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Cutaneous adenoid cystic carcinoma

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Adenoid cystic carcinoma of the external auditory canal (EAC) is a very rare malignant tumour and its diagnosis is frequently missed because of the superficial nature of biopsies.

Adenoid cystic carcinomas of the ceruminous gland are morphologically similar to the more commonly encountered salivary gland tumors.

 As do their more common salivary gland analogs, adenoid cystic carcinomas of the ceruminous glands have a propensity for perineural growth.

This tendency accounts for most cases of intracranial involvement by adenoid cystic carcinomas of the ceruminous gland, as the neoplasm grows along the nerves and by direct extension involves the central nervous system.

It has been proposed for salivary gland neoplasms that the propensity for perineural involvement is related to the expression of neural cell adhesion molecules by the neoplastic tumour cells.

Treatment of adenoid cystic carcinomas of the ceruminous gland  involves complete surgical resection with clear surgical margins.

This objective can be difficult to achieve because of the perineural involvement commonly encountered in these neoplasms.

In the ceruminous gland, cytokeratin shows diffuse positive staining, while myoepithelial cells are stained for smooth muscle actin, desmin, S-100 protein and vimentin.

Adenoid cystic carcinoma in external auditory canal grows slowly, so patients can live with the tumour for a long time. 

It is necessary to do both aggressive surgery and postoperative irradiation.

The long term follow-up indicates that the tumour has a poor prognosis.

                  

Adenoid cystic carcinoma of the external auditory canal.Ann Otolaryngol Chir Cervicofac. 2007 May 21;

OBJECTIVES: To report a case of adenoid cystic carcinoma of the external auditory canal with petrous involvement. MATERIAL AND METHODS: A 50-year-old woman was referred to our department for the management of a necrotizing external otitis. Computed tomography and biopsy revealed an adenoid cystic carcinoma of the external auditory canal. RESULTS: The patient was treated by complete surgical excision. A radiation therapy completed the treatment. Follow-up time was 19 months without evidence of recurrence. Adenoid cystic carcinoma of the external auditory canal is a rare tumor. It's a special type of carcinoma developing from the ceruminous glands. Histogenesis is difficult and unsettled. The most important survival factor is removal of the tumor with histologically free margins. CONCLUSIONS: Lack of specific clinical and radiological signs makes the diagnostic challenging. The adenoid cystic carcinoma is a rare neoplasm of the external auditory canal. An initial aggressive wide "en bloc" surgical resection is mandatory.

Six cases of adenoid cystic carcinoma in external auditory canal.Lin Chuang Er Bi Yan Hou Ke Za Zhi. 2006 Feb;20(4):158-9.

OBJECTIVE: By investigating the clinical and pathological features of adenoid cystic carcinoma in external auditory canal, we can improve our ability of diagnosis and treatment. METHOD: A retrospective analysis was performed on 6 patients with adenoid cystic carcinoma in external auditory canal, including clinical manifestations, the therapy as well as the follow-up results. All cases underwent subtotal temporal bone resection or other aggressive surgical encision. RESULT: All cases were alive for a period from 8 to 21 months after subtotal temporal bone resection or other aggressive surgical excision, without any signs of regional lymph node metastasis and distal metastasis. CONCLUSION: Adenoid cystic carcinoma in external auditory canal grows slowly, so patients can live with the tumor for a long time. But the long-term prognosis is poor. It is necessary to manipulate aggressive surgical excision at the first time to improve cure rate.

Adenoid cystic carcinoma of ceruminous gland: a case report.Indian J Pathol Microbiol. 2006 Oct;49(4):587-9.

Adenoid cystic carcinoma of the external auditory canal (EAC) is a very rare malignant tumor and its diagnosis is frequently missed because of the superficial nature of biopsies. A 30 year old male presented with 6 months history of recurrent painful right ear discharge. On examination there was a nodule of 2 cms in diameter. The microscopic examination of the punch biopsy specimen of tumor revealed typical features of an adenoid cystic carcinoma. While reporting the biopsies of external auditory canal one should keep the adenoid cystic carcinoma as one of the possible differentials, especially if the biopsy is superficial, problem is recurrent and histopathological features suggest the granulation tissue.

Adenoid cystic carcinoma of external auditory canal: a report of 7 cases.Lin Chuang Er Bi Yan Hou Ke Za Zhi. 2004 Apr;18(4):202-3.

OBJECTIVE: To detect the clinical and pathological features of the adenoid cystic carcinoma of external auditory canal, and to improve its diagnosis and treatment level. METHOD: A retrospective analysis was performed on 7 patients with ACCEAC by their clinical manifestation, the therapy and follow-up results as well as the combination with the review of related literature. RESULT: The period from the appearance of primary symptoms to definite diagnosis averaged 5.7 years. All patients were treated by surgical excision and followed up from 2 years 8 months to 9 years 11 months. Five of them were recurrent after operation, 2 of which died. CONCLUSION: Adenoid cystic carcinoma of external auditory canal, which is rarely seen in clinic, is not easy to exact diagnosis in early stage. It progresses slowly and tends to invade parotid and even intracranial cavity. It also has the tendency of recurrence and pulmonary metastasis. So, its necessary to do both aggressive surgery and postoperative irradiation. The long term follow-up indicates that the tumor has a poor prognosis.

A case of highly aggressive adenoid cystic carcinoma of the external auditory canal.Acta Otorhinolaryngol Ital. 2004 Dec;24(6):354-6.

Tumours of the external auditory canal are extremely rare and only 20% of these are of glandular origin. The most frequent histotype is adenoid cystic carcinoma. The rarity of external auditory canal glandular tumours explains the lack of large series reported in the literature and the corresponding large number of case reports from different Authors. Adenoid cystic carcinoma, presenting in the external auditory canal, exhibits the same characteristics as those affecting the major salivary glands, this tumour has an aggressive behaviour characterized by local invasivity and with a metastatic risk of approximately 30%. A rare case of adenoid cystic carcinoma of the external ear is reported. The patient, a 75-year-old male, had right intermittent otorrhea for 6 years. On examination, a vegetating, ulcerated formation which easily bled was found protruding from the right external auditory meatus. Clinical, radiological and pathological features of the tumour are described. A subtotal petrosectomy combined with homolateral elective lymph node neck dissection was performed. Parotid gland, condyle of the mandible and VII cranial nerve were spared since these were free from disease.

Ceruminous gland adenoid cystic carcinoma with contralateral metastasis to the brain. Arch Pathol Lab Med. 2002 Jan;126(1):87-9.

We present the case of a 38-year-old man with an adenoid cystic carcinoma originating from the ceruminous glands of the external ear canal. The patient subsequently presented with a contralateral brain mass that was also diagnosed as adenoid cystic carcinoma. To our knowledge, contralateral metastasis to the brain of a patient with an adenoid cystic carcinoma of the ceruminous glands has not been reported previously. This rare neoplasm should be considered in the differential diagnosis of poorly differentiated carcinomas metastatic to the central nervous system in patients with occult malignant neoplasms.

An immunohistochemical study of adenoid cystic carcinoma of the external auditory canal.Eur Arch Otorhinolaryngol. 1993;250(4):240-4.

Specimens from five cases of adenoid cystic carcinoma of the external auditory canal were studied by immunohistochemical staining, and findings were compared with those from adjacent non-neoplastic tissues containing ceruminous glands. In the ceruminous gland, cytokeratin showed diffuse positive staining, while myoepithelial cells were stained for smooth muscle actin, desmin, S-100 protein and vimentin. The epithelial markers used were cytokeratin, carcinoembryonic antigen and secretory component and stained at various densities the inner cells of the tubular component and duct-lining cells in the cribriform component of tumor tissues. In contrast, the muscular markers, smooth muscle actin and desmin, and the mesenchymal marker, vimentin, were positive in the outer cells of the tubular component and in the cyst-lining cells of the cribriform component. S-100 protein immunoreactivity showed paradoxical results; positive findings occurred in the myoepithelial cells of the ceruminous glands and in the inner cells of the tubular component and duct-lining cells of the cribriform component of the tumor. Present findings demonstrate that adenoid cystic carcinoma of the external auditory canal had dual epithelial and myoepithelial differentiation and can mimic the ceruminous glands of the auditory canal.

Adenoid cystic carcinoma involving the external auditory canal: a clinicopathologic study of 16 cases. Cancer 1982;50:2873–2883.

Sixteen patients with a rare tumor, adenoid cystic carcinoma (ACC) involving the external auditory canal, have been studied. Clinically, most patients complained of ear pain, often of several years duration. On physical examination, a mass or a nodule usually was identified in the ear canal. In most cases, treatment consisted of a wide surgical resection of the auditory canal and adjacent structures. Histologically, these neoplasms had the same appearance as ACC originating in salivary glands. In eight cases, the tumor was confined to the ear canal wall and unquestionably arose in this area, probably within the ceruminous glands. When incompletely excised, these lesions usually recurred locally. Nine patients had a total of 26 local recurrences. Most recurrences were found within two years of the preceding treatment, but the interval in some cases was long, ranging up to 14 years. Of the 16 patients, seven had no evidence of recurrence following surgical resection, two were living with recurrent, unresectable tumor, five had died of disease, one had died of other causes, and one was lost to follow-up. Some patients died of tumor after a prolonged clinical course with multiple recurrences. Death usually was caused by intracranial extension by the tumor, or by pulmonary metastases. Radiotherapy did not appear to cure the lesion, but probably resulted in palliation. An increased incidence of recurrent, unresectable tumor or of death from the neoplasm correlated with the following histologic features: demonstration of tumor on the lines of surgical excision, involvement of the parotid gland, extension into bone, perineural invasion and local recurrence of tumor.

 
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