Учебники / Textbook and Color Atlas of Salivary Gland Pathology - DIAGNOSIS AND MANAGEMENT Carlson 2008

206 Tumors of the Submandibular and Sublingual Glands

Figure 9.8f. Surgical specimen.

In undertaking the level I dissection the cervical skin flap is lifted in a subplatysmal plane and the marginal mandibular branch of the facial nerve is preserved. The anterior belly of the digastric muscle is identified and its fascia dissected free. The fascia is dissected off the mylohyoid muscle, freeing the fat and nodes from the digastric, mylohyoid, and inferior border of the mandible. The posterior edge of the mylohyoid muscle is retracted to identify the lingual nerve, which is preserved if it is uninvolved by cutting the branch to the gland and allowing the nerve to retract into the mouth. The duct is sectioned and the facial vein and artery tied off or dissected free as indicated to release the specimen. In low-grade N0 tumors a neck dissection is usually not indicated, but the excision can be extended to encompass lymph node levels II and III as a supraomohyoid neck dissection. If lymph nodes are clinically involved, then a type I modified radical neck dissection is required.

In high-grade tumors or advanced low-grade tumors with extracapsular infiltration and involvement of the skin, muscle, or mandible extended resections with incorporation of these structures will be necessary to obtain clear margins. These resections will be dictated by the size and extent of the tumor. In N0 cases selective neck dissection levels I-III or I-IV will be used and modified radical neck dissections for clinically positive necks. In the case of ACC, widespread infiltrative growth beyond the palpable tumor makes obtaining clear margins challenging. The propensity for perineural inva-

sion with ACC will necessitate sacrifice of involved nerves, for example, lingual, hypoglossal, and facial, tracing the nerves proximally using frozen section guidance to determine clearance. Unfortunately, “skip” metastases can occur along the nerve and a negative frozen section is no guarantee of success. ACC is more prone to metastasize hematogenously than through lymphatics such that a selective neck dissection is usually sufficient.

Postoperative radiation therapy is administered for high-grade tumors, positive margins, positive nodes, and perineural spread if reresection is not possible. Chemotherapy has not been shown to improve survival in salivary gland cancer.

Prognosis will depend on the histologic grade and the stage of the tumor. Some authors (Anderson, Thrkildsen, and Ockelman et al. 1991) have found a crude 10-year survival of 50% with 10% local recurrence and 39% of cases having metastasized at the time of diagnosis. In the series reported by Rapidis et al. (2004), 8 of 14 patients died during follow-up with a survival rate of 38.5%, but 11 of 14 of these patients presented with stage III or IV disease. Bhattacharyya (2004) analyzed 370 cases of submandibular gland cancer from the SEER database and reported a 59.7% 5-year survival; however, this figure is high, as 42.2% of his cases were ACC with a mean survival of 99 months. In the same series the patients with squamous cell carcinoma had a mean survival of 52 months. Younger age, low-grade histology, and the use of radiation therapy were factors in improving survival. Weber, Byers, and Petit et al. (1990) found a 69% 5-year survival, with extracapsular infiltration and lymph node metastases indicating a poor prognosis. Stages TI-TIVA had a case-specific 5-year survival of 88% compared to 55% for T4B, and 5-year survival of 86% for negative nodes compared to 30% for positive nodes.

Most published series have found a survival benefit conferred by radiation therapy, with 75% of patients receiving adjuvant radiation in one study (Camilleri et al. 1998). Storey, Garden, and Morrison et al. (2001) report actuarial locoregional control of 88% at 5 and 10 years; however, the corresponding disease-free survival rates were 60% and 53% due to 36% of patients with locoregional control developing distant metastases.

Figure 9.9a. Submandibular gland attached only by its duct. The pleomorphic adenoma is large and hangs beneath the gland attached only by the enveloping fascia of the capsule.

Figure 9.10a. 82-year-old man with pain and swelling of the right submandibular region.

Figure 9.9b. Surgical specimen with arrows pointing to the large PA, which has no real attachment to the gland.

Figure 9.10b. CT scan confirms a submandibular gland mass diagnosed as malignant on fine needle aspiration biopsy.

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c

d

Figures 9.10c and 9.10d. Intraoperative figures showing level I excision combined with a selective neck dissection in view of high-grade cytology.

Figure 9.10e. Operative specimen.

f

g

Figures 9.10f and 9.10g. Hematoxylin and eosin stain (f) and Alcian Blue stain (g) confirm a diagnosis of high-grade mucoepidermoid carcinoma.

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Figure 9.10h. One year postoperative view of the patient. He died 3 years postoperatively with distant metastases of the lung.

Nonetheless the median survival time for patients with locoregional control was 183 months compared to 19 months for those patients without locoregional control. In a similar study of adjuvant radiation therapy, cancer-specific survival was 79% and 57% at 5 and 10 years, with local control of 85% and 74%, respectively. Twenty percent of patients (all ACC) developed distant metastases (Sykes et al. 1999). A retrospective study of 87 patients that compared patients who received either initial enucleation of the gland (subcapsular dissection) with no evidence of residual primary or nodal disease followed by postoperative radiation therapy; or who had evidence of gross residual primary or nodal disease, grossly positive margins, or piecemeal removal following initial treatment who underwent definitive surgical resection followed by postoperative radiation therapy, found no difference in locoregional control, diseasespecific survival, or overall survival (Kaszuba,

Zafero, and Rosenthal et al. 2007). This suggests patients without evidence of gross residual disease post-enucleation might be satisfactorily treated with radiation therapy without further surgery.

In a series of 22 patients with ACC of the submandibular gland, disease-free survival at 5 years of 57% and 10 years of 41% and overall survival of 70% and 37%, respectively, were found (Cohen, Damrose, and Huang et al. 2004). These authors concluded that early diagnosis, wide surgical intervention, and postoperative radiotherapy were associated with a favorable prognosis, while not surprisingly large tumor size, positive surgical margins, perineural invasion, and local recurrence were negative prognostic factors.

In comparing submandibular gland cancers to parotid cancer a poorer overall prognosis was associated with submandibular gland tumors (Hocwald, Korkmaz, and Yoo et al. 2001). In addition, the likelihood of developing distant metastasis is greater in the submandibular gland than the parotid (Schwenter et al. 2006); however, this may be due to the higher percentage of ACC. In one large series of 370 cases only 12 (3%) presented with distant metastases, but 24.9% were found to have positive regional nodes (Bhattacharyya 2004). Interestingly, in this retrospective review extraglandular extension and nodal positivity did not affect survival. Goode, Auclair, and Ellis (1998) in a study of 234 cases of MEC of the major salivary glands stated that MECs with equal histopathologic grade had a better prognosis when their tumors were in the parotid gland rather than in the submandibular gland. This finding was confirmed in a Swedish study of 2,465 major salivary gland tumors (Wahlberg, Anderson, and Bjorklund et al. 2002). High-grade MEC is also more common in the submandibular gland, with 32 of 82 cases (39%) occurring in this gland (Bhattacharyya 2004).

SUBLINGUAL GLAND TUMORS

Virtually all sublingual gland tumors will be malignant and as for all salivary tumors, primary surgery is the treatment of choice. Prognosis will be determined by the histologic grade and the stage of the tumor. In the very rare benign tumor or with small low-grade carcinomas a transoral wide local resection may be successfully performed (Blanchaert, Ord, and Kumar 1998). This will be easier to undertake in edentulous patients, and Wharton’s

210 Tumors of the Submandibular and Sublingual Glands

Figure 9.11. Delivering sublingual gland with low-grade malignant tumor via an intraoral wide local excision. Republished with permission from Blanchaert RG, Ord RA, Kumar D. 1998. Polymorphous low-grade adenocarcinoma of the sublingual gland. Int J Oral Maxillofac Surg

27:115–117.

duct will require a sialodochoplasty procedure (Figure 9.11). In most cases due to grade, tumor size, the presence of teeth, and involvement of mandibular periosteum/bone a wider access will be required. If the periosteum is uninvolved and can be safely peeled from the lingual bone a standard “pull through” approach or a lip split with mandibulotomy can be used (Figure 9.12). The functional result of the lip split/mandibulotomy is better than the pull through (Devine, Rogers, and McNally et al. 2001). As both of these methods of access involve entering the neck, a supraomohyoid neck dissection is usually carried out in the N0 neck even for low-grade tumors (Figure 9.13).

When positive nodes are present, type I modified radical neck dissection is required. Both lingual and hypoglossal nerves can be involved by these tumors at an early stage, particularly the ACC. Sacrifice of the nerve with proximal tracing and frozen section guidance as described for the submandibular tumors may be needed. In tumors fixed to periosteum or where minimal cortical erosion is present, an oblique marginal mandibular resection angling the cut to take a greater height of the lingual plate will be utilized. The marginal mandibular resection can be performed with the pull through or mandibulotomy approach. Where the medullary bone is invaded a segmental mandibular resection with a composite en bloc resection of the floor of the mouth is safest and will provide excellent access (Figure 9.14). In these

Figure 9.12a. Adenoid cystic carcinoma of sublingual gland closely approximated to the mandible (seen via an intraoral mirror photograph).

Figure 9.12b. Following lip split and mandibulotomy the periosteum is found to be uninvolved and is stripped from the mandible, which is preserved.

larger soft tissue resections a thin pliable flap such as the radial forearm flap probably gives the best results in maintaining tongue mobility. Where the mandible has been resected a fibular flap (Rinaldo, Shaha, and Pellitteri et al. 2004) or deep circumflex iliac artery (DCIA) flap is appropriate.

Adjuvant radiation therapy is indicated for positive nodes, perineural invasion, extracapsular nodal spread, positive margins, and high-grade histology. Prognosis for these tumors is difficult to assess, as the literature is mostly composed of case reports and small series. Spiro (1995) reported only 3 of 18 patients (16.6%) dying of their tumor with a median follow-up of 74 months. However, Yu, Gao, and Wang et al. (2007) reported distant

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Figure 9.13c. Cosmetic result of lip split incision. Patient is alive and tumor-free 13 years postoperatively.

Figure 9.13a. CT scan shows low-grade mucoepidermoid carcinoma of the right sublingual gland.

Figure 9.13b. This tumor was accessed via a lip split incision and mandibulotomy. Bilateral supraomohyoid neck dissections were undertaken, as can be seen in the surgical specimen.

metastases and local recurrence as the main cause of death with local recurrence rates of 30% and distant metastases 26.7%. In this series 56.7% of tumors were stage III.

It is reasonable to conclude that although 5-year survival from submandibular and sublingual gland cancer is reasonable, the high percentage of ACC found in these glands leads to a continuing decrease in survival at 10 years and beyond due to late local recurrence and distant metastases.

Figure 9.14a. 46-year-old African American man with left cervical lymphadenopathy.

Figure 9.14b. Intraoral view of high-grade mucoepidermoid carcinoma fixed to the right mandible.

Figure 9.14c. CT scan confirms necrotic node.

Figure 9.14d. A modified radical neck dissection and hemimandibulectomy with lip split was performed.

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Figure 9.14e. Postoperative panoramic film showing the reconstruction plate.

Figure 9.14f. Immediately post-chemoradiation.

Figure 9.14g. Mass in anterior mediastinum eroding sternum and manubrium.

Figure 9.14h. Close-up view of manubrial mass. The patient died shortly thereafter due to lung metastases. Re-published with permission from Ord RA. 2000. Salivary gland disease. In: Fonseca R (ed.), Oral and Maxillofacial Surgery, Volume 5, Surgical Pathology. Philadelphia: W.B. Saunders Co., pp. 288–289 (Figures 10-21 a, b, c, d, e, f, g).

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214 Tumors of the Submandibular and Sublingual Glands

Summary

•Submandibular gland tumors comprise only 10% of salivary tumors.

•Most submandibular swellings are inflammatory in etiology.

•50% of submandibular tumors will be malignant.

•Open biopsy should not be used for submandibular gland tumors. FNAB is the preoperative diagnostic method of choice.

•Sublingual gland tumors comprise <1% of salivary tumors.

•90% of sublingual gland tumors are malignant.

•ACC followed by MEC are the commonest cancers in both the submandibular and sublingual glands.

•Surgical management is based on both histologic diagnosis and stage.

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