Multi-institutional Series Research Articles

Pituitary gland duplication syndrome - An international imaging analysis.

Duplication of the pituitary gland is a rare developmental anomaly. Multiple associated craniofacial malformations have previously been reported with the largest series to date consisting of five patients. In this multi-institutional series of ten patients, we present a detailed review of the imaging features and discuss a possible overarching pathogenesis that would explain most of the detected malformations. Inclusion criteria for this retrospective imaging review were the presence of a pituitary stalk and gland duplication and the characteristic appearance of the hypothalamic ventral midline. In addition to the clinical presentation, we recorded the imaging findings of ten patients (9 female) through onsite and online reviews. Genetic analysis was available for six patients. The duplicated pituitary stalk and gland showed normal imaging appearances in all patients. Mammillary bodies were clearly identified lateral to the characteristic prominence of the hypothalamic ventral midline. Strands of tissue extending to the anterior dura ("limited ventral myeloschisis") were noted at the medulla oblongata in 10, and at the cervical spinal cord in 7 patients. The medulla oblongata showed a "butterfly" appearance on axial images in 9 patients. Ten patients had cervical segmentation anomalies ("zipper"-like), 9 anterior-posterior brainstem patterning defects (small pons, elongated medulla), and corpus callosum measurements were abnormal in all patients. Three patients each presented with diencephalic-mesencephalic junction abnormalities and 4 with an anterior mesencephalic "cap". An oropharyngeal teratoma was present in four patients. Genetics was normal in three of the six patients studied; the remainder were found to have mutations in EFNB1 and a gene variant of GIT1, two copies of 7. And 8. exon of SMN1 gene, and 2.126 megabase duplication at bands q11.1 and q11.2 of one chromosome 15, respectively. Duplication of the pituitary gland presents as well-defined craniofacial and cervical spine malformation phenotype. Axial mesoderm duplication generating an excess of Sonic Hedgehog may be the primary embryological driver leading to this condition. CFNS＝ Craniofrontonasal Syndrome; DPG＝ Duplication of the Pituitary Gland; SHH＝ Sonic Hedgehog.

Clear Cell Stromal Tumor of Lung: A Clinicopathologic, Immunohistochemical, and Molecular Characterization of 8 Cases

Clear cell stromal tumor (CCST) is a recently described mesenchymal neoplasm of the lung, characterized by spindle cells with variably clear-to-pale eosinophilic cytoplasm and prominent vascularity, as well as a recurrent YAP1::TFE3 gene fusion in most cases. Diagnosis can be challenging given its rarity and the lack of supportive immunohistochemical markers aside from TFE3. To date, less than 20 cases have been reported and data on clinical behavior are also limited. While most appear to be benign, aggressive behavior has been reported rarely. Here, we present the largest multi-institutional series of CCST to date, comprising a total of 8 cases and including 6 previously unpublished cases. We investigate its clinicopathologic and genomic features, while also assessing the diagnostic utility of immunohistochemistry (IHC) for YAP1 C-terminus (YAP-CT). Five patients were male and three were female. The median age was 59 years (range: 35 - 84). In all cases, a TFE3 rearrangement was demonstrated by either FISH or DNA / RNA sequencing. In 7 tumors, the YAP1::TFE3 fusion was identified by sequencing. We demonstrate that the combination of YAP1-CT loss and TFE3 overexpression by IHC reliably predicts an underlying YAP1::TFE3 fusion in these neoplasms and may be more sensitive than TFE3 FISH. Although the median follow-up time for our study was short (18 months, available in 7 cases), all cases pursued a benign clinical course, with no recurrences or metastases. Our study provides further characterization of this novel entity, supporting its wider recognition.

Psammoma bodies in thyroid: are they always indicative of malignancy? A multi-institutional study.

Traditionally, psammoma bodies (PB) have been considered as tale-tell evidence of papillary thyroid carcinoma (PTC) and are frequently encountered in classic and other subtypes of PTCs. However, the presence of PBs in the thyroid gland does not always indicate malignancy. The leading hypothesis on their origin suggests that PB are remnants of papillary structures that have undergone thrombosis, necrosis, and subsequent calcification. From January 2010 to May 2024, 26 patients with psammoma bodies associated with benign thyroid lesions, mainly thyroid follicular nodular disease (TFND), Hashimoto thyroiditis (HT), Graves' disease, and follicular adenomas, were found. The case cohort included 16 females and 10 males with a median age of 49.3years. The series included 12 TFND, two HT, and 12 follicular adenomas (11 out of 12 were oncocytic adenomas). Twenty-four out of 26 underwent total thyroidectomy. In 24 out of 26 cases, the entire lobes and parenchyma were included and serial cuts at multiple levels were performed in cases with PB but without any evidence of malignancy. Even though the detection of PB is associated with a malignant thyroid lesion, especially PTC and its subtypes, our multi-institutional series showed that in a minority PB can be found in a variety of benign thyroid lesions. Evaluation of the entire thyroid parenchyma at multiple levels is mandatory to exclude sub-centimeter papillary thyroid carcinoma.

Outcomes related to penile prosthesis reservoir removal: a 7-year multi-institutional experience.

The 3-piece inflatable penile prosthesis (IPP) is the most widely used device for erectile dysfunction refractory to medications, containing a reservoir inserted into the retropubic space (RPS) or an alternative/ectopic space (AES). Indications for removal of the reservoir include malfunction, malposition, or infection. In revision cases without infection, reservoir removal is sometimes optional. We reviewed outcomes and complications related to reservoir removal from a large multi-institutional series. We retrospectively reviewed databases at 6 institutions over 7years. Patients with artificial urethral sphincter, urethral sling, or mini-jupette were excluded. Outcomes and complications related to IPP reservoir removal were analyzed. Data were collected, but only reservoir-related complications at surgery were included. Data were compared between the RPS and AES cohorts to evaluate differences with a χ2 test, with significance at P < .05. Of 215 cases, there were 172 RPS and 43 AES reservoirs. The mean patient age was 65.3years. An overall 131 procedures were due to malfunction and 49 to malposition of an IPP component; 35 were secondary to infection. Among those retained (n = 44), reasons included reuse, avoiding surrounding structure damage, and difficult dissection. Among those removed (n = 171), 15 required a counterincision. To determine the statistical difference between those removed from the RPS and an AES, the χ2 test result was P = .00059, indicating a significant difference in the need for a counterincision between the groups. Complications included bladder perforation (n = 1) in the RPS group and an avulsion of the epigastric vessels requiring abdominal exploration (n = 1) in the AES group. To determine the statistical difference between RPS and AES complications, the χ2 test result was P = .365, indicating no significant difference between the groups. Strengths include being a multi-institutional study with high-volume skilled implanters. Limitations include being a retrospective review, with implanters exclusively performing penoscrotal incisions and not utilizing an infrapubic approach. Last, there was a lack of long-term follow-up with these patients. Removal of an IPP reservoir remains safe, with few intraoperative complications. Surgeons should be aware of the inferior epigastric vessels during removal in an AES or be willing to perform a counterincision to avoid injury to surrounding structures. Surgeons should also obtain preoperative imaging to identify the specific location of the reservoir and adjacent anatomy. This is the first multi-institutional study reviewing outcomes related to reservoir removal during IPP revision or removal surgery.

Prognostic stratification of patients with pT4bN0M0 colorectal cancer following multivisceral resection: a multi-institutional case series analysis.

Colorectal cancer (CRC) patients with stage pT4b are a complex group as they show differences in tumor-infiltrated organs. Patients with the same stage often exhibit differences in prognosis after multivisceral resection (MVR). Thus far, some important prognostic factors have not been thoroughly investigated. Here, we identified the prognostic factors influencing CRC patients at the pT4bN0M0 stage to stratify the prognostic differences among patients. A retrospective analysis was conducted on patients diagnosed with locally advanced CRC and who underwent MVR at three medical institutions from January 2010 to December 2021. The prognostic factors affecting the survival of CRC patients at pT4bN0M0 stage were identified by multivariate Cox proportional hazard models. We then classified the prognosis into different grades on the basis of these independent prognostic factors. We enrolled 690 patients with locally advanced CRC who underwent MVR; of these, 172 patients with pT4bN0M0 were finally included. Patients with digestive system [overall survival (OS): hazard ratio (HR)=0.441; 95% confidence interval (CI)=0.217-0.900; P =0.024; disease-free survival (DFS): HR=0.416; 95% CI=0.218-0.796; P =0.008) or genitourinary system invasion (OS: HR=0.405; 95% CI=0.193-0.851; P =0.017; DFS: HR=0.505; 95% CI=0.267-0.954; P =0.035) exhibited significantly better OS and DFS as compared to those with gynecological system invasion, while the OS and DFS were similar between the digestive system and genitourinary system invasion groups (OS: HR=0.941; 95% CI=0.434-2.042; P =0.878; DFS: HR=1.211; 95% CI=0.611-2.403; P =0.583). Multivariate analysis showed that age (OS: HR=2.121; 95% CI=1.157-3.886; P =0.015; DFS: HR=1.869; 95% CI=1.116-3.131; P =0.017) and type of organs invaded by CRC (OS: HR=3.107; 95% CI=1.121-8.609; P =0.029; DFS: HR=2.827; 95% CI=1.142-6.997; P =0.025) were the independent prognostic factors that influenced the OS and DFS of CRC patients with pT4bN0M0 disease. The OS and DFS of patients showing invasion of the gynecological system group were significantly worse ( P =0.004 and P =0.003, respectively) than those of patients with invasion of the nongynecological system group. On the basis of the above-mentioned two independent prognostic factors, patients were assigned to high-risk, medium-risk, and low-risk groups. Subgroup analysis showed that the OS and DFS of the medium-risk and high-risk groups were significantly worse ( P =0.001 and P =0.001, respectively) than those of the low-risk group. Patients with pT4bN0M0 CRC show significant differences in their prognosis. The type of organs invaded by CRC is a valuable indicator for prognostic stratification of CRC patients with pT4bN0M0.

High-grade B-cell lymphoma, not otherwise specified: CNS involvement and outcomes in a multi-institutional series

AbstractLittle is known about the central nervous system (CNS) risk in high-grade B-cell lymphoma, not otherwise specified (HGBL NOS). Hence, we sought to describe the rates of baseline CNS involvement, risk of CNS recurrence after primary therapy, and management strategies in HGBL NOS. In this multicenter retrospective study, we included 160 adults with newly diagnosed HGBL NOS treated between 2016 and 2021 at 20 US institutions. Eleven patients (7%) had baseline CNS involvement at diagnosis (leptomeningeal = 6, parenchymal = 4, and both = 1). Baseline CNS involvement was significantly associated only with MYC rearrangement (OR = 3.5) and testicular (in men) or female pelvic (in women) involvement (OR = 8.1). There was no significant difference in survival outcomes between patients with HGBL NOS with (median PFS = 4 years) or without (median PFS = 2.4 years) baseline CNS involvement (P = 0.45). The cumulative incidence of CNS recurrence at 3 years was 11%. Patients with baseline CNS involvement were at the highest risk (48.5% vs 8% for those without baseline CNS involvement) and were excluded from the risk factors analysis for CNS recurrence. The risk for CNS recurrence was significantly associated with blood or bone marrow involvement, CD5 expression, non–germinal center B-cell subtype, and “dual-expresser lymphoma” phenotype, however, high CNS IPI was not. The prognosis of relapsed HGBL NOS was poor, regardless of whether recurrence was systemic or limited to the CNS, and with currently available salvage strategies, including autologous transplantation and chimeric antigen receptor T-cell modalities, almost all patients with CNS recurrence ultimately succumbed to their disease.

Umbilical Cord Hemangiomas: A Multi-Institutional Case Series With Literature Review.

Umbilical cord hemangiomas are rare lesions, for which data on pregnancy outcome is lacking. This study combines a multi-institution 4-case series with a systematic literature search (n = 52) to determine possible pathologic lesion parameters which may have an effect on pregnancy outcome. Of all 56 pregnancies, lesion size ranged from 0.2 to 23.0 cm with pregnancy outcomes ranging from healthy liveborns (58.9%), liveborns with severe complications largely due to prematurity and/or fluid overload (12.5%), intrauterine/neonatal demise (25.0%), and pregnancy termination (3.6%). Of the 52 cases included for statistical analysis, there was no significant association between fetal outcome and vascular lesion location (P = .12) or fetal outcome and single umbilical artery involvement versus involvement of other vasculature (P = .29). The mean length of vascular lesions that resulted in healthy liveborns did not significantly differ from those resulting in severe fetal complications and/or demise (P = .72). Cases resulting in severe complications and/or demise were significantly earlier at delivery than those resulting in healthy liveborns (P < .001). Combined findings suggest that functional lesion characteristics, such as the degree of turbulent flow generated, have more significance than size, especially in early gestation losses. Moving forward, standardized reporting of pathologic lesion characteristics is paramount to better predict pregnancy prognosis.

Free flap reconstruction following head and neck trauma.

Free flap (FF) reconstruction of traumatic injuries to the head and neck is uncommon. Multi-institutional retrospective case series of patients undergoing FF reconstruction for a traumatic injury (n = 103). Majority were gunshot wounds (GSW; 85%, n = 88) and motor vehicle accidents (11%, n = 11). Majority underwent osseous reconstruction (82%, n = 84). FF failures (9%, n = 9/103) occurred in GSW patients (100%, n = 9/9) and when multiple subsites were injured (89%, n = 8/9). Preoperative antibiotics correlated with lower rates of a neck washouts (4% vs. 19%) (p = 0.01) and 30-day readmissions (4% vs. 17%) (p = 0.02). All FF failures occurred in the setting of a GSW and the majority involved multiple subsites. Preoperative antibiotics correlated with lower rates of postoperative washout procedures and 30-day readmission.

Fibula Free Flap Reconstruction of Cervical Spine Defects: A Multi-Institutional Study.

Cervical spine defects result in spinal instability, putting the spinal cord and vertebral arteries at risk of damage and possibly devastating neurological injuries. The fibula free flap can span the spinal defects for stability. There is a paucity of literature on this technique. Multi-institutional retrospective case series reviewing patients who underwent cervical spine reconstruction with a fibula free flap. Patient demographic information, comorbidities, characteristics of cervical spine defects, and free flap complications were collected. A total of 1187 fibula free flaps across 10 different institutions were reviewed. Thirteen patients (1.09%) underwent cervical spine reconstruction with a fibula free flap. Average age was 52.3 years old with an age range of 12-79 years. There were six males (46.1%) and seven females (53.8%). The most common defect etiology was infection (n = 6, 46.1%). Most commonly involved cervical spine level of the defect was C5 (n = 10) followed by C6 (n = 9) and C4 (n = 8). The majority of reconstructed defects spanned three or more cervical levels, (n = 9, 69.2%). Facial artery was the most common arterial anastomosis (n = 8). Eight patients (61.5%) required a tracheostomy during their postoperative course. None of the patients had symptomatic or radiographic nonunion. This case series demonstrates that a vascularized fibula flap is a potential reconstructive option for cervical spine defects, especially in defects greater than three cervical levels, in the setting of infection, and previously radiated patients. Level 4 Laryngoscope, 2024.

A multi-institutional series of a novel, recurrent TRIM24::MET fusion-driven infant-type hemispheric glioma reveals significant clinico-pathological heterogeneity

Within the past decade, incremental integration of molecular characteristics into the classification of central nervous system neoplasms increasingly facilitated precise diagnosis and advanced stratification, beyond potentially providing the foundation for advanced targeted therapies. We report a series of three cases of infant-type hemispheric glioma (IHG) involving three infants diagnosed with neuroepithelial tumors of the cerebral hemispheres harboring a novel, recurrent TRIM24::MET fusion. Histopathology showed glial tumors with either low-grade or high-grade characteristics, while molecular characterization found an additional homozygous CDKN2A/B deletion in two cases. Two patients showed leptomeningeal dissemination, while multiple supra- and infratentorial tumor manifestations were found in one case. Following subtotal resection (two cases) and biopsy (one case), treatment intensity of adjuvant chemotherapy regimens did not reflect in the progression patterns within the reported cases. Two patients showed progression after first-line treatment, of which one patient died not responding to tyrosine kinase inhibitor cabozantinib. As the detection of a recurrent TRIM24::MET fusion expands the spectrum of renowned driving fusion genes in IHG, this comparative illustration may indicate a distinct clinico-pathological heterogeneity of tumors bearing this driver alteration. Upfront clinical trials of IHG promoting further characterization and the implementation of individualized therapies involving receptor tyrosine kinase inhibition are required.

Editorial Comment on “Early Post-Operative Events After Urethroplasty in Obese Patients: A Multi-Institutional Retrospective Series”

Editorial Comment on “Early Post-Operative Events After Urethroplasty in Obese Patients: A Multi-Institutional Retrospective Series”

Tyrosine kinase inhibitors with and without upfront CNS radiation for brain metastases in oncogene-driven non-small cell lung cancer (TURBO-NSCLC).

2019 Background: Newer generation tyrosine kinase inhibitors (TKI) for NSCLC with EGFR mutations and ALK rearrangements have demonstrated encouraging central nervous system (CNS) activity with CNS objective response rates, greatly improved from 1st generation TKIs. In response to these data, guideline statements have acknowledged a strategy of CNS-penetrant TKI +/- upfront stereotactic radiosurgery (SRS) for the treatment of select patients with BM. However, optimal use of upfront SRS for BM in these patients is controversial since upfront CNS radiation has been the historical standard of care, and there are limited data guiding patient management with upfront TKI alone. Additionally, results from a large multi-institutional series reported inferior overall survival (OS) with the omission of SRS in patients with EGFR-mutated NSCLC treated with first-generation TKI. Methods: Data on TKI-naïve patients with EGFR- and ALK-driven NSCLC with BM treated with CNS-penetrant TKIs +/- upfront SRS were retrospectively collected from 7 centers in the United States. Time to CNS progression (PD), local CNS PD, and OS were analyzed, with multivariable adjustment (MVA) in Fine and Gray and Cox proportional hazards models for baseline factors including age, sex, performance status, mutation, extracranial metastases, prior therapy, neurologic symptoms, and number and size of BM. Results: We identified 317 patients (200 TKI only and 117 TKI+SRS). 250 (79%) and 61 (19%) patients received osimertinib and alectinib, respectively. Patients who received TKI+SRS were more likely to have BM ≥1 cm (p&lt;0.001) and neurologic symptoms (p&lt;0.001) at baseline. The median follow-up from treatment of BM was 23 months and 26 months in the TKI and TKI+SRS groups, respectively. Median OS was similar between the TKI and TKI+SRS groups (median 41 months [95% CI: 35-NR] vs 40 months [95% CI: 40-NR], respectively; p=0.5). On MVA, TKI+SRS was associated with a significant improvement in time to CNS PD (HR 0.63; 95% CI: 0.42-0.96; p=0.033). Local CNS control was significantly improved with TKI+SRS (HR 0.30, 95% CI: 0.16-0.55; p&lt;0.001), whereas no significant differences were observed in distant CNS control. Subgroup analyses demonstrated greater CNS control benefits with TKI+SRS in patients with BM ≥1 cm. Conclusions: This is the largest multi-institutional study comparing strategies of CNS-penetrant TKIs +/- upfront SRS in TKI-naïve patients with oncogene-driven NSCLC. The addition of SRS improved time to CNS PD and local CNS control but not OS. Patients with BM ≥1 cm may benefit the most from upfront integration of SRS. [Table: see text]

Early Post-Operative Events After Urethroplasty in Obese Patients: A Multi-Institutional Retrospective Series

ObjectiveTo compare early urethroplasty outcomes in non-obese, obese and morbidly obese patients undergoing urethroplasty for urethral stricture disease. The impact of obesity on outcomes is poorly understood but will be increasingly important as obesity continues to rise. MethodsPatients underwent urethroplasty at one of five institutions between January 2016 and December 2020. Obese (BMI 30–39.9, n=72) and morbidly obese (BMI > 40, n=49) patients were compared to normal weight (BMI <25, n = 29) and overweight (BMI 25-29.9, n=51) patients. Demographics, comorbidities, and stricture characteristics were collected. Outcomes including complications, recurrence, and secondary interventions were compared using univariate and multivariate analysis. Results201 patients (Mean BMI 34.1, Range 18.4-65.2) with mean age 52.2 years (SD=17.2) were analyzed. Median follow-up time was 3.71 months. Obese patients were younger (p=0.008), had more anterior (p<0.001), iatrogenic and LS-associated strictures (p=0.036). 60-day complication rate was 26.3% with no differences between cohorts (p= 0.788). 9.5% of patients had extravasation at catheter removal, 18.9% reported stricture recurrence, and 7.4% required additional interventions. Obese patients had greater estimated blood loss (p=0.001) and length of stay (p=0.001). On multivariate analysis, smoking associated with contrast leak (OR 7.176, 95% CI 1.13-45.5) but not recurrence or need for intervention (p=0.155, 0.927). ConclusionsObese patients in our cohort had more anterior, iatrogenic, and LS-related strictures. However, obesity is not associated with complications, contrast leak, secondary interventions, or recurrence. Obese had higher blood loss and length of stay. Urethroplasty is safe and effective in obese patients.

Endoscopic Endonasal Approach for Residual and Recurrent Craniopharyngioma after Transcranial Approach: A Multi-institutional Experience

Abstract Objectives While the endoscopic endonasal approach (EEA) has become a well-established surgery for resection of craniopharyngiomas (CP), the utility of this procedure following subtotal resection from open transcranial approach (TCA) surgery has yet to be explored. Here we present a multi-institutional case series of patients who underwent EEA for treatment of recurrent CP originally treated by TCA, demonstrating the viability of this approach as salvage surgery. Design Retrospective cohort. Setting Loyola University Medical Center and University of Pittsburgh Medical Center. Participants Patients who underwent EEA for recurrent CP following an initial TCA between 2003 and 2018. Main Outcome Measures Gross total resection (GTR) rate, surgical complications, visual outcomes, and endocrine outcomes Results Patients who underwent EEA for recurrent CP following a prior TCA had a GTR rate of 77%. EEA reoperation was not associated with worsening of visual outcomes as compared with the primary TCA (0% visual worsening after EEA reoperation vs. 40% after primary TCA, p = 0.055). While primary TCA resulted in worsening endocrinopathies in 100% of patients (including seven patients with permanent diabetes insipidus), subsequent EEA for re-resection of CP resulted in stable (30.8%) or improved (69.2%) endocrine function in all patients (p &lt; 0.001). Conclusions EEA achieves reasonable GTR rates and is an effective, safe surgical option for recurrent CP postinitial TCA. This approach demonstrated stable or improved visual and endocrinologic outcomes in all patients within our two-institution series. Thus, EEA should be considered as an efficacious form of retreatment in cases of progressive and recurrent CP.

Treatment at relapse for synovial sarcoma of children and adolescents: A multi-institutional European retrospective analysis.

Though the prognosis for pediatric patients with localised synovial sarcoma (SS) is generally good, the chances of being cured after relapse are limited. This study describes a retrospective multi-institutional series of relapsing SS patients treated at six selected European referral centers for pediatric sarcoma. The study included 41 patients<21 years with relapsing SS, treated between 2002 and 2022. The analysis included patient's characteristics at first diagnosis, first-line treatments, clinical findings at relapse, and second-line treatment modalities. The first relapse occurred within 3-132 months (median 18 months) after first diagnosis and was local in 34%, metastatic in 54%, and both in 12%. Treatment at first relapse included surgery in 56% of cases, radiotherapy in 34%, and systemic therapy in 88%. In all, 36 patients received second-line medical treatment, that was chemotherapy in 32 cases (with 10 different regimens) and targeted therapy in four. No patient was included in an early-phase clinical trial as second-line therapy-line therapy. Overall response rate was 42%. Median event-free survival (EFS) was 12 months, postrelapse 5-year EFS was 15.8%. Median overall survival (OS) was 30 months, postrelapse 5-year OS was 22.2%. At the Cox's multivariable regression analysis, OS was significantly associated with time and type of relapse. Pediatric patients with relapsed SS have a poor prognosis and generally receive an individualized approach, due to the lack of a uniform standardized approach. New comprehensive strategies are needed to improve the knowledge on the biologic landscape of SS and develop tailored prospective clinical trials.

Endoscopic repair of tracheoesophageal fistulas: A contemporary multi-institutional case series and literature review

ObjectivesRecurrent and primary tracheoesophageal fistulas (TEFs) are a challenging surgical pathology to treat, as standard open surgical approaches are associated with high morbidity and mortality. As such, endoscopic modalities have gained interest as an alluring alternative, yet variable success rates have been reported in the literature. The aim of this study was to provide a contemporary update of the literature and describe our institutional experience with the bronchoscopic obliteration of recurrent and primary TEFs. MethodsRetrospective chart review of all pediatric patients having undergone endoscopic TEF repair at two pediatric academic centers in Montreal, Canada and Lille, France between January 1, 2008 to December 31, 2020. Results28 patients with TEFs (20 recurrent, 8 primary) underwent a total of 48 endoscopic procedures. TEF repair was performed under endoscopic guidance using various combinations of techniques, including fistula de-epithelialization (endoscopic brush, thulium laser, trichloroacetic acid-soaked pledgets or electrocautery), tissue adhesives, submucosal augmentation, esophageal clip and stenting. Successful closure was achieved in 16 patients (57 %), while 12 (43 %) required eventual open or thoracoscopic repair. The mean number of endoscopic procedures was 1.7. There were no major treatment-related complications such as pneumothorax, mediastinitis or death (mean follow-up 50.8 months). ConclusionsEndoscopic repair of recurrent or primary TEFs is a valuable component of our therapeutic armamentarium and may contribute to decreased surgical morbidity in this complex patient population. Families should be counselled that endoscopic results may be more modest than with open or thoracoscopic approaches, and multiple procedures may be required.

Brain magnetic resonance imaging and computed tomography findings in cats and dogs with central nervous system cryptococcosis in Australia: 23 cases (2009-2020).

To describe the imaging findings in Australian cats and dogs with CNS cryptococcosis. 23 cases (10 cats; 13 dogs) with CNS cryptococcosis and brain MRI or CT studies available to review. Retrospective, multi-institutional case series. Brain MRI or CT studies were reviewed by a board-certified radiologist. Imaging findings were described and the differences between cats and dogs explored. Morphologic features were consistent with extra-axial lesions in all (n = 13) dogs and either intra-axial (5/10) or extra-axial (4/10) lesions in cats, with 1 cat having no detectable lesions in low-field brain MRI scans. Meningeal abnormalities were most common, followed by forebrain and cerebellar lesions. Intracranial MRI lesions were typically T2 hyperintense and T1 hypo- to isointense. Four cases had T2 hypointense lesions affecting the brain, sinonasal cavity, or regional lymph nodes. Intracranial CT lesions were mostly soft tissue attenuating. Contrast enhancement was present in all cases with contrast series available, with ring enhancement shown only in cats. Osteolysis was more common in dogs than cats, particularly affecting the cribriform plate. All 13 dogs and many (6/10) cats had at least 1 lesion affecting sinonasal or contiguous tissues, and locoregional lymphadenomegaly was common (7/10 cats; 11/13 dogs). Imaging lesions in cryptococcal meningoencephalitis were extra-axial in dogs but could be intra-axial or extra-axial in cats. Careful examination for extracranial lesions (sinonasal, retrobulbar, facial soft tissue, tympanic bullae, or locoregional lymph nodes) is important to provide alternative safe biopsy sites. T2 hypointense lesions, while rare, should prompt consideration of cryptococcosis.

1077 Operative Management and Outcomes in Spinal Hemangioblastoma: A Multi-institutional Case Series of 59 Patients

INTRODUCTION: Hemangioblastoma is a rare benign neoplasm that accounts for 2-5% of all spinal cord tumors and is associated with von-Hippel Landau (VHL) disease in about 25-30% of patients. Symptomatic spinal hemangioblastomas are typically managed with en bloc microsurgical resection, but the infrequent nature of these lesions has limited the detailed characterization of reported management and surgical outcomes in the literature. METHODS: We retrospectively reviewed all patients with pathology-proven spinal hemangioblastoma. We collected information about demographics, preoperative symptoms and imaging, intraoperative characteristics, and postoperative course. We analyzed what variables influence postoperative changes in motor scores and McCormick scores. RESULTS: We included 59 patients (35 sporadic, 24 VHL). The average age of diagnosis was 48.7 (± 16.1) for sporadic tumors and 35.1 (± 12.6) for those associated with VHL (p = 0.001). Most tumors were cervical (46.6%) or thoracic (34.5%) and both intradural (93.2%) and intramedullary (71.2%). Most patients (69.5%) had multiple spinal tumors. The average lesion volume was 0.98 (± 1.1) cm3. The average symptom duration before surgery was 24.7 (± 73.2) months. Adjuvant therapies included radiation (6.8%) and chemotherapy (1.7%). At 1 month postoperatively, 4.4% had motor loss in any muscle group and 31.1% had motor gain, while 18.6% had a decreased McCormick score and 28.8% had an improved score at 1 month. While 72.9% of patients had numbness/paresthesia preoperatively, only 59.3% did at 1 month. Duration of pre-operative symptoms and time from diagnosis to surgery were not found to influence outcomes. CONCLUSIONS: Overall, surgical planning should be guided by symptom management. In this population, duration of preoperative symptoms and timing of surgery did not influence post-operative functional outcomes.

Perivascular Epithelioid Cell-Family Tumors in Children, Adolescents, and Young Adults: Clinicopathologic Features in 70 Cases.

Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors of uncertain histogenesis expressing smooth muscle and melanocytic markers. The clinicopathologic spectrum in young patients is not well documented. To describe a multi-institutional series of PEComas in children, adolescents, and young adults. PEComas, not otherwise specified (NOS); angiomyolipomas (AMLs); lymphangioleiomyomatosis; and clear cell sugar tumors were retrospectively identified from 6 institutions and the authors' files. Seventy PEComas in 64 patients (median age, 15 years) were identified. They were more common in females (45 of 64 patients), occurring predominantly in the kidney (53 of 70), followed by the liver (6 of 70). Thirty-four patients had confirmed tuberous sclerosis complex (TSC), 3 suspected TSC mosaicism, 2 Li-Fraumeni syndrome (LFS) and 1 neurofibromatosis type 1. Most common variants were classic (49 of 70) and epithelioid (8 of 70) AML. Among patients with AMLs, most (34 of 47) had TSC, and more TSC patients had multiple AMLs (15 of 36) than non-TSC patients (2 of 13). Two TSC patients developed malignant transformation of classic AMLs: 1 angiosarcomatous and 1 malignant epithelioid. Lymphangioleiomyomatosis (5 of 70) occurred in females only, usually in the TSC context (4 of 5). PEComas-NOS (6 of 70) occurred exclusively in non-TSC patients, 2 of whom had LFS (2 of 6). Three were malignant, 1 had uncertain malignant potential, and 2 were benign. All 4 PEComas-NOS in non-LFS patients had TFE3 rearrangements. Compared to the general population, TSC was more prevalent in our cohort; PEComas-NOS showed more frequent TFE3 rearrangements and possible association with LFS. This series expands the spectrum of PEComas in young patients and demonstrates molecular features and germline contexts that set them apart from older patients.

The Utilization of Fine-Needle Vasography to Localize Obstruction of the Male Reproductive Tract

ObjectivesTo report our experience using fine needle vasography (FNV) to identify a site of obstruction and determine candidacy for reconstructive procedures in patients presenting with suspected obstructive azoospermia or cryptozoospermia. MethodsWe report a multi-institutional case series of patients with suspected complete or partial obstructive azoospermia. Patients that were included had azoospermia or cryptozoospermia, presence of palpable vasa, testicles ≥12 ml, and FSH <7.6 IU/L. All patients underwent testicular biopsy prior to or at the time of FNV to confirm spermatogenesis. FNV was performed using a 25-gauge angiocatheter, with radio-opaque dye visualized under fluoroscopy. Descriptive statistics are reported as median and interquartile range (IQR). ResultsA total of 16 patients underwent vasography from 2014-2022 with three surgeons. Twelve patients presented with azoospermia, and four with cryptozoospermia. A total of 7 (44%) men were found to have distal obstruction on FNV. Of the eight men with prior inguinal hernia repairs, two were confirmed to have an obstruction at the level of the inguinal canal. Of the six patients with a history of genitourinary infection, four had an obstruction at the level of the epididymis (with normal FNV), while two had atresia of the vas deferens in the pelvis. ConclusionFNV is an effective, minimally-invasive way to identify the site of complete or partial obstruction in patients presenting with suspected obstructive azoospermia / cryptozoospermia. It additionally permits identification of men who are candidates for epididymovasostomy reconstruction and helps to differentiate between ejaculatory duct obstruction (EDO) and other causes of blockage.