Pneumothorax in lung metastasis of advanced soft tissue sarcoma patients treated with oral pazopanib.

AIM: Data on occurrence of pneumothorax after the use of oral pazopanib in advanced soft tissue sarcoma (STS) with lung metastases are scarce in literature. We aimed to evaluate those in our patients. METHODS: This was a single center retrospective study of incidence of pneumothorax in patients with lung metastases in advanced STS treated with oral pazopanib between July, 2016 and December, 2020. Patients were treated with pazopanib usually from 2nd line onwards with a dose ranging from 400 mg to 800 mg once daily. RESULTS: Total of 34 patients with lung metastasis in a setting of advanced STS were treated with oral pazopanib during the study period. The setting of pazopanib use was 2nd line in four and 1st line in one of them. The starting dose was 600 mg once daily in three patients, 400 mg OD in one patient, and 800 mg OD in one patient. Five patients developed pneumothorax with duration on pazopanib of 6, 7, 24, 6, and 2.5 months, respectively. Three patients had symptoms and required chest tube drainage. None of them were smokers or had any other underlying lung disease. The disease response of those patients was stable disease in four and partial response in one during treatment with pazopanib. One patient had a rechallenge with further pazopanib course without any recurrence of pneumothorax. CONCLUSIONS: Pneumothorax is a rare pulmonary complication after pazopanib use in patients with lung metastasis. Clinicians should be aware of this rare complication as literature is scarce. Rechallenge with pazopanib is feasible after pneumothorax.

MRI Features for Identifying MYCN-amplified RB1 Wild-type Retinoblastoma.

Background MYCN-amplified RB1 wild-type (MYCNARB1+/+) retinoblastoma is a rare but clinically important subtype of retinoblastoma due to its aggressive character and relative resistance to typical therapeutic approaches. Because biopsy is not indicated in retinoblastoma, specific MRI features might be valuable to identify children with this genetic subtype. Purpose To define the MRI phenotype of MYCNARB1+/+ retinoblastoma and evaluate the ability of qualitative MRI features to help identify this specific genetic subtype. Materials and Methods In this retrospective, multicenter, case-control study, MRI scans in children with MYCNARB1+/+ retinoblastoma and age-matched children with RB1-/- subtype retinoblastoma were included (case-control ratio, 1:4; scans acquired from June 2001 to February 2021; scans collected from May 2018 to October 2021). Patients with histopathologically confirmed unilateral retinoblastoma, genetic testing (RB1/MYCN status), and MRI scans were included. Associations between radiologist-scored imaging features and diagnosis were assessed with the Fisher exact test or Fisher-Freeman-Halton test, and Bonferroni-corrected P values were calculated. Results A total of 110 patients from 10 retinoblastoma referral centers were included: 22 children with MYCNARB1+/+ retinoblastoma and 88 control children with RB1-/- retinoblastoma. Children in the MYCNARB1+/+ group had a median age of 7.0 months (IQR, 5.0-9.0 months) (13 boys), while children in the RB1-/- group had a median age of 9.0 months (IQR, 4.6-13.4 months) (46 boys). MYCNARB1+/+ retinoblastomas were typically peripherally located (in 10 of 17 children; specificity, 97%; P < .001) and exhibited plaque or pleomorphic shape (in 20 of 22 children; specificity, 51%; P = .011) with irregular margins (in 16 of 22 children; specificity, 70%; P = .008) and extensive retina folding with vitreous enclosure (specificity, 94%; P < .001). MYCNARB1+/+ retinoblastomas showed peritumoral hemorrhage (in 17 of 21 children; specificity, 88%; P < .001), subretinal hemorrhage with a fluid-fluid level (in eight of 22 children; specificity, 95%; P = .005), and strong anterior chamber enhancement (in 13 of 21 children; specificity, 80%; P = .008). Conclusion MYCNARB1+/+ retinoblastomas show distinct MRI features that could enable early identification of these tumors. This may improve patient selection for tailored treatment in the future. © RSNA, 2023 Supplemental material is available for this article. See also the editorial by Rollins in this issue.

Characteristics and outcomes of gallbladder cancer patients at the Tata Medical Center, Kolkata 2017-2019.

BACKGROUND: The north and north-eastern regions of India have among the highest incidence of gallbladder cancer (GBC) in the world. We report the clinicopathological charateristics and outcome of GBC patients in India. METHODS: Electronic medical records of patients diagnosed with GBC at Tata Medical Center, Kolkata between 2017 and 2019 were analyzed. RESULTS: There were 698 cases of confirmed GBC with a median age of 58 (IQR: 50-65) years and female:male ratio of 1.96. At presentation, 91% (496/544) had stage III/IV disease and 30% (189/640) had incidental GBC. The 2-year overall survival (OS) was 100% (95% CI: 100-100); 61% (95% CI: 45-83); 30% (95% CI: 21-43); and 9% (95% CI: 6-13) for stages I-IV, respectively (p = <0.0001).   For all patients, the 2-year OS in patients who had a radical cholecystectomy followed by adjuvant therapy (N = 36) was 50% (95% CI: 39-64), compared to 29% (95% CI: 22-38) for those who had a simple cholecystectomy and/or chemotherapy (N = 265) and 9% (95% CI: 6-14) in patients who were palliated (N = 107) (p = <0.0001). CONCLUSION: The combined surgical/chemotherapy approach for patients with stage II GBC showed the best outcomes. Early detection of GBC remains problematic with the majority of patients presenting with stage III-IV and who have a median survival of 9.1 months. Our data suggests that the tumor is chemoresponsive and multi-center collaborative clinical trials to identify alternative therapies are urgently required.

Outcomes of clinico-radiologically advanced cancer (cT4b) of buccal mucosa: a retrospective analysis of 104 patients.

Although guidelines recommend non-surgical management for cT4b patients, recent studies have shown that upfront surgery in carefully selected patients can be performed with acceptable long-term survival benefit. In this study, we analysed the survival outcome of curative intent treatment on cT4b patients. Data from 104 patients who were staged cT4b and underwent upfront surgery for squamous cell carcinoma of buccal mucosa were retrospectively analysed. Outcome measures were locoregional recurrence-free survival (LRFS), disease-free survival (DFS) and overall survival (OS). The study cohort comprised 104 patients who had a median age of 52.5 years (range 27-81 years) and included 81 males (77.9%). Thirty-six patients had masticator space involvement on final histopathology, designating them as pT4b. Contrast enhanced computed tomography scan demonstrated 91.67% sensitivity in identifying masticator space involvement, albeit with a lower accuracy of 31.7%. Pathologically, clear margins were achieved in 79 (76%) patients. 62 (59.7%) and 20 (19.2%) patients received adjuvant radiotherapy (RT) and adjuvant chemoradiotherapy respectively. 2-year LRFS, DFS and OS were 61.8%, 60% and 68.1%, respectively. On multivariate analyses, involved margins (hazard ratio (HR) 28.766, p = 0.006), pN2b status (HR 4.68, p = 0.027) and perineural invasion (PNI) (HR 3.001, p = 0.027) showed statistically significant impact on LRFS, involved margins (HR 28.859, p = 0.008) and pN2b status (HR 4.018, p = 0.004) affected DFS. Involved margins (HR 14.139, p = 0.023) and pN2b status (HR 3.166, p = 0.025) showed statistically significant impact on OS. In conclusion, upfront surgery is a feasible option for patients with carcinoma of the buccal mucosa with the involvement of the masticator space. Survival outcomes are better in patients where resection is achieved with clear margins, and regional disease is limited to a single cervical lymph node.

Magnetic Resonance Imaging Can Reliably Differentiate Optic Nerve Inflammation from Tumor Invasion in Retinoblastoma with Orbital Cellulitis.

PURPOSE: To investigate the prevalence and magnetic resonance imaging (MRI) phenotype of retinoblastoma-associated orbital cellulitis. Additionally, this study aimed to identify postlaminar optic nerve enhancement (PLONE) patterns differentiating between inflammation and tumor invasion. DESIGN: A monocenter cohort study assessed the prevalence of orbital cellulitis features on MRI in retinoblastoma patients. A multicenter case-control study compared MRI features of the retinoblastoma-associated orbital cellulitis cases with retinoblastoma controls. PARTICIPANTS: A consecutive retinoblastoma patient cohort of 236 patients (311 eyes) was retrospectively investigated. Subsequently, 30 retinoblastoma cases with orbital cellulitis were compared with 30 matched retinoblastoma controls without cellulitis. METHODS: In the cohort study, retinoblastoma MRI scans were scored on presence of inflammatory features. In the case-control study, MRI scans were scored on intraocular features and PLONE patterns. Postlaminar enhancement patterns were compared with histopathologic assessment of postlaminar tumor invasion. Interreader agreement was assessed, and exact tests with Bonferroni correction were adopted for statistical comparisons. MAIN OUTCOME MEASURES: Prevalence of retinoblastoma-associated orbital cellulitis on MRI was calculated. Frequency of intraocular MRI features was compared between cases and controls. Sensitivity and specificity of postlaminar optic nerve patterns for detection of postlaminar tumor invasion were assessed. RESULTS: The MRI prevalence of retinoblastoma-associated orbital cellulitis was 6.8% (16/236). Retinoblastoma with orbital cellulitis showed significantly more tumor necrosis, uveal abnormalities (inflammation, hemorrhage, and necrosis), lens luxation (all P < 0.001), and a larger eye size (P = 0.012). The inflammatory pattern of optic nerve enhancement (strong enhancement similar to adjacent choroid) was solely found in orbital cellulitis cases, of which none (0/16) showed tumor invasion on histopathology. Invasive pattern enhancement was found in both cases and controls, of which 50% (5/10) showed tumor invasion on histopathology. Considering these different enhancement patterns suggestive for either inflammation or tumor invasion increased specificity for detection of postlaminar tumor invasion in orbital cellulitis cases from 32% (95% confidence interval [CI], 16-52) to 89% (95% CI, 72-98). CONCLUSIONS: Retinoblastoma cases presenting with orbital cellulitis show MRI findings of a larger eye size, extensive tumor necrosis, uveal abnormalities, and lens luxation. Magnetic resonance imaging contrast-enhancement patterns within the postlaminar optic nerve can differentiate between tumor invasion and inflammatory changes.

Germline Biallelic Mismatch Repair Deficiency in Childhood Glioblastoma and Implications for Clinical Management.

We report a case of a 9-year-old boy with glioblastoma with a past history of colon cancer. Germline bi-allelic DNA-mismatch repair deficiency was diagnosed by a lack of immunohistochemical staining for PMS2 in the tumor and normal tissue. Family history was lacking. Sequencing confirmed compound heterozygous PMS2 mutations. A second hit in the DNA-polymerase-ε gene led to complete DNA-replication repair deficiency. This contributed to an ultra-hypermutated phenotype. Temozolomide was excluded from the treatment. PD-1 immunotherapy at recurrence contributed to extending post-relapse survival up to 11 months. Challenges included managing initial immune "flare" related to "pseudo-progression" and access to drug. Family screening diagnosed the sibling with Lynch syndrome. This is the first report of a child with a brain tumor treated with immunotherapy from India. Our report supports the routine inclusion of immunohistochemistry for mismatch repair proteins in the evaluation of pediatric high-grade glioma as this may directly impact the clinical care of these children and families.

Inflammatory Lesions Mimicking Chest Malignancy: CT, Bronchoscopy, EBUS, and PET Evaluation From an Oncology Referral Center.

Infective and inflammatory diseases can mimic malignancy of the lung. Granulomatous inflammations are common causes of pulmonary nodule, mass, or nodal disease. Systemic infection or inflammation also commonly involves the lung that may raise suspicion of a malignant process. Even in patients with a known malignancy, inflammatory diseases can simulate new metastasis or disease progression. Knowledge of the imaging features of these diseases is essential to prevent missed or overdiagnosis of malignancy. Radiologists also need to be familiar with the scope and limitations of bronchoscopy, endobronchial ultrasound, PET-CT, and biopsy to guide clinical management. In this review, we discuss the imaging features and diagnostic approach of common mimickers of chest malignancy that involve the chest wall, pleura, lung parenchyma, and mediastinal nodes.

Imaging Features of Postradiotherapy Changes in Head and Neck Cancers.

Radiation therapy is the mainstay in the treatment of head and neck cancers, in addition to surgery and chemotherapy. Expected radiotherapy changes evolving over time may be confused with recurrent tumor. Conversely, even residual or recurrent tumor in the setting of postradiotherapy changes may be difficult to identify clinically or even by radiological imaging. Therefore, it is important to be familiar with the temporal evolution of these changes. The purpose of this pictorial essay is thus to illustrate distinctly the expected radiotherapy changes and radiotherapy-related complications in the head and neck region and to differentiate them from tumor recurrence on routine cross-sectional imaging techniques (computed tomography and magnetic resonance imaging).

Accuracy of MRI without intracavernosal prostaglandin E1 injection in staging, preoperative evaluation, and operative planning of penile cancer.

PURPOSE: To evaluate the performance of non-erectile MRI in staging and preoperative evaluation of penile carcinomas, compared to postoperative histopathology. METHODS: In this retrospective study, MRI scans of patients who had undergone surgery for penile carcinoma (n = 54) between January 2012 and April 2018 were read by two radiologists; and disagreement was solved in the presence of a third experienced radiologist. Data necessary for preoperative evaluation and staging were collected and compared with final postoperative histology and the type of surgery performed. All MRI had been performed without intracavernosal injection of prostaglandin E1 and with IV Gadolinium, as per local protocol. RESULTS: 54 patients were included in the study (mean age 57.52 ± 12.78). The number of patients with T1, T2, and T3 staging in histopathology were 32, 14, and 8. Moderate interobserver agreement was found for staging, disease-free penile length, and all subsites except urethra, which had weak agreement. Strong agreement of consensus MRI with final histopathological staging was found (49/54, weighted κ = 0.85), with high sensitivity and specificity. Sensitivity and specificity for involvement of corpus spongiosum, corpora cavernosa, and urethra were 95.5% and 93.8%, 87.5% and 97.8%, and 90.9% and 86.1%, respectively. Sensitivity (89.6%) and specificity (100%) of MRI for predicting adequate disease-free penile length were high. CONCLUSION: There were acceptable interobserver agreement and good diagnostic performance of MRI for staging and preoperative assessment without intracavernosal injection, especially for higher stages and higher degrees of invasion which require more extensive surgery.

The RSNA Pulmonary Embolism CT Dataset.

Supplemental material is available for this article.

Outcome of testicular non-seminomatous germ cell tumours: report from a tertiary cancer centre in eastern India.

Non-seminomatous germ cell tumour (NSGCT) is a rare but highly curable malignancy. The literature on the management and outcomes of NSGCT is scarce from India. Here, we report the demography and treatment outcomes of NSGCT treated at our centre. This is a retrospective analysis of testicular and retroperitoneal NSGCT patients treated from March 2011 to December 2019. Patients were staged appropriately with imaging, pre- and post-operative tumour marker. Patients received stage adjusted adjuvant treatment after high inguinal orchiectomy. Patients with advanced disease were risk stratified as per International Germ Cell Cancer Collaborative Group (IGCCCG) classification. A total of 100 patients were treated with a median age of 28 years (Range: 18-51). Primary site was testis in 92 and retroperitoneum in 8 patients. Testicular violation was present in 17 (18%) patients. The stage of the disease was I in 32, II in 19 and III in 49 patients, respectively. IGCCCG risk groups were good in 29 (46%), intermediate in 13 (21%) and poor in 21 (33%) patients. Eleven patients (24%) underwent retroperitoneal lymph node dissection amongst 45 with post-chemotherapy residual disease. After a median follow-up of 26.6 months (range: 2.2-100.7), 3-year event-free survival and overall survival (OS) were 70.7% ± 5.6% and 78.2% ± 5.4%, respectively. S3 tumour marker (p = 0.01) and non-pulmonary visceral metastasis (p < 0.001) emerged as independent poor prognostic factors for OS in multivariate analysis. To conclude, testicular NSGCT has very high cure rate. Two-third patients present with advanced disease and one-third of them had poor risk disease. S3 tumour marker and non-pulmonary visceral metastasis are poor risk factors for OS.

Multimodality treatment outcome in patients with primary malignant mediastinal germ cell tumor in adults.

BACKGROUND: Malignant mediastinal germ cell tumor (MGCT) is rare and has poor outcomes even after multimodality treatment. Data from resource-poor countries are scarce in the literature. AIMS: To evaluate the clinicopathologic features and treatment outcome of primary malignant MGCT at our center. METHODS AND RESULTS: Single institutional data review of patients aged ≥18 years, treated with a diagnosis of malignant MGCT between Nov'2013 and Nov'2019. Risk stratification was done as per International Germ Cell Cancer Collaborative Group (IGCCCG) classification. Patients were treated with platinum based chemotherapy and surgical resection for the residual disease was performed in non-seminomatous histology.28 patients had MGCT with a median age of 25 years (range:18-36) and all were male. Seven patients had superior vena cava obstruction (SVCO) at diagnosis and pre-treatment histological diagnosis was available in 23 (82%) patients. Seven (25%) patients had seminoma histology, all were of good risk as per IGCCCG risk criteria, whereas others had non-seminoma histology with poor-risk group. Seven patients with seminoma histology achieved a complete response after initial treatment. Six patients with non-seminoma histology underwent complete resection of residual disease post-chemotherapy and five revealed residual viable tumors. After a median follow-up of 10.8 months (range:2.9-75), 3-year progression-free survival (PFS) and overall survival (OS) estimate was 61.2% and 94.7% in the whole cohort, respectively and 3-year PFS and OS estimate was 100% in patients with seminoma histology. CONCLUSIONS: This is the largest data set of MGCT patients' outcomes reported from India with multi-modality treatment. All patients were male and one-fourth had SVCO at presentation. Seminoma histology patients had a 100% outcome after initial platinum based chemotherapy. But, those with non-seminoma histology had a poor outcome even with chemotherapy and surgery.

Imaging and Management of Rectal Cancer.

High-resolution phased array external magnetic resonance imaging (MRI) is the first investigation of choice in rectal cancer for local staging, both in the primary and restaging situations. Use of MRI helps differentiate between those with good prognosis, which can be offered upfront surgery and the poor prognostic cases where treatment intensification is needed. MRI identified poor prognostic factors are threatened or involved mesorectal fascia, T3 tumors with >5 mm extramural spread, those with extramural vascular invasion, pelvic sidewall nodes and mucinous tumors. At restaging, use of MRI helps evaluate response and an MR tumor regression grading system is being evaluated. Complete response seen on clinical examination and endoscopy, needs confirmation on MRI using both T2-weighted and diffusion-weighted sequences to justify a "watch and wait" approach. In this subset of patients, MRI also plays a role in monitoring and detecting early regrowth. In those with partial response, MRI helps define surgical margins and can be used as a roadmap to decide between sphincter preserving surgeries and radical sphincter sacrificing surgeries; pelvic exenteration and pelvic sidewall lymph node dissection. Poor responders on MRI may benefit from adjuvant chemotherapy. Use of MRI thus helps in individualizing treatment in rectal cancer.

Centrally inserted tunnelled peripherally inserted central catheter: Off-label use for venous access in oncology patients.

PURPOSE: Vascular access in oncology patients can often be challenging, especially after a few cycles of chemotherapy through peripheral lines which can cause veins to become attenuated. We evaluated the feasibility of centrally placed non-cuffed tunnelled peripherally inserted central catheter in the chest as an alternative to conventional peripherally inserted central catheter. METHOD: Patients referred for peripherally inserted central catheter found to have inadequate peripheral venous access in their arms due to prior chemotherapy, and therefore they were offered placement of the non-cuffed tunnelled peripherally inserted central catheter in the chest. Adult patients were subjected to the procedure under local anaesthesia, while paediatric patients underwent this procedure under general anaesthesia. Ultrasound guidance was used for venous access, and fluoroscopy was used for tip positioning. Using internal jugular vein access, BARD Groshong-valved 4F peripherally inserted central catheter was placed with its tip in the cavo-atrial junction. Proximal end of the catheter was brought out through the subcutaneous tunnel, so that the exit point of the peripherally inserted central catheter lies over the upper chest. Extra length of the catheter was trimmed, and extensions were attached. The device was stabilized with adhesive and sutures. RESULTS: Out of 19 patients, 18 patients were male (4-72 years). Technical success was achieved in 100% cases. No catheter-related bloodstream infection was noted within 30 days of peripherally inserted central catheter. Overall, during 1966 catheter days, no catheter-related bloodstream infection was observed. The purpose of peripherally inserted central catheter was achieved in 15 patients (78.9%) either in the form of completion of chemotherapy (8/15) or maintained peripherally inserted central catheter line till death (7/15). Partial or complete pullout was observed in four patients (20.1%), which required cuffed tunnelled catheter or implantable port. External fracture was noted in one patient, which was successfully corrected using repair kit. No exit site infection, bleeding, catheter occlusion, catheter dysfunction, venous thrombosis, venous stenosis or catheter embolizations were noted in our series. CONCLUSION: Centrally placed tunnelled peripherally inserted central catheter is a promising alternative method, when conventional arm peripherally inserted central catheter placement is not feasible. It is an easy and safe procedure that can be performed under local anaesthesia.

Incidental detection of Zinner syndrome in a patient with nonseminomatous germ cell tumor of testis.

Zinner syndrome is a rare congenital abnormality occurring in males comprising a triad of unilateral renal agenesis, ipsilateral ejaculatory duct obstruction, and seminal vesicle cyst. Most patients remain asymptomatic, and some may present with lower urinary tract symptoms or infertility. We present a case of incidentally detected Zinner syndrome in a patient with nonseminomatous germ cell tumor of testis, an association that is not reported in literature to our knowledge.

Computed tomography angiographic study of internal mammary perforators and their use as recipient vessels for free tissue transfer in breast reconstruction.

CONTEXT: The internal mammary artery perforator vessels (IMPV) as a recipient in free flap breast reconstruction offer advantages over the more commonly used thoracodorsal vessels and the internal mammary vessels (IMV). AIMS: This study was designed to assess the anatomical consistency of the IMPV and the suitability of these vessels for use as recipients in free flap breast reconstruction. PATIENTS AND METHODS: Data from ten randomly selected female patients who did not have any chest wall or breast pathology but had undergone a computed tomography angiography (CTA) for unrelated diagnostic reasons from April 2013 to October 2013 were analysed. Retrospective data of seven patients who had undergone mastectomy for breast cancer and had been primarily reconstructed with a deep inferior epigastric artery perforator free flap transfer using the IMPV as recipient vessels were studied. RESULTS: The CTA findings showed that the internal mammary perforator was consistently present in all cases bilaterally. In all cases, the dominant perforator arose from the upper four intercostal spaces (ICS) with the majority (55%) arising from the 2nd ICS. The mean distance of the perforators from the sternal border at the level of pectoralis muscle surface on the right side was 1.86 cm (range: 0.9-2.5 cm) with a mode value of 1.9 cm. On the left side, a mean of 1.77 cm (range: 1.5-2.1 cm) and a mode value of 1.7 cm were observed. Mean perforator artery diameters on the right and left sides were 2.2 mm and 2.4 mm, respectively. CONCLUSIONS: Though the internal mammary perforators are anatomically consistent, their use as recipients in free tissue transfer for breast reconstruction eventually rests on multiple variables.