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Background: Rosai-Dorfman disease (RDD) is a rare type of histiocytosis that can manifest with diverse symptoms. It usually presents with systemic involvement, and only a few cases have been reported at the level of the skull base. RDD typically follows a benign course during the progression of the disease. In this particular case reported, after the skull base invasion, the disease started to infiltrate the brain parenchyma. Our objective for this case report was to present this particular progression pattern and the nuances of its surgical treatment. In addition, a revision of the current literature was performed about skull base RDD with intracranial invasion and brain parenchyma infiltration not previously described. Case Description: We are presenting the case study of a 57-year-old male patient who was experiencing severe headaches and an increase in volume in the right fronto-orbital region. On clinical examination, no neurologic clinical symptoms were observed. Contrast computed tomography and magnetic resonance imaging showed a tumor mass that affected the right orbit, frontal paranasal sinus, greater sphenoid wing, and right frontal lobe with moderate adjacent brain edema. The patient underwent surgery using an extended pterional approach with intracranial, orbital decompression, and frontal sinus cranialization, accompanied by frontal lobe tumor resection. Neuropathologic diagnosis revealed a Rosai-Dorfman histiocytosis disease. Conclusion: The etiopathogenesis of RDD is still not completely understood. The current literature considers this disease to have a predominantly benign course. Nevertheless, as we have shown in this case, it may, in some cases, present direct parenchymal invasion. We consider that prompt surgical treatment should be ideal to avoid the local and systemic progression of the disease.

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Rosai-Dorfman Disease (RDD) is a rare non-Langerhans histiocytosis, usually self-limited and presenting with massive, painless, bilateral cervical lymphadenopathy, with or without constitutional symptoms. Extranodal disease is frequently present, and may happen in the absence of lymph node involvement, symptomatology and differential diagnosis will depend on the site affected and fatal cases may occur. The authors present two cases of Rosai-Dorfman disease (RDD), diagnosed through immunohistochemistry, with different progressions, one with complete remission and one culminating in death, highlighting the variety of presentations and the diagnostic difficulty. RDD is a rare condition with clinical presentations similar to several diseases, and should be considered in the differential diagnosis of lymphadenopathy with extranodal lesions.

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RESUMEN La enfermedad de Rosai-Dorfman cutánea (ERDC) es una rara proliferación reactiva de histiocitos, que cursa con lesiones cutáneas sin compromiso generalizado. Las manifestaciones clínicas cutáneas son diversas y la variante clínica "tipo tumoración" es la forma más infrecuente. Se describe el caso de un paciente con ERDC que se presentó con esta forma inusual.

ABSTRACT Cutaneous Rosai-Dorfman disease (CRDD) is a rare reactive histiocytic proliferation, which presents with skin lesions without systemic involvement. The clinical cutaneous manifestations are diverse and the "tumor-like" clinical variant is the most infrequent. We describe the case of a patient with CRDD who presented with this unusual form.

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Rosai Dorfman Destombes (RDD) disease is a non-Langerhans histiocytosis. The central nervous system is affected in < 5% of cases. We report the case of a 59-year-old man, who began 8 months before admission with headache, diminished visual acuity in the temporal hemifields, hyposmia, and seizures. Magnetic resonance imaging showed three midline skull-base lesions in anterior, media, and posterior fossae. We performed a complete resection of symptomatic lesions using a bifrontal craniotomy. The histopathological analysis determined RDD, therefore, we started steroid treatment. Our case description is due to the diagnosis and location, one of the rarest reported to date in the literature.

La enfermedad de Rosai-Dorfman-Destombes (RDD) es una histiocitosis no Langerhans. El SNC se ve afectado en menos del 5% de los casos. Presentamos el caso de un hombre de 59 años quien inició ocho meses previos al ingreso con cefalea, hemianopsia bitemporal, hiposmia y convulsiones. La resonancia magnética mostró tres lesiones de la base del cráneo en las fosas anterior, media y posterior. Realizamos una resección completa de las lesiones sintomáticas mediante una craneotomía bifrontal. El análisis histopatológico determinó RDD. Nuestro caso es debido al diagnóstico y localización, uno de los más raros reportados hasta la fecha en la literatura.

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PURPOSE: To describe the clinical features and histopathologic findings of Rosai-Dorfman disease (RDD) with ophthalmic involvement diagnosed at a Mexican ophthalmologic referral center in a period of 62 years. RESULTS: A total of 17 cases of RDD with ophthalmologic manifestations in Mexican-mestizo patients were collected. Female predominance was observed in 12 of 17 patients. The mean age was 36 years with a range of 12-76 years. The median age at presentation was 32 years. The orbit was the most affected location with 15 cases. Conjunctival disease was seen in 4 cases: two as isolated lesions, the rest with orbital or uveal involvement . One case suggested choroidal RDD associated to conjunctival and orbital lesion. Three cases presented multifocal disease. A patient with orbital affection developed extranodal disease elsewhere. No concurrent lymphadenopathy was observed in any patient.Histopathological examination revealed classic RDD findings in all cases: A polymorphous cellular infiltrate composed by lymphocytes, plasma cells and large histiocytes with emperipolesis hallmark of the disease. Immunohistochemically, histiocytes were positive for S-100 protein and CD68 in 15 cases. CONCLUSION: RDD of the eye and ocular adnexa in Mexican-mestizo patients is common in middle-aged women frequently as an isolated orbital lesion. Histopathology is necessary for accurate diagnosis. Most patients had a favourable outcome after medical and surgical treatment. To the best of our knowledge this is the largest case series of RDD of the eye and ocular adnexa in Latin-American patients.

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The case is presented on a 52-year-old male patient, who was seen in the Rheumatology department. He had painless lymph nodes in the cervical, axillary, supraclavicular, and neck region. He also had a fever, and parotid and submaxillary gland enlargement. Complementary studies were performed, showing normocytic-normochromic anemia, thrombocytopenia and eosinophilia, impaired renal function with hypoalbuminaemia and hematuria, ANA 1/5120, Sm+, ACL+. Biopsies were also performed on the compromised tissues, reaching the diagnosis of Rosai-Dorfman Disease and IgG4-related Disease. Differential diagnoses of cervical, axillary and inguinal lymph nodes, with fever, renal and hematological compromise are discussed.

Se describe el caso de un paciente varón de 52 años que consulta al servicio de reumatología por presentar adenopatías indoloras en las regiones cervical, axilar, supraclaviculares y en la nuca, así como fiebre, aumento de tamaño de parótidas y submaxilares. Se realizan estudios complementarios que arrojan como resultado anemia normocítica-normocrómica, trombocitopenia y eosinofilia, alteración de la función renal con hipoalbuminemia y hematuria, FAN 1/5.120, Sm+, ACL+ y biopsia de los tejidos comprometidos, por lo que se arriba al diagnóstico de enfermedad de Rosai-Dorfman y enfermedad relacionada con IgG4. Se discuten diagnósticos diferenciales de adenopatías cervicales, axilares e inguinales, fiebre, compromiso renal y hematológico.

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RESUMEN Introducción: La enfermedad de Rosai-Dorfman es una histiocitosis linfática masiva caracterizada por emperipolesis con inmunohistoquímica positiva para S100 y CD68. Es una entidad clínica rara y de curso benigno más comúnmente presentada en varones. Objetivo: El propósito de este caso clínico es demostrar el tratamiento exitoso de la enfermedad de Rosai-Dorfman con inmunomoduladores y quimioterapia metronómica. Presentación del caso: Paciente masculino de 57 años que acude por linfadenopatía cervical bilateral, a quien se le realiza tomografía y biopsia ganglionar con resultados positivos para S100 y CD68 con marcado fenómeno de emperipolesis. Recibió esquema inmunomodulador y quimioterapia metronómica exitosa con remisión de enfermedad. Conclusiones: Los pacientes diagnosticados con Rosai-Dorfman son muy pocos debido a lo inusual de esta entidad clínica. Es importante mencionar que esta enfermedad es una histiocitosis de células tipo No Langerhans con características de benignidad y buena respuesta al manejo con corticoides y quimioterapia metronómica, terapia instaurada en nuestro paciente con buena evolución.

ABSTRACT Introduction: Rosai-Dorfman disease is a massive lymphocytic histiocytosis characterized by emperipolesis with positive immunohistochemistry for S100 and CD68. It is a rare clinical entity of benign course most commonly presenting in males. Objective: The purpose of this clinical case is to demonstrate successful treatment of Rosai-Dorfman disease with immunomodulators and metronomic chemotherapy. Case presentation: A 57-year-old male patient presenting with bilateral cervical lymphadenopathy underwent CT scan and lymph node biopsy with positive results for S100 and CD68 with marked emperipolesis phenomenon. He received immunomodulatory scheme and successful metronomic chemotherapy with disease remission. Conclusions: Patients diagnosed with Rosai-Dorfman are very few due to the unusual nature of this clinical entity. It is important to mention that this disease is a non-Langerhans cell histiocytosis with benign characteristics and good response to management with corticosteroids and metronomic chemotherapy, therapy established in our patient with good evolution.

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The Rosai-Dorfman disease (RDD) is a bening entity, of unknown etiology, rare, characterized by the overproduction of histiocytes and their subsequent accumulation in the lymph nodes, which may also compromise other organs and systems. It predominantly affects young people. The histological study, which shows emperipolesis, is very indicative but not pathognomonic, but with the immunohistochemistry the diagnosis of the disease is reached, which does not have a well-protocolized treatment. Its evolution is highly variable, with spontaneous remission being a possibility, although in some cases the commitment is extensive, compromising important or vital anatomical structures that requiere vigorous treatment, which can be surgical, radiotherapy and even chemotherapy. We present a case of RDD that affected a 17-year-old girl with upper jaw and right maxillary sinus commitment, which began with loss of teeth, and subsequent appearance of a painless and deforming facial tumour that underwent surgical resection. In this case, the etiological diagnosis was not posible in the preoperative period despite multiple biopsies. An extensive review of the literature is carried out in order to a better understanding of this pathology.

La Enfermedad de Rosai-Dorfman (ERD) es una entidad benigna, de etiología desconocida, poco frecuente, caracterizada por la sobreproducción de histiocitos y su posterior acúmulo en los linfonodos, pudiendo comprometer también a otros órganos y sistemas. Afecta predominantemente a personas jóvenes. El estudio histológico, que muestra emperipolesis, es muy orientativa pero no patognomónica, aunque con la Inmunohistoquímica se llega al diagnóstico de la enfermedad que no tiene un tratamiento bien protocolizado. Su evolución es muy variable, siendo la remisión espontánea una posibilidad, no obstante, en algunos casos, el compromiso es extenso, comprometiendo estructuras anatómicas importantes o vitales que requieren de un tratamiento enérgico, pudiendo ser éste quirúrgico, radioterápico y aún quimioterápico. Se presenta un caso de ERD que afectó a una joven de 17 años, con compromiso de maxilar y seno maxilar derecho, que debutó con pérdida de piezas dentarias y posterior aparición de un tumor facial indoloro y deformante que fue sometido a resección quirúrgica. En este caso no fue posible el diagnóstico etiológico en el preoperatorio pese a múltiples biopsias. Se hace una extensa revisión de la literatura con el objeto de conocer mejor esta patología.

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Resumen La histiocitosis sinusal con linfadenopatías masivas, conocida como enfermedad de Rosai-Dorfman (ERD), es una patología poco frecuente cuya presentación clásica consiste en el desarrollo de grandes adenopatías cervicales bilaterales indoloras. La manifestación extranodal puede involucrar compromiso cutáneo, del sistema nervioso central, hematológico, óseo, de la vía aérea, entre otros. Su diagnóstico es un desafío y requiere una evaluación completa del paciente incluyendo historia clínica, examen físico, exámenes de laboratorio, imágenes y estudio histopatológico que confirme el diagnóstico. El tratamiento debe ser individualizado para cada paciente. Las alternativas incluyen la observación, corticoides sistémicos, radioterapia, quimioterapia, inmunomoduladores y cirugía. Se presenta el caso de una paciente de 65 años con antecedente de enfermedad de Rosai-Dorfman localizada en glándulas lagrimales y linfoma no Hodgkin, que comienza con obstrucción nasal bilateral progresiva refractaria a tratamiento médico, por lo que se decide realizar cirugía, cuyo estudio histopatológico confirmó enfermedad de Rosai-Dorfman.

Abstract Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare pathology, with a classic clinical presentation of painless bilateral massive cervical lymphadenopathy. The extranodal manifestations may involve skin, central nervous system, hematological, bones, and airway compromise, among others. Its diagnosis is challenging and requires a complete evaluation of the patient including medical history, physical examination, laboratory testing, imaging and histopathological study to confirm the diagnosis. Treatment should be individualized for each patient, including follow-up, systemic corticosteroids, radiotherapy, chemotherapy, immunomodulators and surgery. We present the case of a 65-year-old patient with a history of RDD located in the lacrimal glands and lymphoma, which begins with progressive bilateral obstruction refractory to medical treatment, for which it is decided to perform surgery, whose histopathological study confirmed RDD.

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ABSTRACT Introduction: Rosai-Dorfman disease (RDD), also known as sinus histiocytosis, is a rare disorder characterized by histiocyte proliferation. Case presentation: A 33-year-old man consulted the emergency department of a tertiary care institution in Cartagena de Indias, Colombia, due to a 6-month history of progressive deformity in the frontal right side of the face, associated with pain of slow progression, without any other symptoms or dermatological involvement. There were no other major findings on physical examination and laboratory tests performed were negative. Imaging scans obtained showed extensive inflammatory involvement of the frontal bone, which led to suspect osteomyelitis as the first diagnostic possibility. A biopsy of the lesion was performed with negative cultures for bacteria, which allowed establishing a diagnosis of extranodal Rosai-Dorfman disease with isolated bone involvement. Treatment with systemic corticosteroids was indicated with poor response, so methotrexate was added, achieving an evident improvement after 2 months. Conclusions: Little is known about the manifestations of Rosai-Dorfman disease and its treatment in the adult population. The present case report contributes to expanding the literature on this topic, which can present with rare symptoms that may pose challenges for its diagnosis.

RESUMEN Introducción. La enfermedad de Rosai-Dorfman, también conocida como histiocitosis sinusal, es un trastorno poco frecuente caracterizado por la proliferación de histiocitos. Presentación del caso. Hombre de 33 años quien consultó al servicio de urgencias de una institución de tercer nivel de Cartagena de Indias, Colombia, por una deformidad de progresión lenta que apareció 6 meses atrás en el costado frontal derecho de la cara y generaba dolor. Al examen físico no hubo hallazgos mayores y los estudios paraclínicos fueron normales o negativos. Se obtuvieron imágenes que evidenciaron extenso compromiso inflamatorio/infeccioso del hueso frontal, por lo que se sospechó de osteomielitis y se realizó biopsia de la lesión que permitió establecer diagnóstico de enfermedad de Rosai-Dorfman variante extranodal con compromiso óseo aislado. El paciente recibió tratamiento con corticoide sistémico sin obtener respuesta, por lo que se adicionó metotrexato, con lo cual se logró mejoría a los 2 meses. Conclusiones. Las manifestaciones de la enfermedad de Rosai-Dorfman en población adulta son poco conocidas, además no hay mucha información sobre su tratamiento, por lo que el presente caso contribuye a ampliar la literatura sobre esta enfermedad que puede presentarse con sintomatología completamente inusual.

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BACKGROUND: Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytic proliferative disorder classically as a massive cervical lymphadenopathy. However, over the years, extranodal locations were confirmed with the central nervous system involvement in less than 5% of cases, which is marked as a significant differential diagnosis of meningiomas, with which they are widely confused due to the similarity of their radiological images. CASE DESCRIPTION: We report a 37-year-old man and 45-year-old man who were diagnosed with intracranial RDD but whose radiological images mimic meningiomas, requiring anatomopathological and tumor's immunohistochemistry for definitive diagnosis. Moreover, a review of 184 publications with 285 cases of intracranial involvement of this disease was also performed, comparing these findings with those brought in the previous studies. CONCLUSION: Intracranial Rosai-Dorfman tumors should always be remembered as differential diagnosis of meningiomas since they are similar radiologically and macroscopically. Once remembered and diagnosed, the lesion must be treated following the same pattern of resection done in meningiomas and, treatment's differences will not occur in the surgical excision technique, but in complementary chemotherapy implementation, radiotherapy, and even with radiosurgery aid, depending on the case. Thus, it is possible to obtain better results than with just the isolated surgical procedure.

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BACKGROUND: The term "Tolosa-Hunt syndrome" (THS) has been used to refer to painful ophthalmoplegia associated with nonspecific inflammation of the cavernous sinus and many processes can result in a similar clinical picture, including infectious, inflammatory and neoplastic diseases. Rosai-Dorfman disease (RDD) is a lymphoproliferative disorder that rarely affects the central nervous system. We report a case of isolated CNS Rosai-Dorfman disease involving the cavernous sinus and presenting as "Tolosa-Hunt syndrome". CASE PRESENTATION: Our patient presented with horizontal diplopia due to impairment of cranial nerves III, IV and VI and a stabbing/throbbing headache predominantly in the left temporal and periorbitary regions. There was a nonspecific enlargement of the left cavernous sinus on MRI and the patient had a dramatic response to steroids. Biopsy of a frontal meningeal lesion was compatible with RDD. CONCLUSIONS: We highlight the importance of including Rosai-Dorfman disease as a differential diagnosis in cavernous sinus syndrome and demonstrate a satisfactory long-term response to steroid treatment in this disease.

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Rosai-Dorfman disease (RDD) a rare, non-malignant disease of histiocytic proliferation usually presents as a painless lymphadenopathy. It has been associated with autoimmune diseases, viral infections, and malignancies. Management is the treatment of underlying disease. Here we present the case of a patient with HIV infection who presented with massive splenomegaly and lymphadenopathy.

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The association between Rosai-Dorfman Disease (RDD) and cancer was reported for the first time in 1984. However, there are still a small number of reports of this association. We describe a 60-year-old woman who presented with a focal onset motor seizure followed by tonic-clonic generalization and persistent headache. Magnetic Resonance Imaging disclosed an irregular hyperintense lesion in T2 and vasogenic edema in the left parietal region. Immunohistochemical analysis of a biopsy fragment was positive for protein S-100 and CD68 and negative for CD1a, compatible with the diagnosis of RDD. She previously had breast cancer six years earlier and had used tamoxifen for two years and anostrozol for three years after diagnosis of cancer. RDD has been already associated with different cancers, such as Hodgkin's lymphoma, non-Hodgkin's lymphoma, follicular lymphoma, melanoma, adenocarcinoma and small cell lung cancer. As far as we know, this is the first report of an association between breast cancer and RDD.

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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.

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BACKGROUND: The aim of this study is to report a case of cystoid macular oedema (CME) associated with Rosai-Dorfman Disease (RDD). CASE PRESENTATION: A 52-year-old male initially presented with a two-month history of a congested left eye. At presentation, visual acuity was 20/20 in the right eye and 20/80 in the left eye. Biomicroscopy showed conjunctival hyperaemia in the left eye with a slight elevation, suggesting a subconjunctival mass. Retinal fluorescein angiography and optical coherence tomography (OCT) revealed the presence of CME in the left eye. A clinical examination revealed nodular lesions in the ears and a lump in the subcutaneous tissue of the left arm. A biopsy of the subcutaneous lesion showed histological and immunohistochemical characteristics of RDD. The patient was treated with intravitreal triamcinolone (0.1 mL/4 mg). One month after treatment, there was complete regression of the oedema with a significant improvement in visual acuity to 20/20. CONCLUSIONS: This is the first reported case of RDD associated with cystoid macular oedema. Macular oedema responded to intravitreal treatment with triamcinolone.

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Rosai-Dorfman disease also known as sinus histiocytosis with massive lymphadenopathy (SHML) is characterized by distorted lymph node architecture with marked dilation of lymphatic sinuses occupied by numerous lymphocytes, as well as histiocytes with vesicular nucleus and abundant clear cytoplasm with phagocytized lymphocytes or plasma cells, also known as 'emperipolesis'. This disease of unknown etiology progresses with a benign prognosis strictly and only when an early diagnosis and treatment is made. A late diagnosis and a generalized lymph node involvement contribute to a poor prognosis. In this study, we focussed on the cytological characteristics of the Rosai-Dorfman disease and differential diagnoses. We reported a case of a 61-year-old Mexican male with a 9-month history of painless bilateral cervical masses and low-grade fever with the final diagnosis of Rosai-Dorfman disease. The final diagnosis was made by fine needle aspiration (FNA) biopsy of parotid gland and cervical lymph node. In conclusion, FNA biopsy can be enough to make the diagnosis in most cases due to the distinct cytological features of SHML, thereby avoiding more invasive approaches that potentially are unnecessary.

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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.

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OBJECTIVES: The present article provides an overview of the spectrum of abdominal findings of histiocytic disorders that may be observed in multimodality imaging illustrated by clinical cases from our Imaging Center. METHODS: We will review abdominal findings of Langerhans cell histiocytosis, Rosai-Dorfman disease, Erdheim-Chester disease, and hemophagocytic syndrome illustrated by clinical cases from our imaging department with histologic correlation. RESULTS: Abdominal involvement of histiocytic disorders is rare and may occur in the liver, biliary tract, kidney, retroperitoneum, kidney, gastrointestinal tract, and lymph nodes. CONCLUSION: Histiocytic disorders encompass a group of rare diseases with a wide range of manifestations in which the abdominal involvement is quite infrequent. The role of the radiologist is to report the major imaging findings and the differential diagnosis; however, the imaging features are unspecific and biopsy usually is necessary to establish the definitive diagnosis.

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