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

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.

2010 ◽  
Vol 46 (2) ◽  
pp. 138-142 ◽  
Author(s):  
Marjorie E. Milne ◽  
Christina McCowan ◽  
Ben P. Landon

Spontaneous pneumothorax is rarely reported in the cat. This case report describes the use of computed tomography (CT) to diagnose pulmonary bullae in an adult cat with recurrent spontaneous pneumothorax. A large bulla in the right middle lung lobe and several blebs in other lobes were identified by CT. Partial lobectomy of the right middle and right and left cranial lung lobes was successfully performed to remove the affected portions of lung. Histopathological examination suggested bronchopulmonary dysplasia (BPD) as the underlying cause for development of the pulmonary bulla. This is the first case report in the veterinary literature describing the use of CT to identify pulmonary bullae in the cat with BPD as a possible underlying cause.


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. 1-6
Author(s):  
Kyriakos Papavasiliou ◽  
Antonia Bintoudi ◽  
Apostolos Vlahodimos ◽  
Eleftherios Tsiridis ◽  
Prodromos Hytiroglou ◽  
...  

Only three cases of patients suffering from a localized chronic form of Langerhans cell histiocytosis (LCH) successfully treated with radiofrequency ablation (RFA) have been published so far. This is the first case report of a patient with a localized chronic form of LCH of the femur, which was successfully treated with percutaneous image-guided RFA, and who was evaluated pre-RFA and followed up post-RFA for a period of 48 months, in order to validate the safety and efficacy of this method and to obtain imaging studies depicting the actual in situ changes taking place post-RFA. RFA was proved to be a safe and efficient method when treating patients with a localized chronic form of LCH.


2017 ◽  
Vol 8 (1) ◽  
pp. 67-72 ◽  
Author(s):  
Mikael Hofsli ◽  
Nico Gampenrieder ◽  
Steffen Heegaard

Background: To present a rare case of a 2-year-old girl with neurofibromatosis type 1 (NF1) who presented with ptosis of the right upper eyelid along with a tumor in the eyelid. Methods: A magnetic resonance imaging scan of the orbit revealed a solid tumor located extraconally at the site of the right lacrimal gland. A transcranial orbitotomy was performed. Results: Histopathological examination demonstrated expanded nerve branches/fascicles cut in various planes in between normal lacrimal gland acini. These findings were consistent with a plexiform neurofibroma presumably deriving from the lacrimal nerve and/or a supraorbital nerve branch. Conclusion: This is the first case of a plexiform neurofibroma involving the lacrimal gland ever described and the tumor shows similarities with neurofibroma in other salivary glands with a high recurrence rate. Plexiform neurofibromas are frequently seen in patients with NF1 and rarely undergo malignant transformation.


2021 ◽  
Vol 3 (3) ◽  
pp. 24-26
Author(s):  
Y. Labani ◽  
O. Oulghoul ◽  
O. Benhoummad ◽  
Y. Rochdi ◽  
A. Raji

Langerhans cell histiocytosis is a rare hematologic disease due to multiplication and accumulation of Langerhans cells, which are immature dendritic cells. The clinical presentation is highly variable and can range from isolated, self-healing skin or bone lesions to life-threatening multisystem disease. We report 2 cases confirmed by Histological and immunohistochemical evaluation of temporal bone biopsies. The first case is a 2-year-old child with chronic bilateral otorrhea and polyuria-polydipsia syndrome. Physical examination showed bilateral retroauricular redness and induration with filling all of the right and left external ear canal. Computed tomography showed osseous lysis of the temporal bone bilaterally. Magnetic resonance imaging of the brain showed the presence of pituitary infiltration. The second case is 2-year-old girl with chronic left otorrhea. Clinical examination objectified the presence of redness and induration in the left mastoid with the presence of an inflammatory polyp filling the entire left external auditory canal. Computed tomography revealed a left temporal bone lysis. Both patients were treated with chemotherapy.


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.


2016 ◽  
Vol 22 (7) ◽  
Author(s):  
Maraya de Jesus Semblano Bittencourt ◽  
Carolina Moraes Dias ◽  
Thaiane Lima Lage ◽  
Amanda Magno Parijós ◽  
Letícia Brito Mesquita ◽  
...  

2020 ◽  
Vol 2020 ◽  
pp. 1-5
Author(s):  
Stephanie M. Tillit ◽  
Siva S. R. Iyer ◽  
Eric J. Grieser ◽  
John T. LiVecchi

Primary cutaneous mucinous carcinoma (PCMC) is a rare, low-grade malignant neoplasm of the sweat gland, whose history has been controversial regarding eccrine versus apocrine origin. This case report describes a 53-year-old male who presented to the University of Florida, Gainesville, ophthalmology clinic and was referred to the oculoplastics service with a painless, subcentimeter mass on the lateral right upper eyelid including the canthus, consistent with recurrent primary cutaneous mucinous carcinoma of the eyelid. Four years prior, the patient had undergone excisional biopsy of the lesion in a clinic, which revealed residual tumor, but the patient deferred further surgery at the time. The patient underwent surgical excision of the mass with reconstruction without operative complications and with negative surgical margins. PCMC is difficult to diagnose clinically due to its rarity and requires a histopathological examination for confirmation of the diagnosis. This report presents the first case in the literature of primary cutaneous mucinous carcinoma in a patient with human immunodeficiency virus (HIV). With this case report, we aim to raise awareness of primary cutaneous mucinous carcinoma as a potential part of the differential diagnosis for malignant eyelid lesions, including those present in patients with HIV.


2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Yun Hsia ◽  
Huang-Chun Lien ◽  
I-Jong Wang ◽  
Shu-Lang Liao ◽  
Yi-Hsuan Wei

Abstract Background Epibulbar complex choristoma, a rare congenital epibulbar tumor, has many diverse forms. Reviewing the literature, it can present clinically as either a circumferential or isolated epibulbar mass, limbal tumor, lateral canthal mass, aggregate of ectopic cilia in the upper eyelid, eyelid mass mimicking chalazion, or lacrimal caruncle mass. The management depends on the extent of involvement, the risk of amblyopia, and cosmetic concerns. Here, we report an atypical presentation of epibulbar complex choristoma with simultaneous eyelid involvement. Case presentation A 1-month-old full-term boy was brought to our clinic with congenital epibulbar mass of the right eye with simultaneous eyelid involvement. Dilated fundus examination was unremarkable. Survey for linear nevus sebaceous Jadassohn was negative. Due to concerns of possible amblyopia and cosmetics, lamellar keratectomy, sclerotomy, and conjunctivoplasty were performed to remove the epibulbar lesion. The eyelid defect was reconstructed with 6–0 Vicryl sutures. Histopathological examination reported complex choristoma. Upon three-year follow-up, low astigmatism and favorable cosmetics results were achieved. Conclusions Congenital complex choristoma can present clinically as an epibulbar mass with eyelid involvement. The management depends on the extent of involvement, the risk of amblyopia, and cosmetic concerns. The method of eyelid reconstruction should be tailored according to the residual eyelid defect.


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