Prevalence, clinical characteristics, and management of silicone lymphadenopathy: A systematic review of the literature.

INTRODUCTION: Implant-based breast augmentations and reconstructions are one of the most common surgical procedures performed by plastic surgeons in the United States, which has rapidly increased in popularity since the 2000s. Silicone lymphadenopathy (SL) is a complication of breast implants that involves migration of silicone to nearby soft tissue/lymph nodes. Data on its clinical features and management is scarce. METHODS: SL-related search terms were used to find articles in 3 databases. Of 598 articles, 101 studies met the inclusion criteria. Demographics, clinical presentation, workup, and management data were analyzed. RESULTS: Of 279 cases of SL and 107 with information on initial diagnosis, 35 (33%) were incidental. The most common symptom was painless lymphadenopathy, followed by painful lymphadenopathy. 251 (95%) and 13 (5%) patients had silicone and saline implants, respectively. 149 (68%) patients had implant rupture. Axillary lymphadenopathy was the most affected region (136 cases, 72%), followed by internal mammary (40 cases, 21%), cervical/supraclavicular (36 cases, 19%), and mediastinal (24 cases, 13%) regions. 25% of patients underwent fine-needle aspiration, 12% core needle biopsy, and 59% excisional biopsy. 32% of cases underwent explantation and/or implant exchange. The most common indication for surgery was implant rupture. Histology showed multinucleated giant cells, large histiocytes, and silicone accumulation. CONCLUSIONS: SL is a complication associated with breast implants. The majority of patients are asymptomatic, and most cases are managed conservatively. Minority need a biopsy and surgical interventions due to abnormal imaging, persistent symptoms, and/or implant rupture. Workup and management should be tailored to the patient.

Spectrum of B-cell neoplasms associated with immunoglobulin G4-related disease.

Immunoglobulin G4-related disease (IgG4-RD) has rarely been associated with lymphoid neoplasms, the spectrum of which remains unclear. B-cell lymphoid neoplasms (LN) associated with IgG4-RD diagnosed in a 4-year period were analysed. There were five men and three women at a median age of 76.5 (52-90) years; three with synchronous IgG4-RD and LN; three with IgG4-RD preceding LN by 2, 3, and 22 years; and two with LN preceding IgG4-RD by 2.5 and 7 years. All patients presented with disseminated lymphadenopathy. Monoclonal gammopathy of undetermined significance (MGUS)/smouldering multiple myeloma (SMM) was found in three patients, all with an IgGκ paraprotein. Levels of IgGκ and IgG4 correlated. Diffuse large B-cell lymphoma (DLBCL) was found in three patients, with one case showing co-existing lymphoma and IgG4-RD in the same lymph node biopsy. The remaining two cases were marginal zone lymphoma (MZL) developing in a lacrimal gland previously involved by IgG4-RD; and nodular lymphocyte predominant Hodgkin lymphoma (NLP-HL) diagnosed in a lymph node with concomitant IgG4-RD. Low-dose continuous prednisolone was given for MGUS/SMM, with both monoclonal IgGκ and IgG4 responding. Combination chemotherapy was given for DLBCL, with two patients achieving complete response and one patient dying from refractory lymphoma. The patient with MZL refused treatment, whereas the case of NLP-HL responded completely to chemotherapy. Our findings together with previous observations suggest that IgG4-RD has an increased risk of B-cell neoplasms. Patients with IgG4-RD presenting with lymphadenopathy require vigorous investigations to exclude lymphoid neoplasms.

Patient with H syndrome, cardiogenic shock, multiorgan infiltration, and digital ischemia.

BACKGROUND: H syndrome (HS) is a rare autoinflammatory disease caused by a mutation in the solute carrier family 29, member 3 (SCL29A3) gene. It has a variable clinical presentation and little phenotype-genotype correlation. The pathognomonic sign of HS is cutaneous hyperpigmentation located mainly in the inner thighs and often accompanied by other systemic manifestations. Improvement after tocilizumab treatment has been reported in a few patients with HS. We report the first patient with HS who presented cardiogenic shock, multiorgan infiltration, and digital ischemia. CASE PRESENTATION: 8-year-old boy born to consanguineous parents of Moroccan origin who was admitted to the intensive care unit during the Coronavirus Disease-2019 (COVID-19) pandemic with tachypnoea, tachycardia, and oliguria. Echocardiography showed dilated cardiomyopathy and severe systolic dysfunction compatible with cardiogenic shock. Additionally, he presented with multiple organ dysfunction syndrome. SARS-CoV-2 polymerase chain reaction (PCR) and antibody detection by chromatographic immunoassay were negative. A previously ordered gene panel for pre-existing sensorineural hearing loss showed a pathological mutation in the SCL29A3 gene compatible with H syndrome. Computed tomography scan revealed extensive alveolar infiltrates in the lungs and multiple poor defined hypodense lesions in liver, spleen, and kidneys; adenopathy; and cardiomegaly with left ventricle subendocardial nodules. Invasive mechanical ventilation, broad antibiotic and antifungal coverage showed no significant response. Therefore, Tocilizumab as compassionate use together with pulsed intravenous methylprednisolone was initiated. Improvement was impressive leading to normalization of inflammation markers, liver and kidney function, and stabilising heart function. Two weeks later, he was discharged and has been clinically well since then on two weekly administration of Tocilizumab. CONCLUSIONS: We report the most severe disease course produced by HS described so far in the literature. Our patient's manifestations included uncommon, new complications such as acute heart failure with severe systolic dysfunction, multi-organ cell infiltrate, and digital ischemia. Most of the clinical symptoms of our patient could have been explained by SARS-CoV-2, demonstrating the importance of a detailed differential diagnosis to ensure optimal treatment. Although the mechanism of autoinflammation of HS remains uncertain, the good response of our patient to Tocilizumab makes a case for the important role of IL-6 in this syndrome and for considering Tocilizumab as a first-line treatment, at least in severely affected patients.

Primary intratesticular rhabdomyosarcoma, a rare entity: A case report with review of literature.

Paratesticular rhabdomyosarcoma is a very rare mesenchymal tumor. It is an intrascrotal tumor that is localized in paratesticular structures such as the epididymis or spermatic cord. Rhabdomyosarcoma is most often observed in children and adolescents, presenting as a painless scrotal mass. An 18-year-old man presented with a painless left scrotal mass and lump abdomen that had evolved over four months. A histological examination of the lesion revealed rhabdomyosarcoma. Chemotherapy with alternative cycles of Vincristine, Adriamycin, Cyclophosphamide followed by Ifosphamide, Etoposide was given. Paratesticular rhabdomyosarcoma is a rare aggressive tumor manifesting in children and very young adults. Localized forms have a good prognosis whereas metastatic tumors show very poor results. A well-defined treatment based on surgery and chemotherapy yields good results.

A 63-Year-Old Woman With an Acute Exacerbation of Interstitial Pneumonia.

CASE PRESENTATION: A 63-year-old, non-smoking Asian woman presented to our hospital due to abnormal findings on chest radiography. She had no history of dust exposure. Chest radiography and CT imaging showed patchy ground-glass attenuation (GGA) in the bilateral lower lung lobes, a ground-glass nodule in the right lower lung lobe (diameter, 9.8 mm), and some thin-walled cysts in both lungs (Fig 1). Thickening of the interlobular septa, mediastinal lymphadenopathy, and pleural effusion were not evident. Video-assisted thoracic surgery was performed for the examination of the nodule and the background lung disease, and the nodule was histologically diagnosed as lung adenocarcinoma. Simultaneously, the lung background showed diffuse lymphocytic infiltration in the alveolar septum and peribronchovascular interstitium (Fig 2). There were no symptoms suggestive of autoimmune diseases such as dryness, arthralgia, skin rash, or fever. The patient was followed up without treatment for the interstitial lung disease.

Mediastinal lymphadenopathy: a practical approach.

Introduction: Mediastinal lymphadenopathy is secondary to various benign and malignant etiologies. There is a variation in the underlying cause in different demographic settings. The initial clue to the presence of enlarged mediastinal lymph nodes is through thoracic imaging modalities. Malignancy (Lung cancer, lymphoma, and extrathoracic cancer) and granulomatous conditions (sarcoidosis and tuberculosis) are the most common causes. For a confident diagnosis, the clinician must choose from several available options and integrate the clinical, radiological, and pathology findings. An accurate diagnosis is necessary for optimal management.Areas covered: We performed a search of the PUBMED database to identify relevant articles on the causes, imaging modalities, and interventional modalities to diagnose these conditions. We discuss a practical approach toward the evaluation of a patient with mediastinal lymphadenopathy.Expert opinion: Mediastinal lymphadenopathy is a commonly encountered clinical problem. Treating physicians need to be aware of the clinico-radiological manifestations of the common diagnostic entities. Selecting an appropriate tissue diagnosis modality is crucial, with an intent to use the least invasive technique with good diagnostic yield. Endosonographic modalities (EBUS-TBNA, EUS-FNA, and EUS-B-FNA) have emerged as the cornerstone to most patients' diagnosis. An accurate diagnosis translates into favorable treatment outcomes.

[Lymphadenopathy in general practice]. / Lymfadenopathie in de eerste lijn.

Lymphadenopathy (painfull or enlarged lymph nodes) is a common reason for consulting a physician working in primary or secondary health care. Lymphadenopathy can be the reason for consultation, but can also be observed in patients who present with other complaints. The differential diagnosis is very broad and varies from self-limiting benign disorders, where a wait-and-see policy is sufficient, to a more serious and life-threatening disease for which no further delay is warranted. In daily practice it can sometimes be challenging to determine which policy is indicated. In this article, we propose tools in order to assist the primary care physician to determine which policy is needed in patients presenting with lymphadenopathy.

Surgical Experience of Video-Assisted Mediastinoscopy for Nonlung Cancer Diseases.

BACKGROUND: Video-assisted mediastinoscopy (VAM) is a valuable method in the investigation of diseases with mediastinal lymphadenopathy or those localized in the mediastinum. The aim of this study was to determine the diagnostic value of VAM in the investigation of mediastinal involvement of nonlung cancer diseases and to describe our institutional surgical experience. METHODS: Clinical parameters such as age, sex, histological diagnosis, morbidity, and mortality of all patients who underwent VAM for the investigation of mediastinal involvement of diseases except lung cancer between January 2006 and July 2018 were retrospectively reviewed, and the diagnostic efficacy of VAM was determined statistically. RESULTS: During the study period, 388 patients underwent VAM, and 536 lymph nodes were sampled for histopathological evaluation of mediastinum due to mediastinal lymphadenopathy or paratracheal lesions. The most common diagnoses were sarcoidosis (n = 178 [45.9%]), tuberculous lymphadenitis (n = 108 [27.8%]), lymphadenitis with anthracosis (n = 72 [18.6%]), and lymphoma (n = 15 [3.9%]). CONCLUSION: The results of the study show that VAM should be used because of its high diagnostic benefit in mediastinal lymphadenopathies, which are difficult to diagnose, or mediastinal lesions located in the paratracheal region. Despite the increase in the number of new diagnostic modalities, VAM is still the most effective method and a gold standard.

Three Cases of Pediatric Multisystem Inflammatory Syndrome Associated with COVID-19 Due to SARS-CoV-2.

BACKGROUND Coronavirus disease 2019 (COVID-19) infection commonly presents as fever, cough, and shortness of breath in adults. Children are thought to have milder respiratory symptoms and to recover more quickly. We describe a new presentation of COVID-19 infection in children consisting of multisystem inflammation with decreased left ventricular function and evidence of lung disease. CASE REPORT Three children presented with fever, conjunctivitis, dry and cracked lips, rash, and/or cervical lymphadenopathy for at least 5 days. Two of these children required mechanical ventilation, and 1 of the 2 needed extracorporeal membrane oxygenation (ECMO) to support cardiorespiratory function. All of these children had moderate to severe hyponatremia and lymphopenia, which is usually seen in COVID-19. They were treated with intravenous immunoglobulin and high-dose aspirin. All of the children recovered. CONCLUSIONS Early recognition of children with multisystem inflammation is important because they are at increased risk for deterioration. Treatment with intravenous immunoglobulin and aspirin was used because this regimen has been shown to be beneficial in vasculitis of Kawasaki disease. The development of shock due to cardiac involvement may require ECMO.

Management and outcomes of sinus histiocytosis with massive lymphadenopathy (Rosai Dorfman Disease).

Sinus histiocytosis with massive lymphadenopathy (Rosai Dorfman Disease [RDD]), is a rare, benign but clinically heterogeneous histiocytic disorder. Our aims were to analyze the clinical characteristics of the disease and explore the outcomes of patients with RDD followed at our institution. Between January 2000 and February 2019, there were 15 patients with a pathologically confirmed diagnosis of RDD. Median age at diagnosis was 48 years old (range 26-78). The majority (87%, n = 13) of the patients had extranodal disease. Frontline approaches included surgical intervention/complete excision (n = 5, 33%), rituximab monotherapy (n = 5, 33%), observation (n = 3, 20%), and radiation (n = 2, 13%). Two of the five patients underwent surgical excision and were subsequently treated with rituximab. Of the 7 patients who were given rituximab, 64% remained progression free 24 months after the initial rituximab administration. Our review parallels previous reports and highlights rituximab as a favorable option for therapy if ineligible for surgery or radiation.

Lymph node abscess caused by Francisella tularensis - a rare differential diagnosis for cervical lymph node swelling: a case report.

BACKGROUND: Cervical lymph node swelling is quite a common symptom mainly caused by infections in the face or as metastasis of a malignant tumor. In infection cases in particular, rare diseases should never be overlooked. With an incidence of 120 cases in the United States of America (USA) and approximately 25 cases in Germany per year, infection with the pathogen Francisella tularensis is one of these rare diseases that can cause massive lymph node swellings and might even be fatal. CASE PRESENTATION: The example of a healthy 67-year-old German woman who was treated at our university hospital presents a typical progression of a localized form of tularemia. The pathogen could be identified in a universal 16S ribosomal deoxyribonucleic acid (DNA) polymerase chain reaction. Pathogen-specific treatment with lymph node abscess incision, daily rinsing of the abscess cavity, and orally administered antibiotic therapy with doxycycline could cure our patient completely without any remaining complications. CONCLUSION: In patients with cervical lymph node swelling caused by infection it is indispensable to perform specific identification of the pathogen for further local and specific antibiotic treatment. Possible infections with atypical bacteria like Francisella tularensis should never be ignored. Universal polymerase chain reactions are a suitable method for early detection of such rare pathogens.

Recurrence of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome after tonsillectomy: case-based review.

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is a recurrent fever syndrome for which tonsillectomy is a therapeutic option curing the disease in most patients. Recurrence after remission with tonsillectomy is extremely rare. Increasing number of reports on diverse disease manifestations in PFAPA could give us clues about the disease etiopathogenesis. We aimed to describe a patient with recurrence of PFAPA syndrome after tonsillectomy and to review the previous studies including similar cases. We report a 17-year-old boy with PFAPA syndrome who experienced remission for 3 years after tonsillectomy and was later found to harbor an MEFV mutation when the disease relapsed. He responded well to colchicine treatment at relapse. The literature review revealed 14 articles describing 24 similar PFAPA patients. The therapeutic options include single-dose corticosteroids and nonsteroidal anti-inflammatory drugs during attacks, cimetidine, and resurgery. The presented case was the only one heterozygous for an MEFV mutation and treated with colchicine at disease relapse. Albeit rare, the reoccurrence of PFAPA after tonsillectomy could occur. The presence of such patients opposes with the hypothesis that the trigger or immune dysregulation in PFAPA pathogenesis resides in tonsils.

A Rare Case of Pediatric Lacrimal Sac: Squamous Cell Carcinoma.

A case of a 6-year-old boy presenting with epiphora, medial canthal swelling, and lymphadenopathy is reported. Biopsy revealed a poorly differentiated squamous cell carcinoma. The child underwent neoadjuvant chemotherapy and showed tumor progression after a remarkable initial response. This case highlights the atypical age of presentation of potentially fatal lacrimal sac malignancy. Further, it emphasizes the importance of knowing signs of lacrimal sac malignancy among ophthalmologists treating childhood epiphora where inflammatory etiologies are most common. A high index suspicion, thorough clinical examination, and knowledge of the signs of malignancy may avoid delay in diagnosis of this rare but life-threatening malignancy. [J Pediatr Ophthalmol Strabismus. 2019;56:e8-e11.].