Malignant Giant Cell Research Articles

Incidence and predictors of 30-day postoperative complications following musculoskeletal tumor surgery

Objectives: Surgical intervention remains one of the best curative approaches in musculoskeletal (MSK) tumors. However, postoperative complications remain an issue, especially within the first 30-day postoperatively. Hence, the main objective of this study is to estimate the incidence and to identify the predictive factors of 30-day complications following MSK tumor surgery (MTS). Methods: This is a retrospective, descriptive study of 85 patients who had undergone MTS between 2016 and 2019 in our institution. The included patients were those who had MTS caused by a malignant tumor or giant cell tumor. Results: Twenty-eight patients (33%) had a complication during the first 30 days following surgery. The most common complication was surgical site infection in 64% (n = 18 / 28) followed by venous thromboembolism in 11% (n = 3 / 28) and then death in 7% of the patients (n = 2 / 28). Complications were distributed equally among genders. The complication rate was 36% in bone tumor surgeries (n = 22 / 61) and 21% in soft tissue tumor surgeries (n = 4 / 24). Ewing's sarcoma patients had the highest complication rate among bone tumors (36%, n = 8 / 22). Lower extremities' surgeries had the highest complication rate (35%, n = 18 / 51). Furthermore, 80% of the patients with a cardiovascular condition other than hypertension developed a complication (n = 4 / 5, P = 0.0322). Conclusion: One-third of the patients who underwent MTS developed a complication within a 30-day duration. The diagnosis of Ewing's sarcoma, surgery of lower extremities, and preexisting cardiovascular conditions was linked to a higher complication rate.

Clinical characteristics and treatment outcomes in six cases of malignant tenosynovial giant cell tumor: initial experience of molecularly targeted therapy

BackgroundAlthough tenosynovial giant cell tumor (TGCT) is classified as a benign tumor, it may undergo malignant transformation and metastasize in extremely rare occasions. High aberrant expression of CSF1 has been implicated in the development of TGCT and recent studies have shown promising activity of several CSF1R inhibitors against benign diffuse-type TGCT; however, little is known about their effects in malignant TGCT.Case presentationInformation from six consenting patients (3 men, 3 women) with malignant TGCT presenting to Dana-Farber Cancer Institute for initial or subsequent consultation was collected. Median age at initial diagnosis of TGCT was 49.5 years (range 12–55), and median age at diagnosis of malignant TGCT was 50 years (range 34–55). Two patients developed malignant TGCT de novo, while four other cases showed metachronous malignant transformation. All tumors arose in the lower extremities (3 knee, 2 thigh, 1 hip). Five patients underwent surgery for the primary tumors, and four developed local recurrence. All six patients developed lung metastases, and four of five evaluable tumors developed inguinal and pelvic lymph node metastases. All six patients received systemic therapy. Five patients were treated with at least one tyrosine kinase inhibitor with inhibitory activity against CSF1R; however, only one patient showed clinical benefit (SD or PR). Five patients were treated with conventional cytotoxic agents. Doxorubicin-based treatment showed clinical benefit in all four evaluable patients, and gemcitabine/docetaxel showed clinical benefit in two patients. All six patients died of disease after a median of 21.5 months from diagnosis of malignant TGCT.ConclusionsThis study confirms that TGCT may transform into an aggressive malignant tumor. Lymph node and pulmonary metastases are common. Local recurrence rates are exceedingly high. Conventional cytotoxic chemotherapy showed clinical benefit, whereas tyrosine kinase inhibitors against CSF1R showed limited activity. Given its rarity, a prospective registry of malignant TGCT patients is needed to further understand the entity and to develop effective strategies for systemic treatment.

The reconstruction of tumorous partly defect in acetabulum

Objective Investigate the surgical resection, reconstruction technique and follow-up of partly defect by primary bone malignancy in acetabulum. Methods Retrospective analysis 20 cases clinical data of partly defect by primary bone malignancy in acetabulum patients from January 2009 to January 2015. Resect the tumor and reconstruct the acetabulum based on the type of acetabulum tumor: type A, excise pubis and partial acetabulum, use self-femoral-head transplantation and pelvis reconstruct steels to reconstruct the bone defection of acetabulum leading edge, then transplant biological type acetabular cup; type B, excise ischium and trailing edge of acetabulum, transplant self-femoral-head and fixed with cancellous screw, for those can’t maintain the acetabulum stabilization, apply acetabulum enhance ring to immobilize; type C, excise partial ilium and acetabulum superior margo to interrupt its discontinuity, self-femoral-head transplant to reconstruct acetabulum, then use enhance ring and cemented cup to rebuild hip joint. Results Total 20 cases, 13 males and 7 females; the age ranges 23-69 years old, average 48 years; followed up 13-56 months, mean 34 months. Pathology types: 17 cases of chondrosarcoma and 3 cases of malignant bone giant cell tumor. After surgery, 3 cases of chondrosarcoma recurred (15%) , 1 case of malignant bone giant cell tumor relapsed and developed to pulmonary metastasis. 2 cases of acetabulum prosthesis incipient dislocation performed closed replacement then fixed 6 weeks with hip joint brace. 1 cases of prosthesis losing performed hip joint revision. 1 case occurred deep hip infection, which performed debridement and taking out prosthesis. 2 cases self-femoral-head transplant and biological total hip replacement healed well. Postoperative functional MSTS 93 score showed excellent in 13 cases, good in 6 cases, poor in 1 case. Conclusion According to the tumor type and range, determine the resection method and boundary, which is the key to acquire well oncological prognosis. On the premise of sufficient tumor resection, reconstruct function individually based on the type of acetabulum tumor, which is the key to acquire well functional prognosis and prosthesis survival rate. Key words: Bone neoplasms; Acetabulum; Reconstructive surgical procedures

En bloc resection of tumor involving partial sacrum and ipsilateral sacroiliac joint with reservation of contralateral sacral foramina

Objective To evaluate effect of en bloc resection of malignant sacral tumor involving partial sacrum and ipsilateral sacroiliac joint with reservation of contralateral sacral foramina.Methods Between February 2005 and July 2010,16 patients with malignant sacral tumor involving partial sacrum and ipsilateral sacroiliac joint underwent hemi-sacrectomy and functional reconstruction.The operation achieved en bloc resection of tumor with reservation of contralateral sacral foramina.There were 10 males and 6 females,aged from 17 to 70 years (average,37.9 years).There were 5 cases of chondrosarcoma,3 cases of Ewing's sarcoma,2 cases of malignant schwannoma,2 cases of osteosarcoma,1 case of malignant giant cell tumor of bone,1 case of lymphoma and 2 cases of metastatic tumor.Results The surgical time ranged from 4 to 11.5 hours (average,6.5 hours).The average blood loss was 3600 ml (range,1500 to 5500 ml).Five patients underwent type Ⅰ resection,while 11 patients underwent type Ⅱ resection.All patients were followed up for 21 to 59 months (average,34.4 months).Three months after operation,13 patients had difficulty with ankle plantar flexion,while sphincter function was more or less reserved in 16 patients.Four patients (25％)had wound healing complications,which was cured after debridement and drainage.Local recurrence occurred in 7 patients.There were 5 cases of local recurrence among 6 patients who had intralesional margin;4 recurrence lesions located in the sacrum and 1 in soft tissue.Eight patients (50.0％) survived without tumor,2 patients (12.5％)survived with tumor,and 6 patients (37.5％) died of tumor.Conclusion The en bloc resection of tumor involving partial sacrum and ipsilateral sacroiliac joint with reservation of contralateral sacral foramina was feasible and had better outcome compared with total sacrectomy. Key words: Sacrum; Sacroiliac joint; Bone neoplasms; Treatment outcome

P63 Expression in Giant Cell–Containing Lesions of Bone and Soft Tissue

Previous studies have demonstrated p63 overexpression in giant cell tumors of bone and advocate its use as a potential diagnostic marker. Although routine histology is often all that is required to diagnose giant cell tumor of bone, immunohistochemistry could prove useful to distinguish it from other benign and malignant giant cell-containing lesions of bone and soft tissue on needle biopsies and unusual clinical settings. To assess p63 expression in giant cell-containing lesions of bone and soft tissue. p63 immunohistochemistry was performed in 23 giant cell tumors of bone, 8 primary aneurysmal bone cysts, 12 chondroblastomas, 4 giant cell reparative granulomas, 4 osteosarcomas, 15 tenosynovial giant cell tumors, 6 nonossifying fibromas, and 4 pigmented villonodular synovitides. p63 overexpression was identified in 20 of 23 giant cell tumors of bone (86.9%), 5 of 8 primary aneurysmal bone cysts (62.5%), 10 of 12 chondroblastomas (83.3%), 4 of 4 giant cell reparative granulomas (100%), 2 of 4 osteosarcomas (50%), 1 of 15 tenosynovial giant cell tumors (6.6%), 1 of 6 nonossifying fibromas (16.6%), and 1 of 4 pigmented villonodular synovitides (25%). The sensitivity, specificity, positive predictive value, and negative predictive value of p63 immunohistochemistry for the diagnosis of giant cell tumor of bone were 86.95%, 53.36%, 45.45%, and 91.17%, respectively. This study shows that although p63 is expressed by most giant cell tumors of bone, its lack of specificity limits its use as an immunohistochemical marker in the differential diagnosis of giant cell-containing lesions of bone and soft tissue.

Features of long bone diaphysial osteosarcoma on X-ray, CT and MRI

Objective To explore the findings of diaphysial osteosarcoma in long bone on X-ray,CT and MRI, and discuss their clinical features and manifestations for differential diagnosis. Methods Twenty-eight cases with diaphysial osteosarcoma in long bone proved by surgery and pathology were reviewed retrospectively. Eighteen tumors were located in the femur, 4 in fibula, 4 in humerus and 2 in tibia. All of the patients were examined by X-ray, CT and MRI. The imaging manifestations on X-ray, CT and MRI were analyzed, and the relationship of the imaging features with the pathological types was also observed. The imaging signs were correlated with the pathologic findings with a double blind method. Results Of the 28 cases, there were 16 cases with large bone destruction, 22 cases with periosteal reaction on X-ray and CT. On X-ray, 18 cases showed soft tissue mass and 12 cases with neoplastic bone and tumor calcification.While on CT, 22 cases showed soft tissue mass on plain scan and 2 more cases displayed soft tissue mass after the injection of contrast mediun. Sixteen cases showed neoplastic bone and tumor calcification on CT.On MRI, there were 10 cases with bone destruction and periosteal reaction with iso- and hypo-intense on T1WI and iso- signals on T2WI. Twenty-six cases showed soft tissue edema and bone marrow on MRI. The soft mass were iso-signals on T1 WI and iso-hyperintense signals on T2 WI or STIR. The soft tissue edema was found hyperintense signals on T2WI or STIR. The lesions had heterogeneous enhancement especially in bone marrow with edema and adjcent soft tissue. Conclusion The X-ray, CT and MRI can reflect the pathological changes of diaphysial osteosarcoma in long bone from different aspects. Lower incidence, large bone destruction and no pathological fracture were the features of diaphysial osteosarcoma. The osteogenic type is diagnosed easily, but the osteolytic lesion should be differentiated from Ewing sarcoma, malignant giant cell tumor of bone and so on. Key words: Osteosarcoma; Diaphysis; Magnetic resonace imaging; Tomography, X-ray computed; Radiography

Malignant myoepithelioma of the breast

Malignant myoepithelioma of the breast is rare. A 50-year-old Japanese woman was admitted to our hospital because of a right breast tumor (11 × 10 × 5.5 cm). Core needle biopsy revealed malignant spindle cells. A mastectomy was performed. The tumor consisted of malignant spindle, round, pleomorphic and giant cells with many mitotic figures and necrotic areas. Tumor and osteoclast-like giant cells were scattered. Much lymphovascular permeation was seen. In a few areas, particularly on the tumor periphery, there were merges between the tumor cells and myoepithelial cells of the non-tumorous ducts, as if the tumor emanated from the duct myoepithelium. The tumor was invasive into the skin and pectoral muscle. Immunohistochemically, the tumor cells were diffusely positive for vimentin, CD10, α-smooth muscle antigen, and Ki-67 (labeling = 95%). The significant areas of the tumor were positive for S100 protein, p63, p53, CD68, caldesmon, desmin and TGFβ1. A few areas were positive for pancytokeratin (AE1/3), cytokeratin (CK) 5/6, and CK 34βE12. In contrast, the tumor cells were negative for pancytokeratins (WSS, CAM5.2), CK7, CK8, CK14, CK18, CK19, CK20, EMA, CEA, bcl-2, myoglobin, CD34, CD56, CD45, HMB45, GFAP, α-1-antitrypsin, synaptophysin, estrogen receptor, progesterone receptor, HER2/neu, MUC1, MUC2, MUC5AC and MUC6. The author diagnosed the tumor as malignant myoepithelioma, as myoepithelial markers (C10, p63, S100 protein, α-smooth muscle actin, caldesmon) were positive, and also because there was a transition between the tumor cells and myoepithelium of non-tumorous ducts. The grade of the tumor was high. The patient was treated with chemoradiation and was free of disease 5 months after the operation.

Primary Malignant Giant-Cell Tumor of Bone Has High Survival Rate

Primary Malignant Giant-Cell Tumor of Bone Has High Survival Rate

Malignant Osteoclast-like Giant Cell Tumar of the Kidney with Osteosarcomatous Transformation

Malignant Osteoclast-like Giant Cell Tumar of the Kidney with Osteosarcomatous Transformation

Endometrial Adenocarcinoma With a Component of Giant Cell Carcinoma

We report six high-grade endometrial adenocarcinomas with a malignant giant cell component. The patients ranged in age from 43 to 85 years (mean 65); five were postmenopausal. All the patients presented with vaginal bleeding. The tumors all had a giant cell component that was composed of poorly cohesive sheets and nests of bizarre multinucleated giant cells admixed with an approximately equal number of mononucleate tumor cells. A sarcomatoid pattern and a marked inflammatory infiltrate were each present in three cases. All the tumors contained at least focal areas of endometrial adenocarcinoma of one of the usual types. Occasional malignant giant cells were positive for cytokeratins (AE1/AE3 or CAM 5.2) and epithelial membrane antigen in all three tumors tested. Three tumors were in stage I, one stage III and two stage IV. Two of the patients with stage I disease (each with superficial myometrial invasion) were alive and well 6 and 2 years later. Of the remaining four patients, three died of disease 2.5 years, 6 months, and 5 months after presentation, and one was alive with extensive abdominal disease after 1 year. Giant cell carcinoma of the endometrium is an aggressive tumor that should be distinguished from other endometrial tumors with a prominent giant cell component, including trophoblastic tumors, certain primary sarcomas, and malignant mixed müllerian tumors. It is imperative that malignant giant cells and non-neoplastic giant cells not be confused, as is possible in a curettage or biopsy specimen.

Histological and Immunohistochemical Observations of Dedifferentiated Leiomyosarcoma of the Uterus

A case of dedifferentiated leiomyosarcoma of the uterus was examined using immunohistochemistry. The tumor arose in the myometrium, and was a whitish large nodule with hemorrhage and necrosis. Histologically it was a well differentiated leiomyosarcoma with foci showing epithelioid pattern, and in part resembling malignant fibrous histiocytoma (MFH) and giant cell tumor (GCT). Additionally, small round neoplastic cells arranged in an alveolar manner, simulating alveolar rhabdomyosarcoma, were seen in some areas. Neoplastic cells in well differentiated areas expressed desmin, muscle-specific actin and LeuM1, whereas those in epithelioid and poorly differentiated areas lacked these antigens. Instead, tumor cells in epithelioid and small round cell areas were positive for keratin. Interestingly, most tumor cells in well differentiated, epithelioid and small round cell areas were also positive for MB1. However, tumor cells in GCT- and MFH-like areas reacted with none of the antibodies used. Ultrastructurally, some tumor cells possessed various amounts of microfilaments with or without dense patches, whereas others lacked them. These findings suggest that the divergent antigen expression was attributable to different levels of differentiation, and that poorly differentiated components had lost their native features.

ENZYME HISTOCHEMICAL INVESTIGATIONS ON BONE AND SOFT TISSUE TUMOURS

Histochemical staining for three hydrolytic enzymes were performed in 35 bone tumours and 43 soft tissue tumours, malignant as well as benign. Osteosarcoma, intra-osseous as well as extra-osseous, revealed characteristic rich staining for alkaline phosphatase, no matter how dedifferentiated the tumour was. Haemangioendothelioma (and normal endothelium), too, showed strong reaction for alkaline phosphatase whereas haemangiopericytoma did not. Alkaline phosphatase furthermore was found in slight to moderate amounts in fibrous proliferations. All other tumours examined were negative. Acid phosphatase was found in almost every tumour investigated except Ewing sarcoma and chondromyxoid fibroma. However, high activity was characteristic of giant cell tumours and malignant fibrous histiocytoma. The inhibition of acid phosphatase by tartrate was complete except in osteosarcoma and giant cell tumours, where only a partial inhibition was seen. There were non-specific esterase reactions in a variety of tumours, but very strong reactions were characteristic of malignant fibrous histiocytoma and giant cell tumours. The reaction could be completely inhibited by the addition of fluoride. In an era of increasing application of immunohistologic techniques in surgical pathology it might be of value to remember that simple enzyme histochemical stainings may provide helpful diagnostic features in the classification of bone and soft tissue tumours.

The ultrastructure of osteosarcoma. A review of twenty cases.

The ultrastructural features of 20 cases of osteosarcoma are discussed. Characteristically, the malignant cells (osteoblasts) contained large quantities of dilated, anastomosing rough endoplasmic reticulum, ofter forming large lakes. Mitochondria were sparse. Similar features are seen in osteoblasts in normal developing bone. Marked ultrastructural similarities of cells from chondrosarcomatous, fibrosarcomatous and typical osteosarcomatous areas of these tumors strongly supports the concept that these neoplastic cells all arise from the same progenitor cell. Malignant giant cells were readily differentiated from benign osteoclasts. The most significant ultrastructural findings were the varying degrees of calcification in the intercellular areas (osteoid), ranging from an extensive dense deposition of hydroxyapatite crystals which obscured the underlying collagen fibers, to focal collectors, or puffs, of slender needle-shaped hydroxyapatite crystals deposited on or along collagen fibers. The earliest apparent evidence of calcification was the presence of small electron-dense particles on or in collagen fibers.