Clinical Manifestations and Radiological Characteristics on Rosai-Dorfman Disease: A Retrospective Observational Study

2020 ◽  
Vol 5 (5) ◽  
Keyword(s):  
Blood Vessels ◽  
Abdominal Cavity ◽  
Clinical Manifestations ◽  
Ct Images ◽  
High Signal ◽  
Radiological Features ◽  
Imaging Characteristics ◽  
Rosai Dorfman Disease ◽  
Heterogeneous Enhancement ◽  
The Masses

Background and Objective: Rosai-Dorfman disease (RDD) are usually misdiagnosed because of rarity and nonspecific clinical and radiological features. The aim of our study is to explore the clinical and imaging characteristics of RDD to improve diagnostic accuracy. Methods: Clinical and imaging data in 10 patients with RDD were retrospectively analyzed. 7 patients were underwent CT scanning and 3 patients were underwent MR examination. Results: 8 (8/10) patients presented with painless enlarged lymph nodes (LNs) or mass. 3 cases were involved with LNs, 5 cases were involved with extra-nodal tissues, and the remaining 2 cases were involved with LNs and extra-nodal tissue simultaneously. In enhanced CT images, enlarged LNs displayed mild or moderate enhancement, and 2 cases showed heterogeneous ring-enhancement. MR features of 3 patients with extra-nodal RDD, 2 cases showed a mass located in the subcutaneous and anterior abdominal wall respectively, and 1 case showed an intracranial mass. Besides, all lesions showed high signal foci on DWI images, and were characterized by marked heterogeneous enhancement with blurred edge. The dural/fascia tail sign and dilated blood vessels could be seen around all the lesions on enhanced MRI. Radiological features of 2 cases with LN and extranodal tissue involved, one case presented with the swelling and thickening of pharyngeal lymphoid ring and nasopharynx, meanwhile with enlarged LNs in bilateral submandibular area, neck and abdominal cavity, and also companied with osteolytic lesion in right proximal humerus. All these LNs displayed mild and moderate enhancement on CT images. Another case showed enlarged LNs in bilateral neck accompanied with soft tissue mass in the sinuses. Conclusions: RDD occurred commonly in young and middle-aged men and presented with painless enlarged LNs or mass.RDD had a huge diversity of imaging findings, which varied with different location. The radiological features, such as small patches of high signal foci in the masses on DWI images, heterogeneous enhancement and blood vessels around the masses, are helpful in diagnosis of extranodal RDD.

scholarly journals Clinical Manifestations and Radiological Characteristics on Rosai-Dorfman Disease: A Retrospective Observational Study

2020 ◽  
Vol 5 (5) ◽  
Keyword(s):  
Blood Vessels ◽  
Abdominal Cavity ◽  
Clinical Manifestations ◽  
Ct Images ◽  
High Signal ◽  
Radiological Features ◽  
Imaging Characteristics ◽  
Rosai Dorfman Disease ◽  
Heterogeneous Enhancement ◽  
The Masses

Background and Objective: Rosai-Dorfman disease (RDD) are usually misdiagnosed because of rarity and nonspecific clinical and radiological features. The aim of our study is to explore the clinical and imaging characteristics of RDD to improve diagnostic accuracy. Methods: Clinical and imaging data in 10 patients with RDD were retrospectively analyzed. 7 patients were underwent CT scanning and 3 patients were underwent MR examination. Results: 8 (8/10) patients presented with painless enlarged lymph nodes (LNs) or mass. 3 cases were involved with LNs, 5 cases were involved with extra-nodal tissues, and the remaining 2 cases were involved with LNs and extra-nodal tissue simultaneously. In enhanced CT images, enlarged LNs displayed mild or moderate enhancement, and 2 cases showed heterogeneous ring-enhancement. MR features of 3 patients with extra-nodal RDD, 2 cases showed a mass located in the subcutaneous and anterior abdominal wall respectively, and 1 case showed an intracranial mass. Besides, all lesions showed high signal foci on DWI images, and were characterized by marked heterogeneous enhancement with blurred edge. The dural/fascia tail sign and dilated blood vessels could be seen around all the lesions on enhanced MRI. Radiological features of 2 cases with LN and extranodal tissue involved, one case presented with the swelling and thickening of pharyngeal lymphoid ring and nasopharynx, meanwhile with enlarged LNs in bilateral submandibular area, neck and abdominal cavity, and also companied with osteolytic lesion in right proximal humerus. All these LNs displayed mild and moderate enhancement on CT images. Another case showed enlarged LNs in bilateral neck accompanied with soft tissue mass in the sinuses. Conclusions: RDD occurred commonly in young and middle-aged men and presented with painless enlarged LNs or mass.RDD had a huge diversity of imaging findings, which varied with different location. The radiological features, such as small patches of high signal foci in the masses on DWI images, heterogeneous enhancement and blood vessels around the masses, are helpful in diagnosis of extranodal RDD.

scholarly journals Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages

Blood ◽  
2016 ◽  
Vol 127 (22) ◽  
pp. 2672-2681 ◽  
Author(s):  
Jean-François Emile ◽  
Oussama Abla ◽  
Sylvie Fraitag ◽  
Annacarin Horne ◽  
Julien Haroche ◽  
...  
Keyword(s):  
Dendritic Cell ◽  
Clinical Manifestations ◽  
Rare Disorders ◽  
Molecular Alterations ◽  
Imaging Characteristics ◽  
Rosai Dorfman Disease ◽  
New Findings ◽  
Wide Range ◽  
Guidelines And Recommendations

Abstract The histiocytoses are rare disorders characterized by the accumulation of macrophage, dendritic cell, or monocyte-derived cells in various tissues and organs of children and adults. More than 100 different subtypes have been described, with a wide range of clinical manifestations, presentations, and histologies. Since the first classification in 1987, a number of new findings regarding the cellular origins, molecular pathology, and clinical features of histiocytic disorders have been identified. We propose herein a revision of the classification of histiocytoses based on histology, phenotype, molecular alterations, and clinical and imaging characteristics. This revised classification system consists of 5 groups of diseases: (1) Langerhans-related, (2) cutaneous and mucocutaneous, and (3) malignant histiocytoses as well as (4) Rosai-Dorfman disease and (5) hemophagocytic lymphohistiocytosis and macrophage activation syndrome. Herein, we provide guidelines and recommendations for diagnoses of these disorders.

Intravascular malignant lymphomatosis: diffusion‐weighted magnetic resonance imaging characteristics

2005 ◽  
Vol 46 (3) ◽  
pp. 246-249 ◽  
Author(s):  
T. Kinoshita ◽  
S. Sugihara ◽  
E. Matusue ◽  
T. Nomura ◽  
M. Ametani ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
White Matter ◽  
Magnetic Resonance ◽  
Blood Vessels ◽  
High Signal ◽  
Resonance Imaging ◽  
Diffusion Weighted ◽  
Apparent Diffusion Coefficient Values ◽  
Imaging Characteristics ◽  
Weighted Magnetic Resonance Imaging

Intravascular malignant lymphomatosis is an unusual condition in which malignant lymphoma cells form microscopic masses within the blood vessels of the central nervous system. Occlusion of the involved blood vessels can lead to multifocal cerebral infarcts. Diffusion‐weighted magnetic resonance imaging (MRI) reveals a subacute infarction pattern (bright high signal intensity on b = 1000 s/mm2 images and intermediate apparent diffusion coefficient values) in the cerebral deep white matter. We present MRI findings of a 68‐year‐old woman with intravascular malignant lymphomatosis involving the cerebral white matter and the thoracic cord.

scholarly journals Rosai-Dorfman Disease of Bone and Soft Tissue

2021 ◽  
Vol 146 (1) ◽  
pp. 40-46
Author(s):  
Roberto A. Garcia ◽  
Edward F. DiCarlo
Keyword(s):  
Differential Diagnosis ◽  
Soft Tissue ◽  
Clinical Presentation ◽  
Clinical Manifestations ◽  
Imaging Findings ◽  
Imaging Characteristics ◽  
Rosai Dorfman Disease ◽  
S 100 ◽  
Systemic Manifestations ◽  
Variable Clinical Presentation

Context.— Rosai-Dorfman disease is a rare histiocytic proliferative disorder of unknown pathogenesis that may be diagnostically difficult in extranodal sites. It is commonly an unsuspected diagnosis when arising in bone and soft tissue, especially when it presents without associated lymphadenopathy. Its variable clinical presentation and nonspecific imaging findings make the diagnosis quite challenging, particularly in small biopsies. The problem is compounded by its less-characteristic histomorphologic features in comparison with nodal disease. Awareness of the potential diagnostic pitfalls in Rosai-Dorfman disease of bone and soft tissue should raise the degree of diagnostic accuracy. Objective.— To review the clinical manifestations, imaging characteristics, and histomorphologic features of Rosai-Dorfman disease of bone and soft tissue along with a brief discussion of its differential diagnosis, pathogenesis, and current management. Data Sources.— Thorough review of the literature with focus on clinical manifestations, imaging findings, key histomorphologic features, pathogenesis, and treatment. Conclusions.— The diagnosis of Rosai-Dorfman disease of bone and soft tissue may be quite challenging because of its variable clinical presentation and nonspecific imaging findings. It may be asymptomatic without systemic manifestations or associated lymphadenopathy. The definitive diagnosis relies on histopathologic identification of the characteristic S-100–positive histiocytes demonstrating emperipolesis. Bone and soft tissue lesions tend to have lower numbers of characteristic histiocytes and less conspicuous emperipolesis and often demonstrate areas of fibrosis or storiform spindle cell areas resembling fibrohistiocytic lesions. Awareness of these unusual features is necessary in order to consider Rosai-Dorfman disease in the differential diagnosis when confronting these rare and often misleading lesions.

scholarly journals Head and neck osteosarcoma: CT and MR imaging features

2020 ◽  
Vol 49 (2) ◽  
pp. 20190202
Author(s):  
Zhendong Luo ◽  
Weiguo Chen ◽  
Xinping Shen ◽  
Genggeng Qin ◽  
Jianxiang Yuan ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Head And Neck ◽  
Giant Cell ◽  
High Signal ◽  
Imaging Features ◽  
Matrix Mineralization ◽  
Imaging Characteristics ◽  
Mri Features ◽  
Signal Intensities ◽  
Ct And Mri

Objective: This study aims to assess the CT and MRI features of head and neck osteosarcoma (HNO). Methods: 37 HNOs were identified, and the following imaging characteristics were reviewed on CT and MRI. Results: A total of 37 patients(age 41.5 ± 15.0 years old; 16 males, 21 females) were included in the study. Tumours occurred in the maxilla (16, 43.2%), mandible (8, 21.6%), skull base (6, 16.2%), calvarium (5, 13.5%), paranasal sinuses (1, 2.7%) and cervical soft tissue (1, 2.7%). 16 patients received radiotherapy for nasopharyngeal carcinoma. Three patients (8.1%) developed osteosarcomas related to a primary bone disease. 16 of the (43.2%) tumours demonstrated lytic density on CT scans, followed by 13 (35.1%) showing mixed density and 7 (18.9%) with sclerotic density. Matrix mineralization was present in 32 (86.5%). 3 out of 24 (12.5%) tumours showed lamellar periosteal reactions, 21 out of 24 (87.5%) showed spiculated periosteal reactions. 12 tumours showed low signal intensities on T1WI, with 16 having heterogeneous signal intensities. 10 tumours showed high signal intensities on T2WI, and 18 showed heterogeneous signal intensities. With contrast-enhanced images, 3 tumours showed homogeneous enhancement (2 osteoblastic and 1 giant cell-rich), 18 tumours showed heterogeneous enhancement (13 osteoblastic, 4 fibroblastic and 1 giant cell-rich), and 7 tumours showed peripheral enhancement (6 chondroblastic and 1 osteoblastic). These tumours were characterized by soft tissue masses with a diameter of 5.6 ± 1.8 cm. Conclusions: HNO is a rare condition and is commonly associated with previous radiation exposure. This study provides age, sex distribution, location, CT and MRI features of HNO.

scholarly journals Deep Cerebral Venous Thrombosis—A Clinicoradiological Study

2021 ◽  
Author(s):  
Sujana Gogineni ◽  
Dhananjay Gupta ◽  
R. Pradeep ◽  
Anish Mehta ◽  
Mahendra Javali ◽  
...  
Keyword(s):  
Venous Thrombosis ◽  
Care Center ◽  
Tertiary Care ◽  
Clinical Manifestations ◽  
Arterial Occlusion ◽  
Venous System ◽  
Common Risk Factor ◽  
Imaging Characteristics ◽  
Deep Cerebral Venous Thrombosis ◽  
High Index Of Suspicion

AbstractStroke is a common neurological emergency. Almost 80% of strokes are due to arterial occlusion. Venous thrombosis comprises less than 1–2% of all strokes. Involvement of the deep cerebral venous system is still rare and accounts for about 10.9% of all cerebral venous thromboses (CVT). CVT diagnosis is often delayed or missed, because of its variable clinical manifestations. We retrospectively (2015–18) and prospectively (2018–20) reviewed all the cases of CVT in a tertiary care center in south India. Out of a total of 52 CVT cases, 12 were due to the involvement of deep cerebral venous system. Their clinical presentation, imaging characteristics, and outcomes were assessed. The most frequent presentation was headache followed by seizures. Hyperhomocysteinemia was the most common risk factor noted. Imaging characteristics were variable, and a high index of suspicion was required for early diagnosis. All patients had favorable outcome in our study, and except one, all were treated conservatively.

scholarly journals Aggregation-induced emission nanoparticles for in vivo three-photon fluorescence microscopic rat brain angiography

2019 ◽  
Vol 12 (06) ◽  
pp. 1950012 ◽  
Author(s):  
Hequn Zhang ◽  
Weisi Xie ◽  
Ming Chen ◽  
Liang Zhu ◽  
Zhe Feng ◽  
...  
Keyword(s):  
Rat Brain ◽  
Blood Vessels ◽  
Laser Scanning ◽  
Near Infrared ◽  
Brain Disease ◽  
Laser Scanning Microscopy ◽  
High Signal ◽  
Imaging Method ◽  
Photon Fluorescence

Rodents are popular biological models for physiological and behavioral research in neuroscience and rats are better models than mice due to their higher genome similarity to human and more accessible surgical procedures. However, rat brain is larger than mice brain and it needs powerful imaging tools to implement better penetration against the scattering of the thicker brain tissue. Three-photon fluorescence microscopy (3PFM) combined with near-infrared (NIR) excitation has great potentials for brain circuits imaging because of its abilities of anti-scattering, deep-tissue imaging, and high signal-to-noise ratio (SNR). In this work, a type of AIE luminogen with red fluorescence was synthesized and encapsulated with Pluronic F-127 to make up form nanoparticles (NPs). Bright DCDPP-2TPA NPs were employed for in vivo three-photon fluorescent laser scanning microscopy of blood vessels in rats brain under 1550[Formula: see text]nm femtosecond laser excitation. A fine three-dimensional (3D) reconstruction up to the deepness of 600[Formula: see text][Formula: see text]m was achieved and the blood flow velocity of a selected vessel was measured in vivo as well. Our 3PFM deep brain imaging method simultaneously recorded the morphology and function of the brain blood vessels in vivo in the rat model. Using this angiography combined with the arsenal of rodent’s brain disease, models can accelerate the neuroscience research and clinical diagnosis of brain disease in the future.

scholarly journals Current Diagnosis and Management of Cervical Spondylotic Myelopathy

2017 ◽  
Vol 7 (6) ◽  
pp. 572-586 ◽  
Author(s):  
Joshua Bakhsheshian ◽  
Vivek A. Mehta ◽  
John C. Liu
Keyword(s):  
Natural History ◽  
Cervical Spondylotic Myelopathy ◽  
Early Recognition ◽  
Clinical Manifestations ◽  
High Quality ◽  
Diagnosis And Management ◽  
Imaging Characteristics ◽  
Cervical Sagittal Alignment ◽  
Long Term Follow Up ◽  
The Cost

Study Design: Review. Objectives: Cervical spondylotic myelopathy (CSM) is a major cause of disability, particular in elderly patients. Awareness and understanding of CSM is imperative to facilitate early diagnosis and management. This review article addresses CSM with regard to its epidemiology, anatomical considerations, pathophysiology, clinical manifestations, imaging characteristics, treatment approaches and outcomes, and the cost-effectiveness of surgical options. Methods: The authors performed an extensive review of the peer-reviewed literature addressing the aforementioned objectives. Results: The clinical presentation and natural history of CSM is variable, alternating between quiescent and insidious to stepwise decline or rapid neurological deterioration. For mild CSM, conservative options could be employed with careful observation. However, surgical intervention has shown to be superior for moderate to severe CSM. The success of operative or conservative management of CSM is multifactorial and high-quality studies are lacking. The optimal surgical approach is still under debate, and can vary depending on the number of levels involved, location of the pathology and baseline cervical sagittal alignment. Conclusions: Early recognition and treatment of CSM, before the onset of spinal cord damage, is essential for optimal outcomes. The goal of surgery is to decompress the cord with expansion of the spinal canal, while restoring cervical lordosis, and stabilizing when the risk of cervical kyphosis is high. Further high-quality randomized clinical studies with long-term follow up are still needed to further define the natural history and help predict the ideal surgical strategy.

scholarly journals Melioidosis in Bihar, India: unearthing the first of many?

2021 ◽  
Vol 3 (9) ◽  
Author(s):  
Isra Halim ◽  
Prathyusha Kokkayil ◽  
Ravi Kirti ◽  
Rajeev Nayan Priyadarshi ◽  
Asim Sarfraz ◽  
...  
Keyword(s):  
Clinical Manifestations ◽  
Clinical Suspicion ◽  
Radiological Features ◽  
First Case ◽  
Insidious Onset ◽  
Ankle Joints ◽  
The World ◽  
Resource Poor ◽  
Empirical Antibiotics ◽  
The Right

Melioidosis, a disease with protean clinical manifestations, is prevalent in many parts of India, with established endemic hotspots on the southern coast of the country. However, it is still underdiagnosed in many resource-poor regions of the country. We report what is, to the best of our knowledge, the first case of melioidosis diagnosed and treated in Bihar, an economically underdeveloped state in East India. The patient, a 52-year-old diabetic male, presented to the outpatient department with a fever of insidious onset along with pain and restriction of movement in the right shoulder joint and right knee joint, and swelling and tenderness of bilateral ankle joints. Radiological features were suggestive of multiple joint and organ abscesses. A diagnosis of disseminated septicaemic melioidosis was confirmed microbiologically. The patient improved clinically following aggressive treatment with meropenem and cotrimoxazole. The case highlights the need for increased clinical suspicion of melioidosis and adequate diagnostic facilities, as well as the need for early institution of appropriate empirical antibiotics in suspected cases of melioidosis in this region of the world.

scholarly journals Diagnostic difficulties and features of endovideosurgical treatment of a patient with mucocele of the appendix

2020 ◽  
Vol 12 (2) ◽  
pp. 85-90
Author(s):  
Badri V. Sigua ◽  
Vyacheslav P. Zemlyanoy ◽  
Elguja L. Lataria ◽  
Alexey A. Kurkov ◽  
Vyacheslav A. Melnikov ◽  
...  
Keyword(s):  
Benign Tumor ◽  
Abdominal Cavity ◽  
Clinical Manifestations ◽  
Vermiform Appendix ◽  
Diagnostic Methods ◽  
Number Of Patients ◽  
Mucocele Of The Appendix ◽  
Diagnostic Difficulties ◽  
Life Threatening ◽  
The Right

The mucocele of the appendix is the expansion of the appendix with the accumulation of a large amount of mucus. The mechanism and causes of mucocele are not fully understood. According to some authors, such changes in the appendix can occur due to cicatricial narrowing of the lumen of the appendix, compression or blockage of its base. Other authors believe that the mucocele of the appendix is a benign tumor that develops from the remnants of primitive mesenchyme and is sometimes prone to malignancy. Clinical manifestations of mucocele of the appendix are nonspecific. In a number of patients, this disease causes pain in the right abdomen, more often pulling, intermittent. However, the disease is often asymptomatic. In this regard, diagnosis is established only during performing an operation, most often, regarding acute appendicitis. Nevertheless, instrumental diagnostic methods such as ultrasound and computed tomography of the abdominal and pelvic organs make it possible to suspect mucocele. Despite the frequent asymptomatic, non-aggressive course, a number of life-threatening complications can become the outcome of the mucocele of the vermiform appendix. The most formidable complication is the rupture of the appendix with mucus entering free abdominal cavity, followed by the development of peritoneal pseudomyxoma due to implantation of mucus-forming cells. The only option for radical treatment of the mucocele of the appendix is a surgical intervention. A presented clinical case demonstrates the difficulties of diagnosis, as well as the features of surgical treatment of a patient with a mucocele of the appendix.

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