Castleman Disease and Rosai-Dorfman Disease

Miguel A Piris1, Elena Aguirregoicoa1, Santiago Montes-Moreno2

  • 1Pathology Service, Fundación Jiménez Díaz, CIBERONC, Avenida Reyes Católicos, 2, 28040 Madrid, Spain.

Insights

Castleman Disease (CD) and Rosai-Dorfmann Disease (RDD) are distinct lymphoproliferative and histiocytic disorders, respectively. While CD presents heterogeneously, RDD involves specific histiocyte infiltration and may represent a neoplastic process.

Area of Science:

  • Hematology
  • Oncology
  • Pathology

Background:

  • Castleman Disease (CD) is a heterogeneous lymphoproliferative disorder with overlapping features.
  • Rosai-Dorfmann Disease (RDD) is a histiocytic proliferative disorder characterized by specific cellular infiltrates.
  • Both conditions require careful histopathological differentiation.

Purpose of the Study:

  • To delineate the distinct features of Castleman Disease (CD) and Rosai-Dorfmann Disease (RDD).
  • To highlight the diagnostic criteria and potential underlying mechanisms of CD and RDD.

Main Methods:

  • Review of clinical and histopathological characteristics of CD and RDD.
  • Discussion of diagnostic markers such as S100 and CD1a for RDD.
  • Exploration of molecular findings suggesting neoplastic potential in RDD.

Main Results:

  • CD encompasses Hyaline Vascular CD (HVCD), Unicentric Plasma Cell CD, and Multicentric CD, each with unique morphological and clinical aspects.
  • HVCD involves abnormal germinal centers and follicular dendritic cell changes.
  • Multicentric CD is associated with proinflammatory hypercytokinemia and florid presentation.
  • RDD diagnosis relies on S100-positive, CD1a-negative histiocytes, plasma cell aggregates, and emperipolesis.
  • Molecular data suggest RDD may be a low-grade neoplastic process in some cases.

Conclusions:

  • CD and RDD are distinct entities with specific diagnostic features.
  • Understanding these differences is crucial for accurate diagnosis and management.
  • Further research into RDD's etiology may reveal neoplastic origins.

Related Concept Videos

Rous Sarcoma Virus (RSV) and Cancer01:03

Rous Sarcoma Virus (RSV) and Cancer

Rous Sarcoma virus or RSV was discovered by F. Peyton Rous in the year 1911 as a filterable transmissible agent that could cause tumors in chickens. He won a Nobel Prize for this discovery in 1966. His experiments clearly demonstrated that some cancers could be caused by infectious agents and led to the discovery of many more cancer-causing viruses in animals as well as humans.
RSV is a retrovirus that contains two copies of a plus-strand  RNA genome. Its genome consists of four main open...
6.4K
Cardiomyopathy I: Introduction and Classification01:25

Cardiomyopathy I: Introduction and Classification

Cardiomyopathy, or CMP, is a group of diseases affecting the myocardial structure, impairing its ability to pump blood effectively. This condition can lead to arrhythmias, heart failure, or sudden cardiac death.Cardiomyopathies are classified into primary and secondary categories:Primary Cardiomyopathy refers to conditions involving only the heart muscle that are often idiopathic (of unknown cause) or genetic. They primarily affect the myocardium without the involvement of other systemic...
638
Abnormal Proliferation02:23

Abnormal Proliferation

Under normal conditions, most adult cells remain in a non-proliferative state unless stimulated by internal or external factors to replace lost cells. Abnormal cell proliferation is a condition in which the cell's growth exceeds and is uncoordinated with normal cells. In such situations, cell division persists in the same excessive manner even after cessation of the stimuli, leading to persistent tumors. The tumor arises from the damaged cells that replicate to pass the damage to the...
5.3K
Cardiomyopathy IV: Restrictive Cardiomyopathy01:29

Cardiomyopathy IV: Restrictive Cardiomyopathy

Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...
611
mTOR Signaling and Cancer Progression03:03

mTOR Signaling and Cancer Progression

The mammalian target of rapamycin or mTOR protein was discovered in 1994 due to its direct interaction with rapamycin. The protein gets its name from a yeast homolog called TOR. The mTOR protein complex in mammalian cells plays a major role in balancing anabolic processes such as the synthesis of proteins, lipids, and nucleotides and catabolic processes, such as autophagy in response to environmental cues, such as availability of nutrients and growth factors.
The mTOR pathway or the...
4.9K
Rheumatic Heart Disease I: Introduction01:23

Rheumatic Heart Disease I: Introduction

Rheumatic heart disease or RHD is a chronic condition that results from rheumatic fever, causing permanent damage to the heart valves.Etiology and Risk FactorsIt primarily arises from rheumatic fever, an inflammatory disease that can develop after untreated or inadequately treated group A streptococcal (GAS) pharyngitis. Streptococcus spreads through direct contact with oral or respiratory secretions. While the bacteria are the causative agents, factors like malnutrition, overcrowding, poor...
581