Analysis for clinical feature and outcome of chondroblastoma after surgical treatment: A single center experience of 92 cases.

BACKGROUND: Chondroblastoma is a rare, benign, cartilaginous bone tumor derived from epiphyseal chondrocytes. Although the clinical characteristics and experience of surgical treatment of the smaller number of patients has been reported in the literature, it is difficult to draw conclusions about the clinical and radiographic features and the outcome for surgical treatment of this disease due to the rarity of chondroblastoma. This study was aiming to review the epidemiologic characteristics and outcome of surgical management for patients with chondroblastoma. METHODS: We performed a retrospective analysis of 92 patients with chondroblastoma. Clinical data, radiographic images, surgical treatment and outcome were analyzed. Eighty-two patients received the extensive intralesional curettage and ten cases had the En-block resection. RESULTS: The most common site of disease was proximal femur (20.7%, 19/92), followed by distal femur (18.5%, 17/92) and proximal tibia (16.3%, 15/92). The secondary aneurysmal bone cyst component was most common for chondroblastoma of the small irregular bones, such as patella and foot. Four (4.3%, 4/92) cases receiving the extensive intralesional curettage developed the local recurrence, respectively two at the proximal tibia, one at the pelvis and one at the calcaneus. Time to local recurrence were respectively 14.5, 8.8, 27.0 and 5.6 months, with the average 14 months. Kaplan-Meier estimated survivorship curve of local recurrence-free survival rates of one, two and five years were respectively 97.7%, 96.2% and 93.9%. The mean Musculoskeletal Tumor Society (MSTS) score was 29.4. At the last follow-up, no one had the pulmonary metastasis and death associated with the disease. CONCLUSION: Intralesional curettage plus local adjuvants can obtain satisfactory outcome for chondroblastoma. LEVEL OF EVIDENCE: Level IV.

Solitary long-bone epiphyseal lesions in children: radiologic-pathological correlation and epidemiology.

BACKGROUND: Solitary epiphyseal lesions are rare and present with nonspecific imaging features. Knowledge regarding etiologies of pediatric epiphyseal lesions is limited to small studies. OBJECTIVE: The purpose of this study was to determine the relative incidence of pathologies affecting the pediatric epiphysis based on biopsy-proven cases with imaging. MATERIALS AND METHODS: We conducted a retrospective review of imaging studies including the terms "biopsy" or "resection" and entities known to affect the epiphysis and cross-referenced these with pathology reports, recording the relevant clinical data. Two radiologists performed comprehensive imaging review and recorded relevant features. RESULTS: Forty-nine children and adolescents met inclusion criteria. The long-bone epiphyseal lesion etiologies included chondroblastoma (n=22, 45%), nonspecific nonmalignant pathology (n=11, 22%), osteomyelitis (n=9, 18%), lymphoma (n=2, 4%) and 1 case of each of aneurysmal bone cyst, chondrosarcoma, enchondroma, hemangioendothelioma, and non-Langerhans cell histiocytosis. Median age was 13.1 years old (range 1.5-18.6 years). We performed comparative analysis of the two most common lesions in our series, chondroblastoma and osteomyelitis. Chondroblastoma was significantly more likely to be peripherally located (94% vs. 33%, P=0.002) and to demonstrate a discrete T1-weighted hypointense rim (94% vs. 33%, P=0.002); there were no significant differences in enhancement or intrinsic signal properties. Children with chondroblastoma were older (15.1 years vs. 7.3 years, P=0.001), and chondroblastoma lesions were significantly larger, with mean maximum lesion diameter of 25 mm (interquartile range [IQR] 20-30) vs. 12 mm (IQR 11-18) (P=0.001) and lesion volumes of 4.4 mL (IQR 2.4-7.9) vs. 0.4 mL (IQR 0.2-1.4) (P=0.01). CONCLUSION: This study reports the relative frequency of pathology of pediatric solitary epiphyseal lesions and describes several features that might assist in differentiating between chondroblastoma and osteomyelitis.

Clinical features, treatments and long-term follow-up outcomes of spinal chondroblastoma: report of 13 clinical cases in a single center.

PURPOSE: Chondroblastoma (CB) in the spine is extremely rare and there is little published information regarding this subject. We attempt to explore the clinical features of spinal CB and address the importance of total resection, especially total en bloc spondylectomy (TES) for the treatment of spinal CB. METHODS: Clinical data of 13 consecutive CB patients who received surgical treatment in our center between January 2006 and December 2016 were reviewed retrospectively. Recurrence-free survival (RFS) was estimated by Kaplan-Meier method and Log-rank test. RESULTS: The 13 CB patients included 9 men and 4 women with a mean age of 32 years. The lesions were located in the cervical spine in 2 cases, thoracic spine in 5 cases, and lumbar spine in 6 cases. All the patients were treated surgically using either curettage, piecemeal total resection, or TES. Postoperative radiotherapy was administered in 2 cases. The mean follow-up period was 41.6 months. Relapse occurred in 3 (23.1%) cases, resulting in one death in 60 months. The mean RFS duration was 28.7 months. CONCLUSIONS: CB predominantly affects males and various age groups. Spinal CB more commonly involves the thoracic and lumbar segments. Spinal CB usually appears as an aggressive and destructive bony lesion with a soft tissue mass on imaging, forming compression on the spinal cord in some cases. Recurrence is not uncommon for spinal CB. Total resection, especially TES, has been confirmed as a powerful method to control the disease, while curettage is more likely to associate with local recurrence. Radiotherapy does not seem to reduce local recurrence.

Chondroblastoma of bone in the extremities: a multicenter retrospective study.

BACKGROUND: Chondroblastoma is a rare benign cartilage tumor that commonly occurs in children and adolescents. This study was designed to review the epidemiologic characteristics and outcomes of surgical management in a large series of patients with extremity chondroblastoma. METHODS: We performed a multicenter retrospective analysis of 199 patients with extremity chondroblastoma. Clinical data, radiographic images, histological findings, treatment, and outcome were analyzed. RESULTS: There were 145 male patients and fifty-four female patients with a mean age of 18.0 years. The most commonly involved bone was the proximal part of the tibia (fifty-five patients [27.6%]), followed by the proximal part of the femur (fifty-two patients [26.1%]) and the distal part of the femur (thirty-eight patients [19.1%]). Prior to presentation, 73.4% (146 of 199 patients) experienced pain. The mean duration of pain and other symptoms was 8.7 months. The physis was open in 25.7%, it was closing in 22.2%, and it was closed in 52.1% of the patients at the time of presentation. One hundred and twenty-six patients had at least twenty-four months of follow-up; their mean follow-up duration was 62.1 months (range, twenty-four to 190 months). Initial treatment was curettage for 119 patients (94.4%) and en bloc resection for seven patients (5.6%). The local recurrence rate was 5.0% after curettage and 0% after resection. The only significant factor related to recurrence was the location of the lesion in the proximal part of the humerus (p = 0.001). CONCLUSIONS: Chondroblastoma occurs most frequently in the proximal part of the tibia and the proximal part of the femur with significant male predilection. In this series, recurrence was most frequent in the proximal part of the humerus. Our results suggest that curettage and bone-grafting provide favorable local control and satisfactory functional outcome for patients with this disease. LEVEL OF EVIDENCE: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Secondary osteosarcoma: is there a predilection for the chondroblastic subtype?

Osteosarcoma is the most common form of primary bone cancer in the adolescent and young adult patient population. Outcomes in patients with secondary osteosarcoma are inferior compared with outcomes in patients with primary osteosarcoma. The goal of this study was to investigate whether there is a predilection for the chondroblastic histologic subtype in secondary osteosarcoma. A retrospective chart review was performed to identify cases of secondary osteosarcoma treated at 1 institution from 1991 to 2012. Histologic subtypes were evaluated by a pathologist, and a review of the literature was also performed to identify the histologic subclassification of additional series of secondary osteosarcomas. Of a total of 131 cases of osteosarcoma, 9 (6.9%) were identified as a secondary malignancy. Only 2 cases (22%) were identified as chondroblastic variants, although 6 (67%) showed some degree of chondroid differentiation. Of the 3 cases meeting the criteria for postradiation osteosarcoma, 2 (67%) were identified as chondroblastic variants and all 3 showed some degree of chondroid differentiation. Five other studies evaluating histologic subtypes in postradiation osteosarcoma showed a cumulative frequency of 20% for the chondroblastic variant. Although the study results did not support the hypothesis of an association between secondary osteosarcoma and the chondroblastic subtype, the high proportion of cases of postradiation osteosarcoma with the chondroblastic subtype and the even higher proportion showing some degree of chondroid differentiation are noteworthy features of this disease.

Chondroblastoma: a clinico-pathological analysis.

OBJECTIVE: To determine the clinico-pathological and histological features of Chondroblastoma (CB). STUDY DESIGN: Case series. PLACE AND DURATION OF STUDY: The Aga Khan University Hospital, Karachi, from 2000 to 2013. METHODOLOGY: The histological slides of all reported cases of CB were retrieved and reviewed for morphological features. Clinical features were noted from surgical pathology reports. Frequency of features was noted. RESULTS: Sixty one cases of CB were identified. Pain was the most common presenting symptom. The age ranged from 10 to 38 years (mean 20 ± 1.98 years; M: F 2.5:1) with 61% patients in the second decade of life. Forty six cases occurred in long tubular bones; distal femur being most common site. Metaphysis, clavicle, temporal bone and metacarpal were also involved. Histologically, all CBs were composed of round to polygonal cells and scattered osteoclast-type multinucleated giant cells and majority had hemosiderin pigment. Chicken-wire calcifications and coarse calcifications were seen in 85% and 26% of cases respectively. A spindle cell component was seen in 54% of cases. ABC-like areas were seen in 10 cases. Mitosis ranged from 1 to 6/10 HPFs. Recurrence was seen in 2 cases. Recurrent tumor showed similar morphology when compared with the initial tumor. CONCLUSION: CB is a benign tumor but has potential for recurrence. Males are more affected, second decade is more common and distal femur is most common site. Metaphysis, clavicle, temporal bone and metacarpal were the rare sites of CB. Histological features predictive of recurrence were not separately identified.

Distinct H3F3A and H3F3B driver mutations define chondroblastoma and giant cell tumor of bone.

It is recognized that some mutated cancer genes contribute to the development of many cancer types, whereas others are cancer type specific. For genes that are mutated in multiple cancer classes, mutations are usually similar in the different affected cancer types. Here, however, we report exquisite tumor type specificity for different histone H3.3 driver alterations. In 73 of 77 cases of chondroblastoma (95%), we found p.Lys36Met alterations predominantly encoded in H3F3B, which is one of two genes for histone H3.3. In contrast, in 92% (49/53) of giant cell tumors of bone, we found histone H3.3 alterations exclusively in H3F3A, leading to p.Gly34Trp or, in one case, p.Gly34Leu alterations. The mutations were restricted to the stromal cell population and were not detected in osteoclasts or their precursors. In the context of previously reported H3F3A mutations encoding p.Lys27Met and p.Gly34Arg or p.Gly34Val alterations in childhood brain tumors, a remarkable picture of tumor type specificity for histone H3.3 driver alterations emerges, indicating that histone H3.3 residues, mutations and genes have distinct functions.

[Chondroblastoma of bone ]. / Chondroblastomes osseux.

BACKGROUND: Chondroblastoma is a rare and benign cartilaginous tumor of bone often localized in the epiphysis of long bones and affecting young persons between 10 and 20 years. AIM: To study the epidemiological, clinical and radiological aspects of bone chondroblastoma, to assess the outcome of chirurgical treatment and factors associated with recurrence. METHODS: It's a retrospective study, concerning 13 patients with chondroblastoma of bone, attending the orthopedic department of Sahloul hospital between 1991 and 2007. The patients' age, sex, clinical features, radiological aspects, treatment and evolution were recorded. After initial clinical examination, all patients have had plane radiographs. Twelve patients were explored by Computed tomography. The treatment was surgical in every case: curettage of the lesion in 11 cases associated with bone grafting in 5 cases and cementation in one case, one in-bloc resection and one simple biopsy. Histology confirmed the diagnostic in all cases. RÉSULTS: The average age of patients was 18, 2 years and sex ratio was 2, 3. the long bones are the most commonly involved. The main presenting symptoms were pain in all cases and local swelling in 8 cases. Radiographs showed a lytic geographic well-defined lesion in 7 cases. Computed tomography showed better calcifications and soft tissue extension. The mean follow-up of our patients was 8 years (6 mois-18 ans). Two recurrences were noted. Functional results were good in 11 cases. CONCLUSION: Chondroblastoma is a rare and benign cartilaginous tumor of bone which usually involves the epiphysis of long bones. Plane radiographs are sufficient for diagnostic in typical cases. MRI is interesting in unusual presentations and localizations. Curettage and grafting constitute the treatment of choice. Prognosis depends basically on a relatively high rate of recurrence. Aggressive feature of the lesion and incomplete curettage are the most important predictive factors of recurrence.

Chondroblastoma of bone in a pediatric population.

BACKGROUND: Chondroblastoma is a rare benign bone lesion that occurs in young patients and has a high rate of recurrence. The purpose of the present study was to report on eighty-seven cases of chondroblastoma in children and to identify the possible factors that increase the risk of recurrence. METHODS: We retrospectively reviewed eighty-seven cases of chondroblastoma in patients with open physes at the time of diagnosis and treatment. Historical data, complete imaging data, histological findings, and surgical charts were analyzed. Multiple logistic regression was used to identify predictors of recurrence. RESULTS: The series included fifty-three boys and thirty-four girls with a mean age of 12.5 years. Lesions were located in the epiphysis in 68% of the patients, especially in the proximal part of the tibia (twenty-four patients) and the proximal part of the femur (twenty-three patients). Pain was the presenting symptom in 84% of the patients. The treatment consisted of intralesional curettage with autogenous bone-grafting in 63% of the patients. The functional outcome at an average of 62.5 months of follow-up was good for 68.5% of the patients. At a minimum of twenty-four months of follow-up, 32% of the lesions had recurred. Sex, radiographic aggressiveness, an aneurysmal bone-cyst component on histological analysis, and the method of surgical treatment had no significant influence on recurrence. Epiphyseal chondroblastomas were associated with a higher risk of recurrence when compared with metaphyseal, apophyseal, and epiphyseal-metaphyseal lesions (p = 0.004). CONCLUSIONS: Chondroblastoma in growing children is most frequently located in the proximal part of the tibia and the proximal femoral epiphysis. The recurrence rate is high, particularly for strictly epiphyseal lesions. Proximal femoral lesions and tarsal lesions are associated with a poorer outcome.

Vertebral chondroblastoma.

OBJECTIVE: To determine the age distribution, gender, incidence, and imaging findings of vertebral chondroblastoma, and to compare our series with findings from case reports in the world literature. DESIGN AND PATIENTS: Case records and imaging findings of nine histologically documented vertebral chondroblastomas were retrospectively reviewed for patient age, gender, vertebral column location and level, morphology, matrix, edema, soft tissue mass, spinal canal invasion, and metastases. Our findings were compared with a total of nine patients identified from previous publications in the world literature. The histologic findings in our cases was re-reviewed for diagnosis and specifically for features of calcification and secondary aneurysmal bone cyst (ABC). Clinical follow-up was requested from referring institutions. RESULTS: Nine of 856 chondroblastomas arose in vertebrae (incidence 1.4%; thoracic 5, lumbar 1, cervical 2, sacral 1). There were six males and three females ranging in age from 5 to 41 years (mean 28 years). Satisfactory imaging from seven patients revealed the tumor to arise from the posterior elements in four and the body in three. All tumors were expansive, six of seven were aggressive, and the spinal canal was significantly narrowed by bone or soft tissue mass in six. In one patient canal invasion was minimal. Calcification was pronounced in two and subtle in four. The sole nonaggressive-appearing tumor was heavily mineralized. Bony edema and secondary ABC were not seen on MR imaging. None of the cases had microscopic features of significant secondary ABC. Calcification, and specifically "chicken wire" calcification, was identified in two patients. Pulmonary metastases occurred in none. CONCLUSIONS: Vertebral chondroblastoma is a rare neoplasm that presents later in life than its appendicular counterpart. On imaging it is aggressive in appearance with bone destruction, soft tissue mass, and spinal canal invasion. The lesions contain variable amounts of mineral. Secondary aneurysmal cyst bone formation was not a feature in our study group.

Frequency, presentation and postintervention prognosis of chondroblastoma.

OBJECTIVE: To evaluate the frequency of clinico-radiological presentation and the prognosis after treatment of chondroblastoma. DESIGN: A descriptive, observational study. PLACE AND DURATION OF STUDY: The study was carried out at the Department of Orthopaedic Surgery, JPMC, Karachi over the period of 16 years, January 1986 to June 2002. METHODOLOGY: Patients in teenage, presenting with signs and symptoms of a primary bone tumours, were included by retrospectively reviewing the tumour registry record. Detailed scrutiny of clinical record was carried out to analyze the clinico-radiological presentation and posttreatment prognosis criteria of healing was symptom-free centripetal and homogeneous ossification. RESULTS: Seven (1.24%) out of 563 primary bone tumour cases were chondroblastoma. The clinico-radiological presentation in the 2 out of 7 (28.57%) cases was classical, 2 simulated osteomyelitis and other 3 as chondromyxoid fibroma, aneurysmal bone cyst and giant cell tumour. Lesions were treated with curettage and bone grafting. One patient (14.28%) developed recurrence within 2 years after curettage and healed after re-curettage and bone grafting. Five out of 7 (71.42%) tumours showed good response and 2 out of 7 (28.57%) showed satisfactory response. CONCLUSION: Chondroblastoma is very rare benign primary bone tumour with high local recurrence rate. The lesions confined to epiphysis of weight-bearing bone present early with symptoms of mild arthritis. Lesions in non-weight-bearing bones often present late and simulates epiphysio-metaphyseal tumours. Lesions heals with centripetal healing and need more than 2 years follow-up to achieve recurrence-free healing.

Chondroblastoma of bone.

BACKGROUND: Chondroblastoma of bone is a rare lesion, and few large series have been reported. The purpose of this paper is to report forty-seven cases treated by one group of surgeons and to identify factors associated with more aggressive tumor behavior. METHODS: Seventy-three patients with chondroblastoma of bone were treated between 1977 and 1998. We were able to obtain historical data, imaging studies, histological findings, and adequate personal or telephone follow-up to determine the outcome for forty-seven patients. RESULTS: The lesions were distributed widely in the skeleton, but most were in the epiphyses or apophyses of the long bones, especially the proximal part of the tibia (eleven tumors) and the proximal part of the humerus (ten tumors). The principal presenting symptoms were pain and limitation of movement. The treatment consisted of a variety of procedures, but the majority of the patients had intralesional curettage and packing with allograft or autograft bone chips or polymethylmethacrylate. Most of the patients had an excellent functional result, although in three osteoarthritis developed in the adjacent joint. Seven patients (15 percent) had a local recurrence; three of them had a second recurrence and one, a third recurrence. One patient died of widespread metastases, and another who had metastases to multiple sites was alive and disease-free after aggressive treatment of the metastatic lesions. CONCLUSIONS: While the size of the lesion, the age and gender of the patient, the status of the growth plate, and an aneurysmal-bone-cyst component to the tumor had no significant effect on the recurrence rate, lesions around the hip (the proximal part of the femur, the greater trochanter, and the pelvis) accounted for the majority (five) of the seven recurrent tumors and one of the two metastatic lesions.