Journal of Cancer Research and Therapeutics

: 2015  |  Volume : 11  |  Issue : 4  |  Page : 989--992

Giant cell rich osteosarcoma of the cuneiforms

Sandeep Vijayan1, Monappa A Naik1, Shamsi Abdul Hameed2, Sharath K Rao1,  
1 Department of Orthopedics, Kasturba Medical College, Manipal, Manipal University, Karnataka, India
2 Department of Orthopedics, Hamad Medical Corporation, Doha, Qatar

Correspondence Address:
Sandeep Vijayan
Department of Orthopedics, Kasturba Medical College, Manipal, Manipal University, Karnataka - 576 104


Osteosarcoma is the commonest primary malignant bone tumor in children and adolescents. Giant cell rich osteosarcoma is a rare subtype of conventional osteosarcoma. Osteosarcomas commonly involve the metaphysis and meta-diaphysis of long bones. We report a 19-year-old girl with giant cell rich osteosarcoma of the medial and intermediate cuneiform bones. Even though, giant cell rich osteosarcoma is frequently mistaken for osteoclastoma of the bone; age of onset, location of lesion, radiological features, and histological characteristics on a high power field helps to differentiate the two conditions. Appropriate and early diagnosis of this variant possibly averts severe morbidity and mortality to the patient. Nonmetastatic osteosarcomas in the foot have better prognosis and are amenable to limb salvage surgeries.

How to cite this article:
Vijayan S, Naik MA, Hameed SA, Rao SK. Giant cell rich osteosarcoma of the cuneiforms.J Can Res Ther 2015;11:989-992

How to cite this URL:
Vijayan S, Naik MA, Hameed SA, Rao SK. Giant cell rich osteosarcoma of the cuneiforms. J Can Res Ther [serial online] 2015 [cited 2020 May 25 ];11:989-992
Available from:

Full Text


Giant cell rich osteosarcoma, a rare subtype of conventional osteosarcoma is characterized by abundance of osteoclast-like giant cells widely dispersed throughout the tumor, which on low power shows multinucleated giant cells simulating a giant cell tumor (osteoclastoma), but on high power shows the atypical anaplastic stromal cells and malignant osteoid production. [1],[2],[3] To the best of our knowledge, this is the first case report of a giant cell rich osteosarcoma involving the tarsal bones.


A 19-year-old girl presented with progressive swelling of insidious onset on the left foot since 2 years and pain since 4 months, without preceding trauma or constitutional symptoms. A tender, firm to hard swelling with ill-defined margins was noted in the mid-foot. The ankle and subtalar movements were normal and there was no inguinal lymphadenopathy. Radiographs revealed nonhomogeneous, lytic, ballooned out lesion in the medial and intermediate cuneiforms with trabecular destruction, internal septations, and ill-defined sclerotic rim [Figure 1]. Computed tomography (CT) showed an expansile lytic lesion of the medial and intermediate cuneiforms with destruction of the posterior cortex without intralesional calcification [Figure 2]. Magnetic resonance imaging (MRI) showed the lesion abutting the navicular bone and tibialis posterior tendon on the plantar aspect [Figure 3]. Blood investigations and CT thorax and abdomen were normal. Technetium-99 bone scan showed increased uptake only in the left mid-foot. After failed fine-needle aspiration cytology (FNAC), an incisional biopsy was done which showed osteoclast type giant cells distributed uniformly amongst plump stromal cells with areas of hyalinization, fibrosis, and foci of hemorrhage [Figure 4]. Differential diagnosis of giant cell reparative granuloma and giant cell tumor was given by the pathologist.{Figure 1}{Figure 2}{Figure 3}{Figure 4}

She underwent extended curettage and iliac crest bone grafting. Histopathology of excised tumor showed a neoplasm composed of osteoclast-type giant cells admixed with plump stromal cells exhibiting nuclear atypia and forming lacy osteoid, osteoid seams and osteoid trabeculae with mitosis, and foci of vague cartilaginous differentiation [Figure 5]; and the diagnosis of giant cell rich osteosarcoma was made.{Figure 5}

After detailed discussion with the oncologist, patient, and her relatives; it was decided to do limb salvage surgery with neoadjuvant chemotherapy. Six cycles of chemotherapy with doxorubicin and cisplatin was given. After three cycles of chemotherapy, she underwent excision of the naviculum and three cuneiforms and interposition fibular strut grafting [Figure 6], followed by three more cycles of chemotherapy. She was asymptomatic without evidence of local recurrence or distant metastasis at last follow-up, that is, 3 years after the surgery [Figure 7].{Figure 6}{Figure 7}


Giant cell rich osteosarcoma is a rare subtype of conventional osteosarcoma. [1],[2],[3],[4] It is characterized by proliferation of atypical stromal cells with an abundance of osteoclast-like giant cells distributed throughout the tumor. The lesion can be easily mistaken for osteoclastoma.

Osteosacroma, generally involves the metaphysis or metadiaphysis of long bones. [1] The incidence of osteosarcoma in foot is less than 1%. [1],[5],[6],[7] Most reported cases of osteosarcoma in foot (75%) were in calcaneum and metatarsals. [5],[6],[7],[8] Osteosarcoma of the medial cuneiform has been reported only once. [6] However, giant cell rich osteosarcoma in the cuneiform bones has never been reported.

The peak age of osteosarcoma is in the 2 nd decade, whereas, most conventional osteoclastoma occur in 20-50 years. [1],[3],[8] Malignant transformation of a conventional osteoclastoma takes many years without prior history of local radiation. [3] A tumor that appears as an osteoclastoma histologically but arise in a skeletally immature person; should be regarded with great suspicion and sampled widely to rule out osteosarcoma. [9] Osteosarcomas of the foot were noted almost exclusively in adults. [5],[7],[8] Giant cell rich osteosarcoma of the foot can occur in the adolescent age group like present case.

Giant cell rich osteosarcoma demonstrates characteristic features which are helpful in diagnosis. When a giant cell rich osteosarcoma occurs in a common location for osteoclastoma, histology becomes the most important factor in differentiating the two [Table 1]. [1],[2],[3],[4],[9],[10] Around 13-25% of conventional osteosarcomas may show benign osteoclast-like giant cells which are usually arranged in clusters. [1],[9] Hence, it is very important that the specimen be sampled widely to confirm the diagnosis of giant cell rich osteosarcoma. FNAC or Tru-Cut biopsy may be insufficient to give the diagnosis. The diagnosis of giant cell rich osteosarcoma should be made only after ruling out conventional osteosarcoma with giant cells, telangiectatic osteosarcoma with solid areas, and other giant cell rich lesions [Table 2]. [1],[2],[3],[4],[10]{Table 1}{Table 2}

Osteosarcoma in general is a highly malignant and rapidly metastasizing tumor. Osteosarcoma in foot is usually low-grade and survival rate for these patients are higher than those with other locations. [5],[7],[8] The disease was low-grade malignant in present patient too as she was symptomatic for 2 years before the diagnosis without evidence of distant metastasis.

In the past, osteosarcoma of the foot was considered not amenable to limb sparing surgery because of the close anatomic confines and poor compartmentalization of the tumor. [6] Limb ablating surgery was advocated. [6] However, in recent years, with accurate imaging, a good response to preoperative chemotherapy and improvements in limb salvage techniques, limb preservation in treatment of nonmetastatic osteosarcoma has been accepted as an important therapeutic strategy. [5],[7] Limb salvage and ablative surgeries have their own pros and cons [Table 3]. [5],[7] The low-grade, slow-growing nature of the tumor for 2 years without evidence of metastasis, intracompartmental location, favorable response to neoadjuvant chemotherapy, feasibility of getting a wide margin during resection, and reports of better functional and psychological outcomes after limb salvage were main reasons for opting for limb salvage surgery in our patient. With disease-free, 3-year survival of patient and good clinical outcome, we suggest limb salvage surgery in nonmetastatic giant cell rich osteosarcoma of the foot. Even though, vascularized fibular or iliac crest graft might be biomechanically superior, the procedure needs expertise and longer operating time and is associated with more complications. Moreover, unlike in long bone defects, here the length of the graft needed was small. Also, as we used two pieces of graft to reconstruct the defect, we feel harvesting two small segments with its vascular pedicle would have been difficult. In comparison, nonvascularized fibular grafting is technically simpler and will incorporate with recipient bone if the gap is small as in our case. We felt that K wires could give the necessary stability and use of plates to stabilize the grafts might interfere with soft tissue coverage and wound closure necessitating a flap surgery.{Table 3}


Age at onset, location of the tumor, and atypical stroma with osteoid production in histology helps to diagnose giant cell rich osteosarcoma correctly. It occurs in adolescents and can present with localized swelling. Wider fields should be viewed to correctly diagnose it due to the presence of clusters of giant cells in small percentage of conventional osteosarcoma. As being low-grade with good prognosis, local resection in nonmetastatic cases can yield better survival.


The authors would like to express their sincere gratitude to Prof. NJ Mani, Senior Orthopedic Consultant, Baby Memorial Hospital, Kozhikode for timely referral of the patient to our institute. Authors wish to thank Dr. Suman CPS, former Senior Resident of our department for his inputs and Dr. Hitesh Shah, Associate Professor of Orthopedics, Kasturba Medical College, Manipal for copy editing the manuscript.


1Gambarotti M, Donato M, Alberghini M, Vanel D. A strange giant cell tumor. Eur J Radiol 2011;77:3-5.
2Sato K, Yamamura S, Iwata H, Sugiura H, Nakashima N, Nagasaka T. Giant cell-rich osteosarcoma: A case report. Nagoya J Med Sci 1996;59:151-7.
3Shinozaki T, Fukuda T, Watanabe H, Takagishi K. Giant cell rich osteosarcoma simulating giant cell tumour of bone. Kita Kanto Med J 2004;54:147-51.
4Bathurst N, Sanerkin N, Watt L. Osteoclast rich osteosarcoma. Br J Radiol 1986;59:667-73.
5Wang CW, Chen CY, Yang RS. Talar osteosarcoma treated with limb-sparing surgery: A case report. J Bone Joint Surg Am 2011;93:e22.
6Choong PF, Qureshi AA, Sim FH, Unni KK. Osteosarcoma of the foot: A review of 52 patients at the Mayo clinic. Acta Orthop Scand 1999;70:361-4.
7Katagiri H, Takahashi M, Takagi T, Nakagawa M. Osteosarcoma of the talus treated successfully with limb-sparing surgery. A case report. J Bone Joint Surg Am 2008;90:869-74.
8Biscaglia R, Gasbarrini A, Bohling T, Bacchini P, Bertoni F, Picci P. Osteosarcoma of the bones of the foot--an easily misdiagnosed malignant tumour. Mayo Clin Proc 1998;73:842-7.
9Klein MJ, Siegal GP. Osteosarcoma: Anatomic and histologic variants. Am J Clin Pathol 2006;125:555-81.
10Imran AA, Khaleel ME, Salaria SM, Hasan M. Giant cell rich osteosarcoma: Unravelling an elusive, enigmatic entity. Int J Pathol 2012;10:36-8.