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Trachea

Tumors

Adenoid cystic carcinoma


Reviewer: Adriana Handra-Luca, M.D. (see Reviewers page)
Revised: 8 February 2013, last major update October 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
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● #2 most common primary malignancy of trachea after squamous cell carcinoma
● Resembles salivary gland tumor

Clinical features
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● Usually in upper third of trachea
● Intramural (15%) or extramural (85%)
● Symptoms: dry cough, dyspnea, hemoptysis
● May coexist with main bronchus lesion
● 20% invade locally (including thyroid gland); may metastasize to lymph nodes, nasal cavity, liver
● Lengthy clinical course, but poor long term prognosis due to late local recurrences
● Median overall surival 18 years, median disease free survival 10 years
● 75% survival rate if complete resection
● Predictors of survival: margin status, extramural disease, perineural invasion, lymph node metastases (Eur J Cardiothorac Surg 2010;37:1438, Ai Zheng 2004;23:581)

Case reports
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● 44 year old woman with midline neck swelling mimicking thyroid tumor (Lung India 2010;27:167)
● 52 year old woman with tracheal primary and liver metastases (Gut Liver 2009;3:127)
● 63 year old woman with metastasis to nasal cavity (Ear Nose Throat J 2009;88:E9)
● 79 year old woman treated with curettage and radiation therapy (South Med J 2011;104:68)
● Metastatic to placenta (Hum Pathol 1989;20:193)

Treatment
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● Chemotherapy, radiotherapy, surgery (Head Neck 2012;34:1263)

Micro description
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● Cribriform growth pattern of nests of tumor cells with discrete, rounded, “punched-out” gland-like spaces filled with eosinophilic or basophilic material
● Infiltrative, perineural invasion common

Micro images
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Uniform hyperchromatic basaloid cells surrounding acellular spaces containing mucoid and hyaline material


Tubular structures with microcysts surrounding hyalinized stroma

Cytology description
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● Rarely extends to thyroid and presents as thyroid nodule (Am J Clin Pathol 2004;121:551)

Cytology images
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Spherical globules of basement membrane material and hyperchromatic uniform rounded tumor cells with scanty cytoplasm (May Grunwald Giemsa stain)

Positive stains
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● Brain derived neurotrophic factor (Hum Pathol 2002;33:933)

Differential diagnosis
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● Thyroid tumor

End of Trachea > Tumors > Adenoid cystic carcinoma


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