• Investigative Magnetic Resonance Imaging
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• 제24권1호
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• pp.61-65
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• 2020

Langerhans cell histiocytosis (LCH) is generally considered a childhood disease that exhibits various nonspecific clinical and radiological manifestations that mimic infection or malignancy. Here, we present a case of LCH involving the rib in an adult patient. CT and MRI revealed an expansile lytic lesion with periosteal reaction on the left 8th rib, suggesting a malignant bone tumor. Surgical resection was performed and histopathological examination was consistent with LCH. Owing to its rare occurrence in adults and nonspecific aggressive features, LCH should be included in the differential diagnosis of an aggressive-appearing rib lesion in both adults and children.

• 대한세포병리학회지
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• 제18권1호
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• pp.87-91
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• 2007

Langerhans cell histiocytosis (LCH) is a pleomorphic disease entity characterized by local or disseminated atypical Langerhans cells (LCs) found most commonly in bone, lungs, mucocutaneous structures and endocrine organs. Among different sites, unifocal disease confined to a lymph node (LN) is rare. We report a case of LCH confined to a LN in a 38-year-old male who presented with a 2 cm-sized cervical mass. The fine needle aspiration (FNA) smears of cervical LN showed high cellularity having isolated LCs with contorted nuclei and nuclear grooves mixed in multinucleated giant cells, small lymphocytes and eosinophils. Charcot-Leyden crystals were also seen, as were a few dendritic-like cells and intranuclear inclusions. Confirmation of LCH was made by histopathologic studies, positive reactions for S-100 protein and CD1a immunohistochemical staining and by the demonstration of Birbeck granules on electron microscopy. The differentials to be considered include dermatopathic lymphadenitis, sinus histiocytosis with massive lymphadenopathy, Hodgkin's lymphoma and malignant histiocytosis. The characteristic cytomorphologic pattern of LCH in a LN FNA smear plays an important role in suggesting the diagnosis of LCH.

• 대한두경부종양학회지
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• 제33권2호
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• pp.81-84
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• 2017

Langerhans cell histiocytosis (LCH) is a heterogeneous disease, characterized by accumulation of dendritic cells with features similar to epidermal Langerhans cells. It is a rare entity that may involve various organ levels such as the skeletal, pulmonary, hematopoietic and lympho-vascular systems. The patient was a 1-year-old female presented with fever associated with otorrhea and palpable cervical lymph node for 4 days. Neck ultrasonography and Computed tomography imaging revealed multiple enlarged lymph nodes suggesting suspicious malignant morphology. Lymph node biopsy was performed under general anesthesia. Histological and immunophenotypic examination showed the lymph node to be consistent with LCH. The patient was given chemotherapy.

• 대한두경부종양학회지
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• 제19권2호
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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.

• 대한두경부종양학회지
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• 제13권1호
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• pp.9-15
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• 1997

서론 : 혈구탐식증후근은 고열, 범혈구감소증, 간비장종대, 림프절비대 및 혈액응고장애 등을 동반하는 전신적 질환으로 대부분 면역억제 상태에서 바이러스 및 각종 병원체의 감염에 의해서 유발되고 예후가 불량하다. 조직학적으로 림프세망기관의 조직구의 증가와 혈관탐식현상이 빈번히 관찰되고 이와 같은 현상을 보이는 악성조직구증식증과의 감별이 어렵다. 코의 혈관중심위 림프종은 거의 일정한 Epstein-Barr 바이러스(EBV)양성을 보인다고 보고되고 있고 병의 경과중 혈관탐식증추군이 빈번하게 발생되는데 이는 EBV감염에 의해서 유발된다고 보아지고 있다. 재료 및 방법 : 1985년 1월부터 1995년 12까지 강남성모병원과 성바오로병원에서 코의 혈관중심위 림프종으로 진단 받았던 환자 42명 중 혈구탐식증후근을 동반한 10명을 대상으로 임상양상을 관찰하고, 조직표본에 면역조직화학염색법과 교잡반응을 사용하여 악성세포의 표현형을 살펴보고 EBV와의 관련성을 관찰하였다. 결과 : 10명의 환자 중 5명은 혈관중심위 림프종 진단당시, 3명은 재발시기, 2명은 관해 시기에 혈구탐식증후군을 동반하였다. 모든 환자에서 실시된 치료방법에 상관없이 치명적인 경과를 보였으며 중앙생존기간은 18일(2-44일)이었다. 대상 모두에서 T형세포 표현형과 교잡반응상 EBV양성을 보였으며, EBV는 주로 악성림프종세포에 분포양상을 보였다. 결론 : 혈구탐식증추군은 코의 혈관중심위 림프종의 흔한 합병증으로 불량한 예후를 보인다. 임상적 양상 및 조직학적 검사상 악성 조직구증가증과 유사한 소견을 보여 감별이 어렵고, 치명적 결과를 초래하므로 치료에 어려움을 주고 있다. 코의 혈관중심위 림프종과 밀접한 관계를 보이는 EBV에 의해서 유발된다고 보아지고 있으며 치료의 개선을 위하여 앞으로 병인적연구가 필요하다고 생각된다.

• 대한두경부종양학회지
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• 제12권1호
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• pp.16-21
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• 1996

Lymphomatoid granulomatosis, polymorphic reticulosis, midline malignant reticulosis, or lethal midline granuloma have similar histologic features of an angiocentric and angiodestructive lymphoreticular proliferation representing the same nosologic entity. The term 'angiocentric immunoproliferative lesion' (AIL) was proposed by Jaffe, Costa, and Martin. The malignant potential of AILs is originally uncertain, but the facts that AILs have a relatively short survival, and most of them usually progress to an overt malignant lymphoma and survival is inversely proportional to the large, atypical lymphoreticular cells suggest that AILs are malignane. We experienced 17 AILs in nose during 16-year period and retrogradely analized them to recognize the problems in the diagnosis and to establish the further therapeutic strategies. The results were as follows; Twelve of total 17 patients who had diagnosed as histologic grade 1 and 2 had received radiation therapy as an initial treatment and the complete response rate was 91.7%(11/12), but 6 out of 11 had local recurrence and 5 had progress to overt maligant lymphoma within 2years. Three patients with the histologic grade 3 and 2 with unclear histologic grade had received CHOP chemotherapy and there was 1 case with complete response. Two patients with unclear histologic grade had been proved to be malignant histiocytosis by bone marrow biopsy during the clinical course. The overall duration of survival was 2 - 119 months and the 5-year survival rate was 71.9%. And the achievement of initial complete response was the most important prognostic factor of overall survival(P=0.006). Our results suggest that the treatment strategy according to the histologic grading scheme is efficient and more aggressive combination chemotherapy may be necessary to achieve complete response in patients with histologic grade III and II, because most of them progress to overt malignant lymphoma during its process.

• Nuclear Medicine and Molecular Imaging
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• 제41권6호
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• pp.553-560
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• 2007

목적: F-18 FDG PET은 근골격계 종양에서 양성과 악성병변을 감별하는 유용성에 대하여 다양한 결과가 보고되고 있다. 저자들은 F-18 FDG를 이용한 PET/CT로 근골격계 종양의 maxSUV를 분석하고 비교하여 유용성을 알아보았다. 대상 및 방법: 치료 전 46개 병소(연부 조직 종양 양성/악성 : 11/12, 골종양 양성/악성 9/14)에 대하여 F-18 FDG PET/CT를 시행하였으며, 조직학적 검사로 확진하였다. 악성과 양성을 구분하는 maxSUV 절단값은 연부 조직 종양에서는 4.1, 골종양에서는 3.05로 하였다. 결과: 연부 조직 종양에서 양성(R=11; maxSUV $3.4{\pm}3.2$)과 악성(n=12; maxSUV $14.8{\pm}12.2$) 간에 maxSUV는 통계학적으로 유의하게 (p<0.001) 차이가 있었다. 민감도와 특이도는 각각 83%, 91%였다. 그러나 골종양에서는 양성 종양(n=9; maxSUV $5.4{\pm}4.0$)과 악성 골종양(n=14; maxSUV $7.3{\pm}3.2$) 간에 통계학적으로 유의한 차이를 보이지 않았다. 연부 조직 종양에서는 결절성 근막염이 위양성으로 나타났고(maxSUV=12.4) 골종양에서는 섬유성 골이형성증과 랑게르한스세포 조직구증식증 2예 및 골모세포종이 있었다. 결론: 연부 조직 종양에서 maxSUV는 양성과 악성을 감별하는데 유용하였다. 그러나 골종양의 경우에는 maxSUV가 낮은 경우에는 악성을 배제할 수 있었으나, maxSUV가 높은 경우에는 조직학적으로 조직구나 섬유모세포 등이 포함된 종양의 감별진단을 고려하여야한다.

• 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.

• 대한세포병리학회지
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• 제6권2호
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• pp.163-168
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• 1995

Ki-1 positive anaplastic large cell lymphoma is a newly described high-grade lymphoma and is defined by histopathological and immunologic criteria. We experienced a case of systemically involving Ki-1 positive anaplastic large cell lymphoma in a 44 year-old female which initially manifested as pleural effusion. Abdominopelvic CT scan showed the evidence of marked lymphadenopathy in retroperitoneal and both external and inguinal lymph nodes. On cytologic examination of pleural fluid, tumor cells revealed pleomorphic large isolated cells with prominent nucleoli and abundant cytoplasms. The nuclei were large with irregular profiles including some deep invaginations. Also, occasional multilobed/multinucleated and binucleated nuclei were seen. Immunohistochemical examination was performed to differentiate from the undifferentiated adenocarcinoma, Hodgkin's disease, non-Hodgkin's lymphoma and malignant histiocytosis. The neoplastic cells were positive for leukocyte common antigen, CD3, CD30(Ki-1) but negative for cytokeratin, epithelial membrane antigen, and CD15. A histologic diagnosis of Ki-1 positive anaplastic lymphoma was made by biopsies of the inguinal lymph node, polypoid lesions of the stomach and cecum.

• Tuberculosis and Respiratory Diseases
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• 제45권1호
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• pp.184-190
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• 1998

저자들은 발열, 체중감소 등의 증상과 흉부 X-선 사진상 폐경화의 소견이 있었던 환자에서 폐의 병변이 역형성 거대세포 림프종으로 추측되는 1례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.