Tonsillar Kaposi sarcoma (KS) is an extremely rare manifestation of KS, a neoplasm associated with human herpesvirus-8 and often linked to immunocompromised individuals. This study aims to analyze the outcomes and prognostic factors of tonsillar KS using data from the SEER (Surveillance, Epidemiology, and End Results) database, covering cases from 2000 to 2020. A total of 42 patients were identified, with the vast majority being male (97.6%) and a mean age of 40.1 years. Tumors were primarily localized (42.9%), and treatment modalities varied, with radiation therapy being the most common (38.1%), followed by chemotherapy (21.4%) and surgery (19.0%). Survival analysis showed that 21.4% of patients had died by the last follow-up, but the median survival time was not reached, indicating a relatively favorable prognosis. No statistically significant survival differences were observed based on treatment modality or other demographic factors, though there were trends towards worse outcomes for patients with regional disease and longer delays in treatment initiation. The study highlights the rarity of tonsillar KS, its demographic profile, and suggests a generally positive prognosis, although further research is needed to optimize treatment strategies for this rare form of KS.

Background: Spindle cell variants of squamous cell carcinoma represent rare and aggressive malignancies with poorly understood prognostic factors and treatment outcomes. This study leverages machine learning approaches alongside traditional statistical methods to analyze a large cohort from the Surveillance, Epidemiology, and End Results (SEER) database. Methods: We conducted a retrospective analysis of 1086 patients with spindle cell variants of squamous cell carcinoma from the SEER database. Traditional Cox regression and machine learning approaches, including random survival forests (RSF), gradient boosted survival (GBSurv), and DeepSurv models, were employed to identify prognostic factors and predict survival outcomes. Model performance was evaluated using concordance indices and decision curve analysis. Results: Of the 1086 patients included in the analysis, patients were diagnosed with spindle cell variants of squamous cell carcinoma between 1992 and 2021. Median age was 70 years (IQR: 60–77). Primary tumor sites included larynx (21.4%), lung and bronchus (20.2%), and tongue (10.7%). 32.0% had localized disease, 25.4% had regional disease, and 17.2% had distant disease, with 25.4% having unknown stage. Treatment modalities included radiation therapy in 51.9% and chemotherapy in 30.7% of patients. In the multivariate Cox model, kidney and renal pelvis tumors showed the highest risk (HR: 6.28, 95% CI: 2.26–17.45, P < 0.001), followed by urinary bladder (HR: 2.72, 95% CI: 1.56–4.74, P < 0.001) and lung/bronchus sites (HR: 1.94, 95% CI: 1.51–2.50, P < 0.001). The RSF model demonstrated superior discriminative ability (C-index: 0.733, 95% CI: 0.680–0.784) compared to GBSurv (C-index: 0.294, 95% CI: 0.239–0.351) and DeepSurv (C-index: 0.314, 95% CI: 0.255–0.378) approaches. Conclusions: The findings suggest that anatomical site and disease stage significantly influence survival, while current treatment modalities show limited impact on outcomes. The superior performance of the RSF model indicates potential value in using machine learning for risk stratification in clinical practice.

Background: Nuclear protein of the testis (NUT) carcinoma (NC) is a rare and exceedingly aggressive neoplasm, typically presenting as midline tumors such as mediastinal, thoracic, or sinonasal tumors, but can also be seen in other locations like the kidneys, liver, and pancreas. NC is frequently underdiagnosed or misdiagnosed due to its rarity, nonspecific clinical and histopathological presentation, lack of clinically trialed standard-of-care therapy, and, ultimately, a lack of awareness about the condition. Methods: We retrospectively reviewed 45 cases of NC from the Surveillance, Epidemiology, and End Results (SEER) database. Results: The patient pool was predominantly male, with the most common tumor sites being the lungs and bronchus (51.1%), followed by the nasal cavity and paranasal sinuses (28.9%).73.3% of patients received systemic therapy, 62.2% received radiotherapy, and only one-quarter of patients underwent surgery. The median survival time was 8 months (95% CI: 4.09–11.91). Systemic therapy (aHR = 0.268, P = 0.009) and radiotherapy (aHR = 0.335, P = 0.02) were the only independent prognostic factors in this cohort. Conclusion: Our study reiterates the rarity of NC in the United States. We also reveal that when faced with the rare NC, systemic therapy and radiotherapy have been found to relatively prolong NC patient survival. Future research is needed to further guide NC treatment and improve the poor survival associated with the diagnosis.

Introduction: Ewing sarcoma is a rare, aggressive malignancy primarily affecting children and young adults, characterized by significant clinical variability and complex prognostic factors. Methods: In this study, we analyzed data from 4242 patients with Ewing sarcoma from the Surveillance, Epidemiology, and End Results (SEER) database to identify sociodemographic and clinical factors influencing overall and cancer-specific survival. We evaluated the impact of age, tumor location, disease stage, and treatment modalities, including surgery, chemotherapy, and radiotherapy, on survival outcomes. Results: The mean age of the patient sample was 21.54 ± 14.82 years. A total of 2530 (59.6%) patients were male. The most common tumor location was bone (3106, 73.2%), followed by soft tissue (843, 19.9%). In multivariate analysis, tumor location in the retroperitoneum and peritoneum (aHR: 1.655, 95% CI: 0.9604 to 2.640), age (aHR: 1.031, 95% CI: 1.026 to 1.037), distant disease (aHR: 3.337, 95% CI: 2.694 to 4.161), and regional disease (aHR: 1.363, 95% CI: 1.077 to 1.729) were independent predictors of poor cancer-specific survival. Subgroup analyses reveal distinct survival patterns across osseous, soft tissue, and retroperitoneal Ewing sarcoma cases, underscoring the importance of site-specific treatment approaches. Discussion: This study provides valuable insights into the prognostic landscape of Ewing sarcoma, informing both clinical practice and future research focused on enhancing survival outcomes for this challenging malignancy.

Background: Alveolar rhabdomyosarcoma (ARMS) is an aggressive subtype of rhabdomyosarcoma with generally poorer outcomes than embryonal rhabdomyosarcoma. We analyzed a large contemporary cohort to characterize survival outcomes and prognostic factors for ARMS. Methods: We identified patients with histologically confirmed ARMS from the Surveillance, Epidemiology, and End Results (SEER) database. Demographics, tumor characteristics (stage, primary site), and treatments (surgery, radiation, and chemotherapy) were extracted. Cancer-specific survival (CSS) was estimated with Kaplan–Meier methods and compared using log-rank tests. Cox proportional hazards models were used for univariate and multivariate analysis of prognostic factors, with results reported as hazard ratios (HR) and 95% confidence intervals (CI). Results: A total of 592 ARMS patients were included [median age 14 years (interquartile range 7–25); 52% male; 73% White]. Primary tumors most commonly arose in extremities or trunk (64%), followed by head and neck sites (26%) and genitourinary locations (5%). Most patients received multimodal therapy: 44% underwent surgical resection, 70% received radiotherapy, and 92% received chemotherapy. The 5-year CSS for the entire cohort was approximately 43%. Distant metastasis was the strongest independent adverse prognostic factor (aHR: 2.9, 95% CI 2.13–3.92, P < 0.001), and regional nodal involvement also independently worsened CSS (aHR: 1.46, 95% CI 1.07–1.99, P = 0.016) compared to localized disease. Older age (≥20) at diagnosis was associated with higher mortality risk (aHR: 1.82 vs. age <10, P < 0.001). Notably, the use of surgery (aHR: 0.74, P = 0.031) and radiation therapy (aHR: 0.62, P < 0.001) were independently associated with improved CSS after controlling for stage and age. Conclusions: In this large population-based study, ARMS survival remains poor, especially for patients with metastatic disease or in older age groups. Distant metastases and age ≥20 were associated with markedly inferior outcomes, while local therapy with surgery and radiation conferred significant survival benefit.

Glassy cell carcinoma (GCC) is a rare, highly aggressive subtype of poorly differentiated adenocarcinoma, primarily affecting the cervix but also reported in other anatomical sites such as the lung, pancreas, and uterus. Due to its rarity, little is known about the optimal management and prognostic factors influencing outcomes. This retrospective study utilized the SEER database to analyze 43 confirmed cases of GCC from 1992 to 2021, aiming to clarify demographic, clinical, and treatment-related factors associated with survival. The analysis revealed that GCC primarily affects females (90.7%) with a median age of 43 years, and most commonly arises in the cervix (74.4%). Survival analysis using Kaplan-Meier and Cox proportional hazards models indicated that disease stage at diagnosis and age were significant prognostic factors. Specifically, distant-stage disease (HR = 8.44, P < 0.001) and advanced age (HR = 1.043, P = 0.001) were associated with poorer survival. In contrast, treatment modalities such as chemotherapy and radiotherapy did not significantly impact survival outcomes, suggesting that traditional approaches may be inadequate for this carcinoma type. These findings underscore the need for improved early detection and tailored therapeutic strategies. GCC’s unique biological behavior and limited response to conventional treatments highlight the potential value of exploring targeted therapies to enhance patient outcomes in this rare malignancy.