Giant cell fibroblastoma in a 3-year-old boy

Giant cell fibroblastoma (GCF) is a rare soft tissue tumor most often discovered during the first two decades of life. We present a case of a 3- year- old boy with a history of a recurrent lesion in the knee, the tumor growth progressively and enlarged to 2.1 cm in the previous two years before diagnosis. It involved the subcutaneous tissue, had infiltrative borders and extended into the superficial dermis. The tumor was surgically excised with free margins. There was no evidence of local recurrence, and a metastatic workup was negative after 10 years of follow up. We review herein the clinicopathologic features, histogenesis, differential diagnosis and relationship to dermatofibrosarcoma protuberans (DFSP).     

Giant cell fibroblastoma

Two cases of giant cell fibroblastoma in children are described and the available literature on these uncommon neoplasms is summarized. Several local recurrences were noticed in both of our cases, but no metastases occurred. The histologic appearance was distinctive, but no firm conclusions regarding histogenesis could be drawn. Immunostaining methods ruled out a vascular origin. The evidence was not sufficient enough to either confirm or refute a neoplastic histiocytic component of these tumors. The clinical course and pathologic findings suggested that these lesions be classified tentatively with the fibromatoses of childhood.     

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.     

Giant polypoid basal cell carcinoma

A giant polypoid basal cell carcinoma occurring in the back of a 64-year-old man is reported. Three other such cases from the English literature are reviewed.     

胆汁中CEA浓度测定在大肠癌肝转移诊断中的意义

We investigated the clinical features of soft-tissue recurrence in giant cell tumor of bone (GCTB). Among 106 cases with GCTB in our hospital, there were 2 cases occurring soft-tissue recurrence which histories were reported. These two soft-tissue recurrences occurred with the interval of 6.9 years and 2.5 years respectively from primary diagnosis. The clinical presentation was nonspecific masses in soft tissue. Radiographic ossification was not found at periphery or within the masses. Through pathological examination peripheral ossification was found in 1 case and malignant transformation occurred in the other case. Through retrieving and reviewing literatures in PubMed, 19 cases of soft-tissue recurrence with detailed materials were collected and analyzed. Soft-tissue recurrence of GCTB is a rare episode which reflects its locally aggressive nature, the reasons of which are tumor cells implantation and tumor residual. Ossification at periphery or in the masses can be considered as a pathognomonic character of this episode in radiographic and pathohistological examination. The prevention lies in determining tumor extension preoperatively, proper non-tumor manipulations, removing the tumor and irrigating operative wound as completely as possible.     

Giant cell tumour of the tendon sheath of the hand: A pictorial essay

Giant cell tumour of the tendon sheath is a soft tissue mass found occasionally in the hand. Its diagnosis can be readily made preoperatively if the characteristic MRI features are appreciated. This pictorial essay demonstrates and describes the imaging findings correlated with histopathological findings in a group of patients with proven giant cell tumour of the tendon sheath.     

Treatment with steroids of a giant cell granuloma of the maxilla

This case report of giant cell granuloma involving the maxilla is of particular interest for two reasons: the locally aggressive clinical course contrasting with the diagnosis of a benign disease and the spectacular, although transient, response under steroid treatment. Corticotherapy should be further tested in cases of invasive or recurring giant cell granuloma.     

Pleomorphic giant cell carcinoma of the pancreas: A review of nine cases

Nine cases of pleomorphic giant cell carcinoma of the pancreas collected over a 26-year period are analyzed. The tumor presents a distinctive histology. The head of the pancreas appears to be the predominant site of the lesions in this study, and the cell origin is considered to be the ductal epithelium. Its clinical manifestation and rapid, fatal course of usually two months are almost indistinguishable from the more conventional pancreatic adenocarcinoma.     

Giant cell fibroblastoma. A juvenile form of dermatofibrosarcoma protuberans

A clinicopathologic analysis of 28 cases of giant cell fibroblastoma (GCF), a rare mesenchymal tumor occurring predominantly in the first decade of life, is presented. This disease presented as a painless, slowly enlarging, subcutaneous mass. The tumor recurred locally in 47% of the patients; however, metastasis was not detected. On microscopic examination, GCF showed an unique combination of spindle cell patterns, pleomorphic and multinucleated giant cells, myxoid areas, and distinctive sinusoid-like spaces. This unrecognized histomorphologic picture led to a misdiagnosis of sarcoma in 40% of the cases. The histogenesis of this lesion remains uncertain; however, based on both clinical and morphologic similarities, it is proposed that GCF is a juvenile form of dermatofibrosarcoma protuberans (DFSP).     

Giant cell tumour of the temporal bone: Case report and review of the literature

This paper presents an expansile lesion of the temporal bone, in a 14-year-old boy, that was initially diagnosed as intra-osseous meningioma from CT appearance. At histopathology a final diagnosis of giant cell tumour was made. A brief review of the literature is presented for this rare case.     

Giant cell glioblastoma: A glioblastoma subtype with distinct epidemiology and superior prognosis

Giant cell glioblastoma (GC) is an uncommon subtype of glioblastoma multiforme (GBM). Consequently, the epidemiology, natural history, and factors associated with outcome are not well defined. Patients diagnosed with GC from 1988 through 2004 were identified in the Surveillance, Epidemiology, and End Results (SEER) database. Outcomes were examined with Kaplan-Meier survival analysis and Cox models. For comparison, similar analyses were conducted for patients diagnosed with GBM. GC was identified in 1% of 16,430 patients diagnosed with either GC or GBM. Compared with GBM, GC showed similar gender and racial distributions. Likewise, tumor size and location were not significantly different between the two histologies. GC tended to occur in younger patients with a median age at diagnosis of 51 years, compared with 62 years for GBM. Additionally, patients with GC were more likely to undergo complete resection compared with patients with GBM. For both histologies, young age, tumor size, extent of resection, and the use of adjuvant radiation therapy (RT) were associated with improved survival. Cox modeling suggests the prognosis for GC is significantly superior to that for GBM (hazard ratio = 0.76; 95% confidence interval, 0.59–0.97) even after adjustment for factors affecting survival. GC is an uncommon GBM subtype that tends to occur in younger patients. Prospective data defining optimal treatment for GC are unavailable; however, these retrospective findings suggest that resection, as opposed to biopsy only, and adjuvant RT may improve survival. The prognosis of GC is superior to that of GBM, and long-term survival is possible, suggesting aggressive therapy is warranted.     

16例巨大型胃肠间质瘤手术治疗分析

目的：通过对巨大型胃肠间质瘤（GIST）的诊断和手术方式进行探索,为临床治疗巨大型 GIST 提供新思路。方法对16例巨大型 GIST 患者一般资料、影像学资料、手术方式、随访指标进行回顾性调查,并进行统计学分析。结果11例服用甲磺酸伊马替尼患者无复发生存期1 a 以上,而5例未服用伊马替尼治疗患者1 a 内复发,中位无复发生存期为33个月。COX 多因素分析显示,肿瘤部位、术中肿瘤有无破裂、是否使用伊马替尼治疗与患者预后有关（P <0.05）,可作为患者预后的独立因素。结论巨大型 GIST 在选择手术时,应遵循术前影像学评估、术前服用甲磺酸伊马替尼治疗缩小肿瘤和手术切除时边缘切净为3大原则,这对于改善患者预后和延长生存期有益。     

A study of soft tissue sarcomas after childhood cancer in Britain

Among 16 541 3-year survivors of childhood cancer in Britain, 39 soft tissue sarcomas (STSs) occurred and 1.1 sarcomas were expected, yielding a standardised incidence ratio (SIR) of 16.1. When retinoblastomas were excluded from the cohort, the SIR for STSs was 15.9, and the cumulative risk of developing a soft tissue tumour after childhood cancer within 20 years of 3-year survival was 0.23%. In the case-control study, there was a significant excess of STSs in those patients exposed to both radiotherapy (RT) and chemotherapy, which was five times that observed among those not exposed (P=0.02). On the basis of individual radiation dosimetry, there was evidence of a strong dose-response effect with a significant increase in the risk of STS with increasing dose of RT (P<0.001). This effect remained significant in a multivariate model. The adjusted risk in patients exposed to RT doses of over 3000 cGy was over 50 times the risk in the unexposed. There was evidence of a dose-response effect with exposure to alkylating agents, the risk increasing substantially with increasing cumulative dose (P=0.05). This effect remained after adjusting for the effect of radiation exposure.     

A percutaneous instrument for biopsy of soft tissue masses

The communication describes a curved needle which is suitable for obtaining biopsy specimens from various types of soft tissue masses. In 37 out of 40 consecutive patients, a specimen satisfactory for pathologic diagnosis was obtained. No complications occurred in its use.     

Giant Cell Tumor of the Posterior Arch of the Atlas

Two rare cases of giant cell tumor involving the posterior arch of the atlas vertebra are presented.     

Alveolar soft part sarcoma in children and adolescents: A report from the Soft-Tissue Sarcoma Italian Cooperative Group

Background:Alveolar soft part sarcoma (ASPS) is a rare malignanttumor and little is known about its clinical features and management. Wereport on a series of 19 pediatric patients managed over 20 years. Patients and methods:Primary conservative surgery was performedin all patients and was radical in nine, non-radical in three; seven patientsunderwent biopsy alone (3 unresectable tumors, 4 metastatic disease). In twocases radical surgery was performed after primary chemotherapy. Radiotherapywas delivered to 8 patients, chemotherapy to 15. Results:After a median follow-up of 74 months, the five-yearsurvival was 80% for the whole series, 91% for patients withlocalized disease, 100% for patients with tumor 5 cm, and31% for those >5 cm; 16 of 19 patients were alive (12 of 12 withgrossly-resected tumor in first continuous remission). Chemotherapy achievedtwo partial remission among seven evaluable patients. Conclusions:Pediatric ASPS has a more favorable prognosis thanits adult counterpart. In this series, tumor size correlates with metastaticdisease at onset and is the major factor influencing survival. Surgery is themainstay of therapy. The effectiveness of adjuvant therapy remains to beestablished, though radiotherapy may be advisable in cases of inadequatesurgery.