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Overexpression of cytokine receptor-like factor 2 (CRLF2) resulting from its genomic rearrangement is the most frequent genetic alteration found in Philadelphia chromosome-like (Ph-like) B-cell acute lymphoblastic leukemia (B-ALL), a high-risk leukemia. Detection of CRLF2 expression by multiparameter flow cytometry has been proposed as a screening tool for the identification of Ph-like B-ALL. However, the prognostic relevance of flow cytometric expression of CRLF2 in pediatric B-ALL is not very clear. Additionally, its association with common copy number alterations (CNA) has not been studied in detail. Hence, in this study, we prospectively evaluated the flow cytometric expression of CRLF2 in 256 pediatric B-ALL patients and determined its association with molecular features such as common CNAs detected using Multiplex ligation-dependent probe amplification and mutations in CRLF2, JAK2 and IL7RA genes. Further, its association with clinicopathological features including patient outcome was assessed. We found that 8.59% (22/256) pediatric B-ALL patients were CRLF2-positive at diagnosis. Among CNAs, CRLF2 positivity was associated with presence of PAX5 alteration (P=0.041). JAK2 and IL-7R mutations were found in 9% and 13.6% CRLF2-positive patients, respectively. IGH::CRLF2 or P2RY8::CRLF2 fusions were each found in 1/22 individuals. CRLF2-positive patients were found to have inferior overall (hazard ratio (HR) =4.39, P=0.006) and event free survival (HR=2.62, P=0.045), independent to other clinical features. Furthermore, concomitant CNA of IKZF1 in CRLF2 positive patients was associated with a greater hazard for poor overall and event free survival, compared to patients without these alterations or presence of any one of them. Our findings demonstrate that the surface CRLF2 expression in association with IKZF1 copy number alteration can be used to risk stratify pediatric B-ALL patients.

Author(s): Charu Paruthi [1]; Rohini Gupta Ghasi (corresponding author) [1]; Rachna Sehgal [2]; Amitabh Singh [2] Sir, Diffuse leptomeningeal glioneuronal tumor (DLGNT) is a recently described entity. Due to its [...]

Byline: Vivek. Vasudeo, Amitabh. Singh, Ashish. Khanna, Sudhir. Rawal, Sarbartha. Pratihar, Nikhil. Saurabh, Bhuwan. Kumar, Mujahid. Ali, Prerit. Sharma, Shravika. Akotkar, Girish. Sharma, Bhagyashri. Kesarwani Introduction: We retrospectively compared surgical [...]

Byline: Sarbartha. Pratihar, Ashish. Khanna, Vivek. Vasudeo, Rahul. Gupta, Nikhil. Saurabh, Bhuwan. Kumar, Mujahid. Ali, Shravika. Akotkar, Sudhir. Rawal, Amitabh. Singh Introduction: Transrectal ultrasound (TRUS) guided systematic prostate biopsy is [...]

Byline: Vivek. Vasudeo, Ashish. Khanna, Sarbartha. Pratihar, Jiten. Jaipuria, Arnab. Chakraborty, Sudhir. Rawal, Amitabh. Singh Objectives: To present our intermediate to long-term oncological and functional outcomes of robot-assisted retroperitoneal lymph [...]

Renal cell carcinoma (RCC) accounts for 2% of all cancer cases worldwide, and majority are sporadic. The latest World Health Organization (WHO) classification of renal cell tumors (fifth edition, 2022) has molecularly defined renal tumor entities, which includes fumarate hydratase (FH)–deficient RCC. FH‐deficient RCC is an aggressive carcinoma caused by pathogenic alterations in FH gene, seen in 15% of patients with hereditary leiomyomatosis and renal cell cancer syndrome (HLRCC) syndrome. These tumors occur more frequently at a younger age and present at an advanced stage, carrying a dismal prognosis. We report a series of 10 cases of FH‐deficient RCC. The mean age was 49.8 years, and all cases presented in advanced stages (III and IV). Morphologically, the cases had varied architectural patterns with characteristic eosinophilic macronucleoli and perinucleolar halo. On immunohistochemistry (IHC), all showed diffuse nucleo‐cytoplasmic expression of S‐(2‐succino)‐cysteine (2‐SC), with loss of FH in seven cases. FH‐deficient RCCs are aggressive neoplasms and can be diagnosed using specific IHC markers (FH and 2‐SC). These patients should undergo germline testing for FH gene mutation, genetic counseling, and surveillance of family members. [ABSTRACT FROM AUTHOR]

Objective: To report outcomes of multicenter series of penile cancer patients undergoing robot-assisted video endoscopic inguinal lymph node dissection (RA-VEIL). Materials and Methods: In this retrospective analysis from 3 tertiary care centers in India, consecutive intermediate-/high-risk carcinoma penis (CaP) patients with nonpalpable inguinal lymphadenopathy and/or nonbulky (<3 cm) mobile inguinal lymphadenopathy undergoing RA-VEIL were included. Patients with matted/bulky (>3 cm) and fixed lymphadenopathy were excluded. Demographic, clinical, and intraoperative data were recorded. Perioperative complications were graded by the Clavien–Dindo classification (CDC). The International Society of Lymphology (ISL) {0-III} grading was used for the assessment of lymphedema. Incidence and pattern of recurrences were assessed on follow-up. Results: From January 1, 2011, to September 30, 2023, 115 patients (230 groins) underwent bilateral RA-VEIL for CaP. The median age of the cohort was 60 (50–69) years. Clinically palpable (either unilateral or bilateral) inguinal lymphadenopathy was seen in 54 patients (47%). The "per groin" median operative time was 120 (100–140) minutes with median lymph node yield of 12 (9–16). No complications were recorded in 87.8% groins operated, with major complications (CDC 3) seen in 2.6% groins. At a median follow-up of 13.5 months, 13 patients had documented recurrences and there were 10 cancer-related deaths. No port-site recurrences were observed. No/minimal lymphedema (ISL 0/I) was seen in 94% legs. Conclusion: RA-VEIL demonstrates safety and oncologic efficacy in penile cancer patients presenting with clinically nonpalpable and/or nonbulky inguinal lymphadenopathy, with favorable functional outcomes. [ABSTRACT FROM AUTHOR]

Byline: Amitabh. Singh, Ashish. Khanna, Jiten. Jaipuria, Shashikant. Gupta, Sarbartha. Pratihar, Vivek. Vasudeo, Rahul. Gupta, Sudhir. Rawal Purpose: Despite widespread acceptance of robotics in urology, literature on using the minimally [...]