scholarly journals Primary squamous cell carcinoma of major salivary gland: “Sapienza Head and Neck Unit” clinical recommendations

Rare Tumors ◽  
2020 ◽  
Vol 12 ◽  
pp. 203636132097352
Author(s):  
Silvia Mezi ◽  
Giulia Pomati ◽  
Andrea Botticelli ◽  
Francesca De Felice ◽  
Daniela Musio ◽  
...  

Primary squamous cell carcinoma of salivary gland (SCG) is an extremely rare type of malignant salivary gland tumor, which in turn results in scarcity of data available regarding both its treatment and associated genetic alterations. A retrospective analysis of 12 patients with primary SCG was conducted, along with analysis of the association between treatment, clinical/pathological characteristics, and outcomes. Most patients (8) were staged IVa, with the majority of them (10) having G3 fast growing cancer. Local and systemic recurrence were reported in only three out of nine parotid cases (0 out of 2 submandibular SCGs). In two out of eight patients local relapse occurred after integrated treatment, while recurrence occurred in two out of three patients undergoing exclusive surgery. Five patients eventually died. Treatment of resectable disease must be aggressive and multimodal, with achievement of loco-regional control in order to reduce rate of recurrence and improve outcomes. Metastatic disease would require a therapeutic strategy tailored to the molecular profile in order to improve the currently disappointing results.

Pathology ◽  
2016 ◽  
Vol 48 (5) ◽  
pp. 523-525 ◽  
Author(s):  
Tun-Pang Chu ◽  
Wei-Che Chen ◽  
Tao-Yeuan Wang ◽  
Shih-Ping Cheng

2020 ◽  
Vol 38 (15_suppl) ◽  
pp. e18506-e18506
Author(s):  
Andrea Botticelli ◽  
Silvia Mezi ◽  
Giulia Pomati ◽  
Ilaria Zizzari ◽  
Bruna Cerbelli ◽  
...  

e18506 Background: In head and neck squamous cell carcinoma (HNSCC) only a small subset of patients (pts) really benefits from immunotherapy, suggesting the need of validated molecular and immunological predictive biomarkers. The aim of the study was to evaluate both the genomic and immune profile of HNSCC correlating it to early progression to immunotherapy. Methods: This is a pilot study evaluating immune and molecular profile in platinum-refractory HNSCC pts treated with Nivolumab. Blood samples were collected at baseline (T0) and at second cycle of therapy (T1). The immune profile was studied by multiplex assay, evaluating circulating: CD137, CTLA4, PD1, PDL1, PDL2, CD27, TIM3, LAG3, GITR, HVEM, BTLA, CD80, CD28 and IDO. The comprehensive genomic profile was evaluated at baseline on formalin-fixed paraffin-embedded samples of the tumor through Foundation One Cdx test. The results obtained were correlated with early progression (within 3 months from the start of therapy). Results: Ten pts were enrolled in the study. Early progression occurred in 6 pts (60%). Median progression free survival and overall survival were 3 months (1-11) and 6.5 months (1-12), respectively. In all patients ≥ 5 genetic mutations were detected; median number of mutation was 6 (5-14); 8/10 pts had a tumor mutational burden less than 10 Muts/mb. The more frequent genetic alterations involved the cycline-dependent-kynase pathway (9/10), the transcription factor associated gene TP53 (8/10) and the PI3K-Akt-PTEN signaling pathways (5/10). Less frequent alterations involved FGFR family (4/10), NOTCH signaling pathway (3/10) and TERT (4/10). Early progression was slightly associated with the number of mutations detected (p = 0.06). The rising or falling trend of circulating levels of biomarkers were detected in 9/10 pts and included modification in IDO (1/10), LAG3 (3/10), GITR (3/10), BTLA (3/10), CD137 (4/10), CTLA4 (4/10), PD1 (2/10), Tim3 (3/10), CD28 (3/10), HVEM (3/10), CD80 (2/10). No modifications were recorded in PDL1, PDL2 and CD27. Conclusions: Our results, although in a small cohort, highlighted the complexity and heterogeneity of landscape in HNSCC pts. A novel evaluation that combines molecular and immune profile is needed. In the context of a poor prognosis disease the combination of personalized molecular and immune approaches could represent a promising strategy to improve survival.


2020 ◽  
Vol 3 (1) ◽  
Author(s):  
Mingfei Yan ◽  
Phillip Bomeisl ◽  
Hannah Gilmore ◽  
Aparna Harbhajanka

Abstract Background Adenoid cystic carcinoma and primary squamous cell carcinoma are both rare breast neoplasms, which possess drastically different morphological and molecular features as well as distinguishing clinical behaviors and prognosis. Case presentation In this report, we described a rare case in which concurrent adenoid cystic carcinoma and keratinizing squamous cell carcinoma were diagnosed in contralateral breasts in an 85 year-old female patient. The patient had a history of adenoid cystic carcinoma diagnosed 11 years ago, which was treated by partial mastectomy followed by whole breast radiation. The recurrent carcinoma on the same side of the breast was small in size but appeared to involve an intraductal papilloma. Also, a newly occurred large cystic mass was identified on the contralateral breast, which histologically presented as a keratinizing squamous cell carcinoma with no glandular differentiation. No in situ or invasive carcinoma was identified in the overlying skin of the lesion, and no malignancy in a second site was found by PET-CT. Therefore, this lesion was mostly likely a primary squamous cell carcinoma of the breast. Conclusion The concurrence of two such rare neoplasms was likely an incidental finding or was therapy-related. However, more mechanistic studies are needed in order to understand whether predisposing genetic alterations exist in this rare case. Besides, cases of both breast adenoid cystic carcinoma and carcinoma with predominant squamous differentiation diagnosed in our institution were reviewed, which help to better characterize their clinicopathological features.


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