Wide Surgical Resection Research Articles

Malignant maxillofacial bone tumors.

Malignant bone tumors of the head and neck region are rare. Due to the paucity of studies on these tumors, patients with these maxillofacial malignancies can suffer from high morbidity and mortality. Currently, mainstay management of these tumors includes wide surgical resection with margins followed by radiotherapy and/or adjuvant chemotherapy. Although much progress has been made over the last few decades regarding the prognosis of many of these tumors, large multicenter trials are needed to better determine their optimal management. The current review will provide a broad review of the most current epidemiology, pathogenesis, prognosis, and management of the most common bone malignancies of the maxillofacial skeleton.

Use of hamstring autograft to reconstruct the patellar tendon after endoprosthetic replacement of the proximal tibia for bone sarcomas.

The surgical treatment of bone sarcoma involves wide surgical resection and endoprosthetic replacement. Surgical resection for proximal tibia bone sarcoma includes the patellar tendon. The secondary extension lag is a common complication and, although many have been described, the options for reconstruction of the patellar tendon are suboptimal. We propose adding a biological reinforcement, the hamstring tendons, to our extensor mechanism reconstruction technique (polyprolene mesh and gastrocnemius flap). We describe the surgical technique and, using an illustrative case, compare the outcome of this technique with the outcome following reconstruction without reinforcement. Level of evidence: Level III.

Sarcomas of the mandible.

Sarcomas of the mandible are extremely rare tumors, with osteosarcoma being the most common, followed by Ewing's sarcoma MATERIALS AND METHODS: A retrospective review of the clinical records,imaging studies, and pathology slides of patients with sarcoma of the mandible at a Tertiary Care Cancer Center from 1998 to 2014 was undertaken. The impact of neoadjuvant chemotherapy and postoperative radiotherapy with or without chemotherapy was studied, and factors impacting upon local control and disease-specific survival were analyzed. Twenty-two patients were treated over the study period, comprising of 15 males and seven females. External swelling, intraoral growth, or facial numbness were the presenting symptoms. Eighteen patients had osteosarcoma and four had the Ewing's sarcoma. Nine patients received neoadjuvant chemotherapy. All but one patient underwent surgery. Eleven had negative margins, with 90% recurrence-free survival at 3 years, compared to 10 with positive or close margins, leading to 67% recurrence-free survival. None of the patients receiving neoadjuvant chemotherapy developed recurrence and all were alive at 3 years. The impact of postoperative radiotherapy or adjuvant chemotherapy was not statistically significant. Wide surgical resection with negative margins remains the hallmark of surgical treatment.

Dose Characterization of the Investigational Anticancer Drug Tigilanol Tiglate (EBC-46) in the Local Treatment of Canine Mast Cell Tumors.

Mast cell tumor (MCT) is the most common cutaneous neoplasm in dogs and wide surgical resection is the current first-line treatment. However, recurrence is common and often requires more specialist and expensive therapies. Tigilanol tiglate is a novel small molecule drug delivered by intratumoral injection that is currently under development to provide a new option for treating MCT. The aim of this study was to characterize a safe and effective dose of tigilanol tiglate for canine MCT and to gather preliminary data on the drug's pharmacokinetics. A multicenter, open-label, uncontrolled, non-randomized, dose de-escalation design was used. Eligibility was MCT stage I/IIa and a tumor size of 0.1–6.0 cm3. Dosing was based on tumor size (50% v/v tumor) and 3 drug concentrations (1.0, 0.5, 0.2 mg/mL) were evaluated. Twenty-seven dogs were treated in 3 dose de-escalation cohorts (10, 10, and 7 dogs, respectively). Efficacy at 21 days was defined using international accepted solid tumor response criteria (RECIST). Greatest efficacy (90% complete response) was observed at the highest drug concentration (1.0 mg/mL) and adverse events were generally low grade, mild and transient, and directly associated with the mode of action of the drug. Hematological and serum biochemistry were generally unremarkable with plasma concentration curves typical of a non-intravenous parenteral medication. Intratumoral treatment of MCT with tigilanol tiglate at a concentration of 1.0 mg/mL was highly efficacious and well-tolerated. These results support the drug's further development for the treatment of MCT and other solid tumors.

Overall survival and tumor recurrence after surgical resection for primary malignant chest wall tumors: a single-center, single-surgeon experience.

Malignant primary chest wall tumors (PCWTs) comprise a rare group of thoracic tumors with unique anatomical considerations, and experience with wide surgical resection is limited to specialty referral centers and specific diagnoses. We investigated the tumor recurrence and overall survival (OS) for patients with a variety of PCWTs diagnoses at our institution. From 1991 to 2010, patients with malignant PCWT undergoing wide surgical resection for curative intent under a single surgeon were reviewed. Diagnosis and grade (if applicable) of surgical pathology, along with patient demographics, neoadjuvant chemotherapy or radiation therapy, and outcomes (complications, recurrence, and OS) at follow-up were analyzed. One hundred fifteen patients were included in the study. The most common tumor diagnoses included pleomorphic sarcoma and liposarcoma. Negative margins were achieved in 70 (74%) of cases. Postoperative complications were reported in 21 (20%) cases. The 5-year survival rate was 54%, while the 10-year survival rate was 29%. The local and distant recurrence rates were 50% and 38%, respectively. OS was significantly less in patients with any recurrence ( p < 0.001) but not significantly different between pathology grades ( p = 0.28). Wide resection for malignant PCWT is feasible when undertaken for a heterogenous group of diagnoses.

Craniomaxillofacial osteosarcoma — The role of surgical margins

BackgroundOsteosarcomas (OS) in the craniomaxillofacial (CMF) region are typically diagnosed at later age than long-bone OS, but they are reported to have better 5-year survival. Curative treatment warrants wide surgical resection, which is often not possible in the CMF region. The purpose of this article is to present a nationwide series of CMF in Finland to discuss the role of surgery. Patients and methodsAll 21 CMF OS patients managed in Finland from 1992 to 2009 were included. The mean age was 40 years (range 15–72). Data on patient and tumor characteristics, treatment modalities, and survival were recorded. All patients had a minimum follow-up of 5 years or until death. ResultsOS was evenly represented in the mandible and maxillary bones, which together constituted 76% of all sites. Surgery with curative intent was carried out in 20 patients. Clear margins were achieved in only five cases. Eight (40%) of these 20 patients died due to OS, and their average survival time was 1.3 years. Seven (35%) out of the 20 patients received radiotherapy due to close/intralesional surgical margins, and four of them did not develop recurrences during the follow-up. ConclusionsThe results suggest that postoperative radiotherapy may alter the prognosis in CMF OS, particularly in cases with close or intralesional margins. This may increase the survival rates achieved by prompt action in performing radical surgery.

Chordoma Management: A Review of the Literature

Chordoma is an uncommon bone neoplasm. Despite being considered a low-grade or benign bone tumor because of its histology, it is aggressive and locally invasive. It has a poor prognosis because it has a high rate of recurrence and is likely to be malignant. Chordoma is usually recurrent. Although its histology is considered a low-grade tumor or benign tumor, its behaviour of recurrence leads to a poor prognosis and higher likelihood of malignant tumors occurring. Chordoma is believed to emerge from transformed remnants of notochord and appears to favor the axial bone. The most commonly affected parts of chordoma are at the sacrum, vertebra, and skull base. The present gold standard for management of chordoma is a wide surgical resection. This article highlights recent practices and future directions for treatment. Keywords : chordoma, recurrent chordoma, treatment of chordoma DOI: 10.31524/bkkmedj.2019.02.019

Desmoplastic fibroma of the femur with atypical image findings

Rationale:Desmoplastic fibroma of the bone (DFB) is an extremely rare benign bone tumor, which can occur in any bone at all ages. Herein, we report a case of non-specific imaging findings.Patient concerns:A 25-year-old female patient was consulted in the Armed Police General Hospital (Beijing, China) due to repeated pain in the right thigh lasting over 1 year. Imaging examination revealed a space-occupying lesion in the right femur.Diagnosis:Desmoplastic fibroma of the femurInterventions:Wide surgical resectionOutcomes:At the 1-year follow-up visit, no relapse in the tumor was observed.Lessons:In this case report, we described and emphasized the particularity of the case examined and the possible non-specificity of the imaging results of some DFB cases.

Management of Sacrococcygeal Chordoma: A Systematic Review and Meta-analysis of Observational Studies.

Systematic review and meta-analysis of observational studies. The aim of the study is to evaluate different treatment modalities in the management of sacrococcygeal chordoma. Chordomas are primary malignant bone tumors associated with considerable morbidity and mortality. We searched MEDLINE, EMBASE, Cochrane Central-Register of Controlled Trials, and Scopus from inception to July 2015. Eligible studies included patients with sacrococcygeal chordoma treated exclusively with surgery, radiotherapy, or both. Two reviewers independently assessed the eligibility of potential studies, risk of bias, and extracted data. Outcomes of interest were all-cause mortality, progression-free survival, and metastases. We analyzed further surgical outcomes by resection margin. All outcomes were assessed at 60 months and more than 60 months following intervention. We included 33 noncomparative studies reporting on 501 patients (mean age 57 years). Overall mortality rate was (16%) after surgical resection with adjuvant radiotherapy and (28%) after surgical resection, and (43%) after radiotherapy (P = 0.28). All-cause mortality following wide surgical resection was (32%) compared to (40%) after marginal resection (P = 0.51). Overall progression-free survival rate was (58%) after surgical resection with adjuvant radiotherapy and (55%) after surgery (P = 0.92). However, at more than 60 months follow-up, progression-free survival rates were significantly higher (P = 0.024) following surgical resection with adjuvant radiotherapy (74%) in comparison to surgery (55%) and radiotherapy (36%). Overall progression-free survival rates were nonsignificantly higher after wide surgical resection (66%) than marginal resection (33%) (P = 0.16). However, at 60 months follow-up, progression-free survival rates were significantly higher following wide surgical resection (73%) than marginal resection (33%) (P = 0.047). Sacrococcygeal chordoma is a difficult to treat disease entity. Until comparative studies become available, wide surgical resection and multidisciplinary management are the recommended approaches to improve patient outcomes. 3.

P3.16-23 Soft-Tissue Sarcoma of the Chest Wall, Surgical Resection: Analysis Our Cases Period of Time Decembre 2004-July 2017

Soft tissues include muscle, fat, blood vessels, nerves, tendons and joint linings (synovial tissue). Cancerous tumors (sarcomas) of the soft tissue are rare, but there are many types. Because soft tissue sarcomas arise from a number of different kinds of tissue, they can occur anywhere in the body. Outcomes for people with soft tissue sarcomas depend on the type, size, grade, stage and location of the tumor, as well as a person's age and general health. Clasification from grade are :low ,midle ,high diferenciations..Aim of Study: Our objective was to introduce a patient diagnosed with neglectetd giant soft tissue chest wall liposarcoma . Records of 32 patients admitted to our institution from January 2004 to July 2017 were treated in our clinic’ A patient with the initials K.B bored 17/06/1950 .He was admitted to our hospital in thoracal surgery at 19.09.2012 with diagnosis : soft tissue tumor left chest wall .Pacienti without other diseases . We did all necessary examinations , CT , MRI and determined the extent of tissue tumor. Ages ranged from 13 to 86 years (median, 38 years); the ratio of male to female patients was 3:1. The initial complaint was mass or pain in 98% of the cases. Histologic types were as follows: desmoid tumor (n = 5), 15 %; liposarcoma (n = 7), 21%; rhabdomyosarcoma (n = 6), 18%); fibrosarcoma (n =4 , 12%); malignant peripheral nerve tumor (n = 3, 9%); malignant fibrous histiocytoma (n = 1, 3%); tenosynovial sarcoma (n = 1),3 %; hemangiopericytoma (n = 2) ,6%%); alveolar soft part sarcoma (n = 2),6%); and other types(lymphoma) (n = 2),6%). Resection was the primary treatment in 28 cases (87%).All patients were treated by multimodal treatment ,chemoradition therapy and surgery. Local recurrence developed in 25%. Metastases occurred in 15 (46%) of the cases (metachronous in 12, synchronous in 3) and were more common in patients with high-grade disease than in those with low-grade disease Overall 5-year survival was 60%. Five-year survival rate for those with high-grade sarcomas was significantly lower than that for low-grade sarcomas .Tumor size and age of patient were not prognostic. Surgery is important in the treatment of most sarcomas.[Additional treatments, including chemotherapy and radiation therapy, may be administered before and/or after surgery. Thoracic wall soft-tissue sarcomas are best controlled by wide surgical resection.

A Rare Case of Hematochezia: Rectal Melanoma

Introduction. Anorectal melanoma is an aggressive, rare subtype of both anorectal masses and melanomas. We describe a case of rectal melanoma presenting with lower gastrointestinal (GI) symptoms. Clinical Summary. A 60 year old Caucasian male with a history of Hepatitis C (no cirrhosis, on Ledipasvir/sofosbuvir) presented with 3 weeks of progressively worsening abdominal pain, constipation, hematochezia, and narrow stool caliber. On exam, the patient was hemodynamically stable and without lymphadenopathy. Rectal exam was negative for anal fissures, external hemorrhoids, or gross blood, however, noted a palpable mass in the posterior rectal wall. A colonoscopy found 6 polyps (from <5-9mm) throughout the colon that were removed with snare polypectomy, and found to be tubular adenomas. Also, a 7.2cm rectal mass involving the dentate line was visualized. Histology was positive for melanoma, and immunohistochemistry was positive for S100 and melan-A. Rectal endoscopic ultrasound showed the mass abutt the muscularis propria without clear penetration into the serosa. A staging PET scan was unremarkable. In the next 8 months, the patient underwent 4 cycles of pembrolizumab and 2 cycles of ipilimumab with radiation therapy (XRT). Discussion. Rectal melanoma (RM) is a rare cancer representing 1% of anorectal malignancies, and less than 1% of melanomas. Most literature is case series or smaller reviews. Histologically, kit and S-100 mutations are common and BRAF mutations are rare; our patient was only S-100 positive. In some metastatic case studies, kit mutations direct imantinib therapy. First line treatment is wide local surgical resection, sometimes with XRT. The role of chemotherapy is unclear. Diagnosis is difficult as symptoms are nonspecific and lesions are difficult to detect on physical exam. Even when visualized, masses are often non-melanotic. Clinical suspicion is thus essential when evaluating a patient with lower GI symptoms. Unfortunately, anorectal melanoma has a poor prognosis. Median age at diagnosis is 69, median survival is 18 months, and most patients present with at least stage II disease. Wide local excision is safer than abdominoperineal resection but does not have a relative survival advantage. Our patient was not a surgical candidate and during chemotherapy had primary tumor growth and metastases to the lung, liver, and kidney. This case highlights a rare, aggressive GI malignancy and emphasizes maintaining a high clinical suspicion.1552_A Figure 1. melanotic mass area on colonoscopy1552_B Figure 2. rectal mass on endoscopic ultrasound

Intensity-modulated radiotherapy for paranasal sinuses and base of skull tumors

Paranasal sinus and skull base tumors are rare aggressive head and neck cancers, and typically present in the locally advanced stages. As a result, achieving wide surgical resection with clear margins is a challenge for these tumors, and radiotherapy is thus usually indicated as an adjuvant modality following surgery to optimize local control. Given the integral role of radiotherapy in the management of this subgroup of head and neck tumors, the advent of intensity-modulated radiotherapy (IMRT) has led to substantial improvement of clinical outcomes for these patients. This is primarily driven by the improvement in radiation dosimetry with IMRT compared to conventional two dimensional (2D)- and 3D-techniques, in terms of ensuring dose intensity to the tumor target coupled with minimizing dose exposure to critical organs. Consequently, the evident clinical benefits of IMRT have been in reduction of normal tissue toxicities, ranging from critical neurological symptoms to less debilitating but bothersome symptoms of eye infections and radiation-induced skin changes. Another domain where IMRT has potential clinical utility is in the management of a subset of non-resectable T4 paranasal sinus and skull base tumors. For these inoperable lesions, the steep dose-gradient between tumor and normal tissue is even more advantageous, given the crucial need to maintain dose intensity to the tumor. Innovative strategies in this space also include the use of induction chemotherapy for patient selection. In this review, we summarized the data for the aforementioned topics, including specific discussions on the different histologic subtypes of paranasal sinus and skull base tumors.

Cystadenocarcinoma: Report of a Rare Case in Oral Cavity

Cystadenocarcinoma is a rare tumor of the oral cavity, recently reclassified by the World Health Organization as synonymous with adenocarcinoma, not otherwise specified. This report describes a case of cystadenocarcinoma in the buccal mucosa. A 72-year-old woman was referred for diagnosis of a lesion, of unknown duration, located in the right buccal mucosa. Intraorally, the lesion appeared as a well-delimited, mobile, and submucosal nodule. A benign mesenchymal neoplasm was the main hypothesis of diagnosis. Microscopic analysis revealed a salivary gland neoplasm formed by atypical cells arranged in microcystic structures, with frequent intraluminal papillary projections. The tumor cells exhibited positivity for CK7 and negativity for CK20. Based on these features, a diagnosis of cystadenocarcinoma was established. The patient was submitted to wide surgical resection. No recurrence was observed after 12 months. Although rare, cystadenocarcinoma should be considered in the differential diagnosis of oral submucosal nodules.

Extracellular Vesicle RNA Sequencing Reveals Dramatic Transcriptomic Alterations Between Metastatic and Primary Osteosarcoma in a Liquid Biopsy Approach.

Osteosarcoma (OS) is a highly metastasizing bone malignancy despite wide surgical resection of the primary lesion. A liquid biopsy approach to detect residual disease and identify therapeutic targets is still lacking. In this report, we aimed to track the metastasis of OS via extracellular vesicle (EV) RNA profiling in a non-invasive manner. We applied RNA sequencing for 10 matched metastatic and primary OS EV samples, including two pairs of cell lines and three pairs of plasma, and compared the expressed mutation, gene expression, fusion transcript, and alternative splicing (AS) between metastatic and primary OS at the transcriptome-wide level. Additional paired tissue/EVs were sequenced and public datasets were used to validate the EV-based metastatic biopsy. EVs were characterized through size-profiling, immunolabeling, and morphological examination. A drastic increase of mutation burden was observed in metastatic OS versus the non-metastatic counterpart. Hierarchical clustering of the expression profiles differentiated the metastatic EVs from the non-metastatic, with a signature enriched in cell-adhesion signaling and tyrosine kinase pathways. Moreover, 30 cancer-related gene fusions were identified in EV RNA as AS events tend to be more frequently observed in metastatic EVs. Further investigation suggested that over 70% of expressed point mutations from EVs could be validated in paired cell line/EV and tissue/EV analyses, and the expression signature significantly predicted 5-year survivorship of 42 patients from a public dataset. We have demonstrated a liquid biopsy-based approach for tracking cancer transcriptomic alterations, which is a promising source of prognostic and therapeutic biomarkers for metastatic OS. NCT03108677.

Ocular sebaceous gland carcinoma: an update of the literature.

To review and summarize the newest update on ocular sebaceous gland carcinoma (SGC) focusing on diagnosis and treatment. A PubMed search was carried out using the terms "Sebaceous Carcinoma", "Meibomian Gland Carcinoma", "Sebaceous Cell Carcinoma", and "Sebaceous Gland Carcinoma". All studies published in English up to October 2017 were included in this review. Globally, the overall incidence of SGC is increasing making it the third most common eyelid malignancy after basal cell carcinoma (BCC) and squamous cell carcinoma. The mainstay of treatment of ocular SGC is wide surgical resection under frozen section or Moh's micrographic surgery control followed by eyelid reconstruction. Based on histopathological features, SGC can be classified according to growth pattern, cell type, and cytoarchitecture. Based on the growth pattern, they can be classified as trabecular, lobular, papillary, and BCC-like. The cell type can be classified as basaloid, basosquamous, and epidermoid. The SGC cytoarchitecture presents either as a nodular or as an infiltrative lesion. Based on immunohistochemistry, the overexpression of ZEB2, BAG3, androgen receptor, and C-erbB-2 oncoprotein is associated with poor prognosis. The tumor is associated with systemic metastasis in 8-14% and death in 10-30%. Ocular SGC is an aggressive tumor associated with poor prognosis. Early identification and appropriate treatment may help improve the prognosis. New insight into its pathogenesis and the immunohistochemical profile may lead to the development of new effective treatment strategies, along with traditional therapies.

Wide local resection of sacral chordoma through posterior midline approach

This study was performed in 21 patients with sacral chordoma from July 2008 to June 2017 and posterior surgical approach was used for resection. Out of 21 patients, 12 had done subtotal sacrectomy and the remaining 9 had done partial sacrectomy. Their follow-up periods were at least five years. Operative time ranged between two to four hours. All patients recovered well from operation and two to five units of blood transfusion were needed for each. After operation, majority of the patients developed some bowel and bladder dysfunction and five patients developed wound infection. During the follow-up, two patients had tumor recurrence and one patient expired two years after operation. The remaining 18 patients were tumor-free at the 5-years follow-up. Wide surgical resection via the posterior midline approach could be a good management plan for the sacral chordoma. However, complete removal with surgical margin varies according to the location of the tumor.

Primary malignant melanoma of the female urethra − a case report

Introduction. Malignant melanoma accounts for 3% of all cancers in Europe. Malignant melanoma of the genitourinary tract is very rare, accounting for 1% of all cases. In addition, primary malignant melanoma of the urethra accounts for 0.2% of all melanomas and 4% of all urethral cancers. The abnormal migration of melanocytes during the embryonic development may explain their occurrence in the urethra. Also, vulva contains a relatively high concentration of melanocytes, so it may be the reason for higher incidence of malignant melanomas in females than in males, especially located at the meatus or in the distal urethra. Case Report. A 60-year-old woman presented with a solid tumor protruding through the vaginal introitus. Computerized tomography revealed a 5 cm large tumor of the urethra, infiltrating the bladder neck and the anterior vaginal wall. In addition, a bilateral pelvic lymphadenopathy was observed. The patient underwent anterior pelvic exenteration with urethrectomy, bilateral pelvic lymphadenectomy and bilateral ureterocutaneostomy, followed by immunochemotherapy. Nonetheless, the patient died 10 months after the surgery. Conclusion. Urethral melanoma is a tumor with a very poor prognosis and high recurrence rate (71%), even after wide surgical resection, adjuvant radiotherapy, chemo and immunotherapy.

Malignant Small Bowel Tumor - Case Series and Literature Review

Introduction: The diagnosis of malignant small bowel tumors is difficult due to the rarity of these lesions and the lack of presenting signs and symptoms. This fact may delay the treatment and therefore worsen the outcome. Material and methods: This article describes 6 cases of this rare disease, reviewing the literature on its clinic, pathological and treatment aspects. Discussion: Malignancies involving the small intestine are rare and account for less than 3% of the gastrointestinal tract cancers. When the different regions of the intestine are considered, adenocarcinomas are mostly found in the duodenum while stromal tumors are usually spread throughout the entire small bowel. Adenocarcinoma represents 33% of MSBT and is best managed with wide segmental surgical resection and primary and regional node lymphadenectomy. Sarcomas represent 17% of MSBT and are treated with en bloc segmental resection with tumor-free margins, with preservation of the tumor’s pseudocapsule. Secondary neoplastic involvement of the small intestine is more common than primary small neoplasia and its treatment is palliative. Conclusion: MSBT have a rare incidence, however attention and investigation of persistent symptoms may lead to an early diagnosis and treatment.

Epithelioid sarcoma of femoral nerve: A case report

Introduction. Epithelioid sarcoma is a slow-growing malignant soft tissue tumor and occurs approximately in 1% of all soft tissue malignant tumors. This case report describes epithelioid sarcoma in femoral nerve and as we know it is the first described case of epithelioid sarcoma at this anatomical localisation. Case report. A 44-years-old female patient presented with strong pain in her left leg. On magnetic resonance imaging (MRI), tumor was presented as a node 8 cm in the diameter at left femoral nerve between ileopsoas and iliacus muscle, without infiltration of muscle fascia and tendons. Four enlarged lymph nodes described in left iliac fosa, were suspected on secondary tumor deposits. We preformed radical surgical excision of the tumor and femoral nerve transection with local lymp hnode disection. Histopathological analysis revealed epithelioid sarcoma, and tumor stained highly positive for anti-pan cytokeratin antibody (AE1/AE3), vimentin, cancer antigen 125 (CA125), anticytokeratin antibody (MNF116), hematopoietic progenitor cell antigen (CD34) and epithelial membrane antigen (EMA) markers. After the operation patient recived radiotherapy without chemotherapy. Six months postoperatively, there was no evidence of local relapses or distant metastases. Conclusion. Initial wide surgical resection and adjuvant radiotherapy is beneficial in treatment of epitheloid sarcoma.

Salivary Gland Carcinoma Mimicking Secretory Breast Cancer

Mammary analogue secretory carcinoma (MASC) of the salivary gland is a newly classified pathologic entity since 2010. Prior to its recognition, MASC was diagnosed as low-grade cystadenocarcinoma, acinic cell carcinoma, and mucoepidermoid carcinoma. MASC shares common histological and genetic characteristics with secretory carcinoma of the breast and has a distinct feature of the ETV6-NTRK3 fusion gene. Treatment of MASC in salivary gland is mainly wide surgical resection of the tumor. Prognosis of MASC is similar to other low-grade salivary gland carcinomas. Herein, we report a case of MASC developed in a parotid gland with a review of the literature.