대 한방사선 의 학회 지 1991; 27(4) : 513~517 Journal of Korean Radiological Society, July, 1991

Computed Tomography of Lethal Midline Granuloma

Ho Suk Lee, M.D., Tae Ho Kim, M.D., Kyung Jin Sub, M.D., Tae Hun Kim, M.D. Yong Joo Kirn, M.D., Dul‘ Sik Kang, M.D. Department of Diagnostic Radiology, CoJ1ege of Medicine, Kyungpook National University

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In order to clarify the CT findings oflethal midline granuloma (LMG) diagnosed clinically or histopathologically, the authors retrospectively analyzed 12 patients who were seen at Kyungpook National University Hospital from February 1985 to August 1989. CT showed nasal mucosal thickening and/or soft tissue mass (9 cases), spreading of the lesions along the facial subcutaneous fat plane (8 cases), invasion into the (5 cases), bone destruction (5 cases), nasopharyngeal mass lesion (2 cases), and extension of the lesion into the infratemporal fossa (1 case). In spite of the fact that CT does not make definitive diagnosis of LMG, it permits evaluation of the extent of the lesion, detection of the combined lesion, differential diagnosis, and close monitoring of its evolution under treatment.

Index Word8: Nose , Lethal Midline Granuloma 261.622 Paranasal sinuses. Computed Tomography 23.1211 Nose. Computed Tomography 261. 1211

Recent research on the condition historically Introduction known as lethal midline granuloma concludes that it is a form of T-cell .(2) Since the first report ofMcBride (1897)(1). the tenn The prominent histological features ofLMG are in­ lethal midline granuloma (LMG) and it8 various f1ammation, necrosis, and thrombosis with infiltra­ synonyms-progressive lethal granulomatous 비cera­ tion of the atypical histiocytes into the perivascular tion (Stewart. 1933).lethal granulomatous ulceration connective tissue of midline facial tissue (Williams. 1949). idiopathic The purpose of this retrospectlve study was to lethal granulomatous ulceration (Woodburn & Har­ clarify the CT findings of LMG. ris. 1951), nonhealing granuloma (Waltone, 1959), polymorphic reticulosis (Eichel et a 1., 1966), midline Materials and Methods malignant reticulosis (Kassel et al., 1969), midline granuloma (Dellon, 1977), and lymphomatoid Our study included 12 patients with LMG who granulomatosis (DeRemee et a 1., 1978)-have been were seen at Kyungpook National University Hospital used to describe a rare lesion of a nonhealing from February 1985 to August 1989. They were 9 granuloma that ultimately causes necrosis and men and 3 women with an age range of 17 to 71 years destruction of the nose and midface. According to (mean: 40 yrs). WHO (1978), LMG is defined as a progressively Four cases were pathologically proved and 8 cases destructive nontuberculoid granulomatous lesion had nonspecific pathologic findings, but their clinical localized in the nasal cavity, paranasal sinuses, or findings and courses were identical to LMG. palate and infiltrating the soft and hard tissue All patients were examined with a CT/T 8800

이 논문은 1 99 1 년 l 월 4 일 접수하여 1 9 91 년 5 월 17 일 에 채택되었음 Received January 4. accepted May 17. 1991 - 513- Journal of Korean Radiological Society 1991; 27(4): 513-517 scanner (GE, Milwaukee, Wl, U.S.A.) using con­ Table 1. Clinical Features tiguous 5 or 10-mm-thick sections from the frontal Symptoms/Signs No. of cases s inus to the hyoid bone after 50cc bolus and 100cc 8 Nasal stuffiness dripping of contrast (RayvistR 300) administration. 5 Facial swelling and pain 4 Axial or axial with coronal images were obtained. Mucocutaneous u lceration 3 Nasal discharge 2 Results Swallowing difficulty 2 Sore throat 2 The most common clinical features of LMG was Foulodor nasal stuffiness. Other symptoms and signs includ­ ed facial swelling and pain, mucocutaneous ulcera­ nose (1 case). tion, nasal discharge, and fo ul odor (Table 1). The The main histologicaI findings were acute or primary lesion sites were the nasal cavity (8 casesJ, chronic infIammation (12 cases) and focal or massive nasopharynx (2 casesl, palate (1 casesJ, and external necorsis (7 cases). Atypical histiocyte infiltrations

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Fig. 1. a , b. A soft tissue mass lesion is seen in posterior portion ofthe left nasal cavity ‘ is perforated The mucosa of the anterior nasal septum is mildly thickened, and the lesion spreads along the left facial fat plane Soft tissue of slightly lower density in Ieft maxillary sinus suggests infIammation secondary to obstruction of max. illary sinus ostium. Fig.2, Section through the midantrum shows soft tissue mass filling both sides ofthe nasal cavity. Bone destruc­ tions are present involving both medial walls of the maxillary antra.

&나"""" k *‘ 3a 3b 4 • Fig.3. a. b. There is a soft tissue mass occupying nasal cavity with mucosal thickening. Irregular bone destruc. tions are present in the medial wall of the right maxillary antrum Chronic is present in the right maxillary antrum ‘ Fig. 4. A soft tissue mass lesion is involving the anterior portion of the right nasal cavity and nares

- 514- Ho Suk Lee, et al: Computed Tomography of Lethal Midline Granuloma

Fig. 5. Midantral scan shows thicken­ ing of the right nares with spreading of the lesion into the soft tissue of the cheek overlying the right antrum. Fig. 6. An axial section through the junction of the nasopharynx and oropharynx shows soft tissue mass n the right nasopharynx with partial obliteration of the right parapharyngeal space.

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were seen in 4 cases, but fibroid necrosis, granuloma, midline d estructive disease , Wegener's and giant cells were not observed granulomatosis, polymorphic reticulosis, and Prominent CT findings included nasal mucosal nonHodgkin's lymphoma.(3) thickening and/or a soft tissue m ass (9 cases) (Fig. The etiology of LMG has been controversial, and

1.2 ,3 ,4). Spreading ofthe lesions aIong the facial sub­ the proposed pathogeneses have been immunologic cutaneous fat plane was noted frequently (8 cases) disorder(4,5), neoplastic process(6), or close relation (Fig. 1.5). Invasion into the paranasal sinuses was with lymphoma(7). But recent research concludes

observed in 5 cases‘ the maxillary sinus (5/5), that it is a form of T-celllymphoma(2), and the ap­

ethmoid sinus (5/5) , and sphenoid sinus (4/5) (Fig. 2) ‘ plication ofimmunological studies with monoclonal but frontal sinus invasion was not seen. antibody panels is now a standard procedure and

Bone destruction (5 cases) and nasopharyngeal sho 비 d help in avoiding misdiagnosis. Kataura et

mass lesion (2 cases) were also observed (Fig. 2 , 3 .6). a l.(8) studied 3 patients with lethal midline Extension of the lesion into the infratemporal fossa granuloma using monoclonal antibodies against T was seen in 1 case (Table 2) cell subsets, clearly demonstrating that the tumor cells in each case were probably T-cell, not from Table 2. CT Findings histiocytes, reticulum cells, or B-cells. CT findings No. of cases The prominent histologicaI features ofLMG are in­ flammation, necrosis, and thrombosis, a 1l 3 ofwhich Nasal mucosal thickening and/or 9 suggest a similarity to Wegener’ s granulomatosis, soft tissue mass Spreading of the lesions along the 8 but differentiate the lesion from lymphosarcoma(9). facial subcutaneous fat plane True is a typical finding in Wegener’s ζ 니 Invasion into paranasal sinuses granulomatosis but does not appear in the LMG ‘ DF Bone destruction It p이 ymorphic 끼 a hough cells may infiltrate the Mass in nasopharynx ι perivascular connective tissue mimicking vasculitis. Histologically, the lesions ofLMG are separable from

Discussion Wegener’ s granulomatosis by the absence of necrotizing epithelioid granulomas, a tendency The term ‘'lethal midline granuloma" does not toward a necrotizing angioinfiltrativegrowth pattern, properly reflect the current observation that several and abundant atypical lymphoproliferative cellular different diseases can produce noninfectious mid- components( lO). ln LMG cases, only ulceration ofthe

facial destruction. In many cases‘ this is neither lethal upper occurs, whereas the lungs and

nor midline, and granuloma may be a bsen t. Recent. kidneys are never affected. This specific organ in- ly. the disorders producing erosion of the upper volvement is a lso a significant point in differential aerodigestive passage are more accurately classified diagnosis from Wegener‘ s granulomatosis by clinical and histological criteria as idiopathic A recen t study on the CT findings of LMG show - 515- Journal of Korean Radiolog ical Society 1991 ; 27(4): 513-517 ed a more or less complete filling of the air cavities granuloma. Otolaryng Clinics North America 1982: ofthe face by an abnormal soft tissue mass reaching 15:685-692 the nasal cavity and the nasopharynx. the ethmoidal 2. Platt J C. Tomich CE. Campbell S. Malignant lym­ cells. the sphenoidal sinus. the maxillary sinus. and phoma presenting as a midline lethal granuloma. J Oral Maxillofac Surg 1989; 47:511-513 the frontal sinus( 11). These findings are identical to 3. Pickens JP. Modica L. Current concepts of the lethal the results of our study. except for the frontal sinus midline granuloma syndrome. Otolaryngol Head involvement. CT permits the precise evaluation of the Surg 1989; 100:623-630 extent of the lesions. It is known that the lesions can 4. Friedmann I. Sando 1, Balkany T. Clinical records. extend to the intracranium. to the infratemporal Idiopathic pleomorphic midfacial granuloma fossa. and to the orbit and can infiltrate the sub­ (Stewart’s type). J Laryngol Otol 1978; 92:610 cutaneous tissue ofthe face(II). In this study. cranial 5. Fau ci AS. Johnson RE. WolffSM. Radiation therapy or orbital invasion was not observed. but the of midline granuloma. Annals of Internal Medicine spreading ofthe lesion along the facial subcutaneous 1976; 84:140-147 fat plane was frequently seen. Paranasal sinus inva­ 6. Eichel BS. Maeberg TE. The enigma of the lethal sion was not infrequently seen. but it is difficult to midline granuloma. Lary ngoscope 1968; differentiate it from chronic sinusitis caused by 78:1367-1368 obstructive nasal lesions. Presence of major bone 7. Michaels L. Gregory M. Pathology of “ non-healing (midline) granuloma'’. J Clin Patho 1977; destruction with no definite osteosclerotic bone 30:317-327 thickening is helpful for differential diagnosis. 8. Kataura A. Sambe S. Minase T et al. Midfacial T cell The CT permits no definite differentiation of LMG Iymphoma: characterization by monoclonal an­ from other granulomatoses or from nasal or paranasal tibodies. Ann Otol Rhinol Laryngol 1985; sinus tumor lesions. Nevertheless a few differential 94:207-211 points may be mentioned: There is no caJcification 9. Mann WJ. Riede UN. Bocking A. Midline malignant within the granulomatous tissue. osteoscJerotic reac­ reticulosis. Acta otolaryngol (Stockh) 1984; tion at the bone structure leveI. or cervical 97:365-372 Iocoregional Iymph nodes(II). In our study. caJc ifica­ 10. BatsakisJG. Tumors ofthe head and neck. 2nd ed. tion or lymph node enlargement was not observed Williams and Wilkins. 1979; 492-500 The nasal or paranasal sinus carcinomas tended to 11. Ovtchinnikoff S. Blin D, Rouanet JP et al. Computed be bulky and expansile with nonspecific focal bone tomography of lethal midline granuloma: 5 case report. Europ J Radiol 1988; 8 :145-147 d estruction(l2). but these findings were rare in our 12. Silver AJ, Baredes S. Bello JA et al. The opacified s eries. CT is also u seful in detection of combined le­ maxillary sinus: CT findings in chronic sinusitis and sions. distant metastases. or close monitoring of the malignant tumors. Radiology 1987; 163:205-210 LMG under treatment. which is essentially 13. 김귀언， 홍인수， 이도행. 악성 중앙선 세망증의 방사선 r a diotherapy. 치 료. Journal of Korean Cancer Research Assoc ia­ The prognosis of LMG is poor(5.13). However. tion 1981 ;13( 1): 13-18 there are many recent repoπsabout its g∞d response 14. 강현영， 박준식. Midline Grilnuloma 의 방사선 치료 및 to radiotherapy( 14.15). In our 12 patients examined steroid 병용 요법. 대한이비인후과학회지 198 1 ; 24(3) between February 1985 and August 1989. 6 patients : 322-326 showed complete remission on follow-up studies. 15. 이 종담， 이 정 삼， 김 상일 . Polymo rphic reticulosis 의

임상적 관찰. 대한이비인후과학회지 1989 ‘ 32(2) : 335 REFERENCES - 342

1. Alan DK. Anthony SF. Idiopathic midline

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〈국문요약〉 치사성 정중부 육아종의 전산화 단층촬영

경북대학교 의과대학 진단방사선과학교실

이호석 · 김태호 · 서 경진 · 검태헌 · 김용주· 강덕식

치사성 정중부 육아종은 비장， 부비강， 비인강 올 포함한 상기도 및 안면에 국소적 이고 진행성이며 파괴적인 염증 성 병변이 나타나 는 질환으로서 예후가 아 주 불량하며 1 897 년 McBride 가 처음 기술한 이래 비 록 발생 빈 도는 희귀

하나 수많은 학자들에 의해 여러가지 병명이 소개되고 원인 ， 병리소견 둥에 대해 많은 연구가 진행되어 왔다 . 병리 조직학적 소견이 비 특이적인 경우가 많으므로 생검시에는 심부 조직및 궤양변연부 조직을 취해야하며 반복적 생검이 확진에 필수적이다. 저자들은 1985 년 2 월부터 1989 년 8 월까지 경북대학교병원을 방문하여 치사성 정중부 육아종으로 진단받은 12 명 의 환자를 대상으로 전산화 단충촬영 소견을 역행적으로 분석하여 그 특정 에 대해 알아 보고자 하였다. 전산화 단충촬영 소견으로는 비강점막 비 후 와 비강내 연부조직 종괴가 가장 흔한 소견이었고 (9 례) 안변부 피하 지 방층을 따라 염중성 영변이 파급되 는 양상이 8 례에서 관찰되었다 . 부 비강 으로의 병변의 파급과 비 특이적인 골파괴 양상이 각각 5 례에서 관찰되었으며 비인강 종양 과 비슷한 양상을 보인 예도 2 례가 있었다. 그러나 임파선 종 대나 병

변내 석회화 양상은 관창되지 않았다.

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