Adenoid cystic carcinoma

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Classification according to ICD-O-3
8200/3 Adenoid cystic carcinoma
ICD-O-3 first revision online

The adenoid cystic carcinoma is a rare malignant tumor derived from glandular tissues and occurs particularly in the head and neck area. The most common places of manifestation are the salivary glands ( parotid gland , mandibular salivary gland , small oral salivary glands). The tumor can also be observed in many other locations, such as the lacrimal gland , nose and paranasal sinuses , larynx , trachea , bronchi , lungs , skin , mammary gland , external ear canal , cervix , Bartholin's gland or prostate . Typical of adenoid-cystic carcinoma is an initially slow growth, although the tumor shows a pronounced tendency to infiltrate the surrounding tissue and therefore often cannot be completely removed and ultimately relapses .

term

Three years after Robin and Laboulbene first described the tumor entity, the surgeon Theodor Billroth coined the term cylindroma in 1856 , which alludes to the histologically recognizable, cylindrical connective tissue formations within the tumor tissue, which are surrounded by tumor cells. The dermal cylindroma , a benign tumor of the skin appendages, has to be distinguished from this today . The term adenoid-cystic carcinoma was first used by pathologist James Ewing and refers to the typical fine-tissue appearance of the tumor, which forms gland-like (adenoid) and sometimes wide, epithelial- lined (cystic) or non-epithelial-lined (pseudocystic) cavities. The term became generally in use with its inclusion in the WHO classification in 1972 following a proposal by Thackray and Sorbin.

Epidemiology

The age peak of the disease is in the 4th – 6th Decade of life, although the tumor can rarely occur in childhood. Women are affected slightly more often than men (3: 2).

etiology

The underlying causes of tumor formation are unknown. There is also currently no knowledge of genetic or environmental risk factors that favor the development of the disease. At the molecular genetic level, an inactivation of the p53 tumor suppressor gene has been demonstrated in advanced and aggressive forms of adenoid-cystic carcinoma. In a more recent study, the deletion of the chromosomal region 1p32-p36 was identified as the most common genetic abnormality in adenoid-cystic carcinoma.

pathology

Histological picture of adenoid-cystic carcinoma.

Adenoid-cystic carcinomas appear macroscopically as solid, well-circumscribed, but unencapsulated tumors of light brownish color and variable size. In terms of tissue, the tumor is composed of ductal and modified myoepithelial cells that develop tubular, cribriform and solid growth patterns. The tumor always grows infiltratively into the surrounding tissue and spreads in particular along nerve structures (perineural invasion).

diagnosis

A definitive diagnosis can only be made after removal of tumor tissue ( biopsy , fine needle aspiration ) and microscopic examination by the pathologist. Imaging methods such as computed tomography , magnetic resonance tomography or positron emission tomography are used in propagation diagnostics .

Differential diagnosis

The main entities that have to be distinguished from adenoid-cystic carcinoma are pleomorphic adenoma , polymorphic low-grade adenocarcinoma, basal cell adenoma or adenocarcinoma, and basaloid squamous cell carcinoma .

therapy

Therapy is usually based on the complete surgical removal of the tumor with a sufficient safety margin. Postoperatively, radiation therapy can reduce the relapse rate and is therefore recommended as the standard therapy in some studies. Effective chemotherapy for adenoid cystic carcinoma is not yet available. Combination therapies consisting of radiation and chemotherapy are currently being tested.

forecast

The slow but often unstoppable progression of adenoid-cystic carcinoma is reflected in survival rates. The 5-year survival rate is still relatively low at 89%, but drops to 65% after 10 years and to 40% after 15 years. The prognostically significant factors are the histological growth pattern, tumor location, tumor size, clinical stage, bone involvement and the status of the surgical resection margins. Tumors with a tubular or cribriform growth pattern often show a more favorable clinical course than tumors with more than 30% solid tissue patterns. Lymph node involvement is comparatively unusual with a relative frequency of 5 to 25%. Distant metastases, which affect the lungs, bones, brain and liver in decreasing frequency, occur in 25–55% of cases; only about 20% of the affected patients survive 5 years.

literature

  • Johannes Gerhard Heribert Laubenthal: Prognostic significance of clinical-therapeutic factors in adenoid cystic carcinoma of the head and neck area , dissertation, Saarbrücken 2017, doi: 10.22028 / D291-27217 .
  • Daniela Heine: Adenoid cystic carcinoma in the head and neck area - Cancer testis antigen expression and its prognostic and therapeutic relevance , dissertation, Ulm 2018, doi: 10.18725 / OPARU-5426 .

Web links

Individual evidence

  1. ^ JR Molina, MC Aubry, JE Lewis, JA Wampfler, BA Williams, DE Midthun, P. Yang, SD Cassivi: Primary salivary gland-type lung cancer: spectrum of clinical presentation, histopathologic and prognostic factors. In: Cancer , 2007 Nov 15; 110 (10), pp. 2253-2259.
  2. ^ FP Bernardini, MH Devoto, JO Croxatto. Epithelial tumors of the lacrimal gland: an update. In: Curr Opin Ophthalmol. 2008 Sep; 19 (5), pp. 409-413. PMID 18772674 .
  3. ↑ AD Lupinetti, DB Roberts, MD Williams, ME Kupferman, DI Rosenthal, F. Demonte, A. El-Naggar, RS Weber, EY Hanna: sinonasal adenoid cystic carcinoma: the MD Anderson Cancer Center experience. In: Cancer. 2007 Dec 15; 110 (12), pp. 2726-2731.
  4. ^ A. Del Negro, E. Ichihara, AJ Tincani, A. Altemani, AS Martins: Laryngeal adenoid cystic carcinoma: case report. In: Sao Paulo Med J. 2007 Sep 6; 125 (5), pp. 295-296. PMID 18094899
  5. ^ E. Albers, T. Lawrie, JH Harrell, ES Yi: Tracheobronchial adenoid cystic carcinoma: a clinicopathologic study of 14 cases. In: Chest. 2004 Mar; 125 (3), pp. 1160-1165.
  6. ↑ E. Naylor, P. Sarkar, CS Perlis, D. Giri, DR Gnepp, L. Robinson-Bostom: Primary cutaneous adenoid cystic carcinoma. In: J Am Acad Dermatol. 2008 Apr; 58 (4), pp. 636-641. PMID 18342709 .
  7. ^ YM Law, ST Quek, PH Tan, SL Wong: Adenoid cystic carcinoma of the breast. In: Singapore Med J. 2009 Jan; 50 (1), pp. E8 – e11. PMID 19224074
  8. ^ CP Carvalho, AN Barcellos, DC Teixeira, O. Sales Jde, R. Silva Neto: Adenoid cystic carcinoma of the external auditory canal. In: Braz J Otorhinolaryngol. 2008 Sep-Oct; 74 (5), pp. 794-796. PMID 19082366 .
  9. ↑ SA Koyfman, A. Abidi, P. Ravichandran, SA Higgins, M. Azodi: Adenoid cystic carcinoma of the cervix. In: Gynecol Oncol . 2005 Nov; 99 (2), pp. 477-480. PMID 16139349
  10. ↑ FM Woida, A. Ribeiro-Silva: adenoid cystic carcinoma of the Bartholin gland: an overview. In: Arch Pathol Lab Med . 2007 May; 131 (5), pp. 796-798. PMID 17488169
  11. ^ SK Halat, GT MacLennan: Adenoid cystic / basal cell carcinoma of the prostate. In: J Urol . 2008; 179, p. 1576. PMID 18295260
  12. ↑ a b c American Society of Clinical Oncology: Adenoid Cystic Carcinoma. on: cancer.net (07/2008)
  13. ↑ C. Robin, JJA Laboulbene: Mémoire sur trois productions morbides non décrites. In: Compte Rend Soc Biol. 1853; 5, pp. 185-196.
  14. ^ T. Billroth: The cylinder tumor. In: Investigations into the development of blood vessels, together with observations from the royal surgical university clinic in Berlin. G. Riemer, Berlin 1856, pp. 55-69.
  15. ^ Mosby's Medical Dictionary. 8th edition. Elsevier, 2009.
  16. ↑ a b c d e A. K. El-Naggar, AG Huvos: Adenoid cystic carcinoma. In: Genetics & Pathology, Head and Neck Tumours, Tumors of the Salivary Glands. IARCPress, 2000.
  17. ↑ P. Solisch: Morphological-cytological contribution to the biological peculiarity of the cylindroma. In: Arch Klin Exp Ear, nose and larynx healing. 1967; 189 (2), pp. 219-224.
  18. ^ AC Thackray, LH Sobin: Histological typing of salivary gland tumors. Geneva 1972.
  19. ↑ PH Rao, D. Roberts, YJ Zhao, D. Bell, CP Harris, RS Weber, AK El-Naggar: Deletion of 1p32-p36 is the most frequent genetic change and poor prognostic marker in adenoid cystic carcinoma of the salivary glands. In: Clin Cancer Res . 2008 Aug 15; 14 (16), pp. 5181-5187.
  20. ↑ JD Casler, Conley JJ: Surgical management of adenoid cystic carcinoma in the parotid gland. In: Otolaryngol Head Neck Surg. 1992; 106, pp. 332-338.
  21. ↑ K. Triantafillidou, J. Dimitrakopoulos, F. Iordanidis, D. Koufogiannis: Management of adenoid cystic carcinoma of minor salivary glands. In: J Oral Maxillofac Surg. 2006 Jul; 64 (7), pp. 1114-1120. PMID 16781345 .
  22. ↑ K. Terashima, Y. Shioyama, K. Nakamura, S. Ohga, S. Nomoto, T. Yamaguchi, T. Toba, T. Yoshitake, H. Yabuuchi, T. Nakashima, H. Terashima, H. Honda: Long -term local control of recurrent adenoid cystic carcinoma in the parotid gland with radiotherapy and intraarterial infusion chemotherapy. In: Radiat Med. 2006 May; 24 (4), pp. 287-291.
  23. ^ AS Garden, RS Weber, KK Ang, WH Morrison, J. Matre, LJ Peters: Postoperative radiation therapy for malignant tumors of minor salivary glands. Outcome and patterns of failure. In: Cancer. 1994 May 15; 73 (10), pp. 2563-2569. PMID 8174054