Temporal Bone Chondroblastoma: Systematic Review of Clinical Features and Outcomes.

OBJECTIVE: Chondroblastoma is an uncommon benign neoplasm of cartilaginous origin usually involving the long bones. The temporal bone is a rare location for this tumor. The clinical profile, optimal medical and surgical management, and outcomes of treatment for temporal bone chondroblastoma remain unknown. METHODS: We performed a systematic review of the SCOPUS, PubMed, and CENTRAL databases for case reports and case series on patients with histopathologically proven temporal bone chondroblastoma. Data on demographics, clinical manifestation, surgical management, adjuvant treatment, and outcome on last follow-up were collected. RESULTS: A total of 100 cases were reported in the literature, including one described in the current study. The mean age of patients was 42.3 years (range, 2-85 years), with a slight male predilection (1.3:1). The most common clinical manifestations were otologic symptoms (e.g., hearing loss [65%], tinnitus, and otalgia) and a palpable mass. Surgical excision was performed in all cases, with gross total excision achieved in 58%. Radiation therapy was performed in 18% of cases, mostly as adjuvant treatment after subtotal excision. There were no deaths at a median follow-up of 2 years. Among the patients with detailed status on follow-up, 58% had complete neurologic recovery, 38% had partial recovery, and 4% had progression of symptoms as a result of tumor recurrence. CONCLUSIONS: Temporal bone chondroblastoma has a distinct clinical profile from chondroblastoma of long bones. Surgery is the mainstay of treatment, and radiation therapy may be given after subtotal excision. Outcomes are generally favorable after treatment.

Functional and Oncological Outcome After Treatment of Chondroblastoma With Intralesional Curettage.

BACKGROUND: Chondroblastoma is a rare cartilaginous benign bone tumor that commonly arises in long bones of young people. Surgical management is the primary treatment of choice for chondroblastoma and it entails adequate intralesional curettage alone or in combination with adjuvants. This study was performed to describe the epidemiological characteristics and clinical and radiologic results of intralesional curettage of chondroblastoma. METHODS: This was a retrospective study which included an analysis of 91 patients with chondroblastoma who were treated with intralesional curettage and were followed up between 1994 and 2014 for at least 3 years. Epidemiological data, clinical symptoms, radiologic and histologic investigations, surgery, functional outcomes, complications, and local recurrence rate were analyzed. RESULTS: There were 60 males (65.9%) and 31 females (34.1%) with a mean age of 16.4 years. The most commonly involved bone was the proximal tibia in 24 patients (26.4%), followed by distal femur in 20 patients (22%), proximal humerus in 17 patients (18.7%) and proximal femur in 15 patients (16.6%). All patients underwent intralesional curettage. High-speed burr was used in 66 patients (72.5%). The resultant cavity was filled with autogenous bone graft, bone substitute, bone cement or a combination of bone graft and cement. Four patients (4.4%) had complications. Three patients (3.3%) developed local recurrence. Age, site, history of previous intervention or pathologic fracture had no impact on the rate of recurrence. The mean Musculoskeletal Tumor Society (MSTS) score was 28.88 (range, 24 to 30) points. CONCLUSIONS: Thorough curettage using high-speed burr and bone-grafting or bone cement in the treatment of chondroblastoma has good local control, low recurrence rate and excellent functional long-term outcome. LEVEL OF EVIDENCE: Level IV, retrospective case series.

The effects of surgical treatment with chondroblastoma in children and adolescents in open epiphyseal plate of long bones.

BACKGROUND: Chondroblastoma is a rare benign cartilaginous tumor, which primarily occurs in children and adolescents. Chondroblastoma commonly originates in the epiphyseal plate of long bones. An aggressive curettage treatment is recommended to manage lesion, which may jeopardize an open epiphyseal plate and result in limb shortening and deformity as the limb grows and develops. The purpose is to observe surgical effects of chondroblastoma on open epiphyseal plate of long bones in children and adolescents and explore influences on limb growth and development. METHODS: We retrospectively reviewed 18 cases of long bone chondroblastoma with open epiphyseal growth plate during March 2004 to October 2010 in our center. Seven females and 11 males with mean age of 11.6 ± 2.0 years old (8-15 years) were included. Patients, who suffered from trauma and pathological fracture of the epiphyseal plate or congenital diseases such as poliomyelitis, congenital dementia, and cartilage malnutrition, were excluded. All patients were treated with meticulous intralesional curettage and inactivity with alcohol followed by bone grafts. All cases were followed up 8.2 ± 1.7 years (5-11.5 years). RESULTS: All had no local recurrence and distance metastasis. The length of the affected limb was short, 18.47 ± 7.22 mm (1.5-30 mm). There was no obvious relativity with tumor activity (P = 0.061). Meanwhile, there were obvious relativity with the greatest dimension of the lesion (TGD) (P = 0.003), the vertical dimension between edge of lesion and epiphyseal line (TVD) (P = 0.010), and area ratio of lesion to local epiphysis (lesion/growth plate) (P = 0.015). The MSTS93 (Revised Musculoskeletal Tumor Society Rating Scale 93) and SF-36 (Medical Outcomes Study 36-Item Short-Form Health Survey) had been significantly improved (P < 0.01). CONCLUSION: Managing of chondroblastoma located in open epiphyseal plate of a long bone with meticulous curettage, inactivity, and bone grafts can control tumor progression and recurrence effectively. Meanwhile, early detection and prompt surgical treatment intervention, which reduced significantly the tumor to influence limb growth and development, get encouraging limb function. TRIAL REGISTRATION: This is a retrospective study, which was not registered in any trial registry.

Chondroblastoma in adult age.

PURPOSE: Chondroblastoma (CB) is a rare benign tumor that occurs most commonly in the second decade of life. No studies on CB in adulthood have been reported. Our purposes were to report a single-institution experience on CB in adults and to discuss the clinical and imaging findings, type of treatment, oncologic and functional outcomes. PATIENTS AND METHODS: All patients diagnosed and treated for CB from 1981 to 2014 were reviewed. The main inclusion criterion was patients above their 30 years of age at diagnosis. Clinical presentation, imaging, surgical treatment, complications and functional outcome were evaluated. Local recurrences were recorded. RESULTS: There were 15 males (65%) and 8 females (35%) with a mean age of 40 years (range 30-59 years). Tumor involved the small bones (talus, calcaneus, acromion, cuneiform, metatarsals) in 15 patients (65%) and the long bones in eight patients (35%). Treatment consisted of curettage only (6 cases), curettage plus phenol (13 cases), curettage plus radiotherapy (one patient with vertebral tumor), resection (two patients with CB of the acromion) and chopart amputation (one patient with a metatarsal tumor). At a mean follow-up of 8.4 years (range 3-30 years), twenty patients (87%) remained continuously disease-free, whereas three had local recurrences. None of the patients received chemotherapy. CONCLUSION: This study confirms that the occurrence and outcome of adult patients with CB are similar with those affecting younger patients. The main difference consists of the involved bones; flat bones and short tubular bones of the foot, especially the talus and calcaneus, are most commonly involved as opposed to long tubular bones. LEVEL OF EVIDENCE: Therapeutic study, level IV-1 (case series).

Molecular basis for oncohistone H3 recognition by SETD2 methyltransferase.

High-frequency point mutations of genes encoding histones have been identified recently as novel drivers in a number of tumors. Specifically, the H3K36M/I mutations were shown to be oncogenic in chondroblastomas and undifferentiated sarcomas by inhibiting H3K36 methyltransferases, including SETD2. Here we report the crystal structures of the SETD2 catalytic domain bound to H3K36M or H3K36I peptides with SAH (S-adenosylhomocysteine). In the complex structure, the catalytic domain adopts an open conformation, with the K36M/I peptide snuggly positioned in a newly formed substrate channel. Our structural and biochemical data reveal the molecular basis underying oncohistone recognition by and inhibition of SETD2.

Tumors of the coracoid process: clinical evaluation of twenty-one patients.

OBJECTIVE: We present the incidence and management of bone tumors of the coracoid process and discuss the related clinical and imaging findings and treatment. MATERIALS AND METHODS: We present 21 patients (7 males and 14 females; mean age, 39 years) treated for bone tumors of the coracoid process from 1900 to 2010. Mean follow-up was 44 months (range, 12-132 months). Clinical presentation, imaging, surgical treatment, complications, range of shoulder motion, and Musculoskeletal Tumor Society (MSTS) function were evaluated. RESULTS: Bone tumors were benign in 7 (33%) and malignant in 14 (67%). The most common were chondrosarcomas, osteoblastomas, and chondroblastomas. The most common presentation was pain and palpable mass for a mean duration of 11 months. Limb salvage, with or without megaprosthetic reconstruction, was achieved in 20 patients. One patient required forequarter amputation. One patient with chondroblastoma and 2 with chondrosarcoma had local recurrence. The range of shoulder motion varied according to the type of resection: patients with curettage and limited resections without involvement of the abductor mechanism had better shoulder motion, and patients with scapulectomy and proximal humeral resections had significant limitations of motion. The mean MSTS score was 80% (range, 50%-100%). CONCLUSIONS: Chondrosarcomas, osteoblastomas, and chondroblastomas are the most common bone tumors of the coracoid process. Limited resections are associated with nearly normal range of motion and excellent function; however, limited resections are acceptable in only in a small number of patients. In patients with malignant and recurrent lesions, wide resection is required, which is associated with significant limitations of shoulder function.

Condroblastoma de escafoides carpiano / Chondroblastoma of the carpal scaphoids

El condroblastoma es un tumor benigno de origen cartilaginoso que representa menos del 1% de todos los tumores óseos primarios. Se localiza típicamente en la epífisis de los huesos largos. Un 10% de los condroblastomas afectan a huesos del pie y de la mano. El asiento de esta lesión en el carpo es extraordinariamente raro. Se presenta un caso de condroblastoma del escafoides del carpo que fue tratado mediante curetaje de la lesión y relleno con injerto óseo autólogo de cresta iliaca (AU)

A chondroblastoma is a benign tumour of cartilaginous origin which represents less than 1% of all primary osseous tumours. It is typically localised in the epiphysis of the long bones. Some 10% of chondroblastomas affect the bones of the foot and the hand. It is extremely rare for the seat of this lesion to be in the carpal bones. We present a case of chondroblastoma of the carpal scaphoids that was treated through curretage of the lesion and filling with autologous osseous graft from the iliac crest (AU)

Management of chondroblastoma: retrospective review of 28 patients.

BACKGROUND: This study retrospectively reviewed 28 patients who presented with chondroblastoma in our hospital during the period March 1986 to February 2006. We investigated factors associated with local recurrence of the tumors after surgery. METHODS: We recorded the patients' sex and age, the location of the pathology, imaging studies (status of growth plate and activity of lesions), histological findings, and treatment outcomes. RESULTS: Pain was the presenting symptom in all patients. The tumors were generally located in the epiphyses or apophyses of long bones, especially in the proximal humerus (9 patients), proximal tibia (7 patients), and proximal femur (5 patients). Our preferred treatment method was generally aggressive curettage of the lesion followed by bone grafting. There was local recurrence in six patients. A secondary aneurysmal bone cyst was present in two patients. CONCLUSION: Aggressive curettage and bone grafting resulted in local control in most patients. Tumor recurrence was not statistically associated with age, sex, growth plate status, tumor location, or tumor activity. The only parameter associated with increased local recurrence was previous surgery for chondroblastoma in the same location.

Chondroblastoma of bone: long-term results and functional outcome after intralesional curettage.

We undertook this retrospective study to determine the rate of recurrence and functional outcome after intralesional curettage for chondroblastoma of bone. The factors associated with aggressive behaviour of the tumour were also analysed. We reviewed 53 patients with histologically-proven chondroblastoma who were treated by intralesional curettage in our unit between 1974 and 2000. They were followed up for at least two years to a maximum of 27 years. Seven (13.2%) had a histologically-proven local recurrence. Three underwent a second intralesional curettage and had no further recurrence. Two had endoprosthetic replacement of the proximal humerus and two underwent below-knee amputation after aggressive local recurrence. One patient had the rare malignant metastatic chondroblastoma and eventually died. The mean Musculoskeletal Tumour Society functional score of the survivors was 94.2%. We conclude that meticulous intralesional curettage alone can achieve low rates of local recurrence and excellent long-term function.

Chondroblastoma of the patella presenting as knee pain in an adolescent.

We report a case of chondroblastoma of the patella, associated with an aneurysmal bone cyst and pathologic fracture, that presented as knee pain in a 13-year-old girl. This case is the fifth case in the literature that associates patellar chondroblastoma with fracture, which should probably be regarded as a significant feature of this lesion.

Epiphyseal chondroblastoma of bone. Long-term effects on skeletal growth and articular function in 15 cases treated surgically.

The authors describe the long-term sequelae of chondroblastoma in 15 patients with open growth plates, whose age at operation ranged from 8 to 15 years. At follow-up, the youngest patient was 21 and the oldest 48 years old. Upper limb length discrepancy ranging from 2 cm to 10 cm was present in the four patients who had a proximal humeral epiphyseal location; in three of them, the range of motion of the shoulder was also limited and X-rays showed marked irregularities of the humeral head. Lower limb length discrepancy ranging from 0.5 cm to 2 cm was found in five of the eight patients in whom the tumor affected lower limb epiphyses. One patient with proximal tibial epiphyseal involvement also had mild genu valgum. Radiographic osteoarthritis was present only in the trapeziometacarpal joint of a patient in whom the first metacarpal bone affected by the tumor was replaced by a free fibular graft. The abnormalities observed did not cause important functional loss in either the everyday or the working activities of any of our patients.

[Studies on internal pressure and oxygen tension of bone tumors and tumor conditions. II--Growth rate, internal pressure and oxygen tension in bone lesions].

Internal pressure and oxygen tension were measured in 24 patients with bone tumors and tumorous conditions. High internal pressure were observed in most of the rapidly growing lesions. The internal pressure of slowly growing and non-growing lesions were not significantly different from those of normal bone marrow. Oxygen tension was commonly higher in the rapidly growing lesions than in simultaneously obtained peripheral venous samples from the same patients. A high correlation was found between internal pressure and the growth rate of the bone lesions in this study. These data suggest that internal pressure and oxygen tension of the lesions reflect the degree of blood supply to the bone tumors and tumorous conditions.

Periosteal bone apposition in chondroblastoma.

Periosteal bone apposition in chondroblastoma of long bones is more frequent than generally recognized. This should not cause confusion in differential diagnosis. The findings does not seem to have prognostic significance.