가톨릭대학교 의과대학 서울성모병원 이비인후과학교실
유동준, 이성훈, 여상원, 박시내
Department of Otolaryngology-HNS, The Catholic University of Korea College of Medicine, Seoul, Korea
Dong Joon Yoo, MD, Sung-Hun Lee, MD, Sang Won Yeo, MD, PhD, Shi-Nae Park, MD, PhD.
성인에서의 외이도 황색 육아종 1례
A Case of external auditory canal xanthogranuloma in adult
J Korean Skull Base Society 9권 1호 : 23~27, 2014
Xanthogranuloma is one of non-Langerhans cell histocytic disorders. And adult-onset huge xanthogranuloma of the external auditory canal is very rarely reported, especially on this anatomical site. We introduce a case of adult-onset huge xanthogranuloma of the external auditory canal. An 32-year-old female presented with intermittent left bloody otorrhea for two months. Radiological studies showed a solitary mass on the posterior wall of external auditory canal that had no invasion or erosion of temporal bone. The mass that originated from the posterior wall of the external auditory canal was excised and the defect site was reconstructed with ipsilateral superficial temporalis fascia. Pathologically, this huge mass was diagnosed as xanthogranuloma.
We report this case as a very rare case of adult-onset xanthogranuloma in the external auditory canal as well as its excellent long-term result of surgery with the review of the literatures.
논문 접수일 : 2014년 4월 30일 심사 완료일 : 2014년 5월 20일
주소 : Department of Otolaryngology-HNS, Seoul St. Mary’s Hospital, The Catholic University of Korea, College of Medicine,
505 Banpodong, Seochogu, 137-040, Seoul, Korea Tel : 82-2-2258-6215 Fax : 82-2-595-1354
E-mail : snparkmd@catholic.ac.kr
Shi-Nae Park, MD, PhD
교신저자
Xanthogranuloma, EAC, external auditory canal
Key Words
종설1 종설2 종설3 증례1 증례2 증례3 증례4 증례5 증례6
▒ Background
As a member of non-Langerhans cell histocytic disorders, xanthogranuloma usually presents with skin lesion which is asymptomatic, self-healing and brown-yellowish papule.
Xanthogranulomas are known to be predominantly occurred in infancy, so that Helwig and Hekney1 introduced these tumors as the term “juvenile xanthogranuloma”in 1954.
Juvenile type of xanthogranuloma has a tendency of spontaneous regression. In contrast, pathologically same skin lesion that develops in adult periods, are frequently persistent
and even growing. So majority of adult xanthogranuloma which usually involves face are needed to be excised.
Recently, we treated this rare type of benign tumor of adult xanthogranuloma in the external auditory canal by excision of the mass with the fascia graft. Here, we report this case with the long-term follow-up result and the review of literatures.
▒ Case
A 32-year-old female presented with intermittent bloody
A. Otoendoscopic findings of the external auditory canal. About 70% of external auditory canal was obstructed by approximately 0.5x0.5cm sized brown-yellowish round, rubbery and fixed mass.
B. One month later, external auditory canal was totally obstructed by the mass.
Fig. 1
A B
A B C
Fig. 2
otorrhea in the left ear for more than two months. She was in a good general condition and there was no trauma history of external auditory canal. On physical examination, about 70%
of external auditory canal was obstructed by 0.5×0.5cm sized round, rubbery and fixed mass and the mass was attached to postero-inferior auditory canal wall(Fig. 1A). During the follow-up period of one month, the mass has been increased in size which caused total obstruction of the external auditory (Fig. 1B).
Contrast-enhanced computed tomography (CT) of the temporal bone showed solitary mass at the posterior wall of external auditory canal of the left ear and there was no erosion of temporal bone. Magnetic resonance imaging (MRI) of the temporal bone showed us 0.5×0.5×0.5cm sized intermediately enhanced , solitary mass(Fig. 2).
Surgical removal of the mass was performed under local anesthesia. Meatal skin flap was elevated and the mass was excised including normal skin around the mass via transcanal approach(Fig. 3). The mass was originated from the skin of the postero-inferior wall of the external auditory canal and was well encapsulated state. After excising the mass, skin defect of the external auditory canal wall was up to 0.9×1.2 cm. Defective site was reconstructed by superficial temporalis fascia with a minimal supra-auricular incision with the firm packing with gauze soaked with antibiotic-oint. The excised specimen was 0.5×1.1×0.6 cm sized yellowish solid mass with good margin. Histopathologically, the specimen revealed
dermal infiltration of histocytes(Fig. 4A & 4B). And there were increased foamy macrophages(arrows) and multinucleated giant cells(arrows) of which nuclei arranged in a ring, so called “Touton giant cells”.(Fig. 4C, 4D). During the 18 months of follow-up, there was no complication or recurrence in this case and the patency of the reconstructed external auditory canal was excellent(Fig. 5).
▒ Discussion
Histocytic disorders are classified into 3 groups : Langerhans cell disease(class I), non-Langerhans cell histocytic disease(class II), and malignant histocytic disorders(class III). Xanthogranuloma is one of non- Langerhans cell histocytic disease(NLCH) and 80-90% of NLCH are xanthogranuloma.2) Xanthogranuloma usually occurs in infancy and child and Helwig and Hekney1 introduced the term “juvenile xanthogranuloma(JXG)”in 1954. JXG is usually presented with multiple, solitary skin lesions. Head and neck areas are most frequently involving sites. JXG tends to show self-healing. Though rare, orbit, muscle, brain or spinal cord and lung can be involved by this tumor3. If the lesion in these organs interferes normal function, surgical removal, chemotherapy or radiation therapy can be necessary.
In contrast to JXG, skin lesion that develops in adults, so called adult xanthogranuloma(AXG) is frequently persistent.4)
The surgically excised specimen. The specimen was about 0.5x1.1x0.6 in sized mass(A) and filled with sold yellowish materials (B).
Fig. 3
A B
Therefore AXG mostly needs to be treated by surgical removal. AXG is known as a solitary and single cutaneous skin lesion and mainly involves the face(73%) and the trunk(13%).5)Multiple xanthogranulomas in adults are more
rare and recently, Masahiro et al6) reported mechanical stimulation probably played a role in the development of multiple adult xanthogranulomas. However, AXG originated from external auditory canal has been very rarely reported.
Clinical course of this rare type of benign tumor in adult rarely shows spontaneous remission even through some of them showed the regression in the literature.7)
Histopathologically, xanthogranuloma shows dense foamy cells and multinucleated cells with dermal infiltration. A typical pathologic finding of this rare NLCH disease is
“Touton giant cells”.
In our case, we could observe a solitary mass of the external auditory canal wall with rapid growing tendency in a 32-year-old female, which was confirmed as an adult xanthogranuloma. Preoperative CT and MRI showed no local invasion or erosion of temporal bone but space occupying lesion in the external auditory canal. We completely excised the mass and successfully reconstructed skin defect site by
Otoendoscopic finding of external auditory canal checked at 18- month postoperatively shows no recurrence of tumor with excellent patency of the canal.
Fig. 5 A
Histopathologic findings of the excised specimen.
Dermal infiltration of histocytes(A, B H&E, x10, x20) are observed with the increased foamy
macrophages and multinucleated cells. The nuclei of the multinucleated giant cells, so called Touton giant cells, arrange in a ring and they separate peripheral foamy cytoplasms from
eosinophilic core (C,D H&E, x40).
Fig. 4
A B
C D
superficial temporalis fascia. There was no complication or recurrence of the mass during the 18-month follow-up period. We report this very rare and interesting case of huge xanthogranuloma arising from the external auditory canal in an adult, with the imaging, histological findings and a long- term surgical result with brief review of the literatures.
References
1. Helwig E, Hackney VC. Juvenile xanthogranuloma. Am J Pathol 1954;30:625-6.
2. Jose Rodriguez, MD, A. Bernard Ackerman. Xanthogranuloma in Adults. Arch Dermatol 1976;112:43-44
3. Young Woo Park, Eun Jeong Koh, Ha Young Choi. Rapid-Growing Juvenile Xanthogranuloma on the Scalp in 18-Month-Old Girl J Korean Neurosurg Soc 2011;50:271-273
4. Kraus MD, Haley JC, Ruiz R, et al. ‘‘Juvenile’’xanthogranuloma: an immunophenotypic study with a reappraisal of histogenesis. Am J Dermatopathol 2001; 23:104-111
5. Chang SE, Cho S, Choi JC, Choi JH, Sung KJ, Moon KC, et al.
Clinicohistopathologic comparision of adult type and juvenile type xanthogranuloma. J Dermatol 2001;28(8):413-8
6. Masahiro OKA, Tsuneyoshi KAMO, Noriko GOTO, Tohru NAGANO, Chikako NISHIGORI. Multiple adult xanthogranuloma in intertriginous areas J Dermatol 2009;36:176-178
7. Brian F. Worden,, Jose E. Barrera, Sam P. Most. Adult Xanthogranuloma Causing Nasal Obstruction. Arch otolaryngol Head Neck Surg 2010;136(5):509-12
