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Chondroblastoma of Diaphysis of Tibia; Case Report

Heidarali Esmaeili, Haniyeh Amidfar*, Mehrdad Yavarikia, Atiyeh Amidfar

Assissstant of Pathology, Iran

Abstract: Background: Chondroblastoma is a rare benign cartilaginous neoplasm that considered for approximately 1% of all bone tumors and characteristically emerges in the epiphysis of a long bone, especially the hummeros, femurand tibia.Only rare cases have been reported in the diaphyseal region. Case Report: We report a case of diaphyseal one in a 13 years old girl that admitted ith brief pain and deformities in left leg. Radiographic examinations disclosed a multiloculated osteolytic lesion in left tibia. Conclusion: Histologicak findings revealed proliferation of polygonal cells in the cartigenous background with few Giant cells.

Keywords: Chondroblastoma, Diaphysis, chondroblasts

Citation: Heidarali Esmaeili, Haniyeh Amidfar, Mehrdad Yavarikia, Atiyeh Amidfar, Chondroblastoma of Diaphysis of Tibia; Case Report. American Journal of Medical Case Reports, 4 (6). 215-217. January 2016. doi:10.12691/ajmcr-4-6-8

1. Background

Chondroblastoma is a rare benign cartilaginous neoplasm that accountsfor approximately 1% of all bone tumors and characteristicallyarises in the epiphysis of a long bone, particularly the hummeros, femurand tibia. [1]

The tumor has a higher incidence in males than females [2] and occurs predominantly in males under 20 years [3]. The tumor presents in a child, teenager or young adult with pain and swelling around joint, usually, the shoulder, knee or around hip [4].

Typically it is defined as a lytic bone lesion with a predilectionfor the epiphyseal region of long bones of skeletallyimmature individuals, although 50% of the tumors also involve the metaphysis [5].

Numerous studies have shown that chondroblastoma overwhelmingly arises from the epimetaphyseal region. Only rare cases have been reported in the diaphyseal region [6].

The neoplastic cells (chondroblasts) are generally arranged in sheets without alobular architecture, a feature which can help differentiate it from chondromyxoid fibroma, which generally exhibits a lobular architecture. The chondroblastsare generally intermediate-sized, round to polyhedral, and display round to oval nuclei, often with grooves. Perhaps, the most recognizable feature is the presence, albeit sometimes focal, of chicken-wirecalcification. Giant cell density varies from lesion to lesion [5].

The purpose of this study is to present a case of pure diaphysealchondroblastomas in a 13 years old girl.

2. Case Report

A 13-year-old girl was referred to our hospital because of pain and swelling over the proximal region of her left leg for 4 years. There was no historyof previous trauma. On examination, there was pain on pressure over the left leg, which was painless at rest.

The patient was afebrile. Anteroposterior and lateral x-rays demonstrated a lesion with these characteristics: aneccentric, multiloculated, expansil ,osteolytic and well defined lesion in the diaphysis of the left tibia(see Figure 1).

Figure 1. sharply delineated lytic appearance of chondroblastoma

Physical examination was remarkable for a brief pain uponpalpation of the proximal left leg and deformities.

After decalcification an aggregate of red-tan soft tissue wasroutinelyprocessed to hematoxylin and eosin (H&E) glass slides. Microscopy showed proliferation of polygonal cells in the cartigenous background.

Figure 2. Small tumor cells of round shape are accompanied by scattered osteoclasts

Figure 3. Small tumor cells of round shape are accompanied by scattered osteoclasts (Closer view)

Giant cells were few in numberand scattered.

Based on the findings, the diagnosis was chondroblostom.

3. Discussion

In 1927, Kolodny was the first to describe a bone tumor presenting as a “giant cell variant.” Ewing called it a “calcifying giant cell tumor,” and later, in 1931, Codman termed it as “epiphyseal chondromatous giant cell tumor.” The term benign “chondroblastoma” was proposed by Jaffe and Lichtenstein in 1942 to describe this rare, benign, and distinctive tumor composed of immature chondroblasts with a scant chondroid matrix [7].

According to the WHO 2002 definition, “chondroblastoma is a benign, cartilage-producing neoplasm usually arising in the epiphyses of skeletally immature patients.” [4]

Chondroblastomas usually show round or oval, geographic, lucent lesions with sharply marginated borders on radiograph. CT scans can depict matrix mineralization, soft tissue extension, and cortical erosion. The signal intensity characteristics of chondroblastoma on MRI reflect the prominent cellular stroma of the tumor, which has low signal intensity on T1-weighted images and high or variable signal intensity on T2-weighted images. [5]

Resemble those of Langerhanscells. Mitoses are exceptional. Intracytoplasmicglycogen granules are present, sometimes in large numbers. Reticulinfibers surround each individual cell. Recurrent lesions may showsome degree of atypia, a feature that should not be interpreted as asign of malignant transformation. A distinctive microscopic changeis the presence of small zones of focal calcification. These zonesrange from a network of thin lines ('chicken wire') to obviousdeposits surrounded by giant cells. [3]

Mostly, authors describe CB as an eccentric, oval or round well-defined lesion mostly seen in epiphyseal region of the long bones, predominantly in the proximalfemur, proximal tibia, and proximal humerus.

The lesion’s epiphyseal location is an important diagnostic radiological feature. It is well known that the contiguous involvement of the metaphyseal region occursfrequently, but purely metaphyseal and diaphyseal tumors are extremely rare. [4]

In Table 1 [7] the purely metaphyseal and diaphysealchondroblastoma cases that has been reported till 2008 is listed.

A valid clarification for this unusual location was offered by Brien et al. who reported that chondroblastoma is derived from amultipotential mesenchymal cell of the tendon sheaththat, and within an osseous environment, would have atendency to chondroid formation. [1]

Possibly the source of the tumor in our patient was from an embryonic rest of the primitive cartilage prior to its endochondralossification.

Chondroblastoma does not undergo spontaneous resolution if left untreated; therefore, intervention is necessary to stop progression and alleviate pain [8].

The goals for the treatment of patients with chondrosarcoma include minimizing the risks of local recurrence, metastatic disease, and death from disease, all the while maximizing the function of the patient. Because of the relative inefficacy of adjuvant therapies, adequate surgical management of chondrosarcoma is the mainstay of treatment. [9]

The accepted treatment method for chondroblastoma is curettage and filling of the bone defect. [10]

Radiofrequency ablation for chondroblastoma provides an alternative to surgical curettage, and have demonstrated both a clinical improvement in symptoms and the follow-up MRI appearances in some cases. [11]

Radiation therapy is not preferred owing to the tumor’s low sensitivity to radiation and the possibility of inducing a secondary sarcoma, [10] but this therapy is appropriate for the treatment of positive surgical marginsor palliation of disease-related symptoms.

The role for chemotherapy in the treatment of patients with local and advanced chondrosarcoma remains undefined. Although its use in conventional chondrosarcoma has been largely ineffective, recent data have suggested a possible role in certain subtypes of chondrosarcoma, specifically the dedifferentiated and mesenchymal variants. [9]

Table 1.

4. Conclusions

To bring to an end, chondroblastoma should be considered as a differential diagnosis of any primary diaphyseal lesion in a patient presenting with constitutional symptoms.

References

[1]   | Ma X, Dong Y, Zhang C, Zeng B, Ding J. Diaphyseal chondroblastoma in the tibia: One case report and literatures review. Chinese Journal of Clinical Oncology. 2008;5(6):459-61.
[2]   | ROSAI J. Surgical pathology. 2: china; 2011. p. 2035-37.
[3]   | Azorin D, González-Mediero I, Colmenero I, De Prada I, López-Barea F. Diaphyseal chondroblastoma in a long bone: first report. Skeletal radiology. 2006;35(1):49-52.
[4]   | Punit A, Nadkarni S, Doomra T, Punit A. Chondroblastoma of Diaphysis of Radius in a Seven Year Old Child.
[5]   | Clapper AT, DeYoung BR. Chondroblastoma of the femoral diaphysis: report of a rare phenomenon and review of literature. Human pathology. 2007;38(5):803-6.
[6]   | Bali K, Kumar V, Rawall S, Sudesh P. A rare case of metaphyseodiaphyseal chondroblastoma of proximal femur presenting with constitutional symptoms in a six-year-old child. Journal of Pediatric Orthopaedics B. 2012;21(2):174-8.
[7]   | Maheshwari AV, Jelinek JS, Song AJ, Nelson KJ, Murphey MD, Henshaw RM. Metaphyseal and diaphyseal chondroblastomas. Skeletal radiology. 2011;40(12):1563-73.
[8]   | Erickson JK, Rosenthal DI, Zaleske DJ, Gebhardt MC, Cates JM. Primary Treatment of Chondroblastoma with Percutaneous Radio-frequency Heat Ablation: Report of Three Cases 1. Radiology. 2001;221(2):463-8.
[9]   | Riedel RF, Larrier N, Dodd L, Kirsch D, Martinez S, Brigman BE. The clinical management of chondrosarcoma. Current treatment options in oncology. 2009;10(1-2):94-106.
[10]   | Atalar H, Basarir K, Yildiz Y, Erekul S, Saglik Y. Management of chondroblastoma: retrospective review of 28 patients. Journal of Orthopaedic Science. 2007;12(4):334-40.
[11]   | Christie-Large M, Evans N, Davies A, James S. Radiofrequency ablation of chondroblastoma: procedure technique, clinical and MR imaging follow up of four cases. Skeletal radiology. 2008;37(11):1011-7.