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1 KISEP Case Report Korean J Otolaryngol 2005;48:823-7 두경부에생긴과립세포종 9 예 송형민 남순열 김상윤 최승호 Granular Cell Tumors of the Head and :A Case Report of 9 Cases Hyung Min Song, MD, Soon Yuhl Nam, MD, Sang Yoon Kim, MD and Seung-Ho Choi, MD Department of Otolaryngology, University of Ulsan College of Medicine, Seoul, Korea ABSTRACT Granular cell tumor () is a rather uncommon neoplasm. Though s are usually small, solitary and benign in most cases, a small number (1-2%) of cases are malignant. They can originate from anywhere in the body but are most frequently found in the head and neck, particularly in an oral cavity. Histopathologically, is composed of large, round, or polygonal cells with small dense nuclei. Cytoplasm contains abundant eosinophilic granules. In an immunohistochemical study, granules of are positive for S-100 protein. Surgical intervention, wide excision with enough margin, is the treatment modality of choice. Inadequate resection of a tumor may result in local recurrence, even with a benign. We have experienced 9 cases of between December 1997 and June 2003, and report on them with a review of literatures. (Korean J Otolaryngol 2005;48:823-7) KEY WORDS:Granular cell tumor Head and neck neoplams. 823

2 두경부에 생긴 과립세포종 Table 1. Patient clinical features Case Sex Age Chief complaint 1 2 mo Gingiva mass Pedunculated mass P/E Gingiva Location Diameter 0.1 cm Benign mass Clinical Dx 2 10 mass Whitish hard mass 0.5 cm cyst 3 25 mass Nontender hard mass 0.7 cm ibroma 4 44 Incidental Whitish papillomatous mass A-E* fold 0.6 cm Papilloma 5 M 46 mass Hyperkeratotic mass 0.1 cm Pilomatricoma 6 50 acial pain Nasal cavity mass MS & NC 7 61 Parotid mass irm fixed mass Parotid 2.4 cm Parotid tumor 8 M 63 Incidental Whitish polypoid mass Vocal cord 0.3 cm Contact granuloma 9 66 MS cancer mass Nontender mass 1.2 cm *A-E aryepiglottic, MS maxillary sinus, NC nasal cavity, Tbc LAP tuberculous lymphadenopathy Tbc LAP Table 2. Pathology and surgical approach Case Location NAB Treatment Pathology S Gingiva Congenital Negative 2 3 Excision Negative 4 A-E* fold Laryngomicro surgery with laser 5 6 MS & NC mass Subtotal resection craniotomy 7 Parotid Parotid acinic cell carcinoma Superficial parotidectomy 8 Vocal cord Laryngomicro surgery with laser 9 Neoplasm, unspecified Excision *A-E aryepiglottic, MS maxillary sinus, NC nasal cavity, granular cell tumor 산화 단층촬영 소견상 좌측 이하선 내에 경계가 좋고 조영 증강이 잘되는 2 2 cm 크기의 종괴가 관찰되었으며 다형 선종(pleomorphic adenoma)과 같은 양성 종양이 의심되 었다(ig. 1). 세침흡인검사에서 과립 세포질을 가진 상피 세포가 관찰되었으며, 선방세포암종(Acinic cell carcinoma) 로 추정 진단하였다. 절제생검을 위해 이하선 천엽절제술 을 시행하였으며 cm 크기의 경계가 좋은 타원형 모양의 단단한 종괴가 이하선에서 관찰되었다. H & E 염색 에서 간질조직 없이 풍부한 호산성의 거친 과립 세포질을 가진 다각형 모양의 과립세포가 있었으며 대부분의 세포핵 은 작고 진하며 유사분열이나 뚜렷한 핵소체는 관찰되지 않았다(ig. 2). 과립성 세포질은 PAS 염색에 양성이었고 ig. 1. Axial contrast-enhanced neck CT scan image of parotid. This shows a well-defined enhancing mass (white arrow). 면역조직화학 검사상 S-100 단백에 양성을(ig. 3) 보였 으며 과립세포종으로 진단되었다. 수술 후 안면마비 등의 합병증은 없었고 21개월간의 추적관찰기간 동안 재발은 증 례 1(Case 7) 없었다. 61세 여자 환자가 3개월 전부터 시작된 좌측 이하선 부 위의 종괴를 주소로 내원하였다. 과거력과 가족력상 특이 증 례 2(Case 6) 사항은 없었고, 신체 검진상 좌측 이하선 부위에 2 cm 크 46세 여자 환자가 좌측 비폐색 및 좌측 시력감소를 주소 기의 단단하고 고정된 무통성 종괴가 촉지되었다. 경부 전 로 내원하였다. 과거력상 2년 전 모 대학병원에서 좌측 상 824 Korean J Otolaryngol 2005;48:823-7

3 송형민 외 A B ig. 3. Immunohistochemical staining for S-100 protein. Tumor cells are strongly reactive for S-100 protein on immunohistochemistry ( 400). ig. 2. Histological features of granular cell tumor. A The cells do not form a distinctive pattern. Individual cells or small clusters are separated by thin bands of connective tissue (H & E, 100). B Granular cell tumor consisting of polygonal cells with small, mostly eccentrically placed nuclei and abundant coarsely granulated cytoplasm (H & E, 400). ig. 5. Coronal paranasal sinus CT scan image. A mass invaded into the left orbit (solid arrow) and palate (dash arrow). 강 되었으며 아래쪽으로 경구개와 연구개를 침범하고 있으 며 동측의 상악동 벽과 구개 그리고 치조돌기 등에 골파괴 를 초래하고 있었고 동측의 해면정맥동과 안와 그리고 후 두개와로 두개 내 침범을 보이고 있었다(igs. 4 and 5). 경구개의 종괴에 대해 절제생검을 시행하였고 과립세포종 으로 진단되었다. 이비인후과와 신경외과의 협의 수술을 계 획하고 환자에게 수술을 권유하였으나 거부하였다. 3개월 후에 의식변화를 주소로 응급실 통해 신경외과에 입원하였 다. 뇌 자기공명영상에서 3개월 전 촬영한 뇌 전산화단층촬 영과 비교하여 두개 내 병변의 크기가 커졌고 뇌간 및 종 괴 주위에 부종이 관찰되었다. 두개 내 병변에 대한 수술 ig. 4. Axial paranasal sinus CT scan image. The homogeneously well enhancing mass replaced the left nasal cavity and maxillary sinus. This mass destructed the posterior wall of left maxillary sinus and extended to the infratemporal fossa (solid arrow) and made left pterygopalatine fossa widening (dash arrow). 을 우선 시행하기로 하고 종괴에 대해 동맥 색전술을 시행 후 신경외과에서 좌측 후두하 개두술로 아전절제(subtotal resection via left suboccipital craniotomy)를 시행하였다. 수술장 소견에서 노랗고, 매우 단단한 5 7 cm 크기의 경 악동에 생긴 과립세포종으로 수술 받은 병력이 있었다. 비 계가 불분명한 종괴가 뇌조직에 단단하게 붙어있었다. 소뇌 내시경 검사에서 유두상의 종괴가 좌측 비강을 가득 채우 의 종괴는 절제하였으나 뇌간의 종괴는 남겼다. 수술 1일 고 있었으며 경구개에서도 자라난 종괴를 관찰할 수 있었 째 수두증이 생겨 뇌실복강 단락술(V-P shunt)을 시행하 다. 부비동 전산화 단층촬영에서 종괴는 균일하게 조영증 였고 이후 호전 보여 수술 12일째 퇴원하였다. 조직검사에 825

4 두경부에생긴과립세포종 Korean J Otolaryngol 2005;48:823-7

5 송형민외 REERENCES 1) Enzinger M, Weiss SW. Granular cell tumor. In: Soft Tissue Tumors, 3d ed. St. Louis: Mosby;1995. p ) Choi HS, Lim JY, Yoo JB, Kim JH, Shin EA. A case of granular cell tumor of the vocal cord. Korean J Otolaryngol 2002;45: ) Cho SH, Park KS, Woo JS, Jung KY. A case of granular cell tumor of the larynx. Korean J Otolaryngol 2002;45: ) Yang SW, Hong SW, Cho MY, Kang SJ. Malignant granular cell tumor at the retrotracheal space. Yonsei Medical Journal 1999;40: ) Vance S, Hudson RP Jr. Granular cell myoblastoma: Clinicopathologic study of forty-two patients. Am J Clin Pathol 1969;52: ) Das Gupta TK, Chaudhuri PK. Granular cell tumors. In: Tumors of the Soft Tissues. Stamford, CT: Appleton & Lange;1998. p ) Ordonez NG, Mackay B. Granular cell tumor: A review of the pathology and histogenesis. Ultrastruct Pathol 1999;23: ) Abrikossoff A. Uber Myome, ausgehend von der quergestreiften willkurlichen Muskulatur. Virhows Arch Pathol Anat 1926;260: ) eyrter. Ueber eine eigennartige Geschultsform des Nervengewebes im menslichen Verdauunsschlauch. Virchows Arch Pathol Physiol 1935;285: ) isher ER, Wechsler H. Granular cell myoblastoma: Misnomer. Electron microscopy and histochemical evidence concerning its Schwann cell derivation and nature. Cancer 1962;15: ) Lack EE, Worsham G, Callihan MD. Granular cell tumor: A clinicopathologic study of 110 patients. J Surg Oncol 1980;13: ) Nussbaum M, Haselkorn A. Granular cell myoblastoma in parotid gland. N Y state J Med 1972;72: ) Chetty R, Kalan MR. Malignant granular cell tumor of the breast. J Surg Oncol 1992;49: ) Giant MD, Wall RW, Ransburg R. Endobronchial granular cell tumor: Cytology of a new case and review of the literature. Acta Cytol 1979;23: ) Tsuchida T, Okada K, Itoli E, Sato T, Sato K. Intramuscular malignant granular cell tumor. Skeletal Radial 1997;26: ) anburg-smith JC, Meis-Kindblom JM, ante R, Kindblum LG. Malgnant granulkar cell tumor of soft tissue: Diagnostic criteria and clinicopathologic correlation. Am J Surg Pathol 1998;22: ) liss DM, Puterman M, Zirkin H, Leiberman A. Granular cell lesions in head and neck: A clinicopathological study. J Surg Oncol 1989;42: ) Mazur TM, Schultz MS, Myers JL. Granular cell tumor: Immunohistochemical analysis of 21 benign tumors and one malignant tumor. Arch Pathol Lab Med 1990;114: ) Gamboa LG. Malignant granular cell myoblastoma. Arch Pathol 1955; 60: ) Strong EW, McDivitt RW, Brasfield RD. Granular cell myoblastoma. Cancer 1970;25:

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