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 ◽  
...  

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.

2015 ◽  
Vol 2015 ◽  
pp. 1-4
Author(s):  
E. Roos ◽  
M. Maas ◽  
S. J. M. Breugem ◽  
G. R. Schaap ◽  
J. A. M. Bramer

Nonbacterial osteitis is a rare autoinflammatory disease. Often it is mistaken for a tumor or osteomyelitis. We present a case of a twelve-year-old girl referred to our hospital because of a lesion of the right clavicle. The differential diagnoses were sarcoma, osteitis, and Langerhans cell histiocytosis. After biopsy the diagnosis nonbacterial osteitis (NBO) was established. Treatment of choice is a nonsteroidal anti-inflammatory drug. This case report gives a complete follow-up of the disease, showing the pitfalls of the diagnosis.


2016 ◽  
Vol 8 (1) ◽  
pp. 38-40
Author(s):  
Asif Salimov ◽  
Ahmet E Suslu ◽  
Serdar Ozer ◽  
Taner Yilmaz ◽  
Hatice IY Bajin

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.


2020 ◽  
Vol 2020 ◽  
pp. 1-3
Author(s):  
Ibtissem Ben Nacef ◽  
Sabrine Mekni ◽  
Chedi Mhedhebi ◽  
Ines Riahi ◽  
Imen Rojbi ◽  
...  

Langerhans cell histiocytosis (LCH) is a rare sporadic proliferative disorder of Langerhans cells. LCH rarely involves the thyroid gland. We report herein a case of a disseminated chronic form of LCH with a diagnosis established by histological examination of the thyroid gland. It is about a 37-year-old female who underwent total thyroidectomy for a thyroid nodule of the right lobe. Histological study showed a granulomatous thyroiditis, and the immunohistochemistry study revealed a strong positivity of histiocytes for the CD1 antigen and for the S100 protein. The incidence of LCH involving the thyroid gland, either as an isolated lesion or as a part of multisystemic disease, is extremely rare.


Author(s):  
Aakash Mishra ◽  
Sandesh Gyawali ◽  
Sanjeev Kharel ◽  
Aman Mishra ◽  
Sandip Kuikel ◽  
...  

2013 ◽  
Vol 5 (2) ◽  
pp. 74-86
Author(s):  
Mirjana Paravina ◽  
Dragan Jovanović ◽  
Milenko Stanojević ◽  
Ljiljana Nikolić

Abstract Langerhans cell histiocytosis is a disease which results from accumulation or proliferation of a clonal population of cells with the phenotype of Langerhans cells arrested at an early stage of activation that are functionally deficient. The etiology and pathogenesis of the disorder are still unknown. There are ongoing investigations to determine whether it is a reactive or a neoplastic disease. The fact is that neoplastic and reactive processes may have many clinical and pathological similarities. Some emphasize the role of “cytokine storm” in Langerhans cells. Further studies are necessary in all areas, from the etiology and pathogenesis to diagnosis and therapy. Langerhans cell histiocytosis primarily affects bones, but less commonly it may involve other organ systems, or present as a multisystem disease. The clinical course is variable, from benign forms with spontaneous resolution, to chronic disseminated forms with fatal outcome. This is a report of a 29-year-old man with Langerhans cell histiocytosis with an onset at the age of 8, which later progressed to a multisystem disease. Apart from lesions on the skin and exposed mucous membranes, the patient also presented with: diabetes insipidus, granuloma of the right femur and slight bulbar protrusion of the right eye. The patient experienced spontaneous pneumothorax on two occasions. The diagnosis of Langerhans cell histiocytosis was histologically confirmed using electron microscopy by presence of Birbeck granules in the histiocytes. A favorable therapeutic response was obtained after systemic corticosteroid therapy.


2020 ◽  
Vol 8 ◽  
pp. 2050313X2091961
Author(s):  
Janelle Cyr ◽  
Annie Langley ◽  
Dina El Demellawy ◽  
Michele Ramien

In our case report, we discuss a 1-day-old boy presenting with blueberry muffin syndrome diagnosed with Langerhans cell histiocytosis. The diagnosis complicated by an initial difficult-to-interpret biopsy showing only a hint of perifollicular CD1a-positive cells; however, given our team’s strong clinical suspicion of Langerhans cell histiocytosis, a second biopsy of a more mature lesion was done and showed typical histopathology. This case introduces the possibility of perifollicular Langerhans cells early in this condition, demonstrates the importance of appropriate biopsy site selection, and highlights the importance of maintaining a high degree of suspicion when there is poor clinicopathologic correlation. Our case report contains a comprehensive table which reviews the systemic and cutaneous clinical features, as well as the laboratory, pathology, and imaging findings for the differential diagnoses of blueberry muffin baby.


2016 ◽  
Vol 7 (2) ◽  
pp. 158-160
Author(s):  
Nadira Musabbir ◽  
ASM Bazlul Karim ◽  
Kaniz Sultana ◽  
Syeda Afria Anwar

Langerhans’cell histiocytosis (LCH) (Histiocytosis X) is a rare disease of unknown cause characterized by oligoclonal proliferation of Langerhans cells. It occurs mostly in children and young adults and involves one or more body systems such as bone, hypothalamus, posterior pituitary gland, lymph nodes, liver or various soft tissues. The diagnosis is always made by a histological approach. A 3.5-year old male child with LCH involving multiple systems, including the skin, lungs, liver with clinical signs of diabetes insipidus. The patient was diagnosed following a skin biopsy that revealed infiltration of Langerhans cells. This case report would increase the awareness of pediatricians about the multisystem involvement in LCH.Northern International Medical College Journal Vol.7(2) Jan 2016: 158-160


2019 ◽  
Vol 10 ◽  
pp. 234
Author(s):  
Kacper Kostyra ◽  
Bogusław Kostkiewicz

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.


Cases Journal ◽  
2008 ◽  
Vol 1 (1) ◽  
Author(s):  
Khalid A Al-Anazi ◽  
Abdulrahman Alshehri ◽  
Hazza A Al-Zahrani ◽  
Fahad I Al-Mohareb ◽  
Irfan Maghfoor ◽  
...  

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