Management of malignant submandibular gland tumors: A 30-year experience from a single center.

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.

Primary desmoplastic small round cell tumor of the submandibular gland: a case report and literature review.

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.

Hsp27 and Hsp60 in human submandibular salivary gland: Quantitative patterns in healthy and cancerous tissues with potential implications for differential diagnosis and carcinogenesis.

Tumors of the submandibular salivary gland (SMG) are uncommon but sufficiently frequent for the physician to consider them in routine examinations and for the pathologist to be prepared to differentiate them from other tissue abnormalities. However, scarcity of specimens makes training difficult, a situation compounded by the lack of accepted universal diagnostic guidelines. Furthermore, there is little information on the chaperone system (CS) of the gland, despite the increasing evidence of its participation in carcinogenesis as a biomarker for diagnosis and patient follow up, and in the mechanisms by which the tumor cells thrive. We are investigating this aspect of various tumors, and here we describe standardized methods for assessing the tissue levels of two chaperones, Hsp27 and Hsp60, in normal SMG and its tumors. We present illustrative results obtained with immunohistochemistry (IHC) and immunofluorescence-confocal microscopy (IF-CM), which we propose as a platform onto which a data base could be built by adding new information and which would provide material for developing guidelines for tumor identification and monitoring. The initial findings are encouraging in as much as the tumors surveyed showed quantitative patterns of Hsp27 and Hsp60 that distinguished tumoral from normal tissue and certain tumors from the others, and the results from IHC were confirmed by IF-CM.

Surgical treatment of liver and kidney metastasis from an adenoid cystic carcinoma of the submandibular gland: case report.

INTRODUCTION: Adenoid cystic carcinomas (ACCs) account for 10% of all malignant salivary tumours. They are slow-growing but locally aggressive. Reports of liver and renal metastases are rare. CASE DESCRIPTION: A 58-year-old woman who had undergone resection of a left submandibular ACC in 1995 was referred to our centre for follow-up in 2018. A computed tomography scan revealed two lesions: one on segment six of the liver and the other on a kidney. A hepatic wedge resection and right nephrectomy were performed. The postoperative course was uneventful. At 2-year follow-up, the patient was alive and well with no evidence of disease. CONCLUSION: Management of ACC is a therapeutic challenge because of its tendency for distant metastases. The literature recommends regular follow-up imaging and radical surgical treatment but specific guidelines for the approach to recurrence are lacking.

Histopathologic and clinical outcomes of Milan System categories "non-diagnostic" and "non-neoplastic" of salivary gland fine needle aspirations.

INTRODUCTION: The Milan System for Reporting Salivary Gland Cytopathology (MSRSGC) specifies six categories with estimated risks of malignancy (ROM) and suggested management. The estimated ROM is 25% for Non-Diagnostic (ND) category, and 10% for Non-Neoplastic (NN). This study aimed to investigate histopathologic and clinical outcomes of MSRSGC categories ND and NN at the authors' institution. MATERIALS AND METHODS: Cytopathology fine needle aspiration reports from 2008-2020 were searched for the word "salivary", "parotid", and "submandibular". Cases fitting Non-Diagnostic (ND) and Non-Neoplastic (NN) categories were identified. Follow-up cyto-/histopathologic and clinical data were extracted. RESULTS: There were 43 ND and 46 NN cases. The average age was 58.3 years. Neoplastic lesions were found in 13 of 43 (30%) ND and 3 of 46 (6.5%) NN. The rate of malignancy in ND category was 14.0% (6/43) and 0% (0/46) in NN category. Four cases in ND (9.3%) and 6 (13.0%) in NN had no neoplasm and instead had an underlying reactive condition (e.g., chronic sialadenitis) or inflammatory lesion (e.g., lymphoepithelial cyst) on histologic follow-up. There was no follow-up pathology in 46.5% NDs (20/43) and 82.6% NNs (38/46); however, no lesions were apparent clinically with a mean follow-up of 3 years and 1.5 years, respectively. CONCLUSIONS: MSRSGC categories ND and NN are helpful for reporting salivary gland FNA results. With proper clinical and radiologic correlation, ROM of NN is low; however, ROM of ND remains significant. Repeat FNA after correlation for ND cases seems prudent as neoplasms and malignancies may have gone undetected.

Endobronchial sialolipoma. Case report.

BACKGROUND: A 52-year-old woman presented with shortness of breath and cough. An endobronchial sialolipoma was found at the left entrance of the main bronchus. Sialolipoma is an exceedingly rare type of lipoma reported of the minor salivary glands, especially within the bronchus. CASE PRESENTATION: A 52-year-old woman presented with shortness of breath and cough with 6 months´ evolution. Endobronchial endoscopy revealed a tumour at the left entrance of the main bronchus. The entire removal of the tumour was removed using a cryoprobe device. Pathological examination showed a tumour consistent with the diagnosis of sialolipoma due to the presence of mature adipose cells blended with acinar, ductal, basal, and myoepithelial cells. The patient had a favourable outcome. CONCLUSION: The infrequent tracheobronchial presentation of this tumour can be challenging for correct diagnosis.

Solitary fibrous tumour of the submandibular gland: Novel insights from clinical practice on a close mimicker of pleomorphic adenoma and a diagnostic challenge for the cytopathologist.

Spindle cell lesions of the salivary glands are rare and account for less than 3% of the salivary gland aspirates. A definitive classification of salivary gland neoplasms by fine needle aspiration is possible for the most common lesions but remains a challenge for the less common entities. We present a case of a 72-year-old male with a solitary fibrous tumour (SFT) of the submandibular gland mimicking a myoepithelial-rich pleomorphic adenoma (PA). In this article, we discuss novel insights that help differentiate SFT from PA and other spindle cell lesions.

Unusual presentation of submandibular lingual nerve sheath tumour as sublingual stone.

A 60-year-old woman was referred to the otolaryngologist for 18 months of left-sided tongue pain and taste changes. Surgeon-performed ultrasound of the submandibular region revealed a hyperechoic mass. Wharton's duct was dilated proximally and the submandibular gland demonstrated normal vascularity. While these findings were highly suspicious for submandibular gland sialolith, an in-office attempt at sialolithotomy suggested an alternate process or mass. After imaging failed to further elucidate an aetiology, surgical exploration revealed a well-circumscribed submandibular mass associated with the lingual nerve. The mass was removed en-bloc and pathology revealed a schwannoma of the lingual nerve.

Giant submandibular sialolipoma masquerading as huge goitre: a case report.

BACKGROUND: Sialolipoma is a rare tumour which may arise from both major and minor salivary glands and has recently been described as a variant of salivary gland lipomatous lesions. CASE PRESENTATION: We report a 54-year-old male who presented with a 7-year history of large right anterior neck swelling. He was clinically euthyroid and had no compressive or infiltrative symptoms. He sought medical attention due to the discomfort exerted by the weight of the mass and was keen for excision. The swelling appeared like a goitre but physical examination proved otherwise. Imaging was suggestive of a benign tumour arising from the right parapharyngeal fossa. The mass was surgically excised and was noted to be adherent to part of the submandibular gland. Histopathological examination revealed a new variant of benign adipocytic tumour of salivary gland or sialolipoma arising from the submandibular gland. Besides being the largest sialolipoma to be reported, there are also no reports of giant submandibular sialolipomas masquerading as a huge goitre in appearance. CONCLUSION: Submandibular sialolipomas can present in really large sizes and appear as a giant goitre. It is important to differentiate between benign lipomas from liposarcomas and tailor the management accordingly. Surgical enucleation is the preferred choice of treatment for these benign tumours with low recurrence rates.

Schwannoma-Like Pleomorphic Adenoma: Two Cases and a Review of the Literature.

Schwannoma-like pleomorphic adenoma is a rare variant of the common benign salivary neoplasm, pleomorphic adenoma. This entity's cytomorphology closely mimics a schwannoma, potentially making a diagnosis of cytologic preparations or of surgical specimens a challenge. To the best of our knowledge, there are only six previous reports of schwannoma-like pleomorphic adenoma with eight total cases in the English language literature prior to the addition of the two cases detailed in this report. Our report includes what we believe to be the first documented case of this entity occurring in the submandibular gland. One of our cases occurred in the submandibular gland of a 90-year-old woman and the other occurred in the left parotid gland of a 40-year-old woman. We also examine the diagnostic considerations used to differentiate these two entities.

A case of oncocytic lipoadenoma of the submandibular gland and its diagnostic cytology challenges.

Lipomatous tumors of the salivary gland in general are rare and oncocytic lipoadenomas are even much rarer. Most of the reported cases of oncocytic lipoadenoma were located in the parotid gland and to the best of our knowledge, only four cases reported to arise from the submandibular gland. The preoperative interpretation of the aspirates taken from these lesions can be difficult and challenging. We report a case of a 41 year old gentleman who presented with submandibular gland swelling. CT scan of the neck revealed an oval-shape enhancing mass in the inferior aspect of the right submandibular gland measuring 19 × 12 mm. Fine needle aspiration biopsy (FNAB) showed hypocellular smears with few clusters of epithelial cells having oncocytic appearance. The cytologic findings were interpreted as "oncocytic lesion." Submandibular gland excision was done. Histologically, a final diagnosis of oncocytic lipoadenoma was rendered. We present this rare entity to add to the few cases reported to date and to upsurge awareness during cytologic examination of oncocytic salivary gland lesions.

Sialoblastoma of the submandibular gland: a distinct entity?

Sialoblastoma is a rare congenital malignant tumor of the salivary glands. A case of a submandibular sialoblastoma in a 1.5-year-old child is presented. A comparative analysis on 79 pediatric cases reported in the literature suggests a less aggressive behavior for submandibular sialoblastoma in comparison with other sites. Classically, diagnosis is confirmed by open biopsy, but fine-needle aspiration may offer an alternative with reduced morbidity. Expression of AFP and high levels of Ki-67 have been associated with poor prognosis. Whilst early surgical resection with negative margins is widely accepted as first-line treatment, there is no consensus on therapy of recurrence and follow-up. MRI and sonography represent valid tools for the follow-up, which is usually restricted to 3-5 years.Conclusion: Submandibular sialoblastomas may have a different biological profile in comparison with parotid tumors with the absence of metastasis and much lower rate of recurrence. Comprehensive diagnostics should include additional options such as fine-needle aspiration and markers to assess cell proliferation and AFP. Literature suggests that surgery alone is sufficient for the treatment of tumors with low malignancy. Follow-up should be tailored according to the tumor site and might be limited to 3-5 years. What is Known: • Sialoblastoma is a rare congenital malignant tumor with an unpredictable clinical outcome. What is New: • Sialoblastoma of submandibular origin seems to have a less aggressive behavior in comparison with other sites. • Fine-needle aspiration and markers to assess proliferation index (i.e., suggestive of potential more aggressive course/malignancy) should be strongly considered in the diagnostic work-up. • Radical surgery as first-line therapy and a 3-5-year follow-up are acceptable for tumors with a low malignancy.

Synchronous double malignancy of lip and submandibular salivary gland with rare histology.

In the modern era, there is an increase in the incidence of double malignancies owing to the remarkable improvement in cancer diagnostics and patient survival. Double malignancies can be either synchronous or metachronous. Synchronous double malignancy can present either at the same time or within 6 months of diagnosis of the first one. We present a case of double malignancy of lip and submandibular salivary gland, diagnosed in a 55-year-old male, who presented with ulceroproliferative lesion of the upper lip and a hard swelling in the right submandibular gland. The tissue diagnosis was suggestive of squamous cell carcinoma of the lip and mucoepidermoid carcinoma of the submandibular gland. The patient underwent successful wide local excision of the lip and submandibular gland along with prophylactic supraomohyoid neck dissection. This case is reported for the rarity in site and histopathology of double malignancy.

[A case of paraneoplastic limbic encephalitis associated with neck neuroendocrine carcinoma].

A 69-year-old man presented with a history of personality change for several years. He was admitted to our hospital due to partial seizure. A cerebrospinal fluid test and an electroencephalogram showed no specific abnormalities, but brain magnetic resonance imaging revealed abnormal findings in the right temporal pole, bilateral amygdala to hippocampus, and insular cortex. He was diagnosed with limbic encephalitis accompanied by partial seizure, and received infusion of an antiepileptic agent and acyclovir. Additional examinations for malignancy and autoimmune disease were performed, and neck CT and MRI revealed a neck tumor. Neck lymph node biopsy suggested lymph node metastasis of a neuroendocrine neoplasm derived from other organs. He did not want aggressive treatment involving surgical resection and chemotherapy, and thus, conservative treatment was chosen by an otorhinolaryngologist and immunotherapy was not used. After discharge, the neck tumor grew gradually. To manage the focal mass effect, chemotherapy and surgical resection followed by chemoradiotherapy were performed by the otorhinolaryngologist on days 244 and 325 of the disease course, respectively. Histology of resected tissues disclosed neck neuroendocrine carcinoma derived from a submandibular gland. His personality change improved temporarily after surgical resection, but then worsened again with regrowth of the tumor. He died on day 723. After death, a blood test revealed the presence of anti-amphiphysin antibody. This case suggests that neck neuroendocrine carcinoma can induce paraneoplastic limbic encephalitis, and in such cases, early surgical resection of the neck tumor with suspected lymph node metastasis is necessary both to control symptoms associated with encephalitis and to exclude carcinoma derived from the neck itself.

Pleomorphic Adenoma of the Submandibular Gland: A Case Report.

Salivary gland tumours are relatively rare and constitute about 3-4% of head and neck tumours. Most of the tumours arise from parotid glands. Submandibular gland tumours are very rare. Pleomorphic adenoma of the submandibular gland is exceedingly rare tumour. Very few studies have been reported in the literature that is exclusively conducted on pleomorphic adenoma affecting submandibular gland. Patients usually present with a slow growing, painless and mobile mass without any other associated symptoms. Radiologic studies are usually unable to differentiate benign from malignant tumours in most cases. Recurrence is rare with complete en bloc excision of the tumour along with submandibular gland. Prognosis is excellent except for the rare cases of malignant transformation. This paper describes a case of pleomorphic adenoma affecting submandibular gland with brief review of current literature on submandibular gland tumours. Keywords: pleomorphic adenoma; salivary gland; submandibular gland tumours.

Long-term outcomes and prognosis in submandibular gland malignant tumors: A multicenter study.

OBJECTIVES/HYPOTHESIS: Submandibular gland tumors (SMGTs) are rare and characterized by heterogeneity in histologic profiles. The aim of the present study was to retrieve data on submandibular gland (SMG) malignancies and identify factors influencing survival. STUDY DESIGN: Retrospective study. METHODS: A multicenter, retrospective study on patients who underwent primary surgery for SMGTs at three referral centers was performed. RESULTS: Among 204 patients with SMGTs, 50 (24.5%) with SMG malignancies were analyzed in detail. Definitive pT status was: 21 (42%) pT1, 14 (28%) pT2, 10 (20%) pT3, and five (10%) pT4. Nodal metastases and perineural spread were diagnosed in 15 (30%) and 25 (50%) patients, respectively. High-grade lesions were identified in 32 (64%) patients. Follow-up status, available for 49 (98%) patients, was as follows: 23 (47%) patients were alive without disease, 11 (22.5%) died of disease, five (10.2%) alive with disease, and 10 (20.4%) died of other causes. Five- and 10-year overall survival (OS), disease-specific survival (DSS), and recurrence-free survival (RFS) were 66.4% and 57.1%, 76.6% and 72.1%, 69.1% and 62.4%, respectively. At univariate analysis, OS, DSS, and RFS were influenced by pT status, pN status, and perineural spread. OS and RFS were also affected by the presence of facial palsy and pain. RFS was negatively influenced by positive margins. Multivariate analysis confirmed that OS, DSS, and RFS were independently affected by perineural spread, whereas nodal involvement influenced only RFS. CONCLUSIONS: The malignancy rate of SMGTs is comparable to that reported for parotid tumors. Most patients presented with high-grade lesions. pT status, pN status, and perineural spread significantly affected prognosis. LEVEL OF EVIDENCE: 4 Laryngoscope, 128:2745-2750, 2018.

Pleomorphic Adenoma in a Young Female: Case Report / Adenoma Pleomórfico en una Mujer Joven: Reporte de Caso

ABSTRACT: Pleomorphic adenoma (PA) is the most common benign salivary gland neoplasm and it is frequently diagnosed in the third and fourth decade with predilection for women. PA is the most common benign lesion of minor salivary glands in children and adolescents, being the palate one of the most frequently affected site of minor salivary glands. Herein, we present a case report of a PA of the hard palate diagnosed in a 15-year-old female and a review of the Englishliterature of the reported cases of PA in children and adolescents in the hard palate.

RESUMEN: El adenoma pleomórfico (AP) es la neoplasia benigna más común de las glándulas salivales y se diagnostica frecuentemente en la tercera y cuarta década con predilección por las mujeres. El AP es la lesión benigna más común de las glándulas salivales menores en niños y adolescentes, siendo el paladar uno de los sitios más frecuentemente afectados de las glándulas salivales menores. En este trabajo se presenta un relato de caso de un AP de paladar duro diagnosticado en una mujer de 15 años de edad y una revisión de la literatura en inglés de los casos reportados de AP en niños y adolescentes en paladar duro.