-
Autopsy & Case Reports 2023First described by J Rosai and R F Dorfman in 1969, Rosai-Dorfman disease (RDD) is a benign, self-limiting histiocytosis of unknown etiology. It is usually seen in the...
First described by J Rosai and R F Dorfman in 1969, Rosai-Dorfman disease (RDD) is a benign, self-limiting histiocytosis of unknown etiology. It is usually seen in the first two decades of life. The most frequent clinical presentation is painless, bilateral cervical lymphadenopathy accompanied by fever, weight loss, and an elevated ESR. However, RDD without nodal involvement is extremely rare, and the most common extranodal location is the head and neck region, mainly affecting the nasal cavity, pharynx, and paranasal sinuses. Oral location of RDD is occasional; according to our knowledge, only 17 cases of oral Rosai-Dorfman disease without lymph node involvement have been found in the literature. Because of the rarity of these isolated oral presentations, the clinical and radiological aspects need to be more studied. This article aims to present a rare case of oral Rosai-Dorfman disease without nodal involvement, detail the clinical and radiological signs, and the treatment strategy used in our patient.
PubMed: 38149069
DOI: 10.4322/acr.2023.463 -
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 -
Journal of Radiology Case Reports Sep 2023Rosai-Dorfman Disease, otherwise known as sinus histiocytosis with massive lymphadenopathy, is a rare form of non-Langerhans cell histiocytosis with an estimated... (Review)
Review
Rosai-Dorfman Disease, otherwise known as sinus histiocytosis with massive lymphadenopathy, is a rare form of non-Langerhans cell histiocytosis with an estimated incidence of 100 cases per year in the United States. Due to its variable presentation and nonspecific clinical findings, it is particularly difficult to diagnose in pediatric patients. We report a case of an 11-month-old male who presented with a 4-day history of a right groin mass. Ultrasound of the groin and pelvis demonstrated, and MRI of the abdomen and pelvis confirmed an inguinal mass with surrounding lymphadenopathy. Pathology confirmed Rosai-Dorfman Disease and the patient improved after starting oral steroid therapy. To the best of our knowledge, this is the first case of Rosai-Dorfman Disease involving the inguinal region in an infant under 1 year of age reported in the literature. In this case report, we discuss the imaging and histology findings as well as provide a brief literature review for this diagnosis.
Topics: Humans; Male; Child; Infant; Histiocytosis, Sinus; Pelvis; Lymphadenopathy; Diagnosis, Differential; Magnetic Resonance Imaging
PubMed: 38098961
DOI: 10.3941/jrcr.v17i8.4873 -
Pediatric Blood & Cancer Mar 2024
Topics: Humans; Histiocytosis, Sinus; Neoplasms; Germ Cells
PubMed: 38073016
DOI: 10.1002/pbc.30801 -
Photodiagnosis and Photodynamic Therapy Feb 2024Cutaneous Rosai-Dorfman Disease (CRDD), a rare form of Rosai-Dorfman Disease (RDD), lacks established treatment guidelines. In this case report, we detailed a successful...
Cutaneous Rosai-Dorfman Disease (CRDD), a rare form of Rosai-Dorfman Disease (RDD), lacks established treatment guidelines. In this case report, we detailed a successful case of CRDD management in a 50-year-old woman with facial lesions. Our treatment included 5-aminolevulinic acid photodynamic therapy (ALA-PDT) and low-dose oral corticosteroids. Remarkable improvement was evident after six ALA-PDT courses, and a one-year follow-up confirmed sustained remission. This case highlights the potential of combining ALA-PDT with low-dose corticosteroids as a promising therapeutic strategy for CRDD. Further research is necessary to fully elucidate its mechanisms and effectiveness.
Topics: Female; Humans; Middle Aged; Photochemotherapy; Aminolevulinic Acid; Histiocytosis, Sinus; Photosensitizing Agents; Adrenal Cortex Hormones; Skin Diseases
PubMed: 38042238
DOI: 10.1016/j.pdpdt.2023.103914 -
Indian Journal of Otolaryngology and... Dec 2023Langerhans cell histiocytosis (LCH) is a disease characterized by localized and generalized proliferation of the histiocytes. It is a locally aggressive condition. The...
Langerhans cell histiocytosis (LCH) is a disease characterized by localized and generalized proliferation of the histiocytes. It is a locally aggressive condition. The clinical presentation is highly variable and can range from isolated, self-healing skin or bone lesions to life-threatening multisystem disease. It can present as a unifocal or multifocal disease. The majority are present in the head and neck region, but the involvement of Paranasal sinuses is rare. Here we describe a 64-years-old female who presented with a slow-growing left nasal mass for 1 year. Evaluation of the patient was suggestive of malignancy, but the biopsy report turned out to be Langerhans cell histiocytosis; subsequently left, total maxillectomy was done. We hereby present a unique case of LCH with isolated nose and paranasal sinus involvement.
PubMed: 37974832
DOI: 10.1007/s12070-023-04075-8 -
The Journal of Dermatology Apr 2024
Topics: Humans; Histiocytosis, Sinus; Diagnosis, Differential
PubMed: 37950432
DOI: 10.1111/1346-8138.17035 -
Annals of Hematology Jan 2024Rosai Dorfman disease (RDD) is a non-Langerhans cell histiocytic neoplasm characterized by sinus histiocytosis with variable emperipolesis. There is a limited...
Rosai Dorfman disease (RDD) is a non-Langerhans cell histiocytic neoplasm characterized by sinus histiocytosis with variable emperipolesis. There is a limited understanding of the factors that contribute to disease progression. Traditional management of RDD consists of local therapies (resection, radiation) for localized disease and myelosuppression for systemic disease; targeted medications have also recently been introduced into clinical practice as an additional therapeutic modality. The goals of this study are to compare the impact of targeted therapies to conventional management of RDD and identify trends in laboratory data that may provide insight into disease progression. A retrospective analysis was conducted at a single institution over a 20-year period in 35 adult patients with histopathologic evidence of RDD without confounding secondary malignancies. Clinical data points included laboratory evaluation, molecular diagnostics, imaging, and therapies rendered. Binary data was utilized for statistical analysis and comparison of outcomes by treatment type and utilization of targeted agents. Evaluation of treatment response varied based on anatomic disease sites and baseline imaging modality. To standardize the radiographic analysis, we included PERCIST (if PET was utilized) or RECIST assessments (in the cases of CT or MR imaging). Conventional therapies rendered included local treatment (surgery, radiation, intralesional injections), systemic corticosteroids, immunotherapy, and chemotherapy while targeted agents included only small molecule inhibitors. In this analysis, primary disease was identified in cutaneous, osseous, and CNS structures (17, 11, and 6/35 patients respectively). Management consisted of surgery (12/35 patients), steroid and myelosuppressive therapies (9/35 each), immunotherapy (5/35), and targeted molecular agents (5/35). In evaluating outcomes, the proportion of partial responses was substantially higher in recipients of molecular as compared to conventional therapy (4/5 patients compared to 6/29) while complete responses were more common in the conventional therapy cohort (12/29 compared to 1/5). Lastly, an evaluation of peripheral blood absolute monocytes in all patients who had progressed on therapy identified a significant decrease in pre-progression values as compared to values following therapy re-institution (averages of 0.70 and 0.27 K/µL, respectively; p = 0.0002, 95% CI 0.652-0.2360). Larger-scale studies are needed to further evaluate the relevance of the monocyte trends that were identified in terms of their relationship to disease status. This study is the largest analysis of Rosai Dorfman disease, that we are aware of, from a single institution. In this cohort, the utilization of small molecule inhibitors corresponded to a greater increase in partial responses than conventional therapies, although the opposite effect has been observed in complete responses. This finding can be attributed to the recent introduction of targeted agents and shorter follow-up. We anticipate higher complete response rates with the use of small molecule in ongoing analyses over a longer follow-up period. The recognition of relative monocyte elevation prior to disease progression is an intriguing and to our knowledge, novel finding in the field of Rosai Dorfman disease. Future studies aimed at elucidating the implications of this trend are in progress.
Topics: Adult; Humans; Histiocytosis, Sinus; Monocytes; Retrospective Studies; Treatment Outcome; Disease Progression
PubMed: 37940718
DOI: 10.1007/s00277-023-05494-x -
Revisiting Rosai Dorfman disease: A rare histiocytic disorder with nodal and extranodal involvement.SA Journal of Radiology 2023Rosai Dorfman disease is a rare but benign and self-limiting disorder. A case of Rosai Dorfman disease in a 16-year-old female with classic radiological and pathological...
UNLABELLED
Rosai Dorfman disease is a rare but benign and self-limiting disorder. A case of Rosai Dorfman disease in a 16-year-old female with classic radiological and pathological findings is presented. T2W hypointense signal of the soft tissue masses was a distinguishing MRI feature.
CONTRIBUTION
Rosai Dorfman disease is often overlooked in the differentials of lymphadenopathy and soft tissue masses on account of its rarity. This case report comprehensively discusses the imaging approach to this rare disorder.
PubMed: 37928929
DOI: 10.4102/sajr.v27i1.2729 -
Medicine Oct 2023Rosai-Dorfman disease is a benign lymphatic tissue proliferative disease with slow growth and is rarely observed in the clinic. Most of these patients present clinically... (Review)
Review
BACKGROUND
Rosai-Dorfman disease is a benign lymphatic tissue proliferative disease with slow growth and is rarely observed in the clinic. Most of these patients present clinically with enlarged lymph nodes. In patients with spinal extranodal Rosai-Dorfman disease, which is even rare than the disease of lymph nodes, patients may experience numbness and weakness in the extremities.
CASE PRESENTATION
We report a 32-year-old male patient with multi-segmental spinal Rosai-Dorfman disease. On admission, his left fingers had been numb for 2 months. Over a 2-month period, the limb numbness progressed from the left to the right hand. The patient underwent resection of the lesion and internal fixation of the C2-C7 spine. The postoperative outcomes were satisfied and no recurrence was observed at 1-year follow-up.
CONCLUSION
Spinal Rosai-Dorfman disease is a relatively rare tumor of which the pathogenesis is still unclear, and most patients have no specific clinical manifestations. Characteristic imaging findings can indicate this disease, however, a definitive diagnosis still depends on a pathological examination. Currently, total surgical resection of the tumor is a relatively effective and preferred treatment.
Topics: Male; Humans; Adult; Histiocytosis, Sinus; Hypesthesia; Lymphadenopathy; Cervical Vertebrae; Neoplasms
PubMed: 37904362
DOI: 10.1097/MD.0000000000035654