8200/3 Cylindroma, NOS



Definitions

Breast
ICD-O-3 topography code: C50
ICD10: C50
           

A carcinoma of low malignant potential, histologically similar to its counterpart in the salivary gland.





Lip, Oral Cavity and Oropharynx
ICD-O-3 topography code: C00-C06, C09-C10, C14
ICD10: C00-C06, C09-C10, C14
           





Esophagus
ICD-O-3 topography code: C15
ICD10: C15
           

This esophageal neoplasm is infrequent and believed to arise, like the mucoepidermoid variant, from oesophageal glands 4
Click to access Pubmed
Karaoglanoglu N, Eroglu A, Turkyilmaz A, Gursan N (2005)
Oesophageal adenoid cystic carcinoma and its management options.
Int J Clin Pract 59: 1101-3



. Both lesions tend to be of salivary gland type with a generally favourable prognosis 5
 
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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Skin
ICD-O-3 topography code: C44
ICD10: C44
           

Primary cutaneous adenoid cystic carcinoma (ACC) is a rare, slow-growing adnexal neoplasm characterized by an indolent but progressive course. AAC arises in the dermis, most frequently of the scalp, followed by chest and abdomen, and may extend into the subcutaneous fat. The tumour typically presents as a firm, poorly circumscribed nodule measuring between 0.5 and 8 cm (average 3.2 cm). It is composed of infiltrative nests and cords of basaloid cells arranged in glandular, cystic, cribriform and tubular patterns. The cystic spaces are often filled with mucin. Perineural involvement, a characteristic feature of ACC in other organs, is not as frequently observed in the skin.
Primary cutaneous ACC affects middle-aged and elderly patients (mean age: 58 years) and shows a predilection for women. The tumour recurs in up to 70% of cases; wide surgical excision is therefore recommended
6
 
LeBoit PE, Burg G, Weedon D, Sarasin A (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Skin Tumours
3rd Edition
IARC Press: Lyon 2005



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Hypopharynx, Larynx and Trachea
ICD-O-3 topography code: C13, C32, C33
ICD10: C13, C32, C33
           





Salivary glands
ICD-O-3 topography code: C07-C08
ICD10: C07-C08
           





Cervix uteri
ICD-O-3 topography code: C53
ICD10: C53
           

Adenoid cystic carcinoma is characterized by cystic spaces filled with hyaline material or mucin and palisaded by epithelial cells. The neoplastic cells show nuclear pleomorphism and a high mitotic rate. Necrosis is common.
Cervical adenoid cystic carcinoma is rare. It predominantly affects women >60 years of age. Patients typically present with postmenopausal bleeding. Local recurrences as well as distant metastases are frequent. The prognosis is unfavourable
7
 
Tavassoli FA, Devilee P (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Breast and Female Genital Organs.
3rd Edition
IARC Press: Lyon 2003



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Bronchus and lung
ICD-O-3 topography code: C34
ICD10: C34
           





Ovary
ICD-O-3 topography code: C56
ICD10: C56
           





Nasal cavity and Sinuses
ICD-O-3 topography code: C30-C31
ICD10: C30-C31
           





Vulva
ICD-O-3 topography code: C51
ICD10: C51
           

Adenoid cystic carcinoma is a slowly growing, locally very aggressive tumour. It is typically composed of uniform malignant epithelial cells arranged in nests and columns around glandlike spaces. The growth pattern is cribriform. The stroma is hyaline and may form cylinders between rows of tumour cells.
In the vulva, primary adenoid cystic carcinoma is very rare. It can arise at the site of the Bartholin gland, resulting in an enlargement of the Bartholin gland area that may resemble a Bartholin duct cyst. Patients are predominantly older women >50 years of age. Approximately 20% of patients have ipsilateral inguinofemoral lymph node metastases at presentation. The tumour has a high tendency to recur
8
Click to access Pubmed
Copeland LJ, Sneige N, Gershenson DM, Saul PB, Stringer CA, Seski JC (1986)
Adenoid cystic carcinoma of Bartholin gland.
Obstet Gynecol 67: 115-20



9
Click to access Pubmed
DePasquale SE, McGuinness TB, Mangan CE, Husson M, Woodland MB (1996)
Adenoid cystic carcinoma of Bartholin's gland: a review of the literature and report of a patient.
Gynecol Oncol 61: 122-5



10
Click to access Pubmed
Woida FM, Ribeiro-Silva A (2007)
Adenoid cystic carcinoma of the Bartholin gland: an overview.
Arch Pathol Lab Med 131: 796-8



11
 
Tavassoli FA, Devilee P (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Breast and Female Genital Organs.
3rd Edition
IARC Press: Lyon 2003



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Vagina
ICD-O-3 topography code: C52
ICD10: C52