Angiomatoid fibrous histiocytoma: Report of two cases, initially construed as sarcoma with unusual clinico-pathological features.

Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor of uncertain differentiation with low metastatic potential, most commonly occurring in children, adolescents, and young adults, involving extremities. Due to its rare nature and diverse presentation, both clinically and morphologically, it is often misdiagnosed. It becomes important to correctly diagnose this lesion, given its distinct therapeutic implications. Here, we present the clinical, radiologic, and pathologic findings of two rare cases of AFH. Since AFH is a rare soft tissue tumor with low malignant potential, both pathologists and clinicians should be aware of this entity, when encountered with a soft tissue mass in extremities of a child or adolescent, so as to accord appropriate treatment in such cases.

A rare case of malignant peripheral nerve sheath tumor of pleura and review of literature.

Malignant peripheral nerve sheath tumor (MPNST) is a type of soft tissue sarcoma arising from peripheral nerves. They can be found in isolation, but about half of the cases are associated with neurofibromatosis type 1 (NF1), an autosomal dominant genetic disorder. They usually arise from the nerve plexus in extremities and trunk. MPNSTs arising from the viscera or internal organs are very rare. We hereby report a rare case of sporadic MPNST arising from pleural surface in a middle-aged male. The diagnosis of such a rare entity involved multimodal investigations with consideration of wide differential diagnosis.

A Walk with Lu-177 PSMA: How Close we Have Reached from Bench to Bedside?

Metastatic castration-resistant prostate cancer (mCRPC) is a natural sequela in advanced prostate cancer following resistance to standard treatment regimes, where patients develop with rising PSA, bone pains, and high disease volume. Further palliative treatment is the need of the hour for ensuring disease control and quality of life. In recent times, many novel methods have been evolved for these patients. Endo-radioligand therapy with Lutetium 177 prostate-specific membrane antigen 617 (Lu-177 PSMA) based on the Theranostic concept has emerged as a promising tool among these. We present here the current status of Lu177-PSMA for mCRPC patient and future directions.

Role of 18F-Flurodeoxyglucose Positron-Emission Tomography/Computed Tomography in the Evaluation of Early Response to Neoadjuvant Chemotherapy in Patients with Locally Advanced Triple-Negative Breast Cancer.

BACKGROUND: Response evaluation in locally advanced breast cancer is done through different methods ranging from clinical examination to magnetic resonance imaging, however evaluation with positron-emission tomography/computed tomography (PET/CT) in now being incorporated for the response evaluation. The aim of the present study is to correlate response to neoadjuvant chemotherapy (NACT) with PET/CT scan. MATERIALS AND METHODS: The present study is a retrospective analysis of 30 locally advanced, triple-negative breast cancer patients. PET/CT scan was done pretreatment and post three and six cycles of NACT and was correlated with pathologic complete response (pCR). Responding disease was considered when there was at least a 50% reduction in the longest diameter. RESULTS: The median pretreatment size of the breast lesion in CT scan was 3.9 ± 2.3 cm (2-12 cm) and maximum standardized uptake value (SUVmax) on PET/CT was 8.5 ± 5.5 (2.9-24). Among the responders, the median decrease in size of lesion was 3.2 ± 1.3 cm and median reduction in SUV of the tumor among was -8.1 ± 5.4 and was statistically significant when compared with nonresponders (P < 0.001). CT scan has 66% accuracy and PET has 82% accuracy at post three cycles NACT in predicting the pathological response. PET/CT had higher sensitivity and specificity when compared with CT findings alone in response evaluation. CONCLUSION: PET/CT scan can be considered as a sensitive tool for predicting pCRs and further larger trials are required to establish these findings.

Patterns of brain metastasis in anaplastic lymphoma kinase - rearranged and epidermal growth factor receptor-mutated lung cancer patients in magnetic resonance imaging.

INTRODUCTION: The optimal management of neuroparenchymal lesions in cases of lung cancer is exigent as this frequent yet notorious complication negatively impacts the morbidity and mortality index. AIMS: This study is aimed at recognizing various patterns of neuroparenchymal metastasis in patients of lung cancer with epidermal growth factor receptor (EGFR)- and anaplastic lymphoma kinase (ALK)-positive mutations. MATERIAL AND METHODS: The radiological findings of the neuroparenchymal lesions were analyzed and the statistical data were charted. We identified two groups of patients with neuroparenchymal lesions among a cohort of 340 patients having EGFR-positive (68) and ALK-positive (24) mutations (total: 24 + 68 = 92). RESULTS: We observed that among the ALK group, leptomeningeal spread was less compared to EGFR group (2/24 as opposed to 18/68). Morphological heterogeneity and central necrosis in the parenchymal lesion which were associated with unfavorable outcomes were predominant in ALK group (8/24) as opposed to EGFR group (2/68). Ancillary findings but pertinent to survival and morbidity such as presence of perilesional edema, hemorrhage, and hydrocephalus on magnetic resonance imaging were also analyzed. The mutation-specific differential imaging spectrum could be attributed to biological differences between these cancers.

Role of ADC values in assessing clinical response and identifying residual disease post-chemo radiation in uterine cervix cancer.

OBJECTIVES: To evaluate the role of apparent diffusion coefficient (ADC) values in assessing response after chemo-radiotherapy in cervix cancer and investigate the utility of ADC as a tool to identify residual disease, after the treatment completion. METHODS: A prospective study was done in 100 patients with histopathologically proven cancer of uterine cervix who were classified as either complete response (CR) or residual disease posttreatment. MRI was done pretreatment and after 6 weeks post-treatment with chemo-radiation. 53 patients among the cohort also underwent a fluoro-deoxy glucose positron-emission computed tomography (FDG-PET CT). ADC values, change in ADC values, and metabolic activity obtained from FDG-PET CT were correlated with clinical outcome, and statistical analysis was done to determine the better tool for assessing response evaluation between ADC and PET-CT. RESULTS: Residual lesions have notably lower ADC value than that of posttreatment changes. The mean ADC values of residual tumors: 1.26 ± 0.238 × 10-3 mm2/s and mean ADC values of lesions due to posttreatment changes: 1.540 ± 0.218 × 10-3 mm2/s (statistically significant difference between malignant and posttreatment lesions, P < 0.05). ADC has 67% sensitivity, 83% specificity, 35% positive predictive values (PPV), 95% negative predictive values (NPV), and 81% accuracy in differentiating residual disease from post treatment changes. PPV, NPV, sensitivity, and specificity with PET-CT were 93%, 89%, 98%, and 73%, respectively. PPV, NPV, sensitivity, and specificity of contrast MRI were 16%, 91%, 58%, and 59%, respectively. CONCLUSION: Diffusion imaging differentiates residual cervix malignancies from post treatment changes based on ADC values and can be a promising and evocative biomarker. Complimentary use of ADC and PET/CT may increase diagnostic confidence.

Malignant Sertoli cell tumor of the testis masquerading as seminoma with bone metastasis.

Sex cord-stromal tumors of the testes are rare malignancies as compared to germ cell tumors. Pure Sertoli cell tumors are still rare representing <1% of testicular cancers and the malignant forms are too rare. Furthermore, the occurrence of metastasis in such cases is extremely rare with <30 cases reported in literature so far to the best of our knowledge. We present herein a case of malignant Sertoli cell tumor in a 48-year-old male who was initially misdiagnosed as seminoma based on histology and clinical presentation. Four months later, he presented with symptoms due to bony metastasis and found to have widespread metastatic disease which is a very rare presentation of Sertoli cell tumors. Diagnosis of sex cord-stromal tumor requires high index of suspicion as these tumors are most of the times misdiagnosed as germ cell tumors due to their rare incidence and atypical presentation leading to mismanagement. Timely diagnosis at an early stage can provide therapeutic benefit due to lack of well-defined treatment options at advanced stages.

Frequency of T790M mutations after progression on epidermal growth factor receptor tyrosine kinase inhibitor in metastatic non-small cell lung cancer in Indian patients: real-time data from tertiary cancer hospital.

AIM: The aim of this study is to determine the incidence of T790M mutations after progression on epidermal growth factor receptor (EGFR) tyrosine kinase inhibitors (TKI) and median duration on TKI before progression on TKI. METHODS: Records of Rajiv Gandhi Cancer Institute and Research Centre, of patients who were diagnosed with metastatic adenocarcinoma of the lung and progressed on oral EGFR TKIs and underwent T790M mutation analysis in the last 6 months were retrospectively reviewed. The incidence of T790M positivity, sites of progression, and median duration of TKI treatment before progression was calculated. RESULTS: Among 31 patients, 10 patients have undergone rebiopsy, and 24 patients had undergone liquid biopsy by Droplet Digital polymerase chain reaction (ddPCR), and three patients had undergone both tests. Among all, the rate of T790M positivity was 54.8%. Among these 17 patients positive for T790M, seven patients were positive by biopsy, and 11 patients were positive by ddPCR. Among three patients who underwent both, one was positive by both. The most common site of progression among all patients is pleura, and 10% of patients progressed in brain post-TKI. Median progression-free survival on TKI before progression is 289.7 days, highest being 1290 days, and lowest 45 days. CONCLUSIONS: Exact incidence of T790M mutations after progression on TKI s in Asian population is not exactly known and requires large data, as incidence may be different than reported in the Western population. Rebiopsy and ddPCR help to determine the most common type of resistance after progression on TKI, for which effective targeted therapy is available.