Safety and Efficacy of 177Lu-DOTATATE in Children and Young Adult Population: A Single-Center Experience.

PURPOSE: This single-center retrospective study explores the safety and efficacy of 177Lu-DOTATATE in children and young adult population with metastatic/inoperable neuroendocrine tumors (NETs). PATIENTS AND METHODS: This study is a retrospective analysis of all children and young adult patients (≤29 years) with advanced inoperable/metastatic epithelial or nonepithelial NETs who were administered a median of 4 cycles of 177Lu-DOTATATE therapy and low-dose oral capecitabine as a radiosensitizer every 8-12 weeks, except 2 patients who received CAPTEM chemotherapy. The radiological response was assessed using RECIST 1.1 on interim and end-of-treatment 68Ga-DOTANOC PET/CT. The primary endpoint was objective response rate, whereas disease control rate, toxicity profile, progression-free survival, and overall survival were secondary endpoints. RESULTS: Nineteen biopsy-proven NET patients (median age, 22 ± 10 years) with 8 of them adolescents (10-18 years) and the remaining young adults (19-29 years) were included. Fourteen patients had gastroenteropancreatic neuroendocrine tumor (pancreas being most common primary site), whereas the rest had non-gastroenteropancreatic neuroendocrine tumor. A total of 65 cycles of 177Lu-DOTATATE (range, 1-6 cycles) were administered with a median cumulative activity of 600 mCi (range, 100-1000 mCi). The objective response rate and disease control rate were 41% and 94%, respectively. Grade 1 and 2 adverse events were observed in 14 (74%) and 5 (26%) of 19 patients, respectively. In a total of 8 events (42%), 4 events each of disease progression and death occurred during a median follow-up of 80.1 months with an estimated 5-year progression-free survival and overall survival of 54% (95% confidence interval, 30-78) and 63% (95% confidence interval, 39-87), respectively. CONCLUSIONS: 177Lu-DOTATATE appears safe and effective in children and young adults with metastatic/inoperable NETs. Large prospective trials are required to validate these results.

Deep-learning enabled ultrasound based detection of gallbladder cancer in northern India: a prospective diagnostic study.

Background: Gallbladder cancer (GBC) is highly aggressive. Diagnosis of GBC is challenging as benign gallbladder lesions can have similar imaging features. We aim to develop and validate a deep learning (DL) model for the automatic detection of GBC at abdominal ultrasound (US) and compare its diagnostic performance with that of radiologists. Methods: In this prospective study, a multiscale, second-order pooling-based DL classifier model was trained (training and validation cohorts) using the US data of patients with gallbladder lesions acquired between August 2019 and June 2021 at the Postgraduate Institute of Medical Education and research, a tertiary care hospital in North India. The performance of the DL model to detect GBC was evaluated in a temporally independent test cohort (July 2021-September 2022) and was compared with that of two radiologists. Findings: The study included 233 patients in the training set (mean age, 48 ± (2SD) 23 years; 142 women), 59 patients in the validation set (mean age, 51.4 ± 19.2 years; 38 women), and 273 patients in the test set (mean age, 50.4 ± 22.1 years; 177 women). In the test set, the DL model had sensitivity, specificity, and area under the receiver operating characteristic curve (AUC) of 92.3% (95% CI, 88.1-95.6), 74.4% (95% CI, 65.3-79.9), and 0.887 (95% CI, 0.844-0.930), respectively for detecting GBC which was comparable to both the radiologists. The DL-based approach showed high sensitivity (89.8-93%) and AUC (0.810-0.890) for detecting GBC in the presence of stones, contracted gallbladders, lesion size <10 mm, and neck lesions, which was comparable to both the radiologists (p = 0.052-0.738 for sensitivity and p = 0.061-0.745 for AUC). The sensitivity for DL-based detection of mural thickening type of GBC was significantly greater than one of the radiologists (87.8% vs. 72.8%, p = 0.012), despite a reduced specificity. Interpretation: The DL-based approach demonstrated diagnostic performance comparable to experienced radiologists in detecting GBC using US. However, multicentre studies are warranted to explore the potential of DL-based diagnosis of GBC fully. Funding: None.

Validation of a Zero-shot Learning Natural Language Processing Tool to Facilitate Data Abstraction for Urologic Research.

BACKGROUND: Urologic research often requires data abstraction from unstructured text contained within the electronic health record. A number of natural language processing (NLP) tools have been developed to aid with this time-consuming task; however, the generalizability of these tools is typically limited by the need for task-specific training. OBJECTIVE: To describe the development and validation of a zero-shot learning NLP tool to facilitate data abstraction from unstructured text for use in downstream urologic research. DESIGN, SETTING, AND PARTICIPANTS: An NLP tool based on the GPT-3.5 model from OpenAI was developed and compared with three physicians for time to task completion and accuracy for abstracting 14 unique variables from a set of 199 deidentified radical prostatectomy pathology reports. The reports were processed in vectorized and scanned formats to establish the impact of optical character recognition on data abstraction. INTERVENTION: A zero-shot learning NLP tool for data abstraction. OUTCOME MEASUREMENTS AND STATISTICAL ANALYSIS: The tool was compared with the human abstractors in terms of superiority for data abstraction speed and noninferiority for accuracy. RESULTS AND LIMITATIONS: The human abstractors required a median (interquartile range) of 93 s (72-122 s) per report for data abstraction, whereas the software required a median of 12 s (10-15 s) for the vectorized reports and 15 s (13-17 s) for the scanned reports (p < 0.001 for all paired comparisons). The accuracies of the three human abstractors were 94.7% (95% confidence interval [CI], 93.8-95.5%), 97.8% (95% CI, 97.2-98.3%), and 96.4% (95% CI, 95.6-97%) for the combined set of 2786 data points. The tool had accuracy of 94.2% (95% CI, 93.3-94.9%) for the vectorized reports and was noninferior to the human abstractors at a margin of -10% (α = 0.025). The tool had slightly lower accuracy of 88.7% (95% CI 87.5-89.9%) for the scanned reports, making it noninferior to two of three human abstractors. CONCLUSIONS: The developed zero-shot learning NLP tool offers urologic researchers a highly generalizable and accurate method for data abstraction from unstructured text. An open access version of the tool is available for immediate use by the urologic community. PATIENT SUMMARY: In this report, we describe the design and validation of an artificial intelligence tool for abstracting discrete data from unstructured notes contained within the electronic medical record. This freely available tool, which is based on the GPT-3.5 technology from OpenAI, is intended to facilitate research and scientific discovery by the urologic community.

Potential role of 68Ga-PSMA PET/CT in metastatic renal cell cancer: A prospective study.

PURPOSE: Prostate-specific membrane antigen (PSMA), in addition to its utility in prostate cancer, is also an angiogenic imaging marker for hypervascular tumors like renal cell carcinoma (RCC). Our study aims to assess the potential role of 68Ga-PSMA-11 positron emission tomography (PET)/CT in metastatic RCC and compare it with contrast-enhanced computed tomography (CECT). METHODS: Biopsy-proven RCC patients with known or suspected distant metastases who underwent 68Ga-PSMA-11 PET/CT for staging/restaging were prospectively recruited. Those patients who had undergone 18F-FDG PET/CT within six weeks of 68Ga-PSMA PET/CT were also included retrospectively for comparative analysis. A patient-based and lesion-based analysis was done to compare the lesion detection rates of CECT, 68Ga-PSMA-11 PET and 18F-FDG PET. PET-based quantitative parameters were also compared between both the PET modalities. Impact of baseline parameters on survival was assessed using Cox regression analysis. A p-value of < 0.05 was considered significant. RESULTS: Thirty-seven patients with median age 60 years ± 13 years (range = 26-76 years) were included in the study. Twenty-seven patients had clear cell (cc) RCC, six had papillary RCC (pRCC), and one each had an eosinophilic variant of ccRCC, collecting duct RCC, translocation RCC and poorly differentiated RCC. 68Ga-PSMA-11 PET performed better in detecting marrow and equivocal bone lesions and worse in detecting liver lesions compared to CECT. 68Ga-PSMA-11 PET-based angiogenic tumor burden estimation using Total Lesion-PSMA (TL-PSMA) and PSMA-Total volume (PSMA-TV) had a prognostic impact on the survival of patients. 68Ga-PSMA-11 PET also detected more lesions and showed significantly higher SUVmax than 18F-FDG PET. CONCLUSION: 68Ga-PSMA-11 PET/CT performs better than CECT and 18F-FDG PET/CT in metastatic evaluation and has prognostic value in the management of clear cell RCC.

Sunitinib in Tandem With 177 Lu-DOTATATE Therapy in Advanced Pancreatic Neuroendocrine Tumor : A New Treatment Approach.

ABSTRACT: Various systemic treatment options are available for advanced pancreatic neuroendocrine tumors (NETs); however, individual treatment may be suboptimally effective. Sunitinib inhibits multiple kinases and signaling pathways with delay in tumor growth, whereas peptide radioreceptor therapy (PRRT) delivers targeted radiation to the tumors in pancreatic NETs. There is a dearth of literature on the combined or tandem use of these systemic treatment modalities. We present a case of 40-year-old man with advanced pancreatic NET where PRRT or sunitinib as monotherapy had a suboptimal treatment response, but the use of sunitinib in tandem with 177 Lu-PRRT reinforced the response to the treatment.

Lymph node involvement in an ovarian borderline serous tumor: a rare clinicopathologic presentation with management dilemma.

Borderline serous tumor (BST), earlier known as atypical proliferative serous tumor, is an ovarian neoplasm of low malignant potential. Extraovarian spread in the form of peritoneal implants is common in these tumors; however, lymph node (LN) involvement is infrequent. The prognostic implication of LN involvement in BST is controversial. We present a case of a 25-year-old female presenting with dull-aching abdominal pain in the left iliac fossa for the past 3 years, which was associated with constipation and abdominal bloating. Her serum Cancer antigen 125 (CA125) level was 841.3 units/ml. Pelvic ultrasonography and magnetic resonance imaging showed a large well-defined, solid-cystic, abdominopelvic mass arising from the right ovary, measuring 21×18×10 cm. The left ovary was also solid-cystic and measured 7×4×3 cm. A provisional clinico-radiologic diagnosis of ovarian malignancy was rendered. The patient underwent bilateral salpingo-oophorectomy with omentectomy and right-sided pelvic and para-aortic lymph node dissection. Histopathology revealed bilateral ovarian BST with involvement of pelvic and para-aortic lymph nodes. This was followed by adjuvant chemotherapy (in view of stage IIIA). She is disease-free at 3 years of regular follow-up. The prognosis and management of BST with LN is not yet fully elucidated. Nevertheless, the finding of such an involvement mandates thorough sampling of the primary ovarian tumor to exclude a possibility of low-grade serous carcinoma with LN metastasis.

[177Lu]Lu-PSMA-617 Versus Docetaxel in Chemotherapy-Naïve Metastatic Castration-Resistant Prostate Cancer: Final Survival Analysis of a Phase 2 Randomized, Controlled Trial.

The prostate-specific membrane antigen (PSMA) inhibitor [177Lu]Lu-PSMA-617 has been previously demonstrated to be noninferior to docetaxel in achieving a biochemical response in chemotherapy-naïve metastatic castration-resistant prostate cancer patients. Here, we report the final analysis of overall survival (OS) for a phase 2 randomized, controlled trial. Methods: Forty chemotherapy-naïve, PSMA-positive metastatic castration-resistant prostate cancer patients were randomly assigned to [177Lu]Lu-PSMA-617 (n = 20) or docetaxel (n = 20). Thirty-five patients received treatment per the protocol. Survival analysis was done using Kaplan-Meier curves and the Cox regression model. Results: The mean follow-up duration was 33.4 mo. In intention-to-treat analysis, the median OS for the [177Lu]Lu-PSMA-617 and docetaxel arms was 15.0 mo (95% CI, 9.5-20.5 mo) and 15.0 mo (95% CI, 8.1-21.9 mo), respectively (P = 0.905). In per-protocol analysis, the median OS was 19.0 mo (95% CI, 12.3-25.7 mo) versus 15.0 mo (95% CI, 8.1-21.9 mo), respectively (P = 0.712). No significant difference in OS was observed between the 2 arms across the analyzed subgroups. Conclusion: Long-term outcomes with [177Lu]Lu-PSMA-617 administered earlier in the prechemotherapy setting are comparable to those with docetaxel.

Alpha Before Beta: Exceptional Response to First-Line 225Ac-DOTATATE in a Patient of Metastatic Neuroendocrine Tumor With Extensive Skeletal Involvement.

ABSTRACT: The utility of ß-emitter 177Lu-DOTATATE in patients of neuroendocrine tumors (NETs) with widespread skeletal metastases is limited by its relatively modest response rates and a significant concern for hematotoxicity. In such situations, targeted α therapy with 225Ac-DOTATATE can be potentially beneficial. In this report, a 46-year-old man with rectal NET and extensive skeletal metastases was treated upfront with 6 cycles of 225Ac-DOTATATE at 8 weeks' intervals. The patient showed excellent symptomatic, biochemical, and radiological response with no grade 3/4 adverse events. The first-line use of 225Ac-DOTATATE, therefore, presents a novel strategy for metastatic NETs with high skeletal disease burden.

177Lu-PSMA-617 versus docetaxel in chemotherapy-naïve metastatic castration-resistant prostate cancer: a randomized, controlled, phase 2 non-inferiority trial.

PURPOSE: Lutetium-177 prostate-specific membrane antigen-617 (177Lu-PSMA-617) in end-stage metastatic castration-resistant prostate cancer (mCRPC) has reported favourable outcomes. In this study, we aimed to prospectively compare the efficacy and safety of 177Lu-PSMA-617 and docetaxel in chemotherapy-naïve mCRPC patients. METHODS: This was a randomized, parallel-group, open-label, phase 2, and non-inferiority trial. Chemotherapy-naïve patients with mCRPC and high PSMA-expressing lesions on 68 Ga-PSMA-11 PET/CT were randomly assigned in 1:1 ratio to 177Lu-PSMA-617 (6.0-7.4 GBq/cycle, every 8 weeks, up to 4 cycles) or docetaxel (75 mg/m2/cycle, every 3 weeks, up to 10 cycles). The primary end-point was best prostate-specific antigen response rate (PSA-RR), defined according to Prostate Cancer Clinical Trials Working Group-3 as proportion of patients achieving ≥ 50% decline in PSA from baseline. Non-inferiority margin of - 15% was pre-specified for PSA-RR. RESULTS: Between December 2019 and March 2021, 40 of the 45 patients assessed for eligibility underwent randomization. Fifteen of 20 patients in 177Lu-PSMA-617 arm and 20/20 patients in docetaxel arm received treatment per protocol. Of these, best PSA-RR in the 177Lu-PSMA-617 arm was 60% (9/15) versus 40% (8/20) in the docetaxel arm. The difference in the PSA-RRs between the two arms was 20% (95% confidence interval, CI: - 12-47, P = 0.25), meeting the pre-specified criterion for non-inferiority in per-protocol analysis. Further, progression-free survival rates at 6 months were 30% and 20% in the 177Lu-PSMA-617 and docetaxel arms respectively (difference 10%, 95% CI: - 18-38, P = 0.50). Overall, treatment-emergent grade ≥ 3 adverse events occurred less frequently with 177Lu-PSMA-617 than with docetaxel (6/20, 30% versus 10/20, 50%, respectively, P = 0.20). Quality-of-life outcomes improved significantly in 177Lu-PSMA-617 arm compared to docetaxel arm (P < 0.01). CONCLUSION: 177Lu-PSMA-617 was demonstrated to be safe and non-inferior to docetaxel in the treatment of mCRPC and could, thus, be potentially employed earlier in the disease course rather than being solely reserved for advanced end-stage disease. CLINICAL TRIAL REGISTRATION: Clinical Trials Registry-India, CTRI/2019/12/022282.

Malignant uterine perivascular epithelioid cell tumor: histopathologic and immunohistochemical characterization of a rare tumor in a post-menopausal woman.

BACKGROUND: Perivascular epithelioid cell tumors (PEComas) are rare, mesenchymal neoplasms composed of epithelioid cells exhibiting myogenic and melanocytic differentiation. The uterus is an infrequent site of involvement. The most common histopathologic mimics include leiomyosarcoma, endometrial stromal sarcoma, undifferentiated uterine sarcoma, and malignant melanoma. Rendering an accurate histopathologic diagnosis is essential, owing to the prognostic and therapeutic implications. CASE: A 65-years-old post-menopausal woman presented with post-menopausal bleeding, abdominal pain, and heaviness for the last four months. Ultrasound abdomen revealed a large uterine mass replacing the endometrial cavity. She underwent a total abdominal hysterectomy with bilateral salpingo-oophorectomy. RESULT: Microscopically, a circumscribed tumor with tumor cells arranged in sheets and interlacing fascicles, with interspersed fine capillary network, was seen. The individual tumor cells were epithelioid to spindle with moderate pleomorphism, round nuclei, vesicular chromatin, prominent macronucleoli, and moderate cytoplasm. Mitosis was 2-3/50 HPFs. On immunohistochemistry, tumor cells were positive for HMB-45, Melan-A, and smooth muscle actin and were negative for h-caldesmon, TFE3, S-100, CD10, and pan-cytokeratin. Based on the histopathologic and immunohistochemical features, a final diagnosis of malignant uterine PEComa was rendered. CONCLUSIONS: This index report describes the characteristic histopathologic and immunohistochemical features of malignant uterine PEComa and highlights the salient features that distinguish it from other commonly encountered histopathologic mimics.

Effect of COVID-19 on 18F-FDG PET/CT: Is There a Need to Consider COVID-19 Status Before Planning 18F-FDG PET/CT for Oncologic Evaluation?

Incidental detection of coronavirus disease 2019 (COVID-19)-related lung changes on 18F-FDG PET/CT images of oncology patients has been increasingly reported. Most of the case reports or series have stressed the retrospective diagnosis of COVID-19 with the help of 18F-FDG PET/CT lung findings. In this case report, we introduce a different aspect of COVID-19-related lung changes on 18F-FDG PET/CT, interfering with the evaluation of metastatic lung lesions in a patient with renal cell carcinoma.

Evolving role of 225Ac-PSMA radioligand therapy in metastatic castration-resistant prostate cancer-a systematic review and meta-analysis.

BACKGROUND: Targeted radionuclide therapy with Actinium-225-labeled prostate-specific membrane antigen ligands (225Ac-PSMA) has emerged as a promising treatment modality in the management of metastatic castration-resistant prostate cancer (mCRPC). With its high linear energy transfer and short path length, 225Ac induces double-stranded DNA breaks and is expected to have excellent efficacy and safety profile. This systematic review was conducted to precisely evaluate the role of 225Ac-PSMA radioligand therapy (RLT) in mCRPC. METHODS: This systematic review followed the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines. Searches were made using relevant keywords in the PubMed, Embase, and Scopus databases, and articles up to December 2020 were included. Data on efficacy and toxicity were extracted from the individual articles. Random-effects model was used for generating pooled estimates through meta-analysis. RESULTS: Ten articles comprising 256 patients were included. Overall, 62.8% (95% confidence interval, CI: 53.4-71.7%) of the patients treated with 225Ac-PSMA RLT achieved biochemical response, i.e., ≥50% decline in the serum prostate-specific antigen levels from baseline. Molecular response on Gallium-68 PSMA positron emission tomography/computed tomography was noted in 74% (95% CI: 50.1-92.1%) of the patients. The pooled estimates of median progression-free survival and overall survival were 9.1 months (95% CI: 3.6-14.5 months) and 12.8 months (95% CI: 4.5-21.0 months), respectively. The most commonly reported adverse event was xerostomia, which was observed in 72.7% (95% CI: 50.5-90.1%) of the patients. However, clinically significant toxicity was limited with grade ≥3 xerostomia, anemia, leucopenia, thrombocytopenia, and nephrotoxicity occurring in 1.2%, 12.3%, 8.3%, 6.3%, and 3.8% of the patients, respectively. Treatment discontinuation due to adverse events was noted in 20/208 patients. CONCLUSIONS: 225Ac-PSMA RLT is an efficacious and safe treatment option for patients with mCRPC. Future randomized controlled trials are required to establish its therapeutic efficacy and survival benefit vis-à-vis other approved treatment modalities.

Response to Concomitant Enzalutamide and 177Lu-PSMA-617 Radioligand Therapy in ATM-Mutated Metastatic Castration Resistant Prostate Cancer.

ABSTRACT: Radioligand therapy with 177Lu-PSMA-617 has emerged as a promising treatment modality for patients with mCRPC (metastatic castration resistant prostate cancer). However, genomic defects in DNA damage repair mechanisms have been proposed to affect the radiosensitivity of prostate cancers. Patients harboring such deleterious mutations are, thus, unlikely to respond to 177Lu-PSMA-617 alone and would need a more tailored therapeutic approach. We report the case of a 68-year-old man with ATM mutation-positive mCRPC who showed exceptional response to concomitant administration of enzalutamide with 177Lu-PSMA-617.

Metastatic dysgerminoma in a young patient with 46 XY DSD: A rare case report.

The term DSD (Disorders of Sex development) is issued to define those conditions in whom disharmony exists between chromosomal, hormonal and anatomical sex. Parental and patient mental health and quality of life are adversely affected by these conditions. Moreover, individuals with an underlying DSD, especially those with specific Y chromosomal material in their karyotype have an increased risk for developing a germ cell tumor. Here, we present a unique case of 46XY DSD with bilateral dysgerminomas presenting with abdominal mass at the age of 24 years, who was treated with one cycle of chemotherapy comprising of Carboplatin and Etoposide, following which he developed tumor lysis syndrome and later underwent exploratory laparotomy.

Locally advanced gallbladder cancer: a review of the criteria and role of imaging.

Gallbladder carcinoma (GBC) is among one of the gastrointestinal malignancies with extremely dismal prognosis. This is due to the advanced stage at presentation. Majority of the patients with GBC are not considered candidates for surgery because of the locally advanced disease or metastases. However, with the accumulating evidence regarding the role of neoadjuvant chemotherapy, there is a need to correctly identify a subset of patients with locally advanced GBC who will benefit maximally from neoadjuvant chemotherapy and will be successfully downstaged to receive curative (R0) surgery. In this context, there is a lack of consensus and different groups have resorted to criteria for locally advanced disease eligible for neoadjuvant chemotherapy based on personal or institutional experiences. Imaging plays a critical role in the evaluation of patients with GBC as it helps stratify patients into resectable and unresectable. Imaging also has the potential to identify patients with locally advanced GBC and hence facilitate neoadjuvant chemotherapy and improve outcomes. In this review, we evaluate the various criteria for locally advanced GBC and the role of imaging in this scenario.

Expression of CXCR4 Receptors in Refractory Atypical Mediastinal Carcinoid.

Primary neuroendocrine tumor of the mediastinum is a relatively rare entity. In metastatic/inoperable disease, therapeutic options are limited to cytotoxic chemotherapy in poorly differentiated tumors and peptide receptor radionuclide therapy in case of well-differentiated tumors. We present the case of a 52-year-old man with mediastinal atypical carcinoid (grade II) neuroendocrine tumor showing mild somatostatin receptor expression and intense FDG avidity with progressive disease on chemotherapy. Chemokine receptor targeted PET/CT with CXCR4 (Ga-CXCR4) showed tracer avidity in tumor sites higher than the physiological sites, which may pave the way for CXCR4-targeted radionuclide therapy in this subgroup of patients.

225Ac-PSMA-617 Radioligand Posttherapy Imaging in Metastatic Castrate-Resistant Prostate Cancer Patient Using 3 Photopeaks.

PSMA-based radioligand therapies have shown the beneficial effect in metastatic castrate-resistant prostate cancer (mCRPC) patients when they become refectory to the established treatments with associated potential toxicities and high mortality rate. Ac-PSMA therapy is known to be remarkably effective in substantially pretreated mCRPC patients. However, posttherapy imaging is usually not performed as alpha emitters are really difficult to image. We presents a patient of mCRPC treated with Ac-PSMA-617, and his posttherapy whole-body scans acquired by using 3 different photopeaks (78, 218, and 440 keV) fairly demonstrated the tracer's distribution and the efficacy of targeted alpha therapy.

Clinicopathological Profile of Castleman's Disease in Indian Population: Experience From a Tertiary Care Center.

Castleman's disease (CD), also known as angiofollicular lymph node hyperplasia, a rare, non-malignant chronic lymphoproliferative disease characterized by, uni or multicentric lymphadenopathy. There is limited information about the clinicopathological variations and associations of this entity. A total of 50 cases of CD were retrieved from the archives, between the years 2005-2017. The cases were divided into pediatric (0-18 years), young adult (age ≤ 40 years) and older adult groups (age > 40 years respectively). Detailed clinicopathological correlation was done. The age range was 6-74 years. There was a male predominance (M: F-1.6:1). The majority (72%; 36/50) of the patients were adults; 46% young adult and 28% older adult, while only 28% (14/50) were of pediatric. Majority (78%) showed features of unicentric Castleman disease (UCD) while rest 22% presented with multi centric Castleman disease (MCD). Systemic symptoms were more frequent in MCD as compared to UCD cases (p = 0.06). The majority of the cases (40/50; 80%) were of the hyaline vascular type. Two of the cases showed mixed histological feature. Out of 50 patients 29 patient's treatment details are available. Majority unicentric cases were cases surgically excised 14/17 (82.3%). Three patients 3/17 (17.6%) were treated with chemotherapy. In multicentric Castleman's disease group, six of the 12 cases with MCD were managed by chemotherapy. One patient died of progressive disease. Castleman disease has varied clinical presentation and is often associated with other diseases. A high degree of suspicion and careful histological examination is required in order not to miss this entity.