Giant Cell Tumor Of Soft Tissue Research Articles

Giant Cell Tumor of Soft Tissue: An Updated Review.

Giant cell tumor of soft tissue (GCTST) is a locally aggressive mesenchymal neoplasm of intermediate malignancy that predominantly occurs in the superficial soft tissue of the extremities. It is histologically similar to a giant cell tumor of bone (GCTB) and shows a mixture of round to oval mononuclear cells and osteoclast-like multinucleated giant cells. Currently, immunohistochemistry plays a very limited role in the diagnosis of GCTST. Primary or secondary malignant GCTST has recently been described and tumors exhibiting high-grade histological features demonstrate higher rates of distant metastasis. GCTST lacks the H3-3A gene mutations that are identified in the vast majority of GCTBs, suggesting a different pathogenesis. Surgery is the standard treatment for localized GCTST. Incomplete surgical resection is usually followed by local recurrence. Radiation therapy may be considered when the close proximity of critical structures prevents microscopically negative surgical margins. The systemic treatment options for advanced or metastatic disease are very limited. This review provides an updated overview of the clinicoradiological features, pathogenesis, histopathology, and treatment for GCTST. In addition, we will discuss the differential diagnosis of this peculiar neoplasm.

Uterine giant cell tumor of soft tissue: A case report.

Giant cell tumor of soft tissue (GCT-ST) is a rare primary soft tissue tumor. GCT-ST mainly occurs in the trunk and extremities. There is no standard treatment for GCT-ST. This paper reports a rare case of primary uterine GCT-ST. A 48-year-old female patient underwent a transabdominal subhysterectomy for uterine leiomyoma. Postoperative pathological examination showed GCT-ST with unclear tissue boundary (10.0 × 6.0 × 5.0 cm). A small amount of GCT-ST tissue could be seen on the local edge of the leiomyoma. Residual tumor tissue was found around the uterine cavity. The patient reported persistent lower abdominal distension pain 3 months after the operation. Pelvic and abdominal imaging showed a huge tumor and multiple pelvic and abdominal organ metastasis. No pulmonary metastasis was found. Exploratory surgery revealed widespread metastases in the abdominal and peritoneal cavities, involving both ovaries, right tubal serous membrane, appendix serous membrane, bladder, pelvic peritoneum, and abdominal wall incision. After surgery, the patient had 6 cycles of docetaxel and carboplatin but stopped treatments due to economic reasons. The patient died 3 months later because of multiple organs failure. GCT-ST is generally benign but has unpredictable behavior. A massive recurrence with wide invasion is possible after subtotal resection.

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

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. 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. 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. 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.

CT and MRI findings of primary hepatic giant cell tumor of soft tissue: A case report

AbstractPrimary giant cell tumor of soft tissue (GCT-ST) is a low-grade malignant tumor and uncommon cancer originating from soft tissue. Primary hepatic GCT is an extremely rare finding. Only a handful of cases have been reported in the literature. Although GCT-ST has a relatively low malignant potential tumor in the view of histology, some patients who suffered from this tumor have poor prognosis after surgery because of the aggressiveness. Thus, the early diagnosis is important, and a preoperative imaging can supply the evidence of differential diagnosis to evaluate tumor malignancy. In this study, we systematically present a rare case of 56-year-old women with chills, fever, jaundice and anorexia for 3 days. CT showed a huge and multinodular confluent lesion in the left lobe of the liver, and it had a calcification component. This tumor is characterized by fibrous components and calcification, which showed iso-intense with peripheral hypo-intense on T1WI, and mainly inhomogeneous hypo-intense on fat-suppressed T2WI. Moreover, it was mainly hypointense and peripheral linear hyperintense on DWI images with a b-value of 800 (b800) and iso-hyperintense on ADC.

Giant Cell Tumors With HMGA2::NCOR2 Fusion : Clinicopathologic, Molecular, and Epigenetic Study of a Distinct Entity.

Giant cell tumors (GCTs) with high mobility group AT-Hook 2 ( HMGA2 )::nuclear receptor corepressor 2 ( NCOR2 ) fusion are rare mesenchymal tumors of controversial nosology, which have been anecdotally reported to respond to CSFR1 inhibitors. Here, we performed a comprehensive study of 6 GCTs with HMGA2::NCOR2 fusion and explored their relationship with other giant cell-rich neoplasms. Tumors occurred in 4 females and 2 males ranging in age from 17 to 32 years old (median 24). Three lesions originated in subcutaneous soft tissue and 3 in bone. Tumor size ranged from 20 to 33mm (median 27mm). The lesions had a nodular/multinodular architecture and were composed of sheets of mononuclear "histiocytoid" cells with uniform nuclei intermingled with multinucleated giant cells. Mitotic activity was low and nuclear atypia and metaplastic bone were absent. Variable findings included necrosis, cystic degeneration, lymphocytic infiltrate (sometimes forming nodules), and xanthogranulomatous inflammation. On immunohistochemistry, all cases focally expressed pan-keratin and were negative with SATB2 and H3.3G34W. Whole RNA-sequencing was performed in all cases of GCT with HMGA2::NCOR2 fusion and a subset of giant cell-rich tumors (tenosynovial-GCT, n = 19 and "wild-type" GCT of soft tissue, n = 9). Hierarchical clustering of RNA-sequencing data showed that GCT with HMGA2::NCOR2 fusion formed a single cluster, independent of the other 2 entities. Methylome profiling showed similar results, but the distinction from "wild-type" GCT of soft tissue was less flagrant. Gene expression analysis showed similar levels of expression of the CSF1/CSFR1 axis between GCT with HMGA2::NCOR2 fusion and tenosynovial-GCT, supporting their potential sensitivity to CSFR1 inhibitors. Clinical follow-up was available for 5 patients (range: 10 to 64mo; median 32mo). Three patients (60%) experienced local recurrences, whereas none had distant metastases or died of disease. Overall, our study confirms and expands previous knowledge on GCT with HMGA2::NCOR2 fusion and supports its inclusion as an independent entity.

An Unusual Entity: Giant Cell Tumour of Soft Tissue in the Nasal Cavity

Giant cell tumour of soft tissue (GCT-ST) are rare soft tissue tumours, with the first case reported in the 1970s. Involvement of the head and neck region, particularly in the nasal cavity is extremely uncommon. GCT-ST are considered the bone counterpart given the striking histological and immunohistochemical resemblance. Despite that, GCT-ST and GCT of bones are genetically distinct. Microscopically, GCT-ST exhibit numerous osteoclast-type multinucleated giant cells within a background of mononuclear stromal cells. They are locally aggressive and can potentially recur. It is important to distinguish GCT-ST from giant cell reparative granuloma (GCRG) as they both share similar clinical and morphological characteristics, however, they have different treatment modalities. We report a case of GCT-ST occurring in the nasal cavity, an unusual site of presentation.

Giant cell tumor of soft tissue: A case report

Giant cell tumor of soft tissue (GCT-ST) is a rare primary neoplasm of soft tissues. It usually involves superficial and deeper soft tissues of upper and lower extremities, followed by trunk. A 28-year-old female, presented with a painful mass in left abdominal wall for three months. On examination, it measured 4×4cm with ill-defined margins. CECT showed ill-defined enhancing lesion deep to muscle planes with possible invasion of peritoneal layer. Histopathology showed multinodular architecture with intervening fibrous septa and metaplastic bony tissue encasing the tumor. Tumor composed of round to oval mononuclear cells and osteoclast like multinucleated giant cells. Mitotic figures were eight per hpf. A diagnosis GCT-ST of anterior abdominal wall was made. Patient was treated with surgery followed by adjuvant radiotherapy. Patient is disease free at one year follow up. These tumors mostly involve extremities and trunk and usually presents as a painless mass. Clinical features depend upon the exact location of the tumor. Common differential diagnosis includes tenosynovial giant cell tumors and malignant giant cell tumors of soft tissue and GCT of Bone. Diagnosis of GCT-ST is difficult on cytopathology and radiology alone. Histopathological diagnosis should be done to rule out the malignant lesions. Complete surgical resection with clear resection margins is the mainstay of treatment. Adjuvant radiotherapy should be considered in case of incomplete resection. Long follow-up is necessary for these tumors as local recurrence and risk of metastasis cannot be predicted.

Giant Cell Tumor of Soft Tissue on the Forearm Skin: Case Report and Literature Review

Giant cell tumor of soft tissue (GCTST) is a very rare neoplasm that occurs predominantly in in the fifth decade of life presented as a benign looking painless mass associated with a local recurrence rate of 12% and very rare metastasis.
 We report a case of this rare entity presented with unusual presentations in a young boy which was diagnosed by histopathological examination and immunohistochemistry after complete excision raising the awareness of diagnosis and management of this unusual entity.

ABDOMINAL SWELLING IN A YOUNG MALE- A RARE CASE OF SOFT TISSUE GIANT CELL TUMOR

Giant cell tumor of soft tissue (GCT-ST) origin is a rare entity. Very few cases have been described, mostly in thigh, liver and thorax. Here we describe a case report of GCT-ST arising in the iliac region in a 24yr old male, who presented with a painless abdominal swelling. Initial CT scan imaging suggested a solid cystic mass in right lower quadrant of abdomen. The diagnosis of GCT was established by histopathological examination of excised specimen.

Giant cell tumor of soft tissue of the colon: a case report and review of the literature

BackgroundA giant cell tumor (GCT) is a benign neoplasm characterized by mixture of mononuclear cells and multinucleated cells. A GCT of soft tissue (GCT-ST) is developed in various extraosseous sites, but GCT-ST of the gastrointestinal tract is very rare. GCT-ST usually has a benign course, but rare cases reported malignant potential of the tumor. Therefore, complete resection is required to prevent local recurrence or distant metastasis.Case presentationA 53-year-old woman was admitted for follow-up colonoscopy who underwent the colorectal endoscopic submucosal dissection (ESD) of a laterally spreading tumor at the hepatic flexure 6 months ago. A colonoscopy showed a polypoid mass about 3.5 × 2.5 cm at the previous ESD site. As endoscopic finding showed a smooth multi-nodular tumor without submucosal invasion, we performed endoscopic mucosal resection. Based on pathological and immunohistochemical findings, the lesion was diagnosed as a GCT-ST in the colon. Follow-up colonoscopy performed 6 months later revealed no evidence of recurrence.ConclusionThis is the first report of a GCT-ST identified in the colon. Although GCT-ST generally has a benign clinical course, complete resection should be performed to prevent local recurrence and metastasis.

Primary Giant Cell Tumor of Soft Tissue in The Female Breast

A giant cell tumor of soft tissue (GCT-ST) is a rare tumor composed of mononuclear and osteoclast-like multinucleated giant cells. It is a low-grade malignant potential tumor morphologically analog to a giant cell tumor of bone. The superficial and deep soft tissue of extremities is the most frequent site of this tumor which is cured by complete or radical resection. GCT-ST as a primary tumor in the female Breast is exceedingly rare. Presentation of osteoclast-like multinucleated giant cells in the breast tumor has been described in association with the variant of breast carcinoma. We report a case of primary GCT-ST arising in the female Breast without a relationship with the epithelial component. To the best of our knowledge, this is the fifth such case report, and only the second one occurred in a female under 50 years.

Distinct H3F3A Driver Mutation Defines Giant Cell Tumor of Bone: A Necessary Adjunct in the Diagnosis of Such a Lesion in the Hyoid Bone, Presenting as a Base of Tongue Mass

<h3>Introduction</h3> Giant cell tumor of bone (GCTB) is a rare benign tumor mostly affecting the epiphyses of long bones in skeletally mature patients that is rarely described in the head and neck. Conventionally, diagnosis is based on microscopic features, exclusion of mimics, and clinico-radiologic correlation. Morphologic overlap with numerous giant cell-containing lesions of bone and soft tissue complicates the diagnosis. GCTB is characterized by the <i>H3F3A</i> mutation, which can be detected immunohistochemically using H3.3 G34W, a monoclonal antibody specific for this mutation. <h3>Case Findings</h3> A 57-year-old male presented with dysphagia, dysarthria, and right base of tongue (BOT) mass suspicious for carcinoma. Imaging revealed a heterogeneous parapharyngeal lesion extending from BOT to the hyoid bone. A BOT biopsy demonstrated giant cell-rich granulation tissue and non-endothelial lined blood lakes, suggestive of aneurysmal bone cyst (ABC). Fluorescent in situ hybridization (FISH) analysis did not identify <i>USP6</i> rearrangement, effectively eliminating primary ABC and favoring secondary ABC. Base of tongue resection, partial pharyngectomy and hyoid bone resection followed. <h3>Results</h3> Microscopically, the resection revealed a giant cell-rich lesion emanating from the hyoid bone, suggestive of GCTB and thus unlikely soft tissue giant cell tumor. Cellular pleomorphism and atypical mitoses were absent. Stromal cells expressed H3.3 G34W, confirming the diagnosis of GCTB. Zonal features of secondary ABC were juxtaposed to the dominant GCTB. <h3>Conclusions</h3> Hyoid GCTB is exceedingly rare, and such a diagnosis presents a conundrum. H3.3 G34W nuclear staining is an indicator of <i>H3F3A</i>mutation in GCTB that can help distinguish GCTB from other mimics. Prior reports of hyoid GCTB did not employ ancillary studies to identify <i>H3F3A</i> mutation or <i>USP6</i> rearrangements, as these have only been recently available. Our case fulfills current diagnostic criteria for GCTB, and excludes other giant cell-rich lesions. Accurate diagnosis of GCTB in this atypical location allows for appropriate surveillance algorithms.

Giant cell tumor of soft tissue: FNA cytopathology of 4 cases, review of the literature, and comparison with giant cell tumor of bone.

The cytopathology of a giant cell tumor of soft tissue (GCT-ST), a fibrohistiocytic neoplasm distinct from other giant cell-rich soft tissue tumors, is rarely reported. The authors report their experience with a series of 4 GCT-ST fine-needle aspiration (FNA) biopsy cases and compare them with a set from giant cell tumors of bone (GCTBs). The authors' cytopathology files were searched for GCT-ST examples with histopathologic confirmation. FNA biopsy smears were performed and examined with standard techniques. Four cases of GCT-ST presenting as a primary soft tissue mass from 4 patients (3 males and 1 female; age range, 28-75 years, mean age, 53 years) were retrieved. FNA sites included the anterior tibia, buttock, shoulder, and upper back. Three cases were interpreted as suspicious for sarcoma radiographically. The specific diagnoses were atypical giant cell tumor of tendon sheath, suspicious for GCT-ST, atypical myxoid lesion with giant cells, and benign with osteoclast-like giant cells (OLGCs). No case was interpreted as malignant. Aspirates consisted of mononuclear polygonal cells, spindled fibroblast cell clusters, and large OLGCs to the near exclusion of other cell types. OLGCs possessed 10 or more nuclei per cell. A comparison with GCTB aspirates and single case reports from the literature showed comparable cytomorphology. GCT-ST FNA smears mimic those of GCTBs containing a limited population of uniform spindle cell clusters, single dispersed polygonal cells, and cytologically banal OLGCs. GCT-ST should be considered in the differential diagnosis of aspirates containing numerous osteoclast-like giant cells.

Recurrent novel HMGA2-NCOR2 fusions characterize a subset of keratin-positive giant cell-rich soft tissue tumors

Giant cell tumors of soft tissue (GCT-ST) are rare low-grade neoplasms that were at one time thought to represent the soft tissue counterparts of GCT of bone (GCT-B) but are now known to lack the H3F3 mutations characteristic of osseous GCT. We present six distinctive giant cell-rich soft tissue neoplasms that expressed keratins and carried a recurrent HMGA2-NCOR2 gene fusion. Patients were five females and one male aged 14–60 years (median, 29). All presented with superficial (subcutaneous) masses that were removed by conservative marginal (3) or wide (2) local excision. The tumors originated in the upper extremity (2), lower extremity (2), head/neck (1), and trunk (1). Five patients with follow-up (median, 21 months; range, 14–168) remained disease-free. Grossly, all tumors were well-demarcated but not encapsulated with variable lobulation. Histologically, they were composed of bland plump epithelioid or ovoid to spindled mononuclear cells admixed with evenly distributed multinucleated osteoclast-type giant cells. Foci of stromal hemorrhage and hemosiderin were seen in all cases. The mitotic activity ranged from 2 to 14/10 high power fields (median: 10). Foci of necrosis and vascular invasion were seen in one case each. The mononuclear cells were immunoreactive with the AE1/AE3 keratin cocktail and less frequently/less diffusely for K7 and K19 but lacked expression of other lineage-associated markers. RNA-based next-generation sequencing revealed an HMGA2-NCOR2 fusion in all tumors. None of the keratin-negative conventional GCT-ST showed the HMGA2-NCOR2 fusion (0/7). Metaplastic bone (4/9) and SATB2 expression (3/4) were frequent in keratin-negative conventional GCT-ST but were lacking in keratin-positive HMGA2-NCOR2 fusion-positive tumors. The distinctive immunophenotype and genotype of these tumors strongly suggest that they represent a discrete entity, differing from conventional GCT-ST and other osteoclast-rich morphologic mimics. Their natural history appears favorable, although a study of additional cases and longer follow-up are warranted.

Giant cell tumour of soft tissue—a rare presentation of a common pathology

We present the case of a giant cell tumour of soft tissue (GCT-ST) presenting as a slow-growing paraspinal mass. Imaging investigations revealed a well-circumscribed subcutaneous lesion containing fluid–fluid levels and an internal solid nodule. The imaging findings resulted in only a tentative differential which included haematoma or complex epidermoid cyst but failed to provide a definitive diagnosis. The patient underwent an image-guided biopsy from which a histopathological diagnosis of a GCT-ST was made. GCT-ST is a primary soft tissue neoplasm that is clinically and histologically similar to giant cell tumour of bone. Given its rare occurrence, there is very little published literature on the characteristic imaging findings of GCT-ST to help with its diagnosis which is usually only made histologically. The aim of this case report is to highlight our specific imaging findings and add to the limited pre-existing imaging data on GCT-ST.

Overview of « druggable » alterations by histological subtypes of sarcomas and connective tissue intermediate malignancies.

We summarize herein the literature data about molecular targeted therapies in sarcomas and conjunctive tissue intermediate malignancies. For each clinical setting, the level of evidence, the mechanism of action and the target are described. The two major axes include (i) identification of subgroups of tumors with druggable alteration irrespective of the histological diagnosis (e.g. NTRK), and (ii) druggable target of pathway related to the physiopathology of the tumor: denosumab and bone giant cell tumor, imatinib and soft tissue giant cell tumor, mTOR inhibitor and PECOMA.

Myositis Ossificans Masquerading as Soft Tissue Giant Cell Tumour in Fine Needle Aspiration Cytology- A Case Report with Brief Review

Myositis Ossificans Masquerading as Soft Tissue Giant Cell Tumour in Fine Needle Aspiration Cytology- A Case Report with Brief Review

Soft Tissue Giant Cell Tumor - Incidence and Therapeutic Results

Abstract Introduction. Giant cell tumors of soft tissue (GCTs) have a relatively low incidence and their low prognosis is reserved to local relapses and distant metastases. This type of pathology usually affects adults and the elderly and it is localized in the extremities, most frequently in the thigh. Materials and methods. GCT is a relatively low aggressive tumor; approximately 85% of the patients survive at least 5 years after diagnosis. The risk factors for low prognosis are old age, metastases at the time of diagnosis, local relapse. We conducted this study in the University Emergency Hospital, Bucharest for a period of 3 years, between 01.01.2015 and 01.01.2018, which included 20 patients with ages between 22 and 83 years, of whom 9 were women and 11 were men. Results. Excision with safety margins was performed for all patients. During surgery, tissue samples from 6 different areas were sent for extemporaneous examination. After excision, the histopathological examination was performed and the diagnosis of GCT was established. Localized forms were described in 16 cases; diffuse forms were identified in 4 cases and loco-regional recurrences in 3 cases. Pre or postoperative adjuvant treatment was not applied in any of the cases. Conclusions. GCT is a rare, potentially malignant pathology, in which case evolution is unfavorable. From the clinical and imaging point of view, it is difficult to establish this diagnosis due to the large variety of pathologies it can be mistaken for, making biopsy an essential step within the diagnostic algorithm. Election treatment is represented by local excision with safety margins.

Primary Giant Cell Tumor of Soft Tissues : A Case Report and Literature Review

Giant cell tumor of soft tissue (GCT-ST) is a rare tumor of low malignant potential. Grossly and histologically similar to its bone counterpart. Majority has benign clinical course and rarely metastasizes. This is a case of a 46-year-old woman with a giant cell tumor of soft tissue of the knee. This tumor was not suspected clinically and was initially diagnosed as septic infra-patellar bursitis. The diagnosis of GCT-ST was made after histopathological examination. Herein, we describe the clinical, histologic, and immunohistochemical features of this rare neoplasm.

Recurrent primary mediastinal giant cell tumor of soft tissue with radiological findings: a rare case report and literature review

BackgroundGiant cell tumor of soft tissue (GCT-ST), which histologically resembles GCT of bone, is a rare tumor. Usually, it is located in the lower extremities and trunk. GCT-ST, occurring in mediastinum, is extremely rare.Case presentationWe encountered an 18-year-old Chinese woman who had mild dull pain on the left side of back. The following chest computed tomography (CT) showed a heterogeneous mass deeply situated in the posterior mediastinum with compression of the lung and invasion of the adjacent rib. On magnetic resonance imaging (MRI), the tumor exhibited predominantly slight hyperintensity on T2-weighted images and intensely heterogeneous enhancement on contrast-enhanced T1-weighted images. The whole body bone scan showed a mildly increased radiotracer uptake in the proximal portion of the left fifth rib, suggestive of local infiltration by the tumor. Surgical resection of the tumor was performed; subsequently, the tumor was histopathologically proved as GCT-ST. Three months after the operation, the patient developed a local recurrence. A brief discussion about the radiological findings, histopathological features, clinical behavior, and a detailed review of the relevant literature are presented.ConclusionsTo the best of our knowledge, this is the first case about recurrent primary mediastinal GCT-ST, moreover, this is the first report to introduce the MRI findings of primary mediastinal GCT-ST. The present case highlights the ubiquitous distribution of soft tissue giant cell tumor and the importance of considering this tumor in the differential diagnosis of posterior mediastinal neoplasms. Also, a long-term follow-up is required to properly assess the malignant potential of this tumor.