Adenoid cystic carcinoma of the lacrimal gland metastasising to the liver: report of a case
© Zeidan et al. 2006
Received: 02 April 2006
Accepted: 20 September 2006
Published: 20 September 2006
Adenoid Cystic Carcinoma of the lacrimal gland is a rare tumour. Their aggressive behaviour, with a high-risk of local recurrence, and late distant spread of the tumour even after aggressive management has been reported. Metastasis to the liver is rare and when it occurs, it is usually part of widespread metastasis, and therefore surgical treatment is seldom considered.
We report a rare case of an isolated liver metastasis from a lacrimal gland adenoid cystic carcinoma 20 years after resection of the primary tumour. The patient presented with right upper quadrant pain radiating to the back and shortness of breath of 3 months duration. No local recurrence was detected during a 15 year follow-up with computerized tomography (CT) of the head. Abdominal CT scan demonstrated a solitary liver tumour with no other primary source, and the bone scan was normal. The patient was treated with an extended right hemihepatectomy. The histology revealed a predominantly cribriform tumour with focal areas of basaloid type metastatic lacrimal gland adenoid cystic carcinoma.
This case illustrates the unpredictable behaviour of adenoid cystic carcinoma and the need for a life long follow up for these patients after treatment. The possibility of surgical resection for liver metastasis from adenoid cystic carcinoma should always be considered.
Adenoid cystic carcinomas (ACC) of the lacrimal gland are rare malignant tumours accounting for 1.6% of all orbital tumours [1–3]. Despite their rarity they are the second most frequent epithelial neoplasms occurring in the lacrimal gland after pleomorphic adenomas .
There are few studies and reports on lacrimal gland ACC describing time interval to presentation of metastases and length of follow-up required [9, 10]. The liver is considered a rare site of distant metastasis and when it is involved is usually as part of disseminated disease . To our knowledge, neither solitary liver metastasis, nor metastatic liver resection from a lacrimal gland ACC has been reported.
We present a case of an isolated liver metastasis occurring 20 years after initial surgery for lacrimal gland ACC, with no evidence of loco regional, synchronous or metachronous distant metastasis.
In 1985, a 51-year-old male, underwent a radical craniofacial resection for a left lacrimal gland adenoid cystic carcinoma, followed by postoperative radiotherapy. He was followed-up with annual head and orbital CT scans for 15 years with no signs of loco-regional recurrence. Five years after discharge from follow-up, he presented with a 3-month history of right upper quadrant pain, radiating to his back, associated with shortness of breath and reduced exercise tolerance.
ACC in general has a slow biological growth and tends to have a protracted course with a poor outcome, with a reported survival rate of less than 50% at 5 years and 20% at 10 years [11–14]. Lacrimal gland ACC displays a similarly aggressive behavior with an overall survival of 20% at 10 years [15, 16]. Local recurrence risk was shown to be higher than distant recurrence in 8 different studies [8, 17], suggesting local recurrence as the commonest cause of disease progression and death.
In 23 years retrospective study, Ramon et al, reviewed the outcome of 12 patients with low grade ACC, showing that all patients had local recurrence with a mean interval of 3.25 years, and a mean survival of 5 years . In the Bayler series of patients with lacrimal gland ACC, the longest survivors lived 13 and 16 years respectively after exenteration and radiotherapy . In this case our patient is still alive 21 years after the primary resection.
Metastasis to the liver is rare and usually only occurs in conjunction with more widespread metastatic disease . Reported rates of liver metastases in ACC are between 2–20%, with the longest reported period to presentation of only 36 months [10, 21]. No isolated hepatic metastases from lacrimal gland ACC were recorded in these series.
This patient had liver metastasis as the sole presentation of secondary disease 20 years following treatment for his primary with no evidence of local recurrence. Surgical resection of liver metastasis from lacrimal gland ACC has never been reported before.
There is no proven effective chemotherapy for the treatment of metastatic lacrimal gland ACC; however some studies suggested benefit from neoadjuvant and adjuvant chemotherapy in the treatment of primary lacrimal gland ACC, in terms of improving local disease control and overall disease free survival .
This case highlights new aspects on ACC of the lacrimal glands, and a new evidence of the unpredictability of ACC behaviour in general .
Solitary hepatic metastasis can occur extremely late after resection of lacrimal gland ACC. Life long follow-up of patients with ACC is mandatory as treatable single organ recurrence may occur decades after resection of the primary. Resection of such liver metastasis is possible and should be considered.
The written consent was obtained from the patient for publication of this case report.
The authors thank Dr. A Bateman from the Histopathology Department and Dr. D Breen from the Radiology Department in Southampton General Hospital for their help in interpretation of the histological features of the tumour and providing detailed reports on the CT films.
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