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.

Reduced recurrence rate and comparable functionality after wide resection and reverse total shoulder arthroplasty with allograft-prosthetic composite versus curettage for proximal humeral giant cell tumor: a multicenter retrospective study.

BACKGROUND: Giant cell tumors of bone (GCTBs) are rare, aggressive tumors, and the proximal humerus is a relatively rare location for GCTBs; limited evidence exists on which surgical approaches and reconstruction techniques are optimal. In the largest case series to date, we evaluated the recurrence rate of proximal humeral GCTBs and the functional outcomes of different resection and reconstruction options in this multicenter study. METHODS: All 51 patients included in this study received initial surgical treatment for proximal humeral GCTBs from January 2007 to December 2020, with a minimum 2-year follow-up period. Local recurrence and functional outcomes were statistically analyzed in relation to demographic, clinical, and primary surgical variables. Functional outcomes were reported by patients and were assessed by the Musculoskeletal Tumor Society score and QuickDASH instrument (shortened version of the Disabilities of the Arm, Shoulder and Hand instrument). RESULTS: The mean follow-up period was 81.5 months (range, 30-191 months), and the overall recurrence rate was 17.6% (9 of 51 patients). The majority of recurrences (n = 7) occurred in the first 2 years of follow-up. The intralesional curettage group (n = 23) showed a statistically significant difference in the recurrence rate compared with the en bloc resection group (n = 28) (34.8% vs. 3.6%, P = .007). Among shoulders receiving en bloc resection, 16 were reconstructed with hemiarthroplasty; 8, reverse total shoulder arthroplasty (rTSA) with allograft-prosthetic composite (APC) reconstruction; and 4, arthrodesis. On the basis of intention-to-treat analysis, the mean functional Musculoskeletal Tumor Society scores of the groups undergoing curettage, rTSA with APC, hemiarthroplasty, and arthrodesis were 26.0 ± 3.1, 26.0 ± 1.7, 20.3 ± 2.8, and 22.5 ± 1.3, respectively (P < .001 [with P < .001 for curettage vs. hemiarthroplasty and P = .004 for rTSA with APC vs. hemiarthroplasty]) and the mean QuickDASH scores were 14.0 ± 11.0, 11.6 ± 4.5, 33.1 ± 11.8, and 21.6 ± 4.7, respectively (P < .001 [with P < .001 for curettage vs. hemiarthroplasty and P = .003 for rTSA with APC vs. hemiarthroplasty]). CONCLUSIONS: On the basis of our data, en bloc resection followed by reverse shoulder arthroplasty showed a lower recurrence rate and no significant difference in functional outcome scores for proximal humeral GCTBs compared with intralesional curettage. Therefore, we believe that rTSA with APC may be reasonable for the initial treatment of proximal humeral GCTBs.

Sonographic features of diffuse giant cell tumor of the tendon sheath in the shoulder: A case report.

A middle-aged woman presented to our hospital with a chief complaint of a mass on the left shoulder for 1 year. The initial lump was small with no pain or tenderness, and the patient had not sought medical attention for numbness in the left shoulder. Clinical examination showed a mass on the left shoulder measuring 11 × 8 × 3 cm approximately with no apparent skin damage or ecchymosis. No limitations in left shoulder joint movements were observed, and the patient exhibited normal movement of the left elbow joint, wrist joint, and metacarpophalangeal joint. Moreover, the left radial artery was palpable.

A large, locally aggressive giant cell tumour arising from the laryngeal cartilage: A Rare Case Report.

Giant cell tumour is a growth predominantly found in long bones of the body. Giant cell tumour has a rare occurrence in the head and neck. A case of a 31 year old male with no known comorbidities at the ENT Department, Shifa International Hospital, Islamabad presented with anterior neck swelling and hoarseness of voice. Patient was diagnosed as having Giant Cell Tumour of Larynx (GTCL) proven on FNA cytology and post-operative biopsy. GCTL is an uncommon entity with only 45 reported cases in the world.

Denosumab treatment for progressive Enneking stage II cervical giant-cell tumor conservatively.

Cervical giant cell tumor of the bone (GCTB) is a rare, primary benign bone tumor in pediatric patients. Surgery remains the primary choice for treating resectable cervical GCTB. Additional adjuvant therapeutic options are available for patients with unresectable cervical GCTB, including the anti-RANKL monoclonal antibody, denosumab. We represented a case incidentally found in a 7-year-old female, who complained severe craniocervical pain, grade 2-3 dysphagia, dysphonia, hypesthesia, and extremity weakness. The patient showed an impressive clinical response to denosumab, both clinically and radiologically, without adverse events or recurrence. To date, this is the youngest patient ever reported to have a progressive Enneking stage II C3 GCTB treated with denosumab alone. Denosumab can be administered as a single and conservative therapy for pediatric patients with unresectable upper cervical GCTB, avoiding the risks and morbidity of surgical and radiative treatment.

Multimodal management of tenosynovial giant cell tumors (TGCT) in the landscape of new druggable targets.

Tenosynovial giant cell tumor (TGCT) is a rare, benign, locally aggressive synovial based neoplastic process that can result in functional debilitation and end-stage arthrtitis. Although surgical resection is the primary treatment modality, novel systemic therapies are emerging as part of the multimodal armamentarium for patients with unresectable or complex disease burden. This review discusses the pathogenesis of TGCT, potential druggable targets and therapeutic approaches. It also evaluates the safety and efficacy of different systemic therapies.

Keratin-positive giant cell-rich tumors of soft tissue with HMGA2::NCOR2 fusions.

BACKGROUND: Giant cell tumor of soft tissue (GCT-ST) is a rare soft tissue neoplasm that is morphologically similar to but genetically distinct from giant cell tumor of bone. A novel keratin-positive GCT-ST (KPGCT-ST) harboring HMGA2::NCOR2 fusions was recently discovered. Fewer than 30 cases have been described; herein is reported an additional seven. METHODS: Cases diagnosed as GCT-ST were retrieved from institutional archives and consultation files. The histopathologic characteristics were assessed, and the electronic medical record was reviewed. RESULTS: Seven tumors were identified in six women and one man with a median age of 23 years. All patients underwent excision; no recurrences or metastases were noted during a median follow-up period of 7 months. Histopathologically, the tumors were characterized by a multinodular proliferation of keratin-positive mononuclear cells with evenly admixed osteoclast-like giant cells and absent neoplastic bone. A fibrous capsule with lymphoid cuffing was frequently seen. Foamy macrophages, inflammation, hemorrhage, and hemosiderin were variably present. The HMGA2::NCOR2 fusion was detected in all cases. CONCLUSIONS: Our findings support previously reported hypotheses that KPGCT-ST is a spectrum of the same entity as the recently described xanthogranulomatous epithelial tumor. Although follow-up data are limited, to date, KPGCT-ST appears to follow an indolent course.

Giant cell tumor of tendon sheath: A report of 216 cases.

BACKGROUND: In this article on giant cell tumor of tendon sheath (GCTTS), we intend to summarize and analyze the clinical and pathological features of GCTTS hoping to improve clinical management and patient treatment. METHODS: The study retrospectively reviewed 216 patients of GCTTS, registered at the Affiliated Hospital of Southwest Medical University from January 2010 to December 2020. These cases were diagnosed by surgical excision. The clinicopathological features and the prognosis were reviewed in the light of the current literature. RESULTS: Of these 216 GCTTS patients, 72 were males (33.3%) and 144 females (66.7%), with a ratio male-to-female of 1:2. The patients' age ranged from 5 to 82, the average being 41.5 years at diagnosis. A total of 96 cases (44.4%) occurred in the hand region, followed by 35 cases (16.2%) in the knee, 32 cases (14.8%) in the foot, 25 cases (11.6%) in the ankle, 12 cases (5.6%) in the wrist, 12 cases (5.6%) in the leg, 2 cases (0.9%) in the head, 1 case (0.5%) in the forearm, and 1 case (0.5%) inside and outside the spinal channel. Histopathology mainly revealed large synovial-like monocytes, small monocytes, and osteoclast-like giant cells. CONCLUSION: Our results confirm that GCTTS predominantly occurs in the hands of young women. Complete surgical resection with long-term follow-up is the preferred management.

A Computed Tomography Radiomics Nomogram in Differentiating Chordoma From Giant Cell Tumor in the Axial Skeleton.

OBJECTIVE: The aim of the study is to develop and validate a computed tomography (CT) radiomics nomogram for preoperatively differentiating chordoma from giant cell tumor (GCT) in the axial skeleton. METHODS: Seventy-three chordomas and 38 GCTs in axial skeleton were retrospectively included and were divided into a training cohort (n = 63) and a test cohort (n = 48). The radiomics features were extracted from CT images. A radiomics signature was developed by using the least absolute shrinkage and selection operator model, and a radiomics score (Rad-score) was acquired. By combining the Rad-score with independent clinical risk factors using multivariate logistic regression model, a radiomics nomogram was established. Calibration and receiver operator characteristic curves were used to assess the performance of the nomogram. RESULTS: Five features were selected to construct the radiomics signature. The radiomics signature showed favorable discrimination in the training cohort (area under the curve [AUC], 0.860; 95% confidence interval [CI], 0.760-0.960) and the test cohort (AUC, 0.830; 95% CI, 0.710-0.950). Age and location were the independent clinical factors. The radiomics nomogram combining the Rad-score with independent clinical factors showed good discrimination capability in the training cohort (AUC, 0.930; 95% CI, 0.880-0.990) and the test cohort (AUC, 0.980; 95% CI, 0.940-1.000) and outperformed the radiomics signature ( z = 2.768, P = 0.006) in the test cohort. CONCLUSIONS: The CT radiomics nomogram shows good predictive efficacy in differentiating chordoma from GCT in the axial skeleton, which might facilitate clinical decision making.

Comparison of the prognostic factors of total en bloc spondylectomy and total piecemeal spondylectomy in patients with Enneking stage III giant cell tumor in the thoracic and lumbar spine.

PURPOSE: To compare total en bloc spondylectomy with marginal margins against piecemeal spondylectomy with intralesional margins in the surgical treatment of Enneking stage III spinal giant cell tumor (GCT) in terms of local recurrence. METHODS: A retrospective survival analysis of patients with Enneking stage III GCT who underwent TES with marginal margins or total piecemeal spondylectomy with intralesional margins was performed between January 2006 and April 2020. Local recurrence-free survival (LRFS) was the time between the date of surgery and recurrence. Factors with p-values < 0.05 in the univariate analysis were included in the multivariate analysis using proportional hazard analysis. RESULTS: Sixty patients (25 men and 35 women) with a mean age of 35.6 (range 11-71) years were included. The mean follow-up duration was 93 (range 24-198) months. Two patients were lost to follow-up 6 and 14 years after the procedure. Over a 10-year period, the recurrence rate was 13.3%. The 2-, 5-, and 10-year LRFS rates were 95%, 88%, and 78%, respectively. Univariate analysis identified total piecemeal spondylectomy and no adjuvant radiotherapy as prognostic factors for LRFS. Multivariate Cox-regression models showed a significant association between local recurrence and total piecemeal spondylectomy and no adjuvant radiotherapy. CONCLUSION: TES with marginal margins is better than total piecemeal spondylectomy with intralesional margins owing to its lower postoperative recurrence rate. Adjuvant radiotherapy should be administered to reduce postoperative recurrence rates.

Complications and prognosis of primary thoracic and lumbar giant cell tumors treated by total tumor resection.

BACKGROUND: Spinal giant cell tumor (SGCT) is a relatively rare primary tumor. En bloc resection is the preferred surgical procedure for it due to its aggressiveness, meanwhile leading to more complications. We reported the characteristics of perioperative complications and local control of total tumor resection including en bloc resection and piecemeal resection for primary thoracic and lumbar spinal giant cell tumors in a single center over 10 years. METHODS: This is a retrospective cross-sectional and cohort study. Forty-one consecutive patients with SGCTs who underwent total tumor resection from 2010 to 2020 at our institution and were followed up for at least 24 months were reviewed. Surgery data, complication characteristics and local tumor control were collected and compared by different surgical procedure. RESULTS: Forty-one patients were included, consisting of 18 males and 23 females, with a mean age of 34.2 years. Thirty-one had thoracic vertebra lesions, and 10 had lumbar vertebra lesions. Thirty-five patients were primary cases, and 6 patients were recurrent cases. Eighteen patients were treated by total en bloc spondylectomy (TES), 12 patients underwent en bloc resection according to WBB surgical system, and 11 patients underwent piecemeal resection. The average surgical time was 498 min, and the mean estimated blood loss was 2145 ml. A total of 58 complications were recorded, and 30 patients (73.2%) had at least one perioperative complication. All patients were followed up after surgery for at least 2 years. A total of 6 cases had postoperative internal fixation failure, and 4 cases presented local tumor recurrence (9.8%). CONCLUSIONS: Although the surgical technique is difficult and accompanied by a high rate of perioperative complications, en bloc resection can achieve favorable local control in SGCT. When it is too difficult to complete en bloc resection, thoroughly piecemeal resection without residual is also acceptable, given the relatively low recurrence rate.

Update of pediatric bone tumors: osteogenic tumors and osteoclastic giant cell-rich tumors.

There are numerous bone tumors in the pediatric population, with imaging playing an essential role in diagnosis and management. Our understanding of certain bone tumors has rapidly evolved over the past decade with advancements in next-generation genetic sequencing techniques. This increased level of understanding has altered the nomenclature, management approach, and prognosis of certain lesions. We provide a detailed update of bone tumors that occur in the pediatric population with emphasis on the recently released nomenclature provided in the 5th edition of the World Health Organization Classification of Soft Tissue and Bone Tumours.

Tenosynovial giant cell tumors of digits: MRI differentiation between localized types and diffuse types with pathology correlation.

OBJECTIVE: To compare the MRI findings between the localized- and diffuse-type tenosynovial giant cell tumors (TSGCTs) of digits with pathology correlation. METHODS: Twenty-eight patients with newly diagnosed TSGCTs of digits (22 localized and 6 diffuse types) who underwent preoperative MRI and surgical excision were included from Jan. 2015 to September 2021. MRI findings regarding nodularity, margins, morphology of hypointensity with pathology correlation, and disease extent (bone erosion, articular involvement, muscle involvement, tendon destruction, and neurovascular encasement) were assessed. RESULTS: Diffuse type was significantly larger (P = 0.006), more multinodular on both MRI and pathology (P = 0.038, both) with significant agreement, and infiltrative on both MRI and pathology (P < 0.001, both) with substantial agreement, and showed central granular on MRI and strong hemosiderin deposition on pathology (P = 0.022 and P = 0.021) with moderate agreement than localized type. Localized type showed significantly more frequent peripheral capsules on both MRI and pathology (P < 0.001, both) with moderate agreement than diffuse type. However, the septum on both MRI and pathology showed no statistically significant difference between the two groups (P = 0.529 and P = 0.372) without significant agreement. The disease extent was more severe in the diffuse type than the localized type regarding articular involvement (P < 0.001), muscle involvement (P < 0.001), and tendon destruction (P = 0.010). No statistically significant differences were found between the two groups regarding bone erosion (P = 0.196) or neurovascular bundle encasement (P = 0.165). CONCLUSIONS: Diffuse-type TSGCTs of digits presented as locally aggressive lesions with larger, multinodular, infiltrative masses exhibiting stronger hemosiderin deposition and more severe disease extents of articular, muscle, and tendon involvement than the localized type.

Update of pediatric bone tumors-other mesenchymal tumors of bone, hematopoietic neoplasms of bone, and WHO classification of undifferentiated small round cell sarcomas of bone.

There are numerous bone tumors in the pediatric population, with imaging playing an essential role in diagnosis and management. Our understanding of certain bone tumors has rapidly evolved over the past decade with advancements in next-generation genetic sequencing techniques. This increased level of understanding has altered the nomenclature, management approach, and prognosis of certain lesions. We provide a detailed update of bone tumors that occur in the pediatric population with emphasis on the recently released nomenclature provided in the 5th edition of the World Health Organization Classification of Soft Tissue and Bone Tumours. We discuss other mesenchymal tumors of bone, hematopoietic neoplasms of bone, and WHO classification of undifferentiated small round cell sarcomas of bone. We have detailed osteogenic tumors and osteoclastic giant cell-rich tumors, as well as notochordal tumors, chondrogenic tumors, and vascular tumors of the bone in separate manuscripts.

Total en bloc spondylectomy of thoracic giant cell tumor with secondary aneurysmal bone cyst: case reports and review of literature.

Spinal giant cell tumor (GCT) combined with secondary aneurysmal bone cyst (ABC) is a locally aggressive primary bone tumor. Total en bloc spondylectomy has never been reported to treat thoracic GCT combined with secondary ABC. We retrospectively reviewed two cases of spinal GCT combined with secondary ABC. A 41-year-old male patient was presented with back pain due to irregular expansive bone destruction involving the T6 vertebral body and intraspinal space-occupying lesion. Total en bloc spondylectomy of T6 vertebra was performed with good neurological status after the surgery. A 29-year-old female patient was presented with right scapular region pain due to irregular expansive bone destruction involving the T5 vertebral body and intraspinal space-occupying lesion. Total en bloc spondylectomy of T5 vertebra was performed with good neurological status after the surgery. Adjuvant radiation therapy was applied after the surgery without local recurrence at the 12-month or 24-month follow-up. Spinal GCT combined with secondary ABC appears to have a high local recurrence rate. Therefore, total en bloc spondylectomy should be applied to treat thoracic GCT combined with secondary ABC.

A novel three-dimensional-printed patient-specific sacral implant for spinopelvic reconstruction in sacral giant cell tumour.

PURPOSE: Spinopelvic reconstruction after sacral tumour resection is one of the most demanding procedures in sacral tumour surgery. The aims of this study were to evaluate the feasibility of spinopelvic reconstruction with 3D-printed prostheses in sacral giant cell tumours and the clinical outcomes and complications at follow-up. METHODS: We retrospectively analyzed ten consecutive patients with giant cell tumors of the sacrum who underwent intralesional nerve-sparing resection with curative intent and custom implant reconstruction between 2016 and 2021. There were four males and six females with a mean age of 40.2 years (range, 25-62 years) at surgery. A computer-aided-design implant was prepared using 3D printing technology that was both matched to the bone defect and biomechanically evaluated. A 3D-printed surgical guide was used to replicate the resection procedure as planned. We analyzed operational outcomes, oncological outcomes, functional outcomes, complications, and prosthetic outcomes. Pain at rest was assessed according to a 10-cm VAS score. The results of functional improvement were evaluated using the MSTS-93 score at the final follow-up. RESULTS: All patients were observed for 26 to 61 months, with an average follow-up of 43.8 months. No deep infection or prosthetic structural failure occurred in this study. A total of 80% of patients had good neurological function and normal urinary, bowel, and ambulatory functions. The mean MSTS score was 24.1 (range, 22-26). The mean VAS score was 2 (range 0 to 2). Delayed wound healing occurred in three patients, and the wounds healed after debridement. One case had local recurrence and survived tumour-free after resection of the recurrent lesion. An aseptic loosening was found in a patient that did not require secondary surgery. By radiographical assessments, we found that 90% of implants were well osseointegrated at the final follow-up examination. CONCLUSIONS: The 3D-printed sacral implants might provide a promising strategy for spinopelvic reconstruction in sacral giant cell tumours undergoing intralesional nerve-sparing surgery with satisfactory clinical outcomes, osseointegration, and excellent durability.

Resection and reconstruction with and without neoadjuvant denosumab in campanacci grade III giant cell tumors of proximal humerus: a retrospective comparative study.

BACKGROUND: Giant cell tumors (GCT) of the proximal humerus are rarely reported case that requires complex surgeries due to involvement of the shoulder joint. Therefore, we report the first retrospective comparative study where the postoperative functional outcomes, recurrence rate and complications in patients who underwent arthrodesis of shoulder after resection of grade III GCT with and without neoadjuvant denosumab are compared. METHODS: A retrospective review of eight cases of grade III GCT of proximal humerus receiving resection and fibular strut graft and arthrodesis between January 2014 and December 2019 is performed. They were stratified into two groups: one group of four patients received once-weekly denosumab 120 mg for 4-weeks before resection and reconstruction, while the other group of four patients did not receive denosumab before surgery. Primary outcomes included the functional outcomes assessed by revised Musculoskeletal tumor society (MSTS) score and shoulder pain and disability index (SPDI) at 6-weeks and 12-months postoperatively while secondary outcomes included complications and recurrences. RESULTS: There was no significant difference in terms of SPDI at 6 weeks and 12 months, MSTS at 12 months, complications, recurrence among denosumab and non-denosumab groups. At 6-weeks follow-up, a significantly better pain score in SPDI and MSTS was acquired in the denosumab group. CONCLUSIONS: Resection and reconstruction with or without neoadjuvant denosumab for Grade III GCT of proximal humerus had similar functional outcomes and with no major differences in recurrence and complications. Hence, postoperative pain relief while long-term benefits were not discovered with the use of neoadjuvant denosumab.

Xanthogranulomatous epithelial tumors and keratin-positive giant cell-rich soft tissue tumors: two aspects of a single entity with frequent HMGA2-NCOR2 fusions.

Xanthogranulomatous epithelial tumor (XGET) and keratin-positive giant cell-rich soft tissue tumor with HMGA2-NCOR2 fusion (KPGCT) are two recently described neoplasms with both distinct and overlapping clinical and histopathologic features. We hypothesized that XGET and KPGCT may be related and represent a histologic spectrum of a single entity. To test this, we sought to characterize the clinical, radiographic, immunohistochemical, ultrastructural and molecular features of additional tumors with features of XGET and/or KPGCT, which we refer to descriptively as keratin-positive xanthogranulomatous/giant cell-rich tumors (KPXG/GCT). The archives were searched for potential cases of KPXG/GCT. Clinical and imaging features were noted. Slides were assessed for histologic and immunohistochemical findings. Ultrastructural and next generation RNA sequencing-based analysis were also performed. Nine cases were identified arising in seven women and two men [median age of 33 years (range: 12-87)]. Median tumor size was 4 cm (range: 2.4-14.0 cm) and tumors presented in the thigh (2), buttock (1), forearm (2), groin (1), cranial fossa (1), ilium (1), and tibia (1). Morphologically, tumors were most frequently characterized by a fibrous capsule, with associated lymphoid reaction, enclosing a polymorphous proliferation of histiocytes, giant cells (Touton and osteoclast-types), mixed inflammatory infiltrate, hemorrhage and hemosiderin deposition, which imparted a variably xanthogranulomatous to giant cell tumor-like appearance. One case clearly showed mononuclear cells with eosinophilic cytoplasm characteristic of XGET. All cases expressed keratin and 7 of 9 were found to harbor HMGA2-NCOR2 fusions including cases with xanthogranulomatous appearance. One patient developed local recurrence and multifocal pulmonary lesions, which were radiographically suspicious for metastases. Shared clinical, histologic and immunohistochemical features, and the shared presence of HMGA2-NCOR2 fusions supports interpretation of KPXG/GCT as a single entity which includes XGET and KPGCT. Given limited clinical follow-up to date and rare cases with apparently aggressive findings, we provisionally regard these tumors as having uncertain biologic potential.

Approach to Fine Needle Aspiration of Giant Cell-rich Tumors of Soft Tissue.

Giant cells may be found in a wide variety of reactive and neoplastic soft tissue lesions. Because of their distinct histomorphology, they often stand out in procured samples such as fine needle aspirates. The giant cells themselves may be benign or neoplastic. However, the presence, type, and quantity of giant cells are usually not specific and in some cases can even be misleading when making a diagnosis. The aim of this review is to guide the practicing cytopathologist in narrowing their differential diagnosis when encountering one of these challenging giant cell-rich lesions of the soft tissue.

Is pancreatic giant cell tumor resistant to standard chemotherapy?

Pancreatic giant cell tumors (PGCTs), undifferentiated pancreatic carcinoma are rare tumors of the pancreas. PGCTs consist of osteoclastic, pleomorphic and mixed variants. PGCT is usually diagnosed at an advanced stage. PGCT has a worse prognosis than pancreatic ductal adenocarcinoma. Although surgery can be curative, there is no standard treatment approach for advanced PGCT. We present a case of PGCT that is resistant to standard therapy and progresses in a short time.