Treatment for SMARCB1 (INI-1) deficient sinonasal tumor: a single-institution study.

Currently, less than 200 cases of SMARCB1-deficient sinus cancer (SDSC) have been documented. Little information is available about the best treatment options or prognosis for SDSC. From September 2016 to November 2022, the medical records of 22 people with SDSC were evaluated retrospectively. Patient demographics, staging, pathology findings, treatment details, recurrence, metastasis, and survival outcomes were all investigated by the researchers. The 1-, 2-, and 3-year overall survival (OS) rates for the entire cohort were 89.8%, 84.2%, and 45.1%, respectively, as were the 1-, 2-, and 3-year progression-free survival (PFS) rates of 81.8%, 63.8%, and 31.9%. After induction chemotherapy, 66.7% (10/15) of patients exhibited decreased tumor volume. Patients who accepted chemoradiotherapy had a better 2-year OS (100% vs. 72.7%, p=0.048) than those who accepted surgery as a preference. However, there is no difference in 2-year PFS between the two groups (53.0% vs. 75.8%, p=0.59). Patients with progressed or stable disease after induction chemotherapy had a higher risk of developing local recurrence (p=0.007); they also showed poor 2-year PFS (40.0% vs. 82.1%, p=0.019). SDSC had a poor 3-year OS, with a PFS of less than 50%. For locally advanced SDSC, chemoradiotherapy might be managed before surgery, especially in patients who benefit from induction chemotherapy.

Personalized treatment for patients with lacrimal sac squamous cell carcinoma.

OBJECTIVE: Lacrimal sac squamous cell carcinoma (LSSCC) is a rare and poor prognosis malignancy. We aimed to investigate the predictive factors for prognosis and to discuss the optimal treatment mode. METHODS: This retrospective study comprised 84 patients with LSSCC who accepted multidisciplinary treatment. We analyzed the potential prognostic factors and the efficiency of different treatment modes in univariate and multivariate analyses. RESULTS: The 5-year overall survival (OS), progression-free survival (PFS), regional failure-free survival (RFS), and distant metastasis-free survival (DMFS) rates for the entire cohort were 83.7%, 76.3%, 77.2%, and 83.7%, respectively. On univariate analysis, orbital bone erosion, lymph node metastasis, and advanced clinical stage were poor prognostic factors. Multivariate Cox regression analysis showed that orbital bone erosion was a uniquely poor predictor for OS; orbital bone erosion, positive cervical lymph nodes, and old age were poor predictors for PFS. Chemotherapy significantly improved the 5-year OS (90.4% vs. 69.6%, p = 0.03), PFS (82.1% vs. 63.6%, p = 0.036), and DMFS (90.4% vs. 69.6%, p = 0.013), except for RFS (82.5% vs. 65.6%, p = 0.15). Surgery did not improve the 5-year OS (85.6% vs. 79.3%, p = 0.062), PFS (76.0% vs. 76.2%, p = 0.41), RFS (76.1% vs. 79.5%, p = 0.54), and DMFS (85.6% vs. 79.5%, p = 0.096). However, the pre-operative radiotherapy conferred a slightly better OS (p = 0.13) and DMFS (p = 0.16) than post-operative radiotherapy and definitive radiotherapy, but without statistical significance. CONCLUSIONS: Orbital bone erosion and lymph node metastasis were poor prognostic factors in LSSCC. Chemoradiotherapy was vital and effective. Although surgery did not improve survival, multidisciplinary treatment, including surgery, was recommended for LSSCC.

Incidence and patterns of lymph node metastases in head and neck rhabdomyosarcoma: One-institution study.

INTRODUCTION: Head and neck rhabdomyosarcoma (HNRMS) is an aggressive malignant soft tissue tumor that easily develops lymph node metastasis (LNM) and distant metastasis. No literature investigates the pattern of LNM in HNRMS. METHODS: Ninety-five consecutive patients with HNRMS newly diagnosed at one institution between November 2011 and July 2023 were retrospectively reviewed. All the patients underwent head and neck contrast-enhanced MRI and/or CT, PET-CT if necessary. The associations between LNMs and clinical characteristics and histopathological parameters were discovered. RESULTS: 44.2% of patients had evidence of LNM at diagnosis, and the most common LNM occurred in the ipsilateral retropharyngeal space. The primary tumor metastasizes to the retropharyngeal space, and then next to level II is the most common LN drainage basin. In multivariate analysis, only distant metastasis determines the prognosis, other than LN status. CONCLUSIONS: LNM has a high incidence in HNRMS and rarely causes contralateral metastasis for localized lesions or skip metastasis.

Surgery for adult head and neck rhabdomyosarcoma: A retrospective report from one institution.

KEY POINTS: Surgery in adults with head and neck rhabdomyosarcoma does not improve survival rates. Surgery should be performed deliberately and focused on the timing of combined treatment modality. Adult head and neck rhabdomyosarcoma benefits from salvage surgery following chemoradiotherapy.

Could a low-dose definitive radiation therapy be the optimal treatment for choroidal hemangioma?

PURPOSE: To determine the efficacy of low-dose intensity-modulated radiation therapy (IMRT)/volumetric intensity-modulated arc therapy (VMAT) in the treatment of symptomatic choroidal hemangioma (CH). MATERIALS AND METHODS: Fifty-three consecutive patients with CH were retrospectively reviewed. All the patients underwent IMRT/VMAT as a unique treatment. Resolution of subretinal fluid (SRF), improvement of best-corrected visual acuity (BCVA), and reduction in tumor thickness were compared before and after radiotherapy. RESULTS: After definitive radiotherapy, 100 % of SRF and 76.7 % of exudative retinal detachment were resolved. 56.6 % of BCVA improvement in more than two lines was observed. The mean best-corrected visual acuity was 20/280 (range, 20/1200-20/40) at diagnosis and 20/100 (range, 20/1200-20/20) after treatment. The mean tumor thickness decreased significantly from 3.8 mm initially to 1.2 mm after treatment (p < 0.01). 66.0 % of patients were delivered with 21.6 Gy (range, 21.6-42 Gy), 84.9 % of fractional dose was 1.8 Gy (range, 1.8-2 Gy). No radiation-induced keratitis, retinopathy, or optic neuropathy were observed. Initial vision (p = 0.042), duration time of vision (p = 0.004), and tumor thickness (p = 0.049) were prognostic factors for vision recovery. CONCLUSION: Low-dose IMRT/VMAT could effectively induce involution of the CH, with reduction of subretinal fluid and relief of damage to the neurosensory retina, which is an effective treatment mode for CH.

The Role of Elective Nodal Irradiation in Treating Clinically Node-Negative Sinonasal Squamous Cell Carcinoma.

PURPOSE: This study aims to examine the role of elective nodal irradiation (ENI) in clinically node-negative (cN0) sinonasal squamous cell carcinoma (SNSCC) and to define the optimal radiation fields for ENI. METHODS AND MATERIALS: We retrospectively reviewed 368 patients with cN0 SNSCC treated between 2009 and 2021. The study evaluated the impact of ENI on overall survival, progression-free survival, regional failure-free survival, and distant metastasis-free survival, along with the coverage areas of ENI. RESULTS: The majority of patients underwent surgery (316/368, 85.9%), with 276 of 368 (75%) having tumors in the maxillary sinus or nasal cavity and 249 of 368 (67.7%) presenting with T4 disease. Additionally, in 119 of the 368 cases (32.3%), tumors were poorly differentiated. The 5-year overall survival, progression-free survival, regional failure-free survival, and distant metastasis-free survival rates were 59.3%, 54.0%, 57.6%, and 58.8%, respectively. ENI was performed in 217 patients (59%), with 16 experiencing neck relapse during follow-up. Although ENI did not enhance survival rates, it significantly reduced the overall regional failure rate (7.9% vs 1.8%; χ2 = 7.98; P < .01) and the cumulative incidence of regional failure (P = .045). Additionally, the subgroups with maxillary sinus origin (2.3% vs 13.5%; P = .025), T4 stage (1.8% vs 8.5%; P = .028), and poor differentiation (2.4% vs 13.5%; P = .029) had higher cumulative incidences of regional failure in patients without ENI. No significant difference was observed in survival and regional failure rates between patients treated with ENI to levels Ib and II with or without level III, as well as between cN0 patients with nonmidline crossing lesions receiving unilateral or bilateral ENI. CONCLUSIONS: Despite no survival benefit, ENI significantly decreases the regional failure rate in patients with cN0 SNSCC. For primary lesions not crossing the midline, ipsilateral ENI targeting levels Ib and II proves to be an effective strategy.

A disulfidptosis-related glucose metabolism and immune response prognostic model revealing the immune microenvironment in lung adenocarcinoma.

Background: Lung adenocarcinoma accounts for the majority of lung cancer cases and impact survival rate of patients severely. Immunotherapy is an effective treatment for lung adenocarcinoma but is restricted by many factors including immune checkpoint expression and the inhibitory immune microenvironment. This study aimed to explore the immune microenvironment in lung adenocarcinoma via disulfidptosis. Methods: Public datasets of lung adenocarcinoma from the TCGA and GEO was adopted as the training and validation cohort. Based on the differences in the expression of disulfidptosis -related genes, a glucose metabolism and immune response prognostic model was constructed. The prognostic value and clinical relationship of the model were further explored. Immune-related analyses were performed according to CIBERSORT, ssGSEA, TIDE, IPS. Results: We verified that the model could accurately predict the survival expectancy of lung adenocarcinoma patients. Patients with lung adenocarcinoma and a low-risk score had better survival outcomes according to the model. Moreover, the high-risk group tended to have an immunosuppressive effect, as reflected by the immune cell components, phenotypes and functions. We also found that the clinically relevant immune checkpoint CTLA-4 was significantly higher in low-risk group (P<0.05), indicating that the high-risk group may suffer worse tumor immunotherapy efficacy. Finally, we found that this model has accurate predictive value for the efficacy of immune checkpoint blockade in non-small cell lung cancer (P<0.05). Conclusion: The prognostic model demonstrated the feasibility of predicting survival and immunotherapy efficacy via disulfidptosis-related genes and will facilitate the development of personalized anticancer therapy.