Cornelia Nitipir, Ana Maria Popa and Cristina Orlov-Slavu
1. Raskin KA, Schwab JH, Mankin HJ, Springfield DS, Hornicek FJ. Giantcelltumor of bone. J Am Acad Orthop Surg. 2013; 21:118-26.
2. Behjati S, Tarpey PS, Presneau N, Scheipl S, Pillay N, Van Loo P et al. Distinct h3f3a and h3f3b driver mutations define chondroblastoma and giantcelltumor of bone. Nat Genet. 2013; 45:1479-82.
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R. Prejbeanu, D. Crişan, A. Bălănescu, H. Haraguş and B. Deleanu
Introduction. Segmental or intralesional excision with curettage or complete resection can be one method of treatment for giant cell tumor (GCT), but the ideal filling material after curettage or resection remains controversial. The purpose of this retrospective study was to follow the latest results and complications regarding the recurrence or degradation of functional status that underwent cementation. Material and methods. We reported 24 cases with GCTs during the last 15 years. All the patients were treated by intralesional excision or segmental resection followed by acrylic cement filling with or no metal augmentation recurrence-free survival proportions were used to evaluate oncological outcomes. Other parameters including surgical complication, general condition, and radiological classification were analyzed. Results. We followed up 20 cases for at least five years postoperatory (from the 5th to the 9th year). The recurrence-free survival proportions showed that the recurrence rate in this group was for 4 patients (2 of them were at second surgery). 2 patients had degradation of implant cement fixation. Parameters including patients’ age, gender, tumor location, and radiological classification did not affect the surgeons’ treatments in cavity filling after GCT curettage. Conclusions. Cementation should be recommended because of easy usage, have long lasting better results, and the better local tumor control than other methods (ex: bone grafting). The risk of recurrence is low and is not related to the cementation and metal augmentation. The cost-benefit is also in favor of this technique.
D. Rădulescu, A. Bădilă, O. Nuțiu, R. Manolescu, T. Ciobanu, I. Japie and R. Rădulescu
Introduction. Giant-cell tumor of the bone is a benign tumor, but with high local aggressiveness, even with risk of remote metastasis. Material and methods. We present the case of a 57-year-old woman, without significant pathological history, who, after clinical, imagistic and anatomopathological investigations, was diagnosed with giant cell tumor of the right distal radius. The patient underwent surgery and due to the size of the tumor and destruction of the surrounding cortical bone, segmental resection of the tumor in oncological limits was performed. The bone defect was filled with the proximal one third of the ipsilateral fibula, fixed to the remaining radius diaphysis with a plate and screws. Also, the autograft was stabilized to the proximal row of the carpal bones with 2 k-wires for 6 weeks. Postoperatively, clinical and X-ray check-ups were performed at 6, 12, 24 weeks and 1 year after surgery. Results. According to Mayo functional assessment score, the results were good. At 1 year after surgery, the patient gained 85 points, representing a good functional outcome of the surgery. This way, the wrist joint mobility and the carpal cartilage were preserved, providing a barrier against distal migration of any remaining tumoral cells, as well. Conclusions. It can be stated that in aggressive giant cell tumors located at the distal radius, the best therapeutic option is a segmental resection of the lesion followed by the replacement of the bone defect with a proximal fibular autograft. This method provides the best postoperative functional results with a lower risk of local recurrence and does not require microvascular surgery or access to a bone bank.
R.S. Cismașiu, R.M. Bîrluțiu, A. Tudor, D. Pop and C.I. Stoica
Intralesional tumoral procedures in giant cell tumor (GCT) are quoted as having a recurrence rate of up to 60%. Thus, a number of studies suggest that broad resection is associated with a lower local recurrence risk compared to intralesional curettage, increasing the free recurrence interval from 84% to 100%. The TCG involvement of the proximal tibia occupies a particular place through the relationship with the articular line, but especially through the frequent, direct, or indirect interest of the extensor mechanism of the knee. Purpose of the paper. Based on the unique tumor registry of “Foişor” Orthopedic Clinic, we proposed to follow all the cases of TCG with proximal tibial localized operation, beneficiaries of an “en bloc” resection and modular tumor prosthesis reconstructions - registering a number of 5 cases between 2009 and 2017. Material and method. The initial evaluation was performed by radiography, CT-scan and MRI investigations and recurrence cases in which histopathological reassessment became mandatory after post-intralesional techniques were also included in the study. The surgical technique followed tumor resection, tumor reconstruction, and the reconstruction procedure of the extensor of the knee, which involved the modulation of the modular tumor prosthesis with a rotary reversible flap of the medial gastrocnemius with the ankle of the patellar tendon to the prosthesis and flap. Quantification of functional outcomes required the use of the revised Muscle-Skeletal Society Score (rMTS) and postoperative complications were centralized; the most commonly reported being the peroneal nerve palsy. Results and discussions. The results obtained were compared to the orthopedic oncology literature data for such a procedure, following their superposition and discrepancy. The challenge of such an orthopedic oncology intervention is the reconstruction of the extensor, with definite functional implications of limiting the knee extension, as well as avoiding the peroneal nerve palsy. However, the results can be reproducible, with an absent relapse rate.
I.M. Japie, A. Bădilă, T. Ciobanu, R. Manolescu, D. Rădulescu, E.M. Japie, A. Bujdei and C. Cîrstoiu
Introduction. Giant-cell tumor of bone (GCTB) is a benign tumor with an unpredictable evolution, representing 4-5% of all primary bone tumors and 15% of benign bone tumors usually affecting 20-45 years old adults. The predilect location is the distal femur, proximal tibia, and distal radius. Case presentation. We report the case of a 31-year-old male, regardless of medical history, admitted in the emergency department (ED) for significant pain and functional impairment of the right knee, after suffering a traumatic event. Clinical examination and imaging tests established the diagnosis of lateral femoral condyle fracture. Therefore, osteosynthesis with 4 screws was performed. Postoperative evolution was uneventful until one year later when the patient presented to the ED for pain and inflammatory aspect of the right knee, but with no history of trauma during this time. The imagistic exams of the right knee (X-ray, magnetic resonance imaging and scintigraphy) detected a tumor of the lateral femoral condyle that also affected the osteosynthesis material. Thus, the removal of screws and histopathological exam were performed, the latter establishing the diagnosis of GCTB. Taking into consideration radiological and histological aspects of the tumor and relating them to the clinical findings, the GCTB was classified in stage III Enneking. The patient underwent surgery, segmental resection of the tumor in oncological limits and arthroplasty with modular tumoral prosthesis was performed. Postoperative results at 6 and 12 months according to Musculoskeletal Tumor Society Scoring System were very good. The key feature of this case consists of post-osteosynthesis appearance of the GCTB given the fact that only 3 cases of GCTB affecting the screw site were reported in literature.
R. Marinescu, D. Lăptoiu, I. Botezatu, S. Ciumeica, A. Bunea and G. Ştefan
Introduction. GCT resembles an aggressive benign tumor of bone and its evolution based on the histological features is unpredictable. About 50% of the cases are located around the knee (proximal femur and distal tibia), with the proximal humerus and distal radius representing the third and fourth most common sites. Femoral neck location is unusual. We report a case of GCT located at the femoral neck level, in a 19-year-old female. Case presentation. Onset was hidden by pathologic femoral neck fracture and, due to insidious symptoms, proper diagnosis and treatment were neglected for almost six months. After 6 months, the case was referred to our clinic and re-evaluated with complete examination and biopsy. Wide resection and tumoral arthroplasty of the hip was performed. Postoperative complete recovery was achieved and the patient returned to previous activities. After 2 years of normal clinical evolution, a neurological severe issue appeared; at this time, a cerebral metastasis was diagnosed. Once the positive diagnosis was achieved, 120 mg Denosumab treatment was initiated monthly. At 16 months follow-up, the patient was symptom free and continued Denosumab treatment. Discussion. Denosumab is a human monoclonal IGG2 antibody inhibiting osteoclast differentiation, activation, and survival with applicable suppression of bone turnover in patients with multiple myeloma, osteolytic bone disease, and bone metastases from breast and prostate cancer. It is also a useful drug for managing the GCT of bone and one excellent option in metastatic GCT. The long time safety and complications, especially in young female patients, are to be proven. Conclusions. Early diagnosis and accurate management of GCT are mandatory in order to achieve good long-term clinical results. Denosumab treatment may be necessary in order to avoid secondary metastasis or local recurrence.
D.N. Tarniţa, D.C. Grecu, M.C. Tenovici, R.C. Văduva, A.D. Tudora, A. Grecu, I.L. Petrovici and B. Căpitănescu
A 20-year-old patient presented to the emergency service with radial distal epiphysis after a minor trauma. The radiological examination indicated a fracture at the radial distal epiphysis on the background of a tumor that occupied the radial epiphysis in its entirety, with cortical burglary in some places. When consulting the oncologist, a surgical intervention for biopsy material harvesting was performed. The anatomopathological exam showed: multiple fragments microscopically representing a tumor proliferation consisting of two cell populations, mononuclear cells, densely cellular and strobe pattern; areas of infarction, haemorrhage areas, rare intratumoral osteoid formation zones; appearance of giant cell tumor. The immunohistochemical examination confirmed the anatomopathological diagnosis adding, therefore, the aggressive character and the local relapse. The oncologist decided that it did not require oncology treatment but only orthopedic treatment. Orthopedic treatment required repeated surgery at intervals of about 5 months apart, caused by tumor recurrence. The first intervention consisted of 1/ 3 distal radius resection and replacement with a graft harvested from the peroneum. Tumor recurrence after 5 months required extirpation of tumor tissue and filling of caries caused in the graft with a fluid bone substitute. Recurrence after another 5 months required removal of the graft that was invaded by the tumor and cubitusmetacarpal arthrodesis fixed with a screw plate. Currently, the patient is undergoing complementary oncology treatment finally initiated by a medical oncologist.
A.M. Bratu, I.A. Sălcianu, A.I. Nicula, C. Zaharia and A.N. Marinescu
Giant cell tumor of soft tissue (GCTST) is usually of synovial origin. It affects synovial membrane, serous bursae, and tendinous tunnels. The most common localizations are in the hands and forearms. Anatomopathological, GCTST is considered as being composed of a cellular fibroblastic stroma in which the tumor cells are distributed. This type of tumor is composed of a mononuclear complex and osteoclast-like giant multinucleated cells, similar to those found in the giant cell tumor at the bone level. Histologically, some authors consider that GCTST is a strictly benign tumor, consisting of welldefined multinucleated histiocytes admixed with eosinophils, lymphocytes and scattered spindleshaped cells, or hemosiderin deposits in its structure, and tumor cells do not have mitosis or atypia. Other authors consider that GCTST is a type of low-grade sarcoma; this entity was named “malignant fibrous histiocytoma, giant cell type” due to the histological similarity with malignant fibrous histiocytoma. The case of a female patient, suspected of giant cell tumor of the brachioradialis tendon sheath was presented. The MRI aspect of this tumor is not the typical one. The MRI examination consisted of a series of sequences, with T1 and T2 weighted images, fat suppression sequence, performed in all three planes, axial, sagittal, and coronal. Also, the examination was performed native, after the administration of intravenous contrast substance, when the 3D multiplanar sequences were performed. The final diagnosis was the post-operative anatomopathological examination, which confirmed that it was a giant cell tumor. We present this case for its less frequent localization - forearm, and the interest it might have in surgical treatment.
M. Popa, Z. Panti, M. Nica, M. Pleniceanu, B. Şerban, R. Ene and C. Cîrstoiu
Introduction. Giant cell tumors of soft tissue (GCTs) have a relatively low incidence and their low prognosis is reserved to local relapses and distant metastases. This type of pathology usually affects adults and the elderly and it is localized in the extremities, most frequently in the thigh. Materials and methods. GCT is a relatively low aggressive tumor; approximately 85% of the patients survive at least 5 years after diagnosis. The risk factors for low prognosis are old age, metastases at the time of diagnosis, local relapse. We conducted this study in the University Emergency Hospital, Bucharest for a period of 3 years, between 01.01.2015 and 01.01.2018, which included 20 patients with ages between 22 and 83 years, of whom 9 were women and 11 were men. Results. Excision with safety margins was performed for all patients. During surgery, tissue samples from 6 different areas were sent for extemporaneous examination. After excision, the histopathological examination was performed and the diagnosis of GCT was established. Localized forms were described in 16 cases; diffuse forms were identified in 4 cases and loco-regional recurrences in 3 cases. Pre or postoperative adjuvant treatment was not applied in any of the cases. Conclusions. GCT is a rare, potentially malignant pathology, in which case evolution is unfavorable. From the clinical and imaging point of view, it is difficult to establish this diagnosis due to the large variety of pathologies it can be mistaken for, making biopsy an essential step within the diagnostic algorithm. Election treatment is represented by local excision with safety margins.
A Maver, G Čuturilo, Stojanović J Ruml and B Peterlin
transgenic mice resulted in stunted growth and neuromuscular deficits [ 15 ]. Somatic mutations in the H3F3 gene were reported in pediatric glioblastoma [ 16 ] and in giantcelltumors of the bone [ 17 ]. Furthermore, it was recently suggested that the pathogenic mechanism of germline histone mutations is distinct from that of the published cancer-associated somatic histone mutations, and may converge on control of cell proliferation [ 18 ].
In our patient, we identified a heterozygous de novo missense variant in the H3F3A gene (NM_002107.4: c. 185 T>G), which is