Radiating on the Edge: A Rare Case of Adenoid Cystic Carcinoma of the Lacrimal Gland Treated by Eye-Sparing Surgery and Radiation Therapy

Special Article - Radiation Oncology

Ann Carcinog. 2017; 2(1): 1009.

Radiating on the Edge: A Rare Case of Adenoid Cystic Carcinoma of the Lacrimal Gland Treated by Eye-Sparing Surgery and Radiation Therapy

Oertel M¹*, Reinartz G¹, Barth PJ², Kittel C¹ and Eich HT¹

¹Department of Radiation Oncology, Albert-Schweitzer- Campus, Germany

²Department of Pathology, Albert-Schweitzer-Campus, Germany

*Corresponding author: Oertel Michael, Department of Radiation Oncology, Albert-Schweitzer-Campus 1 building A1, 48149 Muenster, Germany

Received: May 05, 2017; Accepted: May 30, 2017; Published: June 08, 2017


About 1/3 of epithelial malignancies of the lacrimal gland are adenous cystic carcinoma making it a frequent malignant tumor only outstripped by lymphoma. Despite that fact, no standard treatment is established so far, but surgery and radiation therapy are used with good results, with the extent of surgery being a controversial issue. Concerning radiooncology, different doses and fractionation schemes have been used in the past. We present a case of an adenoid cystic carcinoma of the left lacrimal gland successfully treated by postoperative radiation therapy.

After a case presentation, we focus on a literature review on the role of radiation therapy in lacrimal adenoid cystic carcinoma.

Keywords: Orbital malignancies; Adenoid cystic carcinoma; Lacrimal tumor; Radiotherapy


ACC: Adenoid Cystic Carcinoma; GTV: Gross Tumor Volume; IMRT: Intensity-Modulated Radiotherapy; PTV: Planning Target Volume

Case Presentation

Adenoid Cystic Carcinomas (ACC) represents the most frequent carcinoma of the lacrimal gland with a percentage of 32% [1]. Symptoms may vary and comprise loss of vision, propoptosis, diplopia, eye lid swelling, epiphora and pain [2,3].

Local therapy as operation or radiation therapy is complicated by the orbital anatomy involving the second, third and sixth cranial nerve and essential structures for ocular function as the lacrimal gland, external and internal muscles, lens and the retina [4]. The following case illustrates the interdisciplinary approach to a rare tumor entity and the considerations made for preserving the orbital structures functionally.


A 25-young gentleman was admitted to our institution with an impaired field of vision nasal-inferior on the left side. Ophthalmic examination showed a vision of 0.4 and a prominent tumor in the superior-temporal quadrant of the left orbita. In the initial MRI, a left bulb protrusion due to a sharp demarcated tumor in the left upper quadrant (2.8*2.5*2.1 cm) was noticed and thought to be a pleomorphic adenoma.

The patient underwent resection via a lateral, cranial transosseus orbitotomy. Intraoperatively, the tumor was found to be superficially and capsulated and was consequently removed, supposedly completely.

Pathological examination revealed an ACC of the lacrimal gland (Figure 1). A focal perineural invasion was found and the resection margins were infiltrated broadly by tumor cells. Subsequently, the carcinoma was staged as pT2, pNx, cMx, L0, V0, Pn1, R1 [5].