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.

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.

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.

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.

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.

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.

Giant cell tumors of the skull base: case series and current concepts.

OBJECTIVE: To study the clinical features, tumor characteristics and outcomes of giant cell tumors (GCTs) in the skull base based on long-term follow-up. We also report the largest series of GCTs in the temporal bone and the lateral skull base. MATERIALS AND METHODS: A retrospective study was conducted of all GCTs managed at the Gruppo Otologico, a quaternary referral skull base institute, in Italy from 1993 to 2013. The clinical features, investigations, surgical management and follow-up were recorded. The surgical approaches used were infratemporal fossa approach (ITFA) type B and D and middle cranial fossa (MCF) approaches. RESULTS AND OBSERVATIONS: A total of 7 patients with GCTs of the skull base were treated at our institution. The principal complaints were hearing loss reported in 6 (85.71%) patients, tinnitus in 5 (71.43%) and swelling in 3 (42.9%). Pure-tone audiometry showed conductive hearing loss in 5 (71.43%) patients. High-resolution CT scan and MRI with gadolinium enhancement were done in all patients. Radiology showed involvement of the ITF and middle ear in 6 (85.71%) patients each, temporomandibular joint in 4 (57.14%) patients, invasions of the squamous part of the temporal bone, mastoid, MCF and greater wing of sphenoid in 3 (42.9%) patients each and the petrous bone in 2 (28.6%) patients. ITFA type B was applied as an approach for tumor removal in 5 (71.43%) patients, including a case where an additional MCF approach was employed, and ITFA type D and the transmastoid approach were applied in 1 (14.3%) patient each. Total tumor removal and successful cure was achieved in 6 (85.71%) patients. Subtotal removal leading to recurrence and eventual mortality was the result in 1 (14.3%) patient. CONCLUSIONS: A thorough knowledge of the anatomy of the skull base and the various skull base approaches is necessary to tackle GCTs. ITFA type B and D combined with MCF approaches provide good exposure of the tumor with minimal postoperative sequelae and good locoregional control. Recurrence due to either subtotal removal or suboptimal treatment may have disastrous consequences for the patient.

A multidisciplinary approach to giant cell tumors of tendon sheath and synovium--a critical appraisal of literature and treatment proposal.

Giant cell tumors deriving from synovium are classified into a localized (GCT of tendon sheath; GCT-TS) and diffuse form (diffuse-type GCT, Dt-GCT). We propose a multidisciplinary management based upon a systematic review and authors' opinion. Open excision for GCT-TS and open synovectomy (plus excision) for Dt-GCT is advised to reduce the relatively high recurrence risk. External beam radiotherapy should be considered in severe cases, as Dt-GCT commonly extends extra-articular.

Use of gluteus maximus adipomuscular sliding flaps in the reconstruction of sacral defects after tumor resection.

BACKGROUND: While performing sacrectomy from a posterior approach enables the en bloc resection of sacral tumors, it can result in deep posterior peritoneal defects and postoperative complications. We investigated whether defect reconstruction with gluteus maximus (GLM) adipomuscular sliding flaps would improve patient outcomes. METHODS: Between February 2007 and February 2012, 48 sacrectomies were performed at He Nan Cancer Hospital, Zhengzhou City, China. We retrospectively examined the medical records of each patient to obtain the following information: demographic characteristics, tumor location and pathology, oncological resection, postoperative drainage and complications. Based on the date of the operation, patients were assigned to two groups on the basis of closure type: simple midline closure (group 1) or GLM adipomuscular sliding reconstruction (group 2). RESULTS: We assessed 21 patients in group 1 and 27 in group 2. They did not differ with regards to gender, age, tumor location, pathology or size, or fixation methods. The mean time to last drainage was significantly longer in group 1 compared to group 2 (28.41 days (range 17-43 days) vs. 16.82 days (range 13-21 days, P < 0.05)) and the mean amount of fluid drained was higher (2,370 mL (range 2,000-4,000 mL) vs. 1,733 mL (range 1,500-2,800 mL)). The overall wound infection rate (eight (38.10%) vs. four (14.81%), P < 0.05) and dehiscence rate (four (19.05%)] vs. three (11.11%), P < 0.05) were significantly higher in group 1 than in group 2. The rate of wound margin necrosis was lower in group 1 than in group 2 (two (9.82%) vs. three (11.11%), P < 0.05). CONCLUSIONS: The use of GLM adipomuscular sliding flaps for reconstruction after posterior sacrectomy can significantly reduce the risk of infection and improve outcomes.

Giant cell tumor with secondary aneurysmal bone cyst: a unique presentation with an ossified extraosseous soft tissue mass.

The authors describe a case of giant cell tumor (GCT) with secondary aneurysmal bone cyst (ABC) in a 44-year-old man with chronic, intermittent knee pain. A unique feature is the presentation of GCT with an ossified extraosseous soft tissue mass. Radiograph demonstrates a multiloculated lytic lesion in the distal meta-epiphyseal region of the femur with an adjacent extraosseous soft tissue mass. The soft tissue mass was partially ossified along its margin and internal septa. MRI demonstrates a multiloculated lesion in the distal femur with multiple fluid-fluid levels and cortical penetration of the lesion. Both the intraosseous lesion and extraosseous soft tissue mass have similar MR signal characteristics. At surgery, the intraosseous component was found to be contiguous with the extraosseous soft tissue mass through a cortical perforation. To the best of our knowledge, this is the first case report of GCT with aneurysmal bone cyst initially presenting with an extraosseous soft tissue mass.

Giant-cell-rich pseudotumour in Paget's disease.

Paget's disease (PD) of the bone is a disorder of bone remodelling that may be polyostotic or monostotic. Although development of a sarcoma in PD is well-recognised, it is less well recognised that pseudosarcomas in bone and soft tissue can also arise in this condition. In this report we document the case of a large giant-cell-rich pseudotumour that developed in the tibia and overlying soft tissues in a case of polyostotic PD. Bone and soft tissues were highly vascular and contained abundant haemorrhage with focal areas of new bone formation and a diffuse infiltrate of osteoclastic giant cells. The lesion has not recurred or produced metastases 3 years after removal. Clinicians should be aware that a benign giant-cell-rich pseudotumour can develop in PD and that it needs to be distinguished from other giant-cell-rich tumours.

Spinal giant cell tumor in tuberous sclerosis: case report and review of the literature.

BACKGROUND: Patients affected by tuberous sclerosis (TS) have a greater incidence of tumors than the healthy population. Spinal tumours in TS are reported very rarely and consist mainly of sacrococcygeal and cervical chordomas. METHOD: Case report. FINDINGS: A 21-year-old man, affected by TS, presented a spinal dorsal T2 tumor that caused medullary compression. He underwent decompressive laminectomy and microsurgical excision of a giant cell tumor and an associated aneurysmal bone cyst. Postoperative hypofractionated radiotherapy was performed on the surgical field. At 2.4 years of follow-up the patient reported total recovery of neurological deficits and was free from tumor recurrence. CONCLUSION: Considering this association, which is the first reported in the literature, spinal magnetic resonance imaging with gadolinium should be performed at the onset of spinal pain in patients affected by TS.

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.

Cisplatin-based chemotherapy for pulmonary metastasized germ cell tumors of the testis--be aware of acute respiratory distress syndrome.

BACKGROUND: Cisplatin-based combination chemotherapy is regarded as standard of care for patients with advanced germ cell tumors. In patients with lung metastases and a high tumor load, an association between induction chemotherapy and the development of a 'tumorassociated' acute respiratory distress syndrome (ARDS) has been hypothesized. CASE REPORT: We report the clinical course of a 19-year-old patient who rapidly developed fatal ARDS during the first cycle of chemotherapy using the PEI regimen (cisplatin, etoposide and ifosfamide) for a metastasized (lung, liver, lymph nodes) germ cell tumor of the testis. CONCLUSION: Further clinical research in order to better define risk factors for developing ARDS in this patient population as well as novel strategies for the prevention and treatment of ARDS in those patients are necessary.

Recurrent and refractory giant cell tumor of the jaw.

Recurrent giant cell tumors refractory to various treatment modalities are challenging dilemmas for the most experienced practitioner. We report a case of a multiply recurrent aggressive giant cell tumor diagnosed at 10 months of age with extensive involvement of the maxillae and mandibles unresponsive to multiple therapeutic modalities. The various treatments attempted in this patient including conventional therapies as well as the original use of bevacizumab (Avastin) are described.

Inflammatory processes in cortical tubers and subependymal giant cell tumors of tuberous sclerosis complex.

Cortical tubers and subependymal giant cell tumors (SGCT) are two major cerebral lesions associated with tuberous sclerosis complex (TSC). In the present study, we investigated immunocytochemically the inflammatory cell components and the induction of two major pro-inflammatory pathways (the interleukin (IL)-1beta and complement pathways) in tubers and SGCT resected from TSC patients. All lesions were characterized by the prominent presence of microglial cells expressing class II-antigens (HLA-DR) and, to a lesser extent, the presence of CD68-positive macrophages. We also observed perivascular and parenchymal T lymphocytes (CD3(+)) with a predominance of CD8(+) T-cytotoxic/suppressor lymphoid cells. Activated microglia and reactive astrocytes expressed IL-1beta and its signaling receptor IL-1RI, as well as components of the complement cascade, such as C1q, C3c and C3d. Albumin extravasation, with uptake in astrocytes, was observed in both tubers and SGCT, suggesting that alterations in blood brain barrier permeability are associated with inflammation in TSC-associated lesions. Our findings demonstrate a persistent and complex activation of inflammatory pathways in cortical tubers and SGCT.