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KMID : 0374920080290010127
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Inje Medical Journal
2008 Volume.29 No. 1 p.127 ~ p.132
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A Rare Case of Plexiform Fibrohistiocytic Tumor
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Kwon Soon-Sung
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Abstract
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Objective: Plexiform fibrohistiocytic tumor is a rare mesenchymal neoplasm of intermediate malignancy. It has a predilection for children and young adults but can occur at any age. The tumor usually involves the upper limbs as a slow-growing, painless mass. Histologically, the tumor is characterized by poorly demarcated dermal or subcutaneous mass with multinodular plexiform growth and fibrohistiocytic cytomorphology. There are three distinct recognized growth patterns: fibrohistiocytic, fibroblastic, and mixed types. The tumor displays uniform immunoreactivity for vimentin and CD68. Only 3 cases are reported in Korea and we present a case of plexiform fibrohistiocytic tumor with literature review.
Methods: An 8-year?old female presented an reddish indurate mass on left chest wall. Considering the linear shape the mass was excised elliptically with safety margin 1cn under the impression of mastocytoma or hemangioma. The specimen was sent to pathologic department and the defect was repaired primarily.
Results: Pathologic report confirmed plexiform fibrohistiocytic tumor and all margins were negative. There were no evidences of recurrence after surgical excision. Local lymph node enlargement and lung metastasis were not detected for 10 month follow up period.
Conclusion: The tumor has a high local recurrence rate but metastasizes only rarely. Complete surgical resection of the tumor, preferably with wider margins, is required to prevent local recurrence. Long-term follow-up is necessary to detect any nodal or pulmonary metastasis.
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KEYWORD
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Plexiform fibrohistiocytic tumor
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