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Systematic Reviews Jan 2021To review and summarize the clinical features, diagnosis, treatment strategies, and prognosis of spinal Rosai-Dorfman disease (RDD). (Review)
Review
PURPOSE
To review and summarize the clinical features, diagnosis, treatment strategies, and prognosis of spinal Rosai-Dorfman disease (RDD).
METHODS
RDD is also termed as sinus histiocytosis with massive lymphadenopathy. We searched the databases of PubMed, Elsevier ScienceDirect, SpringerLink, and OVID. The keywords were Rosai-Dorfman disease and spine/central nervous system. Research articles and case reports with accessibility to full texts regarding spinal RDD were eligible for the inclusion. A total of 62 articles were included, and they contained 69 cases. We extracted the information of interest and analyzed them using SPSS statistics package.
RESULTS
The average age was 33.1 ± 18.3 years. The ratio of males to females was 1.9/1. Overall, 63 cases presented with spine-related symptoms. A total of 27 cases (39.1%) had multi-organ lesions, and 12 cases had records of massive lymphadenopathy. Among 47 cases who first manifested spine-related symptoms, 93.6% were preoperatively misdiagnosed. The disease had a predilection for cervical spine (38.8%) and thoracic spine (40.3%). 62.9% of lesions were dura-based. Surgery remained the mainstream treatment option (78.8%), with or without adjuvant therapies. Total lesion resection was achieved in 34.8% of cases. The rate of lesion recurrence/progression was 19.5%, which was marginally lower for total resection than for non-total resection.
CONCLUSION
Spinal RDD has no pathognomonic clinical and imaging features. Most cases first present with spine-relevant symptoms. Massive lymphadenopathy is not common, but a tendency for multi-organ involvement should be considered. Spinal RDD has a high recurrence rate; thus, total resection is the treatment of choice. Adjuvant therapies are indicated for multi-organ lesions and residual lesions. A wait and watch strategy is recommended for asymptomatic patients. Herein, a workflow of diagnosis and treatment of the spinal RDD is established.
Topics: Adolescent; Adult; Female; Histiocytosis, Sinus; Humans; Male; Middle Aged; Prognosis; Recurrence; Young Adult
PubMed: 33461611
DOI: 10.1186/s13643-021-01581-0 -
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 -
Annals of Surgery Open : Perspectives... Dec 2023In this review, we aim to provide an overview of literature on lymph node (LN) histomorphological features and their relationship with the prognosis in colorectal cancer... (Review)
Review
OBJECTIVE
In this review, we aim to provide an overview of literature on lymph node (LN) histomorphological features and their relationship with the prognosis in colorectal cancer (CRC).
BACKGROUND
Lymph nodes play a crucial role in the treatment and prognosis of CRC. The presence of LN metastases considerably worsens the prognosis in CRC patients. Literature has shown that the total number of LNs and the number negative LNs (LNnegs) has prognostic value in CRC patients. In esophageal carcinoma, LN size seems to be surrogate of the host antitumor response and a potentially clinically useful new prognostic biomarker for (y)pN0 esophageal carcinoma.
METHODS
A comprehensive search was performed in Pubmed, Embase, Medline, CINAHL, and the Cochrane library in March 2021. The PRISMA guidelines were followed. Only studies focusing on histomorphological features and LN size and their relation to overall survival were selected.
RESULTS
A total of 9 unique articles met all inclusion criteria and were therefore included in this systematic review. Six of these studies investigated HMF (eg, paracortical hyperplasia, germinal center predominance, and sinus histiocytosis) and 4 studies LNneg size and their relationship with overall survival. The presence of paracortical hyperplasia and an increased number of large LNnegs is related to a more favorable prognosis in CRC.
CONCLUSION
The results of this systematic review seem to support the hypothesis that there is a relationship between the host antitumor response reflected in different histomorphological reaction patterns visible in LNnegs and LNneg size related to survival in CRC patients.
PubMed: 38144501
DOI: 10.1097/AS9.0000000000000336 -
Head and Neck Pathology Jun 2020Rosai-Dorfman disease (RDD) is a benign, self-limiting histiocytosis of unknown etiology. The classic form of the condition includes a painless cervical lymphaenopathy...
Rosai-Dorfman disease (RDD) is a benign, self-limiting histiocytosis of unknown etiology. The classic form of the condition includes a painless cervical lymphaenopathy accompanied by fever, weight loss and an elevated ESR. Extra nodal RDD (ENRDD) is most frequent in the head and neck. Thirty-eight cases of ENRDD have been described. Seven cases of ENRDD were identified in our pathology biopsy services. The demographic and clinical information was tabulated logically on the basis of age, gender, location and presence or absence of symptoms, treatment and follow-up. Radiographic and histopathological features were also examined. The findings in these cases were correlated with those available from the previously reported cases. Six cases affected women and one case was diagnosed in a male. The age ranged from 22-55 years. Three cases presented as a nasal mass. One of these lesions extended into the paranasal sinuses. One case was located in the maxilla and extended to involve the maxillary sinus. Three cases were diagnosed in the mandible. The maxillary and one mandibular lesion (Case 2) resulted in significant painful irregular bone destruction with a non-healing socket and tooth mobility respectively. One mandibular lesion was asymptomatic (Case 6). The third case affecting the mandible presented as a rapidly expansile mass following a tooth extraction (Case 7). Nasal masses presented with symptoms of obstruction. Nasal masses were excised with no recurrence from up to 2-3 years of follow-up. The mandibular lesions were curetted aggressively. The oral mass in Case 7 was excised synchronously. No recurrence up to 2 years was recorded in Case 2. Follow-up information is not available for Cases 6 and 7. The maxillary lesion was not intervened surgically. The patient has persistent but stable disease for a follow-up period of 2 years. ENRDD is rarely considered in the differential diagnosis in the absence of lymph node involvement. Lesions of ENRDD resemble many other histiocytic and histiocyte-rich lesions of the head and neck. This makes the diagnosis of ENRDD challenging with the potential for under diagnosis or misdiagnosis and delay in treatment.
Topics: Adult; Female; Histiocytosis, Sinus; Humans; Jaw Neoplasms; Male; Middle Aged; Nose Neoplasms; Paranasal Sinus Neoplasms; Young Adult
PubMed: 31368076
DOI: 10.1007/s12105-019-01056-8 -
Orphanet Journal of Rare Diseases Mar 2022Rosai-Dorfman-Destombes disease (RDD) is a rare histiocytic disorder with heterogeneous clinical manifestations and rare neurologic involvement. The existing clinical...
BACKGROUND
Rosai-Dorfman-Destombes disease (RDD) is a rare histiocytic disorder with heterogeneous clinical manifestations and rare neurologic involvement. The existing clinical literature about neurologic RDD has yet to be critically examined.
METHODS
We performed a four-database English-language systematic literature search for cases of RDD neurohistiocytosis, excluding secondary literature. Individual patient data for neurologic symptoms, disease sites, treatments, and responses were captured. Responses to first-line and second-line surgical interventions, post-surgical radiotherapy, and systemic therapies were analyzed.
RESULTS
Among 4769 articles yielded by literature search, 154 articles were fully reviewed, containing data on 224 patients with neurologic RDD. 128 (83.1%) articles were single case reports. 149 (66.5%) patients were male, 74 (33.5%) female, with a median age of 37.6 years (range 2-79). Presenting neurologic symptoms included headache (45.1%), focal neurological deficits (32.6%), visual symptoms (32.1%), and seizures (24.6%). RDD involvement was multifocal in 32 (14.3%) cases. First-line treatment involved resection in 200 (89.6%) patients, with subsequent progression in 52 (26%), including 41 (78.8%) with unifocal disease. No difference was observed in progression-free survival comparing post-operative radiotherapy to no radiotherapy following partial resection. Chemotherapy given alone as first-line treatment led to complete or partial response in 3/7(43%) patients. Second-line treatments led to complete or partial response in 18/37(37.5%) patients. Mutational data were reported on 10 patients (4.46%).
CONCLUSIONS
This review highlights the limited published data about neurologic RDD, which presents with varied symptomatology and outcome. Further study is needed about its mutational landscape, and more effective therapies are needed for recurrent and refractory disease.
Topics: Adolescent; Adult; Aged; Child; Child, Preschool; Female; Histiocytosis, Sinus; Humans; Male; Middle Aged; Nervous System; Young Adult
PubMed: 35236371
DOI: 10.1186/s13023-022-02220-0 -
Current Rheumatology Reports May 2017Rosai-Dorfman disease (RDD) is a rare histiocytic disorder typically presenting as painless cervical lymphadenopathy. Extranodal involvement is common and may also... (Review)
Review
PURPOSE OF REVIEW
Rosai-Dorfman disease (RDD) is a rare histiocytic disorder typically presenting as painless cervical lymphadenopathy. Extranodal involvement is common and may also affect bones. Here, we present a patient with typical nodal disease and multifocal bone manifestations. Further, a systematic literature review was performed to better understand the phenotype, clinical course and treatment options of such patients.
RECENT FINDINGS
RDD is a nonmalignant, classically sporadic histiocytosis. Nevertheless, increasing evidence also suggests familial forms of the disease. According to our literature review, bone involvement is exceedingly rare and heterogeneous. Clinical outcome in terms of mortality seems to be favorable in most cases. Currently, therapy strategies include surgical and immunosuppressive treatments, but the optimal treatment of osseous RDD remains to be defined. Patients with osseous RDD may present to rheumatologists with arthralgia or arthritis. Due to the rarity of the disease, diagnosis and treatment remain challenging.
Topics: Adult; Arthralgia; Bone Diseases; Diagnosis, Differential; Drug Therapy, Combination; Female; Glucocorticoids; Hand; Histiocytosis, Sinus; Humans; Immunosuppressive Agents; Magnetic Resonance Imaging; Radiography
PubMed: 28401384
DOI: 10.1007/s11926-017-0656-6