E Boon1, M H Valstar2, W T A van der Graaf3, E Bloemena4, S M Willems5, C A Meeuwis6, P J Slootweg1, L A Smit2, M A W Merkx1, R P Takes1, J H A M Kaanders1, P J T A Groenen1, U E Flucke1, C M L van Herpen7. 1. Radboud University Medical Centre, Nijmegen, Netherlands. 2. Antoni van Leeuwenhoek/Netherlands Cancer Institute, Amsterdam, Netherlands. 3. Radboud University Medical Centre, Nijmegen, Netherlands; The Institute of Cancer Research and The Royal Marsden NHS Foundation Trust, London UK. 4. Radboud University Medical Centre, Nijmegen, Netherlands; VU University Medical centre, Amsterdam, Netherlands. 5. University Medical Centre Utrecht, Utrecht, Netherlands. 6. Erasmus University Medical Centre, Rotterdam, Netherlands. 7. Radboud University Medical Centre, Nijmegen, Netherlands. Electronic address: Carla.vanherpen@radboudumc.nl.
Abstract
OBJECTIVES: In 2010, a new subtype of salivary gland cancer (SGC), (mammary analogue) secretory carcinoma (SC), was defined, characterized by the ETV6-NTRK3 fusion gene. As clinical behavior and outcome data of this histological subtype tumor are still sparse, we aimed to describe the clinicopathological course and outcome of a series of translocation positive SC patients. PATIENT AND METHODS: We re-evaluated the pathological diagnosis of a subset of SGCs, diagnosed in 4 of 8 Dutch head and neck centers. Subsequently, tumors with a morphological resemblance to SC were tested for the ETV6-NTRK3 fusion gene using RT-PCR. Furthermore, patients prospectively diagnosed with SC were included. The clinical characteristics and outcomes were retrieved from the patient files. RESULTS: Thirty-one patients with ETV6-NTRK3 fusion gene positive SC were included. The median age was 49 years, 17 patients (55%) were male. Eighteen tumors (58%) arose in the parotid gland. One patient presented with lymph node metastasis. All patients underwent tumor resection and 4 patients had a neck dissection. Four patients had re-resection and 15 patients (48%) received postoperative radiotherapy. One patient developed a local recurrence, no regional recurrences or distant metastases were observed. After a median follow-up of 49 months the 5- and 10-year overall survival were 95%, the 5- and 10-year disease free survival were 89%. CONCLUSION: The clinical course of SC is favorable with a low rate of locoregional recurrence and excellent survival. Given the low incidence of nodal metastases, elective neck treatment, i.e. surgery and/or radiotherapy, does not seem to be indicated.
OBJECTIVES: In 2010, a new subtype of salivary gland cancer (SGC), (mammary analogue) secretory carcinoma (SC), was defined, characterized by the ETV6-NTRK3 fusion gene. As clinical behavior and outcome data of this histological subtype tumor are still sparse, we aimed to describe the clinicopathological course and outcome of a series of translocation positive SC patients. PATIENT AND METHODS: We re-evaluated the pathological diagnosis of a subset of SGCs, diagnosed in 4 of 8 Dutch head and neck centers. Subsequently, tumors with a morphological resemblance to SC were tested for the ETV6-NTRK3 fusion gene using RT-PCR. Furthermore, patients prospectively diagnosed with SC were included. The clinical characteristics and outcomes were retrieved from the patient files. RESULTS: Thirty-one patients with ETV6-NTRK3 fusion gene positive SC were included. The median age was 49 years, 17 patients (55%) were male. Eighteen tumors (58%) arose in the parotid gland. One patient presented with lymph node metastasis. All patients underwent tumor resection and 4 patients had a neck dissection. Four patients had re-resection and 15 patients (48%) received postoperative radiotherapy. One patient developed a local recurrence, no regional recurrences or distant metastases were observed. After a median follow-up of 49 months the 5- and 10-year overall survival were 95%, the 5- and 10-year disease free survival were 89%. CONCLUSION: The clinical course of SC is favorable with a low rate of locoregional recurrence and excellent survival. Given the low incidence of nodal metastases, elective neck treatment, i.e. surgery and/or radiotherapy, does not seem to be indicated.
Authors: David Forner; Martin Bullock; Daniel Manders; Timothy Wallace; Christopher J Chin; Liane B Johnson; Matthew H Rigby; Jonathan R Trites; Mark S Taylor; Robert D Hart Journal: J Otolaryngol Head Neck Surg Date: 2018-11-16