Multifocal, Extranodal Sinus Histiocytosis With Massive Lymphadenopathy: An Overview

Abstract

Context.—This article provides an overview of the major pathologic manifestations of sinus histiocytosis with massive lymphadenopathy, including patient characteristics and current knowledge about its pathogenesis, with an emphasis on multifocal and extranodal presentation. Sinus histiocytosis with massive lymphadenopathy is a rare, nonneoplastic, idiopathic, proliferative histiocytic disorder; recognition of this disorder is important to avoid misinterpretation and subsequent unnecessary treatment. This is especially true for primary extranodal manifestation of this rare disorder. Although accurate diagnosis of this entity requires a correlation of clinical, radiologic, laboratory, and pathologic studies in most cases, it remains a disorder primarily defined by its histopathologic features and pathologic manifestations, which are key to the diagnosis. Objective.—To summarize the scientific literature, provide a concise review, and emphasize the diagnostic histopathologic features of extranodal sinus histiocytosis with massive lymphadenopathy. Data Sources.—A comprehensive literature review was undertaken to summarize the clinical and pathologic features of this disorder. Conclusions.—Sinus histiocytosis with massive lymphadenopathy is characterized by a rare, acquired, nonmalignant proliferation of distinctive histiocytes that present with lymphadenopathy or extranodal disease, primarily in children and young adults. It exhibits a broad range of clinical presentations, thus eliciting a wide differential diagnosis. The diverse clinical manifestations and frequent association with subtle or severe immunologic abnormalities suggest an immune-mediated cause. Additional studies are needed to characterize the interplay between death receptors and cytotoxic mediators and to further elucidate the loss of immune hemostasis that may underlie idiopathic histiocytic proliferations such as this.

Rosai-Dorfman Disease Presenting with Solitary Liver Mass Without Lymphadenopathy: Case Report and Literature Review

“…Extranodal involvement is common and may occur in more than 40% of patients, sometimes without associated lymphadenopathy, and only 23% of patients have exclusive extranodal disease 13,23,24 . Virtually every organ can be affected by RDD (respiratory, bone, CNS, genitourinary, orbit, soft tissue, visceral organs, nasal cavity, etc.)…”

Extranodal Rosai–Dorfman Disease Arising in the Right Atrium

“…2 Irrespective of concurrent lymph node involvement, the most frequent extranodal sites in decreasing order are skin and soft tissue (16%); nasal cavity and paranasal sinuses (16%); eye, orbit, and ocular adnexa (11%); bone (11%); salivary gland (7%); central nervous system (7 %); oral cavity (4%); kidney and genitourinary tract (3%); respiratory tract (3%); liver (1%); tonsil (1%); breast (<1%); gastrointestinal tract (<1%); and heart (<1%). 2 The histologic hallmark of RDD is a large, polygonal histiocyte with a round nucleus, vesicular chromatin, fine nuclear membrane, one to several eosinophilic nucleoli and abundant pale cytoplasm. 2 Most times the histiocytes show emperipolesis, a phenomenon characterized by the presence of lymphocytes or other inflammatory cells within the histiocytic cytoplasm of other cells.…”

“…2 The histologic hallmark of RDD is a large, polygonal histiocyte with a round nucleus, vesicular chromatin, fine nuclear membrane, one to several eosinophilic nucleoli and abundant pale cytoplasm. 2 Most times the histiocytes show emperipolesis, a phenomenon characterized by the presence of lymphocytes or other inflammatory cells within the histiocytic cytoplasm of other cells. Emperipolesis is often more difficult to appreciate in extranodal lesions than in lymph nodes.…”

“…The involvement of an extranodal site in association with lymphadenopathy is present in approximately 43% of cases and in only 23% does isolated extranodal RDD occur. 2 A search of the literature revealed 3 prior reports of RDD specifically arising in the right atrium. 3,4 Herein, we present a case of RDD with massive right atrial involvement causing severe hypotension.…”

Extranodal Rosai-Dorfman-Destombes disease mimicking renal cell carcinoma: a case report and review of literature

“…4 Less than 5% of patients with RDD have renal involvement, whereas isolated localization of RDD as a renal mass is very rare. 5 and nausea. He had no other complaints, no known comorbidities, no significant past history and no prior surgeries.…”