Deeply located low-grade fibromyxoid sarcoma with FUS-CREB3L2 gene fusion in a 5-year-old boy with review of literature
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CASE REPORT
Open Access
Deeply located low-grade fibromyxoid sarcoma with FUS-CREB3L2 gene fusion in a 5-year-old boy with review of literature Aiko Kurisaki-Arakawa1, Yoshiyuki Suehara2, Atsushi Arakawa1, Tatsuya Takagi2, Michiko Takahashi1, Keiko Mitani1, Kazuo Kaneko2, Takashi Yao1 and Tsuyoshi Saito1*
Abstract Background: Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft tissue tumor typically affecting young to middle-aged adults. Despite its otherwise benign histologic appearance and indolent nature, it can have fully malignant behavior, and recurrence and metastasis may occur even decades later. Case history: We report a case of LGFMS in the left lower leg of a 5-year-old Japanese boy. A magnetic resonance imaging (MRI) uncovered a well-demarcated intra-gastrocnemial tumor measuring 27 × 20 mm with a slightly high intensity on T1WI and heterogeneously high intensity on T2WI. Histologically, the tumor was composed of bland spindle-shaped cells with a whorled growth pattern. The tumor stroma was variably hyalinized and fibromyxoid with arcades of curvilinear capillaries and arterioles with associated perivascular fibrosis. Although LGFMS is known to affect children under 18 years of age, it is extremely rare in infants and children under 5 years of age. Despite the young age, this patient was accurately diagnosed by the typical histology and the detection of a FUS-CREB3L2 gene fusion. Conclusion: Although LGFMS in children tends to be located superficially, this case presented with an intramuscular tumor in the region of the gastrocnemius. To the best of our knowledge, this is the first case of deep LGFMS arising in a child younger than 5 years of age. The patient is still alive with no evidence of the disease 4 months after diagnosis. Virtual Slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/ 13000_2014_163 Keywords: Low-grade fibromyxoid sarcoma, FUS-CREB3L2, Fusion gene, Young patients
Background Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft tissue tumor that typically affects young to middle-aged adults [1,2]. The median age of onset for LGFMS is 34 years [3], although patients of any age can be affected, and 13-19% of cases occur in patients 18 years and younger [3,4]. However, LGFMS is rare in children during the first 5 years of life [2]. To the best of our knowledge, the youngest case of LGFMS is a tumor arising from the cheek in a 22-month-old girl [4]. Histologically, LGFMS is composed of bland spindle-shaped cells * Correspondence: [email protected] 1 Department of Human Pathology, Juntendo University School of Medicine, Bunkyo-ku, Tokyo, Japan Full list of author information is available at the end of the article
in a whorled growth pattern, arranged in alternating myxoid and collagenized areas along with curvilinear capillaries and characteristic arterioles with perivascular fibrosis. Although most of the tumor cells show benign histological appearance, approximately 10% of the tumor may have scattered large, hyperch
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