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Journal of Clinical Imaging Science 2023Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is an uncommon benign disorder characterized by the accumulation of...
Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is an uncommon benign disorder characterized by the accumulation of activated histiocytes in affected tissues. While RDD typically involves lymph nodes, it may manifest as extranodal involvement. Breast involvement is an exceedingly rare presentation of this condition with <100 reported cases worldwide. This report presents a case of RDD in a 58-year-old male patient who presented with a palpable breast mass. Mammography and ultrasound imaging studies raised concerns for malignancy, prompting a breast biopsy. Histopathological examination revealed S100-positive pale histiocytes exhibiting emperipolesis, consistent with RDD. The management of extranodal RDD is individualized, as no standardized guidelines are currently available. However, surgical excision is recommended for unicentric breast lesions, which was performed in our case, resulting in complete remission. The patient has remained disease-free under surveillance with computed tomography scans. Our case underscores the importance of considering RDD in the differential diagnoses of breast masses and highlights the utility of surgical excision as an effective treatment option, especially for unicentric breast lesions of RDD.
PubMed: 37680249
DOI: 10.25259/JCIS_40_2023 -
Successful anesthesia management with inhalation anesthesia in Rosai-Dorfman disease: a case report.The Journal of International Medical... Aug 2023Rosai-Dorfman disease (RDD) is a rare, benign, non-Langerhans cell histiocytic proliferative disease. RDD with central nervous system involvement is extremely rare....
Rosai-Dorfman disease (RDD) is a rare, benign, non-Langerhans cell histiocytic proliferative disease. RDD with central nervous system involvement is extremely rare. Surgical excision is generally regarded as the appropriate treatment of choice for this disease, especially when the lesion causes neurological compression. RDD can be accompanied by systemic symptoms, such as malaise, fever, weight change, leukocytosis, anemia, and hormonal disturbance, which may be challenging during general management. Little is known regarding peri-anesthesia management of this rare disease. We report a case of a patient in his 20s who had recurrent RDD and had general anesthesia with perioperative management. He was obese and hepatic insufficiency. This case report adds to the literature regarding the perioperative anesthetic management of RDD with central nervous system involvement.
Topics: Anesthesia, Inhalation; Histiocytosis, Sinus; Humans; Male; Young Adult; Perioperative Care; Obesity; Hepatic Insufficiency; Craniotomy; Central Nervous System Diseases
PubMed: 37647362
DOI: 10.1177/03000605231195161 -
CNS Oncology Dec 2023Rosai-Dorfman disease (RDD) is a rare, S100-positive histiocytic proliferation, that can cause both nodal and extranodal illness. We present a case of a 53-year-old male...
Rosai-Dorfman disease (RDD) is a rare, S100-positive histiocytic proliferation, that can cause both nodal and extranodal illness. We present a case of a 53-year-old male patient. Magnetic resonance imaging described a plaque-like meningeal lesion, and the preoperative diagnosis was meningioma. Histologically, dense infiltration of lymphocytes, plasma cells, and histiocytes was seen, furthermore, the presence of emperipolesis in the sample was pronounced. In the histiocytes nuclear and cytoplasmic positivity with S100 protein, and nuclear positivity with Cyclin D1 was observed. The case was concluded as RDD. Morphological appearance of intracranial RDD with imaging procedures can present a differential diagnostic challenge. The correct diagnosis is based on the presence of histiocytes with emperipolesis, and properly defined immunohistochemical characteristics.
Topics: Male; Humans; Middle Aged; Histiocytosis, Sinus; Histiocytes; Emperipolesis; Meningioma; S100 Proteins; Diagnosis, Differential; Meningeal Neoplasms
PubMed: 37551608
DOI: 10.2217/cns-2023-0006 -
Iranian Journal of Otorhinolaryngology Jul 2023Multifocal Langerhans' cell histocytosis is a rare condition that can affect multiple organs and manifest in various scenarios. While the condition is more commonly...
INTRODUCTION
Multifocal Langerhans' cell histocytosis is a rare condition that can affect multiple organs and manifest in various scenarios. While the condition is more commonly found in children, it can also occur in adults.
CASE REPORT
A 43-year-old female presented with refractory otorrhea and had a rubbery neck mass in the left mid-cervical area, as well as an itchy eczematoid lesion in the left parietal area. The otic lesion was eventually resected, and histopathologic examination confirmed the diagnosis of Langerhans histiocytosis.
CONCLUSIONS
Although rare in adults, Langerhans histiocytosis should be considered as one of the differential diagnoses for ear canal polyps. If diagnosed, medical treatment should be pursued.
PubMed: 37497157
DOI: 10.22038/IJORL.2023.65286.3238 -
Asian Journal of Surgery Nov 2023
Topics: Humans; Histiocytosis, Sinus; Diagnosis, Differential
PubMed: 37479651
DOI: 10.1016/j.asjsur.2023.07.046 -
Asian Journal of Surgery Nov 2023
Topics: Humans; Histiocytosis, Sinus; Exome Sequencing; Mutation; Interferon Regulatory Factors
PubMed: 37422400
DOI: 10.1016/j.asjsur.2023.06.071 -
Medecine Tropicale Et Sante... Mar 2023A 8-year-old schoolgirl from West Africa with no previous pathological history was admitted to the haematology department of the Brazzaville University Hospital for the...
A 8-year-old schoolgirl from West Africa with no previous pathological history was admitted to the haematology department of the Brazzaville University Hospital for the management of cervical adenopathy. The diagnosis of sinus histiocytosis or Destombes-Rosaï-Dorfman disease was retained and the patient was treated with PO corticosteroids (methylprednisolone 32 mg/d then 16 mg/d). Given the rarity and uncertain aetiopathogeny of this syndrome, treatment is poorly codified. It includes corticosteroid therapy, immunomodulators and sometimes chemotherapy, radiotherapy or surgery, indicated in case of clinical manifestations of local organ compression. The disease may regress spontaneously. Its benignity does not justify systematic treatment in the absence of complications.
Topics: Humans; Child; Congo; Adjuvants, Immunologic; Africa, Western; Data Compression; Histiocytosis, Sinus; Hospitals, University
PubMed: 37389375
DOI: 10.48327/mtsi.v3i1.2023.340 -
Journal of Dentistry (Shiraz, Iran) Jun 2023Rosai Dorfman disease is generally defined as a massive bilateral painless cervical lymphadenopathy accompanied with both fever and leukocytosis with neutrophilia....
Rosai Dorfman disease is generally defined as a massive bilateral painless cervical lymphadenopathy accompanied with both fever and leukocytosis with neutrophilia. Additionally, it may possibly be associated with polyclonal hypergammaglobulinemia, reversal of CD4/CD8 ratio, the elevated erythrocyte sedimentation rate (ESR), microcytic anemia, and thrombocytosis. Rosai-Dorfman disease is known as a benign self-limiting disease, so no treatment is required in many cases, although it causes death in some cases by involving vital organs like kidney. The treatment is required when there is a life-threatening situation such as airway obstruction or involvement of vital organs such as kidney, liver, and lower respiratory tract. The required treatment choices include steroid therapy, chemotherapy, radiotherapy, and surgery. Surgical treatment is performed for bulk removal to resolve the obstruction caused by the mass as well as taking biopsy for the definite histopathologic diagnosis of disease. A 26-year-old man was referred to oral and maxillofacial surgery (OMFS) clinic of Taleghani hospital with chief complaints of pain and swelling of left submandibular space. According to the patient himself, the swelling had been started three months earlier. After rejecting dental source of the lesion, we decided to remove the mass by excisional biopsy concerning the patient's discomfort. Histopathology report verified Rosai Dorfman disease as definite diagnosis of the mass.
PubMed: 37388202
DOI: 10.30476/dentjods.2022.95184.1844 -
Cureus May 2023Rosai-Dorfman disease (RDD) is a rare histiocytic disorder that can present with painless bilateral symmetrical cervical lymphadenopathy, mimicking lymphomas. RDD is...
Rosai-Dorfman disease (RDD) is a rare histiocytic disorder that can present with painless bilateral symmetrical cervical lymphadenopathy, mimicking lymphomas. RDD is characterized by excessive tissue infiltration by dendritic cells, macrophages, or monocyte-derived cells, with a histopathologic diagnosis based on the presence of CD68+, CD163+, and S100+ histiocytes, which differentiate it from other histiocytic neoplasms. In this case report, we present a young Hispanic female with recurrent subcutaneous growths and lymphadenopathy, initially thought to be lymphoma, who was diagnosed with RDD after a significant diagnostic workup. Treatment initially consisted of surgical excision; however, due to recurrence, the patient was successfully treated with corticosteroids and a steroid-sparing agent, 6-mercaptopurine, with significant improvement in symptoms. RDD should be considered a differential diagnosis for patients with cervical lymphadenopathy, and an interdisciplinary approach is essential to managing this rare disorder effectively. The report highlights the need for an interdisciplinary approach to managing this rare disorder effectively and underscores the importance of multimodal treatment in disease suppression. As a rare disease with slow advancement of defined guidelines for diagnostic and treatment strategies, this case report adds to the existing literature on RDD.
PubMed: 37378192
DOI: 10.7759/cureus.39131 -
Revista Espanola de Enfermedades... Jun 2024We present a case of Rosai-Dorfman disease (RDD) occurred in a 6-year-old male child, characterized by extensive involvement of the esophagus. Eight months ago, the...
We present a case of Rosai-Dorfman disease (RDD) occurred in a 6-year-old male child, characterized by extensive involvement of the esophagus. Eight months ago, the child presented with persistent fever and a diffuse dark red rash. MRI revealed a mass occupying the left nasal cavity and septal sinus and biopsy pathology confirmed the diagnosis of RDD. Following dexamethasone and prednisone treatment, the child experienced dysphagia. 18F-FDG PET/CT revealed multiple lesions with increased metabolism in the left nasal sinus, lymph nodes, widespread skin lesions, and the entire esophagus. Subsequent biopsies of lymph nodes, abdominal skin, and esophageal lesions was consistent with RDD involvement. The child is presently undergoing six cycles of VCR+Ara-c+Dex chemotherapy and the treatment is going well.
Topics: Humans; Histiocytosis, Sinus; Male; Child; Esophageal Diseases; Esophagus; Positron Emission Tomography Computed Tomography
PubMed: 37350657
DOI: 10.17235/reed.2023.9753/2023