• 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.

• Korean Journal of Head & Neck Oncology
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• v.19 no.2
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• pp.179-183
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• 2003

Osteosarcoma is one of the most frequently occurring malignant bone tumor except for multiple myeloma. However, osteosarcoma of the mandible is rare and aggressive malignancy constituting about 5% to 13% of all cases of skeletal osteosarcoma. The authors experienced a case of osteosarcoma of the mandible in a 31-years old male patient. He visited our outpatient clinic with painless mass of the cheek. On the basis of the clinical and imaging findings, the differential diagnosis included giant cell tumor, histiocytosis and malignant bone tumor. In the first operation, the mass of the mandible was excised. Intraoperative microscopic examination of a frozen section did not yield a diagnosis: however, the final definitive histologic examination revealed osteoblastic osteosarcoma. After 15days, wide excision with hemimandibulectomy was completed followed by postoperative radiotherapy and chemotherapy. With our experience, we report the progression of diagnosis and management for mandibular osteosrcoma.

• Journal of Yeungnam Medical Science
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• v.38 no.3
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• pp.245-250
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• 2021

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a self-limiting lymphadenitis. It is a benign disease mainly characterized by high fever, lymph node swelling, and leukopenia. Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease with clinical symptoms similar to those of KFD, but it requires a significantly more aggressive treatment. A 19-year-old Korean male patient was hospitalized for fever and cervical lymphadenopathy. Variable-sized lymph node enlargements with slightly necrotic lesions were detected on computed tomography. Biopsy specimen from a cervical lymph node showed necrotizing lymphadenitis with HLH. Bone marrow aspiration showed hemophagocytic histiocytosis. The clinical symptoms and the results of the laboratory test and bone marrow aspiration met the diagnostic criteria for HLH. The patient was diagnosed with macrophage activation syndrome-HLH, a secondary HLH associated with KFD. He was treated with dexamethasone (10 mg/m²/day) without immunosuppressive therapy or etoposide-based chemotherapy. The fever disappeared within a day, and other symptoms such as lymphadenopathy, ascites, and pleural effusion improved. Dexamethasone was reduced from day 2 of hospitalization and was tapered over 8 weeks. The patient was discharged on day 6 with continuation of dexamethasone. The patient had no recurrence at the 18-month follow-up.

• 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.

• Journal of the Korean Society of Radiology
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• v.84 no.3
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• pp.757-762
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• 2023

First described in 1930 as a lipoid granulomatosis, Erdheim-Chester disease (ECD) is a rare histiocytosis encompassing a group of disorders caused by overproduction of histiocytes, a subtype of white blood cells. This disease most commonly involves the bones and can affect organs in the abdomen; however, biliary involvement is rarely reported. We report a case of ECD with biliary involvement, which rendered it difficult to radiologically distinguish ECD from immunoglobulin G4-related disease.

• Tuberculosis and Respiratory Diseases
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• v.67 no.4
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• pp.345-350
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• 2009

Erdheim-Chester disease (ECD) is a proliferative non-Langerhans cell histiocytosis of multiple organs. This is a rare disease of unknown etiology with a high mortality. We present the case report of a 26-year-old man diagnosed with ECD. He was referred to our hospital with elevated levels of aminotransferases. Although the diagnosis was uncertain, the patient was lost to follow up at that time. One year later, the patient returned to the hospital with generalized edema. Although a specific bone lesion was not found, the patient was experiencing the following: glomerulonephritis, aplastic anemia, hepatitis, and lung involvement. A lung biopsy was performed: the immunohistochemical stain were positive for CD68 and negative for S-100 protein and CD1a. We diagnosed as the patient as havinf ECD. Approximately 50% of ECD cases present with extraskeletal involvement. ECD should be considered as part of the differential diagnosis when multiple organs are involved.

• 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.

• 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.

• 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.