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.

Two Cases of Cutaneous Sarcomatoid Squamous Cell Carcinoma Resembling Cutaneous Giant Cell Tumor of Soft Tissue.

ABSTRACT: Cutaneous sarcomatoid squamous cell carcinoma is well-described with histology resembling pleomorphic undifferentiated sarcoma featuring collagenous or myxoid stroma with or without elements of keratinizing squamous carcinoma. This report presents 2 cases of dedifferentiated squamous cell carcinoma (SCC) composed of sheets of malignant mononuclear cells with malignant osteoclast-like multinucleated giant cells, extravasated blood, and hemosiderin resembling cutaneous giant cell tumor (cGCT). In the first case, an exophytic facial mass of a 96-year-old woman removed by shave showing extensive cGCT-like tumor but with microscopic elements of SCC in situ and positivity for cytokeratin 5/6 in the malignant spindle cells and SCC. The second case involved a 32-year-old man with a pedunculated penile mass removed by shave biopsy, displaying malignant cytology resembling cGCT, focal staining for cytokeratin AE1/AE3 and p63, and CD68 highlighting the osteoclast-like giant cells. Molecular analysis revealed CDKN2A, TP53, and TERT. Upon reexcision, case 2 showed focally invasive keratinizing SCC associated with differentiated penile intraepithelial neoplasia and lichen sclerosus. Skin specimens with an exophytic mass histologically resembling cGCT but with malignant cytology should be meticulously evaluated for elements of SCC. Molecular analysis, detecting mutations like H3F3 or HMGA2-NCOR2 fusion, can aid in distinguishing cutaneous sarcomatoid squamous cell carcinoma from GCT bone or GCT soft tissue.

Giant cell tumor of soft tissue involving thyroid gland: a case report and review of the literature.

BACKGROUND: Giant cell tumor of soft tissue is a low malignant uncommon neoplasm, with histologic features and immunophenotype similar to its bone counterpart. Primary giant cell tumor of soft tissue in the thyroid gland is considered an exceedingly rare entity. CASE PRESENTATION: We describe a case of primary thyroid giant cell tumor of soft tissue in a 69-year-old Chinese female patient. Neck ultrasonography showed a 19 mm × 12 mm × 5 mm nodule with heterogeneous echo and clear boundary located within the left thyroid. Histopathological examination revealed that the neoplasm was composed of two morphological components, mononuclear cells admixed with multinucleated osteoclast-like giant cells. Immunohistochemically, the tumor cells were positive for CD68 and vimentin, but were negative for epithelial membrane antigen, cytokeratin, and additional muscle markers. She underwent left unilateral thyroidectomy, and total thyroidectomy was performed for local recurrence 3 months later. The patient remained well without recurrence or metastasis following up for 12 months. CONCLUSION: The significance of this case lies in its rarity, the challenge of preoperative clinical diagnosis, and the differential diagnosis with other malignancies.

Xanthogranulomatous Epithelial Tumors and Keratin-Positive Giant Cell Rich Tumors of Soft Tissue and Bone: Two Sides of the Same Coin.

Xanthogranulomatous epithelial tumor is a recently described soft tissue tumor characterized by subcutaneous location, partial encapsulation, a xanthogranulomatous inflammatory cell infiltrate, and keratin-positive mononuclear cells. It shares some morphologic features with keratin-positive, giant cell-rich soft tissue tumors. Both have recently been shown to harbor HMGA2::NCOR2 fusions. The relationship between these tumors and their differential diagnosis with other osteoclast-containing soft tissue tumors is discussed.

A Mass on the Sole Revealing a Giant-Cell Tumor of the Tendon Sheath.

A 61-year-old woman presented with a 3-year history of painless soft-tissue mass on the right sole. The patient reported gradual growth, with a rapid increase in size over the past few months, leading to difficulty in walking. She had no history of past trauma. Examination revealed a 4-cm ovoid mass located over the ball of the foot. It was firm in consistency, with well-defined margins, a smooth surface, and an overlying normal skin (Figure 1). An ultrasound image revealed an eccentric, hypoechoic, nonvascular subcutaneous lobular mass. A magnetic resonance imaging (MRI) of the foot revealed a well-defined mass arising from the flexor tendon sheath of the right foot. The lesion was heterogeneously hyperin-tense on T1- and T2-weighted images with an avid contrast enhancement. All of the surrounding soft tissues indicated normal signal intensity patterns. There was no associated bony destruction. Histopathologic examination after complete excision of the mass established a well-circumscribed lesion composed of osteoclast-like giant cells and mononuclear cells in a hyalinized stroma, consistent with a giant cell tumor of the tendon sheath (GCT-TS) (Figure 2). There was no recurrence during a 6-month follow-up period (Figure 3).

Tenosynovial giant cell tumour of the finger: a case report.

Giant cell tumour most commonly occuring in epiphysis of the long bone, present and with pain, tenderness and swelling. It is a solitary lesion with restricted movement and tenderness over the lesion. The tendon sheath is where tenosynovial giant cell tumours typically develop. Because of its remarkably peculiar position, we present a case of giant cell tumour (GCT) tenosynovial of bone in the middle phalaynx in a 33-year-old female with complaints of swelling, pain in ring finger of left hand since 2 months which is rarely seen. After clinical, radiological, pathological investigations tenosynovial giant cell tumour was diagnosed. Following fine needle aspiration cytology, histopathology was utilized to confirm the tumour's diagnosis which was later treated as resection of excision of the tumour with allo/autograft reconstruction. Our case report showed no evidence of recurrence in 2 years of follow-up. Hence our case report proves that early and complete resection of the tumour shows evidence of regain of complete range of motion and decrease recurrence rate.

Giant Cell Tumour of Tendon Sheath of Distal Phalanx of Thumb: A Case Report.

Giant cell tumour of tendon sheath is an uncommon benign soft tissue tumour. Histopathological examination plays a crucial role in the definitive diagnosis of giant cell tumour although pre-operative imaging supports its suspicion. We report a case of a giant cell tumour of the tendon sheath in a 26-year-old man as a painless, firm, localized, slow-growing benign soft tissue tumour of the thumb; managed by complete excision. The patient continues to do well at 7 months post-surgery with no complaints and no signs of recurrence. Giant cell tumour of the phalanges is a locally aggressive entity; therefore delayed or missed diagnosis of giant cell tumour especially of the thumb distal phalanx can be extremely debilitating. Hence, high degree of suspicion and early en bloc resection is the key to its management. Keywords: case reports; giant cell tumors; tendons; thumb.

Giant cell tumor of the patellar tendon sheath in childhood: case report.

The aim of this study was to report a rare case of a giant cell tumor of the patellar tendon sheath. This indicates the diagnostic procedures and treatment options for giant cell tumors of the patellar tendon. This study reported a case of a 13-year-old male patient with a giant cell tumor of the tendon sheath. In our case, open arthrotomy was performed with complete surgical excision of the lesion. Histopathological examination revealed a giant cell tumor. At the last follow-up, 2 years after surgery, no complications were reported. The giant cell tumor of the patellar tendon sheath is an uncommon benign tumor. It mimics common knee symptoms. A differential diagnosis is definitely a challenge. Available operation approaches have demonstrated similar results, which lead to symptom relief and a low recurrence rate.

Rare Presentation of Giant Cell Tumors of the Tendon Sheath: Bilateral Involvement of Tendoachilles.

Giant cell tumor of tendon sheath arises from the synovium of tendon sheaths, joints, or bursa, mostly affects adults between 30 and 50 years of age, and is slightly more common in females. It corresponds to a localized form of pigmented villonodular synovitis (PVNS). Typically occur in the hand where they represent the second most common type of soft tissue tumors after synovial ganglions. Bilateral giant cell tumor of tendon sheath of tendoachilles is a rare presentation. We report the case of a 22-years-old female presenting with pain in both ankles without any history of trauma. On clinical examination, tenderness on both tendoachilles and local indurations were observed. Ultrasonography showed focal thickening of Achilles tendon bilaterally, and Doppler demonstrated increased flow in peritendinous area. MRI findings showed that most of the tumor had intermediate signal intensity and portions of the tumor had low signal intensity. Fine needle aspiration cytology confirmed the diagnosis of giant cell tumor of tendon sheath. Excision biopsy was done with no recurrence on subsequent follow-up.

Outcomes of Surgery for Benign Tumours in The Upper Extremity.

Benign tumours of the upper extremity are common in hand surgeons' practice. The most commonly diagnosed are giant-cell tumours of the tendon sheath and lipomas. THE OBJECTIVE: of this study was an investigation into the distribution of tumours in the upper limb, their symptomatology and outcomes of surgery, particularly regarding the rate of recurrence. MATERIAL AND METHODS: A total of 346 patients, 234 women (68%) and 112 men (32%), who had undergone surgery for tumours located in the upper extremity which were not ganglion cysts were enrolled into the study. The follow-up assessment was performed at a mean of 21 months (range 12-36) post-operatively. RESULTS: The most common tumour in this study was giant cell tumour of the tendon sheath - 96 cases (27.7%), followed by lipoma - 44 cases (12.7%). Most lesions - 231 (67%) were localized in the digits. A total of 79 (23%) recurrences were noted, the most common after surgery for rheumatoid nodules - 43.3% and the giant-cell tumours of the tendon sheath - 31.3%. The independent factors increasing risk of recurrence following the tumour's resection were: histological type of the lesion - the giant-cell tumour of the tendon sheath (p=0.0086) and the rheumatoid nodule (p=0.0027), as well as a combination of incomplete (non-radical) and not "en block" resection of tumours. A brief review of the literature referring to the presented material is offered.

Treatment of Patients Diagnosed with Giant Cell Tumor of Bone: Experience of a Philanthropic Hospital in the State of Piauí, Brazil / Tratamento de pacientes com diagnóstico de tumor ósseo de células gigantes: Experiência de um hospital filantrópico no Piauí, Brasil

Abstract Objective To evaluate the treatment of patients with giant cell tumors of bone treated from 2009 to 2019 in a philanthropic hospital, as well as to try and determine the regional clinical and epidemiological profile, aiming to enrich the Brazilian data set and compare our findings with those of the literature. Methods An analytical, observational, and cross-sectional study with retrospective data collection and a quantitative approach, analyzing medical records of patients with giant cell tumors treated at a philanthropic hospital from 2009 to 2019. Results We evaluated 49 medical records; 55.1% of the patients were women, 53.1% were aged between 20 and 40 years, 69.4% of the cases were Campanacci grade III, and 30.6% affected the proximal end of the tibia. The rate of pathological fractures secondary to the tumor and pulmonary metastasis was low. More than 69% of the patients underwent intralesional surgery. Recurrence occurred in 16.3% of the cases. Conclusion The criteria used for the diagnosis, classification, and treatment at our service followed the standards established by the literature, and they can guide further research and improve local prognosis in the future.

Resumo Objetivo Avaliar o tratamento fornecido a pacientes com diagnóstico de tumor de células gigantes ósseo atendidos no período de 2009 a 2019 em um hospital filantrópico, bem como determinar o perfil clínico e epidemiológico regional, visando enriquecer os dados nacionais e comparar os achados com a literatura existente. Métodos Estudo analítico, observacional e transversal, com coleta retrospectiva e abordagem quantitativa, com análise de prontuários de pacientes diagnosticados com tumor de células gigantes atendidos em um hospital filantrópico no período de 2009 a 2019. Resultados Foram avaliados 49 prontuários, sendo que 55,1% eram de mulheres, com 53,1% dos casos na faixa etária de 20 a 40 anos, 69,4% de casos de grau III de Campanacci, e 30,6% acometendo a extremidade proximal da tíbia. Observou-se baixo índice de fratura patológica secundária ao tumor e de metástase pulmonar. A cirurgia intralesional foi realizada em 69,5% dos pacientes. Houve recidiva em 16,3% dos casos. Conclusão Os critérios usados para diagnóstico, classificação e tratamento em nosso serviço seguiram os padrões estabelecidos pela literatura, e podem orientar novas pesquisas e melhorar o prognóstico local futuramente.

[MULTIPLE GIANT CELL TUMOR OF TENDON SHEATH: A CASE REPORT OF THREE LESIONS ON THE SAME FLEXOR TENDON].

INTRODUCTION: We present a case report of a triple location Giant Cell Tumor of tendon sheath appearance on the same flexor tendon sheath of a single digit. There have been scarce descriptions of multiple Giant Cell Tumors of tendon sheath. Multiple tumors may predispose patients to a higher recurrence rate; therefore, recognition and treatment of this rare entity is important.

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.

Cirugías ablativas por causas oncológicas. 17 años de experiencia en una Unidad Especializada del Estado Monagas, Venezuela / Ablative surgeries for oncological causes. 17 years experience in a Monagas specialized unit

Estudio retrospectivo, sobre pacientes a quienes se les practicó cirugías ablativas por causas oncológicas: un total de 76 casos, predominando en la serie: el sexo masculino, la segunda década de la vida y como motivo de consulta: aumento de volumen con un 73,5%, el osteosarcoma fue la neoplasia más frecuente con un 34%, la fractura patológica acompañó a los tumores en el 50% de los casos, el hueso más afectado fue el fémur, los estadios según Enneking predominantes fueron los II b y III. Se practicaron más cirugías en miembros inferiores, siendo la amputación transfemoral la más frecuente. La intención de los procedimientos fue predominantemente curativa con un 69% sobre las intenciones paliativas, se practicaron más amputaciones en niveles oncológicos radicales que amplias. Se obtuvo una sobrevida global de 53,4% a los dos años de seguimiento, el miembro fantasma estuvo presente como complicación por encima del 77% de los casos operados(AU)

Retrospective study on patients who underwent ablative surgeries for oncological causes: a total of 76 cases, predominantly in the series: male sex, second decade of life and as a reason for consultation: volume increase with 73,5%. Osteosarcoma was the most frequent neoplasm with 34%, pathological fracture accompanied the tumors in 50% of cases, the most affected bone was the femur, the predominant Enneking stages were IIb and III. More surgeries were performed on the lower limbs, with transfemoral amputation being the most frequent. The intention of the procedures was predominantly curative with 69% of the palliative intentions, more amputations were performed at radical oncological levels than extensive ones. An overall survival of 53.4% was obtained at two years of follow-up, the phantom limb was present as a complication in over 77% of operated cases.

Soft tissue aneurysmal bone cyst of left hemithorax: An extremely rare case.

Aneurysmal bone cyst (ABC) is a benign expansile cystic lesion that can affect any bone of the skeleton, especially the femur, tibia, and humerus. Lesions with histologic features of an ABC can be originated within soft tissue in exceedingly rare cases. Extra-skeletal ABC may mimic a variety of benign and malignant lesions and can be confused with other common or rare giant cell-rich tumors of soft tissue. Clinical, radiological and histologic correlation are crucial in reaching the correct diagnosis. Here we report a case of an extra-skeletal ABC arising in left hemithorax in a 13-year-old girl and discuss the common differential diagnosis of this rare entity.

Efficacy of Pazopanib in the Treatment of Metastatic Malignant Giant Cell Tumor of Soft Tissue: A Case Report.

Giant cell tumor of soft tissue (GCT-ST), histologically resembling the GCT of the bone, is a rare tumor. The tumor has been categorized to have low malignancy. Few reports of local recurrence or distant metastasis and the use of chemotherapeutic agents for metastatic GCT-ST exist. Herein, we report the efficacy of pazopanib in a 78-year-old Japanese woman with GCT in the intrinsic back musculature with both post-operative local recurrence and lung metastasis. The patient visited the hospital with a three-month history of a palpable mass in the intrinsic back musculature. Following magnetic resonance imaging, the tumor predominantly exhibited slight hyperintensity on T2-weighted images and intense heterogeneous enhancement on contrast-enhanced T1-weighted images. A percutaneous needle biopsy was performed, and the pathological diagnosis was GCT-ST. The patient underwent surgery, and three months later she presented with not only local recurrence but also multiple lung metastases. The patient was immediately treated with pazopanib 400 mg once daily. One month after initiating treatment, a partial response in the pulmonary lesions was observed, and stable disease (SD) effects lasted for 11 months without severe adverse effects. Therefore, pazopanib treatment for metastatic malignant giant cell tumor of soft tissue achieved reasonable success.

Diagnosis of giant cell-rich bone tumors on core needle biopsy: A practical approach.

BACKGROUND: The differential diagnosis of giant cell-rich bone tumors comprises a broad spectrum of lesions with prominent reactive osteoclast-like and/or neoplastic giant cells, with substantial differences in biologic behavior and clinical management. Evaluation of giant cell-rich bone tumors on small biopsies can be challenging especially in specimens with limited representative material. An accurate diagnosis requires a high level of skill on the part of both radiologist and pathologist as correlation with clinical and radiologic characteristics is critical. The objective of the study was to assess the utility of touch preparations (TP), immunohistochemistry (IHC) for mutation-specific markers H3G34W and H3K36M, and fluorescence in situ hybridization (FISH) for USP6 rearrangements and MDM2 amplification in the diagnostic workup of core needle biopsy specimens. METHODS: A total of 27 core needle biopsies with TPs from patients with primary giant cell-rich bone tumors (16 giant cell tumors of bone (GCTBs) (including 3 with lung metastasis), 3 chondroblastomas (CBs), 4 primary aneurysmal bone cysts (ABCs), 2 non-ossifying fibromas (NOFs), 1 low grade osteosarcoma (OS), and 1 conventional OS with tumor giant cells were analyzed with IHC for H3G34W and H3K36M and in select cases FISH for USP6 rearrangements and MDM2 amplification. RESULTS: In all cases the core biopsies were sufficient for histologic examination and diagnostic workup. 16 of 16 GCTBs were positive for H3G34W and negative for H3K36M, and 3 of 3 CBs were positive for H3K36M and negative for H3G34W. All other cases were negative for H3G34W and H3K36M. 4 of 4 primary ABCs showed rearrangement of USP6 by FISH and the low grade OS showed amplification of MDM2 by FISH. CONCLUSIONS: On-site adequacy assessment of TPs proved to be an accurate, simple, and fast method for obtaining sufficient material for complete diagnostic workup. The application of IHC for H3G34W and H3K36M and FISH for detection of rearrangements of USP6 and amplification of MDM2 can improve the diagnostic accuracy in core needle biopsy specimens.

Intramuscular Tenosynovial Giant Cell Tumor Harboring a Novel CSF1-CD96 Fusion Transcript.

Tenosynovial giant cell tumors typically arise in the synovium of joints, bursae, or tendon sheaths. They may occur in an intra- or extra-articular location and can be divided into localized and diffuse types. The neoplastic nature of the lesion has been supported by a recurrent CSF1 gene rearrangement in a small subset of lesional cells, of which the most common fusion partner is COL6A3. Herein, we report a case of intramuscular localized tenosynovial giant cell tumor harboring a novel CSF1-CD96 fusion transcript, thus expanding the molecular profile of this tumor.