Case Report

Adenoid cystic carcinoma of the lacrimal gland metastatic to the kidney: case report and review of the literature

Scottish Medical Journal 2014, Vol. 59(2) e14–e17 ! The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0036933014530836 scm.sagepub.com

Jasna Bacalja1, Maja Magazin2, Monika Ulamec3,4, Duje Rako5, Davor Trnski6 and Bozˇo Krusˇlin4,7

Abstract Introduction: Adenoid cystic carcinoma (ACC) is a malignant neoplasm that arises within secretory glands. It rarely occurs in the lacrimal gland. Kidney metastases are very rare. Case presentation: We report a case of a 76-year-old female patient treated with right radical nephrectomy because of the tumour mass of the right kidney. Well-circumscribed, firm, grey mass was found on the lower pole of the kidney. Histologically, tumour was considered to be ACC. Retrospective history data showed ACC of the lacrimal gland surgically treated 14 years before with metastasis to the lung operated seven years before. Our diagnosis was metastatic ACC to the kidney. Conclusion: ACC has a remarkable capacity for recurrence. To our knowledge, in the English-speaking area (PubMed), 10 cases of ACC metastatic to the kidney have been described to date, and this is the second reported case of kidney metastasis from primary lacrimal ACC.

Keywords Adenoid cystic carcinoma, lacrimal gland, kidney metastases

Introduction Adenoid cystic carcinoma (ACC) is a relatively uncommon but aggressive malignant neoplasm that comprises about 10% of all epithelial salivary neoplasms, most frequently involving salivary glands.1,2 It usually presents in the fourth to sixth decades with slight female predominance (3 : 2). Except in salivary glands, it can rarely arise in lacrimal gland, trachea, lung, breast, skin and oesophagus.3–10 Kidney metastases are extremely rare, and we report such a case.

Case presentation A 76-year-old female patient presented with haematuria and flank pain. Computed tomography (CT) scan revealed a tumour mass of the right kidney. Right radical nephrectomy was performed. Macroscopically, on the lower pole of the right kidney, a well-circumscribed, firm, grey mass was found, measuring up to 7.8 cm (Figure 1).

Histologically, the tumour was composed of cribriform, tubular and solid formations of atypical epithelial cells with dark compact angular nuclei and frequent mitotic figures (Figures 2 and 3). Tumour cells showed positive immunohistochemical reaction to high molecular weight cytokeratin (CKHMW) and epithelial 1

Expert Pathologist, Department of Pathology, Dubrava University Hospital, Croatia 2 Cytology Trainee, Department of Pathology and Cytology, Sveti Duh University Hospital, Croatia 3 Assistant, School of Medicine, University of Zagreb, Croatia 4 Expert Pathologist, Ljudevit Jurak Department of Pathology, Sestre Milosrdnice Clinical Hospital Center, Croatia 5 Expert Urologist, Department of Urology, Dubrava University Hospital, Croatia 6 Expert Urologist, Department of Urology, Sestre Milosrdnice Clinical Hospital Center, Zagreb, Croatia 7 Professor, School of Medicine, University of Zagreb, Zagreb, Croatia Corresponding author: Jasna Bacalja, Department of Pathology, Dubrava University Hospital, Avenija Gojka Sˇusˇka 6, 10000 Zagreb, Croatia. Email: [email protected]

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Figure 1. Tumour presented as a well-circumscribed, firm, grey mass on the lower pole of the kidney.

Figure 3. Atypical epithelial cells with dark compact angular nuclei and frequent mitotic figures (arrows) (hematoxylin-eosin, 400).

Figure 2. The tumour (right side) is divided from normal renal parenchyma (left side) by fibrous tissue and is composed of cribriform and tubular formations (hematoxylin-eosin, 40).

Figure 4. Lung metastasis of adenoid cystic carcinoma with cribriform growth pattern (hematoxylin-eosin, 40).

membrane antigen (EMA), and negative to synaptophysin, CD 19, CD 15, CK8 and renal cell carcinoma (RCC). Retrospective history data showed ACC of the lacrimal gland treated with surgical enucleation of the left eye and removal of paraorbital soft tissue 14 years ago. The tumour was not seen macroscopically. Histology revealed microscopic focuses of ACC with cribriform and solid pattern of growth. The tumour infiltrated fibrous tissue next to the eyeball with no tumour infiltration of the eyeball nor invasion of perineural space or the optic nerve. Seven years after removal of the primary tumour, the patient was surgically treated for ACC lung metastases. The tumour presented as a solitary mass measuring 2.5 cm in diameter and was situated in the lower

lobe of the right lung. Histology revealed metastatic ACC. There were no metastases in the hilar lymph nodes (Figure 4). Based on the clinical data mentioned before, histological appearance and immunohistochemical analysis, pathohistologic diagnosis was metastatic ACC to the kidney.

Discussion Although it is rare, ACC is the most common malignant tumour in lacrimal gland accounting for 1.6% of all orbital tumours. It is the second most frequent epithelial neoplasm occurring in the lacrimal gland, after pleomorphic adenomas.6,11 It can also arise in accessory lacrimal glands of conjuctiva.12

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Surgery and radiotherapy are considered treatment modalities for ACC developed at any site. The five-year survival rate for ACC is 60%–75%, but the 10-year survival drops at 30%–54%.13–15 Despite any treatment, it seems to be impossible to prevent the development of locoregional recurrence that is the commonest cause of disease progression and death.14–18 All but the smallest ACC (T1) in the lacrimal gland are associated with bone invasion of lacrimal gland fossa.19 The majority of patients eventually develop distant metastases, mostly to the lung (72%), followed by bone and liver metastases.6,13–15,17–19 There are around 90 cases of primary lacrimal ACC reported in the English-speaking literature.3,6,11,12,16,17,19 In a 23 years retrospective study, Font et al. analysed 120 lacrimal gland masses of which there were 12 patients with primary lacrimal ACC. All patients were younger or middle-aged men (mean age 44 years). All had local recurrences with a mean interval of 3.25 years, six patients had distant metastases and seven patients died of the tumours (mean survival, five years). Two patients who had radical surgical procedures with orbital exenteration were alive 13 and 16 years after the initial surgery.11 Lee et al. described 26 cases of primary ACC of the lacrimal gland. They concluded that lower tumour grades and cribriform pattern of growth are associated with longer survival, while solid pattern of growth and bone invasion were associated with a shorter survival. In their study, survival was not affected by surgical or radiation therapy.20 Life-long follow-up is necessary as treatable single organ recurrence may occur decades after resection of the primary tumour as was described by Zeidan et al. In their case, a 51-year-old male patient underwent a radical craniofacial resection for primary lacrimal ACC followed by postoperative radiotherapy. He was followed up annually with head and orbital CT scans for 15 years with no signs of local recurrence. Five years after discharge from follow-up, he developed solid liver metastasis with no signs of local recurrence.21 To our knowledge, in the English-speaking area (PubMed), 10 cases of ACC metastatic to the kidney have been described to date, metastasising from the lacrimal gland, bronchus, lung, parotid gland, breast, salivary gland, palate, oral floor and external auditory canal.2,3,5,7,8,22–26 Kidney metastasis usually presents as a solitary tumour mass and chronic pain with or without haematuria.15,22,23 It can macroscopically mimic RCC as was described by Kala et al. In their case, a 35-year-old female patient was treated with right-sided nephrectomy because of a solitary tumour mass of the right kidney. Based on the clinical, radiologic and macroscopic findings, the tumour was considered to be

RCC. Microscopic examination showed it was a metastasis from salivary gland ACC operated eight years before.23 There was only one case of primary lacrimal ACC metastatic to kidney described by Blo¨chle et al. in 1993. In their case, the patient developed liver and kidney metastases that were surgically treated 25 years after surgical removal of primary lacrimal ACC. A few years before, the same patient was operated for intracranial and pulmonary metastases. That was also the longest survival and follow-up for ACC reported in the literature.3 The youngest patient with renal metastases of ACC was a 21-year-old women described by Manorahan et al. The patient underwent nephrectomy because of metastases from parotid gland ACC that was surgically removed seven years before. Patient later developed pulmonary metastases.24 Four cases were reported having renal metastases in the time the primary ACC was diagnosed. In two cases, primary tumour was in the lung,2,5 whereas in the other two cases, primary tumours were in the oral floor and external auditory canal.25,26

Conclusion This is the second reported case of a primary lacrimal ACC metastatic to the kidney. Despite its small size and favourable histological noninvasive behaviour, the patient developed contralateral pulmonary and renal metastases seven and 14 years after removal of the primary tumour. Our case confirms that life-long follow-up of primary lacrimal gland ACC is necessary even for the smallest and locally noninvasive tumours as recurrence may occur years after resection of the primary tumour. Declaration of conflicting interests None declared.

Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Author contributions BK and MU designed the study. DT and DR collected and analysed clinical data. MM collected pathological data and participated in the manuscript writing. JB reviewed gathered data and wrote the manuscript. BK critically reviewed the whole manuscript.

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Adenoid cystic carcinoma of the lacrimal gland metastatic to the kidney: case report and review of the literature.

Adenoid cystic carcinoma (ACC) is a malignant neoplasm that arises within secretory glands. It rarely occurs in the lacrimal gland. Kidney metastases ...
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