Primary Salivary Gland-type Tumors Research Articles

AN UNEXPECTED CAUSE OF HEMOPTYSIS: RARE TRACHEAL TUMOR

SESSION TITLE: Fellows Lung Cancer Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Leading causes of hemoptysis in the western world are cryptogenic and infectious. We present a case of a young adult with isolated hemoptysis subsequently found to have a rare tracheal tumor. CASE PRESENTATION: A 24 yo male presented to the emergency room with hemoptysis of 3-4 tablespoons of bright red blood. He is a competitive swimmer with past medical history of childhood L5 osteochondroma and no tobacco use history. He had no other symptoms or exercise limitation. Initial chest x-ray and lab work were normal. Chest CT revealed mid-tracheal pedunculated mass obstructing >50% of the tracheal diameter. He was admitted to the hospital where bronchoscopy was performed, revealing a multilobulated and vascular-appearing mass. The patient proceeded to thoracic surgery with trans-cervical resection of the mass on 1 tracheal ring. Pathology revealed carcinoma ex pleomorphic adenoma with negative margins. The malignant component of the tumor was negative for MAML2 rearrangement by fluorescent in situ hybridization and was best characterized as high-grade adenocarcinoma, not otherwise specified. PET-CT and brain MRI were negative for metastatic disease. He received 60 Gy of adjuvant radiation therapy 8 weeks following resection. DISCUSSION: Primary salivary gland-type (P-SGT) tumors arise from the seromucinous submucosal glands of the central airways, and unlike their salivary gland counterparts, tend to be malignant.3 P-SGT of the lung are rare and account for 0.1-0.2% of all lung cancer. Mucoepidermoid carcinoma is the most common, followed by adenoid cystic carcinoma and distantly followed by epithelial-myoepithelial carcinoma. Carcinoma ex pleomorphic adenocarcinoma (Ca ex PA) is exceedingly rare, with 11 cases reported in the literature (4 involving the trachea). This tumor has an abrupt transition from a benign pleomorphic adenoma with bland ductal and myoepithelial components to a high grade malignant carcinoma with marked atypia, necrosis and mitotic activity. This is in contrast to the majority of P-SGT tumors, which are typically low grade and carry an overall prognosis of 56.3% survival at 20 years. Average age of onset for P-SGT tumors is the 5th and 6th decades, including pulmonary Ca ex PA (mean age 54y), which makes this discovery in a young patient highly unexpected. For P-SGT tumors, surgical intervention results in significant improvement in overall survival. The optimal treatment for pulmonary Ca ex PA remains unknown with the majority of reported cases receiving complete resection with varying adjuvant use of radiation and/or chemotherapy. CONCLUSIONS: Ca ex PA of the large airways is extremely rare and, in contrast to the majority of other primary salivary gland-type tumors, is high grade. Optimal treatment strategies remain unknown with resection plus radiation and/or chemotherapy being the most common approach to date. Reference #1: Ittrich H, Bockhorn M, Klose H, Simon M. The Diagnosis and Treatment of Hemoptysis. Dtsch Arztebl Int. 2017;114(21):371-381. Reference #2: Molina JR, Aubry MC, Lewis JE, et al. Primary salivary gland-type lung cancer: spectrum of clinical presentation, histopathologic and prognostic factors. Cancer. 2007;110(10):2253-2259. Reference #3: Falk N, Weissferdt A, Kalhor N, Moran CA. Primary Pulmonary Salivary Gland-type Tumors: A Review and Update. Adv Anat Pathol. 2016;23(1):13-23. DISCLOSURES: No relevant relationships by Mark Allen, source=Web Response No relevant relationships by Jennifer Boland, source=Web Response No relevant relationships by Matthew Cecchini, source=Web Response No relevant relationships by Kimberly Johnson, source=Web Response no disclosure on file for Tobias Peikert

Clinicopathological features of lung salivary gland-type tumors

Objective: To study the clinicopathological characteristics of lung salivary gland-type tumors (SGT), and to compare with the corresponding primary SGT in salivary glands. Methods: Twenty-three cases of lung SGT were retrieved from the files of Peking University First Hospital from January 2004 to September 2018. The morphology, immunophenotype, genotype and outcome of these cases were analyzed. Results: The 23 patients included 13 males and 10 females, with age range of 13-79 years (median 54 years). There were 11 cases of adenoid cystic carcinoma, 10 cases of mucoepidermoid carcinoma (MEC), one case each of clear cell carcinoma and myoepithelioma. The morphology and immunophenotype of lung SGT were very similar to their counterparts in salivary gland. MYB rearrangement was detected in one of 11 adenoid cystic carcinomas. MAML2 rearrangement was detected in all the MECs. EWSR1 rearrangement was detected in the one case of clear cell carcinoma. Of patients with adenoid cystic carcinoma, the survival time was more than 60 months (three cases), 52 months (one case), and 12-36 months (three cases). There was no recurrence and death in seven cases of MEC with follow-up results. One case of clear cell carcinoma recurred after 52 months of follow-up. Conclusions: Although the SGT of lung and their counterparts in salivary gland are very similar in their morphology, immunophenotype, genotype and prognosis, there are also some differences between each other. MYB rearrangement can be detected in most adenoid cystic carcinomas of salivary gland, but rarely in lung adenoid cystic carcinoma. The prognosis of patients with lung MEC is better than that of patients with salivary gland MEC, while the prognosis of patients with lung adenoid cystic carcinoma is worse than that of patients with salivary gland adenoid cystic carcinoma.

Primary salivary gland-type tumors of the lung: A systematic review and pooled analysis.

Introduction:Primary salivary gland-type tumors of the lung (PSGTTL) are rare intrathoracic malignant neoplasms. Their description in literature is largely limited to a few case series and case reports. A systematic review and pooled analysis of the previously reported cases of PSGTTL is presented here.Methods:Electronic database of PubMed using keywords “lung neoplasm” AND “salivary gland tumors” was used to identify the papers documenting the PSGTTL. Filters (publication date from January 1, 1900–-December 31, 2015, Humans and English) were applied to refine the search. A pooled analysis of clinical, pathological, treatment, and survival data was performed.Results:The present systematic review included 5 studies and a total of 233 patients. Mean age of the patients was 41 years (range 6–80 years) and there was a male preponderance (1.3:1). Common pathological types were mucoepidermoid (MEC) (56.6%), adenoid cystic (ACC) (39.5%), and epithelial-myoepithelial cancer (3.8%). Tumors were located in the central airways (trachea and major bronchi) in 43.3% of patients. Weighted median tumor size was 4.2 cm. Surgery was the primary treatment undertaken in 82.4% of the patients, while radiotherapy and chemotherapy were also used in 15.9% and 9.4% of the patients. Lymph node involvement was seen in 15.2% of the patients. Disease recurrences were observed in 21.1% of the patients (12.9% and 37.5% in MEC and ACC, respectively). Three-, 5-, and 10-year weighted overall survival was 86.4%, 81.4%, and 73.6% (93.8%, 90.0%, and 85.0%, respectively, for MEC and 76.7%, 62.8%, and 50.5%, respectively, for ACC).Conclusion:Surgery is the primary treatment of PSGTTL to achieve long-term survival. Role of chemotherapy and radiotherapy in the management of PSGTTL warrants further studies.

Primary Salivary Gland-Type Tumors of the Lung: A Comparative Analysis of Mucoepidermoid and Adenoid Cystic Carcinomas

Primary Salivary Gland-Type Tumors of the Lung: A Comparative Analysis of Mucoepidermoid and Adenoid Cystic Carcinomas

Cytology of Primary Salivary Gland-Type Tumors of the Lower Respiratory Tract: Report of 15 Cases and Review of the Literature.

Primary pulmonary salivary gland-type tumors are rare neoplasms arising from the seromucinous submucosal glands of the lower respiratory tract (LRT), the most common of which are mucoepidermoid carcinoma (MEC) and adenoid cystic carcinoma. They are morphologically indistinguishable from their salivary gland counterpart and recognizing them is a challenge, especially on cytological specimens. We analyzed 15 cases of histologically proven primary salivary gland tumors of the LRT to identify cytomorphological features and define potential diagnostic clues that might assist cytopathologists in the preoperative diagnosis of these neoplasias. Three out of the four cases of adenoid cystic carcinomas showed the characteristic tridimensional cell clusters and hyaline globules, whereas the last one did not show malignant cells; only two cases of MEC presented the three characteristic cell types (i.e., squamous, intermediate, and mucin secreting) on cytology. Since these neoplasms are rare and do not have a completely specific set of cytological features, it is important for practicing cytopathologists to be aware of the possibility of encountering them, in specimens from patients with LRT masses, in order to render the correct diagnosis.

Primary Salivary Gland Type Tumors of the Thymus.

The existence of primary salivary gland type tumors (SGTs), similar to those occurring in the major salivary glands, is well known in the thoracic cavity. When they occur in this anatomic area, these tumors more commonly arise from the lung. However, the existence of these tumors primarily affecting the thymus, although recognized in the literature, is rather not well documented or known. In addition, contrary to the primary lung SGTs, which are predominantly of the malignant type, these tumors when occur in thymus encompass a wider spectrum of biology ranging from benign to low grade, and high grade malignancy. The recognition of SGTs in the thymus, even though rare, is important to properly address treatment and prognosis. Herein, we will discuss the numerous benign a malignant SGTs that have been described in the thymus and highlight the difficulty that these tumors may pose when occurring in the thymic area.

P1.08-029 Surgical Experience of Primary Salivary Gland Tumors of Lung: Experience from Tertiary Care Cancer Center in North India

Primary salivary gland type tumors of lung (PSGTTL) are rare intra-thoracic malignant neoplasm. Their description in literature is largely limited to a few case series/case reports. We herewith present our surgical experience and review its clinical presentation, management options and survival outcomes. We performed a retrospective analysis of prospectively maintained computerized data-base of lung cancer patients at department of surgical oncology, Dr BRA-IRCH, AIIMS, Delhi. A total of nine patients underwent treatment for PSGTTL during the period from January 2012 to December 2015. Details concerning the clinical presentation, preoperative therapy, operative procedure, histopathological examination, postoperative complications and outcome were retrieved and analyzed. Median age of patients was 42 years (range 24-52 years) with male: female ratio of 7:2. Median duration of symptoms before presentation was 12 months (range 4-24 months). Most common symptoms were Hemoptysis (77%) and dyspnea (66%). Fiber-optic bronchoscopy revealed endobronchial growth in all patients with six patients had growth in left main bronchus while one had growth in right main bronchus and two in right intermediate bronchus. Biopsy confirmed adenoid cystic carcinoma in 6 (66%) and muco-epidermoid carcinoma in 3 (33%) patients. Total seven patients underwent R‘0’ resection with pneumonectomy in five, bilobectomy in one, lower lobectomy in one patient. One patient developed pneumonia after left carinal pneumonectomy and succumbed to it. No major postoperative complication was encountered in remaining six patients. One patient refused surgery and one found unresectable in view of dense adhesions between lung and heart. Both patient received chemo-radiation and underwent bronchoscopic debulking and are in follow up. Median pathological tumor size was 3 cm. Median number of node harvested was 10 (range 4-18) however none showed metastasis. None of the operated patient developed relapse and overall eight patients are alive after a median follow up of 18 months. Primary salivary gland type tumors of lung (PSGTTL) are low grade malignancy and greater awareness of these tumors is necessary to avoid misdiagnosis and delay in treatment. Aggressive anatomical lung resection with preservation of functional lung parenchyma offers optimal outcome in such patients.

P2.04-033 Primary Salivary Gland Tumors of the Lung: A Systematic Review and Pooled Analysis: Topic: Esophageal Cancer and Other Malignancies

Salivary gland type neoplasms are known to occur at multiple organ sites in view of basic structural homology among exocrine glands in these anatomic sites. Primary salivary gland type tumors of lung (PSGTTL) are rare intra-thoracic malignant neoplasm. They are believed to arise from the sub-mucosal glands of the trachea-bronchial tree. Their description in literature is largely limited to a few case series/case reports. A greater awareness of PSGTTL is essential for accurate diagnosis and proper clinical management. A systematic review and pooled analysis of previously reported cases of PSGTTL is presented here. We searched the electronic database of Pubmed using key words (“lung neoplasm” [Mesh] AND “salivary gland tumors” [Mesh] to identify the papers documenting the PSGTTL. Filters (publication date from 1900/01/01 to 2015/12/31, Humans, and English) were applied to refine the search. All the articles which were single case reports or had exclusively presented one pathological type of PSGTTL were not included in the review. A pooled analysis of clinical, pathological, treatment and survival data was performed. The present systematic review included 5 studies and a total of 233 patients. Mean age of the patients was 41 years (range 6-80 years) and there was a male preponderance (1.3:1). Common pathological types were mucoepidermoid (MEC) (56.6%), adenoid cystic (ACC) (39.5%), and epithelial-myoepithelial cancer (3.8%). Tumors were located in central airways (trachea and major bronchi) in 43.3% patients. Mean tumor size was 4.2 cm. Surgery was the primary treatment undertaken in 82.4% patients while radiotherapy and chemotherapy was also used in 15.9% and 9.4% patients. Lymph node involvement was seen in 15.2% patients. Disease recurrences were observed in 21.1% patients (12.9% and 37.5% in MEC and ACC respectively). 3-year, 5-year, and 10-year weighted overall survival was 86.4%, 81.4%, and 73.6% (93.8%, 90.0% and 85.0% respectively for MEC; 76.7%, 62.8% and 50.5% respectively for ACC). Surgery is the primary treatment of PSGTTL resulting in good long-term survival. ACC histological type has a poor prognosis with more disease recurrences compared to MEC. Role of chemotherapy and radiotherapy in the management of PSGTTL warrants further studies.

Primary epithelial-myoepithelial carcinoma of the lung: A case report demonstrating high-grade transformation-like changes.

Primary salivary gland-type tumors of the lung are rare; among them, epithelial-myoepithelial carcinomas (EMC) represent a minor histological subtype. The present case documents an EMC that occluded the B8 segment of the left lung in a 72-year-old woman. Macroscopically, the tumor was well-demarcated; however, microscopic examination demonstrated that it had infiltrated the lung parenchyma. The majority of the tumor mass was composed of a myoepithelial overgrowth in conjunction with conventional bilayered ductal structures comprising epithelial and myoepithelial cells. At the advancing edge of the tumor, the myoepithelial overgrowth was observed to be gradually transitioning to a higher-grade component, which demonstrated venous invasion. The Ki-67 labeling index was reduced compared with high-grade transformation (HGT) of salivary gland EMC; p53 was sparsely observed on immunostaining. However, cyclin D1, which is reported to be overexpressed in certain subtypes of salivary gland carcinomas with HGT, was overexpressed in the higher-grade component of the tumor, indicating a potential HGT initiation. The surgical margin was tumor free, and no recurrence has been observed for 4 months. A thorough follow-up is required considering the HGT-like changes and venous invasion of the tumor. Additional studies are required to elucidate the characteristics of pulmonary EMC, with an emphasis on detecting HGT or HGT-like changes.

Left carinal pneumonectomy through median sternotomy: Surgical experience of two patients.

Endobronchial tumors infiltrating the carina is a formidable challenge to surgeons in view of difficult surgical access to the carina, especially on the left side, problems of securing the airway intra-operatively, technically challenging anastomosis due to anatomical location, and high post-operative morbidity and mortality. We present our surgical experience of two cases of left carinal pneumonectomy which was undertaken for resectable primary salivary gland type tumors of lung.

Primary pulmonary myoepithelial carcinoma

Primary salivary gland-type tumors of the lung are rare neoplasms. The pulmonary myoepithelial carcinoma belongs to this group. Since it was first described in 1998, we have identified only seven actual cases reported in the literature so far. We describe the case of a non-smoker 60-year-old Caucasian female, who was referred to our institution for the treatment of three peripheral nodules. Her medical history revealed that a wedge resection, for pulmonary myoepithelial carcinoma, was performed in another center 15 months previously. After resection of the current nodules, the histological findings showed three myoepithelial carcinomas of the lung. There was no vascular or lymphatic invasion. The hilar and mediastinal lymph nodes were negatives. The patient is doing well without any sign of recurrence. Herein we shall discuss the case of a primary pulmonary myoepithelial carcinoma in a female and propose a brief review of the literature.