례동시에 발생한 다중심성 및 다발성 교모세포종의 증례

구연무 ( Youn Moo Koo ),정현호 ( Hyun Ho Jung ),엄민섭 ( Min Sub Uhm ),오지웅 ( Ji Woong Oh ),황금 ( Kum Whang ) 대한뇌종양학회·대한신경종양학회·대한소아뇌종양학회 2011 대한뇌종양학회지 Vol.10 No.2

Objective: Multicentric glioblastoma is an uncommon condition, about 2.3 to 9% of all glioblastoma. There have been few reports about the prognosis of multicentric glioblastoma after multimodal treatment. We describe our experience of one case of multicentric glioblastoma. Case: A 50-year-old male patient presented to the neurosurgery clinic with complex partial seizure attack and partial conduction aphasia. A brain magnetic resonance image (MRI) scan showed two enhanced lesions on left temporal and occipi-tal lobes and one on right frontal lobe. Left temporal lesion was partially resected and the pathologic diagnosis was glio-blastoma. Right frontal lesion was also removed totally and it was glioblastoma. Concomitant chemoradiation therapy (CCRT) with Temodal was started. At the time of 6th cycle, right hemiparesis developed and the follow-up MRI revealed progression of left temporal region. And he expired 54 weeks after diagnosis. Conclusion: We experienced a patient of synchronous multicentric and multifocal glioblastoma who showed 12 months of survival period after CCRT with tumor resection.

Clinical features and outcomes in patients with human immunodeficiency virus-negative, multicentric Castleman’s disease: a single medical center experience

서세영,유창훈,윤덕현,김신,박정선,박찬식,허주령,서철원 대한혈액학회 2014 Blood Research Vol.49 No.4

Background Multicentric Castleman’s disease (CD) is commonly associated with poor prognosis, and well-known prognostic factors are scarce. We performed a retrospective analysis to define the clinical features and prognostic factors for patients with multicentric CD. Methods Between 1990 and 2013, 32 patients with multicentric CD were identified from the database of the Asan Medical Center, Seoul, Korea. Clinicopathologic data were collected by reviewing the medical records. With the exclusion of 4 patients because of unknown human immunodeficiency virus infection status, 28 human immunodeficiency virus-negative patients with multicentric CD were included in this analysis. Results Most of the patients were male (76%) and had a median age of 54 years. Hyaline vascular variant was the most common subtype (N=11, 39%). Hepatosplenomegaly (61%), fever (39%), edema (29%), and ascites (18%) were the most frequently reported symptoms and signs at diagnosis. With a median follow-up of 67 months, the 5-year overall survival (OS) was 77%. Patients with extravascular fluid accumulation (i.e., peripheral edema, ascites, and/or pleural effusions) were significantly associated with a poor survival rate (5-year OS, 94% vs. 56%; P=0.04). The extent of disease involvement was also a significant prognostic factor (5-year OS, 91% for involvement on a single side vs. 73% on both sides of the diaphragm; P=0.03). Other clinicopathologic factors were not significantly associated with patient survival. Conclusion Our findings suggest that the hyaline vascular variant is not a rare subtype of multicentric CD. Extravascular fluid accumulation and disseminated disease involvement seem to be significant prognostic factors.

Clinical features and outcomes in patients with human immunodeficiency virus-negative, multicentric Castleman’s disease: a single medical center experience

서세영,유창훈,윤덕현,김신,박정선,박찬식,허주령,서철원 대한혈액학회 2014 Blood Research Vol.49 No.4

Background Multicentric Castleman’s disease (CD) is commonly associated with poor prognosis, and well-known prognostic factors are scarce. We performed a retrospective analysis to define the clinical features and prognostic factors for patients with multicentric CD. Methods Between 1990 and 2013, 32 patients with multicentric CD were identified from the database of the Asan Medical Center, Seoul, Korea. Clinicopathologic data were collected by reviewing the medical records. With the exclusion of 4 patients because of unknown human immunodeficiency virus infection status, 28 human immunodeficiency virus-negative patients with multicentric CD were included in this analysis. Results Most of the patients were male (76%) and had a median age of 54 years. Hyaline vascular variant was the most common subtype (N=11, 39%). Hepatosplenomegaly (61%), fever (39%), edema (29%), and ascites (18%) were the most frequently reported symptoms and signs at diagnosis. With a median follow-up of 67 months, the 5-year overall survival (OS) was 77%. Patients with extravascular fluid accumulation (i.e., peripheral edema, ascites, and/or pleural effusions) were significantly associated with a poor survival rate (5-year OS, 94% vs. 56%; P=0.04). The extent of disease involvement was also a significant prognostic factor (5-year OS, 91% for involvement on a single side vs. 73% on both sides of the diaphragm; P=0.03). Other clinicopathologic factors were not significantly associated with patient survival. Conclusion Our findings suggest that the hyaline vascular variant is not a rare subtype of multicentric CD. Extravascular fluid accumulation and disseminated disease involvement seem to be significant prognostic factors.

다발성 연골육종 1례 보고

전대근,이종석,김석준,이수용,Jeon, Dae-Geun,Lee, Jong-Seok,Kim, Sug-Jun,Lee, Soo-Yong 대한근골격종양학회 1997 대한골관절종양학회지 Vol.3 No.2

Multicentric chondrosarcoma other than the mesenchymal subtype is rare separate entity. We experienced a case with nonmonomelic synchronous multicentric chondrosarcoma without any preexisting lesions of Oilier's disease or Maffucci's syndrome. To our knowledge, there was no report of synchronous nonmonomelic multicentric chondrosarcoma. A thirty-three year old man had right distal thigh pain of one and half year. Bone scan showed hot lesions on medial condyle of right femur and shaft of left femur. Plain X-ray showed osteolytic lesion on right femur and slight cortical thickening and calcific lesion was observed on left femoral shaft. Curettage and bone cement filling was done on both lesions. The pathology reports were grade I chondrosarcoma on both side of femur. At one month from operation, pathologic fracture of left femur occurred on bone cement-host bone junction. Conservative treatment and radiotherapy of 60Gy was done. At 8 months from operation, nonunion was evident. Segmental resection of left femur with contralateral fibula graft and second look operation on right condyle lesion were done. At 6 months from revision, fracture occurred at host-graft bone junction. We removed previous hardware and applied long DCP and massive autogenous bone graft. Afterwards, the patient looks good and union was progressing. But at 4 years from last operation, hypertrophic nonunion occurred. Another revision was done with condylar plate and bone graft and now he is well without any sign of local recurrence or metastasis.

다발성 유아기 흑색 신경외배엽성 종양의 치험례

최병환(Byoung-Hwan Choi),박수원(Su-Won Park),장수미(Soo-Mi Jang),박봉찬(Bong-Chan Park),손한나(Han-Na Son),손장호(Jang-Ho Son),성일용(Iel-Yong Sung),김종렬(Jong-Ryoul Kim),조영철(Yeong-Cheol Cho) 대한구강악안면외과학회 2010 대한구강악안면외과학회지 Vol.36 No.5

A melanotic neuroectodermal tumor of infancy (MNTI) is a uncommon osteolytic pigmented neoplasm that primarily affects the jaws of newborn infants. Most patients (> 90%) present with the tumor in the first year of life. Approximately 65% form in the maxilla, 11% in the mandible, 5% in the brain and elsewhere. MNTI is normally benign, but up to 15% may recur and a few have metastasized. Approximately 200 cases of MNTI have been reported but only 2 of them presented as multifocal. A case of MNTI in a 7 month old boy was encountered. The chief complaint was maxillary anterior ridge swelling. The incisional biopsy findings were MNTI. Two months after the first operation, mild swelling of another site was observed. The infant was examined periodically since undergoing two procedures with no recurrence. This case demonstrates the possibility of a multicentric MNTI. We report a multicentric MNTI with a review of the relevant literature

Multicentric T cell lymphoma in a Maltese dog

Jung, Ji-Youl,Kang, Sang-Chul,Roh, In-Soon,Sohn, Hyun-Joo,Yun, Young-Min,Kim, Jung-Hun,Lee, Kyoung-Kap,An, Min-Chan,Bae, Jong-Hee,Kim, Jae-Hoon The Korean Society of Veterinary Science 2007 大韓獸醫學會誌 Vol.47 No.1

A case of multicentric high grade T cell lymphoma is reported in a 5-year-old male Maltese dog with generalized lymphadenopathy. The dog showed depression, anorexia, blindness, jaundice, arrhythmia, and hematuria for 8 months. Complete blood count and chemistry profile revealed anemia and increased alanine transferase, alkaline phosphatase, total bilirubin, and total cholesterol. Grossly, most of lymph nodes, spleen, and liver were enlarged and neoplastic masses were occupied in these tissues. Histologically, massive accumulation of small noncleaved neoplastic lymphocytes with high mitotic figures was observed in all lymph nodes and spleen. Infiltration of neoplastic lymphocytes was also noted in the lung, liver, kidney, eye, skin, muscle, and bone marrow of femur. Immunohistochemistry revealed that tumor cells were CD3-positive and but CD79a-negative, consistent with T-cell lineage. In our best knowledge, this is the first report of multicentric lymphoma clarified the origin of tumor cells in Korea.

림프절생검과 골수검사를 통해 진단된 다중심성 캐슬만병 1예

박상혁,허주령,지현숙,서철원,박찬정,장성수 대한혈액학회 2009 Blood Research Vol.44 No.3

Castleman’s disease (CD) is an uncommon lymphoproliferative disorder presenting with a variable spectrum of clinical manifestations. CD is classified into two subtypes, Localized CD and Multicentric CD, by clinical manifestation, and is divided into 2 histopathologic types: a hyaline-vascular type (HV) or a plasma-cell type (PC). Multicentric CD show PC type predominantly and show systemic symptoms, such as fever, night sweat, malaise, ascites, edema, and more than half show multiple lymphadenopathy and hepatosplenomegaly. We report a case of 67 year old man with multicentric CD presented with fever of unknown origin and diagnosed with lymph node biopsy and bone marrow examination with a brief review of the literature.

례전이성 뇌종양과 혼돈된 다발성 교모세포종

이수환 ( Su Whan Lee ),김해유 ( Hae Yu Kim ),이선일 ( Sun Il Lee ) 대한뇌종양학회·대한신경종양학회·대한소아뇌종양학회 2011 대한뇌종양학회지 Vol.10 No.1

Introduction:Multicentric glioblastoma is rare disease entity, which is frequently mimicking metastatic brain tumor. We experienced a case of multicentric glioblastoma which was diagnosed finally after multiple craniotomies for the multicentric intracranial lesions having been believed as metastatic brain tumor. We reported this rare case with literature reviews. Case Report:A 48-year-old man had underwent Novalis radiosurgery for multiple malignant tumors in other institute. He had no other malignant lesion in other part of body. He was followed up for 10 months and was taken brain magnetic resonance image (MRI) which showed progressed and newly developed intracranial lesions. He visited our institute with the images for Gamma Knife radiosurgery. During follow up after second radiosurgery, he developed neurological symptoms due to increasing intracranial lesions. We performed multiple craniotomies for large two lesions. He gradually recovered after surgery. Pathological report was glioblastoma. He underwent chemotherapy without radiotherapy due to previous radiosurgeries. Conclusion:This case`s intracranial lesions were considered as metastatic brain tumor undiagnosed primary tumor due to the multicentricity and formation of new lesions for several months. However this was not based on pathological diagnosis which should be necessary to decide precise treatment. This rare case might give us an education about the importance of consideration of invasive procedures for pathological diagnosis in the case of multiple intracranial lesions without evidence of extracranial lesions.

A case of multicentric reticulohistiocytosis, concomittent with rheumatoid arthritis

( Jae Ho Lee ),( Youngkyoung Lim ),( You Jin Lee ),( Jong Yoon Chung ),( Ji Hye Park ),( Jong Hee Lee ),( Dong Youn Lee ),( Joo Heung Lee ),( Jun Mo Yang ) 대한피부과학회 2015 대한피부과학회 학술발표대회집 Vol.67 No.1

Multicentric reticulohistiocytosis (MRH) is a rare systemic inflammatory granulomatous disease marked by severe and often rapidly progressive polyarticular arthritis and cutaneous papulonodules. These features are similar to those found in some rheumatic diseases, such as rheumatoid arthritis or dermatomyositis, and this can lead to a misdiagnosis. Herein, we report a female patient with polyarthralgia and subsequent skin eruptions, who was eventually diagnosed with MRH. She was on treatment of rheumatoid arthritis from department of rheumatology. this case can bring an educational implication, because MRH presented with polyarthralgia can cause misdiagnosis with rheumatoid arthritis

[P059] A case of multicentric reticulohistiocytosis misdiagnosed as rheumatoid arthritis

( Wooseok Jeong ),( Woojung Jin ),( Seunghyun Moon ),( Hyun Hwangbo ),( Sookkyung Lee ) 대한피부과학회 2017 대한피부과학회 학술발표대회집 Vol.69 No.1

Multicentric reticulohistiocytosis(MRH) is a rare disease of unknown etiology that is characterized by skin and mucosal nodular eruptions and arthritis. MRH can result in severe disfigurement of skin and rapidly destructive, irreversible deforming arthritis. A quarter of patients with MRH have associated with internal malignancy. It is easily possible to confuse MRH with other more common autoimmune inflammatory conditions like rheumatoid arthritis. We report here a case of MRH misdiagnosed as rheumatoid arthritis. A 69-year-old woman presented with multiple joint pain that had lasted for 2 years and skin nodules present for 4 months. She had been diagnosed with rheumatoid arthritis and was being treated at another hospital. She had tender reddish-brown papulonodules on the elbows, hands, ears, and nose. Histopathological findings showed large mononucleated cells with finely granular ground glass appearing cytoplasm. The multinucleated giant cells were large and irregular, with many haphazardly arranged nuclei. In order to screen for associated malignancies or autoimmune diseases, we performed mammography, gastro-colonoscopy, abdomen computed tomography(CT), and autoimmune antibody tests. The test results were within normal ranges. Prompt and accurate diagnosis of MRH is important to prevent irreversible disabilities and negative prognosis due to missing associated malignancies or autoimmune diseases.