Papillary Cystadenoma of Larynx: A Case Report

doi:10.4236/ijohns.2013.26058

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International Journal of Otolaryngology and Head & Neck Surgery, 2013, 2, 280-285

Published Online November 2013 (http://www.scirp.org/journal/ijohns)

http://dx.doi.org/10.4236/ijohns.2013.26058

Open Access IJOHNS

Papillary Cystadenoma of Larynx: A Case Report

Tammam Abou Ali1, Basel Al Sabah2, Kavitha Gopalan2

1Department of ENT, Farwaniya Hospital, Al-Farwaniy a h, Kuwait

2Department of ENT, Zain & Al Sabah Hospital, Kuwait City, Kuwait

Email: [email protected]

Received August 28, 2013; revised September 26, 2013; accepted October 15, 2013

cense, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

ABSTRACT

An uncommon clinical entity o f papillary cystadenoma of the larynx in a lady of 37-year-old is presented. Th e diagnosis

after clinical examination was achieved by video-stroboscopy, CT scan of the neck, biopsy and histopathological ex-

amination. This case study emphasizes the importance of recognition of this lesion by the clinician as the differential

diagnosis includes lesions with similar clinical appearance.

Keywords: Papillary Cystadenoma; Larynx; Minor Salivary Gland Tumors

1. Introduction

Cyst adenomas represent between 2.0% and 4.7% of all

minor salivary gland neoplasms [1,2], and papillary cys-

tadenoma of salivary glands is an uncommon benign,

well-circumscribed epithelial neoplasm resembling a

mucous retention cyst. The median age of occurrence is

53.5 and 55 years [1] with a female prevalence. The cys-

tic lesions have papillary projections arising from undif-

ferentiated epithelium of the intercalated ducts of the

glands [3] which could be the minor salivary glands, the

parotid glands, oral cavity, larynx, nasopharynx or the

lacrimal gland.

We herein present a case of multiple papillary cyst

adenomas of the larynx with gross, histological features

and important differential diagnosis of these lesions.

2. Case Report

A 37-year-old wo man with unr emarkable medical histor y,

presented with dysphonia and mild dysphagia for liquids

since one year which were gradually progressive. She did

not give history of dyspnea, odynophagia, otalgia or

cough. Laryngeal examination and videostroboscopy

revealed multiple pinkish sessile cystic lesions of 1 to 1.5

cm diameter, with normal overlying mucosa, distributed

all over the supraglo ttis giving a cobb le-stone appearance.

The true vocal fo lds were normal in appearance and mo-

bility. The sub glottis was free. No cervical lymphade-

nopathy was noted. Serum biochemistry, full blood count

and erythrocyte sedimentation rate were within normal

limits. Computed tomography scan of neck revealed mild

nodular irregularity of entire supraglottis including both

false vocal cords (more appreciated on the left side) with

normal CT appearance of true vocal cords as shown in

Figure 1. Stroboscopy confirmed the CT findings of

multiple cystic lesions in supraglottis as shown in Fig-

ures 2(a)-(c). Direct laryngoscopy and biopsy of the cys-

tic lesions were done. Figures 3(a) and (b) show the cys-

tic lesions in supraglo ttis while biopsied.

Histopathological analysis revealed benign tumors

(cysts), covered my non-ulcerated mucosa of non-

keratinized squamous epithelium with areas of focal hy-

Figure 1. CT neck.

T. A. ALI ET AL. 281

(a)

(b)

(c)

Figure 2. Stroboscopic pictures of larynx with multiple cys-

tic lesions in the entire supraglottis.

perplasia as shown in Figures 4(a)-(c). Submucosa

showed prominent fibrosis and mild inflammatory cellu-

lar infiltrate. The sub epithelial stroma showed multiple

(a)

(b)

Figure 3. Operative pictures of the supraglottic cysts during

biopsy.

cystic and partially solid nodules. The cystic cavity ex-

hibited papillary growth and finger like processes with

fibro vascular core as seen in Figures 5(a)-(c). The lin-

ing epithelium is mainly columnar with abundant cyto-

plasm and basophilic nucleus. No evidence of mitosis

noted and no evidence of invasion to the surrounding

connective tissue stroma was noted. The established di-

agnosis was papillary cystadenoma.

By endolaryngeal surgery using Kleinsasser’s micro

laryngoscopy technique, multiple cysts in entire suprag-

lottis and false vocal cords were completely excised us-

ing micro instruments as shown in Figure 6. Laser or

powered instruments were avoided for fear of fibrosis

latter. Patient had uneventful post-operative recovery.

Histopathology of the excised lesions confirmed the di-

agnosis of papillary cyst adenoma and no stromal inva-

sion were identified.

The patient is currently being followed up and shows

no signs of recurrence.

3. Discussion

The classification of salivary gland tumors by WHO

(1991) has distinguished two variants of cystadenoma:

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T. A. ALI ET AL.

282

(a)

(b)

(c)

Figure 4. (a), (b) and (c) (LP): shows pieces of mucosa and

sub mucosa, covered by non-keratinized squamous epithe-

lium with no ulceration of the covering mucosa.

papillary cystadenoma and mucinous cystadenoma [4].

(a)

(b)

(c)

Figure 5. (a) and (b) (H&E × 100): cystic cavity exhibiting

papillary projections supported by fibrous connective tissue;

nar cells and surrounding tissue free of invasion.

Histologically, it consists of a multilocular proliferation

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T. A. ALI ET AL. 283

Figure 6. Operative picture of endoscopic excision of the

lesions in the supraglottis.

of glandular epithelium. Epith elial cells may b e co lumnar,

cuboidal, squamous or oncocytic-like. Papillary forma-

tions are frequently seen. Stroma is fibrous, rarely with

prominent lymphocytic elements. Papillary cystadenoma

closely resembles Warthin’s tumour, but is devoid of

lymphoid stroma. Mucinous cystadenoma, like papillary

cystadenoma, is a multicystictumour, but is lined by

mucous-producing goblet cells. There are no atypia, nor

any invasion, in either of the two types of cystadenoma.

Papillary cystadenoma, although rare in salivary glands

outside the larynx, can only occasionally be found in

major salivary glands, the nasopharynx, buccal mucosa,

the lacrimal gland the palate and the tonsillar region.

Laryngeal papillary cystadenoma often show oncocytic

metaplasia and are reactive cystic lesions with hyperpla-

sia rather than true neoplasia [5]. This cellular oncocyto-

sis appears to be age related and is accelerated by smok-

ing.

The rarity of this tumour is reported by many authors.

The incidence of papillary cystadenoma in a review of

426 cases of intraoral minor salivary gland tumours by

Waldron et al. [6] is 4.7% of all minor salivary gland

neoplasms and 8.1% of all benign epithelial minor sali-

vary gland neoplasms. Similarly, Chaudry et al. [7] re-

ported only 2% of occurrence of papillary cystadenoma

in a large study of 800 intraoral minor salivary gland

benign tumours.

Previous reports have shown an age range from 12 to

89 years, and an average age at diagnosis of 57 years [8].

Also, previous series have shown a female predominance

[7,9].

In our case, laryngeal examination along with vide-

ostroboscopy and CT scan of neck guided the clinical

diagnosis of multiple cystic lesions in the supraglottis.

Excision biopsy and histopathology ascribed the definite

diagnosis of papillary cystadenoma. As special stains and

immunohisto chemistry are non-contributory for the di-

agnosis [14], they were not done.

The histologic features of this case of papillary cysta-

denoma were characteristic of the lesion rendering the

diagnosis easier. The lesions were well circumscribed

having cystic spaces with intracystic papillary projec-

tions lined by tall co lumnar cells with b asoph ilic nu cleus.

Oncocytes, squamous epithelium or mucous cells were

not seen in these lesions which are common variants re-

ported. No cellular atypia or mitotic figures were noted

and adjacent stroma was free of invasion ruling out ma-

lignancy.

Clinically, the differential diagnosis encountering cys-

tic lesions of the larynx includes mucous retention cysts,

extra nodal lymphomas, lipomas, papillomas, schwan-

nomas and neurofibromas, adenomatoid hyperplasia of

minor salivary glands etc.

The important histological differential diagnosis of

papillary cystadenoma includes papillary cystadenoma

lymphomatosum, oncocytic cyst adenomas, oncocytomas,

low-grade mucoepidermoid carcinoma, the papillary—

cystic variant of acinic cell carcinoma and cystadenocar-

cinoma [10].

Both papillary cystadenoma and papillary cystade-

noma lymphomatosum (Warthin’s tumour) are cystic

tumors but are differentiated by the presence of numer-

ous oncocytes, p apillary formatio ns and lympho id stroma

with lymph follicles which repr esent the main features of

Warthin’stumour [1]. Oncocytic variants of papillary

cystadenoma exist which could be confused with

Warthin’s tumour or oncocytoma.

Oncocytic cystadenoma is characterized by a smooth

cyst wall composed of eosinophyllic cells with granular

cytoplasm (due to numerous mitochondria). The cyst is

adjacent to normal respiratory epithelium.

Oncocytomas are true adenomas and are composed of

solid Oncocytes without cystic component.

Mucoepidermoid carcinoma is characterized his-

tologically by solid nests composed of a mixture of mu-

cous forming cells, epidermoid cells and sometimes of

intermediate cells which are absent in cystadenoma.

In general, four different cell types constitute acinic

cell carcinoma which includes large acinic cells, vacuo-

lated cells, clear cells and non-specific glandular cells

and in the papillary cystic variant of acinic cell carci-

noma, a configuration with papillae in cystic spaces is

seen which resembles papillary cystadenoma [11]. A

microcystic growth pattern in the intra luminar fronds

which is a characteristic feature of acinic cell carcinoma

is not seen in papillary cystadenoma and helps to differ-

entiate.

The most important differential diagnosis of papillary

cystadenoma is cystadenocarcinoma as both neoplasms

have similar architecture with papillary proliferation of

the epithelial lining and are composed of cells that pos-

sess bland cytomorphologic features [12]. Moreover,

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T. A. ALI ET AL.

284

cystadenocarcinoma often shows little atypia [13]. Dif-

ferentiation of tumou r types depends largely on the id en-

tification of actual infiltration of salivary gland paren-

chyma or surrounding connective tissue by either cystic

or solid epithelium in cystadenocarcino mas.

The suggested management for these benign lesions is

conservative and consists of complete local excision

[5,14]. Open laryngeal surgery like Lateral/Median Thy-

rotomy and pharyngotomy approach or transoral ap-

proach is required for the cartilaginous structures of the

larynx; if the position of larynx is to be altered, or if the

anatomical situation of lesion prevents adequate endo-

scopic visualization .

Laryngeal lesions located in the mucosa or sub muco-

sal space is easily accessible to endoscopic approach

with advantages of avoidance of skin incisions, division

of the thyroid cartilage and tracheotomy.

The surgical approach and excision depend on the tu-

mor size and location; but in general, preservation of

laryngeal function is the primary aim of treatment in the

form of complete excision with tumor-free margins. With

improvements in imaging, histopathology, and surgical

techniques—endoscopy, microscopic magnification, ap-

plication of lasers—there has been marked improvement

in the accuracy of diagnosis and the ability to perform

precision excision surgery for benign tumors. This means

more preservation and restoration of laryngeal function

than was previously possible [15]. As per the recom-

mendations, we completely excised the entire cysts in

supraglottis by endoscopic micro laryngeal surgery and

histopathology reconfirmed the diagnosis as benign pap-

illary cystadenoma. Although papillary cystadenoma are

benign lesions, follow-up is recommended, as recom-

mended for oncocytic cysts since recurrence is possible,

especially in the case of patients with multiple involve-

ments, with a tendency to develop new cysts [16]. In our

case, considering the benign nature of the lesions, annual

revisions were decided for a minimum period of five

years.

4. Conclusion

We present this case of multiple laryngeal papillary cys-

tadenomas, a rare benign lesion presenting with hoarse-

ness and dysphagia as this clinical en tity h as to be kept in

mind while evaluating patients with above mentioned

symptoms. Moreover, the histo-pathological diagnosis is

crucial as many tumours are with similar features and

management of each varies.

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