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Radiologia 2019To review the imaging findings for the different types of pulmonary histiocytosis. In particular, in addition to the well-known pulmonary Langerhans cell histiocytosis...
OBJECTIVE
To review the imaging findings for the different types of pulmonary histiocytosis. In particular, in addition to the well-known pulmonary Langerhans cell histiocytosis related to smoking and its possible appearance in nonsmokers, we focus on non-Langerhans cell histiocytosis in Rosai-Dorfman disease and Erdheim-Chester disease. We also review the etiopathogenesis, histology, clinical presentation, and treatment of pulmonary histiocytosis.
CONCLUSION
Langerhans cell histiocytosis, Rosai-Dorfman disease, and Erdheim-Chester disease are idiopathic diseases in which the proliferation and infiltration of histiocytes is the histologic finding that confirms the diagnosis. Langerhans cell histiocytosis manifests as nodules and cysts that spare the costophrenic angles; it typically appears in smokers. Although it is uncommon in nonsmokers, Langerhans cell histiocytosis should also be considered in nonsmokers treated with chemotherapy and radiotherapy in whom cavitated nodules appear and should be included in the differential diagnosis together with metastatic disease and opportunistic infections. Rosai-Dorfman disease and Erdheim-Chester disease present with less specific thoracic findings such as adenopathies, interstitial thickening, and pleural effusion. In Erdheim-Chester disease, the characteristic extrathoracic manifestations are usually key for the diagnosis.
Topics: Adult; Erdheim-Chester Disease; Female; Histiocytosis, Langerhans-Cell; Histiocytosis, Sinus; Humans; Lung Diseases; Male; Middle Aged; Smoking; Tomography, X-Ray Computed; Young Adult
PubMed: 30686482
DOI: 10.1016/j.rx.2018.11.003 -
Pediatric Dermatology 2000Sinus histiocytosis with massive lymphadenitis or Rosai-Dorfman disease (RDD) is a rare, benign, proliferative histiocytic disease of unknown origin. It predominately...
Sinus histiocytosis with massive lymphadenitis or Rosai-Dorfman disease (RDD) is a rare, benign, proliferative histiocytic disease of unknown origin. It predominately affects the lymph nodes The skin is the extranodal organ most frequently involved, although a few patients present with only lesions of the skin and soft tissues, without involvement of the lymph nodes (purely cutaneous RDD). Ocular involvement is rare, and generally localized to the eyelid. We report an unusual case of cutaneous sinus histiocytosis without nodal involvement and with bilateral uveitis in a girl. As far as we know, this is the first case reported in which cutaneous and ocular RDD occurred without nodal involvement.
Topics: Adolescent; Biopsy; Chronic Disease; Diagnosis, Differential; Female; Histiocytosis, Sinus; Humans; Lymph Nodes; Remission, Spontaneous; Skin; Uveitis
PubMed: 11085666
DOI: 10.1046/j.1525-1470.2000.017005377.x -
American Journal of Ophthalmology Mar 1999To report a case of sinus histiocytosis presenting as multiple, bilateral epibulbar masses.
PURPOSE
To report a case of sinus histiocytosis presenting as multiple, bilateral epibulbar masses.
METHODS
We examined a 4-year-old child with a 2-month history of gradually enlarging, salmon-colored epibulbar masses. Excisional biopsies and a systemic evaluation were performed.
RESULTS
Systemic evaluation was normal; no lymphadenopathy was documented. Histopathologic evaluation disclosed features consistent with sinus histiocytosis. Flow cytometry and gene rearrangement studies confirmed the benign nature of the disease. No tumor recurrence was noted.
CONCLUSION
Sinus histiocytosis may present as isolated epibulbar masses that may be treated with surgical excision.
Topics: Child, Preschool; Conjunctival Diseases; Female; Histiocytosis, Sinus; Humans; Immunohistochemistry; Lymphocytes; S100 Proteins
PubMed: 10088757
DOI: 10.1016/s0002-9394(98)00321-3 -
European Archives of... Feb 2023To investigate the clinical features, diagnosis, treatment and prognosis of otolaryngological extranodal RDD.
PURPOSE
To investigate the clinical features, diagnosis, treatment and prognosis of otolaryngological extranodal RDD.
METHODS
A retrospective analysis was performed on 16 patients who were initially diagnosed and treated with otolaryngological extranodal RDD in our hospital from January 2013 to January 2019.
RESULTS
There were 9 females and 7 males. The mean diagnostic age was 45.35. Nasal, laryngeal and otic RDD were, respectively, accounting for 56.25%, 31.25% and 12.5%. The median interval diagnostic time was individually 1, 0.5 and 0.2 year for nasal, laryngeal and otic RDD. The most common symptoms were separately progressive nasal congestion, dyspnea, otorrhea in nasal, laryngeal and otic RDD. 13 had cervical lymph node swelling on MRI. Surgery or postoperative radiotherapy were conformed. In the end, 14 patients with RDD survived. The survival rate is as high as 87.5%. One of them died of RDD in the fifth year. One case was lost to follow-up 2 years after treatment. Patients survive for at least 2 years and up to 9 years. There was no significant difference in life expectancy of extranodal RDD among different parts of ENT (P = 0.508 > 0.05). The average ages of laryngeal and nasal RDD were similar (P = 0.898 > 0.05). However, the age of both was significantly higher than ear RDD (P = 0.023 and 0.019 < 0.05).
CONCLUSIONS
The average diagnostic age was more than 20 years. Nasal RDD was the most common in this area. All laryngeal RDD had infiltrated subglottis. Functional surgery and postoperative radiotherapy can be used to achieve long-term remission and survival.
Topics: Adult; Female; Humans; Male; Young Adult; Histiocytosis, Sinus; Larynx; Magnetic Resonance Imaging; Prognosis; Retrospective Studies
PubMed: 36153784
DOI: 10.1007/s00405-022-07646-7 -
Vnitrni Lekarstvi 2016Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is a very rare disease belonging to a group of histiocytoses (more precisely non-Langerhans cell...
UNLABELLED
Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is a very rare disease belonging to a group of histiocytoses (more precisely non-Langerhans cell histiocytoses). Rosai-Dorfman disease is characterised by the presence of atypical histiocytic cells in the sinuses of lymph nodes or in the extranodal lymphoid tissue, absorbing lymphocytes and plasma cells. The structure and function of the absorbed cells is not impaired and they can leave histiocytes as viable cells. This effect is called emperipolesis, whereas ingestion of cells with their destruction is called phagocytosis. In our text we describe a patient with this disease located, characteristically, in supraclavicular lymph nodes, but also in mediastinal lymph nodes. Along with lymphadenopathy skin alterations appeared which were both clinically and histologically described as eczema dermatitis. At the same time as lymphadenopathy also strong headaches started which the patient had never suffered before. Within the first-line treatment prednisone was administered, but no effect was achieved. 2-chlorodeoxyadenosine in 5 mg/m2 s. c. dose was used in the second-line treatment, for 5 successive days in monthly intervals. There were four cycles of this treatment administered overall. Therapy was tolerated without any manifestations of toxicity. Already after the 1st cycle skin alterations as well as headaches entirely disappeared. To assess the effect of treatment the PET/CT examination with 18F-fluorodeoxyglucose (FDG-PET/CT) was made. After 4 cycles of treatment the mediastinal lymph nodes diminished to a physiological size and the accumulation of fluorodeoxyglucose in them was assessed as physiological. Lymphadenopathy in the neck area also significantly diminished by 50-75 % and the accumulation of fluorodeoxyglucose was reduced as well, though it did not reach the norm. Therefore we evaluate the effect of treatment as a partial remission with complete disappearance of skin alterations and headaches. The cause of the eczema and headaches has not been clarified, however considering the same time of their arising and then disappearance after the application of 2-chlorodeoxyadenosine the causal connection with Rosai-Dorfman disease is likely.
KEY WORDS
Castlemans disease - lenalidomide - Rosai-Dorfman disease - rituximab - sinus lymphadenopathy with massive lymphadenopathy - thalidomide - 2-chlorodeoxyadenosine.
Topics: Cladribine; Histiocytes; Histiocytosis, Sinus; Humans; Immunosuppressive Agents; Lymph Nodes; Lymphadenopathy; Positron Emission Tomography Computed Tomography
PubMed: 27485849
DOI: No ID Found -
Journal of Korean Medical Science Aug 2009Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare disorder characterized by a nonneoplastic proliferation of distinctive histiocyte cells within lymph...
Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare disorder characterized by a nonneoplastic proliferation of distinctive histiocyte cells within lymph node sinuses and lymphatics in extranodal sites. SHML occurs worldwide and is primarily a disease of childhood and early adulthood. A 26-yr-old man presented with painless palpable lymph node in cervical area. Radiographic studies revealed pleural effusion with lymphadenopathy and calcification in mediastinum. The cervical lymph node biopsy showed dilated sinuses filled with histiocytes with clear cytoplasm. The cells stained positive with CD68 and S-100. These cytologic and immunohistochemical findings were considered consistent with the diagnosis of SHML.
Topics: Adult; Antigens, CD; Antigens, Differentiation, Myelomonocytic; Histiocytes; Histiocytosis, Sinus; Humans; Lymph Nodes; Male; Neck; Pleural Effusion; S100 Proteins; Tomography, X-Ray Computed
PubMed: 19654967
DOI: 10.3346/jkms.2009.24.4.760 -
Zhurnal Voprosy Neirokhirurgii Imeni N.... 2022Histiocytosis is a group of idiopathic diseases accompanied by metabolic disorders and accumulation of metabolic products in histiocytes. Isolated Rosai-Dorfman... (Review)
Review
Histiocytosis is a group of idiopathic diseases accompanied by metabolic disorders and accumulation of metabolic products in histiocytes. Isolated Rosai-Dorfman histiocytosis of central nervous system is observed in less than 5% of cases. The authors report treatment and follow-up of a patient with intracranial Rosai-Dorfman disease. There were symptoms of lesion of the left cerebellopontine angle and epileptic seizures. Preoperative MRI identified two tumors (posterior cranial fossa on the left and right-sided parasagittal neoplasm). The authors carried out total resection of supratentorial tumor, after 3 weeks - subtotal resection of tumor in posterior cranial fossa. No recurrence after total resection was observed. Irradiation of infratentorial tumor with a total focal dose of 50 Gy after 6 months resulted tumor shrinkage throughout 12 months. Radiotherapy with the same dose was repeated throughout subsequent 12-month follow-up period due to progression of this focus. This treatment had a positive effect, but new skull base foci occurred. The authors emphasize the effectiveness of total resection and lower efficiency of subtotal excision combined with radiotherapy.
Topics: Diagnosis, Differential; Histiocytosis, Sinus; Humans; Magnetic Resonance Imaging; Skull Base
PubMed: 35170280
DOI: 10.17116/neiro20228601181 -
Nuclear Medicine Review. Central &... 2014A 23-year-old lady presented with abdominal fullness and distension as well as large abdominal masses in physical exam. Upon ultrasonographic evaluation, two large space...
A 23-year-old lady presented with abdominal fullness and distension as well as large abdominal masses in physical exam. Upon ultrasonographic evaluation, two large space occupying lesions anterior to kidneys, with no clear distinction from renal tissue, as well as bilateral hydronephrosis were found. The findings were confirmed by MRI. Histopathological analysis of renal masses and lymph nodes showed proliferation of histiocytes mixed with occasional multinucleated giant cells, immunostaining of which was positive for S-100 and CD68. We present renal scintigraphy features of this rare case of renal sinus histiocytosis with massive intraabdominal lymphadenopathy (Rosai-Dorfman disease).
Topics: Female; Histiocytosis, Sinus; Humans; Kidney; Magnetic Resonance Imaging; Radionuclide Imaging; Ultrasonography; Young Adult
PubMed: 24610654
DOI: 10.5603/NMR.2014.0011 -
Giornale Italiano Di Dermatologia E... Jun 2016
Review
Topics: Histiocytosis, Sinus; Humans; Male; Middle Aged; Skin Diseases
PubMed: 25812620
DOI: No ID Found -
Human Pathology May 1993We report 14 cases of extranodal sinus histiocytosis with massive lymphadenopathy involving a variety of head and neck sites. The patients ranged in age from 3 to 70...
We report 14 cases of extranodal sinus histiocytosis with massive lymphadenopathy involving a variety of head and neck sites. The patients ranged in age from 3 to 70 years (median, 43 years). Nine cases occurred in women and five occurred in men. The clinical presentation varied depending on the site of occurrence and included nasal obstruction, stridor, proptosis, ptosis, decreased visual acuity, facial pain or tenderness, cranial nerve deficits, mandibular tenderness, and mass lesions. Head and neck sites involved by disease included the nasal cavity, paranasal sinuses, nasopharynx, parotid gland, submandibular gland, larynx, temporal bone, infratemporal fossa, pterygoid fossa, meninges, and orbital region. The majority of patients presented with involvement of more than one site. Nodal involvement was identified in four patients. Special stains for microorganisms were negative. The sinus histiocytosis with massive lymphadenopathy cells demonstrated an immunophenotypic profile supporting derivation from macrophage/histiocytic lineage. Treatment varied and included surgical excision with or without adjuvant therapy (chemotherapy, radiotherapy) or steroids. Several patients required more extensive surgery as a result of extension of their disease to adjacent structures or due to recurrent disease. Twelve patients are alive and either free of disease or have persistent disease. Two patients died, one as a result of complications of disease.
Topics: Adolescent; Adult; Aged; Child, Preschool; Female; Follow-Up Studies; Head; Histiocytosis, Sinus; Humans; Immunohistochemistry; Lymphatic Diseases; Male; Middle Aged; Neck
PubMed: 8491488
DOI: 10.1016/0046-8177(93)90160-i