Radiotherapy for steroid-resistant laryngeal Rosai-Dorfman disease

Rosai-Dorfman disease is a rare lymphoproliferative disorder that can have nodal and extranodal manifestations. In the absence of established guidelines for the management of this condition, various therapeutic modalities are used, including radiotherapy. Radiation dosages and fractionation schedules have not been reported in all instances. We present a case in which glottic and subglottic Rosai-Dorfman lesions causing airway obstruction in a frail steroid-refractory patient were put into complete remission using radiotherapy. The lesions responded transiently to a course of prednisone, but responded completely to external-beam radiation, with minimal side effects to the patient.     

Rosai-Dorfman disease. Extranodal sinus histiocytosis in three co-existing sites. A case report

A 73-year-old woman presented with mild anterior uveitis, ipsilateral optic neuropathy, and ipsilateral skin nodules. A compressive mass at the level of the orbital apex and sphenoid wing was found on cranial magnetic resonance imaging. Biopsy of the skin nodules revealed histopathologic evidence of sinus histiocytosis with massive lymphadenopathy, or Rosai–Dorfman disease (RDD). Systemic investigations failed to show any massive lymphadenopathy, making this a case of extranodal RDD. This is a salient case in that it proposes three simultaneous and separate sites of involvement by extranodal RDD. It also exemplifies that RDD should be a suspect diagnosis even in the absence of lymphadenopathy.     

Rosai-Dorfman病一例及文献分析

目的探讨Rosai-Dorfman病（RDD）的临床表现、诊断、组织特征及治疗。方法研究RDD1例,结合文献回顾分析。结果RDD1例,病理见大量淋巴细胞、浆细胞及组织细胞,并有吞噬淋巴细胞现象,免疫组化S-100强阳性。本病临床表现多样,易误诊。结论RDD是良性的组织细胞增生性疾病,预后主要取决于患者的免疫功能状态、淋巴结受累数目及结外器官受累部位,诊断主要依靠病理及免疫组化,治疗上以综合治疗为主,治疗后应随访。     

Sinus histiocytosis with massive lymphadenopathy: A case of multiple dural involvement

An exeptional case of Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) arising from the meninges in a 60-year-old Japanese man is presented. Computerized tomographic scans and magnetic resonance images demonstrated well-circumscirbed tumorous lesions that were homogeneously enhanced with contrast medium. Systemic examination revealed no abnormalities except for a cervical lymphadenopathy and diabetes mellitus. Microscopic examination of the resected specimens showed proliferated histiocytosis and infiltration of plasma cells and lymphocytes. The histology was characterized by the presence of histiocytes demonstrating lymphophagocytosis and immunoreactivity for S-100 protein staining. Immunohistochemical studies and electron microscopy were useful in confirming the diagnosis. The clinical and histopathological features of this disease are discussed.     

A Rare Case of “Rosai–Dorfman Disease”

Sinus histiocytosis with massive lymphadenopathy (SHML) is a benign proliferating histiocytic disorder, predominantly of lymphnodes with extranodal involvement also seen. We present a case of 35 years old female with history of multiple swellings in neck since 1 month duration. On examination patient had painless bilateral cervical lymphadenopathy. No other ENT manifestations noted. Lymphnode biopsy revealed SHML. Abdominal scan and chest X-ray was done which was normal. This case report highlights the clinical, histological aspects of SHML, Rosai–Dorfman disease.     

骨骼Rosai-Dorfman病的诊疗进展

Rosai-Dorfman病，又称窦组织细胞增生伴巨大淋巴结病，是一种罕见的组织细胞病，通常表现为青少年的无痛性双侧颈部淋巴结肿大。Rosai-Dorfman病发病累及骨骼者不到10%，并且多达75%的骨骼Rosai-Dorfman病患者同时存在软组织病灶。颅骨、颌面骨和胫骨是骨骼Rosai-Dorfman病最常见的发病部位。该病临床表现缺乏特异性，诊断主要依靠常规病理学和免疫组织化学染色检查。临床表现主要是局部疼痛和肿胀。影像学上，通常表现为髓内的溶解性病变，有时伴有周围硬化。目前，Rosai-Dorfman病的病因尚不明确，可能涉及潜在的宿主免疫失调、IgG4相关疾病、多种自身免疫性疾病和基因突变等。目前伴有症状的骨骼Rosai-Dorfman病的治疗方案主要取决于具体病灶位置，主要包括手术刮除或切除，其他治疗方案包括激素治疗和化疗等。由于骨骼Rosai-Dorfman病的临床和影像学表现通常提示恶性病变可能，部分患者可能接受比较激进的治疗。全身PET/CT可以用于Rosai-Dorfman病的分期、随访和评估。     

Rosai-Dorfman disease manifesting as intracranial and intraorbital lesion

Rosai-Dorfman disease is a rare benign idiopathic histioproliferative disorder usually manifesting as massive painless adenopathy. Extranodal involvement of the Central Nervous System (CNS) mimicking a skull base meningioma is rare. A 42-year-old male presented with painless, progressive left visual loss of 4 months duration. Clinically, he had a left ptosis, proptosis and ophthalmoplegia. Magnetic Resonance Imaging (MRI) of the brain with gadolinium revealed a destructive lesion of the left orbital apex, middle cranial fossa and cavernous sinus. He was treated with corticosteroids and underwent debulking. Pathology showed inflammatory infiltrate in the absence of an infectious agent, emperipolesis and a positive S100 stain was consistent with Rosai-Dorfman disease. As there was no improvement following steroids and debulking, he underwent radiation therapy with significant improvement of his symptoms. Although a rare entity, Rosai-Dorfman disease should be considered in the differential of a skull base lesion.     

淋巴结外Rosai-Dorfman病二例报告并文献复习

为了探讨淋巴结外Rosai-Dorfman病的病理学特征、临床表现、诊断、治疗及预后,对2例结外Rosai-Dorf-man病行HE和免疫组织化学染色观察,并对其进行随访。结果显示,光镜下见病灶内有大量含嗜酸性胞质的组织细胞,胞质中可见被吞噬的淋巴细胞、浆细胞或中性粒细胞,S-100和CD68染色( )。治疗方法多样,疗效及预后不同。初步研究结果提示,结外Rosai-Dorfman病是一种少见的组织细胞增生性病变,有一定的病理学特征。由于其临床表现多样,导致诊断较难,治疗效果不尽相同。     

Primary Intracerebral Rosai–Dorfman Disease: A Case Report

A 45-year-old woman presented with an isolated, contrast-enhancing brain lesion in white matter of the right frontal lobe, preoperatively thought to be either a primary brain neoplasm or metastasis. The lesion was demonstrated by histology and immunohistochemistry to be Rosai–Dorfman disease. Central nervous system (CNS) manifestations of this disease are rare. There have been 27 cases of intracranial involvement reported previously. All of them have been dural-based, where the disease clinically and radiologically resembles meningioma. To our knowledge, this is the first case of an isolated intraparenchymal CNS lesion without dural attachment, where the clinical and radiological features resembled an intraparenchymal glial neoplasm, lymphoma or metastatic tumor.     

Historical and pathological overview of Castleman disease

Castleman disease consists of several lymphoproliferative subtypes that share some histological features in the lymph nodes. On the other hand, numerous clinical findings and etiologies make the disease challenging to understand. The origin of the disease is the hyaline vascular-type unicentric Castleman disease (UCD), first reported by Benjamin Castleman et al. in 1954. Although UCD is characterized by localized lesions and lack of symptoms, multicentric Castleman disease (MCD) with multiple lesions and systemic symptoms was reported by Frizzera in 1983. MCD is further divided according to KSHV/HHV8 infection status. In KSHV/HHV8-related MCD, viral infection signals lead to excessive cytokine production, and cause clinical and pathologic abnormalities. Some cases of plasma cell-type KSHV/HHV8-negative MCD can be found in association with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-proteins, and skin changes), which is a paraneoplastic syndrome. The others are idiopathic MCD, which are currently considered a heterogeneous group of diseases with overlapping pathological and clinical features. In this article, we summarize the historical evolution of Castleman disease to help understand the disease concept. We also review the latest ideas and definitions of the subtypes within the MCD spectrum and summarize the histopathological findings.     

Extranodal manifestation of Rosai Dorfman Disease of the nasopharynx

The aim of presenting this case is to highlight the fact that extranodal manifestation of Rosai Dorfman Disease (RDD) without lymphadenopathy was seen in a teenaged girl with recurrent episodes of epistaxis, due to a lobulated mass in the nasopharynx, which mimicked malignancy. This case is a rare presentation because the extranodal manifestation of RDD in multisystem organs are reported in literature, but the nasopharyngeal manifestation without lymphadenopathy is unique. The clinical presentation and surgical management of the case by endoscopic sinus surgery, the aetiopathology, differential diagnosis and review of literature are described here.     

Topical antifungal‐corticosteroid combination therapy for the treatment of superficial mycoses: conclusions of an expert panel meeting

Superficial fungal infections affect 20–25% of people worldwide and can cause considerable morbidity, particularly if an inflammatory component is present. As superficial fungal infections can be diverse, the treatment should be tailored to the individual needs of the patient and several factors should be taken into account when deciding on the most appropriate treatment option. These include the type, location and surface area of the infection, patient age, degree of inflammation and underlying comorbidities. Although several meta‐analyses have shown that there are no significant differences between the numerous available topical antifungal agents with regard to mycological cure, agents differ in their specific intrinsic properties, which can affect their clinical use. The addition of a corticosteroid to an antifungal agent at the initiation of treatment can attenuate the inflammatory symptoms of the infection and is thought to increase patient compliance, reduce the risk of bacterial superinfection and enhance the efficacy of the antifungal agent. However, incorrect use of antifungal‐corticosteroid therapy may be associated with treatment failure and adverse effects. This review summarises available treatment options for superficial fungal infections and provides general treatment recommendations based on the consensus outcomes of an Expert Panel meeting on the topical treatment of superficial mycoses.     

Histopathological study of regional lymph nodes in osteosarcoma.

This paper presents the histological appearance of the lymph nodes in cases of osteogenic sarcoma of the limb bones. The nodes were studied for the degree of sinus histiocytosis. Cases with no sinus histiocytosis showed a poor prognosis while cases with moderate to marked sinus histiocytosis showed a better rate of survival. Since the regional lymph nodes play an important part in the immunological process, preservation of regional nodes is suggested.     

Meningeal Rosai-Dorfman disease: report of three cases and literature review

Rosai-Dorfman disease is a well-recognized clinicopathological entity, which in rare cases affects the central nervous system, where it mimics meningioma. We describe three cases and review the literature. Histological and immunohistochemical confirmation is essential for definitive diagnosis. In addition to emperipolesis (lymphophagocytosis), reactivity for S-100 and CD68 and nonreactivity for CD-1a immunostaining are characteristic features of this histioproliferative disease. In contrast to meningioma, this tumor usually occurs in young males and infiltrates the brain parenchyma.     

The use of acrylic resin oral prosthesis in radiation therapy of oral cavity and paranasal sinus cancer

In radiation therapy of cancers of the oral cavity and the paranasal sinuses, the extent to which the tissues of the oral cavity are included in the radiation treatment portals will determine the severity of the oral discomfort during treatment. This will affect the nutritional status of the patients, and may eventually affect the total dose of radiation which the patients can receive for treatment of their cancers. In cooperation with the Maxillofacial Prosthetic Department, an acrylic resin oral prosthesis was developed. This prosthesis is easy to use and can be made for each individual patient within 24 hours. It allows for maximum sparing of the normal tissues in the oral cavity and can be modified for shielding of backscattered electrons from heavy metals in the teeth. We have also found that acrylic resin extensions can be built onto the posterior edge of post-maxillectomy obturators; this extension can be used as a carrier for radioactive sources to deliver radiation to deep seated tumor nodules in the paranasal sinuses.     

Castleman disease and mimickers: Clinicopathological findings of atypical lymphoproliferative disorders associated with autoimmune disease

Atypical lymphoproliferative disorders (LPDs) related with autoimmune disease (AID) show marked clinicopathological diversity, which are defined as three distinct clinicopathological subtypes such as those resembling Castleman disease (CD), atypical paracortical hyperplasia with lymphoid follicles (APHLF), and atypical lymphoplasmacytic and immunoblastic proliferation (ALPIB). We studied excisional biopsy specimens from 31 patients with atypical LPDs associated with systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and Sjögren syndrome (SjS). The lesions in these 31 cases were classified into 6 (19.4%) cases resembling CD, 14 (45.2%) cases of APHLF, and 11 (35.5%) cases of ALPIB. Five cases (83.3%) resembling CD were in the active stage with systemic symptoms and multicentric lymphadenopathy. Thirteen cases (92.9%) of APHLF showed systemic symptoms, multicentric lymphadenopathy and abnormal laboratory findings. Histologic findings for cases resembling CD were rare in patients with RA and SjS. In AID patients, histologic findings for cases resembling CD or APHLF findings correlated with disease activity and multicentric lymphadenopathy. Six cases (54.5%) of ALPIB were in the active phase with systemic symptoms and multicentric lymphadenopathy. ALPIB tended to be unrelated to AID activity, especially in the majority of patients with no abnormal laboratory findings. Atypical LPDs associated with AID is a group of diseases that may be overdiagnosed and overtreated. The diagnosis of atypical LPDs associated with AID requires an understanding of the histological findings as well as a comprehensive assessment of the presence of systemic symptoms, the distribution of lymphadenopathy, and abnormal laboratory findings.     

儿童噬血细胞综合征的诊断和治疗

目的:提高对儿童噬血细胞综合征(hemophargocytic syndrome,HPS)的认识。方法:回顾性分析24例HPS患者的临床资料。结果:24例HPS患者有15例与感染相关,1例家族性,1例系统性红斑狼疮所致,7例病因未明;临床表现持续性高热24例(100%),肝和(或)脾大24例(100%),血常规二系或三系减少24例(100%),高甘油三酯血症12例,10例患者测血清铁蛋白有9例明显增高,凝血功能异常者18例,丙氨酸转氨酶增高21例,24例骨髓均发现组织细胞增多,有噬血组织细胞,并可见明显吞噬血细胞现象,组织细胞形态基本正常。13例加用糖皮质激素,18例给大剂量人血丙种球蛋白治疗,3例用Vp-16 泼尼松 环孢素A化疗。7例治愈,好转10例,8例死亡。结论:HPS病因复杂,病情凶险,病死率高,临床及实验室特点类似于恶性组织细胞病,骨髓组织细胞形态学特点有助于两者的鉴别。早期诊断和正确治疗方案是提高HPS患者生存率的关键。     

Sinus histiocytosis mimicking metastatic melanoma in lymph nodes of a patient with a large joint prosthesis: Case report and review of the literature

Malignant melanoma metastases to regional lymph nodes may be mimicked by several non-neoplastic processes, including sinus histiocytosis induced by fragments shed from joint prostheses. A patient who had an elective lymph node dissection for malignant melanoma and was found to have “post-prosthesis lymph node histiocytosis” resembling metastatic disease is described. Knowledge of the patient's past history of a total shoulder joint replacement along with the use of polarized light microscopy to identify birefringent particles of prosthetic debris allows for an accurate histologic diagnosis. © 1995 Wiley-Liss, Inc.     

An analysis of the utilisation of chemoprophylaxis against Pneumocystis jirovecii pneumonia in patients with malignancy receiving corticosteroid therapy at a cancer hospital

Pneumocystis jirovecii pneumonia (PCP) is associated with high mortality in immunocompromised patients without human immunodeficiency virus infection. However, chemoprophylaxis is highly effective. In patients with solid tumours or haematologic malignancy, several risk factors for developing PCP have been identified, predominantly corticosteroid therapy. The aims of this study were to identify the potentially preventable cases of PCP in patients receiving corticosteroid therapy at a tertiary care cancer centre and to estimate the frequency of utilisation of chemoprophylaxis in these patients. Two retrospective reviews were performed. Over a 10-year period, 14 cases of PCP were identified: no cases were attributable to failed chemoprophylaxis, drug allergy or intolerance. During a 6-month period, 73 patients received high-dose corticosteroid therapy (> or =25 mg prednisolone or > or =4 mg dexamethasone daily) for > or =4 weeks. Of these, 22 (30%) had haematologic malignancy, and 51 (70%) had solid tumours. Fewer patients with solid tumours received prophylaxis compared to patients with haematologic malignancy (3.9 vs 63.6%, P<0.0001). Guidelines for PCP chemoprophylaxis in patients with haematologic malignancy or solid tumours who receive corticosteroid therapy are proposed. Successful primary prevention of PCP in this population will require a multifaceted approach targeting the suboptimal prescribing patterns for chemoprophylaxis.     

鸡新城疫病毒对人类口腔鳞癌细胞的杀伤作用

目的　检测鸡新城疫病毒 (newcastlediseasevirus ,NDV)对人口腔鳞癌细胞的杀伤作用。方法　利用MTT方法检测NDV对人口腔鳞癌 (oralsquamouscellcarcinoma ,OSCC)颈淋巴结转移癌细胞系 (GNM)及人舌鳞癌细胞系 (TSCCa)细胞株的杀伤性。结果　NDV作用后的人口腔鳞癌细胞株的细胞活性比对照组的细胞活性有显著下降 (P <0 0 1) ,NDV血凝效价值显著升高。结论　NDV能够通过在人口腔鳞癌细胞内增殖杀死癌细胞株。