ABSTRACT
Pseudoporphyria (PP) is a relatively infrequent, photodistributed bullous dermatosis that clinically, histopathologically, and immunologically resembles porphyria cutanea tarda (PCT), but is not accompanied by porphyrin abnormalities in the serum, urine, or stool. It was initially described in patients with renal failure on dialysis as 'bullous dermatosis of hemodialysis.' Pseudoporphyria has been seen in patients with end-stage renal disease on hemodialysis. No treatment has proved efficacious in the management of pseudoporphyria. However, N-acetylcysteine has been anecdotally reported to be effective in the management of hemodialysis-related pseudoporphyria and other porphyric diseases. Our patient had developed multiple skin lesions all over the body when hemodialysis started. The lesions were erythematous with fluid-filled vesicles, and bullae with cutaneous fragility that were evaluated and diagnosed as pseudoporphyria. The patient was treated with available all medication in the literature but was not relieved. However, all skin lesions completely healed within 22 days post renal transplantation. Renal transplantation proved to be the cure for dialysis-induced pseudoporphyria resistant to conventional drug therapy.
ABSTRACT
BACKGROUND: Due to the infrequency of non-clear cell renal cell carcinoma (RCC), there is currently a paucity of high-quality literature to help guide the effective treatment of these tumors. Recently, biomarkers such as platelet to lymphocyte ratio (PLR), lymphocyte to monocyte ratio (LMR), systemic immune inflammation (SII) index and C-reactive protein to albumin ratio (CAR) have been demonstrated to be closely related to poor prognosis of patients with RCC. The objective of this study was to evaluate these biomarkers for determining the progression-free survival (PFS) and overall survival (OS) in patients with metastatic non-clear cell cancer. METHODS: We retrospectively reviewed 31 cases diagnosed with metastatic non-clear cell RCC from January 2012 to December 2017. We assessed the prognostic value (OS and PFS) of pretreatment PLR, LMR, SII index and CAR based on multivariate analysis and Kaplan-Meier survival curve. RESULTS: Median time of OS and PFS were 15.5 months (95% confidence interval (CI): 13.7 - 15.2) and 10.9 months (95% CI: 8.9 - 12.8), respectively. The median PFS (0.001) and OS (P = 0.01) was shorter in patients with PLR > 171, LMR < 2.61. Moreover, median PFS but not OS was significantly lower in SII index > 883 (P = 0.064) and CAR > 0.11 (P = 0.229). Scan to surgery time (3.91 weeks, P = 0.001) was also significantly related to progression. CONCLUSIONS: Elevated pretreatment inflammatory biomarkers such as PLR, LMR, SII index and CAR are significant determinants of shorter PFS and OS (PLR and LMR only) in patients with metastatic non-clear cell RCC treated with cytoreductive nephrectomy.
ABSTRACT
BACKGROUND: Penile cancer is a rare malignancy. The extent of lymph node (LN) metastasis is the most important prognostic factor in penile cancer. However, preoperative prediction of LN involvement in clinically non-palpable LN is still a challenge. In absence of a reliable biomarker, attempts are being made to validate imaging characteristics as a predictive tool. The aim of the present study is to assess the primary penile tumor characteristics with diffusion-weighted magnetic resonance imaging (DWMRI) and its correlations with inguinal LN status and tumor positivity in LN dissection specimen within normal sized LNs. METHODS: Twenty-six patients with carcinoma penis underwent DWMRI of penis and pelvis. The apparent diffusion coefficient (ADC) values of primary tumor were compared with histological characteristics. Inclusion criteria encompassed all cases of clinically non-palpable inguinal LN and normal sized LN on imaging. All palpable inguinal nodes with pelvic lymphadenopathies were excluded from this study. RESULTS: The primary tumor ADC ranged from 0.65 × 10-3 - 1.2 × 10-3 mm2/s (mean: 0.87 × 10-3 ± 0.11 × 10-3 mm2/s). In pT1 and pT3 tumors, mean ADC values were 0.86 × 10-3 ± 0.10 × 10-3 and 0.81 × 103 ± 0.09 × 103 mm2/s, respectively. The mean ADC values for grade 1, grade 2 and grade 3 were 0.89 × 10-3, 0.82 × 10-3 and 0.80 × 10-3 mm2/s, respectively. The ADC value of < 0.95 × 10-3 mm2/s was positively correlated with pathological LN presence within normal sized LN. With mean ADC value of 0.87 × 10-3 ± 0.11 × 10-3 mm2/s, sensitivity and positive predictive values for primary penile cancer were 100% and 84.61%, respectively. The mean ADC value for higher-grade and -stage tumor was low. The sensitivity and specificity of predicting LN metastasis by DWMRI were 87.22% and 80.90%, respectively. CONCLUSION: ADC value of primary tumor can help in prediction of LN metastasis in carcinoma penis with clinically and radiologically normal groin.
