A Rare Presentation of Recurrent Malignant Peripheral Nerve Sheath Tumor with Glandular Differentiation-A Case Report.

Malignant peripheral nerve sheath tumors (MPNST) are rare heterogeneous group of soft tissue neoplasms. In most cases, they originate within the pre-existing neurofibromatosis. The emergence of glandular structures in MPNST is curious and enigmatic. We report a case of recurrent MPNST with glandular differentiation arising in the background of neurofibroma in a 20-year-old lady. By immunohistochemistry, MPNST showed focal positive staining for S100 and negative staining for SOX10 while adjacent neurofibroma showed diffuse positivity for S100 and SOX10. The glandular tumor cells showed positive staining for CDX2, Cam5.2, CK19, and CK7 (focal), while negative for SOX10 and S100. MPNST with glandular differentiation is quite rare which may pose a diagnostic challenge. The glandular differentiation in MPNST should be excluded from the metastasis from second primary with the aid of clinical and radiological correlation.

Primary Circumscribed Meningeal Melanoma Involving the Meckel's Cave: A Report of a Rare Case and Review of Literature.

Primary intracranial meningeal melanomas are rare. Diagnosing primary meningeal melanomas mostly involves comprehensive assessment through clinical and radiological means. This evaluation should encompass a detailed dermal and ophthalmic examination. Any suspicious lesion must be biopsied and examined microscopically. This is crucial not only to differentiate primary intracranial melanoma from other brain tumors but also to rule out metastases from potential sources of primary cutaneous or non-cutaneous melanomas. Surgery is considered the mainstay of treatment. Despite melanomas being generally considered radio- and chemo-resistant tumors, adjuvant radiotherapy and chemotherapy still play a crucial role in their management. The treatment landscape for primary meningeal melanoma is continually evolving, with ongoing research aiming to improve outcomes for patients with this challenging disease.

Development of an MRI-Based Prediction Model for Anti-TNF Treatment Failure in Perianal Crohn's Disease: A Multicenter Study.

BACKGROUND & AIMS: Clinical and radiologic variables associated with perianal fistula (PAF) outcomes are poorly understood. We developed prediction models for anti-tumor necrosis factor (TNF) treatment failure in patients with Crohn's disease-related PAF. METHODS: In a multicenter retrospective study between 2005 and 2022 we included biologic-naive adults (>17 years) who initiated their first anti-TNF therapy for PAF after pelvic magnetic resonance imaging (MRI). Pretreatment MRI studies were prospectively reread centrally by blinded radiologists. We developed and internally validated a prediction model based on clinical and radiologic parameters to predict the likelihood of anti-TNF treatment failure, clinically, at 6 months. We compared our model and a simplified version of MRI parameters alone with existing imaging-based PAF activity indices (MAGNIFI-CD and modified Van Assche MRI scores) by De Long statistical test. RESULTS: We included 221 patients: 32 ± 14 years, 60% males, 76% complex fistulas; 68% treated with infliximab and 32% treated with adalimumab. Treatment failure occurred in 102 (46%) patients. Our prediction model included age at PAF diagnosis, time to initiate anti-TNF treatment, and smoking and 8 MRI characteristics (supra/extrasphincteric anatomy, fistula length >4.3 cm, primary tracts >1, secondary tracts >1, external openings >1, tract hyperintensity on T1-weighted imaging, horseshoe anatomy, and collections >1.3 cm). Our full and simplified MRI models had fair discriminatory capacity for anti-TNF treatment failure (concordance statistic, 0.67 and 0.65, respectively) and outperformed MAGNIFI-CD (P = .002 and < .0005) and modified Van Assche MRI scores (P < .0001 and < .0001), respectively. CONCLUSIONS: Our risk prediction models consisting of clinical and/or radiologic variables accurately predict treatment failure in patients with PAF.

Accumulation of 18 F-FDG at Anomalous Systemic Arterial Supply to Normal Lung on 18 F-FDG PET/CT.

ABSTRACT: Anomalous systemic arterial supply to normal lung is an anatomical variant in which a portion of the lung is supplied by a systemic vessel without a distinct pulmonary sequestration. We report a case of mild to moderate accumulation of 18 F-FDG in the medial basal segment of left lung; corresponding CT images localize this uptake in the tortuous artery arising from the descending aorta with similar uptake to that of descending aorta. Findings are suggestive of anomalous systemic arterial supply to normal segments of the lung. Hybrid PET/CT allows precise anatomical localization and helps in differentiate benign mimics of disease, which can alter patient management.

Progression Rates of LR-2 and LR-3 Observations on MRI to Higher LI-RADS Categories in Patients at High Risk of Hepatocellular Carcinoma: A Retrospective Study.

BACKGROUND. Reported rates of hepatocellular carcinoma (HCC) for LR-2 and LR-3 observations are generally greater than those expected on the basis of clinical experience, possibly reflecting some studies' requirement for pathologic reference. OBJECTIVE. The purpose of this study was to determine rates of progression to higher LI-RADS categories of LR-2 and LR-3 observations in patients at high risk of HCC. METHODS. This retrospective study included 91 patients (64 men, 27 women; mean age, 62 years) at high risk of HCC who had clinically reported LR-2 (n = 55) or LR-3 (n = 36) observations on MRI who also underwent follow-up CT or MRI at least 12 months after the observation was made. A study coordinator annotated the location of a single LR-2 or LR-3 observation per patient on the basis of the clinical reports. Using LI-RADS version 2018 criteria, two radiologists independently assigned LI-RADS categories on the follow-up examinations. Progression rates from LR-2 or LR-3 to higher categories were determined. A post hoc consensus review was performed of observations that progressed to LR-4 or LR-5. Subgroup analyses were performed with respect to presence of prior HCC (n = 34) or a separate baseline LR-5 observation (n = 12). RESULTS. For LR-2 observations, the rate of progression to LR-4 was 0.0% (95% CI, 0.0-6.7%) and to LR-5 was 3.6% (95% CI, 0.4-13.1%) for both readers. For LR-3 observations, the rate of progression to LR-4 was 22.2% (95% CI, 9.6-43.8%) and to LR-5 was 11.1% (95% CI, 3.0-28.4%) for both readers. Fourteen observations progressed to LR-4 or LR-5 for both readers. Post hoc analysis revealed no instances of progression from LR-2 to LR-4; two, from LR-2 to LR-5; eight, from LR-3 to LR-4; and four, from LR-3 to LR-5. The progression rate from LR-3 to LR-5 was higher (p < .001) among patients with (100.0%) than those without (3.0%) a separate baseline LR-5 observation for both readers. The progression rate from LR-2 to LR-5 was not associated with a separate baseline LR-5 observation for either reader (p = .30). Progression rates were not different (p > .05) between patients with versus those without prior HCC. CONCLUSION. On the basis of progression to LR-4 or LR-5, LR-2 and LR-3 observations had lower progression rates than reported in studies incorporating pathology results in determining progression. CLINICAL IMPACT. The findings refine understanding of the clinical significance of LR-2 and LR-3 observations.

Case report of a female child with right nasal chondromyxoid fibroma.

Chondromyxoid fibroma is a rare tumour, representing <1% of all primary bone neoplasm. We report the case of a four-year-old female child with a one-year history of nasal obstruction and facial swelling. A large enhanced lesion with amorphous densities spreading into the right cribriform plate and floor of sphenoid sinus, laterally into the right lamina papyracea, inferolaterally into the medial wall of maxillary sinus, posteriorly into the nasopharynx and superior aspect of oropharynx was observed on CT scan. The mass was excised by Caldwell Luc's endoscopic medial maxillectomy via sublabial approach. CMF was confirmed histopathologically.

Comparison of Bosniak Classification of cystic renal masses version 2019 assessed by CT and MRI.

OBJECTIVE: To compare imaging features in cystic masses imaged with both CT and MRI using Bosniak Classification version 2019 (Bosniak.v2019) and original Bosniak Classification (Bosniak.original). MATERIALS AND METHODS: This IRB-approved, retrospective, cross-sectional study evaluated sixty-five consecutively identified cystic (≤ 25% enhancing) masses imaged by CT and MRI between 2009 and 2019: 35 with histologic diagnosis and 30 Bosniak.v2019 Class 2 and Class 2F cystic masses verified by an expert radiologist (R1) with minimum 5-year stability. Three radiologists (R2, R3, R4) independently evaluated CT, followed by MRI and assigned Bosniak.original and Bosniak.v2019 class in two sessions separated by ≥ 1 month and assessed the following: septa number, septa/wall thickness, and protrusions. Discrepancies were resolved by consensus with R1. RESULTS: There was 70.8% agreement (kappa = 0.60, p = 0.0146) in class assigned by CT versus MRI for Bosniak.original and 72.3% agreement (kappa = 0.63, p = 0.006) for Bosniak.v2019. Increased septa number (p < 0.001) and more protrusions (p = 0.034) were identified on MRI, with no differences in septal/wall thickness (p = 0.067, 0.855) or protrusion size (p = 0.467). For both CT and MRI, Bosniak.v2019 improved specificity (79.0% [95% confidence interval 71.0-87.0%] CT, 70% [62.0-77.0%] MRI) compared to Bosniak.original (63.0% [56.0-69.0%] CT, 66.0% [58.0-74.0%] MRI) with maintained sensitivity and higher overall accuracy. Inter-observer agreement was similar-to-slightly higher for Bosniak.v2019 (K = 0.44 CT, 0.39 MRI) versus Bosniak.original (K = 0.35 CT, 0.37 MRI). CONCLUSION: Class assignment differs in cystic masses evaluated by CT versus MRI for original and v2019 Bosniak Classification with similar-to-slightly higher agreement and improved specificity and higher overall accuracy on both CT and MRI with Bosniak version 2019.

Bosniak Classification version 2019: validation and comparison to original classification in pathologically confirmed cystic masses.

OBJECTIVE: To evaluate Bosniak Classification v2019 definitions in pathologically confirmed cystic renal masses. MATERIALS AND METHODS: Seventy-three cystic (≤ 25% solid) masses with histological confirmation (57 malignant, 16 benign) imaged by CT (N = 28) or CT+MRI (N = 56) between 2009 and 2019 were independently evaluated by three blinded radiologists using Bosniak v2019 and original classifications. Discrepancies were resolved by consensus with a fourth blinded radiologist. Overall class and v2019 features were compared to pathology. RESULTS: Inter-observer agreement was slightly improved comparing v2019 to Original Bosniak Classification (kappa = 0.26-0.47 versus 0.24-0.34 respectively). v2019 proportion of IIF and III masses (20.5% [15/73, 95% confidence interval (CI) 12.0-31.6%], 38.6% [28/73, 95% CI 27.2-50.5%]) differed from the original classification (6.8% [5/73, 95% CI 2.3-15.3%], 61.6% [45/73, 95% CI 49.5-72.8%]) with overlapping proportion of malignancy in each class. Mean septa number (7 ± 4 [range 1-10]) was not associated with malignancy (p = 0.89). Mean wall and septa thicknesses were 3 ± 3 (1-14) and 3 ± 2 (1-10) mm and higher in malignancies (p = 0.03 and 0.20 respectively). Areas under the receiver-operator-characteristic curve for wall and septa thickness were 0.66 (95% CI 0.54-0.79) and 0.61 (95% CI 0.45-0.78) with an optimal cut point of ≥ 3 mm (sensitivity 33.3%, specificity 86.7% and sensitivity 53%, specificity 73% respectively). Proportion of malignancy occurring in masses with the v2019 features "irregularity" (76.9% [10/13], 95% CI 46.2-94.9%) and "nodule" (89.7% [26/29], 95% CI 72.7-97.8%) overlapped. Angle of "nodule" (p = 0.27) was not associated with malignancy. CONCLUSION: Bosniak v2019 definitions for wall/septa thickness and protrusions are associated with malignancy. Overall, Bosniak v2019 categorizes a higher proportion of malignant masses in Class IIF with slight improvement in inter-observer agreement. KEY POINTS: • Considering Bosniak v2019 Class IIF cystic masses with many (≥ 4) smooth and thin septa, there was no association between the number of septa and malignancy (p = 0.89) in this study. • Increased cyst wall and septa thickness are associated with malignancy and a lower threshold of ≥ 3 mm maximized overall diagnostic accuracy compared to ≥ 4 mm threshold proposed for Bosniak v2019 Class 3. • An overlapping proportion of malignant masses is noted in Bosniak v2019 Class 3 masses with "irregularity" (76.9% [10/13], 95% CI 46.2-94.9%) compared to Bosniak v2019 Class 4 masses with "nodule" (89.7% [26/29], 95% CI 72.7-97.8%).

Hepatosplenic mucormycosis post autologous stem cell transplant.

Mucormycosis is a life threatening fungal infection and remains an important cause of morbidity and mortality in immunocompromised patients after hematopoietic stem cell transplant. We report here a case of hepatosplenic mucormycosis in a patient after autologous stem cell transplant. A young man with anaplastic large cell lymphoma underwent autologous hematopoietic stem cell transplant after achieving complete remission with standard chemotherapy and consolidative radiotherapy. He was found to have incidental hepatosplenic hypodensities on follow up imaging, that were proved to be mucormycosis on histopathology after getting CT-guided biopsy of splenic lesions. He was treated with intravenous amphotericin-B followed by complete radiological resolution of hepatosplenic lesions. Although these infections are often life threatening but limited disease may have better outcome if diagnosed and treated early and aggressively.