Angiolymphoid Hyperplasia with Eosinophilia (Epithelioid Hemangioma)

Otic (Aural) Polyp

Age

Wide age range, peak in third to fifth decades, and female predominance

Wide age range, no predilection for either sex

Location

Head and neck lesions most often involve the periauricular soft tissue, auricle, external auditory canal, and scalp

Middle ear. May occasionally extend into the external ear through a perforated tympanic membrane

Symptoms

Subcutaneous nodule, possibly with pain, itching, and bleeding

Symptoms of otitis media: otalgia, hearing loss, fever, and drainage

Signs

Single or multiple subcutaneous nodule(s)

Red, fleshy polyp in the middle ear behind an intact tympanic membrane or extending into external auditory canal through a perforated membrane

Etiology

Unknown. Some appear to arise following trauma

Arise in the setting of chronic otitis media, sometimes with cholesteatoma

Histology

1. 
   

   Nodular proliferation of small vessels and chronic inflammatory cells, often centered on an injured artery (Fig. 3.1.1)

   
   
2. 
   

   The inflammatory infiltrate consists of lymphocytes with germinal centers and numerous eosinophils (Fig. 3.1.2)

   
   
3. 
   

   The proliferative vessels are small and have plump endothelial cells. These endothelial cells take on an epithelioid appearance including eosinophilic cytoplasm, large nuclei with open chromatin, and distinct nucleoli (Fig. 3.1.3)

1. 
   

   Polypoid fragments of granulation tissue. The surface epithelium is often ulcerated and the stroma is edematous (Fig. 3.1.4)

   
   
2. 
   

   Proliferation of small blood vessels with plump endothelial cells (Fig. 3.1.5)

   
   
3. 
   

   No association with an injured artery

   
   
4. 
   

   Heavy infiltrate of lymphocytes and sometimes neutrophils, but generally only rare eosinophils and no germinal center formation (Figs. 3.1.4 and 3.1.5)

   
   
5. 
   

   Background changes of otitis media: chronic inflammation, edema, fibrosis, calcifications, and/or cholesteatoma

Special studies

Nothing helpful in this differential diagnosis

Nothing helpful in this differential diagnosis

Treatment

Surgical excision

Surgical excision, treatment of underlying otitis

Prognosis

Excellent

Excellent

Endolymphatic Sac Tumor

Middle Ear Adenoma (Middle Ear Carcinoid Tumor)

Age

Wide age range, from second to eighth decades

Wide age range, peak in third to fifth decades

Location

Inner ear. May extend to involve the temporal bone and/or middle ear

Middle ear. May occasionally perforate the tympanic membrane and extend into the external ear

Symptoms

Hearing loss, tinnitus, and vertigo

Unilateral hearing loss, bleeding, and tinnitus

Signs

Destructive lesion of the temporal bone

Middle ear mass usually beneath an intact tympanic membrane

Etiology

Most are sporadic but some cases arise in the setting of von Hippel-Lindau syndrome

Unknown

Histology

1. 
   

   Papillary, follicular, and cystic architecture with pink secretions, resembling thyroid tissue (Fig. 3.2.1)

   
   
2. 
   

   The tumor often infiltrates bone (Fig. 3.2.2)

   
   
3. 
   

   Single layer of flattened, cuboidal, or columnar epithelial cells with clear or eosinophilic cytoplasm. The nuclei are uniformly round without significant atypia (Fig. 3.2.3)

   
   
4. 
   

   The stroma contains many capillary-sized vessels (Fig. 3.2.4)

1. 
   

   Proliferation of epithelial cells growing in a complex arrangement of nests, trabeculae, cords, ribbons, sheets, and/or tubules (Fig. 3.2.5). A papillary architecture is not common

   
   
2. 
   

   Bone invasion not typical

   
   
3. 
   

   Subtle biphasic tumor cell population: outer layer of cuboidal to columnar cells and an inner layer of flattened eosinophilic cells, often with luminal secretions (Fig. 3.2.6)

   
   
4. 
   

   Tumor cells often exhibit “neuroendocrine” cytologic features (plasmacytoid cells with fine “salt-and-pepper” chromatin, amphophilic and granular cytoplasm) (Fig. 3.2.6)

   
   
5. 
   

   Generally bland cytologic features with mild pleomorphism and minimal mitotic activity. The nuclear chromatin is finely dispersed (Fig. 3.2.6)

   
   
6. 
   

   The background may include changes of otitis media

Special studies

Positive for cytokeratins (including CK7) and EMA. Negative for neuroendocrine markers, TTF-1, thyroglobulin, and CD10

Positive for neuroendocrine markers synaptophysin, chromogranin, and CD56 (Fig. 3.2.7). Basal cells positive for p40 and CK5/6 but not for S100 or actin. Luminal cells positive for CK7

Treatment

Surgical excision with or without radiation

Surgical excision

Prognosis

Slow growing but progressive tumor growth. High morbidity due to proximity to cranial nerves. Local recurrences are common

Good, but recurrences in approximately 15%. Rare examples have shown aggressive local growth

Glandular Metaplasia in Otitis Media

Middle Ear Adenoma (Middle Ear Carcinoid Tumor)

Age

Any age can be affected, but most frequently young children

Wide age range, peak in third to fifth decades

Location

Middle ear

Middle ear. May occasionally perforate the tympanic membrane and extend into the external ear

Symptoms

Otalgia, hearing loss, fever, and drainage

Unilateral hearing loss, bleeding, and tinnitus

Signs

Inflamed, red, and budging tympanic membrane

Middle ear mass usually beneath an intact tympanic membrane

Etiology

Infectious, most often Streptococcus pneumoniae or Haemophilus influenzae

Unknown

Histology

1. 
   

   Background changes of otitis media including chronic inflammation, edema, fibrosis, and calcifications. Association with cholesteatoma is very common (Fig. 3.3.1)

   
   
2. 
   

   The “glands” actually represent invaginations of surface epithelium into the middle ear stroma (Fig. 3.3.2)

   
   
3. 
   

   Glands are haphazardly arranged and separated by intervening stroma (Figs. 3.3.3 and 3.3.4)

   
   
4. 
   

   The glands may be lined by mucinous cells or ciliated cells (Figs. 3.3.4 and 3.3.5)

1. 
   

   Poorly circumscribed proliferation of epithelial cells growing in a complex arrangement of nests, trabeculae, cords, ribbons, sheets, and/or tubules (Fig. 3.3.6). A papillary architecture is not common

   
   
2. 
   

   Glands are often arranged back-to-back without prominent intervening stroma (Fig. 3.3.6)

   
   
3. 
   

   Tumor cells often exhibit “neuroendocrine” cytologic features including plasmacytoid cells with finely dispersed (“salt-andpepper”) chromatin, eosinophilic granular cytoplasm, and eccentrically placed nuclei (Fig. 3.3.7)

   
   
4. 
   

   Mucinous cells and ciliated cells are not present

Special studies

Glands are negative for neuroendocrine markers and do not have biphasic immunostaining pattern

Positive for neuroendocrine markers synaptophysin, chromogranin, and CD56 (Fig. 3.3.8). Basal cells positive for p40 and CK5/6 but not for S100 or actin. Luminal cells positive for CK7

Treatment

Antimicrobials

Complete excision is curative

Prognosis

Recurrences common, and complications such as mastoiditis can occur if untreated

Good, but recurrences in approximately 15%. Rare examples have shown aggressive local growth