• 대한후두음성언어의학회지
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• 제26권1호
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• pp.58-62
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• 2015

Xanthoma disseminatum (XD) is a benign, non-Langerhans cell histiocytosis characterized by disseminated xanthomatous lesions with face, flexures, and mucosa. Most of XD develops in mucocutaneous lesions including skin, oral cavity and pharynx, however laryngeal involvement is uncommon. While the natural course of XD is usually benign and often self-limiting, but XD develop in critical anatomical locations may result in morbidity and mortality. Localized mucous lesions in oropharynx and larynx lead to dysphagia, dyspnea and air way obstruction. The diagnosis of XD was based on clinical, histological and immunohistochemical findings. The treatment is complex and non-consensual. Local treatment with cryotherapy, radiotherapy, surgery, and carbon dioxide lasers have been attempted with various results. Systemic medication with peroxisome proliferator-activated gamma receptors, statins, fenofibrate, chlorodeoxyadenosine, cyclophosphamide, doxycycline, and cyclosporine have also been reported, but none have proven particularly successful. A 59-year-old man presented with respiratory symptoms because of laryngeal involvement of XD. We had to remove the obstructive lesion for relieving the symptoms. We experienced XD in Larynx that was rare in otorhinolaryngology. So we report this case with review of literatures.

• Journal of Chest Surgery
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• 제38권11호
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• pp.799-802
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• 2005

랑거한스세포 조직구증식증은 호산구성 육아종, Letterer-Siwe병, Hand-Schuller 증후군을 포함하는 질환으로 이전에는 조직구증식증 X로 명명되어 왔다. 이 질환의 임상적 양상은 다양하며, 기본적인 종양의 성질은 양성이나 파종되는 성질이 아주 강하다. 질환의 자연 경과는 예측할 수 없다. 하지만, 뼈에 생긴 단일 병변일 경우 광범위 절제를 통해서 완치율을 높일 수가 있다. 대부분의 랑거한스세포 조직구증식증 환자에서는 주로 두개골 천정에 골용해 병변이 나타나며, 드물게 두개골 기저부와 대퇴골을 침범하기도 한다. 늑골에 생기는 단일 병소의 랑거한스세포 조직구증식증은 비교적 드물다. 본 저자들은 늑골에 생기는 단일 병소의 랑거한스세포 조직구증식증 2예를 외과적 절제술로 치료하였기에 보고한다.

• Investigative Magnetic Resonance Imaging
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• 제13권1호
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• pp.74-80
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• 2009

목적: 이 연구의 목적은 두개골 랑게르한스 세포 조직구증의 특징적인 자기공명 영상소견을 기술하고 단순촬영과 컴퓨터 단층 촬영에서의 영상소견과 비교함에 있다. 대상 및 방법: 총 9명의 환자 중 10예의 병변을 대상으로 자기공명 영상을 분석하였다(연령범위: 5-42세, 평균 연령: 18세, 전예 여자). 9예에서 수술이나 미세 침 흡입 생검술에 의해서 병리학적으로 두개골의 랑게르한스 세포 조직구증이 증명되었다. 모든 환자는 자기공명영상을 시행하였고 이 중 7명 (8예) 에서 컴퓨터 단층 촬영과 단순 촬영을 시행하였다. 두 명의 숙련된 신경영상의학 의사가 영상에서 병변의 위치, 크기, 모양과 특징을 독립적으로 분석하고 병변의 범위와 주변 조직으로의 침범 여부를 비교하였다. 결과: 병변은 모든 두개골에 편향 없이 분포하였다. 자기공명 영상에서 종괴들은 조영증강되는 골 융해성 종괴(10/10)로 주로 판사이 공간에 위치하고(8/10) 두피(9/10)나 경질막(7/10)을 침범하였다. 경질막의 조영증강(7/10)과 비후(4/10)가 모든 병변에서 보였다. 골 융해성 종괴의 크기는 1.1 cm에서 6.8 cm의 범위였으며 모양은 원형(5/10)이거나 난원형(5/10)이었다. 컴퓨터 단층 촬영에서 병변은 판사이 공간의 골 융해성 종괴로(6/8) 두피의 침범(7/8)이 잘 보였다. 컴퓨터 단층촬영에서 골미란이나 골파괴가 자기공명영상보다 좀 더 분명하게 보였지만 연부종괴와 경질막의 조영증강 유무는 판단하기 어려웠다. 이와 대조적으로 단순촬영에서 모든 랑게르한스 세포 조직구증은 도려낸 병터 (punched out) 모양(4/8)이나 beveled-edge 모양(4/8)의 골융해성 종괴로 보였고 두피나 경질막으로의 침범은 판단 할 수 없었다. 결론: 두개골 랑게르한스 세포 조직구증의 특징적인 자기공명 영상소견은 판사이 공간에 위치한 골 융해성 종괴가 경질막과 두피를 침범하는 것으로, 자기공명영상이 단순촬영이나 컴퓨터 단층 촬영 보다 우월한 영상 기법이다.

• Tuberculosis and Respiratory Diseases
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• 제74권3호
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• pp.97-103
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• 2013

A lung cyst is an air-filled lucent structure surrounded by a thin wall. The presence of multiple intrapulmonary cysts is defined as cystic lung disease. Although cystic lung disease is rare, incidental detection has increased significantly in recent years by screening using computed tomography. There are many conditions that can mimic lung cysts and cause cystic lung disease. Clinical, radiographic, and histologic findings are all necessary for a proper diagnosis, and multidisciplinary approaches are frequently required. The aim of this report is to review the causes and characteristics of cystic lung disease to better understand and improve treatment.

• 대한골관절종양학회지
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• 제20권1호
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• pp.32-35
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• 2014

Rosai-Dorfman병은 특발성 양성 조직구 증식성 질환으로 결절형과 결절외형으로 분류되는 질환이며 골 침범은 매우 드물다. 증례는 32세 남자로 좌측 하퇴의 통증을 주소로 내원하였으며 림프절병증은 동반하지 않았다. 임상 및 영상학적으로 전이암, Langerhans 세포 조직구증 등이 의심되어 병변에 대한 절개 생검 시행하였고, 조직 검사 결과 Rosai-Dorfman병으로 진단되어 골소파술 시행하였다. 국내에서 Rosai-Dorfaman병이 사지에 단독으로 발생한 증례는 없기에 저자들은 이를 문헌 고찰과 함께 보고하고자 한다.

• 대한두경부종양학회지
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• 제29권1호
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• pp.18-21
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• 2013

랑게르한스 세포 조직구증은 골수에서 유래하는 랑게르한스 세포 조직구의 이상 증식에 의해 발병하는 희귀한 질병으로 알려져 있다. 비록 모든 장기에서 발생 할 수 있으나 갑상선을 침범하는 경우는 매우 드물다. 18세 남자가 5달전부터 점점 커지는 갑상선 종괴를 주소로 내원하여 세침흡인 세포검사, 총샘검, 경부 전산화단층촬영을 시행하였다. 세침흡인 세포검사에서 악성신생물이 의심되었고, 총생검에서 랑게르한스 세포 조직구증으로 나타났다. 경부 전산화단층촬영에서는 우측 갑상선에서 윤곽이 잘 구분되는 저음영의 종괴와 우측 기관 주위 림프절의 종대가 관찰되었다. 갑상선 전절제술과 우측 중앙 선택적 경부 림프절 청소술이 시행되었다. 랑게르한스 세포 조직구증이 갑상선을 침범하는 경우는 드물지만 갑상선 비대가 있는 환자가 뇌하수체 기능부전의 증상이나 뼈와 폐의 침범과 관련된 증상을 호소한다면 갑상선의 랑게르한스 세포 조직구증 침범을 고려해야 한다. 또한, 다른 장기의 랑게르한스 세포 조직구증을 치료한 과거력이 있는 경우는 갑상선 종괴를 감별 진단하는데 있어 랑게르한스 세포 조직구증을 고려해야 한다.

• Journal of Yeungnam Medical Science
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• 제39권2호
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• pp.153-160
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• 2022

Multicentric Castleman disease (MCD) is an uncommon systemic lymphoproliferative disorder that may cause multiple organ damage. Castleman disease-associated diffuse parenchymal lung disease (DPLD) has not been well studied. A 32-year-old man was referred to our hospital for progressive generalized weakness, light-headedness, and dyspnea on exertion for more than one year. Laboratory evaluations showed profound anemia, an elevated erythrocyte sedimentation rate, and an increased C-reactive protein level with polyclonal hypergammaglobulinemia. Chest radiography, computed tomography (CT), and positron emission tomography-CT scan demonstrated diffuse lung infiltration with multiple cystic lesions and multiple lymphadenopathy. In addition to these clinical laboratory findings, bone marrow, lung, and lymph node biopsies confirmed the diagnosis of idiopathic MCD (iMCD). Siltuximab, an interleukin-6 inhibitor, and glucocorticoid therapy were initiated. The patient has been tolerating the treatment well and had no disease progression or any complications in 4 years. Herein, we report this case of human herpesvirus-8-negative iMCD-associated DPLD accompanied by multiple cystic lesions, multiple lymphadenopathy, and polyclonal hypergammaglobulinemia with elevated immunoglobulin G (IgG) and IgG4 levels. We recommend a close evaluation of MCD in cases of DPLD with hypergammaglobulinemia.

• Korean Journal of Radiology
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• 제2권4호
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• pp.222-230
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• 2001

Central diabetes insipidus (DI) can be the outcome of a number of diseases that affect the hypothalamic-neurohypophyseal axis. The causes of the condition can be classified as traumatic, inflammatory, or neoplastic. Traumatic causes include postoperative sella or transection of the pituitary stalk, while infectious or inflammatory causes include meningitis, lymphocytic hypophysitis, and granulomatous inflammations such as sarcoidosis and Wegener's granulomatosis. Various neoplastic conditions such as germinoma, Langerhans cell histiocytosis, metastasis, leukemic infiltration, lymphoma, teratoma, pituitary adenoma, craniopharyngioma, Rathke cleft cyst, hypothalamic glioma, and meningioma are also causes of central DI. In affected patients, careful analysis of these MR imaging features and correlation with the clinical manifestations can allow a more specific diagnosis, which is essential for treatment.

• Advances in pediatric surgery
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• 제15권2호
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• pp.103-112
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• 2009

Catheter related and perianal problems are common surgical complications encountered during the treatment of pediatric malignancies. However acute surgical abdominal emergencies are rare. The aim of this study is to review acute surgical abdominal complications that occur during the treatment of childhood malignancies. Out of a total of 1,222 patients who were newly diagnosed with malignant disease, between January 2003 and May 2008, there were 10 patients who required surgery because of acute abdominal emergencies. Their medical records were reviewed retrospectively. Hematologic malignancies were present in 7 patients (4 leukemia, 2 lymphoma, 1 Langerhans cell histiocytosis) and solid tumors in 3 patients (1 adrenocortical carcinoma, 1 desmoplastic small round cell tumor, 1 rhabdomyosarcoma). Seven patients had intestinal obstruction, two had gastrointestinal perforation and one, typhlitis. Intestinal obstructions were treated with resection of the involved segment with (N=2) or without (N=3) enterostomy. Two patients had enterostomy alone when resection could not be performed. Intestinal perforation was treated with primary repair. Typhlitis of the ascending colon was treated with ileostomy. Right hemicolectomy was necessary the next day because of the rapidly progressing sepsis. Three patients are now alive on chemotherapy and one patient was lost to followed-up. Among six patients who died, five died of their original disease progression and one of uncontrolled sepsis after intestinal perforation. Although rare, acute surgical abdominal complications can occur in childhood malignancies. Rapid and accurate diagnosis and appropriate operation are required for effective treatment of the complications.

• Clinical and Experimental Pediatrics
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• 제54권4호
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• pp.169-175
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• 2011

Purpose: Acute internal hemorrhage is an occasionally life-threatening complication in pediatric cancer patients. Many therapeutic approaches have been used to control bleeding with various degrees of success. In this study, we evaluated the efficacy of selective internal iliac artery embolization for controlling acute intractable bleeding in children with malignancies. Methods: We retrospectively evaluated the cases of 6 children with various malignancies (acute lymphoblastic leukemia, acute myelogenous leukemia, chronic myelogenous leukemia, T-cell prolymphocytic leukemia, Langerhans cell histiocytosis, and rhabdomyosarcoma), who had undergone selective arterial embolization (SAE) of the internal iliac artery at the Chonnam National University Hwasun Hospital between January 2004 and December 2009. SAE was performed by an interventional radiologist using Gelfoam$^{(R)}$ and/or Tornado$^{(R)}$ coils. Results: The patients were 5 boys and 1 girl with median age of 6.9 years (range, 0.7-14.8 years) at the time of SAE. SAE was performed once in 4 patients and twice in 2, and the procedure was unilateral in 2 and bilateral in 4. The causes of hemorrhage were as follows: hemorrhagic cystitis (HC) in 3 patients, procedure-related internal iliac artery injuries in 2 patients, and tumor rupture in 1 patient. Initial attempt at conservative management was unsuccessful. Of the 6 patients, 5 (83.3%) showed improvement after SAE without complications. Conclusion: SAE may be a safe and effective procedure for controlling acute intractable hemorrhage in pediatric malignancy patients. This procedure may obviate the need for surgery, which carries an attendant risk of morbidity and mortality in cancer patients with critical conditions.