• The Journal of the Korean bone and joint tumor society
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• v.10 no.2
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• pp.130-133
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• 2004

Low-grade fibromyxoid sarcoma (LGFMS) is very rarely seen, and it commonly arises from the deep soft tissues of the lower extremities. Histologically, it is characterized by the presence of bland spindle cells with mainly storiform pattern of growth, set in alternating areas with a myxoid or fibrous stroma. The immunohistochemical reaction of this tumor permits a positive diagnosis of low grade fibromyxoid sarcoma and allows its distinction from a number of other benign and malignant soft tissue tumor. We report this rare case together with the review of the literature.

• The Journal of the Korean bone and joint tumor society
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• v.14 no.2
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• pp.152-156
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• 2008

Osteochodromas are the most common benign osseous neoplasm, covered by a distinct hyaline cartilage cap, originating from the physis. They involve typically metaphyseal or metadiaphyseal region of a long bone, while they involve the foot rarely. In the foot, the osteochondromas mostly occur in short tubular bones such as metatarsal bones and phalanges. They are seldom found in calcaneus. We report a rare case of osteochondroma involving calcaneus.

• The Journal of the Korean bone and joint tumor society
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• v.20 no.2
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• pp.99-103
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• 2014

Li-Fraumeni syndrome (LFS) is an autosomal dominant hereditary disorder characterised by a variety of different tumor types in children and young adults. That contains with a germline mutation in the tumor suppressor gene Tumor Protein p53 (TP53). That is extremely rare. Furthermore, this is sometimes overlooked. Here, we report a case of LFS which was confirmed by mutational analysis of the p53 gene. Also, literature review is intended to improve understanding of this disease entity.

• The Journal of the Korean bone and joint tumor society
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• v.20 no.2
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• pp.80-84
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• 2014

Inclusion body fibromatosis is a rare benign soft tissue neoplasm typically involving fingers and toes of children in mostly less than one year old. Histologic findings include spindle-shaped fibroblasts surrounded by dense stroma and small perinuclear eosinophilic inclusions in the cytoplasm. Although the tumor typically undergoes spontaneous regression, surgery is considered when functional impairment or deformity develops with the lesion. Unfortunately, recurrence rate was reported to be as high as 60 % following tumor excision. Authors would like to present our case where the tumor occurred in relatively older child and kissing lesion was found a few months after the surgery.

• The Journal of the Korean bone and joint tumor society
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• v.13 no.2
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• pp.180-184
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• 2007

The etiology of post-traumatic, and presumably post-fracture, osteolysis of the pubic bone is unknown, although the pathological process is similar to bony destruction related to bone tumor or osteomyelitis. We have seen and wish to report one patient in whom destruction of the pubic bone was a prominent feature of the healing process following trauma.

• The Journal of the Korean bone and joint tumor society
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• v.16 no.1
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• pp.27-36
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• 2010

Purpose: Pigmented villonodular synovitis (PVNS) is a rare soft tissue tumor, which usually arises in larger joints, such as the knee. It has a high recurrence rate after surgical treatment. The purpose of this study is to evaluate and analyze the clinical results of diffuse-type pigmented villonodular synovitis cases that were treated with open total synovectomy. Materials and Methods: Between 1994 and 2006, 21 patients who had diffuse-type pigmented villonodular synovitis were selectively reviewed. Among the 21 cases studied, 14 patients presented at the knee, 5 at the ankle, and 2 at the shoulder and elbow. The mean follow up period was 5.5 years (range, 36-157 months). The average age of the patients was 34 years consist of 7 men and 14 women. Clinical outcomes were analyzed retrospectively, including range of motion and complications. Results: Open total synovectomy and adjuvant electrocautrization were done in all cases except one. During the regular follow-up period after the surgery, two patients showed symptoms of recurrence. After re-operation, only one case was pathologically confirmed as a recurrence. The patient who had partial synovectomy and the other patient who had second operation due to recur rence received additional radiation therapy. Clinical outcome scores were improved in every aspect (p<0.0001). 2 out of 14 Patients who had pigmented villonodular synovitis at the knee developed stiff knee after the surgery. Conclusion: After the open total synovectomy with electrocautrization, a low recurrence rate and satisfactory clinical outcome was achieved, observed in a minimum of 3 years of follow-up.

• The Journal of the Korean bone and joint tumor society
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• v.9 no.1
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• pp.45-51
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• 2003

Purpose: This study was designed to know the ability of thallium-201 scintigraphy to discriminate malignant bone tumor from benign by analysing the quantitative thallium uptake ratio. Materials and Methods: We took thallium-201 scintigraphy prospectively with other imaging studies in 82 bone tumor suspecting patients. The results of scintigraphy were read qualitatively and calculated quantitatively, and retention indexes were estimated. For the statistical analysis the patients were divided as four group; high grade malignant bone tumor, benign bone tumor, giant cell tumor and low grade malignant bone tumor. Results: The mean thallium uptake ratio was 4.14 in early phase and 2.26 in delayed phase in high grade malignant bone tumor group, 1.16 and 1.09 in benign bone tumor, 3.15 and 1.94 in giant cell tumor, and 1.41 and 1.31 in low grade malignant bone tumor. Retention indexes were 0.62, 0.97, 0.66, 0.93 in same order. The thallium uptake ratio and retention indexes were statistically correlated in high grade malignant bone tumor and benign bone tumor group(p<0.001). Conclusion: Thallium-201 scintigraphy proved as useful imaging study to discriminate malignant bone tumor from benign, but had exception in giant cell tumor and low grade malignant bone tumors.

• The Journal of the Korean bone and joint tumor society
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• v.9 no.1
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• pp.77-83
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• 2003

Aggressive fibromatosis is a rare soft tissue tumor with locally invasive and infiltrative characteristics. The mechanism of this invasive nature was not reported until now. Mutations or reduction of PTEN, tumor suppressor gene, in cancer tissues, have been found to be associated with invasiveness and metastatic properties of cancer cells. To know the pattern of expression of PTEN in aggressive fibromatosis, we analysed the expression of PTEN with immunohistochemical stain and immunoblotting. PTEN was homogeneously expressed in the normal musculoaponeurotic tissues, but absent or very faint in tissues of patients with aggressive fibromatosis as evidenced by western blot analysis and immunohistochemical examinations. Although the meaning of decreased PTEN expression in aggressive fibromatosis is not certain, it might be involved in the growth of the aggressive fibromatosis, and associated with phenotype of aggressive fibromatosis.

• The Journal of the Korean bone and joint tumor society
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• v.6 no.4
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• pp.173-177
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• 2000

Malignant rhabdoid tumor is a highly aggressive tumor of children, that often arises in the kidney. Some rhabdoid tumors have been reported in various extra-renal location including the central nervous system, liver, skin, and soft tissues. In case of arising in soft tissues, it may be misdiagnosed as rhabdomyosarcoma. It is important to distinguish malignant rabdoid tumor from rhadomyosarcoma, because malignant rhabdoid tumor has more aggressive behavior and poorer survival rate. And this differential diagnosis can be performed by several immunohistochemistry. Here we report a case of malignant rhabdoid tumor that arose in lower abdominal wall with related articles.

• The Journal of the Korean bone and joint tumor society
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• v.8 no.3
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• pp.69-75
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• 2002

Werner's syndrome is a rare autosomal recessive disorder manifesting as premature aging. It is also known to be characterized by a high frequency of malignant tumors, especially sarcomas. However, Werner's syndrome may be not only a premature aging disease but also a cancer syndrome, because the malignant tumors in these patients are different from those of normal population with respect to involved site, histological type, and age of onset. Recent studies found Werner's syndrome was caused by a mutation of Werner helicase suggesting that WRN helicase may participate in metabolism and repair of DNA. And a dysfunction of WRN helicase may induce the genomic instability causing somatic mutations. Further studies of Werner's syndrome associated with sarcoma might give much informations about the normal aging process and the pathogenesis of sarcomas.