scholarly journals Laryngeal Rosai-Dorfman Disease (Sinus Histiocytosis with Massive Lymphadenopathy): A Retrospective Study of 5 Cases

2017 ◽  
Vol 2017 ◽  
pp. 1-6 ◽  
Author(s):  
Yanyan Niu ◽  
Yongjin Li ◽  
Jian Wang ◽  
Xiaofeng Jin ◽  
Dahai Yang ◽  
...  
Keyword(s):  
Surgical Resection ◽  
Age Of Onset ◽  
Clinical Manifestations ◽  
Hormone Treatment ◽  
Good Prognosis ◽  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease ◽  
Alive With Disease ◽  
Laryngeal Involvement

This study was performed to investigate the clinical manifestations, treatment methods, and prognosis of Rosai-Dorfman disease (RDD) with laryngeal involvement. Five clinical cases of RDD with laryngeal involvement diagnosed between 1986 and 2015 were retrospectively analyzed. The laryngeal lesions of these 5 patients mostly involved the glottis and subglottis, with the main symptoms being a hoarse voice and airway obstruction. In addition, the patients mostly exhibited a unilateral or asymmetric onset that was manifested by a laryngeal submucosal nodular mass. The patients were subjected to a regimen of hormone treatment combined with surgical resection. The median follow-up duration was 101 months (8–384 months). One case was lost, and the remaining 4 subjects are alive with disease. The follow-up examinations revealed that 4 subjects had stable laryngeal conditions, whereas one showed minor progression. RDD with laryngeal involvement is clinically rare and differs considerably from classical RDD in age of onset, gender composition, and extranodal involvement. The regimen of hormone treatment combined with surgical resection can stabilize the patient’s general condition and laryngeal lesion. Tracheotomies are recommended for patients with dyspnea. After their conditions stabilize, decannulation can be successfully performed in most cases. This therapeutic regimen generally delivers a good prognosis.

scholarly journals Rosai-Dorfman disease of the subdural spine with a long segment lesion: A case report and literature review

2017 ◽  
Vol 45 (2) ◽  
pp. 875-881 ◽  
Author(s):  
Ji Tu ◽  
Wen-Tian Li ◽  
Cao Yang
Keyword(s):  
Cervical Lymphadenopathy ◽  
Good Prognosis ◽  
Massive Lymphadenopathy ◽  
First Case ◽  
Rosai Dorfman Disease ◽  
Puncture Biopsy ◽  
Appropriate Therapy ◽  
Systemic Manifestations ◽  
Cluster Of Differentiation

Rosai-Dorfman disease (RDD) or sinus histiocytosis with massive lymphadenopathy is a rare benign disorder usually characterized by massive painless cervical lymphadenopathy and systemic manifestations. Extranodal involvement, especially spinal involvement, is extremely rare. We report a 41-year-old man who presented with only intermittent dorsodynia. His condition was diagnosed as non-specific inflammatory disease on the basis of preoperative puncture biopsy results. We performed total surgical resection. Histopathological findings showed distinctive emperipolesis and immunohistochemistry results were positive for cluster of differentiation CD68 and S100 and negative for CD1a. A good prognosis was confirmed at the 3-month follow-up visit. This is the first case of RDD of the subdural spine with such a long segment lesion. There is still no consensus regarding appropriate therapy for this type of RDD and the preoperative diagnosis remains challenging. The unusual presentation of our case serves as a reference when diagnosing and treating RDD.

Hyalinizing clear cell carcinoma of the nasopharynx operated by trans-oral and trans-palatal approach

2015 ◽  
Vol 129 (S2) ◽  
pp. S95-S97 ◽  
Author(s):  
T Nakashima ◽  
R Yasumatsu ◽  
M Yamauchi ◽  
S Toh ◽  
T Nakano ◽  
...  
Keyword(s):  
Salivary Gland ◽  
Cell Carcinoma ◽  
Surgical Resection ◽  
Clear Cell ◽  
Clear Cell Carcinoma ◽  
Minor Salivary Gland ◽  
Good Prognosis ◽  
Salivary Gland Neoplasm ◽  
Hyalinizing Clear Cell Carcinoma

AbstractBackground:Hyalinizing clear cell carcinoma is a rare minor salivary gland neoplasm. The treatment of choice is surgical resection with or without post-operative radiotherapy. This tumour often demonstrates a good prognosis.Case report:We report a case of hyalinizing clear cell carcinoma arising in the nasopharynx. A 27-year-old female presented with progressive hearing disturbance and tinnitus. On examination, an expansile mass was observed in her nasopharynx. Biopsy was performed and the pathology results returned as clear cell carcinoma.Results and conclusion:Surgical resection was performed trans-orally accompanied by trans-palatal approach. She has no recurrence during more than two years of follow up.

scholarly journals Total thymectomy for thymic lymphoepithelioma-like carcinoma—report of two cases

2019 ◽  
Vol 5 (1) ◽  
Author(s):  
Sachi Kawagishi ◽  
Naoko Ose ◽  
Masato Minami ◽  
Soichiro Funaki ◽  
Takashi Kanou ◽  
...  
Keyword(s):  
Surgical Resection ◽  
Thymic Carcinoma ◽  
Clinical Manifestations ◽  
Staging System ◽  
Early Recurrence ◽  
Anterior Mediastinum ◽  
Total Resection ◽  
Distant Lymph Node Metastasis ◽  
Long Term Follow Up

Abstract Background Thymic carcinoma has been classified into 12 subtypes, thymic lymphoepithelioma-like carcinoma (LELC) is a type of them, and has a pathological organization similar to that of lymphoepithelioma, an undifferentiated type of nasopharyngeal carcinoma. According to a report from the International Thymic Malignancy Interest Group (ITMIG), thymic LELC is a rare tumor and accounts for 6% of all thymic carcinoma cases. We report two cases of surgical resection for thymic LELC and perform a search of other reports of thymic LELC, and clinical manifestations and follow-up data thus obtained are summarized. Case presentation Two patients underwent surgical resection for thymic LELC. In both, tumors were detected in the anterior mediastinum and a total thymectomy was performed. Each was diagnosed with thymic LELC and classified in accordance with the Masaoka staging system as modified stage II. In recent examinations, one patient was doing well after undergoing total resection, whereas early recurrence of distant lymph node metastasis was noted in the other at 5 months after the total resection procedure and died thereafter from a different disease. Conclusion We report two cases of surgical resection for thymic LELC. A successful total resection may positively affect prognosis: thus, long-term follow-up examinations must be performed.

Clinical and molecular spectrum associated with Polymerase-γ related disorders

2022 ◽  
pp. 088307382110670
Author(s):  
Ruchika Jha ◽  
Harshkumar Patel ◽  
Rachana Dubey ◽  
Jyotindra N. Goswami ◽  
Chandana Bhagwat ◽  
...  
Keyword(s):  
Age Of Onset ◽  
Pediatric Population ◽  
Single Gene ◽  
Management Strategies ◽  
Clinical Manifestations ◽  
Progressive External Ophthalmoplegia ◽  
Leigh Disease ◽  
Pathogenic Variants ◽  
Phenotype Analysis

Background POLG pathogenic variants are the commonest single-gene cause of inherited mitochondrial disease. However, the data on clinicogenetic associations in POLG-related disorders are sparse. This study maps the clinicogenetic spectrum of POLG-related disorders in the pediatric population. Methods Individuals were recruited across 6 centers in India. Children diagnosed between January 2015 and August 2020 with pathogenic or likely pathogenic POLG variants and age of onset <15 years were eligible. Phenotypically, patients were categorized into Alpers-Huttenlocher syndrome; myocerebrohepatopathy syndrome; myoclonic epilepsy, myopathy, and sensory ataxia; ataxia-neuropathy spectrum; Leigh disease; and autosomal dominant / recessive progressive external ophthalmoplegia. Results A total of 3729 genetic reports and 4256 hospital records were screened. Twenty-two patients with pathogenic variants were included. Phenotypically, patients were classifiable into Alpers-Huttenlocher syndrome (8/22; 36.4%), progressive external ophthalmoplegia (8/22; 36.4%), Leigh disease (2/22; 9.1%), ataxia-neuropathy spectrum (2/22; 9.1%), and unclassified (2/22; 9.1%). The prominent clinical manifestations included developmental delay (n = 14; 63.7%), neuroregression (n = 14; 63.7%), encephalopathy (n = 11; 50%), epilepsy (n = 11; 50%), ophthalmoplegia (n = 8; 36.4%), and liver dysfunction (n = 8; 36.4%). Forty-four pathogenic variants were identified at 13 loci, and these were clustered at exonuclease (18/44; 40.9%), linker (13/44; 29.5%), polymerase (10/44; 22.7%), and N-terminal domains (3/44; 6.8%). Genotype-phenotype analysis suggested that serious outcomes including neuroregression (odds ratio [OR] 11, 95% CI 2.5, 41), epilepsy (OR 9, 95% CI 2.4, 39), encephalopathy (OR 5.7, 95% CI 1.4, 19), and hepatic dysfunction (OR 4.6, 95% CI 21.3, 15) were associated with at least 1 variant involving linker or polymerase domain. Conclusions We describe the clinical subgroups and their associations with different POLG domains. These can aid in the development of follow-up and management strategies of presymptomatic individuals.

scholarly journals Epidemiological and Clinical Characteristics of Children with Coronavirus Disease 2019

2020 ◽  
Author(s):  
Qin Wu ◽  
Yuhan Xing ◽  
Lei Shi ◽  
Wenjie Li ◽  
Yang Gao ◽  
...  
Keyword(s):  
Pediatric Patients ◽  
Clinical Manifestations ◽  
Epidemiological Data ◽  
Good Prognosis ◽  
Pcr Analysis ◽  
Respiratory Pathogens ◽  
Upper Respiratory Tract ◽  
Radiologic Findings ◽  
Epidemiological Characteristics

BackgroundSevere acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is a newly identified pathogen which mainly spreads by droplets. Most published studies focused on adult patients with coronavirus disease 2019 (COVID-19), but data concerning pediatric patients is limited. This study aimed to determine epidemiological characteristics and clinical features of pediatric patients with COVID-19.MethodsWe reviewed and analyzed data on pediatric patients with laboratory-confirmed COVID-19, including basic information, epidemiological history, clinical manifestations, laboratory and radiologic findings, treatment, outcome and follow-up results.ResultsFrom January 20th to February 27th of 2020, a total of 74 pediatric patients with COVID-19 were included in this study. Of the 68 cases whose epidemiological data were complete, 65 (65/68, 95.59%) cases were household contacts of adults whose symptoms developed earlier. Forty (59.46%) of the infected children were male, and the median age and body weight are 6 (0.10-15.08) years and 24 (4.20-87.00) kg, respectively. Except for one critically ill case, 20 (27.03%) patients did not show any symptoms of infection, 24 (32.43%) patients had acute upper respiratory tract infection and 29 (39.19%) patients were diagnosed with mild pneumonia. Cough (24/74, 32.43%) and fever (20, 27.03%) were the predominant symptoms of 44 (59.46%) symptomatic patients at onset of the illness. Abnormalities in leukocyte count were found in 23 (31.08%) children and 10 (13.51%) children presented with abnormal lymphocyte count. Of the 34 (45.95%) patients who had nucleic acid testing results for common respiratory pathogens, 19 (19 / 34, 51.35%) showed co-infection with other pathogens other than SARS-CoV-2.Ten (13.51%) children had RT-PCR analysis of SARS-CoV-2 for fecal specimens and 8 of them showed prolonged existence of SARS-CoV-2 RNA 11 (5-23) days after nasopharynx swabs turning negative. Abnormalities in chest imaging were observed in 37 (50.00%) patients and 28 (37.84%) of them only presented with nontypical changes.All children had good prognosis with a median hospitalization duration of 11 days and follow-up period of 16.5 days. During the follow-up period, all the patients remained in quarantine at designated site and home for two 14-day obervation periods and showed no clinical manifestation,but 3 of the 8 cases with prolonged fecal shedding of SARS-CoV-2 still showed positive result of feces test.

scholarly journals Clinical Characteristics of Nodular Fasciitis of Ear in Children

2021 ◽  
Author(s):  
Xiaoxu Wang ◽  
Wei Liu ◽  
Lejian He ◽  
Min Chen ◽  
Jianbo Shao ◽  
...  
Keyword(s):  
Surgical Resection ◽  
Clinical Characteristics ◽  
Clinical Manifestations ◽  
Neck Surgery ◽  
Final Diagnosis ◽  
Diagnosis And Treatment ◽  
Nodular Fasciitis ◽  
Gene Test ◽  
The Right

Abstract Purpose Summarized the clinical characteristics and diagnosis and treatment process of three cases of nodular fasciitis of ear, to provide a basis for clinical diagnosis and treatment. Methods Reviewed the clinical manifestations, images, pathology, treatment and postoperative follow up results of three cases of pediatric nodular fasciitis in the Department of Otorhinolaryngology, Head and Neck Surgery, Beijing Children's Hospital, Capital Medical University from 2018 to 2020. Results The average age at diagnosis were 24 months, with two girls and a boy. Two lesions were found in the left ear and one in the right ear. All cases had a history of biopsy before surgery. Two of three cases showed a sign of rapid growth after biopsy and three of which were ineffective in anti-inflammatory treatment. FISH test for USP6 were performed in two of the three cases with positive results. Three lesions show a hypointensity or isointensity on T1-weighted MRI and a heterogeneous hyperintensity on T2-weighted MRI. ‘‘Fascial tail’’ sign was found on image of all three cases. All lesions underwent surgical resection. Follow-up showed no recurrence and had an intact ear appearance. Conclusion The early misdiagnosis rate of nodular fasciitis of the ear is high. Combine clinical features with imaging findings may improve the accuracy of preoperative diagnosis. Besides the appearance of pathology, USP6 gene test is also an important tool in the diagnosis. The final diagnosis should be based on comprehensive assessment. Complete surgical resection can prevent recurrence.

Treatment of the Autonomous „Hot“ Nodule of the Thyroid with 131I

1969 ◽  
Vol 08 (04) ◽  
pp. 339-348
Author(s):  
René. Cárdenas ◽  
Juan. Oliva
Keyword(s):  
Radiation Dose ◽  
Thyroid Carcinoma ◽  
Surgical Resection ◽  
Clinical Manifestations ◽  
Good Prognosis ◽  
The Other ◽  
Variable Degree ◽  
Hot Nodule ◽  
Before And After

SummaryThirty-four women with single autonomous “hot” nodules of the thyroid were treated with 131I. There was a variable degree of thyrotoxicosis in 26 cases while in 3 others there was only local cervical discomfort. The total amount administered varied between 2 and 80 mCi of 131I and the radiation dose to the thyroid from 2 to 450 krads. The nodule and symptoms disappeared in 14 of the patients, while in the other 14 the lesion decreased in size and the clinical manifestations improved. Only in six patients was the result unsatisfactory, without change in the size of the nodule. Results are analysed on the basis of the quantity of radioiodine administered.Modifications of the scintigram before and after treatment are discussed, observing that the “cooling” of the nodule and the reappearance of the surrounding gland are signs of good prognosis.A case is described in which the therapy failed and a thyroid carcinoma was found after surgical resection. Two cases of the series in which there were slight transitory manifestations of post-therapeutic hypothyroidism are also reported.

scholarly journals Soft tissue Rosai-Dorfman disease of the posterior mediastinum

2009 ◽  
Vol 35 (7) ◽  
pp. 717-720 ◽  
Author(s):  
Antônio Luiz Penna Costa ◽  
Natália Oliveira e Silva ◽  
Marina Pamponet Motta ◽  
Rodrigo Abensur Athanazio ◽  
Daniel Abensur Athanazio ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Surgical Excision ◽  
Posterior Mediastinum ◽  
Extranodal Involvement ◽  
Surgical Specimen ◽  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease ◽  
History Of ◽  
Paravertebral Mass

Rosai-Dorfman disease (RDD) consists of sinus histiocytosis with massive lymphadenopathy. Extranodal involvement occurs in up to 43% of cases. However, isolated soft tissue RDD is rare. Isolated mediastinal RDD is exceedingly rare, and there have been only three previous reports. Involvement of the posterior mediastinum in RDD has been reported only in the context of disseminated RDD. Here, we report the case of a 49-year-old female patient with a two-year history of cervical pain and lymphadenomegaly, which resolved spontaneously. A CT scan revealed a left paravertebral mass with a diameter of 6 cm. The patient was submitted to surgical excision of the mass. Microscopic examination and immunophenotyping of the surgical specimen led to a diagnosis of RDD. During a 12-month follow-up period, the patient complained of mild cough and chest pain. Periodic imaging tests showed no sign of recurrence, and no postoperative cervical lymphadenomegaly was detected.

scholarly journals The Clinical and Pathological Features of Children With Microscopic Polyangiitis

2021 ◽  
Vol 9 ◽  
Author(s):  
Qian Li ◽  
Li-Chun Yu ◽  
Feng-Xia Li ◽  
Jing Wang ◽  
Yuan Chen ◽  
...  
Keyword(s):  
Microscopic Polyangiitis ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Antithyroid Drug ◽  
Good Prognosis ◽  
Factors Associated ◽  
Stage Renal Disease ◽  
End Stage ◽  
Lung Impairment

Objective: The aim of this study was to explore the clinical features, pathological characteristics, and the prognosis of children with microscopic polyangiitis (MPA).Methods: Ten children with MPA that were hospitalized in our hospital were included in this study. The children's pre-diagnosis status, clinical manifestations, renal pathology, treatment, and prognosis data were analyzed retrospectively.Results: All 10 cases included female patients with a median age of 8.9 years old at the time of diagnosis. MPO-ANCA antibody was positive in all cases, combined with a positive anti-GBM antibody in two cases. Nine cases had primary AAV and one had antithyroid drug (ATD)-associated MPA (secondary to methimazole). Renal involvement was found in all 10 patients, lung impairment was present in eight cases, and anemia was present in nine patients. Renal biopsies were performed in all 10 patients. Segmental focal or global glomerular necrosis was observed in 70% of the patients (7/10). The treatment mainly included steroid use combined with Cyclophosphamide and Mycophenolate. The follow-up s of the patients revealed normal renal function in eight patients and progression to end-stage renal disease (ESRD) in two patients.Conclusions: Female predisposition and positive MPO-ANCA antibody were prominent in children with MPA. The patients' kidneys and lungs were the most frequently involved organs. Corticosteroid combined with immunosuppressive therapy was recommended for the treatment of MPA. Early diagnosis, prompt aggressive treatment, and regular follow-ups are also very important factors associated with a good prognosis.

scholarly journals A Rare Case of Canine Metastatic Spermatocytic Tumor without Sarcomatous and Anaplastic Variants

2021 ◽  
Vol 71 (2) ◽  
pp. 239-246
Author(s):  
Eun-Joo Lee ◽  
Jae-Hyuk Yim ◽  
Myung-Jin Chung ◽  
Jin-Kyu Park ◽  
Il-Hwa Hong ◽  
...  
Keyword(s):  
Surgical Resection ◽  
Rare Case ◽  
Testicular Tumor ◽  
Good Prognosis ◽  
Small Cells ◽  
Histological Feature ◽  
Abdominal Organs ◽  
Histological Variants ◽  
Mitotic Figures

Abstract Spermatocytic tumor is a rare testicular tumor, which is originated from gonocytes. It is characterized by the histological feature of tripartite which is composed of large, medium, and lymphocyte-like small cells. It is well-known that spematocytic tumor is benign, thus a good prognosis is expected after simple resection. Metastatic spermatocytic tumor is extremely rare and usually accompanied by histological variants including sarcomatous changes and anaplastic variants. In this case, however, we report a canine metastatic spermatocytic tumor without prominent sarcomatous changes and anaplastic variants. The mass was composed of three kinds of cells including large, medium, and small cells with high pleomorphism. The neoplastic cells had an indistinct cytoplasmic border and mitotic figures were frequently observed. The primary spermatocytic tumor metastasized to the abdominal organs one month after the resection, and the dog died 13 months after the surgery. Thus, careful follow-up is recommended after surgical resection of canine spermatocytic tumor even though metastasis in spermatocytic tumors is rare.

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