Pigmented villonodular synovitis/giant cell tumor in the knee.

PURPOSE OF REVIEW: Pigmented villonodular synovitis (PVNS) is a rare diagnosis in pediatric patients and commonly presents with symptoms of swelling and pain. Early diagnosis is important to prevent secondary degeneration into the subchondral bone. This review will analyze the etiology, clinical signs/symptoms, diagnosis, treatment, and recent literature on PVNS in the pediatric population. RECENT FINDINGS: Many theories of PVNS etiology have been described in the literature; however, an inflammatory response has been most widely accepted. PVNS can occur in any joint, but most commonly in the knee. The most common treatment for PVNS is synovectomy, and long-term follow-up is necessary to detect disease persistence or recurrence. SUMMARY: Although uncommon, PVNS does occur in the pediatric population and this diagnosis should be included in the differential of atraumatic joint swelling and pain.

Arthroscopic Management of Giant Cell Tumor of the Calcaneus.

Giant cell tumor of the calcaneal bone is a very rare entity and generally seen in the 30 to 40 years age group. We report a case of a 17-year-old male with giant cell tumor of the calcaneus, presented with left heel pain without another obvious physical abnormality. Radiographs showed a lobulated, well-defined, lytic lesion of the calcaneus with narrow transitional zone without periosteal reaction, no extraosseal spread, and no lung metastases. Arthroscopic procedure was done directly for both diagnostic and curative procedures. All soft, grayish lesions were completely removed arthroscopically using direct lateral portals and the suspected reactive zones debrided using high-speed burr and injected with corticosteroid. Histopathology confirmed the suspected diagnosis. The postoperative clinical course was uneventful with immediate pain relief and full weight bearing and movement allowed soon. The patient had no recurrent pain as well as recurrent radiographic lesions, and normal joint mobility 9 months postoperatively. Considering the accessibility of the lesion, giant cell tumor of the calcaneal bone can be successfully treated arthroscopically using direct lateral approach.Levels of Evidence: Therapeutic, Level IV: Retrospective, case report.

Cystic Metastasis of a Giant Cell Tumor Causing Recurrent Spontaneous Pneumothorax / Metástasis quística de un tumor de células gigantes causante de neumotórax espontáneo recurrente

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Comparison of outcomes of 2 surgical treatments for proximal humerus giant cell tumors: a multicenter retrospective study.

BACKGROUND: The incidence of giant cell tumors in the proximal humerus is low. We evaluated 2 surgical treatments for giant cell tumors of the proximal humerus and postoperative upper-extremity function. METHODS: This study retrospectively analyzed the clinical data of 27 cases of giant cell tumors of the proximal humerus at 4 Chinese medical centers specializing in bone oncology collected between January 2002 and June 2015. All patients were followed up for more than 2 years. The surgical procedures performed for treatment included curettage in 14 patients and segmental resection in 13. The Campanacci grade, occurrence of pathologic fracture, surgical method, complications, and Musculoskeletal Tumor Society score were recorded for each cohort. RESULTS: The recurrence rate was 7.1% in the curettage group and 15.4% in the segmental resection group. Other postoperative complications occurred in 4 patients with segmental resection, including resorption of the osteoarticular allograft in 2, subluxation of the glenohumeral joint in 1, and prosthetic loosening and exposure in 1. A significant difference in postoperative upper-extremity function was noted between the 2 groups (P < .001). CONCLUSIONS: Postoperative upper-extremity function in the curettage group was significantly better than that in the segmental resection group. Segmental resection and reconstruction with a large segmental osteoarticular allograft were considered unadvisable. We suggest that extensive curettage should be selected to treat proximal humerus giant cell tumors as much as possible.

Chondroblastoma-like primary malignant giant cell tumor of the humerus - a case report.

35-year-old woman suffered prolonged pain in the left shoulder, where an aggressively growing tumor of the proximal humerus was revealed thereafter. The lesion caused massive osteolysis of the metaepiphysis with cortical disruption, but no soft tissue extension was evident. Given the unsatisfactory effect, the ongoing neoadjuvant chemotherapy was prematurely ceased and the resection 13 cm long segment of bone with modular prosthesis replacement followed. Histologically, clear-cut malignant tumor with both the presence of numerous reactive osteoclast-like giant cells and geographic structural deposition of chondroid matrix bore a close resemblance to chondroblastoma. Dominant cellular composition formed solid mosaic clusters of large, atypical, frequently binucleated cells with voluminous eosinophilic cytoplasm. Impressive nuclear pleomorphism was accentuated by both the grooving and atypical mitotic figures. Thorough sampling disclosed limited, but sharply contrasting parts, where biphasic arrangement of small uniform stromal elements together with regularly distributed, reactive osteoclasts suggested putative precursor giant cell lesion. Except the osteoclasts, all matrical and stromal cells were strongly SOX9 and D2-40 positive; in contrary desmin, SATB2, S100 and p63 yielded completely negative results. Detected H3F3A c.103G>T mutation in exon 2 finally established true nature of that peculiar neoplastic proliferation and lead to descriptive term of primary chondroblastoma-like malignant giant cell tumor. In the setting of all the microscopic variability, histogenesis and complex differential diagnosis of skeletal (malignant) giant cell lesions, there are discussed e.g. aggressive/malignant chondroblastoma, chondroblastoma-like osteosarcoma or giant cell-rich osteosarcoma and practical impact of specific mutational analysis results as well.

Arthroscopic Excision of Tendinous Giant Cell Tumors Causing Locking in the Knee Joint.

PURPOSE OF THE STUDY Non-osseous giant cell tumors are locally aggressive tumors arising around joints. They are commonly located around synovial joints such as wrist and knee and occasionally cause mechanical symptoms. MATERIAL AND METHODS This retrospective case series includes 7 patients operated due to intraarticular lesion. The mean age of the patients was 28.7 (range 22-37) years. Mean follow-up period was 12 months. RESULTS All patients underwent arthroscopic debridement. They were followed monthly with clinical examination and magnetic resonance imaging (MRI) was obtained at third month for all patients. Patients were contacted through phone call and evaluated with the WOMAC score retrospectively. No recurrence was detected in any patient. CONCLUSIONS Arthroscopic debridement is a safe surgical technique that may replace open surgery in the treatment of intraarticular tendinous giant cell tumors. Key words:tendinous giant cell tumor, arthroscopy, knee locking.

Recurrence of Giant Cell Tumor of The Larynx.

Giant cell tumor of the larynx is a rare tumor. It was first reported by Wessely et al in 1940. Thirty-nine cases have been reported until now and together with the current case 2 recurrences were encountered. In this case report, our aim was to discuss conservative management because of the suspicion of recurrence. A 70-year-old male patient was admitted to our clinic with the complaint of hoarseness. A tumor measuring 1 × 1 cm located in the anterior half right vocal fold and extending to the anterior comissure was found on laryngeal endoscopy. Direct laryngoscopy and biopsy of the mass revealed giant cell tumor on histopathological examination. Tumor resection with cordectomy through laryngofissure and subsequently medialization thyroplasty were performed. Horaseness of the patient improved. On 2-year follow-up, a tumoral lesion suggesting recurrence was found on the vocal cord. Direct laryngoscopy and biopsy confirmed recurrence. Total laryngectomy was performed. This is the second case of recurrent giant cell tumor of the larynx. The therapy of choice should be selected considering the possibility of recurrence.

Tumor-induced Osteomalacia in a 3-Year-Old With Unresectable Central Giant Cell Lesions.

Tumor-induced osteomalacia (TIO) is a rare cause of hypophosphatemia involving overproduction of fibroblast growth factor 23. TIO has been described largely in adults with small mesenchymal tumors. We report a case of TIO in a child who presented with knee pain and radiographic findings concerning for rickets, and was found to have maxillomandibular giant cell lesions. The patient was treated with oral phosphorus and calcitriol, surgical debulking, and intralesional corticosteroids, which resulted in tumor regression and normalization of serum fibroblast growth factor 23 and phosphorus. This case illustrates the occurrence of this rare paraneoplastic syndrome in children and adds to our knowledge about clinical manifestations and pathologic findings associated with pediatric TIO.

Multifocal tenosynovial giant cell tumors in a child with Noonan syndrome.

Noonan syndrome is a genetic disorder with variable expression of distinctive facial features, webbed neck, chest deformity, short stature, cryptorchidism and congenital heart disease. The association of Noonan syndrome and giant cell granulomas of the mandible is widely reported. However, Noonan syndrome may also be associated with single or multifocal tenosynovial giant cell tumors, also referred to as pigmented villonodular synovitis. We report a child with Noonan syndrome, giant cell granulomas of the mandible and synovial and tenosynovial giant cell tumors involving multiple joints and tendon sheaths who was initially misdiagnosed with juvenile idiopathic arthritis. It is important for radiologists to be aware of the association of Noonan syndrome and multifocal giant cell lesions, which can range from the more commonly described giant cell granulomas of the mandible to isolated or multifocal intra- or extra-articular tenosynovial giant cell tumors or a combination of all of these lesions.

Primary pulmonary giant cell tumor: 18F-FDG PET/CT imaging / Tumor de células gigantes pulmonar primario: imágenes 18F-FDG PET/CT

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Tratamiento de tumores de células gigantes / Traitement des tumeurs à cellules géantes / Treatment of Giant Cell Tumors

Introducción: el tumor de células gigantes se presenta en adultos jóvenes, y su capacidad metastásica obliga a realizar un tratamiento quirúrgico, aunque su tasa de recurrencias hace que no haya consenso respecto a la conducta terapéutica. Método: 23 pacientes fueron intervenidos en el periodo de 1996 a 2012 en el hospital universitario Miguel Servet, a los que se les realizó un seguimiento medio de 8,9 años. Resultados: el resultado funcional fue satisfactorio en todos los casos, pues todos los pacientes pudieron reanudar su actividad física habitual. Se detectaron 6 pacientes con recurrencias que fueron tratadas quirúrgicamente; estos pacientes también tuvieron una recuperación total. Un paciente falleció a causa de metástasis pulmonares. Conclusiones: el tratamiento quirúrgico es el más indicado en tumores de células gigantes, se lograron buenos resultados tanto en el tratamiento del tumor primario como de las recidivas(AU)

Introduction: giant cell tumor occurs in young adults, and their metastatic capacity requires a surgical treatment, although the recurrence rate makes no consensus on the therapeutic approach. Method: twenty-three patients were operated from 1996 to 2012 at Miguel Servet University Hospital; they underwent an 8.9 year-mean follow up. Results: the functional result was satisfactory in all cases, for all patients were able to return their usual physical activity. Six patients with recurrences were surgically treated; these patients also had a full recovery. One patient died of pulmonary metastases. Conclusions: surgical treatment is the most suitable approach in giant cell tumors, good results were achieved both in the treatment of primary tumor as well as of recurrence(AU)

Introduction: La tumeur à cellules géantes se rencontre chez des jeunes adultes, et sa capacité de métastase impose un traitement chirurgical, quoique son taux de récurrence empêche qu'il n'y ait pas de consensus sur la conduite thérapeutique à suivre. Méthodes: Dans la période 1996-2012, vingt-trois patients ont été traités chirurgicalement à l'hôpital universitaire Miguel Servet, et ils ont été suivis pendant une période moyenne de 8,9 ans. Résultats: Il y a eu des résultats satisfaisants en ce qui concerne la fonction, parce que tous les patients ont pu reprendre leurs activités quotidiennes. Dans quelques cas (6 patients), on a détecté des récurrences qui ont été traitées chirurgicalement. La récupération de ces patients a été totale, mais un patient est décédé à cause d'une métastase pulmonaire. Conclusions: Le traitement chirurgical est la conduite la plus appropriée dans les cas de tumeur à cellules géantes. On a obtenu de bons résultats dans le traitement de la tumeur primaire et des récidives(AU)

Pediatric giant cell tumor of the tendon sheath of the craniocervical junction involving the occipital condyle.

INTRODUCTION: Giant cell tumor of the tendon sheath (GCTTS), also called pigmented villonodular synovitis, is a common lesion of the synovial membrane of the hand joint, but it uncommonly involves the axial skeleton, especially in pediatric populations. Furthermore, GCTTS originating from the occipital condyle has not been reported previously. CASE REPORT: A 15-year-old girl presented with a palpable neck mass for 1 year, and imaging studies revealed a less demarcated and heterogeneously enhanced mass in the suboccipital region. The tumor was originating from the occipital condyle that eroded the skull and atlas, and it was completely resected via a far lateral transcondylar approach followed by transarticular screw fixation. After the resection, we performed occipitocervical fusion to prevent spinal instability. The patient made an uneventful recovery after surgery. Recurrence has not been observed after 5 years of follow-up. DISCUSSION: We report this rare case and briefly review the general features and unusual locations of GCTTS with recommendations for treatment modalities.

Primary hyperparathyroidism associated with a giant cell tumor: One case in the distal radius.

Hyperparathyroidism can present itself as brown tumors (or osteolytic expansive lesions) that usually disappear after normalization of calcium and phosphate levels. It rarely occurs simultaneously with a giant cell tumor. The authors report one case of a localized form at the distal radius in a patient being followed for primary hyperparathyroidism. The diagnostic challenges related to the clinical and radiological similarities of these two pathological entities are discussed, as they can lead to delays in therapeutic management.

Giant Cell Tumor of the Patella Tendon Sheath Presenting as a Painful Locked Knee.

BACKGROUND: The giant cell tumor of the tendon sheath (GCT-TS) is a benign proliferative synovial tumor manifesting as an intra-articular solitary nodule. When it involves the infrapatellar fat pad it can present acutely as a painful locked knee. CASE REPORT: A 26-year-old white male presented with a 2-week history of painful locking in his right knee. Clinical examination revealed lack of extension by approximately 20°. To help establish the diagnosis, an MRI scan of the right knee was performed, showing a large (5×4×2 cm), oval, well-circumscribed mass with a low-intensity homogenous signal. The size of the mass prohibited the removal by arthroscopy and we therefore proceeded with an open arthrotomy. Histological examination showed a tendosynovial giant cell tumor of the patella tendon sheath. At the latest follow-up, 2 years postoperatively, there was no local tumor recurrence. CONCLUSIONS: These rare tumorous lesions should be included in the differential diagnosis of painful locking knee, especially in the absence of definite traumatic history.

Quantitative analysis of the concentrations of IGFs and several IGF-binding proteins in a large fibrous abdominal tumor and the circulation of a patient with hypoglycemia.

The syndrome of nonislet cell tumor induced hypoglycemia (NICTH) represent extreme cases of excessive expression and production of incompletely processed high-molecular-mass pro-IGF-II forms (big IGF-II) by an often large tumor. Tumor-derived big IGF-II is responsible for enhanced insulin-like effects in the body through complicated mechanisms, leading to hypoglycemia. Case studies on NICTH usually focus on measurements of diagnostic parameters in the circulation of patients. Some studies have also reported on qualitative immunohistochemical analysis of tumor tissue, in particular with respect to the expression of IGF-II at the mRNA or protein level. However, quantitative data on the concentrations of IGFs and IGFBPs in tumor specimen causing NICTH, in relation to their corresponding plasma levels are lacking. Such an analysis would provide an estimate of the total potential of (big) IGF-II retained by the tumor and more insight in the relative levels of different IGFBPs and their origin in the circulation, that is, systemically induced by tumor related factors or directly tumor-derived. Here we investigated quantitatively the levels of IGFs and IGFBPs in a large, 1.76 kg weighing, solitary fibrous tumor from a typical case of NICTH using highly specific immunometric assays. Besides a high level of big IGF-II, patient's plasma also contained increased levels of both IGFBP-2 and -6 which declined after removal of the tumor. These IGFBPs have a higher affinity for (pro-) IGF-II than IGF-I and exhibit intrinsic IGF-independent bioactivities. Tumor tissue contained high amounts of big IGF-II and IGFBP-6, exceeding that in patient's circulation many-fold. A relatively low tumor content of IGFBP-2 was found suggesting that the preoperative high levels in plasma were attributable to systemic mechanisms. The background literature and possible implications of these findings are briefly discussed. Based on the present results we postulate that tumor tissue is not the source of the elevated levels of IGFBP-2 often seen in NICTH patients. Large tumors that cause NICTH can produce IGFBP-6 leading to enhanced levels of this IGFBP in the circulation. Hence, the measurement of IGFBP-6 in plasma may serve as an additional marker of this disease pattern.

A Case of Mediastinal Granular Cell Tumor with Horner's Syndrome.

Granular cell tumor (GCT) is found in various organs but is rare in the mediastinum. We report a case of mediastinal GCT in a 19-year-old woman who presented with left ptosis and miosis. CT and MRI revealed a 29-mm well-circumscribed tumor located close to the first thoracic vertebra with features suggesting a neurogenic tumor. The tumor was completely excised using single-port video-assisted thoracoscopic surgery. Histopathological and immunohistochemical analysis revealed that the tumor was a benign GCT. Postoperatively, left ptosis and miosis had improved slightly. To our knowledge, this is the first report regarding mediastinal GCT presenting with preoperative Horner's syndrome.

Giant cell tumor of the sixth thoracic vertebra: case report.

Giant cell tumor is an uncommon but most aggressive benign tumour of the spine with unpredictable outcome and challenging treatment. Spinal giant cell tumors located above the sacrum are rare and treatment recommendations are still unclear. We report a rare case of this lesion in an adult and discuss the management and outcome of such uncommon tumors. A 31-year-old woman presented with progressive motor weakness of both lower limbs with back pain during the past month, associated with sphincter disturbances for the past two days. She was diagnosed with a lytic heterogeneously enhancing mass depending mainly on the T6 posterior arch with small vertebral body involvement. The tumor extent reached surrounding soft tissue and the spinal canal with marked spinal cord compression. A posterior approach was realized as an emergency. Histological examination showed evidence of a giant cell tumor and a complementary irradiation was used. The patient improved well post operatively. There was no recurrence or metastasis over 5 years of follow-up.

Clinical characteristics and evolution of giant cell tumor occurring in Paget's disease of bone.

Patients with Paget's bone disease (PDB) have an increased risk of developing giant cell tumor (GCT). This study was performed to evaluate the clinical characteristics and evolution of GCT complicating PDB and to compare these clinical characteristics to those observed in two large PDB cohorts, the PDB Italian Registry and the United Kingdom's Multi-Centre Randomised Controlled Trial of Symptomatic Versus Intensive Bisphosphonate Therapy for Paget's Disease (PRISM) study. A systematic literature review identified 117 cases of PDB complicated by GCT (PDB-GCT), which involved the skeletal sites affected by PDB (110 patients) or the extraskeletal tissues adjacent to affected bones (7 patients). In contrast to what previously reported for GCT patients without GCT patients (83.2%) were white and one-fourth of them (24.8%) had multifocal GCTs. Compared to PDB patients without GCT, PDB-GCT patients showed a higher male/female ratio (2.1 versus 1.2) and more severe disease (age at PDB onset 52.1 ± 12.1 versus 63.3 ± 10.6 years; number of affected sites 6.1 ± 2.9 versus 2.34 ± 1.6; prevalence of polyostotic PDB 93.3% versus 60.6%). The mortality rate of PDB-GCT patients was higher than those occurring in GCT patients without PDB (about 50% versus 0% to 5% at 5 years) or in PDB patients without GCT (log rank = 29.002). Moreover, up to 98% of PDB-GCT cases had elevated total alkaline phosphatase levels at neoplasm diagnosis, suggestive of active PDB. Importantly, PDB-GCT patients from Southern Italy (45.6% of all GCT patients) showed a higher prevalence of multifocal GCT (51.7%) and of positive familial history for PDB (70.8%) and GCT (65.0%). Finally, indirect evidence suggests a decline in the incidence of GCT in PDB patients. The occurrence of GCT in PDB patients is associated with severe disease and reduced life expectancy of affected patients. The increased prevalence of familial diseases in PDB-GCT patients from Southern Italy suggests a founder effect. The observed changes over time in the incidence of GCT in PDB patients could be related to improved clinical management and/or living conditions of patients.