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Archives of Pathology & Laboratory... Jan 2022Rosai-Dorfman disease is a rare histiocytic proliferative disorder of unknown pathogenesis that may be diagnostically difficult in extranodal sites. It is commonly an... (Review)
Review
CONTEXT.—
Rosai-Dorfman disease is a rare histiocytic proliferative disorder of unknown pathogenesis that may be diagnostically difficult in extranodal sites. It is commonly an unsuspected diagnosis when arising in bone and soft tissue, especially when it presents without associated lymphadenopathy. Its variable clinical presentation and nonspecific imaging findings make the diagnosis quite challenging, particularly in small biopsies. The problem is compounded by its less-characteristic histomorphologic features in comparison with nodal disease. Awareness of the potential diagnostic pitfalls in Rosai-Dorfman disease of bone and soft tissue should raise the degree of diagnostic accuracy.
OBJECTIVE.—
To review the clinical manifestations, imaging characteristics, and histomorphologic features of Rosai-Dorfman disease of bone and soft tissue along with a brief discussion of its differential diagnosis, pathogenesis, and current management.
DATA SOURCES.—
Thorough review of the literature with focus on clinical manifestations, imaging findings, key histomorphologic features, pathogenesis, and treatment.
CONCLUSIONS.—
The diagnosis of Rosai-Dorfman disease of bone and soft tissue may be quite challenging because of its variable clinical presentation and nonspecific imaging findings. It may be asymptomatic without systemic manifestations or associated lymphadenopathy. The definitive diagnosis relies on histopathologic identification of the characteristic S-100-positive histiocytes demonstrating emperipolesis. Bone and soft tissue lesions tend to have lower numbers of characteristic histiocytes and less conspicuous emperipolesis and often demonstrate areas of fibrosis or storiform spindle cell areas resembling fibrohistiocytic lesions. Awareness of these unusual features is necessary in order to consider Rosai-Dorfman disease in the differential diagnosis when confronting these rare and often misleading lesions.
Topics: Diagnosis, Differential; Emperipolesis; Histiocytes; Histiocytosis, Sinus; Humans; Lymphadenopathy
PubMed: 34965285
DOI: 10.5858/arpa.2021-0116-RA -
Journal of the National Comprehensive... Nov 2021Adult-onset histiocytoses (AOH), primarily Rosai-Dorfman disease (RDD), Erdheim-Chester Disease (ECD), and adult Langerhans cell histiocytosis (ALCH), are a group of... (Review)
Review
Adult-onset histiocytoses (AOH), primarily Rosai-Dorfman disease (RDD), Erdheim-Chester Disease (ECD), and adult Langerhans cell histiocytosis (ALCH), are a group of related histiocytic neoplastic disorders featuring multisystemic manifestations. The disorders are largely incurable, and are essentially chronic neoplastic diseases with a variable prognosis. Prompt diagnosis and treatment is important to prevent debilitating and even life-threatening complications. Survivorship issues abound in AOH, due to their multisystemic manifestations and the sometimes recalcitrant chronic inflammation, which can lead to other debilitating complications such as fatigue, weakness, and pain. Because these disorders are rare, few healthcare professionals are proficient in their management; therefore the aim of these guidelines is to offer guidance on how to manage patients, and how to create survivorship care plans through the efforts of an interdisciplinary team.
Topics: Adult; Erdheim-Chester Disease; Histiocytosis, Langerhans-Cell; Histiocytosis, Sinus; Humans; Neoplasms; Prognosis; Survivorship
PubMed: 34781266
DOI: 10.6004/jnccn.2021.7096 -
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 -
Head and Neck Pathology Dec 2019Sinus histiocytosis with massive lymphadenopathy, or Rosai-Dorfman disease, is a rare, benign type of non-Langerhans cell histiocytosis. The radiological findings are...
Sinus histiocytosis with massive lymphadenopathy, or Rosai-Dorfman disease, is a rare, benign type of non-Langerhans cell histiocytosis. The radiological findings are often nonspecific, potentially mimicking malignancies. The diagnosis is ultimately made based on pathology, in which the lymph nodes are characterized by a dilated subcapsular sinus filled with histiocytes that can exhibit emperipolesis. Immunohistochemically, the histiocytes are variably CD68 positive and reliably negative for CD1a. The features of head and neck sinus histiocytosis with massive lymphadenopathy are exemplified in this radiology-pathology correlation sine qua non article.
Topics: Child; Histiocytosis, Sinus; Humans; Male
PubMed: 29855802
DOI: 10.1007/s12105-018-0941-3 -
Archives of Pathology & Laboratory... Mar 2003Context.-We investigated expression of the adhesion molecule CD31 in sinus histiocytosis with massive lymphadenopathy (SHML) and Langerhans cell histiocytosis (LCH)...
Context.-We investigated expression of the adhesion molecule CD31 in sinus histiocytosis with massive lymphadenopathy (SHML) and Langerhans cell histiocytosis (LCH) because (1) SHML and LCH cells express a variety of cellular adhesion molecules and (2) SHML has been characterized as a reactive histiocytic proliferation, and tissue macrophages (histiocytes) are known to express CD31. Objective.-The purpose of this study was to determine whether SHML and LCH cells express CD31 and whether dual staining with CD31 and S100 facilitates diagnosis of these disease states. Methods.-Formalin-fixed, paraffin-embedded archival tissues were immunohistochemically stained via the labeled streptavidin-biotin method using antibodies against CD31 and S100 protein after heat-induced epitope retrieval. Archival tissues included SHML (n = 2), LCH (n = 10), malignant melanoma (n = 5), sinus hyperplasia (n = 4), granulomas (n = 4), granular cell tumor (n = 6), and normal skin (n = 4). Results.-Normal Langerhans cells in the epidermis were CD31(-)/S100(+); neoplastic Langerhans cells in LCH were CD31(+)/S100(+). Histiocytes in granulomas and in sinus hyperplasia were CD31(+)/S100(-); abnormal histiocytes in SHML were CD31(+)/S100(+). S100(+) tumors (malignant melanoma and granular cell tumor) were CD31(-). Conclusions.-The spectrum of cell types that express CD31 is expanded to include SHML and LCH. We speculate that up-regulation of CD31 in neoplastic Langerhans cells contributes to the migratory capability of LCH cells. CD31 may be a useful nonlysosomal marker of macrophages and their neoplastic counterparts (true histiocytic sarcomas). An immunohistochemical staining panel that includes CD31 and S100 facilitates the diagnosis of SHML and LCH.
Topics: Cell Movement; Formaldehyde; Histiocytosis, Langerhans-Cell; Histiocytosis, Sinus; Humans; Immunohistochemistry; Lymphatic Diseases; Paraffin Embedding; Platelet Endothelial Cell Adhesion Molecule-1; S100 Proteins; Tissue Fixation
PubMed: 12653580
DOI: 10.5858/2003-127-0341-SHWMLA -
European Journal of Case Reports in... 2017Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare and benign cause of lymphadenopathy and was first described in 1969. This disease commonly presents in...
UNLABELLED
Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare and benign cause of lymphadenopathy and was first described in 1969. This disease commonly presents in children and young adults with supra-diaphragmatic lymphadenopathy mainly at cervical nodal sites, composed of a population of histiocytes. Since its description, over 400 cases have been described, but there are few reports in the literature on its association with lymphomas. This case report describes a case with co-existing SHML and anaplastic large cell lymphoma (ALCL). The diagnosis of SHML in our patient did not alter the clinical outcome and the patient responded well to treatment for ALCL. Clinicians should maintain a high index of suspicion in cases of infra-diaphragmatic SHML for the presence of occult lymphoma.
LEARNING POINTS
A rare presentation of sinus histiocytosis with massive lymphadenopathy (SHML) with anaplastic large cell lymphoma in the same lymph node at the same time is described.SHML has no proven clinical significance.A diagnosis of SHML in the presence of infra-diaphragmatic lymphadenopathy should raise a high index of suspicion for lymphoma.
PubMed: 30755945
DOI: 10.12890/2017_000605 -
The American Journal of Pathology Feb 1985The light-microscopic, ultrastructural, and immunohistochemical features of immature sinus histiocytosis were studied in 10 lymph nodes with the histologic picture of...
The light-microscopic, ultrastructural, and immunohistochemical features of immature sinus histiocytosis were studied in 10 lymph nodes with the histologic picture of toxoplasmic lymphadenitis and compared with the features of lymphoid cells present in the marginal zone of the splenic white pulp. Areas of immature sinus histiocytosis consisted largely of medium-sized lymphoid cells with markedly irregular nuclei and abundant pale cytoplasm. Using a panel of monoclonal antibodies, the predominating lymphoid cells were found to carry the B-cell phenotype B1+Ba1-sIgM+sIgD-OKIa1+. Admixed were variable numbers of larger, blastic lymphoid cells, small lymphocytes, histiocytic elements, and polymorphonuclear granulocytes. The marginal zone of the splenic white pulp was composed of a similar mixture of cells, and marginal-zone lymphocytes demonstrated an analogous immunohistochemical phenotype. Our results indicate that immature sinus histiocytes are B-lymphoid cells that are closely related to marginal zone lymphocytes. As such, immature sinus histiocytes may have a role similar to that of marginal-zone lymphocytes, which have been claimed to transport antigens or immune complexes toward the follicular center or to serve as precursors of plasma cells. We suggest that immature sinus histiocytosis represents an abnormal expansion of the marginal zone, normally present at the sinusoidal pole of lymphoid follicles. The reason for this marginal-zone hyperplasia, recognized as immature sinus histiocytosis in a variety of reactive lymph node conditions, may be a maturation arrest in the normal development of immature sinus histiocytes into small, sIgM+ sIgD+ lymphocytes.
Topics: Histiocytes; Histocytochemistry; Humans; Immunochemistry; Lymph Nodes; Lymphatic Diseases; Microscopy, Electron; Phenotype; Spleen
PubMed: 3970140
DOI: No ID Found -
Seminars in Diagnostic Pathology Mar 2016Various hematolymphoid lesions involve the sinonasal tract, including aggressive B, T, and NK-cell neoplasms; myeloid sarcoma; low-grade lymphomas; indolent... (Review)
Review
Various hematolymphoid lesions involve the sinonasal tract, including aggressive B, T, and NK-cell neoplasms; myeloid sarcoma; low-grade lymphomas; indolent T-lymphoblastic proliferations; and Rosai-Dorfman disease. Differentiating aggressive lymphomas from non-hematopoietic neoplasms such as poorly differentiated squamous cell carcinoma, olfactory neuroblastoma, or sinonasal undifferentiated carcinoma may pose diagnostic challenges. In addition, the necrosis, vascular damage, and inflammatory infiltrates that are associated with some hematolymphoid disorders can result in misdiagnosis as infectious, autoimmune, or inflammatory conditions. Here, we review hematolymphoid disorders involving the sinonasal tract including their key clinical and histopathologic features.
Topics: Biomarkers, Tumor; Biopsy; Histiocytosis, Sinus; Humans; Immunohistochemistry; Leukemia-Lymphoma, Adult T-Cell; Lymphoma; Nasal Cavity; Nose Neoplasms; Paranasal Sinus Neoplasms; Prognosis
PubMed: 26472692
DOI: 10.1053/j.semdp.2015.09.008 -
Head and Neck Pathology Mar 2016Hematolymphoid neoplasms of the sinonasal tract are rare and the majority represents non-Hodgkin lymphomas. This review will focus on morphologic, immunophenotypic, and... (Review)
Review
Hematolymphoid neoplasms of the sinonasal tract are rare and the majority represents non-Hodgkin lymphomas. This review will focus on morphologic, immunophenotypic, and genetic characteristics of the most common types of non-Hodgkin lymphoma, namely diffuse large B cell lymphoma and extranodal natural killer/T-cell lymphoma, nasal type, but also include the discussion of less frequent other hematolymphoid entities, such as extranodal plasmacytomas and Rosai-Dorfman disease.
Topics: Histiocytosis, Sinus; Humans; Lymphoma, Non-Hodgkin; Nose Diseases; Paranasal Sinus Neoplasms; Plasmacytoma
PubMed: 26830397
DOI: 10.1007/s12105-016-0698-5 -
Reviews in Urology 2019This case explores a rare initial presentation of Rosai-Dorfman disease isolated to the peri-renal space. Also described as sinus histiocytosis with massive... (Review)
Review
This case explores a rare initial presentation of Rosai-Dorfman disease isolated to the peri-renal space. Also described as sinus histiocytosis with massive lymphadenopathy, Rosai-Dorfman disease is non-neoplastic and most often presents with massive cervical lymphadenopathy, but the disease can affect any organ system. Not often considered by those in the urology community or found in urology journals, this report reviews a clinical presentation of Rosai-Dorfman disease affecting bilateral kidneys and the fundamental histopathology needed for its diagnosis.
PubMed: 31239831
DOI: No ID Found