지방섬유종증
Lipofibromatosis - A Case Report -
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Lipofibromatosis is a recently described rare benign fibrofatty tumor of childhood. It typically forms as an ill defined, slowly growing, painless mass. We present here the case of lipofibromatosis that occurred in a 21-year-old male who had complained of a bulging enlarged mass involving the right thigh and prepatella area for the previous 1 year. Magnetic resonance imaging showed an ill-defined reticular infiltration in the subcutaneous layer with subtle linear enhancement and high T2 signal intensity. The mass was surgically excised and it displayed an 11.0×5.5×1.5 cmsized adipose appearance without encapsulation. Microscopically, the tumor was composed of alternating streaks of mature adipose tissue and a fibroblastic component that mainly involved the septa of adipose tissue. On immunohistochemical study, the fibroblastic component was positive for S-100, CD99, CD34, actin and bcl-2. He has shown an eventful recovery for 6 months after surgery.
Abstract
Lipofibromatosis is a recently described rare benign fibrofatty tumor of childhood. It typically forms as an ill defined, slowly growing, painless mass. We present here the case of lipofibromatosis that occurred in a 21-year-old male who had complained of a bulging enlarged mass involving the right thigh and prepatella area for the previous 1 year. Magnetic resonance imaging showed an ill-defined reticular infiltration in the subcutaneous layer with subtle linear enhancement and high T2 signal intensity. The mass was surgically excised and it displayed an 11.0×5.5×1.5 cmsized adipose appearance without encapsulation. Microscopically, the tumor was composed of alternating streaks of mature adipose tissue and a fibroblastic component that mainly involved the septa of adipose tissue. On immunohistochemical study, the fibroblastic component was positive for S-100, CD99, CD34, actin and bcl-2. He has shown an eventful recovery for 6 months after surgery.
참고문헌 (20)
- Fetsch JF, "A clinicopathologic study of 45 pediatric soft tissue tumors with an admixture of adipose tissue and fibroblastic elements, and a proposal for classification as lipofibromatosis", Am J Surg Pathol, 24, pp.1491~1500 (2000)
- Miettinen M, "Lipofibromatosis, in World Health Organization classification of tumours: pathology and genetics of tumours of soft tissue and bone", pp.85~ (2002), IARC
- Deepti AN, "Lipofibromatosis: report of a rare paediatric soft tissue tumour", Skeletal Radiol, 37, pp.555~558 (2008)
- Greene AK, "Diffuse lipofibromatosis of the lower extremity masquerading as a vascular anomaly", Ann Plast Surg, 62, pp.703~706 (2009)
- Kenney B, "Chromosomal rearrangements in lipofibromatosis", Cancer Genet Cytogenet, 179, pp.136~139 (2007)
- Fletcher CD, "Peripheral nerve sheath tumors: a clinicopathologic update", Pathol Annu, 25 Pt 1, pp.53~74 (1990)
- Puri R, "Solitary neurofibroma of the larynx", Otolaryngol Head Neck Surg, 117, pp.713~714 (1997)
- Koc C, "Bilateral ventricular neurofibroma of the larynx", J Laryngol Otol, 110, pp.385~386 (1996)
- O’Sullivan MJ, "Malignant peripheral nerve sheath tumors with t(X;18): a pathologic and molecular genetic study", Mod Pathol, 13, pp.1336~1346 (2000)
- Falahatkar S, "First reported case of adrenal neurofibroma in Iran", Urol J, 4, pp.242~244 (2007)
- Sasaki D, "Lipofibromatosis arising in a pediatric forearm: a case report", Ups J Med Sci, 110, pp.259~266 (2005)
- Weiss SW, "Enzinger and Weiss’ soft tissue tumors. 5th ed", pp.431~434 (2008), CV Mosby
- Collins MH, "Lipoblastoma", lipoblastomatosis: a clinicopathologic study of 25 tumors, 1997, pp.21, 1131~1137
- Weiss SW, "Enzinger and Weiss’s soft tissue tumors. 5th ed", pp.477~514 (2008), CV Mosby
- Elgar F, "Well-differentiated liposarcoma of the retroperitoneum: a clinicopathologic analysis of 20 cases, with particular attention to the extent of low-grade dedifferentiation", Mod Pathol, 10, pp.113~120 (1997)
- Dei Tos AP, "Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma: analysis of six cases", Am J Surg Pathol, 18, pp.913~921 (1994)
- Fletcher CD, "Fibrous hamartoma of infancy: a histochemical and immunohistochemical study", Histopathology, 12, pp.65~74 (1988)
- Kabasawa Y, "A male infant case of lipofibromatosis in the submental region exhibited the expression of the connective tissue growth factor", Oral Surg Oral Med Oral Pathol Oral Radiol Endod, 103, pp.677~682 (2007)
- Taran K, "Lipofibromatosis presenting as a neck mass in eight-years old boy: a case report", Pol J Pathol, 59, pp.217~220 (2008)
- Lassmann H, "Some electron microscopic and autoradiographic results concerning cutaneous neurofibromas in von Recklinghausen’s disease", Arch Dermatol Res, 255, pp.69~81 (1976)