• Journal of the Korean Society of Laryngology, Phoniatrics and Logopedics
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• v.26 no.1
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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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• v.38 no.11 s.256
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• pp.799-802
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• 2005

Langerhans cell histiocytosis (LCH) involves disorders previously referred as 'histiocytosis X' (including eosinophilic granuloma, Letterer-Siwe, and Hand-Schuller syndrome). Its clinical patterns are various and it is a basically benign tumoral condition but with a strong tendency toward dissemination and destruction. Its natural history is unpredictable. But, in solitary bone lesion, wide resection with tumor-free margin is required in order to provide the best chance for a cure. In the majority of patients LCH is a osteolytic lesion with a predilection for calvarium and is rarely seen in the skull base and the femur. LCM of rib, especially if solitary, is relatively rare. We report two rare cases of solitary LCH developed in the rib, which were successfully treated by surgical resection.

• Investigative Magnetic Resonance Imaging
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• v.13 no.1
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• pp.74-80
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• 2009

Purpose : To evaluate the characteristic MR imaging findings of Langerhans cell histiocytosis (LCH) in the skull and to compare them with those of plain radiography and computed tomography. Materials and Methods : A total of 10 lesions in 9 patients (Age range; 5-42 years, Mean age; 18, all women) with Langerhans cell histiocytosis in the skull were included in our study. Nine lesions in nine patients were histologically confirmed by surgery or fine needle aspiration biopsy. All patients performed with MRI, and plain radiography and CT scan were done in 7 patients (8 lesions). Two experienced neuroradiologists reviewed the radiological examinations independently with attention to location, size, shape and nature of the lesions in the skull and compared the extent and extension of the lesions to adjacent structures. Results : The lesions were distributed in all of the skulls without predilection site. On MRI, the masses were shown as well-enhancing soft tissue masses (10/10) mainly in diploic spaces (8/10) with extension to scalp (9/10) and dura mater (7/10). Dural enhancement (7/10) and thickening (4/10) were seen. The largest diameter of the soft tissue masses ranged 1.1 cm to 6.8 cm, shaped as round (5/10) or oval (5/10). On CT scans, the lesions were presented as soft tissue masses involving diploic space (6/8) and scalp extension (7/8) were also well visualized. Although bony erosion or destruction was more clearly seen on CT rather than those of MRI, enhancement of soft tissue masses and dura were not well visualized on CT. In contrast, all of the lesions in LCH were seen as punched out (4/8), beveled-edge appearance (4/8) osteolytic masses in plain radiography, but scalp and dural extension could not be seen. Conclusion : Characteristic MR findings in patients with LCH are soft tissue mass in diploic space with extension to dura and scalp, and MRI would be better imaging modality than plain radiography or CT.

• Tuberculosis and Respiratory Diseases
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• v.74 no.3
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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.

• The Journal of the Korean bone and joint tumor society
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• v.20 no.1
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• pp.32-35
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• 2014

Rosai-Dorfman disease (RDD) is an idiopathic histioproliferative disorder of lymph node and extranodal site. Bone involvement is very rare. We report a case of extranodal RDD of the tibia in 32-year old male. The patient presented with pain with no evidence of lymphadenopathy. Clinico-radiologic diagnosis was metastatic carcinoma or Langerhans cell histiocytosis, but, histopathologic examination confirmed the diagnosis with RDD. We performed curettage on the osteolytic lesion of tibia. In South Korea, there was no report about RDD of the extremity and we want to report this case with review of the literature.

• Korean Journal of Head & Neck Oncology
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• v.29 no.1
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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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• v.39 no.2
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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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• v.2 no.4
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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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• v.15 no.2
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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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• v.54 no.4
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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.