• Investigative Magnetic Resonance Imaging
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• v.24 no.1
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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.

• The Korean Journal of Cytopathology
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• v.18 no.1
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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.

• Korean Journal of Head & Neck Oncology
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• v.33 no.2
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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.

• 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.

• Korean Journal of Head & Neck Oncology
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• v.13 no.1
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• pp.9-15
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• 1997

Malignant histiocytosis(MH)-like hemophagocytic syndrome(HS) is a fatal complication of nasal angiocentric lymphoma(AL) and difficult to distinguish from MH. Ten of total 42 patients with nasal AL had HS and 9 of them were initially suspected to have MH. Five patients had HS as initial manifestation, 3 at the time of relapse, and 2 during the clinical remission of lymphoma. Four patients were treated by combination chemotherapy(CHOP) and others had only supportive care. Immunohistochemical study and in situ hybridization were performed on the specimen obtained from 10 patients. The median survival of all patients from HS was 18 days(range 2 - 44 days) and all had fatal outcome regardless of the treatment-modality. All cases were positive for UCHL1(CD45RO) and Epstein-Barr virus (EBV) by EBER in situ hybridization. MH-like HS is a fatal complication of nasal AL and has a high association with EBV. Reactivation of EBV may contribute to HS and further investigation of predictive factors and effective treatment of HS should be pursued in future.

• Korean Journal of Head & Neck Oncology
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• v.12 no.1
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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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• v.41 no.6
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• pp.553-560
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• 2007

Purpose: We evaluated the standard uptake value (SUV) of F-18 FDG at PET/CT for differentiation of benign from malignant tumor in primary musculoskeletal tumors. Materials and Methods: Forty-six tumors (11 benign and 12 malignant soft tissue tumors, 9 benign and 14 malignant bone tumors) were examined with F-18 FDG PET/CT (Discovery ST, GE) prior to tissue diagnosis. The maxSUV(maximum value of SUV) were calculated and compared between benign and malignant lesions. The lesion analysis was based on the transverse whole body image. The maxSUV with cutoff of 4.1 was used in distinguishing benign from malignant soft tissue tumor and 3.05 was used in bone tumor by ROC curve. Results: There was a statistically significant difference in maxSUV between benign (n=11; maxSUV $3.4{\pm}3.2$) and malignant (n=12; maxSUV $14.8{\pm}12.2$) lesions in soft tissue tumor (p=0.001). Between benign bone tumor (n=9; maxSUV $5.4{\pm}4.0$) and malignant bone tumor (n=14; maxSUV $7.3{\pm}3.2$), there was not a significant difference in maxSUV. The sensitivity and specificity for differentiating malignant from benign soft tissue tumor was 83% and 91%, respectively. There were four false positive malignant bone tumor cases to include fibrous dysplasia, Langerhans-cell histiocytosis (n=2) and osteoid osteoma. Also, one false positive case of malignant soft tissue tumor was nodular fasciitis. Conclusion: The maxSUV was useful for differentiation of benign from malignant lesion in primary soft tissue tumors. In bone tumor, the low maxSUV correlated well with benign lesions but high maxSUV did not always mean malignancy.

• 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 Korean Journal of Cytopathology
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• v.6 no.2
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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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• v.45 no.1
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• pp.184-190
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• 1998

Anaplastic large cell lymphoma(ALCL) is a recently recognized large cell lymphoma characterized by large pleomorphic cells, with prominent nucleoli, expressing the CD30(Ki-1) antigen. It is often misdiagnosed as metastatic carcinoma, malignant histiocytosis or Hodgkin's disease. Primary pulmonary ALCL is extremely rare although it frequently involves extranodal sites, e.g. skin, bone, gastrointestinal tract, soft tissue and lung. We report a case of ALCL suspected as primarily involving lung.