ABSTRACT
OBJECTIVE: The authors investigated the expression of IgG4 and IgG in cutaneous Rosai-Dorfman Disease (CRDD) to further improve the understanding of this disease. METHODS: The authors retrospectively reviewed the clinicopathological features of 23 CRDD patients. The authors diagnosed CRDD by the presence of emperipolesis and immunohistochemical (IHC) staining of histiocytes consisting of S-100(+)/CD68(+)/CD1a(-) cells. The expressions of IgG and IgG4 in cutaneous specimens were assessed by IHC (EnVision) and quantitatively calculated by a medical image analysis system. RESULTS: All 23 patients, including 14 males and 9 females, were confirmed to have CRDD. Their ages ranged from 17 to 68 years (mean 47.91 ± 14.16). The most frequently affected skin regions were the face, followed by the trunk, ears, neck, limbs, and genitals. In 16 of these cases, the disease presented as a single lesion. IHC staining of sections showed that IgG was positive (≥ 10 cells/High-Power Field [HPF]) in 22 cases, while IgG4 was positive (≥ 10 cells/HPF) in 18 cases. Moreover, the IgG4/IgG proportion ranged from 1.7% to 85.7% (mean 29.50 ± 24.67%, median 18.4%) in the 18 cases. STUDY LIMITATIONS: In the majority of studies, as well as in the current study, the design. RDD is a rare disease, so the sample size is small. In the next studies to come, the authors will expand the sample for multi-center verification and in-depth study. CONCLUSION: The positive rates of IgG4 and IgG and the IgG4/IgG ratio assessed through IHC staining may be important in understanding the pathogenesis of CRDD.
Subject(s)
Histiocytosis, Sinus , Skin Diseases , Male , Female , Humans , Adolescent , Young Adult , Adult , Middle Aged , Aged , Histiocytosis, Sinus/diagnosis , Immunoglobulin G/metabolism , Retrospective Studies , Skin Diseases/pathology , Histiocytes , S100 ProteinsABSTRACT
The cutaneous form of Rosai-Dorfman disease is very rare in childhood. The clinical spectrum is highly variable and histopathological study with immunohistochemistry is essential for the diagnosis. We present the case of a 3-year-old boy with the diagnosis of cutaneous Rosai-Dorfman disease and review the pediatric cases published in the literature.
Subject(s)
Histiocytosis, Sinus , Skin Diseases , Male , Humans , Child , Child, Preschool , Histiocytosis, Sinus/diagnosis , Histiocytosis, Sinus/pathology , Skin Diseases/diagnosis , Skin Diseases/pathology , Skin/pathology , ImmunohistochemistrySubject(s)
Breast Diseases/diagnosis , Breast/pathology , Histiocytosis, Sinus , Image-Guided Biopsy/methods , Ultrasonography, Mammary/methods , Breast Neoplasms/diagnosis , Diagnosis, Differential , Female , Histiocytes/pathology , Histiocytosis, Sinus/diagnosis , Histiocytosis, Sinus/immunology , Humans , Immunohistochemistry , Middle AgedABSTRACT
RESUMEN La enfermedad de Rosai-Dorfman (ERD) o histiocitosis sinusal con linfadenopatía masiva es una enfermedad infrecuente, de etiología desconocida caracterizada por linfadenopatías cervicales masivas bilaterales. El compromiso extraganglionar puede ocurrir en diferentes sitios, incluida la vía aérea, en la cual la localización nasosinusal es la más frecuente, pero puede comprometer también otros sitios de la vía aérea superior. Dentro de éstos, el compromiso laríngeo es muy poco frecuente. Se presenta el caso de una paciente de 82 años con antecedentes de ERD localizada en los ganglios linfáticos cervicales en remisión espontánea, que desarrolló posteriormente una obstrucción subaguda de las vías respiratorias. La tomografía computarizada y la nasofaringolaringoscopía mostraron lesiones subglóticas que obstruían severamente las vías respiratorias. Se realizó una traqueostomía de emergencia y biopsia de las lesiones, confirmando el diagnóstico de ERD extranodal.
ABSTRACT Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy is a rare disease of unknown etiology characterized by bilateral massive cervical lymphadenopathy. Extranodal involvement can occur in different sites including airway where nasosinusal involvement is common but this disease may occur in other sites of the upper airway. Laryngeal involvement is rare. We present the case of an 82-year-old female patient with a history of Rosai-Dorfman disease located in cervical lymph nodes in spontaneous remission that subsequently develops airway obstruction. Computed tomographic scan and nasopharyngolaryngoscopy showed subglottic lessons that severely obstructed the airway. Emergency tracheotomy and biopsy were performed, confirming the diagnosis of extranodal RDD. Patient was observed and tracheostomy was maintained with good tolerance.
Subject(s)
Humans , Female , Aged, 80 and over , Histiocytosis, Sinus/diagnosis , Laryngeal Diseases/diagnosis , Airway Obstruction/surgery , Tracheostomy , Tomography, X-Ray Computed , Histiocytosis, Sinus/surgery , Laryngeal Diseases/surgery , LaryngostenosisABSTRACT
Sinus histiocytosis with massive lymphadenopathy, generally known by the name of Rosai-Dorfman disease is a rare benign condition principally affecting cervical lymph nodes. Concurrent extra-nodal disease frequently occurs, however, solitary extra-nodal disease involving the mandible is exceedingly rare with less than five reported cases in the English literature. We describe a case of primary involvement of the mandible in a 27-year-old female, and discuss the differential diagnosis of this disease with other histiocytic lesions.
Subject(s)
Humans , Female , Adult , Histiocytosis, Sinus/diagnosis , Mandible/pathology , Diagnosis, Differential , Erdheim-Chester Disease , Histiocytosis, Langerhans-Cell , Histiocytosis, Sinus/complications , Rare DiseasesABSTRACT
A 75-year-old male presented with generalized erythematous, scaly plaques and painless lymphadenopathy. Rosai-Dorfman disease was suspected based on clinical manifestations and confirmed by histopathologic and immune reactivity studies performed on the biopsy obtained from the left supraclavicular lymph node. The patient was also diagnosed with multiple myeloma according to urine electrophoresis, serum light chain assay, and bone marrow biopsy, which were initially performed for evaluation of anemia. This report highlights the dermatological manifestations of Rosai-Dorfman disease with generalized painless lymphadenopathy.
Subject(s)
Histiocytosis, Sinus/pathology , Multiple Myeloma/pathology , Aged , Biopsy , Histiocytosis, Sinus/diagnosis , Humans , Male , Multiple Myeloma/diagnosis , Skin/pathologyABSTRACT
Abstract: A 75-year-old male presented with generalized erythematous, scaly plaques and painless lymphadenopathy. Rosai-Dorfman disease was suspected based on clinical manifestations and confirmed by histopathologic and immune reactivity studies performed on the biopsy obtained from the left supraclavicular lymph node. The patient was also diagnosed with multiple myeloma according to urine electrophoresis, serum light chain assay, and bone marrow biopsy, which were initially performed for evaluation of anemia. This report highlights the dermatological manifestations of Rosai-Dorfman disease with generalized painless lymphadenopathy.
Subject(s)
Humans , Male , Aged , Histiocytosis, Sinus/pathology , Multiple Myeloma/pathology , Skin/pathology , Biopsy , Histiocytosis, Sinus/diagnosis , Multiple Myeloma/diagnosisABSTRACT
Resumen La enfermedad de Rosai Dorfman es una entidad rara caracterizada por linfadenopatías no dolorosas de predominio cervical y submandibular. En el pasado se había considerado como una reticuloendoteliosis maligna, hasta que fue descrita como una entidad clinico-patológica, aislada en 1969 por Rosai y Dorfman. Se presenta el caso de una niña de 4 años de edad, conocida sana, con un cuadro agudo de sensación febril, masa cervical y leucocitosis. Los estudios de laboratorio no identificaron malignidad, ni infección como etiología. Recibió tratamiento con antibióticos sin resolución de su adenopatía, lo cual motivó a que se efectuara una biopsia excisional. El estudio histológico reveló una histiocitosis sinusal con linfoadenopatía masiva. Se decidió dar manejo conservador ya que tuvo una resección completa.
Abstract Rosai Dorfman's disease is a rare entity characterized by non-painful lymphadenopathies predominantly cervical and submandibular. In the past it had been considered a malignant reticuloendotheliosis, until it was described as a clinical-pathological entity, isolated in 1969 by Rosai and Dorfman. We present the case of a 4-year-old girl, known to be healthy, with an acute condition of febrile sensation, cervical mass and leukocytosis. Laboratory studies did not identify malignancy or infection as an etiology. She was treated with antibiotics without resolution of his adenopathy, which led to an excisional biopsy. The histological study revealed a sinus histiocytosis with massive lymphadenopathy. It was decided to give conservative management since it had a complete resection.
Subject(s)
Humans , Female , Child, Preschool , Histiocytosis, Sinus/diagnosis , Histiocytosis, Sinus/diagnostic imaging , Costa RicaABSTRACT
Rosai-Dorfman disease is a benign lymphoproliferative disease with rare occurrence in the overall population. The characteristic findings of the disease are emperipolesis on microscopy and S-100 protein positivity on immunohistochemistry. The present report describes the case of a 57-year-old man who presented with repeated seizures, urinary retention, and ataxia. Satisfactory results were obtained with surgical treatment. The diagnosis was confirmed by immunohistochemistry.
Subject(s)
Humans , Male , Middle Aged , Histiocytosis, Sinus/surgery , Meningeal Neoplasms/surgery , Meningioma/surgery , Immunohistochemistry , Histiocytosis, Sinus/diagnosis , Diagnosis, Differential , Meningeal Neoplasms/diagnosis , Meningioma/diagnosis , MicroscopyABSTRACT
La enfermedad de Rosai-Dorfman; también conocida como linfadenopatía masiva con histiocitosis sinusal; es un raro desorden de etiología desconocida; generalmente asociado a un agrandamiento de los ganglios linfáticos superficiales y/o profundos. La mayoría de los pacientes no requiere tratamiento. Presentamos el caso de un niño de 10 meses de vida, que ingresa con una tumoración cervical izquierda de 25 días de evolución.
Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare dis order of unknown etiology, generally associated with enlargement of superficial and/or deep lymph nodes. Most patients do not require treatment. We report the case of a 10 month old infant who was admitted with a left cervical tumor of 25 days duration.
Subject(s)
Humans , Male , Infant , Histiocytosis, Sinus/diagnosis , Uterine Cervical Neoplasms/diagnosis , Hypertrophy , Lymph Nodes/pathologyABSTRACT
Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare dis order of unknown etiology, generally associated with enlargement of superficial and/or deep lymph nodes. Most patients do not require treatment. We report the case of a 10 month old infant who was admitted with a left cervical tumor of 25 days duration.
Subject(s)
Histiocytosis, Sinus/diagnosis , Uterine Cervical Neoplasms/diagnosis , Female , Humans , Hypertrophy , Infant , Lymph Nodes/pathologyABSTRACT
Background: Rosai Dorfman disease is uncommon and consists in a benign fibrous and hematopoietic tissue proliferation, usually located in the head or neck. It is self-limited and the treatment is the surgical excision of the lesion. Case Report: We report a 69 years old woman consulting for a palpable right breast nodule with a mammography and mammary ultrasound informed as BIRADS 4 and 5, respectively. A core biopsy was informed as a mammary Rosai Dorfman disease. Two years later, due to a lesion growth, the patient was subjected to a partial mastectomy. The pathological study of the surgical piece confirmed the diagnosis of the core biopsy. This disease may resemble a malignant disease and the clue for its diagnosis is the pathological study.
Introducción: La enfermedad de Rosai Dorfman es una entidad infrecuente, que consiste en la proliferación benigna del tejido fibroso y hematopoyético ubicado generalmente en cabeza y cuello. Se trata de una enfermedad benigna, autolimitada, cuyo tratamiento generalmente se limita a la resección quirúrgica, con elevada tasa de recidiva. Objetivo: Exposición de un caso de Enfermedad de Rosai Dorfman mamario, inicial-mente sugerente de patología maligna. caso clínico: Mujer 69 años derivada de atención primaria por nódulo palpable en mama derecha, mamografía y ecografía mamaria BIRADS 4 y 5, respectivamente. Biopsia Core informa Enfermedad de Rosai Dorfman mamaria. A los dos años de diagnóstico se objetiva lesión de mayor tamaño. Se realizó mastectomía parcial con biopsia de pieza quirúrgica compatible con diagnóstico de Biopsia Core previa. Discusión: La similitud clínica de esta enfermedad con patologías malignas mamarias, asociado a su baja prevalencia, dificultan la pesquisa de casos como el expuesto. En los estudios de imágenes raramente se sospecha. La clave del diagnóstico son los hallazgos histológicos en el que característicamente se observa emperipolesis con tinciones inmunohistoquímicas positivas para S 100 y negativo para CD 1. Dado que es una enfermedad benigna y autolimitada su tratamiento generalmente se limita a la resección quirúrgica. Este permite extirpar el tumor en estudio, realizar el diagnóstico, y aliviar la sintomatología producida por el efecto masa de la lesión. A pesar de lo anterior la recidiva es alta, por lo que el seguimiento debe ser a largo plazo.
Subject(s)
Humans , Female , Aged , Breast Diseases/surgery , Breast Diseases/diagnosis , Histiocytosis, Sinus/surgery , Histiocytosis, Sinus/diagnosis , Diagnosis, Differential , Mastectomy, Segmental , Breast Neoplasms/diagnosisABSTRACT
BACKGROUND: Adrenal gland involvement is a very rare extranodal presentation of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Its pathogenesis still remains unknown with many theories about its probable cause. Symptoms and signs of adrenal involvement presentation are uncertain. Although, it is a disease with a good prognosis and a favorable clinical course in most cases, the low index of suspicion and difficulty in radiological differential diagnosis lead to radical treatment. CLINICAL CASE: We present a case of Rosai-Dorfman disease with right adrenal plus left infrahiliar nodal involvement. It was treated with adrenalectomy and node exeresis. The infrahilial involvement evolution was appropriate. CONCLUSIONS: Rosai-Dorfman disease is a rare disease and the extra-nodal presentation is even more rare. The incidence and evolution of this disease in the adrenal gland is unknown. The presence of this pathology should be considered if the patient shows adrenal gland affected and lymph node growth, in order to consider the individualized treatment, which may vary (treatment with corticosteroids, chemotherapy and/or radiotherapy).
INTRODUCCIÓN: la afectación extranodal de la glándula suprarrenal es una forma infrecuente de la histiocitosis sinusal con linfadenopatía masiva o enfermedad de Rosai-Dorfman. Aún se desconoce la etiopatogenia de esta enfermedad. Los síntomas y signos de presentación en la afectación adrenal son inciertos. Aunque es una enfermedad de buen pronóstico y curso clínico favorable, el bajo índice de sospecha y el difícil diagnóstico radiológico diferencial llevan a tratamiento radical en la mayoría de los casos. CASO CLÍNICO: se describe el caso de una paciente con enfermedad de Rosai-Dorfman en quien se identificó tumor adrenal derecho de 97 × 99 × 68 mm y tumor nodal infrahiliar izquierdo de 61 × 58 × 57 mm. El tratamiento incluyó adrenalectomía y vigilancia activa del tumor infrahilial mediante tomografía y gammagrama renal. La evolución de la paciente fue adecuada. CONCLUSIONES: la enfermedad de Rosai-Dorfman es una patología poco común y su presentación extranodal es todavía más extraña. La incidencia y evolución de la enfermedad en glándula suprarrenal se desconocen. Esta patología debe ser considerada en glándula suprarrenal cuando hay afección adrenal y crecimiento nodal, para plantear el tratamiento individualizado que puede variar entre vigilancia activa, tratamiento con corticosteroides, quimioterapia y radioterapia.
Subject(s)
Adrenal Gland Diseases/etiology , Histiocytosis, Sinus/complications , Female , Histiocytosis, Sinus/diagnosis , Humans , Middle AgedSubject(s)
Histiocytosis, Sinus/diagnosis , Lymphadenitis/diagnosis , Diagnosis, Differential , Humans , Infant , Male , NeckABSTRACT
A previously healthy 3-year-old girl developed rhinorrhea, painful right neck swelling, and fever. Contrast-enhanced CT of the neck revealed cervical lymphadenopathy, for which lymphoma was considered. FDG PET/CT images showed intense activity not only in the neck but also in the thorax, abdomen, and pelvis. Biopsy was performed, and the diagnosis of Rosai-Dorfman disease was made.
Subject(s)
Fluorodeoxyglucose F18 , Histiocytosis, Sinus/diagnosis , Lymphoma/diagnosis , Multimodal Imaging , Positron-Emission Tomography , Tomography, X-Ray Computed , Child, Preschool , Diagnosis, Differential , Female , Histiocytosis, Sinus/diagnostic imaging , Humans , Lymphoma/diagnostic imagingABSTRACT
Se presenta una paciente de 50 años de edad con lesión tumoral redondeada, no dolorosa en región inguinal, con crecimiento progresivo y formación de una placa eritematosa, con áreas de pigmentación e infiltración de tejidos profundos, así como aparición de otras lesiones de aspecto tumoral. El estudio anatomopatológico evidenció un infiltrado histiocitario con fenómenos de linfohistiofagocitosis (emperipolesis) y el inmunohistoquímico mostró positividad para vimentina, S-100, CD68 y lisozima. Se revisó la evidencia científica disponible en la literatura mundial para determinar las manifestaciones clínicas más frecuentes, así como los medios diagnósticos más empleados en la actualidad y el tratamiento de esta enfermedad. Es importante considerar a la enfermedad de Rosai-Dorfman cutánea como un posible diagnóstico para lo que es indispensable un profundo estudio de la biopsia, así como la realización de estudios de inmunohistoquímica, dada la benignidad y buen pronóstico que la caracterizan(AU)
A 50 year-old patient with a painless round tumoral lesion on inguinal region with progressive growth and formation of an eritematous plaque with areas of pigmentation, infiltration of deep tissues and apparition of other lesions with tumoral aspect is described. The anatomopathological study showed a hystiocitic infiltrate with linfohystiofagocitosis phenomenon (Emperipolesis) and the immunohystochemical made evident positive for Vimentine, S-100, CD68 and Lisozime. The available scientific evidence of the international literature was reviewed to determine the most frequent clinical manifestations of this disease, as well as most updated means of diagnosis employed and the treatment of this disease. It is important to consider the cutaneous Rosai-Dorfman disease as one of the possible diagnosis so a profound study of the biopsy is essential, as well as the immunohystochemical studies due to the mildness and good prognosis of this disease(AU)