Osteoclast-like giant cell tumors of the pancreas and liver

Osteoclast-like giant cell tumors （OGCT） are rare abdominal tumors, which mainly occur in the pancreas. The neoplasms are composed of two distinct cell populations and frequently show an inhomogenous appearance with cystic structures. However, due to the rarity of these tumors, only very limited clinical data are available. Imaging features and sonographic appearance have hardly been characterized. Here we report on two cases of osteoclast-like giant cell tumors, one located within the pancreas, the other within the liver, in which OGCTs are extremely rare. Both patients were investigated by contrast sonography, which demonstrated a complex, partly cystic and strongly vascularized tumor within the head of the pancreas in the first patient and a large, hypervascularized neoplasm with calcifications within the liver in the second patient. The liver OGCT responded well to a combination of carboplatin, etoposide and paclitaxel. With a combination of surgical resection, radiofrequency ablation and chemotherapy, the patient＇s survival is currently more than 15 too, making him the longest survivor with an OGCT of the liver to date.

Thoracic giant cell tumor after two total en bloc spondylectomies including one emergency surgery:A case report

BACKGROUND For patients with acute paraplegia caused by spinal giant cell tumor(GCT)who require emergency decompressive surgery,there is still a lack of relevant reports on surgical options.This study is the first to present the case of an acute paraplegic patient with a thoracic spinal GCT who underwent an emergency total en bloc spondylectomy(TES).Despite tumor recurrence,three-level TES was repeated after denosumab therapy.CASE SUMMARY A 27-year-old female patient who underwent single-level TES in an emergency presented with sudden severe back pain and acute paraplegia due to a thoracic spinal tumor.After emergency TES,the patient's spinal cord function recovered,and permanent paralysis was avoided.The postoperative histopathological examination revealed that the excised neoplasm was a rare GCT.Unfortunately,the tumor recurred 9 months after the first surgery.After 12 months of denosumab therapy,the tumor size was reduced,and tumor calcification.To prevent recurrent tumor progression and provide a possible cure,a three-level TES was performed again.The patient returned to an active lifestyle 1 month after the second surgery,and no recurrence of GCT was found at the last follow-up.CONCLUSION This patient with acute paraplegia underwent TES twice,including once in an emergency,and achieved good therapeutic results.TES in emergency surgery is feasible and safe when conditions permit;however,it may increase the risk of tumor recurrence.

Multimodal imaging in the diagnosis of bone giant cell tumors:A retrospective study

BACKGROUND Giant cell tumor of bone is a locally aggressive and rarely metastasizing tumor,and also a potential malignant tumor that may develop into a primary malignant giant cell tumor.AIM To evaluate the role of multimodal imaging in the diagnosis of giant cell tumors of bone.METHODS The data of 32 patients with giant cell tumor of bone confirmed by core-needle biopsy or surgical pathology at our hospital between March 2018 and March 2023 were retrospectively selected.All the patients with giant cell tumors of the bone were examined by X-ray,computed tomography(CT)and magnetic resonance imaging(MRI),and 7 of them were examined by positron emission tomography(PET)-CT.RESULTS X-ray imaging can provide overall information on giant cell tumor lesions.CT and MRI can reveal the characteristics of the internal structure of the tumor as well as the adjacent relationships of the tumor,and these methods have unique advantages for diagnosing tumors and determining the scope of surgery.PET-CT can detect small lesions and is highly valuable for identifying benign and malignant tumors to aid in the early diagnosis of metastasis.CONCLUSION Multimodal imaging plays an important role in the diagnosis of giant cell tumor of bone and can provide a reference for the treatment of giant cell tumors.

Management of a rare giant cell tumor of the distal fibula: A case report

BACKGROUND Aggressive giant cell tumor of the distal fibula is so rare that no consensus on a surgical strategy has been reached.Thus,an appropriate treatment strategy is still important to discuss.CASE SUMMARY A 61-year-old man who had been experiencing progressive swelling of the left lateral malleolus accompanied by pain for half a year was presented at our hospital.He had never been treated prior to coming to our hospital.Preoperative imaging revealed a 10 cm×6 cm mass located in the body of the distal fibula.Pathological biopsies confirmed it was a giant cell tumor.Preoperative examination revealed he had dilated cardiomyopathy with class 3 cardiac function.The cardiologist and anesthesiologist determined that he could tolerate the operation,but the operation should be as short and minimally invasive as possible.With the patient’s consent,we performed a tibiotalar fusion and followed up with him for 2years,finding no recurrence and a satisfactory recovery.CONCLUSION Tibial talus fusion is an effective method for the treatment of distal fibula tumors.

Osteoclastic and pleomorphic giant cell tumors of the pancreas:A review of clinical,endoscopic,and pathologic features

Giant cell tumors of the pancreas come in three varieties-osteoclastic,pleomorphic,and mixed histology.These tumors have distinctive endoscopic,clinical,and cytological features.Giant cell tumors have a controversial histogenesis,with some authors favoring an epithelial origin and others favoring a mesenchymal origin.The true origin of these lesions remains unclear at this time.These are also very rare tumors but proper identification and differentiation from more common pancreatic adenocarcinoma is important.The risk factors of these tumors and the prognosis may be different from those associated with standard pancreatic adenocarcinoma.Recognition of these differences can significantly affect patient care.These lesions have a unique appearance when imaged with endoscopic ultrasound(EUS),and these lesions can be diagnosed via EUS guided Fine Needle Aspiration(FNA).This manuscript will review the endoscopic,clinical,and pathologic features of these tumors.

Hernia mesh prevent dislocation after wide excision and reconstruction of giant cell tumor distal radius

Giant cell tumor(GCT) remains as major health problem. GCT which located at the lower end of the radius tends to be more aggressive. Wide excision and reconstruction of the wrist in stage 3 of distal radius GCT lesion is an optimal modality to prevent tumor recurrence. However, dislocation often occurs as its complication. We are reporting patient with GCT of distal radius treated with wide excision and reconstruction using nonvascularized fibular graft and the addition of hernia mesh. Circumferential non-absorbable polypropylene hernia mesh was applied, covered radioulnar joint and volar aspect of radius, and served as additional support to prevent dislocation. During five years and two months of follow-up, we found no dislocation in our patient. Furthermore, good functional outcome was obtained. Our finding suggests that the addition of hernia mesh after wide excision and reconstruction with nonvascularized fibular graft may benefit to prevent dislocation and provides an excellent functional outcome.

Three-dimensional-printed custom-made patellar endoprosthesis for recurrent giant cell tumor of the patella:A case report and review of the literature

BACKGROUND Giant cell tumor(GCT)is a benign lesion and rarely involves the patella.This disease is characterized by a relatively high recurrence rate after primary treatment.En bloc resection has been a predominant option for recurrent GCT.However,total patellectomy can lead to disruption of the knee.Therefore,exploration of functional reconstruction of the extensor mechanism is worthwhile.CASE SUMMARY A 54-year-old woman presented with right knee pain and swelling,and was diagnosed as having a GCT in the patella following curettage and autograft.Medical imaging revealed a lytic and expanded lesion involving the whole patella with focal cortical breaches and pathological fracture.Based on the combination of histological,radiological,and clinical features,a diagnosis of recurrent GCT in the patella was made(Campanacci grade III).After a multidisciplinary team discussion,three-dimensional(3D)-printed custom-made patellar endoprosthesis was performed following en bloc resection for reconstructing the extensor mechanism.The patient was followed for 35 mo postoperatively.No evidence of local recurrence,pulmonary metastasis,or osteoarthritis of the right knee was observed.The active flexion arc was 0°-120°,and no extension lag was detected.A favorable patellar tracking and height(Insall-Salvati ratio 0.93)were detected by radiography.CONCLUSION We depict a case of a GCT at the right patella,which was successfully treated by patellectomy and 3D-printed custom-made endoprosthetic replacement.The patella normal reconstruction,the precise-fit articular design,and gastrocnemius flap augmentation could lead to satisfactory knee function and a low rate of complications in the short-term follow-up.

Three-dimensional printed talar prosthesis with biological function for giant cell tumor of the talus:A case report and review of the literature

BACKGROUND Giant cell tumors(GCT)are most commonly seen in the distal femur.These tumors are uncommon in the small bones of the hand and feet,and a very few cases have been reported.A giant cell tumor of the talus is rarely seen clinically and could be a challenge to physicians.CASE SUMMARY We report a rare case of GCT of the talus in one patient who underwent a new reconstructive surgery technique using a three-dimensional(3D)printing talar prosthesis.The prosthesis shape was designed by tomographic image processing and segmentation using technology to match the intact side by mirror symmetry with 3D post-processing technologies.The patient recovered nearly full range of motion of the ankle after 6 mo.The visual analogue scale and American Orthopaedic Foot and Ankle Society scores were 1 and 89 points,respectively.CONCLUSION We demonstrated that 3D printing of a talar prosthesis is a beneficial option for GCT of the talus.

Localized Giant Cell Tumor of the Tendon Sheath: A Rare Case of Anterior Knee Pain

This report describes a 61-year-old female with a giant cell tumor of the tendon sheath (GCT-TS). MRI showed that an elliptical abnormal signal was observed over the infrapatellar region of the right knee. We directly do arthroscopy to remove the tumor. An oval irregular mass of about 2.0 cm × 1.5 cm × 1 cm in the right knee joint was found. It was hard and had a dark red surface and the pedicle of the mass was connected with the joint capsule and infrapatellar fat pad. Nodular GCT-TS occurs less frequently in large joints than the small joints of the fingers and toes. The current report demonstrates the unique characteristics of the GCT-TS that extends around the ankle and invades the knee and proximal humerus.

Surgical treatment of giant cell tumors of long bone combined with inserted microwave antennas induced hyperthermia

AIM：To evaluate the surgical treatment methods of giant cell tumors(GCT) of long bone in conjunction with inserted microwave antennas induced hyperthermia.METHODS:46patients,included the surgical procedures,the oncology results,the functions of the limbs and the complications were analyzed.RESULTS:Follow-up 3.5 to 9 years(mean 5.5years).All patients were evaluated according to oncological and orthopaedic criteria.Two tumors were recurred.Orthopaedic furction were perfect in 44 patients and were fair in 2.Infection was found in 2 patients.CONCLUSION:The surgical procedure to treat the giant cell tumors of long bone by inserted microwave antennas induced hyperthermia is a definitive surgical method which is safe and confident.

The surgical Treatment and Outcome of Pathological Fracture in Patients with Giant Cell Tumor of Bone

Between 1992 and 2008, we treated 35 patients with giant cell tumor (GCT) of bone, seven of the 35 pre-sented with a pathological fracture. The fractures were located in the femur in five, and in the humerus and radius in one patient each. The surgical treatments were curettage in six cases and wide resection in the distal radius case. Two of the seven patients developed local recurrence, giving a local recurrence rate of 29%. The local recurrence rate in GCT patients without a pathological fracture was 21%. There was a tendency for there to be a higher recurrence rate associated with fractured GCT, but no statistically significant difference was observed between the two groups. Therefore, it was considered that a pathological fracture was not a risk factor for local recurrence in GCT.

Systemic Therapy in Patients with Resectable and Unresectable Cases of Giant Cell Tumor: A Systematic Review

Background: Giant cell tumor (GCT) is a common benign tumor of the appendicular and axial skeleton that represents 5% of all primary bone tumors. In recent years, the combination of conventional aggressive curettage with targeted adjuvant anti-osteoclastic agents including bisphosphonates and denosumab have led to lower recurrence rates in patients with GCT in a small number of retrospective case series. Furthermore, efficacy of the same anti-osteoclastic agents has been shown in cases of unresectable GCT of bone, leading to decreased rates of tumor progression and stabilization of disease. This review assesses whether the current literature weakly, moderately, or strongly supports a targeted systemic treatment as the standard of care in patients with GCT. Methods: We conducted a current search of the MEDLINE database for literature pertaining to systemic GCT treatment. Our inclusion criteria were as follows: 1) studies that reported on a series of patients with resectable or unresectable cases of GCT;2) a subset of patients must have been treated with systemic bisphosphonate or RANK-L inhibitor therapy;3) each series had a minimum of 10 patients with histopathologically confirmed GCT;4) each series stated their follow-up period. Results: Overall 6 studies, reporting on a total of 487 patients, were selected for inclusion in this review. For analysis, these 6 retrospective studies were subdivided into series where all GCT patients had resectable tumors (n = 4) and series where patients had a mix of resectable and unresectable tumors (n = 2). The overall recurrence rate of GCT in patients with resectable tumors treated with adjuvant systemic bisphosphonates was 6.7% compared to 48.4% in patients not treated with adjuvant systemic bisphosphonates (p 0.0001). In patients with both resectable and unresectable primary aggressive, recurrent, or metastatic GCT disease, systemic bisphosphonate and denosumab demonstrated good efficacy with decreased rates of disease progression and recurrence. In general the side effects of bisphosphonates were mild while denosumab had a more severe side effect profile. Conclusions: Systemic treatment with bisphosphonates or denosumab in cases of GCT is promising, but there is a lack of high-level evidence with sufficient follow-up supporting their use. We believe the current literature provides moderate support to recommend a short course of adjuvant peri-operative systemic bisphosphonate treatment for patients with resectable primary GCT and moderate support to recommend adjuvant peri-operative (resectable) and non-operative (unresectable) use of denosumab in cases of primary aggressive, recurrent, or metastatic GCT. With either systemic treatment, patients should be well counseled on all potential side effects in addition to alternative treatment, which includes the option of no systemic treatment.

Recurrent Giant Cell Tumor of the Distal End Radius: A Case Report and Surgical Treatment with Wide Resection and Reconstruction with Non-Vascularised Autologous Proximal Fibular Graft

Giant cell tumour of the distal radius is the 3rd most common site after proximal tibia and distal femur. It is locally aggressive and is associated with a high rate of recurrence. Although it is usually treated with various modalities of treatment, wide resection and reconstruction with proximal fibular autograft is most commonly accepted in recurrent cases. The following is a case report of such a case with surgical management.

Benign giant-cell tumor of the common bile duct:A case report

Primary giant-cell tumors rarely arise in the common bile duct. We herein report a case of primary giant-cell tumor of the common bile duct. The patient was an 81-year-old male who was diagnosed with a well-defined 1.2-cm mass projecting into the lumen of the middle common bile duct. Excision of the gallbladder and extrahepatic bile duct and a Roux-en-Y cholangiojejunostomy were performed. Histologically, the tumor had no association with carcinomas of epithelial origin and was similar to giant-cell tumors of the bone. The tumor consisted of a mixture of mononuclear and multinucleated osteoclast-like giant cells. The mononuclear cells showed no atypical features, and their nuclei were similar to those of the multinucleated giant cells. CD68 was expressed on the mononuclear and multinucleated osteoclast-like giant cells, whereas CD163 immunoreactivity was restricted to the mononuclear cells. Six months after the operation, the patient was still alive and had no recurrence. The interest of this case lies in the rarity of this entity, the difficulty of preoperative diagnosis, and this tumor’s possible confusion with other malignant tumors.

Malignant giant cell tumors of the tendon sheath of the right hip:A case report

BACKGROUND Malignant giant cell tumor of the tendon sheath(MGCTTS)is an extremely rare malignant tumor originating from synovial and tendon sheath tissue with highly aggressive biological behavior and a high rate of local recurrence and distant metastasis which should be considered a highly malignant sarcoma and managed aggressively.How to systemically treat MGCTTS remains a challenge.In this case,a patient with MGCTTS suffered a recurrence after 2 surgical resections received adjuvant chemotherapy and radiation therapy,but the treatment outcome remained poor.More clinical trials and better understanding of the biology and molecular aspects of this subtype of sarcoma are needed while novel medicines should be developed to efficiently target particular pathways.CASE SUMMARY A 52-year-old man presented with persistent dull pain in the right groin accompanied by limited right hip motion starting 6 mo ago.Two months before his attending to hospital,the patient’s pain worsened,presenting as severe pain when standing or walking,limping,and inability to straighten or move the right lower extremity.Surgical excision was performed and MGCTTS was confirmed by pathology examination.Two recurrences occurred after surgical resection,moreover,the treatment outcome remained poor after adjuvant chemotherapy and radiation therapy.The patient died only 10 mo after the initial diagnosis.CONCLUSION MGCTTS is characterized by a joint mass with pain and limited motion.It typically grows along the tendons and infiltrated into the surrounding muscle and bone tissue,with a stubborn tendency to relapse,as well as pulmonary metastasis.Radically surgical resection provides a choice of treatment whereas post-operation care should be taken to preserve the function of the joint.Chemotherapy and radiotherapy can be used as alternative treatments when radical resection cannot be performed.

Expect the unexpected:Brown tumor of the mandible as the first manifestation of primary hyperparathyroidism

Hyperparathyroidism(HPT)is a condition in which one or more parathyroid glands produce increased levels of parathyroid hormone(PTH),causing disturbances in calcium homeostasis.Most commonly HPT presents with asymptomatic hypercalcemia but the clinical spectrum may include disturbances reflecting the combined effects of increased PTH secretion and hypercalcemia.Brown tumors are rare,benign,tumor-like bone lesions,occurring in 1.5%to 4.5%of patients with HPT,as a complication of an uncontrolled disease pathway,and are nowadays rarely seen in clinical practice.The tumor can appear either as a solitary or multifocal lesion and usually presents as an asymptomatic swelling or a painful exophytic mass.Furthermore,it can cause a pathological fracture or skeletal pain and be radiologically described as a lytic bone lesion.The diagnosis of a brown tumor in HPT is typically confirmed by assessing the levels of serum calcium,phosphorus,and PTH.Although when present,brown tumor is quite pathognomonic for HPT,the histologic finding often suggests a giant cell tumor,while clinical presentation might suggest other more frequent pathologies such as metastatic tumors.Treatment of brown tumors frequently focuses on managing the underlying HPT,which can often lead to regression and resolution of the lesion,without the need for surgical intervention.However,in refractory cases or when dealing with large symptomatic lesions,surgical treatment may be necessary.

Retrospective analysis of patients with rare-site and metastatic giant cell tumor

A giant cell tumor occurs mainly in the proximal tibia,humerus,distal radius bone and the pelvic bone.It is rarely observed in such sites as the ribs and the temporal bone.The condition is primarily treated with surgical excision and functional reconstruction.The effect of chemotherapy on lung metastases and locally advanced giant cell tumors has remained unknown.We collected and analyzed the data of six patients with rare giant cell tumors located in the head and neck patients.After an average follow-up of 42.6 months after surgery （14 to 90 months）,no local recurrence or metastasis was observed.We also collected and analyzed the data of five patients with metastatic giant cell tumors who were undergoing surgery for the primary tumor before; of three patients who had experienced multiple chemotherapy cycles,one had spontaneous regression,and one survived for long timer despite progression.The other two patients had their major metastatic lesions resected by surgery,and presented long-term survival during the follow up.In addition,this study reports one patient with locally advanced giant cell tumor of the rib,who has undergone successful surgical resection following two cycles of chemotherapy with ifosfamide and liposomal doxorubicin.Complete resection of the lesion at the head and neck is the key to relapse-free survival.The prognosis of lung metastases in patients with giant cell tumors is relatively satisfying.Neoadjuvant chemotherapy is also conducive to the surgery for locally advanced lesions and improvement of the quality of life.

Functional Outcomes of Limb Salvage Surgery in Patients with Giant Cell Tumor of Bone of the Lower Extremities: A Retrospective Study

Giant cell tumor of the bone(GCTB)is a benign,locally aggressive neoplasm that is relatively rare,with a propensity to result in progressive bone destruction,and is associated with a high risk of recurrence.There is no widely held consensus regarding its ideal treatment.Worldwide,there are varying techniques ranging from intralesional curettage to resection of the lesion,supplemented with combinations of numerous adjuncts and fillers,depending on the resected amount and integrity of bone,as well as the preference of the surgeon.This was a cross-sectional study that included 20 patients who underwent limb salvage surgery for giant cell tumor of the bone of the lower extremities from January 2009 to February 2020 at two tertiary hospitals.The mean follow-up period was 37.3 months(SD=2.84).The extended curettage(EC)group had a mean Musculoskeletal Tumor Rating Scale(MSTS)score of 28.18(SD=7.51)which is considered as an excellent outcome,while the resection(RS)group had an mean MSTS score of 19.67(SD=11.02),which is considered as a good outcome.EC resulted to a total of eight complications(47%),while RS had one complication(33%).Prevalence of recurrence was noted to be 11.75%among those who underwent EC,while no recurrence was noted among those in the RS group.Use of bone cement as a filler was noted to have less recurrence as compared with the use of bone grafts,however were both were noted to result in excellent functional outcomes.Despite the prevalence of complications and recurrence of GCTB of the salvaged extremity in those who underwent EC,there is still report of excellent functionality.It is hence important to disclose all these possible outcomes and to stress the importance of compliance to follow-up for monitoring of these events.

Tenosynovial giant cell tumor involving the cervical spine: A case report

BACKGROUND Tenosynovial giant cell tumors(TGCTs)are a frequent benign proliferative disease originating from the synovial membrane.However,TGCTs rarely occur in the spine.The purpose of this paper is to report a case of TGCT occurring in the cervical spine.Although the disease is rare,it is essential to consider the possibility of TGCT in axial skeletal lesions.Awareness of spinal TGCTs is important because their characteristics are similar to common spinal tumor lesions.CASE SUMMARY A 49-year-old man with a 2-year history of neck pain and weakness in both lower extremities was referred to our ward.Imaging revealed a mass extending from the left epidural space to the C4-5 paravertebral muscles with uneven enhancement.The tumor originated in the synovium of the C4-5 lesser joint and eroded mainly the C4-5 vertebral arch and spine.Puncture biopsy was suggestive of a giant cellrich lesion.The patient had pulmonary tuberculosis,and we first administered anti-tuberculosis treatment.After the preoperative requirements of the antituberculosis treatment were met,we used a posterior cervical approach to completely remove the mass after fixation with eight pedicle screws.The mass was identified as a TGCT by postoperative immunohistochemical analysis.Recurrence was not detected after 1 year of follow-up.CONCLUSION Spinal TGCTs are often misdiagnosed.The radiological changes are not specific.The ideal treatment comprises complete excision with proper internal fixation,which can significantly reduce postoperative recurrence.

Traumatic giant cell tumor of rib: A case report

BACKGROUND Giant cell tumor(GCT)of the anterior rib origin is extremely rare.We report the first case of trauma-induced GCT of the rib.CASE SUMMARY A 22-year-old female developed a mass over the right anterior chest wall with pain 3 mo after a falling injury with blunt trauma of the right chest wall.Chest computed tomography(CT)showed a tumor originating from the right 6th rib with bony destruction,and a CT-guided needle biopsy revealed a GCT.We completely resected the tumor with chest wall and performed reconstruction.The pathological diagnosis was GCT of the bone.Twelve months after surgery,no signs of recurrence were observed.CONCLUSION GCT of the rib after trauma has not been reported.Meticulous history-taking and image evaluation are essential for the differential diagnosis of unusual chest wall tumors.