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.

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.

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.

Coexistence of Lymphoepithelial Carcinoma of the Parotid Gland and Submandibular Gland Pleomorphic Adenoma.

Lymphoepithelial carcinoma is a variant of undifferentiated carcinoma with characteristic dense lymphoid stroma in which nasopharynx is site of predilection. Racial and geographic association and Epstein-Barr virus positivity in endemic areas are other characteristics of this rare neoplasm. Lymphoepithelial carcinoma accounts for only 0.4% of malignant salivary gland tumors. The authors present a patient with Epstein-Barr virus positive lymphoepithelial carcinoma of the parotid gland in a nonendemic region. Besides this, synchronous pleomorphic adenoma in the contralateral submandibular gland caused a challenge in making initial therapeutic decision.

Dermoid Cyst of the Lateral Neck Included Within the Submandibular Gland.

Dermoid cysts are benign lesions of congenital origin, and those in the head and neck region are usually present as a midline neck mass. They rarely appear in the lateral neck. This article describes the clinical presentation and histopathologic features of an extremely rare case of lateral dermoid cyst included within the submandibular gland in a 58-year-old man. The etiology of the cyst is also discussed.

Lipomas of the submandibular space: case report of a sialolipoma and review of diagnostic investigations available.

This report describes and discusses the radiologic investigations available for the investigation of fatty tumors of the submandibular triangle, with a focus on sialolipomas. A 33-year-old woman presented with a 3-year history of a slowly growing, painless mass in her right submandibular region. She underwent multiple imaging modalities before having a histologic diagnosis of a sialolipoma. To the best of the authors' knowledge, this is the first reported case using ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI) for investigation of a sialolipoma of the submandibular gland region. Ultrasound and fine-needle aspiration offer a tentative diagnosis of a lipoma without the associated radiation and cost associated with CT and MRI, respectively. CT and MRI offer the definite diagnosis of a lipoma, with MRI depicting better delineation and the ability to identify glandular elements of the tumor. Importantly, the development of sarcomatous change within the lipoma cannot be out ruled at imaging and requires a histologic specimen.

Primary Epithelioid Angiosarcoma of the Submandibular Gland-A Case Report with Histology-Cytology Correlation and Comprehensive Molecular Analysis.

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.

Conventional MRI findings for predicting submandibular pleomorphic adenoma.

BACKGROUND: Although pleomorphic adenomas account for over 90% of all benign submandibular gland tumors, the imaging features of submandibular pleomorphic adenomas have not been reported in a large number of cases. PURPOSE: To assess the conventional magnetic resonance imaging (MRI) findings for predicting the submandibular pleomorphic adenoma. MATERIAL AND METHODS: MR studies of 42 pleomorphic adenomas and 28 other types of tumor were reviewed. MR images were assessed for the presence of hyperintense areas on T2-weighted images (first sign), a well-defined margin (second sign), and presence of crescent-shaped compression of the ipsilateral normal submandibular gland (third sign). RESULTS: For identifying submandibular pleomorphic adenoma, the sensitivity, specificity, positive predictive value, negative predictive value, and accuracy were 97.6%, 50.0%, 74.5%, 93.3%, and 78.6% for the first sign, 95.2%, 46.4% 72.7%, 86.7%, and 75.7% for the second sign, and 23.8%, 100%, 86.7%, 46.7%, and 54.3% for the third sign, respectively. Combining the first and second findings achieved to 85.7% specificity and 90.9% accuracy. CONCLUSION: Although non-specific, submandibular pleomorphic adenomas usually have hyperintense areas on T2-weighted images and well-defined margins. In addition, crescent-shaped compression of the ipsilateral normal gland seems to represent a highly specific sign.

Pleomorphic adenoma occurring outside the submandibular gland: a case report of an accessory submandibular gland.

The accessory submandibular gland is a rare anatomic variant and the incidence of pathology reported within an accessory submandibular gland is even rarer. This report describes the case of a 22-year-old woman who presented with a slowly enlarging mass in the submandibular triangle, which on ultrasound examination suggested that it was close to, but not arising from, the submandibular gland. Fine-needle aspiration cytology was consistent with a pleomorphic adenoma. At surgery, the tumor was found to be entirely separate from the submandibular gland. This case presents an extremely rare occurrence of an accessory submandibular gland and, to the authors' knowledge, is the first report of a pleomorphic adenoma occurring within an accessory submandibular gland.

Submandibular gland excision: long-term clinical outcome in 139 patients operated in a single institution.

In transcervical resection of the submandibular gland for benign lesions, only a limited risk of damage to neural structures can be accepted and a cosmetically satisfactory result is mandatory. In this retrospective case series, we evaluated 139 patients operated over a 10-year period and completed long-term clinical follow-up of 113 of these patients after a median of 81 months. In all patients, the operation was effective. We found a 4.3 % risk of reoperation for wound infection or postoperative hematomas and an 18.7 % risk of early paresis of the marginal branch of the facial nerve, which decreased to 2.7 % on long-term follow-up. We found a 4.4 % risk of permanent lingual nerve paresis, and no patients had damage to the hypoglossal nerve. Xerostomia was found in 22.1 % of the patients and could be quantified by the easily performed biscuit test. Only 2.5 % reported an unsatisfactory cosmetic result and all scars were ≤ 6 on the Vancouver Scar Scale. Problems with scarring were more common if there had been postoperative infection. We continue to use the lateral transcervical approach as standard in our institution for patients who cannot be managed by gland-sparing procedures.

Submandibular triangle masses.

OBJECTIVE: The study aims to analyze the demographic data of a large case series operated on because of submandibular triangle mass for more than 10 years and presents a review of the literature. MATERIALS AND METHODS: The charts of patients who underwent surgical intervention for submandibular triangle mass between January 2000 and November 2010 were reviewed. The medical history, age, sex, duration of symptoms, clinical presentation, preoperative investigations, and histopathologic diagnosis were reviewed. RESULTS: The study included 66 subjects; 12 patients (18.2%) with submandibular sialolithiasis, 18 patients (27.2%) with sialadenitis, 10 patients with lymphadenitis (15.1%), and 26 patients (39.3%) with tumors. Of the tumors, 23% was malignant and 77% was benign. Benign tumors of submandibular gland comprised 22.7% and malign tumors of submandibular gland comprised 3% of all submandibular mass. The most common benign tumor was pleomorphic adenoma. The most frequent histopathologic diagnoses of submandibular masses originated from the submandibular gland, and these comprised 71.2% of all submandibular mass pathologies. The main symptom was a painless mass. Ultrasonography was the most common preoperative diagnostic procedure. Fine-needle aspiration biopsy was performed in 26 patients. A clear diagnosis could not be provided in 3 (12%) patients. CONCLUSION: Infectious conditions and benign tumors are more frequent than malign tumors in the submandibular region. The histopathologic diagnoses mainly consisted of submandibular sialadenitis, sialolithiasis, pleomorphic adenoma, and lymphadenitis. Ultrasonography is the first option of radiologic evaluation. Fine-needle aspiration biopsy is a very useful and usually sufficient diagnostic procedure for histopathologic diagnosis. Excisional biopsy can be performed when the fine-needle aspiration biopsy failed.

Warthin tumor in an unusual site: a case report.

Warthin tumor (papillary cystadenoma lymphomatosum, adenolymphoma) is a benign neoplasm from salivary glands and is almost restricted to the parotid gland and the periparotid lymph nodes. Its etiopathogenesis has been associated with tobacco smoke. Several authors believe that the tumor develops from heterotopic salivary gland tissues or through the attraction of a heavy lymphoid reaction. Most cases involve the lower pole or the tail of the parotid. Since its description, a few cases have been reported in other sites, like the submandibular salivary gland. This study aimed to describe a rare presentation of a Warthin tumor and discuss its etiology and differential diagnosis. A 72-year-old male patient presented an asymptomatic, nodular mass adjacent to the submandibular salivary gland. Based on clinical diagnosis of cervical lymphadenopathy, the patient underwent a surgical excision of the lesion. Histopathological exam revealed a benign neoplasm from salivary glands composed of two components: epithelial and lymphoid. The findings were consistent with Warthin tumor. Warthin tumor from submandibular salivary gland is a rare lesion. Professionals should not misinterpret their site of origin, as if they were from the lower pole or the tail of the parotid.

Pulmonary blastoma with submandibular, scrotum and adrenal metastases: case report.

A 62-year-old patient diagnosed with pulmonary blastoma with submandibular, scrotum and adrenal metastases was admitted to Sotiria General Hospital in Athens. No other such case has been published to date. The patient started receiving chemotherapy, but the scrotum metastasis grew rapidly and erupted. This led to sepsis despite surgical excision of infected and necrotic tissues and intravenous antibiotics. Treatment strategy in pulmonary blastoma should be defined by a multidisciplinary team, and surgical treatment should be considered as quickly as possible when such a tumor is suspected.

Bilateral carcinoma in situ of Wharton's duct after chronic obstructive sialadenitis: inflammation as the cause of malignancy?

A case of bilateral carcinoma in situ of Wharton's duct after chronic sialadenitis is reported. The patient, a 54-year-old man, complained of recurrent pain and swelling in the left lower submandibular region. Computed tomography showed large stones in the hilar area of both submandibular glands. The patient underwent bilateral submandibular excision. Histologic and immunohistochemical examination revealed squamous metaplasia with areas of carcinoma in situ in both right and left ducts adjacent to the calculus. To the best of our knowledge, this is the first case report in the literature describing an association between obstructive sialadenitis and carcinoma in situ of Wharton's duct. We discuss etiologic factors and chronic inflammation as a possible cause of malignancy.

Cytopathologic analysis of stroma-poor salivary gland epithelial/myoepithelial neoplasms on fine needle aspiration.

OBJECTIVE: Fine needle aspiration (FNA) diagnosis of salivary gland neoplasms with epithelial/myoepithelial cells but rare or no stroma is usually difficult. Our aim was to study the cytomorphology of this cohort of FNA cases and evaluate the clinical follow-up. STUDY DESIGN: The diagnostic terminology for this group of aspirates was 'favor an epithelial/myoepithelial neoplasm of the salivary gland'. The cytologic smears of 32 such cases were retrieved and showed cellular smears with bland-appearing or mildly atypical epithelial and myoepithelial cells without typical chondromyxoid stroma seen in pleomorphic adenoma (PA). RESULTS: Twenty of the 32 cases had histologic follow-up. Ten of these 20 cases were PAs, including 8 cellular PAs. Two cases were basal cell adenomas, 1 case myoepithelioma and 1 case benign adenoma, not otherwise specified. Among the 6 malignant tumors on surgical resections, there were 3 epithelial-myoepithelial carcinomas, 1 myoepithelial carcinoma, 1 basal cell adenocarcinoma and 1 adenoid cystic carcinoma. CONCLUSIONS: Although 14 of the 20 (70%) cases were benign neoplasms, a substantial amount of cases (30%) were malignant salivary gland neoplasms. The generic diagnostic terminology of 'epithelial/myoepithelial neoplasm of the salivary gland' and appropriate clinical follow-up are recommended for these cases.

Diagnosis of submandibular gland tumors: a comparison of CT and ultrasound.

OBJECTIVES: This study aims at determining whether CT is superior to ultrasound in providing more accurate information in the investigation of submandibular gland tumours. MATERIALS AND METHODS: A retrospective study was carried out between 2005 and 2010. Thirty-five (35) patients, who presented with submandibular gland swelling and had diagnostic ultrasound or CT/MRI prior to surgery and histological examination of tissues removed at surgery were investigated to determine the correlation between radiologic diagnosis and histologic diagnosis. RESULTS: CT was used in the pre-op assessment of 22 patients, 12 of which had correct diagnosis when compared with the histology findings while ultrasound was used in pre-op assessment of 13 patients, 12 of which concurred with the histological diagnosis. With regards to ability to detect malignant lesion, ultrasound had much higher sensitivity than CT and comparable specificity. Therefore, in this study, ultrasound provided a better guide in the pre-op radiologic assessment of submandibular gland swelling. CONCLUSION: Our evaluation of pre-operative diagnosis of CT and ultrasound in the assessment of submandibular swellings reveal that ultrasound is better in determining the nature of the lesion.

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.

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.

Submandibular gland MALT lymphoma associated with Sjögren's syndrome: case report.

Lymphoma is a common disease of the head and neck. Mucosal-associated lymphoid tissue (MALT) lymphoma constitutes a rare type of extranodal lymphoma. The Waldeyer's ring is one of the most common sites of occurrence, but MALT lymphoma may also arise in salivary glands, lung, stomach, or lacrimal glands. In the oral cavity, it may be confused with swellings from dental infection or sinus inflammation. Often, the patient will seek a dentist because of mobile teeth or because a denture no longer fits. We report a case of a female patient with salivary gland dysfunction and pain of several years' duration, who, after numerous tests and hospitalizations, was diagnosed with Sjögren's syndrome. She later developed mucosal-associated lymphoid tissue lymphoma. We discuss the diagnosis, treatment, and prognosis of this entity. MALT lymphoma is rare in salivary glands. In primary-Sjögren's syndrome, predisposition of the patient for development of malignant non-Hodgkin's lymphoma (4% to 10%) is well established. In this case, long-standing sialadenitis and Sjögren's syndrome seem to be the etiological factors. In cases of chronic infection of salivary glands and the presence of autoimmune syndromes, MALT lymphoma should be considered in the differential diagnosis. Consults should be called to ophthalmology, rheumatology, and head and neck oncologists for proper workup, staging, and treatment.