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Cureus Apr 2017Rosai-Dorfman disease (also known as sinus histiocytosis with massive lymphadenopathy) is a rare benign proliferative disorder of histiocytes that typically involves the...
Rosai-Dorfman disease (also known as sinus histiocytosis with massive lymphadenopathy) is a rare benign proliferative disorder of histiocytes that typically involves the lymph nodes and can also involve extranodal sites. Rosai-Dorfman disease confined to the breast is extremely rare, but important to recognize as it can mimic malignancy. We present the case of a 63-year-old woman who presented with a palpable breast lump that was highly suspicious for malignancy based on mammogram and ultrasound appearance. Biopsy revealed inflammatory tissue with lymphoplasmacytic and histiocytic predominance. The diagnosis of Rosai-Dorfman was made based on characteristic staining of histiocytes with S-100 and the presence of emperipolesis. Early recognition of this benign disease entity spared the patient further investigation and surgical intervention.
PubMed: 28503389
DOI: 10.7759/cureus.1153 -
Iranian Journal of Otorhinolaryngology Jul 2023Multifocal Langerhans' cell histocytosis is a rare condition that can affect multiple organs and manifest in various scenarios. While the condition is more commonly...
INTRODUCTION
Multifocal Langerhans' cell histocytosis is a rare condition that can affect multiple organs and manifest in various scenarios. While the condition is more commonly found in children, it can also occur in adults.
CASE REPORT
A 43-year-old female presented with refractory otorrhea and had a rubbery neck mass in the left mid-cervical area, as well as an itchy eczematoid lesion in the left parietal area. The otic lesion was eventually resected, and histopathologic examination confirmed the diagnosis of Langerhans histiocytosis.
CONCLUSIONS
Although rare in adults, Langerhans histiocytosis should be considered as one of the differential diagnoses for ear canal polyps. If diagnosed, medical treatment should be pursued.
PubMed: 37497157
DOI: 10.22038/IJORL.2023.65286.3238 -
The American Journal of Case Reports May 2024BACKGROUND Langerhans cell histiocytosis (LCH) is a rare and uncontrolled proliferation of dendritic cells of myeloid origin. The incidence of LHC was estimated at 5...
BACKGROUND Langerhans cell histiocytosis (LCH) is a rare and uncontrolled proliferation of dendritic cells of myeloid origin. The incidence of LHC was estimated at 5 cases per million children ages 0-15 years old. The most common places for this tumor are the jaw, vertebra, pelvis, and the extremities. The disease with multisystem involvement can present a mortality rate of 20% and one-third of children have multisystem involvement. We present a case with unusual bone involvement of the anterior cranial base with a challenging diagnosis and a complex surgical approach. CASE REPORT We report the case of a 6-year-old boy who manifested the disease with daily holocranial headache, worse in the frontal region and refractory to analgesia for 10 days, strabismus homonymous, diplopia, and right palpebral ptosis. The tumor affected the sphenoid sinus, internal carotid artery, and sella turcica, and made contact with the pituitary gland. A joint surgery with Otorhinolaryngology and Neurosurgery was performed by nasal endoscopic access to the skull base by means of the right medial turbinectomy (for the access) and right sphenoid opening, septectomy and opening of the left sphenoid to work with 4 hands and, after resection of lesion, inside the sphenoid. CONCLUSIONS This patient had rare bone involvement from LCH and atypical clinical presentation next to the important and delicate structures of the anterior skull base, but had a satisfactory outcome.
Topics: Humans; Histiocytosis, Langerhans-Cell; Male; Sphenoid Sinus; Child
PubMed: 38764221
DOI: 10.12659/AJCR.942681 -
Histopathology Jul 2022The sinonasal tract is a common extranodal site for Rosai-Dorfman disease (RDD). Recently, histiocytes with features of RDD were identified in the clinical setting of...
AIMS
The sinonasal tract is a common extranodal site for Rosai-Dorfman disease (RDD). Recently, histiocytes with features of RDD were identified in the clinical setting of chronic sinusitis. This study evaluates whether this phenomenon should be considered part of the RDD spectrum or classified separately as RDD-like histiocytes.
METHODS AND RESULTS
We prospectively collected 13 cases showing histological features of RDD in chronic sinusitis patients and identified 14 with similar findings (3.5%) via retrospective review of 403 sinus contents over 2 years. All 27 cases displayed nodular aggregates of eosinophilic histiocytes with intermixed lymphoplasmacytic inflammation, prominent eosinophils and emperipolesis. The histiocytes were positive for S100 protein and cyclin D1 and negative for CD1a and CD207. All patients presented with severe chronic sinusitis without tumour formation or systemic symptoms. Twelve patients with follow-up (55%) required repeat sinus surgery compared with just 43 other sinusitis patients evaluated (11%); features of RDD were present in their additional specimens. Two cases that underwent targeted next-generation sequencing (20%) had oncogenic mutations in NF1 and KEAP1.
CONCLUSIONS
Overall, these findings confirm diagnostic histological and immunohistochemical features of RDD in a subset of chronic sinusitis specimens. While patients uniformly lack systemic involvement or tumefactive growth, they have a high risk of recurrent sinus disease. Although the relatively subtle nature of the findings raises consideration of separate classification, the presence of occasional oncogenic mutations and evidence of consistent MAPK/ERK pathway activation via cyclin D1 positivity suggests that this phenomenon represents a unique limited manifestation of RDD.
Topics: Cyclin D1; Histiocytosis, Sinus; Humans; Kelch-Like ECH-Associated Protein 1; NF-E2-Related Factor 2; Sinusitis
PubMed: 35426462
DOI: 10.1111/his.14664 -
Gastric Cancer : Official Journal of... Nov 2023The status of regional tumour draining lymph nodes (LN) is crucial for prognostic evaluation in gastric cancer (GaC) patients. Changes in lymph node microarchitecture,... (Meta-Analysis)
Meta-Analysis Review
The clinical importance of the host anti-tumour reaction patterns in regional tumour draining lymph nodes in patients with locally advanced resectable gastric cancer: a systematic review and meta-analysis.
BACKGROUND
The status of regional tumour draining lymph nodes (LN) is crucial for prognostic evaluation in gastric cancer (GaC) patients. Changes in lymph node microarchitecture, such as follicular hyperplasia (FH), sinus histiocytosis (SH), or paracortical hyperplasia (PH), may be triggered by the anti-tumour immune response. However, the prognostic value of these changes in GaC patients is unclear.
METHODS
A systematic search in multiple databases was conducted to identify studies on the prognostic value of microarchitecture changes in regional tumour-negative and tumour-positive LNs measured on histopathological slides. Since the number of GaC publications was very limited, the search was subsequently expanded to include junctional and oesophageal cancer (OeC).
RESULTS
A total of 28 articles (17 gastric cancer, 11 oesophageal cancer) met the inclusion criteria, analyzing 26,503 lymph nodes from 3711 GaC and 1912 OeC patients. The studies described eight different types of lymph node microarchitecture changes, categorized into three patterns: hyperplasia (SH, FH, PH), cell-specific infiltration (dendritic cells, T cells, neutrophils, macrophages), and differential gene expression. Meta-analysis of five GaC studies showed a positive association between SH in tumour-negative lymph nodes and better 5-year overall survival. Pooled risk ratios for all LNs showed increased 5-year overall survival for the presence of SH and PH.
CONCLUSIONS
This systematic review suggests that sinus histiocytosis and paracortical hyperplasia in regional tumour-negative lymph nodes may provide additional prognostic information for gastric and oesophageal cancer patients. Further studies are needed to better understand the lymph node reaction patterns and explore their impact of chemotherapy treatment and immunotherapy efficacy.
Topics: Humans; Stomach Neoplasms; Hyperplasia; Histiocytosis, Sinus; Clinical Relevance; Lymph Nodes; Prognosis; Esophageal Neoplasms; Neoplasm Staging
PubMed: 37776394
DOI: 10.1007/s10120-023-01426-w -
Cureus Feb 2023Rosai-Dorfman Disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is an uncommon histiocytic condition characterized by massive...
Rosai-Dorfman Disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is an uncommon histiocytic condition characterized by massive histopathological aggregation of CD1-a negative, CD68-positive, and S100-positive histiocytes. It was initially described by Destombes in 1965 under the term "adenitis with lipid excess." However, it is named after Rosai and Dorfman who reported further histopathological features of the disease in 1969. The diagnosis of this non-Langerhans cell histiocytosis can be challenging and requires high clinical suspicion. The diagnostic process usually involves imaging, tissue biopsies, and genetic testing as needed. In this case series, we are presenting three cases of rare disease. Case 2 had both nodal and extranodal forms, which makes this case rarer than cases 1 and 3, which present with extranodal lesions.
PubMed: 36960255
DOI: 10.7759/cureus.35193 -
Cureus Dec 2022Sinus histiocytosis with massive lymphadenopathy (SHML), an alternative term for Rosai-Dorfman disease (RDD), is a rare benign idiopathic...
Sinus histiocytosis with massive lymphadenopathy (SHML), an alternative term for Rosai-Dorfman disease (RDD), is a rare benign idiopathic immune-related lymphoproliferative condition. The central nervous system (CNS) has been documented to be involved in RDD, although lymph nodes are the organs that are most frequently and primarily associated with the disease manifestation. Nonetheless, CNS involvement in RDD is rare and poorly understood. As a result, there is a lack of a solid basis for therapeutic approaches for CNS involvement in RDD. Here, we present a case of RDD with cerebral involvement, a rare presentation of RDD with atypical symptoms. A brief assessment of the radiographic appearance, histological findings, and the peculiar manifestations of the disease is provided.
PubMed: 36660540
DOI: 10.7759/cureus.32605 -
Ear, Nose, & Throat Journal Aug 2019In this article, we explore the disease spectrum and clinical characteristics of and the diagnosis and endoscopic approach to treating isolated sphenoid sinus disease...
In this article, we explore the disease spectrum and clinical characteristics of and the diagnosis and endoscopic approach to treating isolated sphenoid sinus disease (ISSD) in children. To these ends, we review a case series of 19 patients (mean age: 8.1 ± 4.9 years, range: 1.1-15 years, median age: 6.7 years, 13 males, 6 females) who underwent surgical treatment at our hospital for ISSD during the 4 years between 2012 and 2016. The symptoms of pediatric sphenoid sinus disease tend to be variable and nonspecific and include atypical headache, nasal congestion, epistaxis, postnasal drip, snoring, and impaired vision. Headache is the presenting symptom in 42% of patients, but headaches occurred in no specific or typical location. Ten patients underwent preoperative endoscopic examination, and abnormalities in the sphenoethmoidal recess were found in 6 (60%) of these 10 patients. All 19 patients underwent ultra-low-dose paranasal sinus computed tomography (CT) imaging, and 9 patients with suspected tumors or sphenoid mucoceles were further examined by magnetic resonance imaging (MRI). The endoscopic transostial approach was performed in all 19 patients: 16 patients received excision of inflammatory sphenoid sinus disorders and benign tumors, including sphenoid sinusitis, sphenoid sinus mucocele, sphenoid sinus polyp, and ossifying fibroma; 3 patients with suspected tumors received biopsies to detect rhabdomyosarcoma, Langerhans cell histiocytosis, and juvenile xanthogranuloma. No intraoperative or immediate postoperative complications were observed. Children with opacified sphenoid sinus identified by radiographic imaging presented a variety of pathologies. The most common lesions were associated with inflammatory disease. Because the symptoms of pediatric sphenoid sinus disease tend to be variable and nonspecific, CT remains the standard for evaluating sphenoid sinus disease, and ultra-low-dose paranasal sinus CT imaging is recommended and can provide images of equal or better quality compared with those obtained by standard dose CT. In addition, MRI is an essential adjunct in the diagnosis and selection of treatment for suspected tumors of the sphenoid sinus. The endoscopic transostial approach was especially suitable for the management of pediatric benign isolated sphenoid sinus lesions.
Topics: Adolescent; Child; Child, Preschool; Endoscopy; Female; Humans; Infant; Magnetic Resonance Imaging; Male; Mucocele; Nasal Polyps; Paranasal Sinus Diseases; Paranasal Sinus Neoplasms; Sphenoid Sinus; Sphenoid Sinusitis; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 31012343
DOI: 10.1177/0145561319841227 -
Revista Espanola de Enfermedades... Jun 2024We present a case of Rosai-Dorfman disease (RDD) occurred in a 6-year-old male child, characterized by extensive involvement of the esophagus. Eight months ago, the...
We present a case of Rosai-Dorfman disease (RDD) occurred in a 6-year-old male child, characterized by extensive involvement of the esophagus. Eight months ago, the child presented with persistent fever and a diffuse dark red rash. MRI revealed a mass occupying the left nasal cavity and septal sinus and biopsy pathology confirmed the diagnosis of RDD. Following dexamethasone and prednisone treatment, the child experienced dysphagia. 18F-FDG PET/CT revealed multiple lesions with increased metabolism in the left nasal sinus, lymph nodes, widespread skin lesions, and the entire esophagus. Subsequent biopsies of lymph nodes, abdominal skin, and esophageal lesions was consistent with RDD involvement. The child is presently undergoing six cycles of VCR+Ara-c+Dex chemotherapy and the treatment is going well.
Topics: Humans; Histiocytosis, Sinus; Male; Child; Esophageal Diseases; Esophagus; Positron Emission Tomography Computed Tomography
PubMed: 37350657
DOI: 10.17235/reed.2023.9753/2023 -
Radiology. Imaging Cancer Jun 2022
Topics: Diagnosis, Differential; Histiocytosis, Sinus; Humans; Sciatic Nerve
PubMed: 35748761
DOI: 10.1148/rycan.220046