Adenoid cystic carcinomas are malignant tumors that occur in both the major and the minor salivary glands. A laryngeal location is rare because of the paucity of accessory salivary glands in this area. Adenoid cystic carcinomas account for less than 1% of all malignant tumors in the larynx, and only about 120 cases have been reported in the literature. These tumors have a slight female predisposition, and their peak incidence is in the fifth and sixth decades of life. In this article, we describe a case of laryngeal adenoid cystic carcinoma and discuss its clinical characteristics and treatment. We report on a case of laryngeal adenoid cystic carcinoma in a 55 year-old female patient who presented with dyspnea and hoarseness. Features of the diagnostic and therapeutic evaluation are described and the clinical management of such cases is outlined. The clinical course, definitive treatment strategy and surgical procedure, and also adjuvant treatment with irradiation are discussed. Although the tumor is radiosensitive, it is not radiocurable.125295-

Altemani, Albina

Del Negro, André

Ichihara, Edson

Martins, Antônio Santos

Tincani, Alfio José

Sao Paulo Medical Journal

English

Repositorio da Producao Cientifica e Intelectual da Unicamp

ABSTRACT
Sao Paulo Med J. 2007;125(5):295-6.
C
A
S
E
 
R
E
P
O
R
TAndré Del Negro
Edson Ichihara
Alfi o José Tincani
Albina Altemani
Antônio Santos Martins
Laryngeal adenoid cystic carcinoma: 
case report
Head and Neck Service, Department of Surgery, Faculdade de Ciências 
Médicas da Universidade Estadual de Campinas (FCM-Unicamp), 
Campinas, São Paulo, Brazil
CONTEXT: Adenoid cystic carcinomas are malig-
nant tumors that occur in both the major and the 
minor salivary glands. A laryngeal location is 
rare because of the paucity of accessory salivary 
glands in this area. Adenoid cystic carcinomas 
account for less than 1% of all malignant tumors 
in the larynx, and only about 120 cases have 
been reported in the literature. These tumors 
have a slight female predisposition, and their 
peak incidence is in the fi fth and sixth decades 
of life. In this article, we describe a case of 
laryngeal adenoid cystic carcinoma and discuss 
its clinical characteristics and treatment.
CASE REPORT: We report on a case of laryngeal 
adenoid cystic carcinoma in a 55 year-old female 
patient who presented with dyspnea and hoarse-
ness. Features of the diagnostic and therapeutic 
evaluation are described and the clinical man-
agement of such cases is outlined. The clinical 
course, defi nitive treatment strategy and surgical 
procedure, and also adjuvant treatment with 
irradiation are discussed. Although the tumor is 
radiosensitive, it is not radiocurable.
KEY WORDS: Adenoid cystic carcinoma. Larynx. 
Salivary gland. Radiotherapy. Laryngectomy.
INTRODUCTION
Adenoid cystic carcinomas are malignant tu-
mors that occur in both the major and the minor 
salivary glands. A laryngeal location is rare because 
of the paucity of accessory salivary glands in this 
area. Adenoid cystic carcinomas account for less 
than 1% of all malignant tumors in the larynx, 
and only about 120 cases have been reported in 
the literature until now.1,2 Two-thirds of these 
laryngeal tumors occur in the subglottis.3 
These tumors have a slight female predisposi-
tion, and their peak incidence is in the fi fth and 
sixth decades of life.4 There is no distinct risk factor 
that predisposes patients towards this malignancy.4 
Patients usually present with a history of mild 
to severe dyspnea at diagnosis, and histological 
confi rmation is obtained during tracheotomy to 
treat tumors that are up obstructed airways. 
CASE REPORT
A 55 year-old woman who was a former 
smoker was referred to our service with an 
18-month history of progressive dyspnea 
and hoarseness. The patient had no history 
of cough or dysphagia. Fiber bronchoscopy 
detected a vegetative lesion in the posterior 
commissure of the larynx that extended down-
wards to the trachea. During clinical investiga-
tion, the patient developed acute respiratory 
failure, which was treated as community-ac-
quired pneumonia. On physical examination, 
she showed inspiratory laryngeal stridor. 
Fiberoptic laryngoscopy showed an en-
dophytic lesion in the subglottic area with 
laryngeal stenosis of 80%. Tracheotomy was 
performed under local anesthesia and a le-
sion biopsy obtained. The pathology report 
revealed that the specimen was consistent with 
an adenoid cystic carcinoma of the larynx. 
Further investigation using esophagos-
copy did not show any suspicious lesions, 
although there was diffi culty in introducing 
the esophagoscope through the left piriform 
sinus. Computed tomography (CT) con-
fi rmed the presence of a submucosal mass in 
the subglottic area. Neither physical examina-
tion nor neck CT detected any evidence of 
node involvement. The fi ndings from chest 
X-ray were normal. 
We performed total laryngectomy without 
neck dissection and left thyroid lobectomy 
with isthmusectomy. Additionally, the lower 
margin of the third tracheal ring was sent for 
frozen-section biopsy, which did not show any 
evidence of disease. 
The fi nal pathology report showed nega-
tive margins in the subglottis, cricoid cartilage 
and tracheal ring. The tumor affected both 
the vocal folds and the posterior commissure 
and invaded both the left thyroid lobe and the 
extralaryngeal soft tissues of the neck, with a 
maximum width of 2.5 centimeters. Although 
there was no lymphatic spreading, perineural 
invasion (which is a feature of adenoid cystic 
carcinoma) was grossly present (Figure 1).
DISCUSSION
Adenoid cystic carcinoma usually occurs 
as a largely asymptomatic, nonulcerated sub-
mucosal mass. As a result, diagnosis is often 
delayed and, in the larynx, subglottic tumors 
have the opportunity to invade deeply before 
they are diagnosed. 
Figure 1. Histological section of la-
ryngeal adenoid cystic carcinoma. 
(400 x magnifi cation and hematoxylin-
eosin staining).
296
Sao Paulo Med J. 2007;125(5):295-6.
Preoperative histopathological analysis is 
essential because the symptoms do not differ 
greatly from squamous cell carcinoma. Neck 
metastasis is rare, occurring in only 10 to 
15% of the cases.1 Since early perineural and 
hematological spreading is common, local 
recurrences and distant metastases (especially 
to the lung) are common and sometimes arise 
years after the primary tumor has been diag-
nosed and treated. Therefore, these patients 
require long-term follow-up.
An extensive review of the literature revealed 
that there have been about 120 well-documented 
cases of adenoid cystic carcinoma of the larynx. 
Morais Pérez et al.2 reported only 80 cases up to 
1999, and after that year we found only another 
40 cases reported in the literature. According 
to Dexemble et al.,3 64% of laryngeal adenoid 
cystic carcinoma cases occur in the subglottis, 
25% in the supraglottis, 5% in the glottis and 
6% in the transglottic area. The fi rst case of 
hypopharyngeal adenoid cystic carcinoma was 
reported in 2004 by Kufeld et al.5
The histopathological pattern of adenoid 
cystic carcinoma is classifi ed into three distinct 
subtypes: cribriform, which is the most com-
mon; tubular, which has the best prognosis; and 
solid, which carries the worst prognosis.4
As is true of adenoid cystic carcinoma 
originating elsewhere, distant metastases 
are occasionally stable and slow-growing. 
Therefore, with aggressive surgery, the fi ve-
year survival rates for patients with laryngeal 
adenoid cystic carcinoma have been reported 
to range from only 12 to 17%.1-3,6
In view of the rarity of laryngeal adenoid 
cystic carcinoma, the treatment options are 
still controversial. Previous case reports contain 
different management strategies and few data 
on follow-ups. Yet most authors agree that the 
treatment of choice is wide-margin local exci-
sion (partial or total laryngectomy, depending 
on the location and size of the tumor).1-3,5,6 In 
the absence of neck metastasis, elective neck dis-
section is unnecessary. Radical neck dissection 
is indicated for patients who have clinically or 
histologically confi rmed nodal metastases.1-6
The role of radiotherapy is still open to 
debate. These tumors have been shown to be 
radiosensitive but usually not radiocurable. 
Therefore, radiotherapy alone usually has little 
role in treatment.1,6 It is possible that radio-
therapy may cure adenoid cystic carcinoma 
in a prepubescent larynx,1 but this carries the 
risk of causing a condition of fi brotic infantile 
larynx and leaving the patient with no useful 
voice, airway or swallowing function. 
Since our patient had a large tumor with 
perineural invasion, she was scheduled for 
adjuvant radiotherapy.
CONCLUSION
Laryngeal adenoid cystic carcinoma is a 
rare entity and, in many cases, the diagnosis 
is only made when the disease has become ad-
vanced. Therefore, a high degree of suspicion 
is essential for early diagnosis.
In our opinion, although other treatment 
options have been suggested, the best tumor 
management includes wide-margin surgery 
with postoperative radiotherapy for advanced 
lesions that present perineural spreading or 
close or positive margins.
For these patients, long-term follow-up 
is essential because of the possibility of late 
hematological metastasis.
1. Javadi M, Bafrouee FM, Mohseni M, Asghari A. Laryngeal 
adenoid cystic carcinoma in a child: a case report. Ear Nose 
Throat J. 2002;81(1):34-5.
2. Morais Pérez D, Cortejoso Hernández A, Borau Clavero M, 
Martín Sigüenza G. Carcinoma adenoide quístico de laringe. 
[Adenoid cystic carcinoma of the larynx]. Acta Otorrinolaringol 
Esp. 1999;50(8):660-3. 
3. Dexemble P, Huth J, Rebufy M, Chabrol A. Carcinome adénoïde 
kystique du larynx. A propos de deux cas. [Cystic adenoid 
carcinoma of the larynx: two cases]. Ann Otolaryngol Chir 
Cervicofac. 2003;120(4):244-8.
4. Tincani AJ, Del Negro A, Araújo PP, et al. Management of 
salivary gland adenoid cystic carcinoma: institutional experience 
of a case series. Sao Paulo Med J. 2006;124(1):26-30.
5. Kufeld M, Junker K, Sudhoff H, Dazert S. Kollisionstumor eines 
adenoidzystischen Karzinoms des Hypopharynx und eines Plat-
tenepithelkarzinoms des Larynx. [Collision tumor of a hypopha-
ryngeal adenoidcystic carcinoma and a laryngeal squamous cell 
carcinoma]. Laryngorhinootologie. 2004;83(1):51-4.
6. Olofsson J, van Nostrand AW. Adenoid cystic carcinoma of 
the larynx: a report of four cases and a review of the literature. 
Cancer. 1977;40(3):1307-13.
Sources of funding: None
Confl ict of interest: None
Date of fi rst submission: May 25, 2006
Last received: September 9, 2007 
Accepted: September 12, 2007
REFERENCES
AUTHOR INFORMATION 
André Del Negro, MD. Attending surgeon, Head and Neck 
Service, Department of Surgery, Faculdade de Ciências 
Médicas da Universidade Estadual de Campinas, Campi-
nas (FCM-Unicamp), São Paulo, Brazil.
Edson Ichihara. Undergraduate student, Faculdade de 
Ciências Médicas da Universidade Estadual de Campinas 
(FCM-Unicamp), Campinas, São Paulo, Brazil.
Alfi o José Tincani, MD, PhD. Assistant professor, Head and 
Neck Service, Department of Surgery, Faculdade de 
Ciências Médicas da Universidade Estadual de Campinas 
(FCM-Unicamp), Campinas, São Paulo, Brazil. 
Albina Altemani, MD, PhD. Assistant professor, Department 
of Pathology, Faculdade de Ciências Médicas da Univer-
sidade Estadual de Campinas (FCM-Unicamp), Campinas, 
São Paulo, Brazil. 
Antônio Santos Martins, MD, PhD. Chief, Head and Neck 
Service, Department of Surgery, Faculdade de Ciências 
Médicas da Universidade Estadual de Campinas (FCM-
Unicamp), Campinas, São Paulo, Brazil. 
Address for correspondence: 
André Del Negro
Rua Governador Pedro de Toledo, 2.157 — Apto. 141
Piracicaba (SP) — Brasil — CEP 13400-075 
Tel. (+55 19) 3435-1898
E-mail: docdnegro@yahoo.com.br
Copyright © 2007, Associação Paulista de Medicina
RESUMO
Carcinoma adenóide cístico de laringe: relato de caso
CONTEXTO: Carcinoma adenóide cístico (CAC) é um tumor maligno que ocorre tanto nas glândulas sali-
vares maiores quanto nas menores. Localização laríngea é rara devido à paucidade de glândulas salivares 
acessórias nesta região. O carcinoma adenóide cístico representa menos de 1% das lesões malignas da 
laringe e apenas 120 casos foram relatados na literatura. O CAC tem freqüência discretamente superior 
no sexo feminino e seu pico de incidência ocorre entre a quinta e sexta décadas de vida. Neste artigo, 
descrevemos um caso de CAC laríngeo e discutimos suas características clínicas e seu tratamento.
RELATO DE CASO: Relatamos o caso de CAC numa paciente de 55 anos que apresentava disfonia e 
dispnéia. Características diagnósticas e avaliação terapêutica são descritas e a conduta clínica defi nida. 
Evolução clínica, estratégia terapêutica e procedimento cirúrgico são discutidos, bem como o tratamento 
adjuvante com radioterapia. O tumor, apesar de radiossensível, não é radiocurável.
PALAVRAS-CHAVE: Carcinoma adenóide cístico. Laringe. Glândulas salivares. Radioterapia. Laringectomia. 


Laryngeal Adenoid Cystic Carcinoma: Case Report.

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Laryngeal Adenoid Cystic Carcinoma: Case Report.

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