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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 of the bone (GCTB) is a common primary benign tumor, but in some cases, it behaves aggressively, resulting in tumor recurrence. The standard treatment for GCT is thorough curettage with adjuvant treatment such as phenol, liquid nitrogen, high-speed burr, or methylmethacrylate cement. This article presents the case of a 30-year-old male with GCT of the right distal femur, which demonstrated a complete necrosis of GCTB. Interestingly, the specimen also showed adipocytic lineage, and strong expression of apoptotic markers by [terminal deoxynucleotidyl-transferase dUTP nick-end labelling (TUNEL) and caspase-3] and peroxisome proliferator-activated receptor gamma (PPARγ). To the Authors' knowledge, this is the first reported case of complete necrosis of GCTB concurrent with adipocytic lineage and high expression of PPARγ. PPARγ is a master regulator of fat differentiation. PPARγ possesses antitumor activity through suppression of tumor proliferation and invasion and induction of differentiation and apoptosis. Although we could not conclude on the exact cause of complete necrosis and high expression of PPARγ in this case, we focused on the medical history, where this patient took zaltoprofen (240 mg/day) for four weeks before the biopsy to alleviate his pain. Zaltoprofen is a propionic-acid derivative non-steroidal anti-inflammatory drug, and it is reported to act as a direct ligand for PPARγ. We speculated that one of the possible mechanisms of PPARγ activation in this case was induction by zaltoprofen, at least in part. Although further analysis using cultured tumor cells with ligands specific to the receptor is necessary, PPARγ may be a novel therapeutic target in GCTB.

Giant cell tumor of the tendon sheath (GCTTS) is one of the most common benign soft tissue tumors in the extremities, and is frequently associated with bone abnormalities. Although extrinsic bone erosion is the most common bone abnormality associated with GCTTS, intraosseous invasion of GCTTS occurs rarely and may mimic a primary bone tumor, making diagnosis more challenging. We report an unusual imaging presentation of GCTTS mimicking a primary intramedullary metatarsal tumor and review the literature on the frequency of bone involvement of GCTTS.

Osteoclast like-giant cell tumor of the salivary gland is an extremely rare tumor with distinct pathological features and unknown histogenesis. The neoplastic nature of these tumors in itself is questionable. We present the twentieth case in English literature of primary osteoclast like-giant cell tumor with accompanying low to intermediate grade salivary duct carcinoma of parotid gland, metastasizing to the ipsilateral cervical lymph node. As far as we know this is the second case with lymph node metastasis. Due to the rarity of the tumor its exact biological course is uncertain. We present and discuss this rare case with special emphasis on the histology, immunohistochemistry, and histogenesis.

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., (Copyright © 2012 Wiley Periodicals, Inc.)

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.

Background: Giant cell tumor (GCT) of the mobile spine is a benign tumor, but it can be potentially aggressive. There is not much published information on GCT of the mobile spine as a result of rarity of the disease, and there are controversies over prognostic factors of the condition., Methods: A retrospective analysis of GCT of the mobile spine was performed by survival analysis. Recurrence-free survival (RFS) was defined as the interval between the date of surgery and the date of recurrence. The postoperative RFS rate was estimated by the Kaplan-Meier method. Factors with P values of ≤0.1 were subjected to multivariate analysis for RFS by proportional hazard analysis. P values of ≤0.5 were considered statistically significant., Results: A total of 102 patients with GCT of the mobile spine were included in the study. The mean follow-up period was 39.9 (median 26.0, range 2-153) months. Thirty-eight patients developed recurrence. The univariate and multivariate analysis suggested that age less than 40 years, total spondylectomy either by en bloc or piecemeal method, and administration of bisphosphonate were independent favorable prognostic factors. Subgroup analysis by excluding patients before the year 2000 further confirmed our findings., Conclusions: The removal of the entire osseous compartment either by en bloc or piecemeal method in combination with the long-term use of bisphosphonate could significantly reduce the recurrence rate of GCT of the mobile spine. Age less than 40 years is a favorable prognostic factor for GCT in the mobile spine.

Diffuse-type giant cell tumour (DTGCT) is a benign but destructive proliferative disorder of the synovium of joint capsules, tendons and bursae. Lesions originating from the articular synovium may grow intra-articularly or expand widely extra-articularly. Rare cases may develop only in peri-articular tissues. Temporomandibular joint (TMJ) involvement is exceptional and only two cases of purely extra-articular TMJ-DTGCT have been reported. The authors report an additional case of extra-articular TMJ-DTGCT. The clinical features, histopathological characteristics, imaging modalities and diagnostic methods for DTGCT are discussed together with a literature review based on the clinical aspects of TMJ involvement., (Copyright © 2012 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)

We report of a benign, recurrent tendon sheath tumour. Histologically, three different morphological components were observed., (© Georg Thieme Verlag KG Stuttgart · New York.)

Recognition of the different types of multinucleated giant cells in neoplastic and pseudotumoral lesions of the skin may be helpful in the differential diagnosis of these tumors. In this review, we will analyze the different types of multinucleated giant cells that can be found in nonepithelial cutaneous tumors and, more importantly, the clinicopathological context in which they are found. Touton giant cells are typically present in juvenile xanthogranuloma, necrobiotic xanthogranuloma, and some subtypes of xanthomas. Giant cells with a ground glass appearance are typically present in the solitary reticulohistiocytoma and multicentric reticulohistiocytosis. Osteoclast-like cells are found in giant cell tumors (GCT) of soft parts, plexiform fibrohistiocytic tumor, and atypical fibroxanthoma. Floret-like cells are present in giant cell fibroblastoma, pleomorphic lipoma, multinucleate cell angiohistiocytoma, and giant cell collagenoma., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Purpose: To document tumor type, biological/clinical behavior, management, and outcomes in children with primary jaw tumors., Materials and Methods: A retrospective analysis of children with primary benign jaw tumors evaluated at Massachusetts General Hospital and Children's Hospital Boston from 1991 to 2009 was conducted. Patients were included if they were aged 16 years or younger and had adequate records and follow-up. Patient charts, radiographs, and pathology reports were reviewed. Demographic data; clinical, radiographic, and histopathologic findings; treatment; and outcomes were recorded. Predictor variables were tumor type, clinical behavior (nonaggressive/aggressive), and treatment. Outcome variables included presence or absence of recurrence and complications. Descriptive statistics were computed., Results: There were 102 patients (44 male and 58 female patients) with a mean age of 8.3 years (range, 6 months to 16 years). Tumors were grouped by tumor type: mesenchymal (n=96), neurogenic (n=5), vascular (n=5), or hematopoietic (n=3); in addition, when appropriate, they were classified as nonaggressive (n=54) or aggressive (n=27). Treatment was based on the tumor's clinical/biological behavior and radiographic features and whether it was solitary or multifocal. Patients with nonaggressive tumors were treated by enucleation, debulking/contouring, or observation, and the recurrence rate was 0%. Aggressive tumors underwent en bloc resection or enucleation with systemic adjuvant therapy, and the recurrence rate was 7.1%. Mean follow-up was 2.4 years., Conclusions: The results of this study indicate that primary jaw tumors in children exhibit variable biological/clinical behavior, often not predicted by descriptive histologic findings. Management of these tumors should therefore be guided by clinical/biological behavior., (Copyright © 2013 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.)

Context: Bone giant cell tumors (GCTs) are among the most common benign bone tumors and affect mostly young patients. They represent a rare etiology of head and neck cancer., Objective: We report the case of a 38-yr-old male with a GCT of the thyroid cartilage, initially treated as a thyroid cancer., Case Illustration: The patient had incomplete initial surgery, and a substantial tumor residue was observed at postoperative morphological evaluation. Given the potential risks associated with complete definitive surgery and recent data supporting the use of the receptor activator of nuclear factor κB ligand inhibitor, we proposed treatment with denosumab. Three months after initiating denosumab, computed tomography scan imaging showed a significant modification of the lesion with several calcifications. The patient underwent partial laryngectomy, and examination of the surgical specimen revealed a complete histological response., Results: A review of the literature was conducted to identify previous studies pertaining to GCTs, focusing on reports related to their management., Conclusion: Denosumab emerges as a new treatment for patients with GCTs. Additional clinical trial data are needed to establish the real efficacy and long-term safety of this treatment for the management of GCT.

Giant cell tumor of tendon sheath (GCT-TS) in the foot and ankle has been reported as rare. The purpose of the present study was to describe 20 cases of GCT-TS in the foot and ankle treated in the orthopedic department of Huashan Hospital. From January 2007 to January 2012, a retrospective review was performed of 20 cases of GCT-TS in the foot and ankle, all of which were treated by local excision, including both arthroscopy and local excision. The follow-up of all cases was 3 months to 5 years. Of the 20 patients, 14 were female and 6 were male, with a mean age of 38.7 (range 15 to 59) years. Seventeen presented with a painless, solitary, well-defined soft tissue mass with good or poor mobility. Three had discomfort on weightbearing, because the mass was on the plantar aspect of the foot. One patient had neurologic symptoms. A correct preoperative diagnosis was made in only 5 cases. Magnetic resonance imaging proved to be the most useful noninvasive preoperative investigation, because GCT-TS has a characteristic appearance that allows for planned local excision to be performed. Four cases recurred after surgical excision, for a recurrence rate of 20%. After reviewing the published data and comparing them with our results, we concluded that a definite preoperative diagnosis of GCT-TS in the foot and ankle would help in planning the surgical treatment and that complete local excision is probably the only method to prevent recurrence., (Copyright © 2013 American College of Foot and Ankle Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: An extensive differential diagnosis exists for swelling of the fingers. One of the rare causes of such a swelling is a multisystem disorder called sarcoidosis., Case Description: We describe a 40-year-old female patient who was first seen at our outpatient clinic with a painless swelling of the middle phalanx of her right middle finger. The diagnosis initially made was enchondroma, which was treated conservatively. Thirteen years later, the patient returned with pain and progressive swelling of this same finger. Physical examination and imaging revealed a giant-cell tumour of the tendon sheath, which was excised. The diagnosis of sarcoidosis was made after histological analysis., Conclusion: The initial manifestation of sarcoidosis is rarely observed in bone and soft tissue. For this reason, symptoms arising from such tissues will not always lead to the consideration of this diagnosis. The typical radiological abnormalities of bone combined with swelling of soft tissue should, however, give rise to the consideration of this diagnosis.

Tenosynovial giant cell tumors are benign tumors that are often localized on the palmar sites of the hand. The involvement of large joints such as the knee and ankle are rare. We present an 18-year-old male patient referred to the orthopedics clinic with a mass on his right ankle. No differential diagnosis could be made radiologically. The marginally excised lesion was histopathologically diagnosed as a tenosynovial giant cell tumor. There was no local recurrence during a follow-up of 12 months.

Objective: Giant cell tumors of the maxillofacial skeleton are uncommon, they are usually late manifestation of primary hyperparathyroidism. A series of five clinical cases in four women and one man presenting as the giant cell lesions in the maxilla and/or mandible are discussed., Methods: Biopsy of the lesions, biochemical and hormonal analyses, densitometry and parathyroid scintigraphy were carried out., Results: Biopsy of the lesions showed giant cell tumor of bone. The medical history and laboratory analyses showed primary hyperparathyroidism. Bone density loss was documented and scintigraphy revealed the presence of parathyroid adenomas in four cases. Surgical treatment of hyperparathyroidism, and in the second step - after 6-12 months - the subsequent excision of residual brown tumors in all cases was performed., Conclusions: One should have in mind that osteolytic bone lesions may be due to metabolic disease of the bone. Accurate diagnosis enabling the proper treatment should be carried out, avoiding unnecessary harm to the patients.

Giant cell tumor is colonized by aneurismal bone cyst in only 15% of cases and cervical localisation accounts for less than 1% of giant cell tumors. We are reporting a rare case of a C2 hypervascularized giant cell tumor colonized by an aneurismal bone cyst treated with an effective preoperative Onyx embolization followed by a full tumor resection. The patient experienced a moderate cervical spine injury 2 months prior admission followed by a progressive stiff neck and cervicalgia. CT and MRI identified a lytic lesion of the body and lateral masses of the C2 with encasement of both vertebral arteries. The angiography showed a hypervascularization of the lesion from the vertebral and external carotid arteries as well as a thrombosis of the V3 segment of the right vertebral artery at the C1 level. A posterior occipito-C3/C4 fixation and a tumor biopsy were performed. Histopathological examination concluded to a giant cell tumor colonized by an aneurismal bone cyst. Three weeks later, the patient developed a right upper extremity deficit. The MRI showed an increased C1-C2 stenosis and an increase of the hypervascularization. Three sessions of embolization by the onyx were performed. During surgery a near total tumor devascularisation was observed and a complete resection of the tumor was achieved through an anterolateral approach. Reconstruction consisted of a cementoplasty of the C2 body and odontoïd process with an anterior C3-prosthesis plate. The postoperative course was uneventful., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Objective: To investigate surgical methods and therapeutic effects of giant cell tumor of tendon sheath in finger., Methods: From July 2002 to December 2010,70 patients with giant cell tumor of tendon sheath in finger which confirmed by operation and pathology,were retrospectively analyzed. There were 29 males,41 females with an average of 42 years (ranged, 16 to 61), and the course of disease ranged form 4 months to 6 years (mean 11 months). The method of surgery and anesthesia were observed., Results: All wounds were got stage I healing,no necrosis occurred. Vascular crisis occurred in 6 cases (8.6%), inconformity of diagnosis in 18 cases (25.7%), changing of anesthesia due to situation of tumor in operation in 17 cases (24.3%). The patients were followed up from 2.2 to 10.5 years. Among them, 8 cases (11.4%) recurred, and diagnosied by the second operation without malignant change., Conclusion: The best anesthesia for giant cell tumor in finger should choose brachial plexus to fully expose,complete resection and less harmful damage; while the operation should complete resection at the stage I, and followed up actively, the second operation can be carried out for recorrenced.

Biopsies traditionally are performed under ultrasound (US), computed tomography (CT), or fluoroscopic guidance. In situations in which lesions are difficult to visualize with US or CT guidance, magnetic resonance (MR) imaging often can provide better imaging results. The authors describe a case in which a recurrent calf mass not well visualized under fluoroscopy, CT, or US was identified on MR imaging. In the absence of real-time needle visualization, percutaneous interventions under MR guidance have been limited by prohibitively long imaging times. A novel guidance system providing real-time MR guidance of needle position was used to procure a core biopsy specimen of the lesion., (Copyright © 2012 SIR. Published by Elsevier Inc. All rights reserved.)

Objective: To analyze the manifestation and investigate the value of MRI in the diagnosis of giant cell tumor of tendon sheath (GCTTS)., Methods: Twenty patients with GCTTS proved by operation and pathology were retrospectively analyzed. There were 8 males and 12 females. The average age was 35.5 years, range from 15 to 61 years. All the patients underwent MRI examination., Results: Among the 20 cases, 16 patients had the tumor in knee joint, 2 patients had the tumor in interphalangeal articulation of foot and ankle joint respectively. Nineteen patients had limited tumor and 1 patient had diffuse tumor. The soft tissue mass localized beside lower extremity osteoarticulation was displayed on MRI images. On T1WI, the signal intensities of GCTTS almost equaled to those of skeletal muscle in 15 cases and were slightly lower than those of skeletal muscle in 5 cases. On T2WI, the signal intensities tended to range between those of skeletal muscle and fat in 4 cases, almost equaled to those of skeletal muscle in 13 cases, and were slightly lower than those of skeletal muscle in 3 cases. In the 16 patients with gadolinium-enhanced images on T1WI, 5 patients showed homogeneous enhancement and 11 patients showed inhomogeneous enhancement. Four patients had adjacent bone destruction., Conclusion: The location, shape and inner signal characteristic of GCTTS localized beside lower extremity osteoarticulation could be demonstrated clearly by MRI examination, which is valuable for clinical diagnosis, guiding treatment and follow-up visit.

Purpose: To determine whether the clinical behavior of giant cell lesions (GCLs) or their anatomic location can be differentiated by histologic criteria alone., Materials and Methods: We performed a retrospective study of patients with GCLs treated at Massachusetts General Hospital between 1993 and 2008. Predictor variables were histologic parameters: number of giant cells (GCs) per high-power field, number of nuclei per GC, GC size, stromal cellularity, stromal type, presence of hemorrhage and reactive osteoid, and blinded pathologists' prediction of location and behavior. Outcome variables were clinical behavior (aggressive or nonaggressive) and GCL location, that is, maxillofacial (MF) or axial/appendicular (AA). Descriptive and bivariate statistics were computed with statistical significance set at P ≤ .05., Results: The sample included 88 subjects: 41 MF GCLs (35 aggressive) and 47 AA GCLs (28 aggressive). Aggressive AA lesions had more GCs per high-power field, larger mean GC size, and increased stromal cellularity, and they more frequently had a mononuclear stroma when compared with aggressive MF lesions (P < .05). There were no significant histologic differences between aggressive and nonaggressive MF lesions or between nonaggressive MF and nonaggressive AA lesions. Aggressive AA lesions had more nuclei/GC than nonaggressive AA lesions (P = .03). Using histologic criteria only, blinded pathologists predicted clinical behavior in only 45% of cases (κ = 0.19, P = .09). They predicted a lesion's location in 82% of cases with fair agreement (κ = 0.44, P < .01)., Conclusions: Results of this study indicate that histologic differences between aggressive and nonaggressive GCLs are insufficient for pathologists to differentiate them consistently regardless of location., (Copyright © 2012 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.)

A 38-year-old woman presented with nausea and abdominal distension 15 months after surgical resection of osteoclast-like giant cell tumor in the left lobe of the liver. An 18F-FDG PET/CT scan was performed to evaluate the recurrent tumor. The images demonstrated the recurrent malignancy in the liver. In addition, metastatic lesions in both the abdomen and the chest were revealed.

A 43-year-old woman was admitted for concern of postcurettage recurrence of left sacral giant cell tumor (GCT). Both MRI and FDG PET revealed a large lesion in the left sacrum with characteristics concerning for local recurrence. Preoperative staging CT showed no evidence of remote metastases. Subsequent restaging CT and FDG PET demonstrate the interval development of a 5 × 6-mm pulmonary nodule in the right lung base within 40 days after curettage with hypermetabolic features on PET, concerning for pulmonary metastasis of GCT, which was later confirmed by biopsy. As an exception, benign GCT can demonstrate metastases, rapid growth rate, and enhanced FDG avidity on PET for both primary and metastatic lesions. Our case illustrates the importance of recognizing these exceptional features when interpreting FDG PET of GCT to prevent the misinterpretation of metastases as perhaps other etiologies, such as infection.

Giant cell tumor (GCT) derived stromal cells (GCTSCs) have been identified as the neoplastic cell population of GCTs. Within these stromal cells a subpopulation has been identified that shares several features with mesenchymal stem cells (MSCs) indicating that these neoplastic cells develop from MSCs. Although spontaneous transformations of MSC have already been observed in vitro and in vivo the underlying molecular mechanisms are poorly understood. As microRNAs are crucially involved in tumorigenesis and the modulation of stem cell fate and behavior, they represent promising candidates for the regulation of this process. Therefore, the aim of this study was the comparative analysis of the microRNA expression profiles of GCTSCs and MSCs in order to identify differentially expressed microRNAs and their target genes. We could identify a microRNA signature consisting of 26 differentially expressed microRNAs that perfectly separates these two cell types. One of the microRNAs with the most pronounced differences in expression levels was miR-224. We could confirm the already known regulation of the apoptosis inhibitor API5 by miR-224 and could further identify three novel miR-224 target genes (SMAD5, SLMAP, H3.3B). The involvement of these genes in the regulation of apoptosis resistance, proliferation, differentiation and the regulation of gene transcription suggests pivotal roles of these genes in the neoplastic transformation of MSCs during GCT development., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)

Giant cell tumor of tendon sheath (GCTTS) is a common soft tissue lesion and presents as a firm, slow-growing, non-tender mass adjacent to the tendon sheath. It can be further classified into diffuse or localized types based on its growth pattern. Using cytomorphologic analysis, we assessed the feasibility of fine-needle aspiration (FNA) as an initial diagnostic modality for GCTTS. Forty-one cases of image-guided FNA of GCTTS were retrospectively retrieved from the archives of The Johns Hopkins Hospital. Clinical information, imaging studies, histopathology, and other ancillary studies were also reviewed. The majority of the cytology specimens demonstrated hypercellularity. A uniform population of spindled to polygonal stromal cells constituted more than half of the cellularity. Other cell types frequently present included multinucleated giant cells, hemosiderin-laden macrophages, and foamy histiocytes. Nuclear grooves or intranuclear pseudo-inclusions present in stromal cells were also distinctive cytomorphologic features, but only present in less than a quarter of the cases. Our study has shown the usefulness and accuracy of imaging-guided FNA as a diagnostic tool for GCTTS. Diagnostic accuracy can be optimized with a thorough review of clinical history, careful physical examination, and radiologic correlation., (Copyright © 2012 Wiley Periodicals, Inc.)

Giant cell tumour of tendon sheath is a benign soft tissue lesion most commonly found in the flexor aspect of hand and wrist. Being rare in foot and ankle, the unusual presentation of this lesion may sometimes mimic other lesions like lipoma, synovial sarcoma, malignant fibrous histiocytoma, synovial cyst and ganglion. Hence it is important to include this lesion in differential diagnoses especially if the lesion is found to be anchored to any of the surrounding tendons. This article describes the unusual occurrence of giant cell tumour of the tendon sheath of peroneus brevis which is rarely described in literature.

Spine tumors in the athlete are rare but should be included in the differential diagnosis of any patient in the adolescent or young adult age group with persistent or severe pain. Presenting symptoms are often nonspecific, so a high index of suspicion is needed to diagnose these lesions in a timely fashion. Early recognition and referral to an appropriate multidisciplinary spine tumor center can greatly improve prognosis for these challenging cases.

Giant cell tumour of the tendon sheath (GCTTS) is a slowly progressing benign tumour arising from synovial cells of tendon sheaths. It is one of the most common soft tissue tumours in the hand. We report a retrospective study of 27 proven GCTTS of the hand. The mean length of follow-up was 4.5 years (17 months-8.5 years). Radiographic findings are useful and may prove of great diagnostic value. The positive diagnosis was provided by the pathology examination after complete excision that was performed in all patients. The recurrence was noted in two surgically managed cases. The excision should be meticulous and complete in order to avoid recurrence.

Purpose: Giant cell tumor of the tendon sheath (GCTTS) has a high recurrence after excision and can be a management challenge. Although experience with radiation therapy for GCTTS is limited, it is purported to control infiltrative cases and prevent recurrence. We describe our approach to primary and recurrent GCTTS, as well as our identification of infiltrative cases and their treatment with radiation therapy., Methods: We reviewed 58 patients (32 men and 26 women) with GCTTS in the hand excised at 1 center between 1998 and 2009. Mean age at the time of excision at our center was 50 years. A total of 14 patients who had undergone primary excision at other centers were referred to our center with recurrent disease. Intraoperatively, we found infiltrative disease in 4 patients undergoing primary excision at our center and in 10 of the 14 patients referred to our institution with recurrent disease. All infiltrative (4 primary and 10 recurrent) cases were referred for radiation therapy., Results: Of 14 patients with infiltrative tumors, 10 received radiation therapy (3 patients declined and 1 had a major comorbidity that precluded therapy). Radiation dose was either 35 Gy in 14 fractions or 48 Gy in 24 fractions. At 3.1 years' follow-up, none of the 10 patients treated with radiation therapy had recurrence. No long-term complications were associated with radiation therapy, and hand function was not adversely affected. We identified 4 recurrences, 2 of which were in patients with primary tumors without infiltrative features. Of the 4 patients who were referred for radiation but did not receive it, 2 patients developed recurrence by 2 years after referral., Conclusions: In cases of infiltrative GCTTS, radiation therapy may provide local tumor control with preservation of hand function. Radiation therapy may be particularly helpful when further surgery is not a good option., Type of Study/level of Evidence: Therapeutic IV., (Copyright © 2012 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.)

We report the successful use of a supercharged free fibula for tibial reconstruction and ankle arthrodesis. A 28-year-old woman underwent resection of a giant cell tumor of the distal tibia and reconstruction using a methyl methacrylate cement spacer 12 years prior. The spacer eroded into her ankle joint causing significant pain with ambulation. Therefore, she required ankle arthrodesis but lacked distal tibia bone stock. The ipsilateral fibula was harvested for reconstruction and transferred on its distal blood supply into the bony tibial defect. The proximal blood supply of the fibula flap was then anastomosed to the posterior tibial vessels to supercharge the blood supply. An Ilizarov was placed for external fixation. The combination of a supercharged free fibula and stable external fixation for tibial reconstruction led to timely bony union and ambulation, as well as avoiding the potential complications that can occur with other reconstructive options.

Background: Pigmented villonodular synovitis (PVNS) (also known as diffuse-type giant cell tumor) and tenosynovial giant cell tumors (TGCT) are rare, usually benign neoplasms that affect the synovium and tendon sheaths in young adults. These tumors are driven by the overexpression of colony stimulating factor-1 (CSF1). CSF1 is expressed by a minority of tumor cells, which, in turn attract non-neoplastic inflammatory cells that express CSF1 receptor (CSF1R) through a paracrine effect., Methods: Imatinib mesylate (IM) blocks CSF1R, and previous case reports indicated that it also exerts antitumor activity in PVNS. The authors conducted a multi-institutional retrospective study to assess the activity of IM in patients with locally advanced/metastatic PVNS/TGCT., Results: Twenty-nine patients from 12 institutions in Europe, Australia, and the United States were included. There were 13 men, the median age was 41 years, and the most common site of disease was the knee (n = 17; 59%). Two patients had metastatic disease to the lung and/or bone. Five of 27 evaluable patients had Response Evaluation in Solid Tumor (RECIST) responses (overall response rate, 19%; 1 complete response and 4 partial responses), and 20 of 27 patients (74%) had stable disease. Symptomatic improvement was noted in 16 of 22 patients (73%) who were assessable for symptoms. Despite a high rate of symptomatic improvement and a favorable safety profile, 6 patients discontinued because of toxicity, and 4 patients decided to discontinue IM for no clear medical reason., Conclusions: IM displayed interesting activity in patients with PVNS/TGCT, providing proof of concept for targeting CSF1R in this disease. The authors concluded that the benefits of alleviating morbidity in patients with localized PVNS/TGCT must be balanced against the potential toxicity of chronic drug therapy., (Copyright © 2011 American Cancer Society.)

This article describes a case of a giant cell tumor that expanded into the thoracic cavity and through the spinal canal into the vertebrae. A 36-year-old man presented with a 6-month history of back pain and dyspnea. Plain chest radiographs showed a huge mass accompanied by right pleural effusion. The mass involved the 12th thoracic spine, and the spinal cord was severely compressed. The tumor was resected with a 2-stage procedure. As a first stage to separate the tumor from the anterior vital structures under direct vision, thoracic surgeons performed a right thoracotomy with chest wall reconstruction from the 8th to 11th ribs. The right lung and inferior vena cava were gently retracted, and the tumor was carefully detached from these structures. We were not able to separate the tumor from the right diaphragm due to severe invasion; therefore, we performed partial resection of the right diaphragm with the tumor. After excision of the anterior part of the tumor, the thoracic wall was reconstructed with the right eighth rib and Marlex mesh. When the patient's general condition improved 2 weeks later, spondylectomy by posterior approach was performed. We achieved excision of a giant cell tumor that had expanded into the thoracic cavity and through the spinal canal into the vertebrae. The patient had achieved full rehabilitation with no neurological or respiratory abnormalities at 7 years postoperatively., (Copyright 2012, SLACK Incorporated.)

Objective: Analyze the immunophenotype of the different cells in the various subtypes of giant cell tumor of tendon sheath (GCTS) and investigate the value of clusterin in pathological diagnosis and histogenesis of giant cell tumor of tendon sheath., Methods: A total of 104 cases of GCTS from the surgical pathology files of Shanghai Jiaotong university affiliated the sixth people's hospital were identified. Immunohistochemistry (IHC) for clusterin, desmin, CD163, CD68, p63, p53, Ki-67 and CD35 was performed on all cases, using EnVision technique., Results: All cases of GCTS were researched, including 44 cases of localized type (L-GCTS), 32 cases of diffused type (D-GCTS), 26 cases of pigmented villonodular synovitis (PVNS) and 2 cases of malignant type. There was a slight female predominance in all these subtypes, and the male to female ratio was about 38:66. L-GCTS usually occured within the small joints (90.9%, 40/44), while D-GCTS, PVNS and M-GCTS commonly occured within the large weight-bearing joints [68.8% (22/32), 100% (26/26) and 2/2 respectively]. Of 74 cases with follow-up, the recurrence rates of L-GCTS, D-GCTS, PVNS and M-GCTS respectively were 30.3% (10/33), 30.4% (7/23), 18.8% (3/16) and 2/2. The different subtypes of GCTS had the same cell components, including the large synovial-like mononuclear cells, the small histiocytoid cells, foamy histiocytes cells, inflammatory cells, fibroblasts and the osteoclast-like multinucleated giant cells. There were obvious differences among immunophenotype of the various cell components in GCTS: the large synovial-like mononuclear cells were strong positive for clusterin, partly positive for desmin and Ki-67, and negative for CD163. The small histiocytoid cells were strong positive for CD163 but negative for clusterin and desmin. The osteoclast-like multinucleated giant cells were strong positive for CD68 but negative for clusterin, CD163 and desmin. Normal synoviocytes were strong positive for clusterin, partly positive for desmin. The number of the large synovial-like mononuclear cells that were positive for clusterin in D-GCTS were more than that in L-GCTS (P < 0.01) and PVNS (P < 0.05)., Conclusions: GCTS was synovial tumors, not belonged to the category of fibrohistiocytic lesions. The true tumor cells may be the large synovial-like mononuclear cells, and the number of the cells in the D-GCTS was obviously more than that in L-GCTS and PVNS. This may be the reason that the biological behavior of D-GCTS was more aggressive, destructive and recurrent. Clusterin was an useful marker in pathological differential diagnosis of GCTS.

The following case report was selected from the New Zealand Bone and Soft Tissue Tumour Registry to highlight some key concepts and findings in musculoskeletal imaging with radiological-pathological correlation. The presentation follows a question and answer format followed by clinical information, selected images, diagnosis, discussion and teaching points., (© 2012 The Authors. Journal of Medical Imaging and Radiation Oncology © 2012 The Royal Australian and New Zealand College of Radiologists.)

A 57-year-old man presented with jaundice. Abdominal computed tomography showed a 10-cm left hepatic lobe heterogeneous solid mass with low attenuated areas in the mass, multiple liver metastases and lung metastasis. Serology for hepatitis B and C were negative. Serum alpha-fetoprotein, CEA and CA19-9 were normal. The patient died a few weeks later of progressive liver failure and an autopsy was performed. Histologically, the tumor consisted of sarcomatoid mononuclear cells and osteoclast-like giant cells. The liver tissue surrounding the tumor showed no cirrhotic pattern. The osteoclast-like giant cells were uniformly and strongly immunoreactive with CD68. The mononuclear cells demonstrated expression of vimentin but were negative for CAM5.2. The MIB-1 index was 20% for the mononuclear cells. In conclusion, the histopathological diagnosis revealed an osteoclast-like giant cell tumor of the liver.

Cellular cannibalism refers to a phenomenon where a living cell is phagocytosed into a tumoral cell, where it eventually dies. With the exception of cells in suspension, cellular cannibalism has only been observed with malignant tumors. The finding of occasional images of cannibalism in our daily biopsies of giant cell tumors of the tendon sheath led us to examine this phenomenon further in a retrospective study of 66 cases from our archives. In each case, four morphological features were evaluated: evidence of giant cells, cannibalism, xanthomatous cells, and hemosiderin deposits. Five cases were randomly selected for further immunohistochemical study with the following antibodies: CD68, vimentin, leukocytary common antigen (LCA), Bcl-2 oncoprotein, p53, caspase-3, and Bax. Patients included 35 (53.03%) females and 31 (46.97%) males. Mean age was 50.73 years (range from 14 to 75 years). Giant cells were found in all cases but one (98.48%). Cannibalism was found in 56 cases (84.34%) and this phenomenon was graded as 1 in 35 cases, 2 in 13 cases, and 3 in six cases. The internalized cells frequently appeared apoptotic. Immunohistochemical analysis revealed that the internalized cells as well as the cannibal cells expressed CD68.

Giant cell tumor of the tendon sheath (GCTTS) is a relatively uncommon lesion. GCTTS composed largely epithelioid histiocytes are very rare. In the literature, the author could not find such cases. A 73-year-old man presented with a mass of right thumb, and resection of the mass was performed. Grossly, the mass was encapsulated and yellowish, and measured 1.5 x 2 x 2 cm. Microscopically, the mass was composed of cellular and hypocellular zones. The former was composed of spindle cells and osteoclast-like giant cells, while the latter of epithelioid clear histiocytes. The area of the former was 20%, and the latter 80%. Pigment was seen in the former elements. Mitotic figures were seen in 3/per 30 high power fields (HPFs) in the former element and 2/per 30 HPFs in the latter element. Histochemically, the pigment was hemosiderin positive with Prussian blue staining. Immunohistochemically, both the elements were negative for cytokeratin (CK) CE1/3, CK CAM5.2, CEA, HMB45, alpha-smooth muscle antigen, p53, CD10, TTF-1, and CDX2. Both the elements were positive for CD68 and Ki-67 (cellular element 30% and hypocellular element 20%). The histiocytes of the hypocellular element and osteoclast-like giant cell of the cellular element were positive for CD45. S100-protein positive Langerhans cells and CD45-positive lymphocytes were scattered. The pathological diagnosis was GCTTS. In the author's experience, GCTTS composed largely epithelioid histiocytes are very rare. In the literature, the author could not find such cases. Thus, the author reports herein this case.

Tenosynovial giant cell tumor (TGT) rarely arises from the posterior coloumn of the cervical spine. Most lesions of TGT involve the tendon sheath and joint lining of the small joints of the fingers and hands, and consecutively the knee, ankles and feet, and hips. Rate of extra-articular presentation is about 5-15% in all cases. In this report, a case with paraparesis caused by TGT in the cervico-thoracic junction is presented. The clinical manifestations, diagnosis, and treatment of this unusual condition are discussed. In the treatment of TGT of the vertebral column, the main aim should be total surgical excision of the tumor.

Osteoclastoma-like giant cell tumor of the renal pelvis, similar to the entity more commonly occurring in bone, is very rare, having been reported in twelve previous cases to our knowledge. This is the first report of this entity, to our knowledge, to include its cross-sectional imaging appearance. A hyperdense area within the lesion on non-contrast CT may correspond with extensive hemorrhagic content of the lesion identified histologically. As in most prior cases, an adjacent smaller urothelial carcinoma of the renal pelvis was also identified. In the limited reported cases, this entity has exhibited highly aggressive behavior with poor prognosis., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Objective: To study the relation of the sex, age, location and chemotherapy with recurrence of the tumor., Methods: From January 2000 to August 2010, 47 patients with giant cell tumor of tendon sheath in upper extremity were retrospectively analyzed. Statistical analysis of sex, age at presentation, lesion location, chemical inactivation, surgical complications, tumor recurrence and pathological findings were explored. There were 28 females and 19 males, ranging in age from 17 to 78 years, with an average of 38.15 years. All the patients underwent surgical excision. Fourteen patients received intraoperative chemically inactive treatment. All the patients had routine follow-up to observe the wound healing, pathological findings,tumor recurrence, and received necessary imaging examinations., Results: All the patients were followed up, and the duration ranged from 22 to 129 months, with a mean time of 53.89 months. Four patients who received intraoperative alcohol inactivation appeared wound complications such as wound swelling, discharge of necrotic tissue, delayed wound healing. Fifteen patients had active growth of tumor tissue, 1 patient had low-grade malignant giant cell tumor of tendon sheath. The recurrence rate was significantly higher in the group which preoperative X-ray was found to have bone destruction (P = 0.003); patients receiving chemically inactivation had lower risk of recurrence after surgery than patients not receiving chemically inactivation (P = 0.042)., Conclusion: The recurrence rate of giant cell tumor of tendon sheath in upper limb was closely related to tumor growth site, bone destruction and chemical inactivation. Local excision of giant cell tumor of tendon sheath was the effective treatment. How to identify the patients at high risk of recurrence, how to reduce the recurrence rate and the functional restoration after wide resection are the priorities and difficulties of future researches.

Giant cell tumors of the head and neck are rare. We describe the case of a 50-year-old man who presented with otalgia and eustachian tube dysfunction. After his symptoms persisted despite aggressive medical management, further investigation revealed the presence of a firmness in his left parotid tail. Computed tomography identified a heterogeneously enhancing mass in the left masticator space. The tumor was removed surgically, and it was diagnosed on histopathology as a giant cell tumor. The patient recovered uneventfully and was closely followed for signs of recurrence. To the best of our knowledge, ours is the first reported case of a giant cell tumor in the masticator space. We review the literature on giant cell tumors of the head and neck, with particular attention given to cases involving the temporomandibular joint.

Background: The histologic type of nasopharyngeal carcinoma can be an important factor in the prognosis. The effects, if any, of the degree of cellular pleomorphism is uncertain., Case: In this report, we present a case of nonkeratinizing, undifferentiated nasopharyngeal carcinoma with monstrous tumor giant cells. An extremely high level of aneuploidy and dense cytoplasmic accumulation of Epstein-Barr virus capsid antigen were found in tumor cells. The patient was treated with concomitant chemoradiotherapy., Conclusion: Nine years of follow-up with no recurrences suggests that this extreme morphologic variation may impart no adverse prognostic effect.