The presence of two light chain bands on immunofixation is associated with poor outcomes in newly diagnosed multiple myeloma patients.

We aimed to describe the clinical and biological characteristics and the prognosis of patients presenting with an additional light chain (LC) band along with a complete monoclonal protein on immunofixation (IF).An 8-year descriptive study was conducted to assess all cases with confirmed monoclonal gammopathies (MG). We studied those with an entire M-protein with 2 bands of LC of the same isotype based on the results of IF. Data were collected from patients' files.Among 548 cases of MG, we found 32 cases (5.8%) with an additional LC band. We included 28 patients (5%) with a confirmed diagnosis of multiple myeloma (MM). The m/f ratio was 2.5 with a median age of 63 years [32-80 years]. All MM patients had anemia, 16 (57%) had renal failure, 14 (50%) had lytic lesions, 9 (32%) received hemodialysis, and 7 (25%) had hypercalcemia. The free-kappa-lambda ratio was abnormal in all cases: median = 0.07 [0.002-58.57]. The mean overall survival (OS) was 22 months ± 38.76.Fifteen MM patients (48%) received chemotherapy, and 7 (22%) autologous stem cell transplants (SCT). Patients who received SCT had an OS higher than those who received other treatments (p = 0.038). OS was low in patients with high ß2microglobulin levels (rho = -0.791; p = 0.001), and abnormally low free-kappa-lambda ratio (rho = -0.852;p = 0.04).The presence of an additional LC band with a complete monoclonal protein seems to identify newly diagnosed MM patients with poor outcomes and frequent renal impairment.

Clinical behavior and survival outcome of urothelial bladder cancer in young adults.

Background: Bladder cancer (BC) is rare in young adults and therefore natural history of BC is still debatable. This study aimed to determine clinical behavior and prognosis of BC in patients <40 years. Materials and Methods: We reviewed patients (<40 years) managed with urothelial BC from 2003 to 2019. Patients with nonurothelial histology were excluded. Clinical behavior and prognosis such as recurrence, progression, and survival were assessed. The recurrence is defined as a newly diagnosed occurrence of BC at previous or new site(s). Cancer progression is defined as an increase in staging or grade. Results: Fifty-five patients inclusive of 45 males and 10 females with a median age of 30.0 (interquartile range [IQR] 25.0-33.0) years were included. The median follow-up was 3.5 (IQR: 1.5-7.0) years. Fifty-one (92.72%) patients were diagnosed with nonmuscle-invasive BC while four (7.27%) patients were diagnosed with muscle-invasive disease. Three out of four patients with muscle-invasive BC died of metastatic disease. According to stage and grade, there were 42 (76.36%) Ta, 9 (16.36%) T1 and 4 (7.27%) having T2 stage while 41 (74.54%) low grade and 14 (25.45%) were having high grade disease. Thirty-six (65.45%) patients remained stable, 13 (26.63%) patients progressed, and 6 (10.90%) patients regressed to lower stage and grade. Higher stage and grade (P = 0.0431) and tumor size >3 cm (P = 0.0454) were significant for recurrence, and higher stage and grade (P = 0.0012) and tumor size >3 cm (P = 0.0055) were associated with tumor progression. Conclusion: BC in younger adults is mostly low stage and low grade. We should be vigilant in patients with higher stage and grade as it is related with recurrence, progression, and metastatic disease.

Testicular fibroma: A case report and literature review.

Sex cord-stromal tumors are the second most common testicular tumors after germ cell tumors. They account for about 2%-5% of adult testicular tumors. Most of these tumors are benign. The most common sex cord-stromal tumor is Leydig cell tumor. In contrast, testicular fibroma is a very rare type of sex cord-stromal tumors. Histologically, testicular fibromas resemble their ovarian counterparts; however, they are much less common than ovarian fibromas. To the best of our knowledge, <50 cases of testicular fibromas are reported in the English literature. Herein, we report a rare case of testicular fibroma with acellular collagen plaque in a 51-year-old male presenting as a painless testicular mass.

Trends in the surgical management of renal cell carcinoma in a contemporary tertiary care setting.

BACKGROUND: In the last three eras, the incidence of renal cell carcinoma (RCC) has increased, due to increased radiological studies. The expected 5-year survival rate has become better, associated with the identification of small size renal masses. However, this survival improvement may be secondary to improved surgical techniques and medical therapies for these malignancies. OBJECTIVES: The objective was to report the trends of clinical presentation, peri-operative, oncological outcomes, and surgical management trends for RCCs over the period. METHODS: After Institutional Review Board approval, a retrospective study for adult patients was conducted, who presented with renal mass and were managed between 2008 and 2019. Variables, including demographics, perioperative and pathological outcomes analyzed using descriptive statistics for continuous variables reported as mean ± standard deviation and categorical variables values compared by Chi-square test. Survival Analysis calculated using the Kaplan-Meier method. The level of significance is set at P-value < 0.05. RESULTS: A total of 588 patients underwent surgical treatment for kidney cancer from January 2008 to January 2019. 237 (40.30%) were females and 351 (59.69%) males. The clinical presentation was higher as an incidental diagnosis of 58.67%. 71.25% of patients were from outside Riyadh city. Pathology was mostly clear cell RCC 61.22% and grade 2 (57.48%). Tumor size, surgery time, and length of hospital stay showed a significant difference between the three periods (both P > 0.05). Robotic surgery performed more than open (P < 0.0001). There was no significant difference in the survival time, when compared to patients by the regions and when compared by the primary tumors (Log-Rank P = 0.4821). Patients from the Riyadh region (median = 54.0) had a significantly higher recurrence time (Log-Rank P < 0.0001). CONCLUSION: There was a rising trend in the incidence of RCC associated with comorbidities and incidental diagnosis. In our study period we found increase in the trend of minimal invasive approach. The size of the tumor, blood loss and operative time decreases over the period of time. The Robotic assisted nephrectomy approach has become increased over the period of time duration in present study.

An unusual case report of pulmonary adenoid cystic carcinoma metastasis to the kidney. Case report and literature review.

Adenoid cystic carcinoma (ACC) is a malignant neoplasm, frequently affecting the salivary glands, and rarely occurring in other locations. ACC is characterized by slow growth, perineural invasion, local and late recurrence after original treatment. However, renal metastasis of ACC is very rare. To our best knowledge, only 11 cases of ACC metastasis to the kidney have been reported in the English literature to date. Herein, we presented a rare case of a 70-year-old man with renal metastasis from ACC of the right lung after 3 years of primary presentation. Our patient underwent right radical nephrectomy and histologically confirmed as Metastatic Adenoid Cystic Carcinoma.

Familial lecithin-cholesterol acyltransferase (LCAT) deficiency; a differential of proteinuria.

BACKGROUND: Lecithin cholesterol acyltransferase (LCAT) is an important enzyme in cholesterol metabolism that is involved in the esterification of cholesterol. A lack of this enzyme results in deranged metabolic pathways that are not completely understood, resulting in abnormal deposition of lipids in several organs. Clinically, it manifests with proteinuria, dyslipidemia and corneal opacity with progressive chronic kidney disease resulting in end-stage renal disease. CASE PRESENTATION: We herein present a case of a 30-year-old male with proteinuria that was not responsive to empiric management with angiotensin-converting enzyme (ACE) inhibitors and oral steroids. Physical examination revealed corneal ring opacity involving both eyes. Urinalysis revealed an active sediment. The 24-h proteinuria was 3.55 grams. Family history was positive for renal disease and dyslipidemia. Viral serology for human immunodeficiency virus (HIV), hepatitis C virus (HCV) and hepatitis B virus (HBV) were negative. Serum complements were normal and anti-nuclear antibody (ANA) was negative. We elected for a renal biopsy that revealed characteristic features of LCAT deficiency. The diagnosis of LCAT deficiency was established with a combination of clinical and pathological findings. CONCLUSIONS: Currently renal prognosis is poor but conservative management with ACE inhibitors and lipid lowering therapy in addition to steroids has been shown to retard progression to end-stage renal disease. However newer therapies such as gene replacement and recombinant LCAT replacement are being studied with promising preliminary results.

Poorly differentiated ovarian sertoli-leydig cell tumor in a 16-year-old single woman: a case report and literature review.

Sertoli-Leydig cell tumor (SLCT) of ovary is an exceedingly unusual neoplasm that belongs to a group of sex cord-stromal tumors of ovary and accounts for less than 0.5% of all primary ovarian neoplasms. Very few case reports have been documented in the literature so far. Herein, we report a case of primary poorly differentiated ovarian Sertoli-Leydig cell tumor (SLCT) involving the left ovary in a 16-year-old single woman who presented with a 3-month history of a pelviabdominal mass, acne, hirsutism, and menstrual irregularities. In addition, a literature review on ovarian SLCTs is provided.

Posterior fossa tumor in a 2 year-old girl.

We report a case of a 2 year-old girl who presented with three weeks' history of deterioration of walking, then became unable to walk and later she developed projectile vomiting. Neurological examination revealed bilateral papilledema, nystagmus, and truncal ataxia with intention tremor. Radiological studies showed an enhancing mass in the posterior fossa extending from the cerebellum to the roof of the fourth ventricle. The tumor was diagnosed as an embryonal tumor with abundant neuropil and true rosettes (ETANTR). The tumor cells in the neuroblastic component were diffusely positive for synaptophysin and CD56, with scattered positive cells for glial fibrillary acidic protein. The true rosettes were only positive for vimentin. Ki67 showed high index (over 90%) in the true rosettes, while the neuroblastic areas were up to 15%. Our patient developed recurrent disease 6 months after resection and chemotherapy. ETANTR is a very rare aggressive embryonal CNS tumor that combines features of neuroblastoma and ependymoblastoma. We review the thirteen cases reported in the literatures. This case represents the second report of an ETANTR arising in the cerebellum.