• Archives of Plastic Surgery
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• 제45권5호
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• pp.479-483
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• 2018

Malignant peripheral nerve sheath tumor (MPNST) is a very rare type of sarcoma, with an incidence of 0.001%. MPNST has a 5-year survival rate near 80%, so successful reconstruction techniques are important to ensure the patient's quality of life. Sarcoma of the forearm is known for its poor prognosis, which leads to wider excision, making reconstruction even more challenging due to the unique anatomical structure and delicate function of the forearm. A 44-year-old male presented with a large mass that had two aspects, measuring $9{\times}6cm$ and $7{\times}5cm$, on the dorsal aspect of the right forearm. The extensor compartment muscles (EDM, EDC, EIP, EPB, EPL, ECRB, ECRL, APL) and invaded radius were resected with the mass. Tendon transfer of the entire extensor compartment with skin defect coverage using a $24{\times}8cm$ anterolateral thigh (ALT) perforator free flap was performed. The patient was discharged after 18 days without wound complications, and has not complained of discomfort during supination, pronation, or wrist extension/flexion through 3 years of follow-up. To our knowledge, this is the first report of successful reconstruction of the entire forearm extensor compartment with ALT free flap coverage after resection of MPNST.

• 대한두경부종양학회지
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• 제26권1호
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• pp.37-40
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• 2010

Ectopic hamartomatous thymoma is a rare benign tumor of the lower neck occurring in the male adult predominantly. The origin of this tumor has been debated, but it is now believed to arise from remnants of the cervical sinus of His from early development. They are composed of epithelial, adipocytic, and spindle cells in variable amounts. Recognition of ectopic harmatomatous thymoma is important and needs to be differentiated from high-grade sarcomas such as synovial sarcoma or glandular malignant peripheral nerve sheath tumor. We here report on a case of ectopic hamartomatous thymoma arising in the left lateral neck of 33-year-old male patient.

• Clinical and Experimental Pediatrics
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• 제57권9호
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• pp.410-415
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• 2014

Purpose: Neurofibromatosis 1 (NF1) is an autosomal dominant condition caused by an NF1 gene mutation. NF1 is also a multisystem disorder that primarily affects the skin and nervous system. The goal of this study was to delineate the phenotypic characterization and assess the NF1 mutational spectrum in patients with NF1. Methods: A total of 42 patients, 14 females and 28 males, were enrolled in this study. Clinical manifestations and results of the genetic study were retrospectively reviewed. Results: Age of the patients at the time of NF1 diagnosis was $15.8{\pm}14.6$ years (range, 1-62 years). Twelve patients (28.6%) had a family history of NF1. Among the 42 patients, $Caf\acute{e}$-au-lait spots were shown in 42 (100%), neurofibroma in 31 (73.8%), freckling in 22 (52.4%), and Lisch nodules in seven (16.7%). The most common abnormal finding in the brain was hamartoma (20%). Mental retardation was observed in five patients (11.9%), seizures in one patient (2.4%), and plexiform neurofibromas (PNFs) in four patients (9.5%). One patient with PNFs died due to a malignant peripheral nerve sheath tumor in the chest cavity. Genetic analysis of seven patients identified six single base substitutions (three missense and three nonsense) and one small deletion. Among these mutations, five (71.4%) were novel (two missense mutations: p.Leu1773Pro, p.His1170Leu; two nonsense mutations: $p.Arg2517^*$, $p.Cys2371^*$; one small deletion: $p.Leu1944Phefs^*6$). Conclusion: The clinical characteristics of 42 Korean patients with NF1 were extremely variable and the mutations of the NF1 gene were genetically heterogeneous with a high mutation-detection rate.