The urachus is a vestigial structure that connects the bladder to the allantois in utero and subsequently obliterates into the fibrous median umbilical ligament. Urachal adenocarcinoma (UrC) is an extremely rare malignancy, comprising less than 1% of all bladder tumors, and may arise from any remnant component of the urachus—most often from the dome of the bladder. 1 Paner GP Lopez-Beltran A Sirohi D Amin MB Updates in the pathologic diagnosis and classification of epithelial neoplasms of urachal origin. Adv Anat Pathol. 2016; 23: 71-83 Crossref PubMed Scopus (50) Google Scholar ,2 Pinthus JH Haddad R Trachtenberg J et al. Population based survival data on urachal tumors. J Urol. 2006; 175: 2042-2047 Crossref PubMed Scopus (111) Google Scholar The clinical course of UrC involves nonspecific signs and symptoms often leading to eventual detection only after local spread. Overall prognosis is poor with an estimated 5-year survival of 20% to 50%. 2 Pinthus JH Haddad R Trachtenberg J et al. Population based survival data on urachal tumors. J Urol. 2006; 175: 2042-2047 Crossref PubMed Scopus (111) Google Scholar ,3 Gopalan A Sharp DS Fine SW et al. Urachal carcinoma: a clinicopathologic analysis of 24 cases with outcome correlation. Am J Surg Pathol. 2009; 33: 659-668 Crossref PubMed Scopus (197) Google Scholar Diagnostic criteria are debated but frequently emphasize the presence of a tumor at the dome of a bladder with no evidence of a primary neoplasm elsewhere. 3 Gopalan A Sharp DS Fine SW et al. Urachal carcinoma: a clinicopathologic analysis of 24 cases with outcome correlation. Am J Surg Pathol. 2009; 33: 659-668 Crossref PubMed Scopus (197) Google Scholar , 4 Sheldon CA Clayman RV Gonzalez R Williams RD Fraley EE Malignant urachal lesions. J Urol. 1984; 131: 1-8 Crossref PubMed Scopus (434) Google Scholar , 5 Mostofi FK Thomson RV Dean AL Mucous adenocarcinoma of the urinary bladder. Cancer. 1955; 8: 741-758 Crossref PubMed Scopus (277) Google Scholar In fact, metastatic adenocarcinomas of unknown primary origin may often be due to an unidentified urachal malignancy, although there remains a paucity of data on its incidence. 6 Sahu KK Pandey D Mishra AK et al. Mystery of neck lump: an uncommon presentation of urachal cancer. BMJ Case Rep. 2019; 12e230215 Crossref Scopus (3) Google Scholar ,7 Suzuki K Kai Y Matsuda M et al. A rare case of urachal carcinoma with multiple lung metastasis that required differentiation from primary lung carcinoma. Respirol Case Rep. 2022; 10: e0890 Crossref PubMed Scopus (1) Google Scholar In the most recent classification of genitourinary tumors, urachal epithelial malignancies are divided histologically into glandular, nonglandular and mixed neoplasms with further subtypes; the majority of urachal malignancies are glandular adenocarcinomas with mucinous or enteric features. 1 Paner GP Lopez-Beltran A Sirohi D Amin MB Updates in the pathologic diagnosis and classification of epithelial neoplasms of urachal origin. Adv Anat Pathol. 2016; 23: 71-83 Crossref PubMed Scopus (50) Google Scholar ,8 Humphrey PA Moch H Cubilla AL Ulbright TM Reuter VE The 2016 WHO Classification of tumours of the urinary system and male genital organs—Part B: prostate and bladder tumours. Eur Urol. 2016; 70: 106-119 Abstract Full Text Full Text PDF PubMed Scopus (1036) Google Scholar ,9 Netto GJ Amin MB Berney DM et al. The 2022 World Health Organization classification of tumors of the urinary system and male genital organs—Part B: prostate and urinary tract tumors. Eur Urol. 2022; 82: 469-482 Abstract Full Text Full Text PDF PubMed Scopus (59) Google Scholar The prognostic relevance of these subtypes is currently unclear. 1 Paner GP Lopez-Beltran A Sirohi D Amin MB Updates in the pathologic diagnosis and classification of epithelial neoplasms of urachal origin. Adv Anat Pathol. 2016; 23: 71-83 Crossref PubMed Scopus (50) Google Scholar ,10 Benerjee N Parmar K Vaiphei K Primary signet-ring cell carcinoma of the urinary bladder. Autops Case Rep. 2021; 11e2021264 Crossref PubMed Google Scholar The mainstay of treatment for all subtypes is en bloc resection. 4 Sheldon CA Clayman RV Gonzalez R Williams RD Fraley EE Malignant urachal lesions. J Urol. 1984; 131: 1-8 Crossref PubMed Scopus (434) Google Scholar There is remarkable molecular overlap between UrC and colorectal adenocarcinoma (CRC). Global transcriptome profiling and targeted exon sequencing of UrC has shown high similarity of RNA expression (via clustering analysis) as well as genetic alterations (eg, APC, SMAD4, KRAS, microsatellite instability) between UrC and CRC. 11 Kardos J Wobker SE Woods ME et al. Comprehensive molecular characterization of urachal adenocarcinoma reveals commonalities with colorectal cancer, including a hypermutable phenotype. JCO Precis Oncol. 2017; 1 (PO.17.00027) Google Scholar The enteric subtype of UrC also demonstrates a histologic pattern of stratified columnar epithelium similar to that of CRC. Thus, 5-fluorouracil (5-FU) based therapies have often been employed with moderate efficacy in metastatic cases of UrC. 12 Siefker-Radtke AO Gee J Shen YU et al. Multimodality management of urachal carcinoma: The M. D. Anderson Cancer Center Experience. J Urol. 2003; 169: 1295-1298 Crossref PubMed Scopus (223) Google Scholar The role of targeted therapies and immunotherapies has increasingly been investigated in contemporary single case reports. We present the cases of 5 patients with UrC and review literature regarding emerging therapies.

BackgroundSickle cell disease (SCD) is a diverse group of blood disorders with significant global disease burden. Contemporary interest in the underlying inflammatory paradigm of SCD has emphasized the role of the neutrophil-lymphocyte ratio (NLR) as a prognostic inflammatory marker.MethodsWe retrospectively reviewed 268 hospitalized patients with SCDs of different genotypes (HbSS, HbS & beta;(0) thalassemia, HbS & beta;(+) thalassemia, and HbSC), totaling 3329 hospital admissions over a 10-year period. Patients were stratified into SS/S & beta;(0) and S & beta;(+)/SC groups for statistical analysis of parameters collected at steady state and at hospital admission.ResultsAt steady state, per unit increase of hemoglobin values was associated with reduced odds of & GE; 2 hospital admissions per year in SS/S & beta;(0) and S & beta;(+)/SC groups; per unit increase in platelet count and white blood cell count was associated with increased odds only in the SS/S & beta;(0) group. The NLR had no association in either group. During admission, a cutoff of NLR = 3.5 discerned infection with a sensitivity of 60% and specificity of 57%. Performance improved when excluding patients on outpatient hydroxyurea therapy (cutoff of NLR = 3.5; sensitivity of 68% and specificity of 64%).ConclusionThis study supports the utility of NLR as an accessible adjunctive clinical tool in SCD prognostication.

Abstract Background Thymic epithelial tumors are rare and include thymomas and thymic carcinomas. There is scarce literature characterizing prognostic factors and long‐term outcomes in these tumors. Aims This review aims to describe disease features of thymomas and thymic carcinomas and to report clinical differences among thymoma histological subtypes. Methods and Results A retrospective chart review was performed at the University of Florida Shands Hospital, a tertiary care academic medical center in Gainesville, Florida, USA. The review included clinical data of adults with thymic epithelial tumors diagnosed between 2001 and 2021. Significant associations among demographics, histology, stage, and outcomes were investigated. Thymoma subgroup analysis was performed using histological subtype and sex. Forty patients with thymoma and seven patients with thymic carcinoma were included in the final analysis. Among those with thymomas, patients with subtype B1, B2, or B3 tumors were younger, had larger tumors, and presented with higher stage disease when compared to those with subtypes A or AB. Tumor recurrence was most common in subtype B2 and B3 tumors (50.0% and 16.7% vs. 0%; p < .01). However, there was no significant difference in overall survival between histologic subtypes. Compared to females, males with thymomas had superior overall survival (103.0 vs. 62.9 months; p = .021) despite presenting with larger tumors (9.8 vs. 5.8 cm; p = .041). Concomitant myasthenia gravis was associated with increased recurrence but not worsened mortality. Compared to thymomas, patients with thymic carcinoma presented with higher‐stage disease and had poorer 5‐year survival (50.0% vs. 93.1%; p < .01). Conclusion This study affirmed pathologic stage and resectability as prognostic factors for thymic epithelial tumors. New findings include inferior overall survival in female patients and higher recurrence rates in those with thymomas and concomitant myasthenia gravis.

Background Primary cardiac tumors are often benign and commonly present as cardiac myxomas (CMs) or papillary fibroelastomas (CPFEs). There is a paucity of prognostic indicators for tumor burden or potential for embolic cerebrovascular events (CVEs). This study was performed to address these gaps. Methods Medical records at the University of Florida Health Shands Hospital between 1996 and 2021 were screened to identify patients with CMs or CPFEs. Clinical features, echocardiographic reports, and CVE outcomes were quantitatively assessed. Results A total of 55 patients were included in the study: 28 CM (50.9%) and 27 CPFE (49.1%) patients. Baseline patient characteristics were similar among patients. The neutrophil–lymphocyte ratio was correlated ( p < 0.005 in all cases) to three metrics of tumor size in both CM ( r = 64–67%) and CPFE ( r = 56–59%). CVEs were the presenting symptom in 30 (54.5%) patients. CVE recurrence was high; the 5-year CVE recurrence rate in patients with tumor resection was 24.0% compared to 60.0% without resection. No baseline patient characteristics or tumor features were associated with an initial presentation of CVEs compared to any other indication. Univariate analysis indicated that prolonged duration to surgical resection, left atrial enlargement, male sex, and a neutrophil–lymphocyte ratio >3.0 at the follow-up were significantly associated with 5-year CVE recurrence. Left atrial enlargement and a neutrophil–lymphocyte ratio >3.0 at the follow-up remained significantly associated with 5-year CVE recurrence in multivariate analysis. Conclusion The neutrophil–lymphocyte ratio may prognosticate tumor size and recurrence of neurologic events. An increased risk of CVE within 5 years of mass resection is almost exclusive to patients initially presenting with CVEs.

e16306 Background: Duodenal adenocarcinoma (DA) is a rare malignancy with poor outcomes. Tumor markers are used to assess disease response and to monitor for recurrence. Specifically, CA-19-9 and CEA have been validated for use in pancreatic cancer and colorectal cancer, respectively. However, these tumor markers have never been validated in patients with DA. We aim to assess the association of these biomarkers with clinical outcomes in patients with DA. Methods: This is a retrospective cohort study. After obtaining IRB approval (IRB202102705), we accessed the University of Florida medical records of patient treated for DA from January 1, 2006, until December 31, 2021. CA 19-9 and CEA were collected as continuous variables and were analyzed as binary variables: normal vs. high, using the maximum normal value as a cut-off (normal CA 19-9 < = 35 U/ml; CEA < = 3 ng/ml). Analysis was conducted using Kaplan Meyer curves, log-rank test and Cox proportional hazards model. Results: A total of 68 patients were included in the final analysis. Median age was 67 years and median follow-up was 22.2 months. CA 19-9 and CEA were elevated in 36.8% and 48.5% of patients, respectively. Patients with an elevated CA 19-9 had a median overall survival (OS) of 8.5 months vs. 27.4 months in patients with normal levels (HR 1.67; 95%CI 0.94–2.99; p = 0.081). Patients with an elevated CEA had a median OS of 13.4 months vs. 16.8 months in patients with normal level normal levels (HR 1.43; 95%CI 0.81–2.52; p value = 0.221). In a sensitivity analysis, a concomitant elevation of both tumoral markers was significantly associated with worsened OS (HR 1.9; 95%CI 1.05–3.06; p = 0.035). Conclusions: In patients with duodenal adenocarcinoma, elevation of both CA 19-9 and CEA was associated with a statistically significant worse overall survival. CA 19-9 level had a higher prognostic impact on OS than CEA levels. To our knowledge, this is the first study to evaluate the role of CA 19-9 and CEA in patients with DA. Further research is required for validation.[Table: see text]

Adrenal insufficiency is one of the most common endocrine disorders that presents in patients with HIV. Aetiologies of adrenal dysfunction include opportunistic infection, malignancy, such as lymphoma or Kaposi sarcoma, and chronic cytokine-mediated disruption of the hypothalamic-pituitary-adrenal axis. In the case of lymphoma, the manifestation of adrenal insufficiency is most often via primary neoplastic infiltration. However, a spectrum of associated cytokine-mediated abnormal immune responses and coagulopathies may independently contribute to adrenal insufficiency. Literature regarding the presence of the endocrine disorder in patients with both HIV and lymphoma is scarce. We report a case of adrenal insufficiency in a patient with well-controlled HIV and advanced Hodgkin lymphoma without primary adrenal involvement with suboptimal response to corticosteroids who exhibited improvement following initiation of chemotherapy, demonstrating that chemotherapy should not be delayed until adrenal insufficiency resolves and in fact may aid in resolution of adrenal dysfunction.

Synchronous colorectal cancer is a rare subtype of colorectal carcinoma defined by the presence of 2 or more primary tumors simultaneously or within 6 months of initial detection. The overall impact of a synchronous presentation on prognosis is not yet clear. Surgical resection is the primary treatment. However, higher rates of local recurrence and metastasis in synchronous colorectal cancer demand greater exploration of the role of adjuvant therapy. The increased frequency of microsatellite instability observed in synchronous colorectal cancer also affects therapy selection. Similarly, activating PIK3CA mutations are regularly noted in colorectal cancer, but their role in a synchronous presentation has not yet been described. We report a case of a young patient with a synchronous recto-sigmoid colorectal carcinoma complicated by microsatellite instability and an activating PIK3CA mutation-a presentation as of yet unreported in literature. We also review the impact of these molecular events on the efficacy of several chemotherapies and targeted therapies.