scholarly journals Unusual Location for Langerhans Cell Histiocytosis: Basisphenoid extended to Parapharyngeal Space

2016 ◽  
Vol 8 (1) ◽  
pp. 38-40
Author(s):  
Asif Salimov ◽  
Ahmet E Suslu ◽  
Serdar Ozer ◽  
Taner Yilmaz ◽  
Hatice IY Bajin
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Parapharyngeal Space ◽  
Langerhans Cell ◽  
Unknown Etiology ◽  
Endoscopic Endonasal ◽  
Unusual Location ◽  
First Case ◽  
Rare Location ◽  
Magnetic Resonance Imaging Mri ◽  
The Right

ABSTRACT Langerhans cell histiocytosis (LCH) is a rare disease with unknown etiology involving abnormal proliferation of histiocytes. We hereby describe an LCH that has a rare location. A 4-year-old female patient was referred to our clinic with headache lasting for 2 months. Magnetic resonance imaging (MRI) showed an expansile mass on the level of basisphenoid extended to the right parapharyngeal space with dense contrast enhancement. The patient underwent endoscopic endonasal transsphenoidal surgery for biopsy of the mass. Immunohistochemical and pathological studies confirmed LCH diagnosis. This is the first case report of LCH extended to the parapharyngeal space in the current literature. How to cite this article Salimov A, Suslu AE, Ozer S, Yilmaz T, Bajin HIY. Unusual Location for Langerhans Cell Histiocytosis: Basisphenoid extended to Parapharyngeal Space. Int J Otorhinolaryngol Clin 2016;8(1):38-40.

Langerhans' cell histiocytosis – A rare cause of sudden onset unilateral sensorineural hearing loss

1999 ◽  
Vol 113 (12) ◽  
pp. 1098-1100 ◽  
Author(s):  
I. Hore ◽  
R. B. Mitchell ◽  
G. Radcliffe ◽  
R. Quiney ◽  
T. Walker
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Langerhans Cell Histiocytosis ◽  
External Beam Radiotherapy ◽  
Sudden Onset ◽  
Langerhans Cell ◽  
Sensorineural Hearing ◽  
Magnetic Resonance Imaging Mri ◽  
One Year ◽  
The Right

AbstractLangerhans' cell histiocytosis is a rare disorder of unknown aetiology in which pathological Langerhans' cells accumulate and destroy local tissue. We report a 38-year-old female who presented with a sudden onset of left sensormeural hearing loss. Magnetic resonance imaging (MRI) revealed a contrast-enhancing lesion in the left mastoid and a second lesion in the hypothalamus. Following left mastoid exploration and biopsy a definitive diagnosis of Langerhans' cell histiocytosis was made and the patient was treated with external beam radiotherapy. Subsequent right femur and right mastoid involvement were successfully treated with steroids and cytotoxic chemotherapy. At one year follow-up the patient had residual left-sided sensorineural hearing loss with normal hearing in the right ear. To our knowledge, Langerhans' cell histiocytosis has not been previously reported as a cause of unilateral sudden onset sensorineural hearing loss. It should be considered in the differential diagnosis of this condition.

scholarly journals Langerhans cell histiocytosis of the orbit and frontal sinus of the adult woman: A first case report in Poland

2019 ◽  
Vol 10 ◽  
pp. 234
Author(s):  
Kacper Kostyra ◽  
Bogusław Kostkiewicz
Keyword(s):  
Case Report ◽  
Frontal Sinus ◽  
Langerhans Cell Histiocytosis ◽  
Histopathological Examination ◽  
Langerhans Cell ◽  
Radical Excision ◽  
Multisystem Disease ◽  
Upper Eyelid ◽  
First Case ◽  
The Right

Background: Langerhans cell histiocytosis (LCH) is a term describing a clonal proliferation of pathologic Langerhans cells (histiocytes), which may manifest as unisystem (unifocal or multifocal) or multisystem disease. LCH is a rare cause of the orbital tumor with the predilection to its lateral wall which is particularly common in children. Case Description: We report an unusual case of a 33-year-old woman, 6 months after childbirth, who presented with the edema of the right orbit and upper eyelid with headaches. On physical examination, the patient had a right superior and lateral swelling of the eyelid and the orbit and right enophthalmos, without blurred vision. Magnetic resonance imaging showed well-defined, expansile, intensely homogeneously enhancing mass lesion in the right superolateral orbital rim with the destruction of the upper wall of the orbit, growing into the frontal sinus and frontal part of the cranium with the bold of the dura mater in this region. Radical excision of the tumor was achieved through a right fronto-temporo-orbito-zygomatic craniotomy. Histopathological examination had confirmed the diagnosis of the LCH. The patient was discharged home with a modified Rankin Scale score of 0. Conclusion: The main purpose of this case report is that LCH should be considered as one of the possible causes of quickly appearing tumor of the orbit in adults.

scholarly journals Multisystem Langerhans cell histiocytosis coexisting with metastasizing adenocarcinoma of the lung: A case report

2013 ◽  
Vol 70 (12) ◽  
pp. 1159-1161
Author(s):  
Aleksandra Lovrenski ◽  
Mirna Djuric ◽  
Istvan Klem ◽  
Zivka Eri ◽  
Milana Panjkovic ◽  
...  
Keyword(s):  
Case Report ◽  
Lung Adenocarcinoma ◽  
Langerhans Cells ◽  
Langerhans Cell Histiocytosis ◽  
Incidental Finding ◽  
Lung Parenchyma ◽  
Langerhans Cell ◽  
Heavy Smoker ◽  
Unknown Etiology ◽  
The Right

Introduction. Langerhans cell histiocytosis (LCH) is an uncommon disease of unknown etiology characterized by uncontrolled proliferation and infiltration of various organs by Langerhans cells. Case report. We presented a 54-year-old man, heavy smoker, with dyspnea, cough, hemoptysis, headache and ataxia, who died shortly after admission to our hospital. On the autopsy, tumor was found in the posterior segment of the right upper pulmonary lobe as well as a right-sided occipitoparietal lesion which penetrated into the right ventricle resulting in internal and external hematocephalus. Histologically and immunohistohemically, the diagnosis of primary lung adenocarcinoma with brain metastasis was made (tumor cells showed positivity for CK7 and TTF-1 which confirmed the diagnosis). In the lung parenchyma around the tumor, as well as in brain tissue around the metastatic adenocarcinoma histiocytic lesions were found. Light microscopic examination of the other organs also showed histiocytic lesions involving the pituitary gland, hypothalamus, spleen and mediastinal lymph nodes. Immunohistochemical studies revealed CD68, S-100 and CD1a immunoreactivity within the histiocytes upon which the diagnosis of Langerhans' cells histiocytosis was made. Conclusion. The multisystem form of LCH with extensive organ involvement was an incidental finding, while metastatic lung adenocarcinoma to the brain that led to hematocephalus was the cause of death.

Unusual Location of Central Nervous System Langerhans Cell Histiocytosis: Case Report

2003 ◽  
Vol 48 (3) ◽  
pp. 213
Author(s):  
E Yup Kim ◽  
Jae Kyu Lee ◽  
Chan Kyo Kim ◽  
Chang Hyun Lee ◽  
Chang Ho Kang ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Case Report ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Unusual Location

scholarly journals Langerhans Cell Histiocytosis with Isolated Central Diabetes Insipidus, Low Grade Fever and Sellar Erosion

2021 ◽  
Author(s):  
İclal Okur ◽  
Hasan Ari ◽  
Semra Çetinkaya ◽  
Betül Emine Derinkuyu ◽  
Gizem Çağlar ◽  
...  
Keyword(s):  
Diabetes Insipidus ◽  
Langerhans Cell Histiocytosis ◽  
Bone Structure ◽  
Sella Turcica ◽  
Langerhans Cell ◽  
Central Diabetes Insipidus ◽  
Bone Lesions ◽  
Warning Sign ◽  
Low Grade ◽  
First Case

Langerhans cell histiocytosis (LCH) is a rare disease of the monocyte-macrophage system. Although it is known that bone involvement is seen very frequently in cases with LCH, our case is the first case with a lytic-destructive lesion in the bone structure forming sella turcica. A 4-year-old, 5-month-old male patient who applied to our outpatient clinic was diagnosed with Langerhans cell histiocytosis in further examination after the diagnosis of central diabetes insipidus (CDI) was made. On cranial magnetic resonance imaging (MRI), widespread lytic-destructive bone lesions were observed in the bone structure forming the sella (sphenoid bone), sellar destruction not previously described in the literature. Sellar erosion has not been reported before in cases diagnosed with LCH in the literature. The presence of low-grade fever in a patient presenting with isolated CDI is a warning sign for the diagnosis of LCH.

Adult-onset Neurological Langerhans Cell histiocytosis and Response to Treatment

2021 ◽  
Vol 8 (1) ◽  
Author(s):  
Al-Hader R ◽  
◽  
Suneja A ◽  
Memon AB ◽  
Mukherje A ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Brain Magnetic Resonance Imaging ◽  
Langerhans Cell ◽  
Response To Therapy ◽  
Response To Treatment ◽  
Adult Onset ◽  
Multisystem Disease ◽  
Clonal Proliferation ◽  
Magnetic Resonance Imaging Mri ◽  
Mass Lesions

Introduction: Langerhans Cell Histiocytosis (LCH) is a rare form of cancer that mostly affects children and rarely adults. LCH involves an abnormal clonal proliferation of Langerhans cells in the bone marrow. These cells are capable of migrating from the skin to lymph nodes. Therefore, it is characterized as a multisystem disease. Neurological manifestations are not common, and often patients’ present with endocrine dysfunction with neuroimaging findings of hypothalamic and pituitary masses can mimic pituitary adenoma. Here, we discuss two instances of unusual adult-onset, primary neurological LCH in patients with a positive response to therapy-these two patients presented with mass lesion and neurodegenerative form of LCH, respectively. LCH can manifest features of mass lesions or neurodegeneration on brain Magnetic Resonance Imaging (MRI). Since it is rare in adults, it is crucial to identify this condition as timely treatment can have a better prognosis.

scholarly journals Ureteral iliac artery fistula in idiopathic retroperitoneal fibrosis: A case report

2020 ◽  
Vol 92 (2) ◽  
Author(s):  
Eugenio Di Grazia ◽  
Tiziana La Malfa ◽  
Gherardo Gasso
Keyword(s):  
Iliac Artery ◽  
Retroperitoneal Fibrosis ◽  
Common Iliac Artery ◽  
Unknown Etiology ◽  
Gross Hematuria ◽  
Idiopathic Retroperitoneal Fibrosis ◽  
First Case ◽  
Life Threatening ◽  
Uncommon Condition ◽  
The Right

Ureter-arterial fistula (UAF) is an uncommon condition. The presentation is usually a life-threatening intermittent massive gross hematuria and the diagnosis is still a challenge for urologist. Idiopathic Retroperitoneal fibrosis (IRF) is a condition of unknown etiology characterized by a highly fibrotic retroperitoneal mass that frequently causes ureteral obstruction. To our knowledge we report the first case describing the UAF in a patient suffering from IRF. We hypothesize that inflammation and fibrosis resulted in fixation of the ureter to the adjacent artery causing a fistulous path. UAF was managed by deploying a 10 x 59 mm endo-graft at the intersection of common iliac artery bifurcation with the right ureter. Post treatment course was uneventful.

scholarly journals Langerhans cell histiocytosis of the rib presenting with pathological fracture: a case report

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Tao Zuo ◽  
Ping Jiang ◽  
Junjie Yu ◽  
Ke Zhao ◽  
Yong Liu ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Pathological Fracture ◽  
Soft Tissues ◽  
Langerhans Cell ◽  
Rib Fracture ◽  
Unknown Etiology ◽  
Case Presentation ◽  
Left Chest ◽  
Rib Tumor

Abstract Introduction Langerhans cell histiocytosis (LCH) is a rare neoplastic hyperplasia with an unknown etiology. It is clinically rare for patients with solitary rib lesion and pathological fracture; moreover, its diagnosis and treatment are quite difficult. The purpose of this study is to present a case for the pathogenesis, clinical features, imaging, and treatment of this disease. Case presentation A 52-year-old female patient complained of left chest pain for one week. CT showed a fracture in the left 5th rib. The rib tumor was then resected and the surrounding muscles and soft tissues were accordingly resected. The patient was diagnosed with pathological rib fracture, and the patient was pathologically diagnosed with LCH. After surgery, no local recurrence or distant metastasis was reported during the two-year follow-up. Conclusions LCH should be treated by observation, chemotherapy, radiotherapy, or surgery, or using a combination of several methods. Moreover, primary tumor should be considered when rib fracture without trauma and tumor metastasis.

scholarly journals Langerhans-Cell Histiocytoses - Epidemiology, Classification, Clinical Features, Diagnosis, Complications, Treatment and Prognosis

2016 ◽  
Vol 9 (1) ◽  
pp. 3-16 ◽  
Author(s):  
Vera E. Papochieva ◽  
Dimitrinka S. Miteva ◽  
Penka I. Perenovska ◽  
Guergana Petrova
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Eosinophilic Granuloma ◽  
Langerhans Cell ◽  
Unknown Etiology ◽  
Bone Lesions ◽  
Multisystem Disease ◽  
The Past ◽  
System Disease ◽  
Young Smokers ◽  
Underlying Pathology

Summary Histiocytoses comprise a group of diverse diseases of unknown etiology with various clinical presentation and evolution. The underlying pathology is characterised by accumulation and infiltration of variable numbers of cells of the monocyte-macrophage line in the affected tissues and organs. Histiocytoses are divided into three major classes: Langerhans cell histiocytosis (LCH), non- Langerhans cell histiocytosis, and malignant histiocytic disorders. The term LCH (also known in the past as histiocytosis X) encompasses the following rare diseases: Eosinophilic Granuloma, Hand-Schuller-Christian disease, Letterer-Siwe disease, Hashimoto-Pritzker disease, in which accumulation of pathologic Langerhans cells (LCs) leads to tissue damage. LCs usually reside in the skin and ensure protection against infections by destroying foreign substances. LC accumulation is caused by antigen stimulation and inadequate immune response. Thus, clinical LCH manifestations range from isolated disease with mono- or multifocal bone lesions to disseminated multisystem disease. LCH is a rare disease, affecting mainly children and young smokers, aged 20-50 years. Lung involvement in LCH usually presents as a mono-system disease and is characterized by Langerhans cell granulomas (LCG) infiltrating and impairing the distal bronchioles. The definite diagnosis is based on lung biopsy of CAT selected LCG areas. So far, there is no an effective treatment, but the better understanding of the mechanisms involved in the pathogenesis of the disease would help in the development of effective therapeutic strategies in the future.

A case of fixation amnesia in Langerhans cell histiocytosis involving the central nervous system

2020 ◽  
Vol 12 (6) ◽  
pp. 117-123
Author(s):  
L. V. Lukina ◽  
V. A. Mikhailov ◽  
N. I. Ananyeva ◽  
G. E. Mazo ◽  
L. I. Sitnik ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Langerhans Cell Histiocytosis ◽  
Correct Diagnosis ◽  
Brain Lesion ◽  
Clinical Findings ◽  
Langerhans Cell ◽  
Unknown Etiology ◽  
Bone Lesions ◽  
The Central Nervous System

Langerhans cell histiocytosis (LCH) is a rare disease with hitherto unknown etiology and pathogenesis. It is extremely rare for clinicians to encounter histiocytic lesions of the central nervous system (CNS); the proportion of cases of which is only 1–4% of all polysystemic and multifocal bone lesions. The paper describes a clinical case of fixation amnesia in a female patient with focal brain lesions in LCH. It depicts the most characteristic clinical features and presents an algorithm for the diagnosis of histiocytic brain lesion. The results of the experimental psychological examination of the patient are considered in detail and the clinical presentations of fixation amnesia are described. There are neuroimaging data showing the lesions in the hypothalamic-pituitary region and temporal bone, which involve the auditory structures. The clinical findings have led to the conclusion that both the clinical and neuroimaging patterns of histiocytic lesions in the CNS are non-specific, which complicates the diagnostic search in LCH. For correct diagnosis and timely treatment, it is necessary to perform a biopsy of the pathological focus, followed by histological and immunohistochemical examination of the material.

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