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.

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.

Rare Facial Clefts

2015 ◽  
Vol 87 (8) ◽  
Author(s):  
Marta Fijałkowska ◽  
Bogusław Antoszewski
Keyword(s):  
Surgical Treatment ◽  
Soft Tissue ◽  
Clinical Presentation ◽  
Treatment Plan ◽  
General Rule ◽  
Common Type ◽  
Facilitated Communication ◽  
Facial Clefts ◽  
Therapeutic Problem ◽  
Variable Clinical Presentation

AbstractIn 1976 Dr. Paul Tessier described numeric classification for rare craniofacial clefts. He first emphasized that a fissure of the soft tissue corresponds, as a general rule, to a cleft of the bony structure. The classification, easy to understand, became widely accepted because the recording of the malformations was simple and facilitated communication between observers.was to present our own experience with treatment of patients with rare facial clefts.Our Department has 11 patients with rare craniofacial clefts under its care. This group includes 8 boys and 3 girls. The patients aged from 2 months to 18 years at the time of the first consultation.In two patients the cleft was median, in seven patients it was one-sided and in two – bilateral. The most common type of cleft was number 6, and the rarest were 2, 3, and 7. All patients underwent surgical treatment.Atypical facial clefts are rare congenital anomalies, however because of functional and aesthetic disturbances they constitute a serious medical and therapeutic problem. Facial clefts are characterized by variable clinical presentation and require individualized treatment plan.

scholarly journals Sinonasal Hemangiopericytomas: Clinicopathologic and Imaging Findings

2005 ◽  
Vol 84 (2) ◽  
pp. 99-102 ◽  
Author(s):  
Enrique Palacios ◽  
Santiago Restrepo ◽  
Luciano Mastrogiovanni ◽  
Giovanni D. Lorusso ◽  
Rafael Rojas
Keyword(s):  
Soft Tissue ◽  
Retrospective Review ◽  
Soft Tissue Sarcomas ◽  
The Body ◽  
Vascular Tumors ◽  
Imaging Findings ◽  
Mesenchymal Tumors ◽  
Imaging Characteristics ◽  
Neoplastic Lesions ◽  
Pathologic Findings

Hemangiopericytomas are rare soft-tissue neoplastic lesions that can arise in any part of the body. They are mesenchymal tumors that account for 3 to 5% of all soft-tissue sarcomas and 1% of all vascular tumors. They originate in extravascular cells (pericytes). Some 15 to 30% of all hemangiopericytomas occur in the head and neck; of these, approximately 5% occur in the sinonasal area. We describe our brief retrospective review of 7 histologically proven cases of sinonasal hemangiopericytoma, and we discuss the imaging characteristics and clinical and pathologic findings in these patients.

scholarly journals Intramuscular lipoma: a review of the literature

2014 ◽  
Vol 6 (4) ◽  
Author(s):  
Shane McTighe ◽  
Ivan Chernev
Keyword(s):  
Differential Diagnosis ◽  
Soft Tissue ◽  
Clinical Features ◽  
Common Type ◽  
Fatty Tissue ◽  
Review Of The Literature ◽  
Mesenchymal Tumors ◽  
Intramuscular Lipoma ◽  
Imaging Characteristics ◽  
Well Differentiated

Lipomas are the most common type of soft tissue mesenchymal tumors. They are typically located subcutaneously and consist of mature fatty tissue. When they occur under the enclosing fascia, they are called deep-seated lipomas. Infrequently, lipomas can arise inside the muscle and are called intramuscular lipomas. Intramuscular lipomas have been commonly investigated and categorized in the same group as other deep-seated and superficial lipomatous lesions. Their clinical, histological and imaging characteristics may resemble well-differentiated liposarcomas, further adding to the difficulties in the differential diagnosis. This article summarizes the available literature and describes the typical epidemiological, pathological and clinical features of intramuscular lipomas, as well as delineating their treatment and prognosis.

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.

scholarly journals Dermatofibrosarcoma Protuberans: Insights into a Rare Soft Tissue Tumor

2016 ◽  
Vol 6 ◽  
pp. 16 ◽  
Author(s):  
Aliya Sarhan Al Barwani ◽  
Sawsan Taif ◽  
Reem Ahmed Al Mazrouai ◽  
Khamis Salim Al Muzahmi ◽  
Asif Alrawi
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Tumor ◽  
Clinical Presentation ◽  
Treatment Options ◽  
Dermatofibrosarcoma Protuberans ◽  
High Rate ◽  
Imaging Findings ◽  
Full Extent ◽  
Radiological Appearance ◽  
Characteristic Features

Dermatofibrosarcoma protuberans (DFSP) is a rare soft tumor which originally represents a cutaneous sarcoma. It grows slowly and presents usually as nodular superficial lesion on the trunk or the extremities. Although these tumors are locally aggressive with high rate of recurrence following surgery; the prognosis is considered excellent when it is effectively treated. The radiological appearance of this tumor has rarely been studied and findings infrequently discussed in the literature probably because many lesions underwent resection before imaging. Although imaging is infrequently performed for this lesion; it can show characteristic features and demonstrate the full extent. Imaging may also play a role in the differentiation of this tumor from more serious soft tissue lesions such as more aggressive sarcomas and hemangioma. In this article, we discuss the imaging findings of DFSP that can aid in its diagnosis and its variable appearances. In addition; the clinical presentation and treatment options are also described with review of the previous literature.

scholarly journals Rare case of extra-peritoneal brown fat (hibernoma) mimicking dumbbell-shaped liposarcoma

2021 ◽  
pp. 20200162
Author(s):  
Alex Kiu ◽  
Tiffany Fung ◽  
Sungmi Jung ◽  
Rehana Jaffer ◽  
Marie-Helen Martin
Keyword(s):  
Differential Diagnosis ◽  
Adipose Tissue ◽  
Soft Tissue ◽  
Rare Case ◽  
Clinical Presentation ◽  
Soft Tissue Tumour ◽  
Radiological Features ◽  
Tissue Tumour ◽  
Brown Adipose ◽  
Patient Stress

Hibernomas are a very rare and benign soft tissue tumour that originate from brown adipose tissue. While they are not histologically malignant, they may be indistinguishable from aggressive tumours such as liposarcomas on imaging. It is, therefore, important to consider it as a differential diagnosis when a suspicious fatty lesion is seen on imaging. This may prevent unnecessary invasive surgery and patient stress. This paper illustrates the clinical presentation, radiological features, and histological diagnosis of a patient with a rare dumbbell-shaped hibernoma in the pelvis.

scholarly journals Atopic Eczema Simulating Mammary Paget’s Disease in an Adult: Case Report

10.3823/2546 ◽  
2018 ◽  
Vol 11 ◽  
Author(s):  
Debora Bacellar Cruz Nunes ◽  
Adriana Kamilly Leitão Pitman Machado ◽  
Michelle Fonseca Goiabeira ◽  
Samira Oliveira Silveira ◽  
Yandra Sherring Einecke ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Clinical Presentation ◽  
Present Report ◽  
Paget’S Disease ◽  
Clinical Manifestations ◽  
Breast Tumors ◽  
Paget's Disease ◽  
Adult Case ◽  
Intense Pruritus ◽  
Mammary Paget’S Disease

An atopic breast eczema is usually bilateral, with intense pruritus and good response to corticosteroids. The differential diagnosis with mammary Paget’s disease is fundamental, mainly in unilateral clinical manifestations, since it is frequently associated to adjacent breast tumors. The aim of the present report was to present a clinical condition of an atopic breast eczema through an exuberant and unilateral clinical presentation, emphasizing the need of the differential diagnosis for malignant diseases at this location.

scholarly journals Sinonasal Involvement in Systemic Vasculitides and Cocaine-Induced Midline Destructive Lesions: Diagnostic Controversies

2013 ◽  
Vol 4 (2) ◽  
pp. ar.2013.4.0051 ◽  
Author(s):  
M. Armengot ◽  
A. Garćia-Lliberós ◽  
M. J. Gómez ◽  
A. Navarro ◽  
A. Martorell
Keyword(s):  
Differential Diagnosis ◽  
Clinical Manifestations ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Response To Treatment ◽  
Wegener Granulomatosis ◽  
Systemic Manifestations ◽  
Perinuclear Anca ◽  
Histological Results ◽  
Antibody Levels ◽  
Sinonasal Involvement

Multiple systemic diseases produce various clinical manifestations in the sinonasal area. They usually appear as difficult-to-diagnose disease processes with slow, atypical clinical courses. The aim of this study was to evaluate the sinonasal manifestations of systemic vasculitides, highlighting key points for diagnosis and differential diagnosis with other pathological entities, especially cocaine-induced midline destructive lesions (CIMDL). A retrospective study was performed of 10 patients treated in our hospital during the last 5 years with an initial diagnosis of systemic vasculitides with sinonasal involvement: eight patients with granulomatosis with polyangiitis (GPA; new nomenclature for Wegener granulomatosis) and two patients with Churg-Strauss syndrome (CSS). The study variables were clinical presentation, nasal endoscopy results, maxillofacial scan results, nasal biopsy results, erythrocyte sedimentation rate, and autoimmune antibody levels. The definitive diagnosis was GPA in six (60%) patients, CSS in two (20%) patients, and CIMDL in two (20%) patients. Nasal symptoms were similar in all patients, but nasal polyps were present in only one patient with CSS. Systemic manifestations were absent in patients with CIMDL. Likewise, peripheral eosinophilia was observed only in the two patients with CSS. Specific positive biopsy specimens were obtained in six patients (all six patients with GPA, one with CSS, and one with CIMDL). Antineutrophil cytoplasmic antibodies (ANCA) were positive in all patients with GPA (proteinase 3 antigen in five patients and myeloperoxidase in one patient), and perinuclear ANCA was positive in one patient with CIMDL; however, this patient showed an undefined pattern. Finally, the response to treatment was adequate in all patients excluding those with CIMDL. GPA and CIMDL syndromes pose a difficult differential diagnosis because they have common clinical, serological, and histological presentations. Negative histological results do not exclude the diagnosis of sinonasal vasculitides. The absence of systemic manifestations and the lack of response to treatment will lead to the confirmation of CIMDL syndrome in a cocaine user. Otolaryngologists play an important role in the early and differential diagnosis of these diseases.

A RARE CASE OF ROSAI-DORFMAN DISEASE IN HYPOTHYROIDISM PATIENT A DIAGNOSTIC COEXISTENCE

2021 ◽  
pp. 31-32
Author(s):  
Gridhati Srinivas ◽  
Tarun Kumar Dutta
Keyword(s):  
Weight Loss ◽  
Differential Diagnosis ◽  
Soft Tissue ◽  
Rare Case ◽  
Cervical Lymphadenopathy ◽  
Nodal Involvement ◽  
Nodal Disease ◽  
Rosai Dorfman Disease ◽  
Night Sweats ◽  
Rule Out

Rosai-Dorfman disease (RDD) is a rare, benign, and predominantly nodal disease that most commonly presents as bilateral, painless cervical lymphadenopathy; although inguinal, axillary, mediastinal, and hilar lymphadenopathy has also been reported. Apart from nodal involvement, RDD has extra nodal manifestations involving bone, soft tissue, and liver as well as constitutional symptoms of fever, night sweats, and weight loss, which make it reasonable to rule out the infectious, autoimmune, and malignant conditions as the differential diagnosis.

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