Adenoid cystic carcinoma of salivary and lacrimal gland origin: localization, classification, clinical pathological correlation, treatment results and long-term follow-up control in 84 patients

Anticancer Res. 2003 Mar-Apr;23(2A):931-40.


Aim: Adenoid cystic carcinoma (ACC) is a malignant tumor arising from glands. In the maxillofacial region, the salivary glands are particularly affected by ACC and, less frequently, the lacrimal glands. The aim of this study was to determine the outcome of patients with ACC in the maxillofacial region classified according to an internationally accepted staging system.

Materials and methods: Over a period of more than 31 years, 84 patients with ACC underwent surgical treatment or a combined treatment in a University Hospital (primaries: 70; local recurrence: 13; distant metastasis: 1).

Results: In salivary glands the major glands were affected in 20 individuals, the minor glands in 50, while the glands of the maxillary sinus gave rise to ACC in 8. The lacrimal glands were affected in 6 patients [45 females (53.6%), 39 males (46.4%); age: 13 to 93 years, mean 55.9 years]. Reclassified TNM-stage (UICC, 1987) at the time of diagnosis varied (T0:1, T1:14, T2:13, T3:12, T4:30, NX:1, N0:53, N1:10, N2:5, N3:0, MX:5, M0:59, M1:5). The history of symptoms ranged from 1.82 to 7.3 years, depending on the localization, without any specificity of features. However, swelling and pain were the most frequently recorded findings (> 73%). The therapy of choice has to be the resection of the primary tumor with safety margins. The resection status is important for determining the local control. The resection of the related efferent lymphatics has to be included in the therapeutic concept in patients suspected of having metastasis of the regional lymph nodes. However, hematogenic spread was frequently recorded in our patients, even after several years. An excellent prognosis is only in ACC which is detected early and radically resected. Neither localisation nor the histological subtypes, but tumor stage, had statistically significant impact on prognosis. The differences in survival between surgically-treated patients and those who received radiotherapy as an adjunct were not significant.

Conclusion: The TNM classification and the analysis of histopathological subtypes provide some information on the tumor biology. The prognosis is uncertain in ACC. Improvement of the current diagnostics and therapy hopefully will come from the development of tumor markers and future molecular genetic investigations.

MeSH terms

  • Adult
  • Age Distribution
  • Age of Onset
  • Aged
  • Carcinoma, Adenoid Cystic / classification
  • Carcinoma, Adenoid Cystic / mortality
  • Carcinoma, Adenoid Cystic / pathology*
  • Carcinoma, Adenoid Cystic / therapy
  • Combined Modality Therapy
  • Eye Neoplasms / classification
  • Eye Neoplasms / mortality
  • Eye Neoplasms / pathology*
  • Eye Neoplasms / therapy
  • Female
  • Follow-Up Studies
  • Humans
  • Lacrimal Apparatus* / pathology*
  • Male
  • Medical Records
  • Middle Aged
  • Neoplasm Metastasis
  • Neoplasm Recurrence, Local
  • Neoplasm Staging
  • Retrospective Studies
  • Salivary Gland Neoplasms / classification
  • Salivary Gland Neoplasms / mortality
  • Salivary Gland Neoplasms / pathology*
  • Salivary Gland Neoplasms / therapy
  • Survival Analysis
  • Time Factors