Unnecessary orchiectomy due to atypical sarcoidosis manifesting as a unilateral scrotal mass: a case report and literature review.

Sarcoidosis is a disease characterised primarily by lung tissue involvement. Extrapulmonary involvement, particularly in the genitourinary tract, is extremely rare, particularly when it comes to primary disease detection in this location. The gold standard in establishing a definitive diagnosis of sarcoidosis is a combination of the clinical picture, the results of imaging methods, and histopathological examination from the biopsy taken (thus ruling out other causes of granulomatous inflammation). However, it is common for the biopsy to be infeasible or for the patient to refuse such an examination, resulting in the neglect of this critical verification. We introduce the case of a young 29-year-old man of Czech nationality who had been complaining for some time about non-specific pain above the pubic bone and in the lower abdomen, which was combined with a painless enlargement of the right half of the scrotum. Due to suspected malignancy, it was, after considering clinical, imaging, and laboratory findings, decided to perform a radical orchiectomy as a treatment option. The histological examination revealed that it was not cancer, but rather a rare genitourinary form of extrapulmonary sarcoidosis. In this case, radical resection had been, therefore, unnecessary. We also present a review of the literature on published extrapulmonary, genitourinary, and testicular sarcoidosis cases. All the above demonstrates the importance of considering a possible atypical sarcoidosis manifestation and histological confirmation before pursuing radical solutions.

Sudden onset of sarcoidosis after successful surgical treatment of Cushing's syndrome.

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[A Case of Two-Stage Operation for Esophageal Cancer with Sarcoidosis].

A 69-year-old man with dysphagia was diagnosed with advanced esophageal cancer by upper gastrointestinal endoscopy. He had undergone pancreatic tail and partial transverse colon resection for pancreatic cancer, and right hilar lymph node biopsy and partial lower lobe resection for the diagnosis of pulmonary sarcoidosis. Contrast-enhanced computed tomography(CT)scan showed no change over time in lymph node enlargement in the mediastinum, so metastasis of esophageal cancer was considered to be negative. Therefore, the diagnosis of advanced esophageal cancer, Mt, type 2, T2N0M0, cStage Ⅱ, was made, and surgery was performed after 2 courses of DCF therapy. Because of the adhesions in the thoracic cavity and possible problems with elevation of the gastric tube and blood flow due to resection of the pancreatic tail, it was decided to perform two-stage operation. Although imaging studies over time, as in the present case, can help in the diagnosis, it is difficult to distinguish whether enlarged lymph nodes are reactive changes or metastases. In this study, we experienced a case of thoracic esophageal cancer complicated by sarcoidosis with enlarged mediastinal lymph nodes.

Outcomes of catheter ablation for ventricular tachycardia in patients with sarcoidosis: Insights from the National Inpatient Sample database (2002-2018).

INTRODUCTION: Data on utilization, major complications, and in-hospital mortality of catheter ablation (CA) for sarcoidosis-related ventricular tachycardia (VT) are limited. We sought to determine the outcomes of sarcoidosis-related VT, and incidence and predictors of complications associated with the CA procedure. METHODS: We queried the 2002-2018 National Inpatient Sample database to identify patients aged ≥18 years with sarcoidosis admitted with VT. A 1:3 propensity score-matched (PSM) analysis was used to compare patient outcomes between CA and medically managed groups. Multivariable regression was performed to determine independent predictors of in-hospital mortality and procedural complications associated with the CA procedure. RESULTS: Of 3220 sarcoidosis patients with VT, 132 (4.1%) underwent CA. Patients who underwent CA were younger, male predominant, more likely Caucasian, had differences in baseline comorbidities including more likely to have heart failure, less likely to have prior myocardial infarction, COPD, or severe renal disease, had a higher mean household income, and more likely admitted to a larger/urban teaching hospital. After PSM, we examined 106 CA cases and 318 medically managed cases. There was a trend toward a lower in-hospital mortality rate in the CA group when compared to the medically managed group (1.9% vs. 6.6%, p = 0.08). The most common complications were pericardial drainage (5.3%), postoperative hemorrhage (3.8%), accidental puncture periprocedure (3.0%), and cardiac tamponade (2.3%). Independent predictors of in-hospital mortality and procedural complications among the CA group included congestive heart failure (odds ratio [OR], 13.2; 95% confidence interval [CI], 1.7-104.2) and mild to moderate renal disease (OR, 3.9; 95% CI, 1.1-13.3). CONCLUSIONS: Compared to patients with sarcoidosis-related VT who received medical therapy alone, those who underwent CA have a trend for a lower mortality rate despite procedure-related complications occurring as high as 9.1%. Additional studies are recommended to better evaluate the benefits and risks of VT ablation in this group.

Cochlear Implantation Outcomes in Patients With Retrocochlear Pathology: A Systematic Review and Pooled Analysis.

OBJECTIVE: To review the current literature regarding cochlear implantation in patients with retrocochlear pathologies and extract speech perception scores between 6 months and 1 year after surgery. DATABASES REVIEWED: PubMed/MEDLINE, Embase and Cochrane CENTRAL via Ovid, CINAHL Complete via Ebsco, and Web of Science. METHODS: The review was conducted according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Search strategies included keywords and subject headings to maximize retrieval and reflect cochlear implants and retrocochlear pathologies. Patients with previously resected vestibular schwannoma (VS) were excluded. RESULTS: There were 2,524 abstracts screened against inclusion criteria, and 53 studies were included, with individual data available for 171 adult patients. Pathologies included were either observed or irradiated VS (previously operated tumors were excluded) (n = 99, 57.9%), superficial siderosis (n = 39, 22.8%), neurosarcoidosis (n = 11, 6.4%), and previous central nervous system or skull base radiation (n = 22, 12.9%). Mean (standard deviation) postoperative consonant-nucleus-consonant (CNC) word scores were 45.4% (24.2) for observed VS, 44.4% (20.8) for irradiated VS, 43.6% (21.0) for superficial siderosis, 89.5% (3.0) for neurosarcoidosis, and 30.0% (30.2) in patients with previous central nervous system or skull base irradiation. Irradiated compared with observed VS had similar postoperative CNC word scores (effect size, 0.06; p = 0.71). Age, sex, maximal tumor dimension, and neurofibromatosis type 2 status did not significantly impact cochlear implant performance in patients with VS. Eighty-two percent of patients with reported device usage were daily users, and overall, 82% of cases benefitted from cochlear implantation. CONCLUSION: Cochlear implantation in patients with concomitant retrocochlear pathology generally results in improved speech discrimination scores sustained over time.

First Case Described in the Medical Literature: Male Urethral Sarcoidosis. Description of a Case and Review of the Literature.

OBJECTIVE: Although the sarcoidosis is a multisystemic disease that theoretically can affect almost any organ, the presence of sarcoidosis in the male urethra has not been described in the medical literature. We present the first male case of urethral sarcoidosis. METHOD: A 46 years old male undergoing follow up due to lower urinary tract symptoms was diagnosed of endobronchial sarcoidosis during the preoperative study for internal urethrotomy. After surgery, he presented clinical improvement for one year. Given the worsening, a new internal urethrotomy was tried. As it was impossible due to complexity they took a biopsy of the urethra. The pathology report described non-caseating granulomas compatible with sarcoidosis. After that, medical and endoscopic management of the urethral sarcoidosis was attempted. As it didn't achieve an adequate control, the patient was derivate to the "complex urethral unit" of the Cruces University Hospital. Once it was valuated, it was decided to start immunotherapy and subsequently an urethroplasty with a double oral mucosa graft was performed. OUTCOMES: During the postoperative period, a urethral catheter was maintained for two weeks. It was removed after no urinary leakage was observed in de cystourethrography. After that the patient remains with good evolution until today. CONCLUSIONS: Urethral affectation by sarcoidosis is a therapeutic challenge itself. For a better symptom control and to reduce the recurrences, a dual approach using systemic treatment in combination with local surgical treatment seems necessary.

First Case Described in the Medical Literature: Male Urethral Sarcoidosis. Description of a Case and Review of the Literature

Objective: Although the sarcoidosis is a multisystemic disease that theoretically can affect almost any organ, the presence of sarcoidosis in the male urethra has not been described in the medical literature. We present the first male case of urethral sarcoidosis. Method: A 46 years old male undergoing follow up due to lower urinary tract symptoms was diagnosed of endobronchial sarcoidosis during the preoperative study for internal urethrotomy. After surgery, he presented clinical improvement for one year. Given the worsening, a new internal urethrotomy was tried. As it was impossible due to complexity they took a biopsy of the urethra. The pathology report described non-caseating granulomas compatible with sarcoidosis. After that, medical and endoscopic management of the urethral sarcoidosis was attempted. As it didn't achieve an adequate control, the patient was derivate to the "complex urethral unit" of the Cruces University Hospital. Once it was valuated, it was decided to start immunotherapy and subsequently an urethroplasty with a double oral mucosa graft was performed. Outcomes: During the postoperative period, a urethral catheter was maintained for two weeks. It was removed after no urinary leakage was observed in de cystourethrography. After that the patient remains with good evolution until today. Conclusions: Urethral affectation by sarcoidosis is a therapeutic challenge itself. For a better symptom control and to reduce the recurrences, a dual approach using systemic treatment in combination with local surgical treatment seems necessary (AU)

Objetivo: Pese a que la sarcoidosis es una enfermedad multisistémica que teóricamente puede afectar a casicualquier órgano, no ha sido descrita en la literatura médicala presencia de sarcoidosis en uretra masculina. Presentamos el diagnóstico y tratamiento del primer caso masculinode sarcoidosis uretral.Método: Varón de 46 años en seguimiento por clínicamiccional de vaciado al que se le diagnosticó de sarcoidosis endobronquial durante el estudio preoperatorio para larealización de una uretrotomía interna. Tras la intervenciónpresentó mejoría clínica durante un año. Ante el empeoramiento, se intentó repetir una nueva uretrotomía internasiendo esta imposible y decidiéndose realizar una biopsia.El informe anatomopatológico describió granulomas no caseificantes compatibles con sarcoidosis. Posteriormente seintentó un manejo médico y endoscópico de la sarcoidosis uretral que no logró un adecuado control por lo que sederivó a la unidad de uretra compleja del Hospital Universitario Cruces. Ante los hallazgos se decidió comenzar coninmunoterapia y posteriormente se realizó una uretroplastiacon doble injerto de mucosa oral.Resultado: Durante el postoperatorio se mantuvo unasonda uretral durante dos semanas. Posteriormente se retirótras la realización de una cistouretrografía miccional seriada donde no se apreciaba fuga urinaria permaneciendo elpaciente con buena evolución hasta hoy en día.Conclusiones: La afectación uretral por sarcoidosissupone actuar ante una patología que de por sí es un retoterapéutico. Para un mejor control sintomatológico y reducir las recidivas parece necesario un abordaje doble mediante un tratamiento sistémico farmacológico en combinación con tratamiento quirúrgico local (AU)

Meta-Analysis of Catheter Ablation Outcomes in Patients With Cardiac Sarcoidosis Refractory Ventricular Tachycardia.

Cardiac sarcoidosis (CS) frequently leads to ventricular tachycardia (VT), which is often refractory to antiarrhythmic and/or immunosuppressive medications and requires catheter ablation. We conducted a systematic review and meta-analysis to evaluate the role of catheter ablation in patients with refractory VT undergoing catheter ablation. We searched PubMed, Embase, and Scopus databases from their inception to December 31, 2021 with search terms "cardiac sarcoidosis" AND "electrophysiological studies OR ablation." Fifteen studies were ultimately included for evaluation. Patient demographics, VT mapping, and acute and long-term procedural outcomes were extracted. A total of 15 studies were included in our meta-analysis, with a total of 401 patients, of whom 66% were male, with ages ranging from 39 to 64 years. A total of 95% of patients were on antiarrhythmics and 79% of patients were on immunosuppressants. Left ventricular ejection fraction ranged from 35% to 49% and procedure duration ranged from 269 to 462 minutes. Ablation was reported using both irrigated and nonirrigated catheter tips. A total of 25% of patients (84/339) underwent repeat ablation. Acute procedural success was achieved in 57% (161/285). Procedure complications occurred in 5.7% (17/297) procedures. VT recurrence after first ablation was 55% (confidence interval 48% to 63%, 213/401); VT recurrence after multiple ablations was 37% (81/220). The composite end point of death, heart transplant, and left ventricular assist device implantation was 21% (confidence interval 14% to 30%, 55/297). In conclusion, catheter ablation is a useful modality in patients with CS with refractory VT. However, patients with CS presenting with refractory VT after undergoing VT ablation carry a poor prognosis.

Short- and long-term outcomes after heart transplantation in cardiac sarcoidosis and giant-cell myocarditis: a systematic review and meta-analysis.

Heart transplantation (HTx) is a valid therapeutic option for end-stage heart failure secondary to cardiac sarcoidosis (CS) or giant-cell myocarditis (GCM). However, post-HTx outcomes in patients with inflammatory cardiomyopathy (ICM) have been poorly investigated. We searched PubMed, Scopus, Science Citation Index, EMBASE, and Google Scholar, screened the gray literature, and contacted experts in the field. We included studies comparing post-HTx survival, acute cellular rejection, and disease recurrence in patients with and without ICM. Data were synthesized by a random-effects meta-analysis. We screened 11,933 articles, of which 14 were considered eligible. In a pooled analysis, post-HTx survival was higher in CS than non-CS patients after 1 year (risk ratio [RR] 0.88, 95% confidence interval [CI] 0.60-1.17; I2 = 0%) and 5 years (RR 0.72, 95% CI 0.52-0.91; I2 = 0%), but statistically significant only after 5 years. During the first-year post-HTx, the risk of acute cellular rejection was similar for patients with and without CS, but after 5 years, it was lower in those with CS (RR 0.38, 95% CI 0.03-0.72; I2 = 0%). No difference in post-HTx survival was observed between patients with and without GCM after 1 year (RR 1.16, 95% CI 0.05-2.28; I2 = 0%) or 5 years (RR 0.98, 95% CI 0.42-1.54; I2 = 0%). During post-HTx follow-up, recurrence of CS and GCM occurred in 5% and 8% of patients, respectively. Post-HTx outcomes in patients with CS and GCM are comparable with cardiac recipients with other heart failure etiologies. Patients with ICM should not be disqualified from HTx.

Heart transplantation outcomes in cardiac sarcoidosis.

BACKGROUND: Cardiac sarcoidosis (CS) is a progressive inflammatory cardiomyopathy that can lead to heart failure, arrhythmia, and death. There is limited data on Orthotopic Heart Transplantation (OHT) outcomes in patients with CS. Here we examine outcomes in patients with CS who have undergone OHT at centers throughout the United States from 1987 to 2019. METHODS: This was an analysis of 63,947 adult patients undergoing OHT captured in the United Network for Organ Sharing (UNOS) registry. Patients were characterized as cardiac sarcoidosis (CS) or Non-CS. Baseline characteristics were compared using chi-square and Kruskal-Wallis Tests. Outcomes of interest included primary graft failure, patient survival, treated graft rejection, hospitalization for infection, and post-transplant malignancy. RESULTS: During the study period 227 patients with CS underwent OHT. Patients with CS were younger, had higher proportion of non-white patients, and received transplants at more urgent statuses. After multivariable modeling there was no difference in survival (HR 0.86, CI 0.59-1.3, p = 0.446) or graft failure (HR 0.849, CI 0.58-1.23, p = 0.394) between patients with CS and Non-CS. Patients with CS had lower odds of rejection (OR 0.558, CI 0.315- 0.985, p = 0.0444). Patients with CS had similar odds of hospitalization for infection and post-transplant malignancy, as Non-CS patients. CONCLUSIONS: Patients with CS and Non-CS had similar post OHT survival, odds of graft failure, hospitalizations for infection, and post-transplant malignancy. Results of this study confirm the role of heart transplantation as a viable option for patients with CS.

Long-term survival after heart transplantation for cardiac sarcoidosis.

BACKGROUND: Cardiac sarcoidosis is an increasingly common indication for a heart transplant, but there is a paucity of knowledge with regard to long-term outcomes following transplant. METHODS: We utilized the Organ Procurement and Transplantation Network database to retrospectively analyze adult patients undergoing first-time, single-organ heart transplant between January 1999 and March 2020. RESULTS: Of the 41,447 patients that underwent heart transplant during the study period, 289 (0.7%) were transplanted for a primary diagnosis of restrictive cardiomyopathy due to cardiac sarcoidosis (RCM-Sarcoidosis). RCM-Sarcoidosis was associated with 33% reduced risk of mortality over 10 years compared to non-RCM indications in a multivariable Cox proportional hazards model (p = .03). Ten-year survival functions were improved among RCM-Sarcoidosis compared to this reference group (73.4% [64.2%-80.6%] vs. 59.5% [58.8%-60.1%], p = .002). Among patients transplanted after 1999 who had at least 10 years of follow-up (n = 19,489), median survival of RCM-Sarcoidosis patients was 11.9 [8.3-14.6] years while that of non-RCM patients was 9.9 [4.0-13.1] years. RCM-Sarcoidosis was not associated with an increased risk of secondary outcomes such as graft failure, rejection, or infection. The incidence of retransplant was comparable between RCM-Sarcoidosis and non-RCM patients (1.38% vs. 1.50%, p = .93). CONCLUSIONS: These data suggest that long-term outcomes following transplant for cardiac sarcoidosis are favorable compared to heart transplant for other indications.

[A Case of Sarcoid‒Like Reaction with Multiple Lymphadenopathy by Rectal Cancer after an Operation].

We are reporting on a case of lymphadenopathy after surgery for rectal cancer. The case was a 66‒year‒old female. Laparoscopic high anterior resection(D3 dissection)was performed for rectal cancer(pT1bpN0M0, pStage Ⅰ)in April 2018, and she was followed up with on an outpatient basis. In July of the same year, a painless mass had formed in the right groin. An abdominal contrast‒enhanced CT showed lymph node swelling around the right groin and external iliac artery, but the tumor markers, CEA 2.3 ng/mL and CA19‒9 <2 U/mL, were within the standard values. An inguinal lymph node biopsy was performed during the same month. Pathological examination revealed no cancer cells and formation of epithelioid granuloma with giant cells. There was no suspicion of systemic sarcoidosis based on the test results and clinical findings. From the above, the patient was diagnosed with sarcoid reaction due to the tumor. Abdominal contrast‒enhanced CT scan 2 months after the biopsy showed lymph node shrinkage and there was no recurrence 2 years after the biopsy.

Natural History of Patients Diagnosed with Cardiac Sarcoidosis at Left Ventricular Assist Device Implantation or Cardiac Transplantation.

To our knowledge, natural history has not been reported for cardiac sarcoidosis (CS) diagnosed by pathologic evaluation of the apical core at left ventricular assist device (LVAD) implantation or cardiac transplantation. We retrospectively identified 232 consecutive patients meeting CS criteria. Of these patients, 54 were diagnosed by pathologic confirmation of CS, 10 after evaluation of the apical core (LVAD implant) or explanted heart (transplant). We compared clinical characteristics at initial evaluation and outcomes for these 10 patients with those of 10 patients with known CS before LVAD implant/transplant. In the study group, five patients (50%) had confirmed extracardiac sarcoidosis before LVAD implant/transplant; five had not been diagnosed with sarcoidosis. Mean (standard deviation) left ventricular ejection fraction at initial evaluation was 23% (16%), and left ventricular end-diastolic dimension was 61 (10) mm. Four patients died during follow-up; however, no survival difference was found for the 10 patients diagnosed incidentally and the group with a previous diagnosis or institutional LVAD/transplant cohorts. Patients diagnosed with CS on pathological examination of the apical core/explanted heart may have severe dilated cardiomyopathy as the initial presentation. Outcomes for patients with CS after advanced heart failure therapies may be comparable with those of non-CS patients.