Background Ceruminal gland carcinomas are rare neoplasms confined to the skin lining the cartilaginous part of the external auditory canal. Design Retrospective. Results The patients included 11 men and 6 women, aged 33–82 years (mean, 59.5 years). Patients presented clinically with a mass of the outer half of the external auditory canal (n = 14), hearing changes (n = 5), drainage (n = 4), or paralysis of the facial nerve (n = 3). The polypoid masses ranged in size from 0.5 to 3 cm in greatest dimension (mean, 1.8 cm). Histologically, the tumors demonstrated a solid to cystic pattern, composed of an infiltrating glandular to cribriform arrangement of epithelial cells. Histologic features included a dual cell population (although not the dominant histology), increased cellularity, moderate to severe nuclear pleomorphism, irregular nucleoli, increased mitotic figures (mean, 3/10 HPF), including atypical forms, and tumor necrosis (n = 2). Tumors were divided into three types of adenocarcinoma based on pattern of growth and cell type (ceruminous, NOS [n = 12], adenoid cystic [n = 4], mucoepidermoid [n = 1]). CK7 and CD117 highlighted the luminal cells, while S1-00 protein showed a predilection for the basal cells of ceruminous and adenoid cystic carcinomas. Metastatic adenocarcinoma or direct extension from salivary gland neoplasms are the principle differential considerations. Surgical resection was used in all patients with radiation used in four patients. Eleven patients were alive or had died of unrelated causes without evidence of disease (mean, 11.2 years); six patients had died with disease (mean, 4.9 years), all of whom had developed local recurrence. Conclusion Ceruminous-type carcinomas, with the exception of ceruminous mucoepidermoid carcinoma, all demonstrated a dual cell population of basal myoepithelial-type cells and luminal apocrine cells. The specific histologic sub-type does not influence the long-term patient outcome.
Benign fibro-osseous lesions (BFOLs) are a particularly challenging set of diagnoses for the pathologist. This diverse collection of diseases includes fibrous dysplasia, ossifying fibroma and cemento-osseous dysplasia. While all three conditions have similar microscopic presentations, their treatment and prognosis differ, demanding an accurate and definitive diagnosis. A practical and systematic approach considering the patient's history, demographics, intraoperative presentation, and gross appearance with an emphasis on radiology and histology will be discussed.
Ceruminous gland adenomas are the most common external auditory canal tumors. They demonstrate a dual cell population of basal myoepithelial-type cells and luminal ceruminous (ceruminal) cells. Cerumen pigment, CK7, and p63 can help to distinguish this tumor from other neoplasms that occur in the region. Complete surgical excision results in an excellent long-term clinical outcome.
Primary sinonasal tract mucoepidermoid carcinomas (MEC) are uncommon tumors that are frequently misclassified, resulting in inappropriate clinical management. The design of this study is retrospective. Nineteen cases of MEC included 10 females and 9 males, aged 15-75 years (mean, 52.7 years); males, on average were younger by a decade than females (47.2 vs. 57.7 years). Patients presented most frequently with a mass, obstructive symptoms, pain, and/or epistaxis present for a mean of 12.6 months. The majority of tumors involved the nasal cavity alone (n=10), maxillary sinus alone (n=6), or a combination of the nasal cavity and paranasal sinuses (n=3) with a mean size of 2.4 cm. Most patients presented at a low clinical stage (n=15, Stage I & II), with only 4 patients presenting with Stage III disease. Histologically, the tumors were often invasive (bone or perineural invasion), with invasion into minor mucoserous glands. Surface involvement was common. The neoplastic cells were composed of a combination of squamoid cells, intermediate cells, and mucocytes. Cystic spaces were occasionally large, but the majority were focal to small. Pleomorphism was generally low grade. Necrosis (n=5) and atypical mitotic figures (n=6) were seen infrequently. Over half of the tumors were classified as low grade (n=11), with intermediate (n=4) and high grade (n=4) comprising the remainder. Mucicarmine was positive in all cases tested. Immunohistochemical studies showed positive reactions for keratin, CK5/6, p63, CK7, EMA, and CEA in all cases tested, while bcl-2 and CD117 were rarely positive. GFAP, MSA, TTF-1, and S100 protein were non-reactive. p53 and Ki-67 were reactive to a variable degree. MEC need to be considered in the differential diagnosis of a number of sinonasal lesions, particularly adenocarcinoma and necrotizing sialometaplasia. The patients were separated into stage I (n=9), stage II (n=6), and stage III (n=4), without any patients in stage IV at presentation. Surgery occasionally accompanied by radiation therapy (n=2) was generally employed. Six patients developed a recurrence, with 5 patients dying with disease (mean, 2.4 years), while 14 patients are either alive (n=9) or had died (n=5) of unrelated causes (mean, 14.6 years). MEC probably arises from the minor mucoserous glands of the upper aerodigestive tract, usually presenting in patients in middle age with a mass. Most patients present with low stage disease (stage I and II), although invasive growth is common. Recurrences develop in about a third of patients, who experience a shorter survival (mean, 6.5 years). The following parameters, when present, suggest an increased incidence of recurrence or dying with disease: size ≥ 4.0 cm (P=0.034), high mitotic count (P=0.041), atypical mitoses (P=0.007), mixed anatomic site (P=0.032), development of recurrence (P=0.041), high tumor grade (P=0.007), and higher stage disease (P=0.027).
The keratocystic odontogenic tumor is a benign developmental tumor with many distinguishing clinical and histologic features. These characteristics are reviewed in the setting of a typical presentation. The newly acknowledged neoplastic potential and its implications for treatment strategies are also discussed. HistoryA 20-year-old male presented for a routine dental exam with no complaints. Panoramic radiograph films were performed in accordance with standard procedures. A radiolucent lesion was identified in the right maxilla and presumed to be a dentigerous cyst. With this working diagnosis, the lesion was curetted and submitted for microscopic evaluation.
A case of compound odontomas of the anterior maxilla is discussed. Multiple tooth like structures were discovered upon surgical excision.
Sinonasal tract angiosarcoma is a rare tumor, frequently presenting in middle-aged patients as a large mass usually involving the nasal cavity with characteristic histomorphologic and immunophenotypic features. Sinonasal tract angiosarcoma will often have a poor prognosis making appropriate separation from other conditions important.
External auditory canal (EAC) osteomas are rare, benign bony neoplasms that occur in wide range of patients. While chronic irritation and inflammation have been suggested as causal factors in several cases, significant data is lacking to support these suspicions. Symptoms are rare and can include hearing loss, vertigo, pain and tinnitus. Diagnosis is made based on a combination of clinical history and examination, radiographic imaging, and histopathology. Osteomas of the EAC are usually found incidentally and are unilateral and solitary. Computed tomography reveals a hyperdense, pedunculated mass arising from the tympanosquamous suture and lateral of the isthmus. Histopathologically, EAC osteomas are covered with periosteum and squamous epithelium, and consist of lamalleted bone surrounding fibrovascular channels with minimal osteocysts. Osteomas have historically been compared and contrasted with exostoses of the EAC. While they share similarities, more often than not it is possible to distinguish the two bony neoplasms based on clinical history and radiographic studies. Debate remains in the medical literature as to whether basic histopathology can distinguish osteomas of the EAC from exostoses. Surgical excision is the standard treatment for EAC osteomas, however close observation is considered acceptable in asymptomatic patients.Keywords Osteoma Á External auditory canal Á Exostosis Á Radiology Á Pathology HistoryA 28-year-old male was referred to the Otorhinolaryngology clinic for a left ear mass that was incidentally found during a routine audiology evaluation. The patient related that his primary care physician had first noticed the lesion 2 years prior. He denied a history of trauma to the ear or hearing loss, but stated recurrent left-sided otitis media as well as frequent cerumen impactions. Clinical examination revealed a hard, irregularly shaped mass of the left anterior auditory canal measuring approximately 2.0 mm. The mass and surrounding skin were normal in color and no surface ulcerations were noted. The right ear showed no abnormalities. The patient's health history was otherwise unremarkable and he denied participating, more than occasionally, in water sports. Radiographic FeaturesA non-contrast computerized tomographic (CT) scan of the skull base and temporal bones was obtained using 1 mm axial and coronal slices. The CT scan was remarkable for a non-obstructing, partially pedunuclated, hyperdense mass of the left anterior external auditory canal (Fig. 1). Review of the sections demonstrated the mass originated at the tympanosquamous suture (Fig. 2). The mass measured 1.0 cm by 0.6 cm, which was best appreciated on the axial images. The remainder of the structures of the middle and external ear were unremarkable, and the right ear showed no pathologic changes.
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