymoma) - analysis of four cases and literature review |
|
| Louis T. C. Chow | | APMIS. 2016; 124(6): 487 | | [Pubmed] | [DOI] | |
|
|
|
|
|
|
|
|
|
|
|
|
| Article Access Statistics | | | Viewed | 3688 | | | Printed | 183 | | | Emailed | 2 | | | PDF Downloaded | 183 | | | Comments | [Add] | | | Cited by others | 3 | | |
|
|
