Adenoid cystic carcinoma of Bartholin's gland, a case report with genomic data and literature review.

Adenoid cystic carcinoma of the Bartholin's gland (ACCBG) is a rare, slowly but aggressive malignancy. We reported the case of a 31-year-old woman who was treated by local excision and then hemi-vulvectomy, with positive margins and perineural invasion. Radiation therapy (RT) was then performed delivering 45Gy in 25 fractions in bilateral inguinal lymph nodes and 64.8Gy in 36 fractions on the vulvar area. After 30 months, there was no local relapse (LR) but the patient presented a histologically documented lung recurrence. Genomic profiling of the tumor showed a MYB-NFIB fusion transcript and a somatic mutation of PLCG1. A treatment by Lenvatinib was started. We conducted a literature review of 100 published cases. Patients were mainly treated by radical vulvectomy (30%), hemi-vulvectomy (17%), wide or local excision (21% and 24%, respectively) or other. Forty-four percent of patients received postoperative RT, more frequently in case of positive margin (71.9% versus 29.5%). RT may reduce the risk of LR regardless of margin status, with 15.4% vs. 41.9% of LR with or without RT, respectively, in patients with negative margins, and 13% vs. 33.3% of LR with or without RT, respectively, in patients with positive margins. The risk of relapse of any type was 40.9% in patients who received adjuvant RT vs. 48.2% in patients who did not. Median time to relapse was 24 months (range 6-156 months). The most frequent metastatic sites were lung (76.7%) and bone (26.7%). Optimal treatment for ACCBG is still not clearly defined but pooling the data from published case report help us better understand this rare disease and help in the therapeutic decision.

Hypofractionated postoperative stereotactic radiotherapy for large resected brain metastases.

PURPOSE: The aim of the present retrospective study was to report outcomes after hypofractionated stereotactic radiotherapy (HSRT) for resected brain metastases (BM). PATIENTS AND METHODS: We reviewed results of patients with resected BM treated with postoperative HSRT (3×7.7Gy to the prescription isodose 70%) between May 2013 and June 2020. Local control (LC), distant brain control (DBC), overall survival (OS), leptomeningeal disease relapse (LMDR), and radiation necrosis (RN) occurrence were reported. RESULTS: Twenty-two patients with 23 brain cavities were included. Karnofsky Performance status (KPS) was≥70 in 77.3%. Median preoperative diameter was 37mm [21.0-75.0] and median planning target volume (PTV) was 23 cm3 [9.9-61.6]. Median time from surgery to SRT was 69 days [7-101] and 48% of patients had a local relapse on pre-SRT imaging. Median follow-up was 17.5 months [1.6-95.9]. One and two-year LC rates were 60.9 and 52.2% respectively. One and 2-year DBC rates were 45.5 and 40.9%. Median OS was 16.5 months. Four patients (18.2%) presented LMDR during follow-up. RN occurred in 6 patients (27.2%). Three factors were associated with OS: ECOG-PS (P=0.009), KPS (P=0.04), cystic or solid nature of the metastasis before surgery (P=0.037). Several factors were related to RN occurrence: PTV diameter and volume, Normal brain V21, V21 and V24 isodoses volumes. CONCLUSION: HSRT is the most widely used scheme for larger brain cavities after surgery. The optimal dose and scheme remain to be defined as well as the optimal delay between postoperative SRT and surgery. Dose escalation may be necessary, especially in case of subtotal resection.

Efficacy and Tolerance of Carboplatin plus Cetuximab (Simplified EXTREME Regimen) in Patients with Recurrent and/or Metastatic Head and Neck Squamous Cell Carcinoma.

AIMS: The EXTREME regimen is the standard for recurrent and/or metastatic head and neck squamous cell carcinoma (HNSCC). However, many patients have a poor performance status and/or comorbidities, making them unfit for this regimen. We have treated them with carboplatin and cetuximab (simplified EXTREME regimen) since 2007. Our aim was to assess the efficacy and tolerance of this regimen in this frail population. MATERIALS AND METHODS: A retrospective chart review of all patients treated with the simplified EXTREME regimen for recurrent and/or metastatic HNSCC in three academic hospitals between 2007 and 2017 was carried out. The primary end point was overall survival. Secondary end points were progression-free survival (PFS), overall response rate (ORR) and toxicity. RESULTS: One hundred and three patients were included. The median age was 63 years, 40% had performance status 2-3. The median follow-up was 30.2 months. The median overall survival and PFS were 7.2 and 3.7 months, respectively. The median overall survival was 10.1 months in patients with performance status 0-1 versus 4.6 months in patients with performance status 2-3 (P = 0.01). ORR was 39%. Acute grade 3-4 haematological and non-haematological toxicity rates were 25.2% and 27.2%, respectively. Patients with grade 1 or more skin toxicity had a higher ORR (hazard ratio = 3.44; P = 0.03), a prolonged overall survival (hazard ratio = 0.37; P < 0.0001) and PFS (hazard ratio = 0.29; P < 0.0001). During treatment, 29% of patients had pain reduction, 13.5% had weight gain and 17.2% had an improvement in performance status. CONCLUSIONS: This is the largest cohort of patients treated with simplified EXTREME for HNSCC. It was well tolerated, with a high ORR. Interestingly, skin toxicity correlated with treatment efficacy.

Neoadjuvant treatment of pancreatic adenocarcinoma: Chemoradiation or stereotactic body radiation therapy?

Despite recent advances, the prognosis of pancreatic adenocarcinomas remains poor, even for patients with resectable tumors. For these latter, new approaches based on neoadjuvant treatment have been developed. Two components are used: chemotherapy and radiation therapy (RT). Indeed, pre-operative RT has many advantages in terms of efficacy and tolerance. It increases notably the chances of subsequent complete tumor resection. Several prospective trials are currently ongoing to clarify its place in the therapeutic arsenal. Another crucial question is to know which is the best RT technique: conventional normofractionated chemoradiotherapy or hypofrationated stereotactic body RT?