• Title/Summary/Keyword: Eosinophilic granuloma

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MULTIPLE LANGERHANS CELL HISTIOCYTOSIS AT THE LUNG AND MANDIBLE: CASE REPORT (폐와 하악골에 다발성으로 발생한 Histiocytosis X)

  • Hwang, Jin-Hyuk;Lee, Bu-Kyu
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • v.31 no.3
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    • pp.279-282
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    • 2005
  • Hitiocytosis X, recently termed Langergans cell histiocytosis, is a rare disease histologically characterized by an abnormal proliferation of histiocytes, and of which the pathophysiology is still ambiguous. The clinical presentation is highly varied. Typically, there is a solitary or multiple bony involvements, and less frequently, lesions are found in other organs such as the lungs, liver, lymph nodes, skin, and mucosae. Interestingly, so far, there has been no report which shows multiple developments in both soft and hard tissues. In this report, we present a case of hitiocytosis X in both mandible and lung. This may provide some information for understanding the pathophysilogy of the histiocytosis X.

REPORT OF EXPERIENCE WITH KIMURA'S DISEASE (기무라씨 질환, 5 예 보고)

  • Seel David J.;Park Yoon-Kyu;Lee Kwang-Min
    • Korean Journal of Head & Neck Oncology
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    • v.5 no.1
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    • pp.39-46
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    • 1989
  • Kimura's Disease is a chronic inflammatory and proliferative condition producing subcutaneous masses especially in the head and neck area. This report of our experience with 5 patients with this disease is the first in the Korean surgical literature. Kimura's Disease is thought to be part of the larger spectrum of the entity known as angiolymphoid hyperplasia with eosinophilia (ALHE). It is characterized pathologically by hyperplastic lymphoid follicles, eosinophilic infiltration, and vase 비 ar proliferation. It produces masses which are most common in the area of the parotid, submandibular gland and upper neck. These masses occupy the subcutaneous tissues but also extend into salivary tissue and into upper neck nodes. One of our patients had masses in the groin. The tumors are extremely vascular due to the presence of new proliferative vessels and sinusoids. The average age of our 5 patients was 35, but all but one case were younger than 38 years of age. The male: female ratio was 3 : 2, and the average duration of symptoms was 5,2years. All patients had peripheral blood eosinophilia. All had multiple masses, sometimes symmetrical. The management was surgery alone in one case, surgery and steroids in one case, surgery and radiotherapy in two cases, and all three modalities in one case. The relationship of this entity to ALHE and our experience in the management of this disease are presented. A clinicopathological discrepancy alerted us to the existence of Kimura's Disease. A nineteen-year old male presented with subcutaneous masses over both mastoid areas present for 3 years (Case III). When biopsy on each side was reported as 'eosinophilic granuloma' we submitted the slides to an internationally expert pathologist. Symmetrically occurring tumors in the peri-parotid subcutaneous areas did not fit any category of neoplasm or granuloma known to us. The diagnosis, made by Dr. Gist Fan at the Ochsner Clinic, was Kimura's Disease. We found two additional cases in a review of soft tissue eosinophilic granuloma previously reported at Presbyterian Medical Center, and since then have diagnosed two new cases. These five cases constitute the basis for this, the largest series to be reported in Korea. These vascular, tumor-like lesions of the skin, subcutaneous areas and subjacent structures of the head and neck have been a variety of names, such as angiolymphoid hyperplasia with eosinophilia, eosinophilic hyperplastic lymphogranuloma, angioblastic lymphoid hyperplasia with eosinophilia, histioid hemangioma, and epithelioid hemangioma. The history of this disease spectrum dates back to 1937 when Kimm and Szeto (1) reported 7 cases of 'eosinophilic hyperplastic lymphogranuloma' in the Proceedings of the Chinese Medical Journal. In 1948 Kimura and his associates(2) reported additional cases in Japan under the title 'On the unusual granulation combined with hyperplastic changes of lymphatic tissue.' From then until 1966 several hundred cases were reported in China and Japan. The first report from the West was by Wells and Whimster(3) in the British Journal of Dermatology, in 1969. These authors coined the term, angiolymphoid hyperplasia with eosinophilia (ALHE). Since that time a debate has ensued as to whether Kimura's Disease and ALHE are distinct entities, or whether Kimura's is part of the larger spectrum of ALHE, perhaps a later or advanced phase. From the clinical perspective, surgeons should be aware of the diagnosis of Kimura's Disease not only as part of the differential diagnosis of head and neck tumors but also because these lesions are indolent, and generally require conservative surgical removal as part of the management program. CASE I. A 37-year-old female company employee presented in August 1982 with submental swelling of 12 years' duration and with inguinal swelling of 7 years' duration. The submental mass measured 5x5cm. and the inguinal mass was 8x4cm. in size. Peripheral eosinophilia varying from 14% to 40% was found. On August 20, 1982, the submental mass was removed and a superficial groin dissection was done. In May 1983 an intraoral lesion of the palate was removed. The patient is free of disease. CASE II. A 23-year-old unemployed man visited this hospital for the first time in July, 1984, with swelling of the right cheek present for 6 years. The mass was soft and ill-defined but measured 10x20cm. and extended from the submandibular upper neck to the zygomatic arch, and from the mastoid to the cheek, over the parotid gland. Eosinophilia varying from 27% to 29% was noted in the peripheral blood. On March 21, 1986, the lesion was resected. The procedure comprised an extended superficial parotidectomy from the temporalis fascia to the upper neck. Post-operatively radiotherapy 3000 rad tissue dose was administered using the 6 MeV linear accelerator. The patient remains free of disease. CASE III. A 19-year-old student came to the clinic with masses over both mastoid areas, present 3 years. On the right there were two adjacent lesions, one over the mastoid, the other in the upper jugular level of the neck. On the left it was a single mass over the mastoid. Eosinophilia varied from 13 to 32% in the peripheral blood, and 11.6% in the bone marrow. Incisional biopsy revealed 'eosinophilic granuloma' and a trial of predisolone was employed. The mass increased in size so a small dose of radiation (600 rads) was used, with substantial regression,. The lesion on the left was excised and follwed by 1000 rads radiotherapy. Finally recurrent tumor on the right side was removed on November 5, 1985. The patient remains free of disease. CASE N. A 29-year-old local merchant had had swelling of both upper necks since childhood. At the time of his first visit on March 17, 1986, the right submandibular mass measured 5x3.5cm. and the ,right upper neck and parotid tail mass measured 2.5cm. On the left there were masses in the upper neck, the largest of which measured 2.5cm, and of the parotid tail, 2.0cm. in size.(See Fig. 1) Peripheral eosinophilia of 39% was recorded. Left side partial parotidectomy and resection of the upper neck and subdigstric mases was done on May 2, 1986. The mass involving the right parotid tail and upper neck nodes was removed on Angust 7,1986. Postoperatively the patient was placed on prednisolone 30 mg. per day. No definite masses are palpable. CASE V. A 66-year-old housewife informed us, at the time of her first visit in May, 1986, that she had had multiple neck masses since 10 years ago. On the right side there was a 2.5cm. subcutaneous mass of the upper neck, over the upper jugular chain. On the left there was a 9x4.5cm. mass involving the entire parotid, the post-auricular area and the upper neck. A third mass presented in the submental area and measured 3.5cm. (See Fig. 2) Eosinophilia of 51% was noted in the peripheral blood. partial excision of the left upper neck lesion and complete excision of the submental mass were performed on june 6, 1986. post-operatively she was placed on 20 mg. of prednisolone daily, but when the mass re-grew after two months she was referred to Radiation Therapy for a 2500 rad course of treatment. A barely palpable thickening remains.

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A case of extragastrointestinal anisakiasis involving a mesocolic Iymph node (횡행결장간막의 림프절을 침범한 고래회충유충증의 장외감염 1례)

  • Kim, Hui-Jeong;Park, Chan-Il;Jo, Seung-Yeol
    • Parasites, Hosts and Diseases
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    • v.35 no.1
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    • pp.63-66
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    • 1997
  • In a 43-year-old Korean man who underwent radical gastrectomy due to a malignant stromal tumor, was found to have an enlarged Iymph node at transverse mesocolon. The Iymph node exhibited histologically necrotizing eosinophilic granuloma formed around a track containing sections of a nematode larva. The well preserved nematode sections revealed polymyarian muscle cells, Y-shaped lateral cord, a large excretory gland cell, intestine and eosinophilic cuticle. The nematode sections were identified as a larva of Anisnkis species In Korea, thIns is the first case of elrtragas trio into stinal anisakiasis.

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USE OF CORTICOSTEROIDS IN THE TREATMENT OF LOCALIZED LANGERHANS CELL HISTIOCYTOSIS OF THE MANDIBLE : CASE REPORT (Corticosteroid를 이용한 하악에 발현된 Langerhans Cell Histiocytosis의 치료 : 증례보고)

  • Choi, Jung-Yong;Lee, Sang-Hwa;Yoon, Hyun-Joong
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.31 no.3
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    • pp.249-253
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    • 2009
  • Langerhans cell histiocytosis(LCH), previously known as histiocytosis X, is rare, proliferative disorder in which the accumulation of pathologic Langerhans cell leads to tissue infiltration and destruction. The jaw is involved 10~20% of all LCH and most common oral symptoms are jaw swelling or a palpation mass. Most of subjects are males and disease appears to start before the age of 10 years. An 11 years old girl was referred from pediatric department. Histological examination confirmed the diagnosis LCH. She was treated intra-lesional injection of corticosteroid. The overall outcome was excellent. After a follow-up period of 20 month, the patient present with no evidence of residual disease on Panoramic view and whole body bone scan. We report this early recognized LCH case treated by corticoid injection in good progress with literature review.

A Pathological Survey on Liver Lesion of Slaughtered Native Cattle with Special Reference to Fascioliasis (한우(韓牛)의 도축검사례(屠畜檢査例)에 의한 간병변조사(肝病變調査) : 특히 간질증병변(肝蛭症病變)에 관하여)

  • Joh, Tae-Sun;Park, Bong-Joh;Kang, Su-Wha;Bak, Ung-Bok
    • Korean Journal of Veterinary Research
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    • v.16 no.1
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    • pp.35-44
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    • 1976
  • The livers of 300 cases of Korean native cattle which were collected at slaughted house in Pusan were examined by gross and histopathological means to study patterns of pathological changes of the organ. 1. The most dominated lesion of the liver was those of fascioliasis showing incidence rate as 907 per cent of inspected cattle. 2. On age distribution of the fascioliasis lesion in the liver, higher incidence was seen in animals that were from 6 to 9 years of age. 3. Significant changes of the hepaticparenchyma caused by immature flukes included thrombosis, migrational tracks such as hemorrhagic foci with massive eosinophilia and hepatic necrosis. The changes of chronically infected livers of cattle were recognized as proliferation of periductal eosinophilic granuloma of various degrees in each portal triad. And this easential cholangiohepatitis might develop into multiple focal cirrhosis or diffuse perilobular cirrhosis. 4. Most cases with gross lesion of swelled bile ducts on visceral surface of the liver showed histological evidences of fascioliasis lesion throughout hepatic lobes.

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Fine Needle Aspiration Cytology of Langerhans Cell Histiocytosis (Langerhans세포 조직구증의 세침흡인 세포학적 소견 - 1 예 보고 -)

  • Kwak, Jeong-Ja;Jin, So-Young;Lee, Dong-Wha
    • The Korean Journal of Cytopathology
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    • v.4 no.2
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    • pp.140-145
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    • 1993
  • Langerhans cell histiocytosis or histiocytosis X is a disease of unknown etiology characterized by proliferation of mature histiocytes. While a few descriptions of the cytologic features of eosinophilic granuloma ocurring in the bone have been published, reports of cytologic findings of lymph node-based Langerhans cell histiocytosis are very rare. We report the cytologic findings of a case of Langerhans cell histiocytosis diagnosed by fine needle aspiration cytology from the left supraclavicular and right inguinal lymph nodes in a 65-year-old male. Cytologic smears showed characteristic reticuloendothelial cells which have elongated, folded, grooved nuclei and abundant pale cytoplasms. Particularly, nuclei were highly irregular and multilobated. A few mitotic figures were present. The cytologic diagnosis was confirmed by tissue biopsies from the left supraclavicular and right inguinal lymph nodes. Proliferation of histiocytes were also present in the skin. Immunohistochemistry for S-100 protein, vimentin, $\alpha1-antichymotrypsin$ and lysozyme showed positive staining. Electron microscopy disclosed Birbeck granules.

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A Case of Bilateral Retro-Auricular Kimura's Disease (양측 후이개 종물로 발병한 Kimura씨 병 1예)

  • Kim, Yoonjoong;Jeon, Hyoung Won;Kim, Min-Soo;Ahn, Soon-Hyun;Jeong, Woo-Jin
    • Korean Journal of Head & Neck Oncology
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    • v.30 no.2
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    • pp.83-86
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    • 2014
  • Kimura's disease is a chronic inflammatory disease with unknown etiology. The most common clinical presentation of Kimura's disease is non-tender unilateral mass in the head and neck area, but bilateral involvement has rarely been reported. Histopathological confirmation should be taken for the diagnosis. Various management options are available, including surgical excision, steroid therapy, and radiotherapy. We report a male patient diagnosed as Kimura's disease involving bilateral retro-auricular region. Simultaneous bilateral neck involvement is an extremely rare finding. For cosmetic reasons, the patient was treated surgically, with satisfactory postoperative results. When evaluating patients with mass lesion in bilateral parotid/retro-auricular area, Kimura's disease should be considered as differential diagnosis.

CLINICAL STUDY OF HISTIOCYTOSIS-X OF THE JAW BONES (악골에 생긴 조직구증-X의 임상증례 고찰)

  • Kim, Jong-Chul;Bae, Yang-Il;Jung, Ki-Young
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.18 no.4
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    • pp.734-740
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    • 1996
  • Histiocytosis-X is a disorder characterized by proliferation of cells, phenotype of Langerhans' cells. Traditionally, these diseases include three subtypes of commen histologic features and clinical findings respectively. Eosinophilic granuloma shows a solitary or multiple skeletal lesions. Hand-Schuller-Christian disease often fatal due to widespread extraskeletal lesions. Patient' chief compaints are frequent bone pain and swelling. During rescent 10 years in our hospital, 13 patients, 11 male and 2 female, were diagnosed as Histiocytosis-X. And we evaluated clinical features, radiographic finding, histological characteristics and treatment modalities according to the each subtypes. Major treatment methods of the eosinophic granulma was a chemotherapy combined with local currettage, wide resection of the lesion should be performed carefully in selective cases.

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Langerhans cell histiocytosis

  • Oh, Su-Jin;Kim, Cheoul-Hun;Choi, Soel-Hi;Hoe, Jun;Park, Sung-Hwan;Jang, Chang-Dug;Shin, Sang-Hun;Hwang, Hee-Sung
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.18 no.4
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    • pp.647-651
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    • 1996
  • Langerhans cell histiocytosis(LCH) appears to arise from Langerhans cell and comprises a spectrum of clinical disease previously described in the literature by a variety of eponyms including histiocytosis X, eosinophilic granuloma, Hand-Schuller-Christian disease, and Letterer-Siwe syndrome. This rare disorder occurs in all groups, predominently affecting children & young adults. LCH has a wide spectrum of clinical features. The differentiation of several forms of this disease is primarily a clinical and not a histologic one. The radiographic characteristics include the appearance of solitary "intraosseous" lesions, the multiplicity of "alveolar bone" lesions, the bone lesions, periosteal new bone formation, and slight root resorption. Prognosis of a single bone lesion, is known to be excellent. In contrast, disseminated disease has seen associated with a chronic course, a high rate of morbidity and late consequences, and possible mortality. Treatment of LCH remains problematic. Treatment of multisystem disease, where organ function is being compromised has generally been with high-dose systemic corticosteroids or multiple chemotherapy.

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LANGERHANS CELL HISTIOCYTOSIS IN THE JUVENILE MANDIBLE (소아의 하악골을 침범한 랑거한스세포 조직구 증식증의 임상적 고찰)

  • Kang, Yeon-Hee;Park, Se-Hyun;Seo, Dong-Jun;Cha, In-Ho;Yi, Choong-Kook;Kim, Hyun-Sil;Kim, Jin;Kim, Hyung-Jun
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.30 no.6
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    • pp.577-583
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    • 2008
  • Langerhans cell histiocytosis (LCH) is characterized by proliferation of pathological Langerhans cells within different organs. It mainly affects children, but adult cases also occur, with an incidence rate of one to two per million. LCH results from the clonal proliferation of Langerhans cells. And its etiopathogenesis is still unknown. The hypothesis that it is a neoplastic or inflammatory disease, as well as the existence or not of immunological, viral or genetic predisposing factors, has been widely discussed in the literature, but no conclusive proof has ever been provided. Although lesions may appear in tissues of various origins such as skin, hypothalamus, liver, lung, or lymphoid tissue, bone is the most common site of the disease. The head and neck are affected in almost 90% of cases. The maxillary and mandibular bones are affected in 5 to 10% of cases. In our report, we present four cases of LCH in patients aged 3, 4, 7 and 9 years respectively, with primary manifestation in maxillofacial area.