Idiopathic cystic chondromalacia, also known as endochondral pseudocyst of the auricle, is
a benign cystic degenerative lesion of the auricular cartilage. The lesion presents as a unilateral,
painless swelling along the upper half of the ear, usually in the area of the scaphoid or triangular
fossae adjacent to the helix. The disorder affects young to middle aged adult men more often than women.
Trauma is an associated, although not proven, etiologic factor.
Macroscopically, the lesion is a well-defined, cystic cavity within the auricular
cartilage, often filled with clear to yellow viscous fluid, described as similar to olive oil, which can
Microscopically, the skin surface is intact. The lesion is defined by an empty, irregularly shaped
cavity or cleft, most often in the central area of the cartilage. Because the space is not lined by an
epithelium it is called a "pseudocyst." Granulation tissue (a rich vascular proliferation with
erythrocytes, histiocytes, and mixed inflammatory cells) is present in most lesions, usually at the edge
of the cleft. Necrotic chondrocytes and the disappearance of elastic fibers may be seen at the edge of
the cavities. The remaining cartilage is generally unremarkable.
The clinical and histologic differential diagnosis includes relapsing polychondritis,
chondrodermatitis nodularis helicis chronica (CNCH) and Kimura disease.
Patients with relapsing polychondritis (RP; systemic chondromalacia or polychondropathy) present at
any age with one or more of the following progressive degenerative changes in the cartilages of the body:
recurrent bilateral auricular chondritis, inflammatory polyarthritis, nasal chondritis, ocular
inflammation, tracheal or laryngeal chondritis, and cochlea and/or vestibular damage. Other
immunologically mediated diseases may be associated with RP (systemic lupus erythematosus, rheumatoid
arthritis, Sjögren's syndrome). Although the gross findings are non-specific, there is an overall
thickening to the cartilages (ear, nose, epiglottis, cricoid and tracheal rings). There is a loss of
cartilage basophilia assuming a more eosinophilic quality. There is fragmentation of the cartilage with
necrosis and lysis of the cartilaginous plates. The outer perichondrium (lining of cartilage) is
permeated by a spectrum of inflammatory cells, including neutrophils, eosinophils, lymphocytes and plasma
cells, frequently associated with edema or gelatinous cystic degeneration, although not "true" cystic
degeneration. As the disease progresses, the degenerated fibrillar cartilage imperceptibly blends with
the surrounding inflammatory cells leading to granulation type tissue and fibrosis. Other disorders,
including sarcoid, infectious agents (tuberculosis, syphilis, various fungi, bacteria and viruses),
lymphoma, and Wegener's granulomatosis should be excluded. Obtaining cultures or performing
histochemical stains can help to rule out infectious organisms, while performing clinical tests for
cartilage matrix proteins or antibodies to type II collagen (found uniquely in cartilage) may help in
defining relapsing polychondritis. Corticosteroids or immunosuppressive agents are used to treat
patients depending upon the severity of the disease.
CNCH (also called Winkler's disease) affects men more commonly than women and usually in an older age
group at initial presentation. It usually presents as a painful, raised nodule on the superior helix
with central skin ulceration. Painful nodule affecting only one side (probably due to perichondrial
involvement), usually presenting as a raised discrete ovoid nodule about 1.5 cm in greatest dimension.
Vascular compromise and solar damage have been considered in the etiologic role. Histologically, there
is ulceration, hyperkeratosis, granulation tissue, and inflammation down to, but not including the
cartilage. Granulation tissue is identified at the base of the ulcer along with mixed inflammatory
cells. There may be involvement of the perichondrium, but not of the cartilage itself. Fibrinoid
necrosis can be seen. The treatment of choice is steroids or complete surgical excision.
Kimura disease is a rare form of chronic inflammatory disorder of unknown etiology involving
subcutaneous tissues, predominantly in the head and neck region and frequently is associated with
regional lymphadenopathy and/or salivary gland involvement. This condition has a predilection for males
of Asian descent and may clinically simulate a neoplasm. Most patients have peripheral blood
eosinophilia and elevated serum IgE levels. The solitary masses invariably are associated with a lymph
node and subcutaneous soft tissues. Characteristic histologic features of Kimura disease include
preserved nodal architecture, florid follicular hyperplasia with reactive germinal centers, eosinophilic
infiltrates, proliferation of post-capillary venules, sclerosis, polykaryocytes, proteinaceous deposits
in the germinal centers, eosinophilic folliculolysis, germinal center necrosis, prominent eosinophilic
microabscess, and IgE deposition in the germinal centers. Therefore, without a "lymph node" present,
Kimura disease can be effectively eliminated from consideration.
Curettage and pressure dressings have been used in the past, although excision or curettage is usually
the treatment of choice. Anteriolateral wall excision produces the best cosmetic result rather than
Ceruminal Gland Adenomas
Tumors arising from the ceruminal glands of the external ear canal can present a diagnostic dilemma
because of their varied clinical and histological manifestations. While common in animals, tumors of
this type are rare in humans and therefore are seldom seen by general surgical pathologists. Further
adding to the confusion for pathologists and clinicians alike, is the variable nomenclature used to
describe benign tumors of ceruminal gland origin: ceruminoma, ceruminal adenoma, syringocystadenoma
papilliferum, mixed tumor, apocrine adenoma, and cylindroma. The varied clinical behavior, treatment
alternatives, and long term patient prognosis of the different types of ceruminal gland neoplasms
suggests that "ceruminal" appear somewhere in the diagnosis to convey the anatomic site of origin and the
underlying tumor type.
There is an equal gender distribution with a wide age at initial presentation although the
6th decade seems to be the mean age at initial presentation. Patients most frequently present
with a mass lesion in the outer one-half of the external auditory canal accompanied by hearing changes
and occasionally pain. Symptoms will be present for a variably duration with an average of just over one
Since the ceruminal glands are only found in the outer one third to one half of the
external auditory canal, this is the location for these apocrine derived neoplasms. Therefore, it is
important to obtain an accurate location of the biopsy from the surgeon, to exclude glandular neoplasms
from the parotid gland or middle ear. As benign tumors, extension into the mastoid bone, middle ear, and
base of the skull is not identified. Given the confines of the canal, the tumors are about 1 cm in
Ceruminal adenomas are circumscribed, although not truly encapsulated. Surface
involvement can be seen, but ulceration often will obscure this relationship. These benign ceruminal
neoplasms can be divided into three major groups based on specific histologic findings: ceruminal
adenoma, ceruminal pleomorphic adenoma, and syringocystadenoma papilliferum. Ceruminal adenomas contain
neoplastic cells arranged in a variety of different patterns. Whereas a glandular pattern predominates
in most lesions, cystic spaces lined by a dual cell population are frequently noted. Ceruminal adenomas
demonstrate a dual cell population composed of a inner luminal epithelial cells subtended by
basal/myoepithelial cells adjacent to the basement membrane. The luminal cells frequently reveal
apocrine-type decapitation secretion and/or contained yellow-brown cerumen (wax) granules. These latter
two features assist enormously in confirming the ceruminal origin of the neoplastic cells. The tumors
tend to have a low to moderate cellularity composed of cells with mild to moderate nuclear pleomorphism.
Nucleoli can be found, necrosis is not seen, and rare mitotic figures can be counted. The presence of a
more abundant myoepithelial cell population juxtaposed next to areas of myxoid-chondroid matrix material
in the presence of ceruminal apocrine cells within the "duct-like" structures, confirms the diagnosis of
a ceruminal pleomorphic adenoma. The apocrine decapitation secretion and cerumen (wax) is usually
identified within the luminal cells of the duct-like structures of the pleomorphic adenomas. The
syringocystadenoma papilliferum is similar to its skin counterpart and is quite rare in this location.
The lesion cells usually react strongly and diffusely with keratin while only weakly and
focally with epithelial membrane antigen. The dual cell population can be accentuated by strongly
immunoreactive with CK7 in the luminal cells contrasted to strong and diffuse immunoreactivity with S-100
protein, CK5/6 and p63 in the basal-myoepithelial cells.
Paraganglioma, meningioma, schwannoma, middle ear adenoma and ceruminal gland adenocarcinoma are the
principle differential diagnostic considerations given the anatomic location of ceruminal gland
primaries. The "zellballen" architectural, slightly basophilic cytoplasm, nuclear pleomorphism, and
chromogranin/synaptophysin and S-100 immunoreactivity will define a paraganglioma. Meningioma has a
whorled or meningothelial growth, psammoma bodies, intranuclear cytoplasmic inclusions, and a single cell
population (EMA is weakly expressed as well). Schwannoma usually affects the cerebropontine angle and
not the external auditory canal, has palisading, a single cell population and a lack of glandular growth.
Middle ear adenoma can have a similar biphasic tumor growth with a number of patterns similar to
ceruminal adenoma. However, it arises within the middle ear, the nuclear chromatin distribution is
"neuroendocrine," there are no decapitation apocrine secretions, there is a lack of cerumen, and the
cells are immunoreactive with chromogranin and human pancreatic polypeptide. The most difficult
differential is with ceruminal gland adenocarcinoma, whether "not further specified" type or adenoid
cystic type. The ceruminal gland adenocarcinomas are much more cellular (sometimes difficult to assess
on a small biopsy), have a more solid and infiltrating growth, have increased mitotic figures, including
atypical forms, demonstrate moderate to severe nuclear pleomorphism, have prominent nucleoli in many
cells, develop tumor necrosis, and in general lack cerumen. While any one of these features of ceruminal
gland adenocarcinoma can be seen in ceruminal adenoma, it is the aggregate of the findings in either
entity that helps with the diagnosis.
While complete surgical removal of the tumor is ideal, it is frequently impossible due to
the complex anatomy of the ear and temporal bone. Therefore, in a small percentage of patients, residual
tumor may develop a recurrence. After additional surgery, the patients enjoy a disease free survival.
Table 1: Microscopic features in a recent review of 41 cases from the AFIP
|Growth pattern |
| Glandular ||37|
| Cystic ||32|
| Infiltrating ||11|
| Solid ||6|
| Papillary ||8|
|Dual Population ||39|
|Surface origin ||8|
|Mitotic figures (mean per 10 HPF) ||0.3|
|Atypical mitotic figures present ||0|
| Mild ||30|
| Moderate ||11|
| Severe ||0|
| Low ||17|
| Moderate ||20|
| High ||4|
|Prominent nucleoli ||16|
|Ceruminal apocrine decapitation secretion ||40|
|Necrosis present ||0|
|Cerumen/wax granules ||36|
|Associated findings: |
| Cholesteatoma ||2|
| Dense, sclerotic fibrosis ||6|
Neuroendocrine Adenoma of the Middle Ear—NAME (Middle ear adenoma)
Adenomas and carcinoid tumors of the middle ear are rare neoplasms with similar, if not
indistinguishable morphologies. First described in 1976 these tumors have been the subject of much
debate with regard to their similarities, differences, etiologies, and prognoses. Benign neuroendocrine
lesions of the middle ear have been described by many names, including carcinoid tumor, middle ear
adenoma, adenomatous tumor of the middle ear, adenocarcinoid, and amphicrine tumor, with carcinoid tumor
and middle ear adenoma being the most accepted. It is probably best to think of these tumors as a single
entity which display numerous different architectural patterns but similar cytomorphologic and
immunohistochemical reactivity patterns.
Arguments against the diagnosis of a carcinoid tumor within the middle ear have stemmed primarily from
its lack of paraneoplastic syndromes (i.e. "carcinoid syndrome") and lack of documented metastasis to
regional lymph nodes or distant sites. Paraneoplastic systemic alterations due to the elaboration of
hormonal products are a well-known feature of carcinoid tumors, especially the so-called "carcinoid
syndrome" consisting of flushing, diarrhea, sweating, wheezing, and abdominal pain. When systemic
changes are present, lung tumors tend to be large (>3.5 cm). The correlation of large tumor size to
increased levels of hormone elaboration is logical, although these large tumors also have a greater
frequency of liver metastasis that increases the chance of a paraneoplastic syndrome. The lack of
systemic changes associated with tumors of the middle ear may be a function of their relatively small
size (average <1.0 cm), since they do have immunoreactivity (although perhaps not systemic production)
for hormone products such as serotonin, ACTH, glucagon, HPP, and somatostatin. The lack of documented
metastasis of the tumors of the middle ear may be explained by the small size of the neoplasms and the
relative lack of vascularity of the middle ear cavity. There is no easily identifiable progenitor cell
within the middle ear, although an undifferentiated, pluripotential endodermal stem cell may still be
present which may give rise to these neoplasms.
Although the neuroendocrine features of this tumor are not adequately depicted by "Middle Ear
Adenoma", it is a correct description of the benign nature of the tumor and its location.
"Neuroendocrine Adenoma of the Middle Ear" (NAME) may be an excellent potential alternative as it
provides correct morphologic and behavioral depictions of this rare neoplasm.
Patients present in the 5th decade of life (average) with an equal gender distribution.
Clinical presentation includes decreased hearing acuity, fullness, and tinnitus as the most common
Patients present with unilateral disease. Most of the lesions are excised in a piecemeal fashion,
with an overall small size (< 1cm) due to the anatomic confines of the middle ear. The tumor by
definition occur within the middle ear and occasionally they may extend into the external auditory canal
or into the mastoid bone and eustachian tube. There is usually intimate association with the ossicular
chain. T umors are described macroscopically as avascular, soft or rubbery unencapsulated masses with a
variegated cut surface. The lesions tended to be gray-tan, brown-red, or pale yellow.
Tumors are unencapsulated and most are of moderate cellularity. The tumors are predominantly composed
of cuboidal-to-columnar cells with indistinct cytoplasmic borders. Flattened, irregular, plasmacytoid,
and spindled morphologies are frequently described. The cytoplasm is eosinophilic and homogenous to
finely granular. The nuclei tend to be round to oval with minimal pleomorphism. Moderate to severe
nuclear pleomorphism is only occasionally observed. The chromatin is distributed in a "salt-and-pepper"
pattern, most suggestive of a neuroendocrine origin, especially in the cells comprising the trabecular
and solid patterns and those comprising the external cellular layer of the ductal structures. The nuclei
are centrally or eccentrically placed, giving some cells a "plasmacytoid" appearance. Nucleoli are
inconspicuous and mitoses are essentially absent. Architectural patterns include glandular spaces,
trabeculae, festoons, ribbon-like patterns, anastomosing cords, and solid sheets with variable
cohesiveness. The architecture varies between tumors and even within the same tumorwith most tumors
displaying more than one pattern, although the glandular pattern predominates. The glandular pattern
consists of duct-like structures with focal "back-to-back" gland configuration. The ducts are lined by a
dual cell population composed of an inner (luminal), flattened, slightly more intensely eosinophilic cell
surrounded by a basally positioned cuboidal to short columnar cell. The glandular lumen are occasionally
filled with an amorphous, pink substance. Papillary structures are not identified. The "infiltrative"
pattern is characterized by small irregular groups and strands of cells in a moderately desmoplastic
stroma. This pattern give the illusion of tumor cells dissecting the collagen bundles in an uncontrolled
and aggressive fashion. The cells tend to be smaller than those within the other patterns with a higher
nuclear-to-cytoplasmic ratio, however, other features of true aggressiveness such as mitotic activity,
pleomorphism, necrosis, bone, perineural, or lymph-vascular space invasion are not noted. Definitive
surface derivation is not seen.
The tumor cells are immunoreactive with a variety of keratin antibodies, including cytokeratin
cocktail, CK7, and CAM 5.2. Specifically, CK7 immunoreactivity specifically highlights the inner
(luminal) layer of the glandular cells with a uniform and intense result, although it is only variably
present. Neuroendocrine marker immunoreactivity includes chromogranin, NSE, synaptophysin, serotonin,
glucagon, Leu-7, and human pancreatic polypeptide (HPP). Neuroendocrine markers are positive within the
basal cell layer of the glandular elements while more diffusely positive in the other architectural
patterns. Reactivity, however, is not uniform and varies both between cases and in level of intensity.
Electron microscopy demonstrated two distinct cell types, so-called Type A and Type B cells. The Type
A cells are noted within the apical aspect of the glandular lumina and rarely scattered within the solid
areas. These cells exhibit a flattened apical pole, and thin, elongated microvilli. Most significantly,
these cells demonstrate exocrine activity with apically oriented 275-600 nm secretory granules composed
of a flocculent dense core and an electron-lucent rim, representative of mucus granules. The granules
are occasionally noted to fuse with the apical cellular membrane, thereby discharging their secretory
product. The Type B cells are noted in the basal aspect of the glandular structures and are the
predominant cell within the solid, trabecular, and ribbon-like formations. They are most noteworthy for
their basally located 110-350 nm cytoplasmic dense-core granules neurosecretory granules that are
surrounded by a thin halo and distinct limiting membrane. Furthermore, a transitional form exhibiting
features of both Type A and Type B cells has also been described.
At surgery, the tumors are usually tightly adherent to the middle ear cavity, commonly encasing the
ossicular chain. Complete surgical removal of the neoplasm, to include encased ossicles (if present),
should be the treatment of choice. When the ossicular chain is involved but not removed, recurrence
(re-growth) of the lesion is much more likely. Perforation of the tympanic membrane with extension into
the external auditory canal is an occasional feature. However, destruction of or invasion into the
surrounding bone has only rarely been reported, is not extensive in nature, and tends to bony erosion
rather than destruction. The recurrence rate for these neuroendocrine neoplasms of the middle ear is
quite small (<15%). When they do recur, it is usually related to re-growth of an incompletely excised
tumor as opposed to an inherent biologic aggressiveness. Even if facial paralysis is identified, it does
not seem to portend an adverse clinical outcome. These findings suggest mass-related compression of the
facial nerve rather than direct neural invasion. In summary, if one defines "malignancy" by clinical
aggressiveness and potential for metastatic spread, then the tumors of the middle ear should be regarded
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