Adenoid Cystic Carcinoma

Like the mucoepidermoid carcinoma, the adenoid cystic carcinoma is a very diverse tumor with three histologic variants. These have been described morphologically rather than by grade as is the case with the mucoepidermoid carcinoma, and include the tubular, cribriform, and solid variants. The adenoid cystic carcinoma is characteristically slow growing, with a high propensity for recurrent disease. It is highly infiltrative, exhibits profound neurotropism, and is associated with a dismal long-term survival rate. This malignancy was first described by Theodor Billroth in 1859 and referred to as "cylindroma" (Tomich 1991). In 1953 Foote and Frazell proposed the currently accepted nomenclature, adenoid cystic carcinoma. Of the 600 cases of adenoid cystic carcinoma in the AFIP files, 312 were noted in the major salivary glands, and 288 were noted in the minor salivary glands. The palate was the most common site affected in the minor salivary glands, followed by the tongue. Adenoid cystic carcinoma accounts for 8.3% of all

Figure 10.7a. A lesion of the right tuberosity region.

d palatal salivary gland tumors and 17.7% of all malignant palatal salivary gland tumors in the AFIP series (Tomich 1991).

From a surgical standpoint, adenoid cystic carcinoma is probably the most challenging salivary gland tumor for the surgeon (Ord 1994). While straightforward to perform in most cases, c

Figures 10.7c and 10.7d. Computerized tomograms identified an enhancing mass located lateral to the right tuberosity.

Figure 10.7b. The biopsy of the lesion from Figure 10.7a showed intermediate-grade mucoepidermoid carcinoma.

Figures 10.7c and 10.7d. Computerized tomograms identified an enhancing mass located lateral to the right tuberosity.

Figure 10.7e. Definitive tumor surgery involved a transoral partial maxillectomy and coronoidectomy en bloc.

Figure 10.7f. The cancer from Figure 10.7e.

Figure 10.7g. The resultant defect was obturated and allowed to contract significantly over time.

Figures 10.7h, 10.7i, and 10.7j. Soft tissue reconstruction was accomplished with a buccal fat flap and advancement of the mucosa.

Figure 10.7k. The reconstruction healed well as noted at 1 year postoperatively.

radical resection is fraught with recurrences and ultimate distant metastases. This notwithstanding, palatal tumors should be managed with radical maxillectomy, observing 1-2 cm linear margins, and with resection of the greater palatine neurovascular bundle to foramen rotundum with frozen section guidance (Figure 10.9). The presence or absence of tumor in association with this nerve should be documented as far superior as possible. Cervical lymph node metastases are considered to be rare such that prophylactic neck dissection is not required in the patient with an N0 neck. Postoperative radiation therapy is generally considered advisable for all patients with adenoid cystic carcinoma of the minor salivary glands, regardless of the adequacy of the resection (Dragovic 1995; Ord 1994; Triantafillidou et al. 2006). The prognosis associated with adenoid cystic carcinoma of the minor salivary glands is inferior to that of the major salivary glands (Ampil and Misra 1987; Nas-cimento, Amaral, and Prado et al. 1986). In addition, it has been found that the best prognosis for the adenoid cystic carcinoma is associated with the tubular variant, while the solid variant is associated with the worst prognosis (Perzin, Gullane, and Clairmont 1978). It has also been pointed out that perineural invasion of major nerves and positive margins at surgery, in addition to the solid variant of adenoid cystic carcinoma, are associated with increased treatment failures (Fordice et al. 1999). Typical survival statistics for adenoid cystic carcinoma in general include 60% for 5-year sur vival, 30% for 10-year survival, and 7% for survival at 20 years (Ord 1994). It has been pointed out that adenoid cystic carcinoma of minor salivary gland sites has a worse prognosis, with a 0% survival at 20 years (Ord 1994). The presence of perineural spread has a significant impact on survival. Five-year survival rates of patients with perineural spread have been found to be 36.9%, while 5-year survival rates have been found to be 93.8% in patients without perineural spread (Ord 1994).

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