Langerhans cell histiocytosis in an 18-month-old child presenting as periorbital cellulitis

Langerhans cell histiocytosis (LCH) is a rare multi-system disease. It presents infrequently as a childhood orbital tumor, and can mimic more common inflammatory orbital disease processes. We report the clinical, histopathological, and electron microscopic findings of orbital LCH in an 18-month-old child, along with a review of the recent literature regarding molecular pathogenetic analysis of LCH. The child presented with a two-week history of progressive left periorbital edema and redness. He was initially diagnosed and treated empirically for bacterial periorbital cellulitis, but subsequently underwent ophthalmological consultation after he failed to improve. Histopathological examination of an orbital biopsy specimen revealed numerous Langerhans-type cells, which stain positive for CD1A and CD207 (langerin). Electron microscopic examination demonstrated characteristic Birbeck granules within the Langerhans-type cells. Three year follow-up did not demonstrate recurrence or disease progression.     

Langerhans Cell Histiocytosis of the Orbit: Five Clinicopathologic Cases and Review of the Literature

Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells intermixed with inflammatory cells, in particular eosinophils, that may manifest as unisystem (unifocal or multifocal) or multisystem disease. Orbital involvement typically manifests as a solitary lesion that carries a favorable prognosis. We describe the clinical and histologic spectrum of LCH of the orbit in our five cases. One patient exhibited multifocal unisystem disease; the other four had a localized process. Typical histologic features included numerous histiocytes with varying degrees of giant cell formation and scattered eosinophilic granulocytes. The presence of Langerhans cells was confirmed by CD1a and S100 immunohistochemistry. Transmission electron microscopy demonstrated characteristic intracytoplasmic Birbeck granules. We review the different ophthalmic manifestations of LCH and treatment strategies. As LCH may solely involve the orbit, treatment is based on the degree of organ involvement. LCH should included in the differential diagnosis in tumors of the ocular adnexae, especially in young children.     

Langerhans cell histiocytosis of the caruncle

A 37-year-old man presented with the complaint of a small non-tender mass growing steadily on the left caruncle for 3 months. This was a soft, reddish-yellow tumour, occupying the whole left caruncular area without impinging on the conjunctiva. Histopathological evaluation of the excisional biopsy specimen showed that the tumour was largely composed of Langerhans cells mixed with eosinophils and mature lymphocytes. There were strong CD1a and S-100 positivity on immunohistochemical studies. Langerhans cell histiocytosis is an exceedingly rare tumour of the caruncle in the adult and this lesion cannot be distinguished from other amelanotic lesions on clinical grounds alone. Simple excision of the lesion provided a 4-year disease-free period.     

眼部组织细胞增生症诊疗进展

眼部组织细胞增生症是一组罕见的成人及儿童均可发病的异质性疾病,其临床及影像学表现无特异性,临床上该类疾病的诊疗存在巨大困难。随着对组织细胞增生症分子机制的研究,BRAF^V600E突变基因的发现使人们对组织细胞增生症有了进一步的认识,也使多数患者能够得到针对性的治疗。诊断的金标准发展为组织病理活检联合突变基因的检测,治疗方式不再是单一的手术切除治疗,靶向治疗对MAPK-ERK通路中突变基因阳性的患者也有极好的疗效。但是眼部组织细胞增生症类型众多且常常涉及多个系统,临床表现多样,对其早期、正确地诊断仍面临挑战。因此本文就近年眼部组织细胞增生症的诊疗进展进行文献复习,总结眼部组织细胞增生症的临床病理特征并对最新的治疗进展进行综述,旨在提高临床医生对该疾病的诊疗水平。     

儿童眼眶朗格汉斯细胞组织细胞增生症的临床特征及治疗分析

目的 探讨儿童眼眶朗格汉斯细胞组织细胞增生症(LCH)的临床特征及治疗方案.设计回顾性病例系列.研究对象2013年4月-2019年7月空军军医大学第二附属医院经病理确诊的眼眶LCH患者7例(8眼).方法 整理、分析患者一般情况、临床表现、影像学资料、病理特征、治疗方案及随访结果.主要指标临床表现,病理特征、治疗方案和随...     

Langerhans cell histiocytosis of the optic chiasm

Langerhans cell histiocytosis (LCH) is a rare disease with clonal proliferation of dendritic histiocytes forming a pseudotumoral growth. LCH occurs most frequently in infancy or early childhood. It can present either as a localized self-limiting form or as a diffuse form with multisystem involvement. Herein an unusual case of LCH involving the optic chiasm is presented. To the authors' knowledge, this is the first reported case of LCH presenting with visual disturbance. The clinical, radiological and histopathological findings are discussed.     

绿色荧光蛋白标记的恶性组织细胞增生症瘤细胞眼前房移植模型

目的建立绿色荧光蛋白(GFP)标记的恶性组织细胞增生症瘤细胞cy15在眼前房生长及浸润的模型,探讨cy15细胞在前房生长的规律及GFP标记活细胞的优势。方法将带有GFP的逆转录病毒MP71-GFP-PRE转入cy15瘤细胞,获得稳定表达GFP的cy15GFP细胞系。20只BALB/C小鼠(20只眼)每只眼前房注入2μl细胞密度为2×106个/ml的cy15GFP瘤细胞悬液,术后分别在裂隙灯和荧光显微镜下观察瘤细胞在前房的生长情况,连续观察30d,分别在瘤细胞植入前房后第15d、20d、30d时处死小鼠做眼球HE病理切片。结果成功地建立了稳定表达GFP的cy15GFP细胞系。接种的20只BALB/c鼠眼前房均可见肿瘤细胞生长,借助于荧光显微镜能动态地观察到肿瘤细胞在活体小鼠眼前房的生长浸润过程。结论建立的GFP标记的肿瘤细胞眼前房接种模型为研究眼内肿瘤细胞生长与浸润的规律以及抗肿瘤药物的筛选提供了一种新的手段。     

Masquerade of eyelid tumours

Space-occupying processes in the eyelids can either be due to lesions which are specific for the eyelids, such as a chalazion or a meibomian carcinoma, or to non-specific lesions of the skin or ocular adnexa. An incorrect interpretation on clinical or histological grounds is often made; in other words, some eyelid processes masquerade as others. The correct diagnosis should be made on the basis of case history, localisation, the appearance of the tumour and the histological findings, so that adequate treatment can be given. One must be aware of the danger of mistaking one space-occupying process for another. In the Leiden Ophthalmological Clinic a study was made of the incidence of eyelid tumours in the period 1981–1987, with special attention to the most important masquerade phenomena observed. In a group of 956 eyelid tumours histological examination was necessary in 126 cases. Basal-cell carcinoma was the most frequent diagnosis, but other less common tumours such as a trichilemmoma, an eosinophilic granuloma, a haemangio-endothelioma, a metastasis from a mammary carcinoma and a granuloma annulare were also diagnosed.     

Histiocytic lesions of the orbit: A study of 9 cases

Purpose

To describe the clinical presentation, treatment, and outcome of patients with histiocytic lesions of the orbit.

Cherubism: Report of Three Cases and Literature Review

Purpose: To report 3 cases of cherubism, one of whom underwent surgery for orbital manifestations, and to provide a literature review.

Case reports: Our patients were normal at birth and developed painless enlarging of the cheeks and jaws when they were 4–5 years old. Ophthalmologic examinations showed mild proptosis, superior globe displacement and inferior scleral show in all cases. Cases 2 and 3 had lower lid skin discoloration. Computed tomography (CT) scans demonstrated bilateral multicystic lesions in the maxilla and mandible with cortical thinning in all cases. In Case 3, left eye hyperglobus and anisometropic amblyopia was seen. In this case, the CT scan showed a round, well-defined and homogeneous mass, involving the anterior and superior walls of the maxillary sinus on the left side, extending into inferior orbit. Debulking of the mass was performed at the surgery. The pathologic findings were compatible with the diagnosis of giant cell reparative granuloma. He returned 1 year after surgery with recurrence of the mass.

Discussion: A few cases were reported in the literature with histopathologically proven orbital cherubism. To our knowledge, lower lid skin discoloration in Cases 2 and 3 and anisometropic amblyopia in case 3 were not described elsewhere in cherubism cases. We recommend that all cases with cherubism must be examined by an ophthalmologist to diagnose and treat possible orbital manifestations.     

A Merkel cell tumor of the eyelid

The Merkel cell is a distinctive nondendritic, nonkeratinocytic, epithelial clear cell believed to migrate from the neural crest to the epidermis and dermis, which is usually located in or near the basal layer of the epidermis and associated with nerve terminations. Merkel first described these cells in 1875 as "Tastzellen" occurring in the snout of a mole. They are believed to function as slowly adapting mechanoreceptors that mediate the sense of touch. Tumors arising from Merkel cells have been reported to occur on the head and neck area, the trunk, arms, and legs, and resemble a primary cutaneous lymphoma or cutaneous metastasis of a lymphoma or a carcinoma. Electron microscopy, to locate the characteristic membrane-bound, dense core neurosecretory granules, is needed for accurate diagnosis. These tumors must be treated aggressively to minimize the chance of local recurrence and nodal or visceral metastases. The authors present a case of Merkel cell tumor occurring on the eyelid. The clinical history, light and electron microscopic findings are shown.     

Langerhans cell histiocytosis of the orbit

Langerhans cell histiocytosis (LCH) accounts for less than 1% of all orbital tumours. Though rare, orbital involvement in LCH is not uncommon. Most reports so far have been in Western literature. We report here a case of LCH of orbit with intracranial extension. To the best of our knowledge, this is the first case of single system multifocal LCH reported from India.     

Intraosseous capillary hemangioma of the frontal bone

A 31-year-old woman with a history of slowly progressive proptosis associated with blepharoptosis was found radiographically to have a lytic lesion of the frontal bone. Complete surgical excision of the bony mass was performed. Histopathologically the tumor was a capillary hemangioma. Follow-up information two years later showed that the patient was alive and well, without clinical evidence of recurrence. Plain x-ray films appear to be more reliable for diagnosis than computed tomography exhibiting the characteristic sunburst pattern of intraosseous hemangiomas.     

Annular elastolytic giant cell granuloma of conjunctiva: A case report

Annular elastolytic giant cell granuloma is a condition characterized histologically by damaged elastic fibers associated with preponderance of giant cells along with absence of necrobiosis, lipid, mucin, and pallisading granuloma. It usually occurs on sun-damaged skin and hence the previous name actinic granuloma. A similar process occurs on the conjunctiva. Over the past three decades only four cases of conjunctival actinic granuloma have been documented. All the previous patients were females with lesions in nasal or temporal bulbar conjunctiva varying 2-3 mm in size. We report a male patient aged 70 years presenting with a 14 mm × 7 mm fleshy mass on right lower bulbar conjunctiva. Clinical differential diagnoses were lymphoma, squamous cell carcinoma in situ and amyloidosis. Surgical excision followed by histopathology confirmed it to be a case of actinic granuloma. This is the first case of isolated conjunctival actinic granuloma of such a large size reported from India.     

Primary bone tumors of the orbit

Primary tumors of orbital bone constitute 0.6% to 2% of all orbital tumors. Our experience over a 24-year period in the Orbital Clinic at the University of British Columbia yielded 62 (1.9%) cases from a total of 3,340 orbital tumors. Although a heterogeneous group, primary orbital bone tumors may be classified on a clinicopathologic basis into benign fibro-osseous or cartilaginous, reactive, neoplastic and vascular disorders. Presentation is usually a gradual mass effect, with infiltration and acute hemorrhage being features of malignant and reactive lesions respectively. The two most commonly encountered entities were fibrous dysplasia and osteoma, accounting for 22 cases. Although both these conditions rarely present a diagnostic challenge, nonspecific histologic and radiologic appearances can result in poor characterization of several of the rarer lesions. Hence, close cooperation between clinician, radiologist and pathologist is essential for accurate diagnosis.