Angiosarcoma of the submandibular salivary gland: case report and review of the literature

OBJECTIVES: The purpose of this report is to describe an example of angiosarcoma (AS) of the submandibular salivary gland. The clinical and immunohistopathologic features of these lesions are also reviewed. MATERIAL AND METHODS: A 17-year-old male high school student was admitted to our clinic with a 1-year history of a slow-growing, tender mass in the left submandibular region. Physical examination on initial presentation revealed a diffuse soft tissue mass 6 cm in diameter involving the left submandibular region. MRI analysis revealed a mass located superolateral to the submandibular salivary gland, measuring 4.0 x 2.0 cm2. The mass was excised completely together with the left submandibular salivary gland. Histopathologic analysis led to a diagnosis of AS. Immunohistochemical studies were also used to determine endothelial cell differentiation. Owing to the lateralized nature of the lesion, a left functional neck dissection was performed and postoperative radiotherapy was planned. RESULTS: AS is a malignant tumor of endothelial cell origin that may occur in any region of the body. The commonest sites include the extremities and the retroperitoneal space, with only 4% of AS tumors arising in the head and neck area. The submandibular salivary gland is an extremely rare location for this tumor. Based on a literature review, this case report represents only the second reported case of AS of the submandibular salivary gland. CONCLUSIONS: In most cases, radical surgery and postoperative radiotherapy are advocated to treat patients with AS tumors, with lymph node clearance recommended in cases of lateralized lesions In some patients, distant metastasis may occur after many years, which necessitates long-term follow-up. The prognosis is poor in most cases of AS.     

Large cell neuroendocrine carcinoma of the submandibular gland: Case report and literature review

Large cell neuroendocrine carcinoma (LCNEC) of the salivary gland is extremely rare. We report on a case of LCNEC in the submandibular gland. A 58-year-old male had a four-month history of an enlarging mass in his left submandibular region. He underwent lymph node resection and metastasis of LCNEC was suspected. Magnetic resonance imaging of the neck showed a solid submandibular gland tumor with marginal blurring. Positron-emission tomography and upper gastrointestinal endoscopy showed no evidence of malignancy other than in the left submandibular gland. He underwent left submandibular gland resection and left upper neck dissection. The final diagnosis was LCNEC of the submandibular gland; surgical margin was negative. Fourteen months later he is free of tumors. This is the first report of LCNEC of the submandibular gland. LCNEC of the salivary gland shows high-grade malignancy like that of the lung. According to past reports, two of four patients died despite multidisciplinary treatments. There are no standard treatments for LCNEC of the salivary glands. More studies are needed to define prognostic factors and establish therapeutic methods.     

A case of bilateral aplasia of the submandibular glands associated with a unilateral submandibular hemangioma

Congenital aplasia of the major salivary gland is a rare condition. We report on a case of bilateral aplasia of the submandibular glands associated with a left submandibular hemangioma. A 62-year-old woman came to our department complaining of a 3-year history of left submandibular swelling. She had no notable family or personal medical history. On physical examination of the head and neck region, a 30 mm, non-tender mass was palpated in the left submandibular area. On imaging examinations including ultrasonography and CT, lack of the right submandibular gland and a left submandibular mass with calcification were demonstrated. Fine needle aspiration cytology resulted in blood elements only. Functioning tissue could not be observed in the bilateral submandibular glands on technetium pertechnetate scintigraphy. We performed a left submandibular tumor extirpation. Intraoperatively, the left submandibular gland and duct were missing. On pathologic examination, the tumor was found to be a hemangioma. The parotid and sublingual glands were recognized bilaterally on postoperative MRI. Her postoperative condition was satisfactory. This condition may be due to the dysfunction of several factors, such as fibroblast growth factors, related to gland differentiation.     

Malignant salivary gland tumors: squamous cell carcinoma of the submandibular gland in a child.

PURPOSE: Malignant neoplasms of the salivary glands are extremely unusual in the pediatric age group. METHODS: We report an 11-year-old boy who presented with a mass in the left submandibular region and a second mass in the jugularfacial venous angle. RESULTS: Histologic evaluation determined that this was a squamous cell carcinoma of the submandibular gland with metastasis to a cervical node. Review of the literature was undertaken to identify the rate of malignant salivary gland tumors in children. Malignant salivary gland tumors are extremely rare in children. When malignancy does occur, nearly 90% are present in the parotid gland. The submandibular gland is effected in 7.7% of cases. The most common malignancy in salivary glands of children is the mucoepidermoid carcinoma. Squamous cell carcinoma occurs in less than 2% of cases. CONCLUSIONS: Salivary gland tumors are very rare in children in contrast to adults. The proportion of malignant tumors in children is higher than that observed in adults; however, it is not possible to draw conclusions concerning treatment from the few case reports that exist. A higher rate of local recurrence and cervical lymph node metastasis may be expected in children.     

Large-cell undifferentiated carcinoma of the submandibular gland

Large-cell undifferentiated carcinoma (LCUC) arising in the submandibular gland is so rare that there have been only a few reported cases. We encountered a 65-year-old Japanese male, whose left submandibular gland was enlarged due to LCUC. Exenteration of the left submandibular gland together with selective neck dissection was performed, followed by postoperative radiotherapy. Current literature concerning the histopathological and clinical features of this neoplasm was reviewed.     

Anatomic study of submandibular gland transfer in an attempt to prevent postradiation xerostomia

Xerostomia is the most common negative sequela after radiotherapy for head and neck cancer and results from destruction of normal salivary gland tissue by radiation. We hypothesized that transferring a single submandibular gland outside the radiation field to the submental region may preserve its function, thus preventing xerostomia and its consequences. This anatomic study was designed to evaluate different methods of submandibular gland transfer to the submental region. Eight submandibular glands were dissected in four fresh human cadavers. The anatomy and blood supply of the region were documented. Each gland was transferred to the submental region by either free microvascular transfer or based on retrograde flow through the distal facial vessels. Both methods of transfer resulted in complete relocation of the submandibular gland into the submental region.     

Significance of Unilateral Submandibular Gland Excision on Salivary Flow in Noncancer Patients

Objectives/Hypothesis: Normal salivary flow is important for maintaining good oral hygiene. Lack of normal flow predisposes to an increased incidence of dental caries and symptomatic xerostomia. The submandibular glands are responsible for 70% of resting salivary flow. Removal of the submandibular gland is most commonly performed for sialolithiasis, chronic sialoadenitis, or as part of a neck dissection. The effect of unilateral gland resection for benign disease on resting salivary flow has not been well examined in the literature. Study Design: Case controlled study involving patients who had undergone a unilateral submandibular gland resection matched with normal control subjects. Method: Stimulated and unstimulated salivary flow rates in surgical and control subjects were measured in a controlled setting. Results: Unstimulated salivary flow rates were 0.805 mL/min in the control group and 0.405 mL/min in the surgical group (P = .01). Stimulated salivary flow rates were not significantly different between the two groups (P > .05). Fifty-seven percent of patients in the surgical group and 14% in the control group had xerostomia (P < .05). Conclusion: Unilateral submandibular gland excision results in a decreased rate of resting salivary flow and an increase in subjective xerostomia.     

Sialoblastoma: A very rare cervical mass in neonates: A case report

Sialoblastomas are very rare epithelial tumors of the salivary gland that usually occur in the parotid or submandibular gland. Most sialoblastomas present in the neonatal period or early childhood. The biological behavior of these tumors is variable, with a potential for local and systemic recurrences. Only ten cases in the submandibular gland have been reported so far. We are reporting a case of sialoblastoma in a newborn male child presenting in the right submandibular region. Ultrasound visualized a homogeneous solid mass. MRI showed a well-defined soft tissue solid mass in right submandibular region. Alpha fetoprotein was 18.8 ng/ml (normal 0.4–15). Fine needle aspiration showed cylindrical connective tissue with groups of basilar cells. Mitosis was not seen. The most probable diagnosis was sialoblastoma.     

Ectopic submandibular gland presenting as a swelling in the floor of mouth

The authors describe a patient who presented with a left sublingual swelling. Ultrasonic and sialographic investigation showed no salivary tissue in the expected left submandibular region, but a large salivary gland in the left floor of the mouth deep to the mylohyoid muscle. This is likely to represent an ectopically positioned submandibular gland.     

Risk of nodal metastases from malignant salivary gland tumors related to tumor size and grade of malignancy

Indications for elective treatment of the neck are not well defined in salivary gland tumors. We retrospectively reviewed 153 cases of malignant salivary gland tumors treated from 1965 to 1985 at the Hospital de Oncología, Mexico City. There were 106 parotid cancers, 26 in the submandibular gland and 21 in minor salivary glands. Median follow-up was 48 months. In T1-2 tumors there was a 12% incidence of nodal metastases as compared with 27% in T3-4 cancers (P = 0.01). Thirty-six elective neck dissections were performed. Patients with high-grade tumors had an increased risk (50%) of occult node metastases, while no cases were found in low-grade carcinomas (P < 0.05). The risk of neck recurrence was higher in N+ (23.5%) than in NO patients (3.2%). The 5-year actuarial survival was significantly better in lowgrade tumors (78%), T1-2 tumors (85%) and negative nodes (63%) than in high-grade neoplasms (49%), T3-4 tumors (35%) and positive nodes (36%) (P = 0.001, P = 0.001 and P = 0.04, respectively).Presented at the combined meeting of the Society of Head and Neck Surgeons and the European Organization for Research and Treatment of Cancer, Paris, France, 25–28 May 1994     

Submandibular gland transfer: a new method of preventing radiation-induced xerostomia

OBJECTIVE: Radiation-induced xerostomia is a significant morbidity of radiation therapy in the management of patients with head and neck cancers. We have recently reported a method of transfer of one submandibular gland to the submental space in a small pilot series of eligible surgical patients. The submental space was shielded during postoperative radiation therapy. The transferred gland continued to function after the completion of radiation therapy and none of the patients developed xerostomia. The purpose of this article is to present the technique of submandibular gland transfer in detail and to evaluate the postoperative survival and function of the transferred submandibular glands. DESIGN: Prospective clinical trial. METHODS: The submandibular gland was transferred on eligible patients as part of their surgical intervention. The patients were followed clinically, with salivary flow and radioisotope studies. RESULTS: We performed the surgical transfer of the submandibular salivary gland in 24 of 25 patients placed on the protocol. All the glands survived transfer and functioned well postoperatively as demonstrated on the salivary flow and the radioisotope studies. The surgical transfer was relatively simple and added 45 minutes to the surgical procedure. There were no complications attributed to the submandibular gland transfer. CONCLUSIONS: We have successfully demonstrated that the submandibular gland can be surgically transferred to the submental space with its function preserved. The gland seems to continue functioning even after radiation therapy with the appropriate shielding. This surgical transfer procedure has the potential to change the way we currently manage patients with head and neck cancer.     

Metastasis in submandibular gland from a leiomyosarcoma of the uterus

Two general types of metastases should be distinguished in metastatic salivary gland tumors: one of them are tumors originating in the head and neck region, and the other are tumors from distant tumor sites. Distant metastasis affecting the submandibular gland are a rare entity. We report a case of the uterus leiomyosarcoma which metastatised in the submandibular gland.     

The quantitative analysis of the human papillomavirus DNA load in submandibular gland lesions with droplet digital polymerase chain reaction

Background: The relationship between infection with human papillomavirus (HPV) and tumorigenesis of salivary gland remains controversial.

Objectives: This study explored the relationship between HPV and salivary gland lesions as well as that of the HPV infection status and p16^INK4A immunoreactivity. The HPV DNA loads were also quantitatively evaluated.

Materials and Methods: Tissue samples from 31 submandibular gland lesions were evaluated. p16^INK4A immunohistochemical (IHC) staining, nested polymerase chain reaction (PCR), DNA sequencing, and droplet digital PCR (ddPCR) were performed.

Results: Non-neoplastic lesion, benign tumors, and malignant tumors were noted in 9, 16, 6 cases, respectively. p16^INK4A immunoreactivity was higher in malignant tumors than in benign tumors (50.0% vs. 6.3%). Single PCR with MY09/11 found that all samples were negative. Nevertheless, nested PCR revealed a high HPV-DNA positivity rate of 96.8%. No relationship between the HPV status and p16^INK4A immunoreactivity was shown. HPV-18 was the only subtype identified in this study. ddPCR showed significantly lower HPV-18 DNA loads in submandibular gland lesions than in oropharyngeal cancers.

Conclusions: HPV-DNA positivity and p16^INK4A-immunoreactivity were not correlated in submandibular gland lesions. The loads of HPV DNA detected in this study were small. HPV positivity therefore may not be associated with tumorigenesis of the submandibular gland.     

Speichelgangkarzinome der Glandula submandibularis

BACKGROUND: Salivary duct carcinoma (SDC) is a rare adenocarcinoma of the salivary glands which usually occurs in the parotid gland (approx. 80%), but is also found in the submandibular gland (approx. 12%) and the minor salivary glands. Due to the low incidence of this tumour-particularly in terms of involvement of the submandibular and minor salivary glands-only limited clinical data is available. PATIENTS/METHOD: In a retrospective investigation, follow-ups of six patients with a SDC of the submandibular gland were analysed and the surgical measures evaluated for their efficiency. RESULTS: At the time of diagnosis, varying tumor stages were present (2xT1, 3xT2, 1xT1). Initial lymph node metastasis was observed in four (66.6%) of the six patients. In all patients, the submandibular gland was resected and neck dissection performed, in two cases (33,3%) this was followed by radiotherapy. Four patients developed a life-threatening local recurrence. Only one patient developed distant metastasis. Two thirds of the patients died due to tumor-related disease after an average of 35.5 months. CONCLUSION: The clinical course of patients with SDC of the submandibular gland exhibits a high (66.6%) rate of local recurrence, crucial for the following clinical course. The treatment of these patients, therefore, must include radical tumor resection as well as tissue resection with a wide margin.     

Studies on the regranulation of salivary gland serous cells. An electron microscopical study in the rat.

The degranulation and regranulation process was investigated after alpha-adrenergic stimulation on the rat submandibular gland. The submandibular gland in rat contains both serous and mucous cells. It has earlier been shown that serous cells filled with heavy-metal granules, are markedly more radiosensitive than cells without granules. In experiments with emptying the serous cell of their content of granules by administering an alpha-adrenergic stimulant, cyclocytidine, there has been found a decrease in irradiation damage in salivary gland tissue after irradiation. Injection of cyclocytidine, 150 mg/kg, was given i.p. to the rat. After 1 h there was almost complete depletion of granules in the serous cells, no morphological aberration was seen in the mucous cells. This effect still remained after 6 h. A beginning of regranulation with apical granules was seen 12 h after injection. After 24 h an almost complete regranulation had occurred in the salivary gland serous cells. The mucous cells did not show any morphological aberration. Our intent is to reduce unwanted salivary gland damage in patients with head and neck cancer when treated with radiotherapy. Depletion of heavy-metal granules in serous cells, before irradiation may diminish morphological destruction in the salivary glands. As a nearly total degranulation is present between 1-6 h after stimulation, this should be the optimal time for radiotherapy.     

A rare malignancy of the parotid gland: oncocytic carcinoma

Oncocytic carcinomas are uncommon; they represent 11% of all oncocytic salivary gland neoplasms, 0.5% of all epithelial salivary gland malignancies and 0.18% of all epithelial salivary gland tumors. A 65-year-old woman was admitted to our ENT clinic with a history of a painless left preauricular mass. No lymph node was palpable on either side of her neck. Computed tomography demonstrated a 3×3-cm solid lesion in the left parotid gland. As oncocytes and atypical cells were seen at the cytologic examination of a fine-needle aspiration biopsy, this finding suggested a malignant epithelial tumor. Total parotidectomy by preserving the facial nerve was performed. The result of the pathologic examination was oncocytic carcinoma regarding histopathologic and immunohistochemical findings and especially the invasive growth pattern. We preferred a close follow-up to an elective neck dissection, and reserved the neck dissection for a recurrence. The prognosis of oncocytic carcinomas is not well known because of their low incidence. Further investigation of the prognosis of patients with oncocytic carcinoma of the parotid gland is warranted as more cases are reported.     

Sialoblastoma: a rare submandibular gland neoplasm

A sialoblastoma is a rare congenital epithelial tumor that arises in a major salivary gland. To our knowledge, only 24 cases of sialoblastoma have been previously reported in the English-language literature. We report a new case, that of a 15-month-old boy who presented with a submandibular mass. Surgical excision of the mass was undertaken. Intraoperatively, the mass appeared to be adjacent to the submandibular gland, but it had not invaded it. The mass was excised, and the submandibular gland was left in place. Pathology identified the tumor as a sialoblastoma. However, pathology also revealed that residual tumor was present at the surgical margin. The patient was returned to the operating room for excision of the left submandibular gland and the level I lymph nodes. Following revision surgery, the surgical margins were negative. The patient remained disease-free at the 1-year follow-up. Despite the need for revision surgery, this case provides support for the idea that surgery alone is sufficient for curative treatment.     

Sensory preservation in neck dissection: outcomes of a sub-sternocleidomastoid approach

Objective: Cutaneous anesthesia in early postoperative period is common after neck dissection even if the cervical nerve (CN) rootlets are preserved. The aim of this study was to evaluate if the preservation of the terminal branches of CNs using sub-sternocleidomastoid (SCM) approach combined with medially placed skin incision can prevent early postoperative anesthesia.

Material and methods: A retrospective chart review was performed on 129 neck dissections in 87 head and neck cancer patients.

Results: The early postoperative sensory preservation rates for the ear tab, submandibular, lateral neck, and sub-clavicular areas of CN rootlet-preserved necks (n?=?86) were 75.6%, 20.9%, 74.4%, and 86.0%, respectively, compared with 37.2%, 2.3%, 2.3%, and 4.7%, respectively, in CN rootlet-resected necks (n?=?43). In CN rootlet-preserved necks, the sub-SCM approach (n?=?54) showed 81.5%, 27.8%, 92.6%, and 94.4% preservation rates, respectively, compared with 65.6%, 9.4%, 43.8%, and 71.9%, respectively, using the conventional subplatysmal approach (n?=?32). The rates were significantly better in the submandibular, lateral neck, and sub-clavicular areas after sub-SCM approach.

Conclusions: Preservation of CN rootlets is a required element for sensory preservation in neck dissection. The sub-SCM approach can effectively prevent early postoperative cutaneous anesthesia following CN-preserving neck dissection.     

Tumors of the major salivary glands.

Tumors of the major salivary glands are reviewed according to classification, location, surgical procedure and end results. Our data of the incidence of benign and malignant tumors show that the most commonly involved area is the parotid gland and the most frequent is of the mixed variety. In the parotid region 80 percent are benign and 20 percent are malignant; whereas, in the submandibular gland, the malignant and benign tumors are equally distributed. The need for an extensive surgical attack and inclusion of contiguous structure is dictated by the nature of the malignant disease. The role of postoperative irradiation is discussed as is the indication for neck dissection. Management of the facial nerve, relative to malignant tumors of the parotid gland, is considered in detail.     

Spontaneous regression of lung lesions after excision of the submandibular gland in a patient with chronic sclerosing sialadenitis

Chronic sclerosing sialadenitis, which clinically resembles a salivary gland tumor, is considered a salivary gland lesion of the IgG4-related inflammatory disease. Corticosteroids are effective for the treatment of chronic sclerosing sialadenitis, but the natural clinical course of this disease is not fully understood. We report a 67-year-old man with chronic sclerosing sialadenitis who showed lung lesions, which spontaneously regressed without steroid therapy after excision of the submandibular gland. He presented with several weeks' history of continuous swelling in the right submandibular region. Physical examination showed a hard swelling of the right submandibular gland; the cytological finding was Papanicolaou class III. A chest X-ray demonstrated bilateral infiltration of the right middle and left lower lobes. The right submandibular gland was excised under general anesthesia for definite diagnosis, and histopathological examination revealed an IgG4-positive plasmacytic infiltration accompanied by periductal fibrosis and interlobular sclerosis, consistent with chronic sclerosing sialadenitis. Five months after operation, the lung lesions regressed spontaneously.