Acta Oto-Laryngologica. 2015; 135: 489–493

ORIGINAL ARTICLE

Adenoid cystic carcinoma of the larynx: a report of six cases with review of the literature

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WEI LIU1 & XIAOHONG CHEN2 1

Department of Otolaryngology, Beijing Children’s Hospital, Capital Medical University, Beijing Pediatric Research Institute, Beijing Key Laboratory for Pediatric Diseases of Otolaryngology, Head and Neck Surgery and 2Department of Otolaryngology, Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, Key Laboratory of Otolaryngology, Head and Neck Surgery, Ministry of Education, Beijing Institute of Otolaryngology, Beijing, PR China

Abstract Conclusion: Adenoid cystic carcinoma (ACC) in the larynx is an extremely rare entity. Although the 5-year disease-specific survival rates for laryngeal ACC are high, distant metastasis might occur up to more than 5 years post treatment. Therefore patients with ACC require long-term follow-up. Objective: To summarize the characteristics of laryngeal ACC by analyzing six cases in a single center. Methods: This was a retrospective analysis of six patients with laryngeal ACC who were treated in Beijing Tongren Hospital between 1998 and 2013. Results: The mean age at diagnosis was 44 years, ranging from 15 to 61 years. Dyspnea was the most common complaint. All patients underwent combined-modality treatment with surgical resection and external beam radiation. The range of follow-up time varied from 1 to 7 years (median 5 years). Follow-up inspections indicated that none of them had local or regional recurrence. Distant metastasis occurred in 33.7% (2/6) of the patients, with one lung metastasis 5 years after operation and one liver metastasis 4 years after operation.

Keywords: Treatment, follow-up, dyspnea, laryngeal carcinoma

Introduction Salivary gland carcinomas of the larynx are rare, and account for < 1% of laryngeal malignancies [1]. Adenoid cystic carcinomas (ACCs) are the most common malignant tumors that occur in minor salivary glands. It was Billroth in 1856 who coined the term ‘cylindroma,’ which became a widely accepted term for this tumor [2]. Laryngeal ACC is extremely rare because of the paucity of accessory salivary glands. This kind of tumor is characterized by multiple recurrences and late distant metastasis. Because of frequent early perineural and hematologic spread, local recurrences, and systemic metastases (especially to the lung), which sometimes arise years after diagnosis and treatment, patients with laryngeal ACC require long-term follow-up [3].

This study aimed to review our long-term experience of treating ACC of the larynx at Beijing Tongren Hospital. Material and methods We performed a retrospective analysis of six patients with laryngeal ACC treated in Beijing Tongren Hospital between 1998 and 2013 after obtaining the approval of the institution’s research ethics board. The clinical and pathologic data were obtained from the hospital records. We telephoned the patients or their family members to obtain additional follow-up data. Statistical analysis was performed through SPSS 17.0 software. The Kaplan–Meier method was used to construct disease-specific survival curves for the sites.

Correspondence: Dr Xiaohong Chen, Department of Otolaryngology, Head and Neck Surgery, Beijing Tongren Hospital, Beijing 100730, China. Tel: +86 10 58269331. Fax: +86 10 58269107. E-mail: [email protected]

(Received 10 October 2014; accepted 13 November 2014) ISSN 0001-6489 print/ISSN 1651-2251 online  2015 Informa Healthcare DOI: 10.3109/00016489.2014.990583

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Table I. Patients’ characteristics. Patient no.

Sex

Age (years) 46

Pharyngeal paresthesia

Site

1

Male

2

Male

61

Dyspnea

Subglottis

3

Female

56

Pharyngeal paresthesia

Supraglottis

4

Female

47

Dyspnea

Subglottis

5

Female

15

Dyspnea and hoarseness

Subglottis

6

Male

39

Pharyngeal paresthesia and hoarseness

Subglottis

Supraglottis

had positive microscopic margins and received postoperative external beam radiation with 66 Gy.

Results Acta Otolaryngol Downloaded from informahealthcare.com by Nyu Medical Center on 06/09/15 For personal use only.

Symptoms

Patients’ characteristics The mean age of the patients with laryngeal ACC was 44 years, ranging from 15 to 61 years. Three of the six patients were male and three were female. About 66.7% (4/6) of the cases had subglottic ACC and the other lesions (2/6) were in the supraglottis. None of the patients had evidence of distant metastasis at diagnosis. Patients’ characteristics are summarized in Table I. Dyspnea was the most common complaint followed by pharyngeal paresthesia, hoarseness, and dysphagia. All patients underwent combinedmodality treatment with surgical resection and external beam radiation. All the six patients received postoperative external beam radiation with a range of 60–66 Gy. Surgical resection Treatment details and outcomes are presented in Tables II and III. Two patients with subglottic ACC underwent total laryngectomy. The other four patients with ACC in supraglottis underwent horizontal partial laryngectomy. Four of these patients also underwent modified radical neck dissection because of palpable cervical lymph nodes. Two of them who had thyroid infiltration underwent total or subtotal thyroidectomy. Four patients had negative microscopic margins and received postoperative external beam radiation with 60 Gy. The other two patients

Histopathology The postoperative histopathology confirmed that all the six patients had laryngeal ACC. Pathologic subtyping revealed solid (1/6), tubular (3/6), and cribriform (2/6) patterns. All the lymph nodes had reactive hyperplasia without evidence of metastatic ACC. Only one patient (no. 5) had recurrent laryngeal nerve invasion (Figure 1). Follow-up, survival, and vital status The range of follow-up varied from 1 to 7 years (median 5 years). Follow-up inspections indicated that none of the six patients had local or regional recurrence. Distant metastasis occurred in 33.7% (2/6) of the patients, with one lung metastasis 5 years after operation and one liver metastasis 4 years after operation. The patient with lung metastasis eventually died from his disease (Figure 2). Pathologic subtyping of this case was solid pattern. The patient with liver metastasis accepted interventional therapy and was still alive during follow-up. Discussion ACC arises from mixed seromucinous glands in the larynx, which have been shown to decrease from the supraglottis to the glottis and subglottis [4].

Table II. Type of surgery and radiation. Patient no.

Site

Type of surgery

Surgical margins

1

Supraglottis

Horizontal partial laryngectomy + bilateral neck dissection

2

Subglottis

Total laryngectomy + left neck dissection

Negative

60

3

Supraglottis

Horizontal partial laryngectomy + bilateral neck dissection

Negative

60

4

Subglottis

Total laryngectomy + bilateral thyroidectomy

Negative

60

5

Subglottis

Total laryngectomy + bilateral subtotal thyroidectomy

Positive

66

6

Subglottis

Total laryngectomy + bilateral neck dissection

Negative

60

Positive

Radiation dose (Gy) 66

Adenoid cystic carcinoma of the larynx

491

Patient no.

Local recurrence

Distant recurrence

1

No

Lung

2

No

No



6

Alive

3

No

Liver

4

6

Alive

4

No

No



4

Alive

5

No

No



1

Alive

6

No

No



4

Alive

No. of years to recurrence

Length of follow-up (years)

5

6

Figure 1. Patient no. 5: the tumor invaded the recurrent laryngeal nerve (hematoxylin and eosin stain, original magnification 200).

Survival analysis Survival function 0

1.0

Survival proportion

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Table III. Outcome at follow-up.

0.8

0.6 0.4 0.2 0.0 0.0

1.0

2.0

3.0 Years

4.0

5.0

6.0

Figure 2. Disease-specific survival curve.

Despite these findings, the subglottis appears to be the most common site of origin. According to Dexemble et al. [5], 64% of laryngeal ACCs occur in the subglottis, 25% in the supraglottis, 5% in the glottis, and 6% in the transglottic area. The age range

Vital status Dead

is wide, from 29 to 75 years, with the peak incidence in the fifth decade [6]. Although it is rare under 20 years of age [7], Javadi et al. [8] reported a 12-year-old boy with a subglottic ACC. One of our patients was 15 years old at the time of diagnosis. The ratio of males to females is nearly equal. The symptoms and signs of laryngeal ACC are related to the anatomic location. Our results indicated that dyspnea was more frequently associated with subglottic tumors. Tumors in the supraglottis presented with pharyngeal paresthesia. However, ACC usually occurred as a large asymptomatic, nonulcerated submucosal mass [9]. As a result, diagnosis is often delayed. The treatment options for laryngeal ACC are still controversial. Conventional treatment is surgery with or without postoperative radiotherapy. In the present study, all the six patients accepted surgical resection and postoperative radiotherapy. Two patients with subglottic ACC underwent total laryngectomy. The other four patients with supraglottic ACC underwent horizontal partial laryngectomy. The long-term follow-up confirmed that none of the six patients had regional metastasis at the time of presentation. We consider that if the tumor locates in the supraglottis, partial laryngectomy is recommended. However, if it originates from the subglottis, a total laryngectomy is necessary. Because the incidence of lymph node metastasis is rare, elective neck dissection in the absence of palpable lymph nodes is not recommended for ACC of the larynx [10,11]. In our series, four patients with palpable lymph nodes accepted neck dissection. All the lymph nodes had reactive hyperplasia without evidence of metastatic ACC. Chen et al. [12] found that omission of postoperative radiotherapy and, when delivered, a dose less than 60 Gy, were independent predictors of local recurrence. Moukarbel et al. [13] observed that two of three patients who were treated only with surgery and did not receive radiotherapy developed locoregional recurrence. In the present series, all the patients accepted postoperative radiotherapy. The patients with positive microscopic

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margins received external beam radiation with 66 Gy. In the past, we tried our best to achieve free margins. However, we must balance the treatment goals with each patient’s quality of life. Moukarbel et al. [13] observed that they were able to achieve free margins in only one of four patients who had conservative surgery. The rate of positive margins with the total laryngectomy group was high as well (50%). Still they were able to control the disease locoregionally for many years in many of the patients who had positive margins (in both groups, partial and total). In our series, two patients had positive margins and there were no local recurrences. However, one of the patients had distant metastasis 5 years after treatment. The histopathological pattern of ACC is classified into three distinct subtypes: cribriform, which is the most common; tubular, which has the best prognosis; and solid, which carries the worst prognosis [14]. In addition, the solid variant of ACC should be differentiated from basaloid squamous cell carcinoma, which has a high incidence of neck metastasis [15]. In our cases, only one patient with the solid histopathological pattern died from lung metastasis. This confirmed that the solid type had the worst prognosis. A lot of investigation has confirmed that ACC frequently undergoes early perineural and hematologic spread. As a result, local recurrences and distant metastases are common. Sometimes, the metastases arise many years after the primary tumor has been treated. Moukarbel et al. [13] reported that their review of 15 cases showed that the 5- and 10-year disease-specific survival rates were 69% and 49%, respectively. Alavi et al. [16] reviewed five patients with laryngeal ACC, showing an actuarial 2-year survival of 100% and a 5-year survival of 75%. Cohen et al. [17] found a 42.8% 5-year survival rate in their series of laryngeal sialogenic tumors. In our series, the 5-year disease-specific survival rate was 100%; however, one patient died 6 years after operation. The discrepancy in survival rates may result from the small number of patients in all series. Combined, these case series indicate that the 5-year disease-specific survival rates are high. However, distant metastasis may occur up to more than 5 years post treatment. Moukarbel et al. [13] reported that the rate of distant metastasis was 66.7%, and distant metastasis occurred up to more than 11 years after treatment. In our cases, at last follow-up, none of the six patients had local or regional recurrence. Distant metastasis occurred in 33.3% (2/6) of the patients, with one lung metastasis 5 years after operation and one liver metastasis 4 years after treatment. Therefore, patients with ACC require long-term follow-up.

Conclusion ACC in the larynx is an extremely rare entity. If the tumor locates in the supraglottic region, partial laryngectomy is feasible. Neck dissection is not perceived as an integral part of treatment. Pathologic subtyping with solid pattern indicates the worst prognosis. The 5-year disease-specific survival rates in laryngeal ACC are high. However, distant metastasis may occur up to more than 5 years post treatment; therefore, patients with ACC require long-term follow-up. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

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salivary gland adenoid cystic carcinoma: institutional experience of a case series. Sao Paulo Med J 2006;124: 26–30. [15] Kaya S. Larenksinseyrekgörülen benign ve malign tümörleri. In: Kaya S, editor. Larenks hastal1klar1. Ankara: Bilimsel T1p Yay1nevi; 2002. p 492–539.

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[16] Alavi S, Namazie A, Calcaterra TC, Blackwell KE. Glandular carcinoma of the larynx: the UCLA experience. Ann Otol Rhinol Laryngol 1999;108:485–9. [17] Cohen J, Guillamondegui OM, Batsakis JG, Medina JE. Cancer of the minor salivary glands of the larynx. Am J Surg 1985;150:513–18.

Adenoid cystic carcinoma of the larynx: a report of six cases with review of the literature.

Adenoid cystic carcinoma (ACC) in the larynx is an extremely rare entity. Although the 5-year disease-specific survival rates for laryngeal ACC are hi...
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