RESUMO
Objective:To investigate the clinical effect of retroauricular hairline approach in submandibular gland resection assisted by endoscope. Methods:A total of 18 patients with submandibular gland benign tumors treated in our hospital from September 2022 to September 2023 were selected. A 5 cm incision was designed in the retroauricular hairline, and the flap was turned over directly to the anterior edge of sternocleidomastoid muscle, then the flap was turned over to build the cavity through endoscopic surgery, and submandibular gland resection was completed with the assistance of endoscope. After operation, a negative pressure drainage was placed into the hairline, and the wound was closed by continuous intracutaneous suture. The clinical effect was evaluated after 3-6 months follow-up. Results:All patients underwent submandibular gland resection with endoscopic assistance as planned, and none of them converted to submaxillary incision during operation. The operation time was 65-97 min, with an average of 75 min. Intraoperative bleeding was 10-20 mL, with an average of 14 mL.No tongue numbness, wound infection, or tumor recurrence occurred after operation. However, there were 10 patients with ear numbness and discomfort of auricle after surgery, which gradually recovered after 6-9 months of follow-up. Two patients had crooked mouth after surgery, and the symptoms were gradually relieved after follow-up. All incisions healed in stage I and were concealed. Conclusion:Endoscopic retroauricular hairline approach is a choice for submandibular gland resection with good cosmetic effect, less trauma and fewer complications.
Assuntos
Endoscopia , Glândula Submandibular , Humanos , Masculino , Feminino , Endoscopia/métodos , Glândula Submandibular/cirurgia , Adulto , Pessoa de Meia-Idade , Retalhos Cirúrgicos , Neoplasias da Glândula Submandibular/cirurgia , Endoscópios , Resultado do Tratamento , Duração da CirurgiaRESUMO
Leiomyoma is a benign tumour of smooth muscle origin. Leiomyoma arising in major salivary gland is under-reported. We report a case of a woman in her 40s with a submandibular gland tumour which represented a diagnostic challenge during preoperative assessment. The core needle biopsy of submandibular gland tumour revealed a spindle cell tumour suggestive of an undifferentiated tumour. As a malignancy could not be excluded, the submandibular gland tumour was removed en bloc with surrounding lymph nodes in level Ib of the neck. Leiomyoma may be included in the differential diagnosis of spindle cell salivary gland tumours, particularly when there are no signs of infiltration and preoperative investigations are inconclusive.
Assuntos
Leiomioma , Neoplasias da Glândula Submandibular , Glândula Submandibular , Humanos , Feminino , Leiomioma/cirurgia , Leiomioma/patologia , Leiomioma/diagnóstico , Leiomioma/diagnóstico por imagem , Neoplasias da Glândula Submandibular/patologia , Neoplasias da Glândula Submandibular/cirurgia , Neoplasias da Glândula Submandibular/diagnóstico , Diagnóstico Diferencial , Glândula Submandibular/patologia , Glândula Submandibular/cirurgia , Glândula Submandibular/diagnóstico por imagem , AdultoRESUMO
Adenoid cystic carcinoma (ACC) is a rare tumour of the salivary glands characterised by distant metastases, mainly to lungs and bone. Isolated metastasis to the liver is unusual. We present the case of a woman with an ACC of the submandibular gland (pT1N0) who underwent radical submandibular gland excision and selective neck dissection. Preoperative imaging identified a liver lesion with features suggestive of a haemangioma. Two-year postoperatively, a surveillance CT neck/trunk showed an increase in size of the left liver lobe lesion. Subsequent MR liver and US-guided biopsy confirmed the lesion to be metastatic ACC. The patient underwent a successful left lateral liver sectionectomy. She remains disease-free 2.5 years after her liver resection. A literature search revealed only four other similar cases. This report highlights that even early-stage ACCs of the salivary gland may present with synchronous solitary liver metastasis which can be effectively treated with curative surgery.
Assuntos
Carcinoma Adenoide Cístico , Neoplasias Hepáticas , Neoplasias da Glândula Submandibular , Humanos , Carcinoma Adenoide Cístico/secundário , Carcinoma Adenoide Cístico/patologia , Carcinoma Adenoide Cístico/cirurgia , Carcinoma Adenoide Cístico/diagnóstico por imagem , Feminino , Neoplasias Hepáticas/secundário , Neoplasias Hepáticas/patologia , Neoplasias Hepáticas/cirurgia , Neoplasias Hepáticas/diagnóstico por imagem , Neoplasias da Glândula Submandibular/patologia , Neoplasias da Glândula Submandibular/secundário , Neoplasias da Glândula Submandibular/cirurgia , Tomografia Computadorizada por Raios X , Pessoa de Meia-Idade , Glândula Submandibular/patologia , Glândula Submandibular/cirurgia , Hepatectomia , Imageamento por Ressonância Magnética , Esvaziamento CervicalRESUMO
BACKGROUND: Angiosarcoma is a sarcoma that occurs in a range of tissue types, and only rarely in the salivary glands, showing a predilection for the parotid glands of older patients. Preoperative diagnosis may be challenging, especially on cytology, with significant morphological overlap with high-grade primary salivary gland carcinomas. The molecular alterations of this rare salivary gland neoplasm are also not well-characterized. METHODS AND RESULTS: We present a case of right submandibular gland swelling in a 73-year-old male. On fine needle aspiration, including immunohistochemical stains on cell block, the tumor was initially diagnosed as poorly differentiated carcinoma. Resection of the submandibular gland revealed epithelioid angiosarcoma. We performed molecular work-up of the tumor, utilizing targeted next-generation sequencing, DNA methylation profiling and fluorescence in-situ hybridization. Histopathologic assessment revealed an infiltrative tumor comprising solid sheets of epithelioid cells. The tumor cells formed haphazardly anastomosing vascular channels with intracytoplasmic lumina containing red blood cells. On immunohistochemistry, the tumor cells were positive for CD31, CD34 and ERG. Approximately 40% of the tumor cells showed nuclear expression of GATA3. A pathogenic TP53 R267W mutation was detected on next-generation sequencing. DNA methylation analysis did not cluster the tumor with any known sarcoma type. Copy number analysis showed possible MYC amplification and CDKN2A losses, although only the latter was confirmed on fluorescence in-situ hybridization. CONCLUSION: Epithelioid angiosarcoma is an important differential diagnosis to high-grade salivary gland carcinoma. In particular, GATA3 expression may be encountered in both angiosarcoma and high-grade salivary gland carcinomas and cause diagnostic confusion. Identification of TP53 mutations and CDKN2A losses suggest shared oncogenic pathways with soft tissue angiosarcomas, and should be further investigated.
Assuntos
Hemangiossarcoma , Neoplasias da Glândula Submandibular , Humanos , Masculino , Idoso , Hemangiossarcoma/genética , Hemangiossarcoma/patologia , Hemangiossarcoma/diagnóstico , Neoplasias da Glândula Submandibular/patologia , Neoplasias da Glândula Submandibular/genética , Neoplasias da Glândula Submandibular/diagnóstico , Biomarcadores Tumorais/análise , Biomarcadores Tumorais/genética , CitologiaRESUMO
BACKGROUND: While the accurate prediction of the overall survival (OS) in patients with submandibular gland cancer (SGC) is paramount for informed therapeutic planning, the development of reliable survival prediction models has been hindered by the rarity of SGC cases. The purpose of this study is to identify key prognostic factors for OS in SGC patients using a large database and construct decision tree models to aid the prediction of survival probabilities in 12, 24, 60 and 120 months. MATERIALS AND METHODS: We performed a retrospective cohort study using the Surveillance, Epidemiology and End Result (SEER) program. Demographic and peri-operative predictor variables were identified. The outcome variables overall survival at 12-, 24-, 60, and 120 months. The C5.0 algorithm was utilized to establish the dichotomous decision tree models, with the depth of tree limited within 4 layers. To evaluate the performances of the novel models, the receiver operator characteristic (ROC) curves were generated, and the metrics such as accuracy rate, and area under ROC curve (AUC) were calculated. RESULTS: A total of 1,705, 1,666, 1,543, and 1,413 SGC patients with a follow up of 12, 24, 60 and 120 months and exact survival status were identified from the SEER database. Predictor variables of age, sex, surgery, radiation, chemotherapy, tumor histology, summary stage, metastasis to distant lymph node, and marital status exerted substantial influence on overall survival. Decision tree models were then developed, incorporating these vital prognostic indicators. Favorable consistency was presented between the predicted and actual survival statuses. For the training dataset, the accuracy rates for the 12-, 24-, 60- and 120-month survival models were 0.866, 0.767, 0.737 and 0.797. Correspondingly, the AUC values were 0.841, 0.756, 0.725, and 0.774 for the same time points. CONCLUSIONS: Based on the most important predictor variables identified using the large, SEER database, decision tree models were established that predict OS of SGC patients. The models offer a more exhaustive evaluation of mortality risk and may lead to more personalized treatment strategies.
Assuntos
Árvores de Decisões , Programa de SEER , Neoplasias da Glândula Submandibular , Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Estudos Retrospectivos , Neoplasias da Glândula Submandibular/patologia , Neoplasias da Glândula Submandibular/terapia , Idoso , Prognóstico , Adulto , Taxa de Sobrevida , Estadiamento de Neoplasias , Algoritmos , Análise de SobrevidaRESUMO
Sublingual gland herniation into the submandibular space through a mylohyoid muscle defect is a common anatomical variation; however, salivary gland cancers that arise from a herniated sublingual gland have not been described yet. Here, we report three patients with salivary gland cancers originating from a herniated sublingual gland. All tumors were detected as palpable submandibular masses, located anterior to the submandibular gland, medial to the mandible, and lateral to the mylohyoid muscle, with contact with the sublingual gland through a mylohyoid muscle defect. Intraoperative findings confirmed that the masses were derived from herniated sublingual glands. Pathological examination showed one case of mucoepidermoid carcinoma and two cases of adenoid cystic carcinoma. Imaging findings of the tumor location, in addition to the continuity with the sublingual gland through the mylohyoid muscle defect, are crucial for accurately diagnosing the tumor origin, which is essential for determining the appropriate clinical management.
Assuntos
Neoplasias das Glândulas Salivares , Glândula Sublingual , Humanos , Hérnia/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Neoplasias das Glândulas Salivares/diagnóstico por imagem , Neoplasias das Glândulas Salivares/cirurgia , Neoplasias das Glândulas Salivares/patologia , Glândula Sublingual/diagnóstico por imagem , Glândula Sublingual/patologia , Glândula Sublingual/cirurgia , Neoplasias da Glândula Sublingual/diagnóstico por imagem , Neoplasias da Glândula Submandibular/diagnóstico por imagem , Neoplasias da Glândula Submandibular/patologia , Neoplasias da Glândula Submandibular/cirurgia , Tomografia Computadorizada por Raios XRESUMO
The aim of this report was to describe a rare example of sporadic intestinal-type adenocarcinoma of the major salivary glands and oral cavity. A 23-year-old female patient presented an asymptomatic, progressive-growing mass involving the floor of mouth and the left submandibular gland. Fine-needle aspiration cytology, imaging exams, and surgical specimen findings were consisted with the diagnosis of primary intestinal-type adenocarcinoma, despite its similar immunohistochemical results with colorectal adenocarcinoma. Adjuvant chemotherapy and radiotherapy were performed, but the patient developed multiple metastatic lesions after one year of initial the intervention and deceased following 13 months of follow-up, despite several therapeutic efforts. We verified that sporadic cases of primary intestinal-type adenocarcinoma still lack information regarding etiology and tumorigenesis, especially in young and females. A complete diagnostic workflow is indispensable to rule out the presence of primary colorectal adenocarcinoma.
Assuntos
Adenocarcinoma , Neoplasias Colorretais , Neoplasias da Glândula Submandibular , Feminino , Humanos , Adulto Jovem , Adulto , Adenocarcinoma/diagnóstico por imagem , Adenocarcinoma/terapia , Glândulas Salivares/patologia , Neoplasias da Glândula Submandibular/patologia , Glândula Submandibular/patologia , Neoplasias Colorretais/patologiaRESUMO
INTRODUCTION: Benign submandibular gland tumors pose challenges in balancing treatment effectiveness and preserving gland function. This study aimed to compare gland-preserving surgery, extracapsular dissection (ECD), with total excision in managing these tumors, focusing on function preservation and recurrence rate. METHODS: Fifty consecutive patients with treatment-naïve benign submandibular gland tumors were alternatively allocated to receive ECD (n = 25) or total excision (n = 25) without randomization procedures. Intraoperative findings, postsurgical complications, subjective satisfaction, and gland function were assessed. Follow-up data were collected for a median duration of 55 months (24-80 months) to monitor recurrences. RESULTS: ECD demonstrated significant advantages, including shorter operation time, reduced bleeding, and preservation of the facial artery and vein (P < 0.05). Both groups exhibited acceptable postsurgical pain and taste sensations. Complications were minimal and similar between the two groups. ECD resulted in superior facial contour satisfaction (P = 0.030) and preserved gland function, as evidenced by salivary scintigraphy. No recurrences were observed in either group during the follow-up period. CONCLUSIONS: ECD is a practical approach for benign submandibular gland tumors, offering favorable functional outcomes, reduced surgical morbidity, shorter operation times, and improved cosmetic results.
Assuntos
Neoplasias da Glândula Submandibular , Humanos , Neoplasias da Glândula Submandibular/cirurgia , Dissecação/métodos , Resultado do Tratamento , Complicações Pós-Operatórias/epidemiologia , Estudos RetrospectivosRESUMO
Null.
Assuntos
Adenocarcinoma , Neoplasias da Glândula Submandibular , Humanos , Adenocarcinoma/patologia , Neoplasias da Glândula Submandibular/patologiaRESUMO
Primary submandibular gland tumors are relatively rare. Due to its low incidence and broad spectrum phenotypic, biological and clinical heterogeneity types, a wide range of options have been developed to treat this tumor. To date, however, efficacious standard treatment regimens are lacking. Here, the authors present a case of a patient with an advanced submandibular gland tumor. Histological and imaging results diagnosed the case as stage IV submandibular gland adenocarcinoma with multiple metastases. The patient was subjected to systemic platinum-based chemotherapy combined with sintilimab. A primary lesion complete response was observed after six cycles of treatment. This case affirms the efficacy of the PD-1 inhibitor sintilimab combined with platinum-based chemotherapy as a first-line treatment for advanced submandibular gland tumors.
Primary submandibular gland tumors are very uncommon. There is a lack of standard treatment plans due to the low incidence and diverse clinical situations. The authors report a case of an advanced submandibular gland tumor patient who received platinum-based chemotherapy combined with sintilimab as the initial treatment plan. The tumor and multiple lung metastases significantly shrank after six cycles of treatment. This case indicates the regimen is effective for advanced submandibular gland tumor patients.
Assuntos
Adenocarcinoma , Neoplasias da Glândula Submandibular , Humanos , Neoplasias da Glândula Submandibular/tratamento farmacológico , Anticorpos Monoclonais Humanizados/uso terapêutico , Inibidores de Checkpoint Imunológico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêuticoRESUMO
We report the case of a 10-year-old girl with a painless slowly growing mass that had developed over the course of 2 years in the left submandibular area. Physical examination revealed a firm painless submandibular mass with no other associated signs. Ultrasound graphics were in favor of a sebaceous cyst. Fine-needle aspiration and magnetic resonance images (MRI) concluded to a pleomorphic adenoma of the submandibular gland. The patient underwent left submandibulectomy. Histopathological examination confirmed the diagnosis of pleomorphic adenoma arising from the submandibular gland. The postoperative course was uneventful. Pleomorphic adenoma of the submandibular gland represents a diagnostic and therapeutic dilemma in children. Preoperative evaluation, including MRI and fine-needle aspiration, is recommended. Total submandibulectomy is also recommended to minimize recurrences and to avoid malignant transformation.
Assuntos
Adenoma Pleomorfo , Neoplasias da Glândula Submandibular , Feminino , Humanos , Criança , Glândula Submandibular/diagnóstico por imagem , Glândula Submandibular/cirurgia , Glândula Submandibular/patologia , Adenoma Pleomorfo/diagnóstico por imagem , Adenoma Pleomorfo/cirurgia , Neoplasias da Glândula Submandibular/diagnóstico por imagem , Neoplasias da Glândula Submandibular/cirurgia , Cabeça/patologia , Biópsia por Agulha FinaRESUMO
Endoscope-assisted surgery is becoming a preferred technique in salivary gland surgery. However, this technique has not yet been applied in submandibular gland (SMG) preservation surgery. This retrospective study was performed to evaluate the outcomes of endoscope-assisted gland-preserving surgery through a hairline incision in patients with benign SMG tumours. The study included 38 patients with benign SMG tumours who underwent tumour excision with gland preservation: 19 who underwent local excision of the tumour through an endoscope-assisted hairline approach and 19 who received the conventional cervical approach. The feasibility of the surgical procedure, perioperative patient variables, and postoperative appearance and functional outcomes were evaluated. Patients in both groups had their tumours removed successfully with tumour-free margins. The intraoperative blood loss, postoperative amount of drainage, mean length of the incision, and unstimulated saliva flow rate did not differ between the two groups. There was no difference in the stimulated saliva flow rate between the preserved gland and unaffected SMG. The aesthetic result was better in the endoscope-assisted hairline incision group. No tumour recurrence occurred during follow-up (range 12-52 months). Thus, gland-preserving tumour dissection appears to be a safe method for benign SMG tumours, with good functional results. Furthermore, the endoscope-assisted hairline incision is a feasible method with excellent cosmetic results.
Assuntos
Neoplasias da Glândula Submandibular , Humanos , Neoplasias da Glândula Submandibular/cirurgia , Neoplasias da Glândula Submandibular/patologia , Estudos Retrospectivos , Recidiva Local de Neoplasia/patologia , Estética Dentária , Endoscopia/métodos , Glândula Submandibular/cirurgia , Glândula Submandibular/patologiaAssuntos
Carcinoma Intraductal não Infiltrante , Neoplasias da Glândula Submandibular , Carcinoma Intraductal não Infiltrante/patologia , Cabeça/patologia , Humanos , Glândula Submandibular/diagnóstico por imagem , Glândula Submandibular/patologia , Neoplasias da Glândula Submandibular/diagnóstico , Neoplasias da Glândula Submandibular/patologiaRESUMO
BACKGROUND: As a debilitating syndrome, paraneoplastic cerebellar degeneration (PCD) remains challenging to treat. Further, anti-Yo antibody (directed against human cerebellar degeneration-related protein 2) detection in patients with PCD is associated with unsatisfactory responses to existing therapies. Here, we present the case of a 60-year-old woman who developed PCD with anti-Yo antibodies and a submandibular gland tumor. CASE PRESENTATION: A 60-year-old woman presented with a 5-day history of unsteadiness of gait and inadequate coordination of her extremities, along with truncal instability. Although walking without aid was possible, dysmetria of all four limbs, trunk, and gait ataxia was observed. While routine biochemical and hematological examinations were normal, the patient's blood was positive for anti-Yo antibodies. When the neurological symptoms deteriorated despite administration of intravenous methylprednisolone, fluorodeoxyglucose-positron emission tomography (FDG-PET) and computed tomography (CT) images with contrast enhancement were performed, which showed a tumor in the left submaxillary gland. She underwent total left submandibular gland resection, including the tumor; histological and immunohistochemical results revealed a salivary duct carcinoma. She was administered intravenous methylprednisolone, followed by 10 plasma exchange sessions, intravenous immunoglobulins, and cyclophosphamide therapy. Following treatment, her symptoms were not alleviated, even after the reduction of anti-Yo titers. CONCLUSIONS: Although tumor detection was delayed, early tumor detection, diagnosis, and PCD treatment are essential because any delay can result in the progression of the disorder and irreversible neurological damage. Therefore, we recommend that the possibility of a salivary gland tumor should be considered, and whole-body dual-modality CT, including the head and neck, and FDG-PET should be performed at the earliest for patients with well-characterized paraneoplastic antibodies when conventional imaging fails to identify a tumor.
Assuntos
Degeneração Paraneoplásica Cerebelar , Neoplasias da Glândula Submandibular , Anticorpos Antineoplásicos , Autoanticorpos , Feminino , Fluordesoxiglucose F18 , Humanos , Metilprednisolona , Pessoa de Meia-Idade , Degeneração Paraneoplásica Cerebelar/complicações , Degeneração Paraneoplásica Cerebelar/diagnóstico , Neoplasias da Glândula Submandibular/complicaçõesRESUMO
Adenoid cystic carcinoma of the submandibular gland is a relatively rare malignancy of the oral and maxillofacial region. It is an insidious disease characterized by regional spread, perineural invasion, and potential distant metastases. Because of the aggressive nature of this lesion, it is often impossible to completely excise the tumor, necessitating combination therapy of surgery and radiation. The consequences of surgical ablation coupled with radiation treatment can result in complex considerations in the dental care of these patients. This case report outlines a patient who has recovered from multiple surgeries and radiation therapy, requiring mandibular dental implant reconstruction for a return to normal comfort and function.
Assuntos
Implantes Dentários , Reconstrução Mandibular , Neoplasias da Glândula Submandibular , Humanos , Mandíbula/cirurgia , Glândula SubmandibularRESUMO
OBJECTIVE: Submandibular gland (SMG) malignancies are exceedingly rare. Lymph node metastasis is one of the most important determinants of outcome in SMG malignancies. The aim of this study was to investigate the overall rate of occult neck nodal metastasis in SMG malignancies. STUDY DESIGN: The study design is a meta-analysis of all studies on patients with a primary SMG malignancy, without evidence of neck nodal metastasis, who underwent an elective neck dissection (END). The search strategy identified 158 papers that appeared in print from January 1980 to July 2020. All eligible patients from the Tel-Aviv Medical Center were analyzed and consolidated into a case series. A total of 12 retrospective studies that included 306 suitable patients met inclusion criteria. RESULTS: The risk for occult metastasis in primary SMG malignancies was 0.0% to 50.0%, with a fixed effect model of 19.52% (95% CI, 14.9%-24.5%). The analyzed studies included a wide range of pathologies. The most common malignancies were adenoid cystic carcinoma and mucoepidermoid carcinoma. CONCLUSIONS: The overall rate of occult neck metastasis in SMG malignancies is relatively high, and an END should be the default intervention in these cases. An END is unwarranted in tumors judged clinically to be low stage and low grade.
Assuntos
Neoplasias da Glândula Submandibular , Glândula Submandibular , Humanos , Esvaziamento Cervical , Estadiamento de Neoplasias , Estudos Retrospectivos , Neoplasias da Glândula Submandibular/patologia , Neoplasias da Glândula Submandibular/cirurgiaRESUMO
OBJECTIVE: Owing to histologic heterogeneity, biological behavior, and rarity, recommendations for the treatment of malignant submandibular gland tumors (MSGT) are inconsistent. The aim of this study was to present a single-center experience in the treatment of MSGT with an emphasis on surgical treatment, including indication on elective neck dissection (END). STUDY DESIGN: Twenty-four MSGT were primary surgically treated (gland excision with neck dissection). Their records were retrospectively collected and analyzed. RESULTS: The most frequent histology was adenoid cystic carcinoma (41.6%), followed by mucoepidermoid carcinoma (25%) and carcinoma ex pleomorphic adenoma (16.7%). There were 18 elective and 6 therapeutic neck dissections. Histopathologic examination confirmed 29% (7/24) of positive neck dissection specimens. The Kaplan-Meier analysis presented rates of disease-specific survival, disease-free survival, and overall survival (OS) of 81%, 78%, and 52% at 5 years, respectively. Patients undergoing postoperative radiotherapy had significantly higher OS rates compared with patients treated with surgery alone (P = .0209). CONCLUSION: Results of this study suggest that END has questionable benefit in early stage MSGT. Elective selective neck dissection levels I-III is recommended in high-grade and advanced stage MSGT without evidence of multilevel lymphadenopathy.
Assuntos
Adenoma Pleomorfo , Carcinoma Adenoide Cístico , Neoplasias das Glândulas Salivares , Neoplasias da Glândula Submandibular , Adenoma Pleomorfo/patologia , Carcinoma Adenoide Cístico/cirurgia , Humanos , Estudos Retrospectivos , Neoplasias das Glândulas Salivares/patologia , Neoplasias das Glândulas Salivares/cirurgia , Neoplasias da Glândula Submandibular/diagnóstico , Neoplasias da Glândula Submandibular/cirurgiaRESUMO
BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is a sporadic, highly malignant tumor with a poor prognosis. The abdomen and pelvis have been reported as the primary localization sites. However, to the best of our knowledge, there are few reports on primary DSRCT in the submandibular gland. CASE PRESENTATION: We report a case of a 26-year-old Chinese man with a mass in the right submandibular gland. Imaging studies showed a hypoechoic mass in the right submandibular region. Intraoperative pathology revealed that the tumor tissue was composed of small round tumor cells and a dense desmoplastic stroma. On immunostaining, the tumor cells showed markers of epithelial, mesenchymal, myogenic, and neural differentiation. The EWSR1 gene rearrangement was detected by fluorescence in situ hybridization. Based on the overall morphological features and immunohistochemical findings, a final diagnosis of DSRCT was made. The patient was treated with comprehensive anti-tumor therapy mainly based on radiotherapy and chemotherapy. CONCLUSIONS: DSRCT is an uncommon malignant neoplasm with rare submandibular gland involvement. In this report, we have described a case of DSRCT in the submandibular gland and reviewed the literature on DSRCT over the past 5 years. Considering the importance of differential diagnosis between DSRCT, especially with rare extra-peritoneal involvement, and small round blue cell tumors, a full recognition of the clinicopathological features will help to better diagnose this neoplasm.