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Papillary Apocrine Cystadenoma of the External Auditory Canal:Papillary Apocrine Cystadenoma of the External Auditory Canal:A Case Report and Review of the LiteratureA Case Report and Review of the Literature

Katherine Johnson, MD1; Stephanie Johnson, RN, BSN2; Gary Moore, MD1

1University of Nebraska Medical Center, 2Creighton University, Omaha NE

Introduction Tumors with Apocrine Differentiation

Figure 1: Diagram of Adnexal Structures of the Skin

Figure 2: Axial CT Scan of Right Temporal Bone

Figure 3: Coronal CT Scan of Right Temporal Bone

ABSTRACT

Objectives:1) Identify the histologic features of papillary apocrine cystadenoma2) Describe clinical and histologic features that differentiate this tumor from other adnexal tumors with apocrine differentiation3) Understand the clinical behavior of these benign tumors

Study Design: Case report with review of the literature.

Methods: Chart review of a patient at a tertiary care neurotologist clinic with review of the English literature on adnexal tumors with apocrine differentiation.

Results: There are a variety of adnexal benign tumors that demonstrate apocrine differentiation. To date, only 2 other cases of papillary apocrine cystadenoma arising from the external auditory canal exist in the literature.

Conclusions: This is only the third case of papillary apocrine cystadenoma of the external auditory canal in the literature. This benign tumor usually presents as a cystic mass in the external canal. A complication of this tumor is external otitis or hearing loss. Complete surgical excision is recommended.

Apocrine Cystadenoma/ Hidroadenoma - Caused by adenomatous cystic proliferation of

apocrine glands (Not a retention cyst)-Affects adults, no sex predominance- Common sites include eye, face, ears, scalp,

chest and shoulders- Clinical features: gray bluish cyst with smooth

walls, dome shape with clear or milky fluid- Histologic findings include papillary projections

into a cyst cavity

Moll Gland Cyst- Also referred to as apocrine hidrocystoma of

the eyelid- Caused by obstruction of the apocrine sweat

gland apparatus- Affects adults, no gender or racial differences

in etiology, -Physical Exam: solitary papule, flesh colored,

typically near medial canthus, multiple- Clinical history: slow growing mass, rarely

recur following complete excision

Papillary Syringocystadenoma-Typically affects children in puberty- Physical Exam: verrucous, skin-colored

papules on face, scalp, or ears- Can develop malignant transformation

Hidroadenoma Papilliferum- Typically affects Caucasian women- Common Sites: Anogential region, head and

neck- Clinical history-slow growing mass-Physical Exam: rounded papule 1-40mm in

size, may ulcerate

A 68 year old female with history of right sided aural fullness was noted to have a mass located in the lateral external auditory canal. It was flesh colored in appearance and caused a near-total obstruction of the external meatus. The tympanic membrane was visualized beyond the mass and was normal in appearance. Facial strength was intact bilaterally.

An audiogram demonstrated high frequency sensorineural hearing loss without conductive component. A CT scan was obtained that demonstrated a right-sided external auditory canal mass with dimensions 1.1x 1.2x 1.8cm in size (See Figures 2 and 3).

The mass did not change following administration of antibiotics. Patient then underwent surgical excision. Intraoperatively, the mass was noted to be cystic in nature with a clear fluid. Final pathology demonstrated papillary apocrine cystadenoma. (See Figure 4a/b) No re-growth present at nine months postoperatively.

Adnexal structures of the skin may develop into benign neoplasms.

Typical presentation of adnexal tumors with apocrine differentiation is that of a cystic, well rounded mass in the skin, most commonly head and neck region.

Clinical behavior is a slow growing mass that does not recur following complete excision.

The outer one-third of the external auditory canal is cartilaginous and contains numerous structures. In this region, sebaceous glands, hair follicles and cerumen-producing apocrine glands are found. The medial two-thirds of the external auditory canal is cartilaginous and contains thin epithelium that is affixed to the bony periosteum. This region of the external canal is devoid of glands and hair follicles, and the epithelium is contiguous with the tympanic membrane.

The skin of the lateral external canal contains the epidermis, dermis, and subcutaneous tissue. The epidermis is stratified squamous epithelium that forms a protective barrier. Within the dermis, nerve fibers, blood vessels, and adnexal structures are found. (See Figure 1) The associated tissue surrounding a hair follicle is known as a pilosebacous unit, which is comprised of both the hair follicle and sebaceous gland. Sebaceous glands are responsible for producing sebum. Apocrine glands, found external ear canal, are responsible for producing cerumen. Other locations for apocrine glands include the eyelid (Moll’s gland) and axilla. These glands are small during childhood, but become more active following puberty.

Eccrine glands are not extensively found in the external auditory canal. These glands are responsible for regulating body temperature throughout the body. In the external ear canal, they are thought to provide a protective function. Neoplasms of any of the adnexal structures of the skin are possible.

Case Review

1. Sugiyama, A, et al. “Apocrine cystadenoma and Apocrine hidradenoma: examination of 21 cases with emphasis on nomenclature according to proliferative features.” J. Cutan Pathol. 2007; 34 (12): 912-17.

2. Lee et al. “Tubular apocrine adenoma with syringocystadenoma papiliferum arising from the external auditory canal” J Laryngol Otol. 2005; 119 (12): 1004-1005.

3. Onishi, T. “Immunohistochemical analysis of cytokeratin expression in apocrine cystadenoma or hidradenoma.” J Cutan Pathol. 1999; 26(6): 295-300

4. Mituishi, T et al. “ Apocrine cystadenoma arising in the ear.” J Dermatol. 1996: 23 (8): 583-84.

5. Dorland's Medical Dictionary for Health Consumers: Diagram of Skin. 1/14/2012. http://medical dictionary.thefreedictionary.com/_/viewer.asp x?path=dorland&name=skin.jpg,

Conclusions

References

Katherine Johnson, MDUniversity of Nebraska Medical CenterEmail: kijohnso@unmc.eduPhone: 402-559-7777

CONTACTCONTACT

Figure 4 a & b: Low power view demonstrating cystic structure, Higher power demonstrating papillary projections into cyst cavity.