Langerhans Cell Histiocytosis of the Clavicle in an Adult: A Case Report and Review of the Literature

Langerhans cell histiocytosis (LCH) usually occurs in children under the age of 10 years with a predilection for the skull, spine, rib and humerus. Solitary LCH occurring in an adult clavicle is uncommon with limited reports to date. The lesion in our patient was curetted with the intent to make a diagnosis, which subsequently lead to the remission of the symptom and the disease. At the final follow-up after 1 year, no local recurrence or metastasis is observed.Key Words: Langerhans cell histiocytosis, Clavicle, Adult     

朗格汉斯细胞组织细胞增生症3例临床分析

目的探讨朗格汉斯细胞组织增生症的临床特点及治疗方法。方法回顾分析3例朗格汉斯细胞组织细胞增生症并复习相关文献。结果 2例患者以头部肿物为首发症状,手术切除病灶后经过化疗及免疫治疗存活至今,另1例患者经过颈椎穿刺活检确诊,未行化疗,死于并发症。结论朗格汉斯细胞组织细胞增生症是一组原因不明的罕见疾病,其病理基础是单核巨噬细胞系统中朗格汉斯细胞增殖,由于病变年龄、细胞增生程度、累及组织和器官的不同,其症状表现不一样,预后也不一样。     

宫颈朗格汉斯细胞组织细胞增生症的临床病理分析

目的 探讨女性生殖道朗格汉斯细胞组织细胞增生症的临床病理学特征,以加深此病在宫颈等少见部位的认识.方法 对1例宫颈LCH进行HE及免疫组化染色,观察其临床病理特征,并复习相关文献.结果 患者女性,31岁,确诊为宫颈LCH,镜下朗格汉斯细胞弥漫增生,肿瘤细胞中等大小,细胞质淡粉染,核呈卵圆形,可见核沟,核仁不明显;背景可...     

朗格汉斯组织细胞增生症放疗的疗效观察

目的探讨放疗对朗格汉斯组织细胞增生症的疗效及不良反应。方法回顾性分析21例经病理检查确诊的朗格汉斯组织细胞增生症患者的临床资料。结果非成人组14例(27个病变部位),中位单次放疗剂量180 cGy,中位总放疗剂量1 600 cGy;局部控制率96.3%,完全缓解率为74.1%。成人组7例(9个病灶),中位单次放疗剂量200cGy,中位总放疗剂量2 000 cGy;局部控制率100.0%,完全缓解率66.7%。放疗剂量≤2 000 cGy者与>2 000 cGy者局部控制率及完全缓解率比较,无显著性差异(P>0.05)。中位随访时间4年。随访期间无严重放疗并发症发生;无野内复发。结论放疗是朗格汉斯组织细胞增生症的有效治疗手段,其局控率高,野内复发及严重放疗并发症少见。     

New Insights Into the Molecular Pathogenesis of Langerhans Cell Histiocytosis

Langerhans cell histiocytosis (LCH) is a rare proliferative disorder characterized by an accumulation of cells sharing the major phenotypic features of cutaneous Langerhans cells. Given its variable clinical evolution, ranging from self‐limiting lesions to multisystemic forms with a poor prognosis, in the last decades it has been debated whether LCH might not have a neoplastic rather than an inflammatory nature. However, although the fundamental events underlying the pathogenesis of LCH are still elusive, recent advances have strikingly improved our understanding of the disease. In particular, the identification of multiple interplays between LCH cells and their tumor microenvironment, along with the recognition of the lesional cytokine storm as a key determinant of LCH progression, has substantiated new opportunities for devising targeted therapeutic approaches. Strikingly, the detection of the rapidly accelerated fibrosarcoma isoform B^V600E gain‐of‐function mutation as a genetic alteration recurring in more than 50% of patients has fueled the paradoxical picture of LCH as a tumor of the antigen‐presenting cells that can evade rejection by the immune system. Thus, new evidence regarding the ontogeny of LCH cells, as well as a better understanding of the putative immune system frustrating strategy in LCH, may help to define the precise pathogenesis.     

Efficacy of BRAF‐Inhibitor Therapy in BRAFV600E‐Mutated Adult Langerhans Cell Histiocytosis

Langerhans cell histiocytosis (LCH) is a rare histiocytic neoplasm. To date, there is a lack of U.S. Food and Drug Administration–approved treatments in adult LCH to establish optimal first‐line therapy. We conducted a retrospective, single‐center case series evaluating the use of BRAF inhibitors in adult patients with BRAF ^V600E‐ LCH proven by biopsy. Our case series is the first to report the use of BRAF inhibitors as first‐line therapy in adults with LCH. We also report the efficacy with single‐agent dabrafenib in adult LCH. All but one of our patients had favorable response to targeted therapy.     

成人朗格汉斯组织细胞增生症放射治疗的疗效合并文献复习

目的 分析成人朗格汉斯细胞组织细胞增生症(LCH)的放射治疗(RT)效果合并文献复习.方法 回顾性分析经病理检查确诊的1例成人LCH患者的临床资料及放射治疗效果.结果 病理检查提示:“C7椎体”骨与软组织中见淋巴细胞、浆细胞、嗜酸性粒细胞、巨噬细胞及多核巨细胞浸润,血管丰富,充血出血,伴纤维组织增生.免疫组化标记显示:病变中含有朗格汉斯细胞、组织细胞、T和B淋巴细胞及浆细胞.骨组织脱钙后见类似病变及细胞免疫表型.确诊后给予前后路联合颈7椎体切除椎管减压+肿瘤切除病检+前路钢板、钛网植骨固定+后路颈7椎体附件切除椎弓根钉内固定术和放射治疗.术后予瘤床区(P-CTV)放射治疗,瘤床区(P-CTV)放疗剂量:200 cGy/f,1次/天,5次/周,共予(P-CTV)放疗剂量:2 200 cGy/11f.患者症状达到基本缓解,照射部位无放射治疗的副作用出现.结论 放射治疗可以有效治疗成人LCH.即使是低RT剂量疗效也很好,而且副作用少.     

Adult Epiglottic Abscess: A Case Report

We report a case of Amyloidosis of larynx in a 60-year-old adult who reported to us with hoarseness of voice of 3 years long duration and no other symptoms. Patient was treated successfully with endoscopic curettage of the mass with no inadvertent ill effects.     

Highlights of the Management of Adult Histiocytic Disorders: Langerhans Cell Histiocytosis,Erdheim-Chester Disease,Rosai-Dorfman Disease,and Hemophagocytic Lymphohistiocytosis

Histiocytic disorders are an exceptionally rare group of diseases with diverse manifestations and a paucity of approved treatments, thereby leading to various challenges in their diagnosis and management. With the discovery of novel molecular targets and the incorporation of targeted agents in the management of various adult histiocytic disorders, their management has become increasingly complex. In an attempt to improve the understanding of the clinical features and management of common adult histiocytic disorders (Langerhans cell histiocytosis, Erdheim-Chester disease, Rosai-Dorfman disease, and hemophagocytic lymphohistiocytosis), we created this document based on existing literature and expert opinion.     

Orbito-Ethmoidal Rhabdomyosarcoma in an Adult Patient: A Case Report and Review of the Literature

We report a patient who presented to the ENT service complaining of nasal obstruction, exophthalmos, edema and ipsilateral facial congestion. Imaging studies revealed an aggressive noncalcified solid mass centered in the left nasoethmoidal region and heterogeneous avid enhancement following contrast media injection. Subsequently, a biopsy confirmed the presence of solid alveolar rhabdomyosarcoma. The patient was treated with chemoradiation therapy for 7 weeks. Due to the advanced stage of the disease, the patient was enrolled in a palliative care and pain control program.Key words: Computed tomography, Magnetic resonance imaging, Ethmoidal region, Rhabdomyosarcoma, Radiochemotherapy     

Haemorrhagic Pseudocyst of the Adrenal in an Adult (A Case Report)

An adult female patient presenting with a cystic abdominal mass and intermittent fever, was found at surgery to have a large cyst of the left adrenal gland. The imaging findings and pathology of this uncommon entity are discussed.     

Eosinophilic Granuloma of Mandible in an Adult (A Case Report)

A case of mono-ostotic eosinophilic granuloma localised to the mandible of an adult patient is described. The role of low dose radiation therapy as a curative modality is discussed.     

A Rare Case of an Osteoid Osteoma of the Rib Treated under Computed Tomography Guidance: A Case Report and Review of the Literature

Osteoid osteoma (OO) usually occurs in the extremities of young adults. The tumor can arise in any part of the skeletal tissue; however, it is rarely found in the rib, with limited reports to date. In this report, we present a rare case of OO arising in the rib, which was successfully treated under computed tomography guidance with minimal invasiveness. At the final follow-up after 4 years, no local recurrence was observed.Key Words: Osteoid osteoma, Rib, Computed tomography guidance