Orbit, 2014; 33(4): 283–285 ! Informa Healthcare USA, Inc. ISSN: 0167-6830 print / 1744-5108 online DOI: 10.3109/01676830.2013.842254
C ASE REPORT
Simultaneous Diagnosis of Ipsilateral Adenoid Cystic Carcinoma of the Lacrimal Gland and Orbital Cavernous Hemangioma: Case Report Diego Strianese, Mariantonia Ferrara, Adriana Iuliano, and Giulio Bonavolonta` Department of Neuroscience and Reproductive Sciences and Odontostomatology – University of Naples Federico II, Italy
ABSTRACT To describe a patient with coexistence of ipsilateral adenoid cystic carcinoma of the lacrimal gland and orbital cavernous hemangioma. The patient presented a 2-month history of painful swelling of left upper eyelid, which did not resolve on a course of oral steroid. On clinical examination, she had moderate medial and inferior dislocation of the eye globe, edema of the temporal superior eyelid and moderate restriction upgaze. Magnetic resonance imaging identified two left orbital tumors: one was a lesion that arose from the lacrimal gland, and the second was a well-defined, ovoid, intraconal mass. The patient underwent lateral orbitotomy and excision of both lesions. Postoperative histological features demonstrated two different lesions: an adenoid cystic carcinoma of the lacrimal gland and a cavernous hemangioma. The clinical presentation of the present case was strongly suggestive of a lacrimal gland malignancy, although the imaging features did not show typical invasive aspects. This is the first report of coexistence of lacrimal gland adenoid cystic carcinoma and cavernous hemangioma in the ipsilateral orbit. Keywords: Adenoid cystic carcinoma, cavernous hemangioma, coexistence
INTRODUCTION
Case Report
Adenoid cystic carcinoma (ACC) accounts for 32% of epithelial tumor of the lacrimal gland1 and it represents the 2% of all orbital lesions.2 Classically, it consists of 3 histological subtypes: cribriform, tubular, and solid subtype. New pathological tools have been described to better define prognosis of those tumors3.Cavernous haemangioma (CH) is the most common primary orbital tumor in adults, accounting for 9% of all orbital lesions.2 It is considered a proliferating hamartoma with the characteristics of low-flow arteriovenous malformations. We report the first case of coexistence in the ipsilateral orbit of a ACC and a CH.
A 53-year-old woman presented a 2-month history of painful swelling of her left upper eyelid, which had not resolved on a previous course of oral corticosteroids. Distance visual acuity measured 2/25 OD and 20/30 OS and 2 mm of relative proptosis were noted, with medial and inferior displacement of the globe and with no other significant orbital signs. On magnetic resonance imaging (MRI), two separate soft tissue lesions were noted in the left orbit. The first appeared to be arising from the lacrimal gland measuring 3.5 mm 2 mm, and was isointense and slightly heterogeneous on T1-weighted and STIR windows and almost hyperintense and
Received 10 April 2013; Revised 10 August 2013; Accepted 19 August 2013; Published online 28 April 2014 Correspondence: Diego Strianese, Department of Ophthalmological Sciences, University ‘‘Federico II’’ of Naples, Italy, Via Pansini n. 5, 80131, Naples, Italy, Tel: (þ39) 081-7462389, Fax: (þ39)081-7462389, E-mail: [email protected]
283
284 D. Strianese et al. heterogeneous on T2-weighted MR image. This tumor showed an inhomogeneous enhancement by Gd-DTPA administration. The second lesion was a well-defined, ovoid, intraconal mass of 2 mm 1 mm, dislocating medially the optic nerve. It appeared isointense to muscle on T1-weighted imaging and hyperintense to muscle on T2-weighted and STIR windows with inhomogeneous enhancement of Gd-DTPA (Figure 1). Since the mass arising from lacrimal gland showed well defined margins on MRI imaging, an excisional biopsy through lateral orbitotomy was performed, in order to remove the intact lesion. This appeared firm, gray and surrounded by a pseudocapsule, which
allowed the dissection from the adjacent structures and the removal of the lacrimal gland along with the whole lesion. The second lesion was found posteriorly to the globe into the intraconal space. The lesion was ovoid, reddish and well circumscribed by thin capsule. It was removed without traction on adjacent structures (Figure 2). No postoperative complications were registered. Pathological examination revealed that the lesion arising from the lacrimal gland was an ACC with cribriform histological pattern, while the intraconal lesion was a CH. The patient underwent postoperative radiotherapy for the ACC and no recurrences were registered during 3-year follow-up.
FIGURE 1. Both the adenoid cystic carcinoma and the cavernous hemangioma appear isointense on T1-weighted images and hyperintense on T2-weighted.
FIGURE 2. The two lesions excised. Orbit
Double Orbital Pathology: A Rare Occurrence
Comment The clinical presentation of malignant neoplasms of the lacrimal gland is characterized by rapid progression, pain, and radiographic evidence of bone destruction.4,5 The majority of cavernous hemangiomas are isolated lesions, although they may occur bilaterally or as multiple tumors. The clinical course is characterized by a slow progressive growth, that leads to compression or displacement of ocular or orbital structures and causing proptosis. Asymptomatic lesions have been found incidentally on radiological imaging.6 However, intralesional hemorrhage can rarely occur, and, where present, suggests pathology other than CH.7 The history, being that of 2 months of progressive painful proptosis and swelling of the upper eyelid, strongly suggested a lacrimal gland malignancy. However, the imaging findings did not fully support such a diagnosis, with only the T2-weighted images suggesting a malignant process. In this patient, identification of the CH was a coincidental finding, and, in the absence of co-pathology (ACC) the painless and slowly progressive changes due to the CH might have passed unnoticed for years to decades, as has is well-documented in the literature.8 Treatment of ACC requires complete removal of the lacrimal gland. Incisional biopsy should be avoided, even though in cases with uncertain preoperative diagnosis is the key to rule out other malignancies such as orbital lymphoma involving lacrimal fossa which require different treatment modalities and outcome.9,10 In the present patient, the tumor was completely removed and no recurrence occurred within 3 years of follow-up. The advisability of removal of an asymptomatic orbital CH remains controversial due to the associated surgical morbidity.7 However, in this case, due to the rare presence of two orbital lesions, the decision to operate had already been made. Thus far, no cases of coexistence of two different pathology in the same orbit are described in literature at present.
!
2014 Informa Healthcare USA, Inc.
285
DECLARATION OF INTEREST The authors report no conflicts of interest. No financial support was received for this submission. The authors alone are responsible for the content and writing of the paper. Diego Strianese and Giulio Bonavolonta` had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. Contributions of authors: Design of the observation: D. Strianese; Collection and management of the data: D. Strianese, G. Bonavolonta’; Preparation, review, or approval of the manuscript: M. Ferrara, A. Iuliano.
REFERENCES 1. Weis E, Rootman J, Joly TJ, et al. Epithelial lacrimal gland tumors: pathologic classification and current understanding. Arch Ophthalmol 2009;127:1016–1028. 2. Bonavolonta` G, Strianese D, Grassi P, et al. An analysis of 2,480 space-occupying lesions of the orbit from 1976 to 2011. Ophthal Plast Reconstr Surg 2013;29:79–86. 3. Strianese D, Baldi G, Staibano S, et al. Expression of apoptosis-related markers in malignant epithelial tumours of the lacrimal gland and their relation to clinical outcome. Br J Ophthalmol 2007;91:1239–1243. 4. Paulino AF, Huvos AG. Epithelial tumors of the lacrimal glands: a clinicopathologic study. Ann Diagn Pathol 1999;3: 199–204. 5. Wright JE, Rose GE, Garner A. Primary malignant neoplasms of the lacrimal gland. Br J Ophthalmol 1992;76: 401–407. 6. Mc Nab A. Wright JE. Cavernous haemangiomas of the orbit. Aust N Z J Ophthalmol 1989;17:337–345. 7. Selva D, Strianese D, Bonavolonta G, Rootman J. Orbital venous-lymphatic malformations (lymphangiomas) mimicking cavernous hemangiomas. Am J Ophthalmol 2001;131:364–370. 8. Harris GJ. Cavernous hemangioma of the orbital apex: pathogenetic considerations in surgical management. Am J Ophthalmol 2010;150:764–773. 9. De Cicco L, Cella L, Liuzzi R, et al. Radiation therapy in primary orbital lymphoma: a single institution retrospective analysis. Radiat Oncol 2009;4:60. doi: 10.1186/ 1748-717X-4-60. 10. Strianese D, Tranfa F, Finelli M, et al. Hepatitis C virus infection in ocular adnexal lymphomas. Arch Ophthalmol 2010;128(10):1295–1299.
Copyright of Orbit is the property of Taylor & Francis Ltd and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
C ASE REPORT
Simultaneous Diagnosis of Ipsilateral Adenoid Cystic Carcinoma of the Lacrimal Gland and Orbital Cavernous Hemangioma: Case Report Diego Strianese, Mariantonia Ferrara, Adriana Iuliano, and Giulio Bonavolonta` Department of Neuroscience and Reproductive Sciences and Odontostomatology – University of Naples Federico II, Italy
ABSTRACT To describe a patient with coexistence of ipsilateral adenoid cystic carcinoma of the lacrimal gland and orbital cavernous hemangioma. The patient presented a 2-month history of painful swelling of left upper eyelid, which did not resolve on a course of oral steroid. On clinical examination, she had moderate medial and inferior dislocation of the eye globe, edema of the temporal superior eyelid and moderate restriction upgaze. Magnetic resonance imaging identified two left orbital tumors: one was a lesion that arose from the lacrimal gland, and the second was a well-defined, ovoid, intraconal mass. The patient underwent lateral orbitotomy and excision of both lesions. Postoperative histological features demonstrated two different lesions: an adenoid cystic carcinoma of the lacrimal gland and a cavernous hemangioma. The clinical presentation of the present case was strongly suggestive of a lacrimal gland malignancy, although the imaging features did not show typical invasive aspects. This is the first report of coexistence of lacrimal gland adenoid cystic carcinoma and cavernous hemangioma in the ipsilateral orbit. Keywords: Adenoid cystic carcinoma, cavernous hemangioma, coexistence
INTRODUCTION
Case Report
Adenoid cystic carcinoma (ACC) accounts for 32% of epithelial tumor of the lacrimal gland1 and it represents the 2% of all orbital lesions.2 Classically, it consists of 3 histological subtypes: cribriform, tubular, and solid subtype. New pathological tools have been described to better define prognosis of those tumors3.Cavernous haemangioma (CH) is the most common primary orbital tumor in adults, accounting for 9% of all orbital lesions.2 It is considered a proliferating hamartoma with the characteristics of low-flow arteriovenous malformations. We report the first case of coexistence in the ipsilateral orbit of a ACC and a CH.
A 53-year-old woman presented a 2-month history of painful swelling of her left upper eyelid, which had not resolved on a previous course of oral corticosteroids. Distance visual acuity measured 2/25 OD and 20/30 OS and 2 mm of relative proptosis were noted, with medial and inferior displacement of the globe and with no other significant orbital signs. On magnetic resonance imaging (MRI), two separate soft tissue lesions were noted in the left orbit. The first appeared to be arising from the lacrimal gland measuring 3.5 mm 2 mm, and was isointense and slightly heterogeneous on T1-weighted and STIR windows and almost hyperintense and
Received 10 April 2013; Revised 10 August 2013; Accepted 19 August 2013; Published online 28 April 2014 Correspondence: Diego Strianese, Department of Ophthalmological Sciences, University ‘‘Federico II’’ of Naples, Italy, Via Pansini n. 5, 80131, Naples, Italy, Tel: (þ39) 081-7462389, Fax: (þ39)081-7462389, E-mail: [email protected]
283
284 D. Strianese et al. heterogeneous on T2-weighted MR image. This tumor showed an inhomogeneous enhancement by Gd-DTPA administration. The second lesion was a well-defined, ovoid, intraconal mass of 2 mm 1 mm, dislocating medially the optic nerve. It appeared isointense to muscle on T1-weighted imaging and hyperintense to muscle on T2-weighted and STIR windows with inhomogeneous enhancement of Gd-DTPA (Figure 1). Since the mass arising from lacrimal gland showed well defined margins on MRI imaging, an excisional biopsy through lateral orbitotomy was performed, in order to remove the intact lesion. This appeared firm, gray and surrounded by a pseudocapsule, which
allowed the dissection from the adjacent structures and the removal of the lacrimal gland along with the whole lesion. The second lesion was found posteriorly to the globe into the intraconal space. The lesion was ovoid, reddish and well circumscribed by thin capsule. It was removed without traction on adjacent structures (Figure 2). No postoperative complications were registered. Pathological examination revealed that the lesion arising from the lacrimal gland was an ACC with cribriform histological pattern, while the intraconal lesion was a CH. The patient underwent postoperative radiotherapy for the ACC and no recurrences were registered during 3-year follow-up.
FIGURE 1. Both the adenoid cystic carcinoma and the cavernous hemangioma appear isointense on T1-weighted images and hyperintense on T2-weighted.
FIGURE 2. The two lesions excised. Orbit
Double Orbital Pathology: A Rare Occurrence
Comment The clinical presentation of malignant neoplasms of the lacrimal gland is characterized by rapid progression, pain, and radiographic evidence of bone destruction.4,5 The majority of cavernous hemangiomas are isolated lesions, although they may occur bilaterally or as multiple tumors. The clinical course is characterized by a slow progressive growth, that leads to compression or displacement of ocular or orbital structures and causing proptosis. Asymptomatic lesions have been found incidentally on radiological imaging.6 However, intralesional hemorrhage can rarely occur, and, where present, suggests pathology other than CH.7 The history, being that of 2 months of progressive painful proptosis and swelling of the upper eyelid, strongly suggested a lacrimal gland malignancy. However, the imaging findings did not fully support such a diagnosis, with only the T2-weighted images suggesting a malignant process. In this patient, identification of the CH was a coincidental finding, and, in the absence of co-pathology (ACC) the painless and slowly progressive changes due to the CH might have passed unnoticed for years to decades, as has is well-documented in the literature.8 Treatment of ACC requires complete removal of the lacrimal gland. Incisional biopsy should be avoided, even though in cases with uncertain preoperative diagnosis is the key to rule out other malignancies such as orbital lymphoma involving lacrimal fossa which require different treatment modalities and outcome.9,10 In the present patient, the tumor was completely removed and no recurrence occurred within 3 years of follow-up. The advisability of removal of an asymptomatic orbital CH remains controversial due to the associated surgical morbidity.7 However, in this case, due to the rare presence of two orbital lesions, the decision to operate had already been made. Thus far, no cases of coexistence of two different pathology in the same orbit are described in literature at present.
!
2014 Informa Healthcare USA, Inc.
285
DECLARATION OF INTEREST The authors report no conflicts of interest. No financial support was received for this submission. The authors alone are responsible for the content and writing of the paper. Diego Strianese and Giulio Bonavolonta` had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. Contributions of authors: Design of the observation: D. Strianese; Collection and management of the data: D. Strianese, G. Bonavolonta’; Preparation, review, or approval of the manuscript: M. Ferrara, A. Iuliano.
REFERENCES 1. Weis E, Rootman J, Joly TJ, et al. Epithelial lacrimal gland tumors: pathologic classification and current understanding. Arch Ophthalmol 2009;127:1016–1028. 2. Bonavolonta` G, Strianese D, Grassi P, et al. An analysis of 2,480 space-occupying lesions of the orbit from 1976 to 2011. Ophthal Plast Reconstr Surg 2013;29:79–86. 3. Strianese D, Baldi G, Staibano S, et al. Expression of apoptosis-related markers in malignant epithelial tumours of the lacrimal gland and their relation to clinical outcome. Br J Ophthalmol 2007;91:1239–1243. 4. Paulino AF, Huvos AG. Epithelial tumors of the lacrimal glands: a clinicopathologic study. Ann Diagn Pathol 1999;3: 199–204. 5. Wright JE, Rose GE, Garner A. Primary malignant neoplasms of the lacrimal gland. Br J Ophthalmol 1992;76: 401–407. 6. Mc Nab A. Wright JE. Cavernous haemangiomas of the orbit. Aust N Z J Ophthalmol 1989;17:337–345. 7. Selva D, Strianese D, Bonavolonta G, Rootman J. Orbital venous-lymphatic malformations (lymphangiomas) mimicking cavernous hemangiomas. Am J Ophthalmol 2001;131:364–370. 8. Harris GJ. Cavernous hemangioma of the orbital apex: pathogenetic considerations in surgical management. Am J Ophthalmol 2010;150:764–773. 9. De Cicco L, Cella L, Liuzzi R, et al. Radiation therapy in primary orbital lymphoma: a single institution retrospective analysis. Radiat Oncol 2009;4:60. doi: 10.1186/ 1748-717X-4-60. 10. Strianese D, Tranfa F, Finelli M, et al. Hepatitis C virus infection in ocular adnexal lymphomas. Arch Ophthalmol 2010;128(10):1295–1299.
Copyright of Orbit is the property of Taylor & Francis Ltd and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
![[Adenoid cystic carcinoma of the lacrimal gland].](/assets/img/hold.png)
