Symposium: Tumors of the Lids and Orbit 1
1
1
LACRIMAL GLAND AND FOSSA LESIONS: AN APPROACH TO DIAGNOSIS AND MANAGEMENT WILLIAM B. STEWART, MD SAN FRANCISCO, CALIFORNIA
and GREGORY B. KROHEL, MD ALBANY, NEW YORK JOHN E. WRIGHT, MD LONDON, ENGLAND BOTH BY INVITATION
Lesions of the fossa of the lacrimal gland present special problems in diagnosis and management. Clinical recognition and differentiation of benign mixed cell lacrimal gland tumors from other inflammatory and neoplastic lesions of the lacrimal gland fossa are feasible and should provide the basis for the approach to proper management. Lesions conforming to the clinical picture of benign mixed cell lacrimal gland tumors need not undergo incisional biopsy, but require en bloc excision with adjacent tissues through a lateral orbitotomy to ensure complete excision and to prevent late recurrence. Incisional biopsy is indicated and should not be delayed in suspected infectious and noninfectious inflammatory lesions Submitted for publication Oct 25, 1978. From the Ophthalmic Plastic and Reconstructive Surgery Service, Department of Ophthalmology, Pacific Medical Center, San Francisco (Dr Stewart); the Neuro-Ophthalmology Service, Department of Ophthalmology, Albany Medical College, Albany, NY (Dr Krohel); and the Orbital Surgery Service, Moorfields Eye Hospital, London (Dr Wright). Presented in combination with the American Society of Ophthalmic Plastic and Reconstructive Surgery at the 1978 Annual Meeting of the American Academy of Ophthalmology, Kansas City, Mo, Oct 22-26. Reprint requests to Department of Ophthalmology, Pacific Medical Center, PO Box 7999, San Francisco, CA 94120 (Dr Stewart).
that have not responded rapidly to medical therapy, and in other lesions suspected of being neoplastic and not amenable to total local excision. An expedient approach to these lesions is essential if the prognosis of epithelial lacrimal gland neoplasms is to be improved.
INTRODUCTION
THE differential diagnosis of lesions occurring in the superotemporal orbit is large. Proper management of all lacrimal gland and fossa lesions requires recognition of benign mixed lacrimal gland tumors as a clinical entity. These tumors can often be differentiated from other neoplastic and inflammatory lesions affecting the superotemporal orbit on clinical and radiographic grounds. The recognition of benign mixed tumors is essential because these lesions should be totally excised en bloc with adjacent tissues to avoid the risk of incomplete excision and the possibility of recurrences with malignant potential,l- 3 However, all other lesions in this region, apart from acute dacryoadenitis and der-
886
86 !979
VOLUME MAY
SYMPOSIUM ON ORBITAL DISEASES
moid cysts, require incisional biopsy, through an anterior approach, so that a definitive tissue diagnosis can be made. Following appropriate biopsy, histologic as well as clinical criteria can be used in therapeutic decision making without either significant risk of incomplete excision of a mixed cell tumor by unwanted incisional biopsy or further invasive spread of malignant lesions caused by unnecesary delays in treatment. MATERIALS AND METHODS
Clinical records and histopathologic findings of 82 patients with lesions of the fossa of the lacrimal gland seen in the Orbital Clinic at Moorfields Eye Hospital, London, from 1968 through 1978 were reviewed. Clinical characteristics were identified that would allow recognition of benign mixed lacrimal gland tumors from other lesions of the superotemporal orbit. There were 31 cases of benign or malignant epithelial lacrimal gland masses with adequately documented history, verified radiologic findings, and confirmed histopathologic diagnosis. All of these patients were examined and operated on by at least one of the authors (J.E.W.) The other cases were nonepithelial lesions such as acute and chronic nongranulomatous inflammation, granulomatous inflammation, idiopathic inflammatory pseudotumor, and lymphoma, as well as ectodermally derived lesions such as dermoid cysts. Orbital involvement in some of these cases was not confined to the lacrimal fossa. Evaluation of patients with lacrimal gland fossa symptoms and signs included general medical examination, indicated blood studies,
887
and plain and tomographic radiologic studies of the orbits. Orbital ultrasonography, venography, and computerized tomography were performed in selected cases. RESULTS
Fourteen patients in the series had benign mixed lacrimal gland tumors. Seven men and seven women, aged 21 through 56 years, were involved. Duration of symptoms was greater than one year in 13 of the 14 patients. Only one patient with a benign mixed tumor had pain. Thirteen of the 14 patients had "pressure" changes (characterized by enlargement of the lacrimal gland fossa without destruction of bone) on plain roentgenograms or axial tomography. In addition, sclerosis of bone was present in one patient in association with pressure changes. Malignant neoplasms accounted for 17 of the 31 epithelial tumors. These eight men and nine women had a wide age distribution; however, it was most noteworthy that six patients were under 30 years of age. Five patients were over 60 years of age. Thirteen of the 17 patients with malignant epithelial tumors had symptoms for less than one year. Twelve patients with malignant lesions complained of pain. Of the 17 patients with malignant tumors, 12 had pressure changes, two showed no bone changes, and three showed destructive changes in the superotemporal orbit on plain roentgenograms or axial tomography. In addition, three patients with pressure changes and malignant tumors had calcification in the lacrimal gland fossa, and two patients had sclerosis of bone adjacent to the lacrimal fossa.
STEW ART ET AL
888
Six of the malignant neoplasms were considered inoperable when first observed at Moorfields Eye Hospital because of extensive involvement of bone or evidence of spread beyond the limits of even the most extensive surgical approach. All of the patients with tumor spread beyond potential surgical margins had an interval of more than three months from the time they were initially seen by an outside ophthalmic service until referral or definitive tissue diagnosis, or both. The average interval was 41.4 months. For those patients who had resectable tumors, the interval was only two months. The nonepithelial lesions in the review included acute dacryoadenitis, idiopathic inflammatory pseudotumor, and lymphomatous lesions. These lesions represented a broad and varied spectrum of clinical appearance. Ages of the patients ranged from the first through the eighth decades. Generally symptoms had been present less than six months. Inflammatory signs and discomfort. were relatively common. Roentgenographic abnormalities were unusual. Table 1 summarizes the ages, sex, duration of symptoms, presence of pain, and radiologic findings in those patients with benign and malignant epithelial lacrimal gland neoplasms. DISCUSSION
Although the clinical appearance of cases with benign mixed lacrimal gland tumors has been included in the excellent reviews by Zimmerman,1 Forrest, 4 Davies, 5 Reese, 6 and Henderson/ lr 409 l further emphasis must be placed on improving clinical diagnosis.
OPHTH AAO
Benign mixed lacrimal gland tumors tend to occur in patients aged from late 20s to early 50s, with a slowly progressive, painless upper lid swelling or proptosis, or both, without inflammatory symptoms or signs. A careful, detailed history will reveal a duration of symptoms in excess of 12 months. Examination of old photographs will sometimes reveal a ptosis or displacement of the globe that will help date the onset of symptoms. This pattern of presentation, when compared with the other lacrimal fossa lesions, is the most useful diagnostic feature. When considered with the roentgenographic findings, this long duration is virtually pathognomonic. These slow-growing tumors displace the normal lacrimal gland elements and often cause a pressure enlargement of the lacrimal fossa without destruction of overlying bone. 8 1° Careful tomographic study may reveal this characteristic change when plain reontgenograms are negative. (This occurred in one patient in our series.) Although described as a rare entity by Duke-Elder, 11 acute dacryoadenitis occurs with sufficient frequency to be considered in the differential diagnosis of lacrimal fossa lesions. 12 • 13 Furthermore, suspicion of this lesion may cause delays in biopsy by therapeutic trials and periods of observation. While bacterial and viral dacryoadenitis is more commonly seen in children and in younger persons, 11 • 12 epithelial lacrimal gland tumors are extremely rare in children.l 4 Acute dacryoadenitis has a short history measured in days rather than months, may be related to trauma, and is associated with local tenderness, erythema, lid swelling, conjunctival chemosis, discharge or
889
SYMPOSIUM ON ORBITAL DISEASES
VOLUME 86 MAY 1979
TABLE
1
SUMMARY OF PATIENTS WITH BENIGN AND MALIGNANT EPITHELIAL LACRIMAL GLAND NEOPLASMS MALIGNANT EPITHELIAL TUMORS BENIGN MIXED CELL LACRIMAL GLAND TUMORS AGE (YR)
0-20
3 4 5 2
20-30 30-40 40-50 50-60
MALIGNANT OTHER CARCINOMAS CARCINOMAS
~!XED CELL ADENOCYSTIC
TUMORS
1 1
2 1 2
1 1
1 2 4
2
3 4
5 3
1
3
3 2 2 1
60-70 Over 70 SEX
DURATION OF SYMPTOMS (MO)
7 7
M
w 0-3 3-6 6-9
Over PAIN
18
Present Not Present
ROENTGENO-
2 1
1 2
9-12 12-18
Norma]
GRAPHIC FINDINGS Pressure . (PLAIN AND DestructiOn TOMOGRAPHIC)
11
1
1
1 13
1 1
6 1
5 3
2
1 5 1
5 2
1
13
suppuration, enlarged preauricular and cervical nodes, and systemic findings such as pyrexia, malaise, upper respiratory involvement, and abnormal blood studies. 11 •12 Radiologic studies, m the absence of osteomyelitis or associated fracture, would be expected to be normal. Acute dacryoadenitis is usually unilateral and tends to resolve rapidly. 12 Secondary dacryoadenitis related to adjacent infections, mumps, infectious mononucleosis, or herpes zoster should be associated with characteristic systemic abnormalities. Chronic dacryoadenitis may
1
follow acute inflammation or be caused by thyroid ophthalmopathy, trachoma, syphilis, tuberculosis, or sarcoidosis.U Usually the diagnosis can be made on the basis of local or systemic findings; however, biopsy, particularly in chronic cases, may be necessary. Malignant tumors compose about one half of epithelial lacrimal gland masses. Epithelial lacrimal gland neoplasms (benign and malignant) account for about 7% of lesions in surveys of proptosis and orbital tu-
STEW ART ET AL
890
mors. Of these, approximately one half are benign mixed cell tumors, one third are adenacystic carcinomas, and the remainder are malignant mixed and other carcinomas. In general, patients with these carcinomas have a poor prognosis.L 4.7
1
1
LACRIMAL GLAND AND FOSSA LESIONS: AN APPROACH TO DIAGNOSIS AND MANAGEMENT WILLIAM B. STEWART, MD SAN FRANCISCO, CALIFORNIA
and GREGORY B. KROHEL, MD ALBANY, NEW YORK JOHN E. WRIGHT, MD LONDON, ENGLAND BOTH BY INVITATION
Lesions of the fossa of the lacrimal gland present special problems in diagnosis and management. Clinical recognition and differentiation of benign mixed cell lacrimal gland tumors from other inflammatory and neoplastic lesions of the lacrimal gland fossa are feasible and should provide the basis for the approach to proper management. Lesions conforming to the clinical picture of benign mixed cell lacrimal gland tumors need not undergo incisional biopsy, but require en bloc excision with adjacent tissues through a lateral orbitotomy to ensure complete excision and to prevent late recurrence. Incisional biopsy is indicated and should not be delayed in suspected infectious and noninfectious inflammatory lesions Submitted for publication Oct 25, 1978. From the Ophthalmic Plastic and Reconstructive Surgery Service, Department of Ophthalmology, Pacific Medical Center, San Francisco (Dr Stewart); the Neuro-Ophthalmology Service, Department of Ophthalmology, Albany Medical College, Albany, NY (Dr Krohel); and the Orbital Surgery Service, Moorfields Eye Hospital, London (Dr Wright). Presented in combination with the American Society of Ophthalmic Plastic and Reconstructive Surgery at the 1978 Annual Meeting of the American Academy of Ophthalmology, Kansas City, Mo, Oct 22-26. Reprint requests to Department of Ophthalmology, Pacific Medical Center, PO Box 7999, San Francisco, CA 94120 (Dr Stewart).
that have not responded rapidly to medical therapy, and in other lesions suspected of being neoplastic and not amenable to total local excision. An expedient approach to these lesions is essential if the prognosis of epithelial lacrimal gland neoplasms is to be improved.
INTRODUCTION
THE differential diagnosis of lesions occurring in the superotemporal orbit is large. Proper management of all lacrimal gland and fossa lesions requires recognition of benign mixed lacrimal gland tumors as a clinical entity. These tumors can often be differentiated from other neoplastic and inflammatory lesions affecting the superotemporal orbit on clinical and radiographic grounds. The recognition of benign mixed tumors is essential because these lesions should be totally excised en bloc with adjacent tissues to avoid the risk of incomplete excision and the possibility of recurrences with malignant potential,l- 3 However, all other lesions in this region, apart from acute dacryoadenitis and der-
886
86 !979
VOLUME MAY
SYMPOSIUM ON ORBITAL DISEASES
moid cysts, require incisional biopsy, through an anterior approach, so that a definitive tissue diagnosis can be made. Following appropriate biopsy, histologic as well as clinical criteria can be used in therapeutic decision making without either significant risk of incomplete excision of a mixed cell tumor by unwanted incisional biopsy or further invasive spread of malignant lesions caused by unnecesary delays in treatment. MATERIALS AND METHODS
Clinical records and histopathologic findings of 82 patients with lesions of the fossa of the lacrimal gland seen in the Orbital Clinic at Moorfields Eye Hospital, London, from 1968 through 1978 were reviewed. Clinical characteristics were identified that would allow recognition of benign mixed lacrimal gland tumors from other lesions of the superotemporal orbit. There were 31 cases of benign or malignant epithelial lacrimal gland masses with adequately documented history, verified radiologic findings, and confirmed histopathologic diagnosis. All of these patients were examined and operated on by at least one of the authors (J.E.W.) The other cases were nonepithelial lesions such as acute and chronic nongranulomatous inflammation, granulomatous inflammation, idiopathic inflammatory pseudotumor, and lymphoma, as well as ectodermally derived lesions such as dermoid cysts. Orbital involvement in some of these cases was not confined to the lacrimal fossa. Evaluation of patients with lacrimal gland fossa symptoms and signs included general medical examination, indicated blood studies,
887
and plain and tomographic radiologic studies of the orbits. Orbital ultrasonography, venography, and computerized tomography were performed in selected cases. RESULTS
Fourteen patients in the series had benign mixed lacrimal gland tumors. Seven men and seven women, aged 21 through 56 years, were involved. Duration of symptoms was greater than one year in 13 of the 14 patients. Only one patient with a benign mixed tumor had pain. Thirteen of the 14 patients had "pressure" changes (characterized by enlargement of the lacrimal gland fossa without destruction of bone) on plain roentgenograms or axial tomography. In addition, sclerosis of bone was present in one patient in association with pressure changes. Malignant neoplasms accounted for 17 of the 31 epithelial tumors. These eight men and nine women had a wide age distribution; however, it was most noteworthy that six patients were under 30 years of age. Five patients were over 60 years of age. Thirteen of the 17 patients with malignant epithelial tumors had symptoms for less than one year. Twelve patients with malignant lesions complained of pain. Of the 17 patients with malignant tumors, 12 had pressure changes, two showed no bone changes, and three showed destructive changes in the superotemporal orbit on plain roentgenograms or axial tomography. In addition, three patients with pressure changes and malignant tumors had calcification in the lacrimal gland fossa, and two patients had sclerosis of bone adjacent to the lacrimal fossa.
STEW ART ET AL
888
Six of the malignant neoplasms were considered inoperable when first observed at Moorfields Eye Hospital because of extensive involvement of bone or evidence of spread beyond the limits of even the most extensive surgical approach. All of the patients with tumor spread beyond potential surgical margins had an interval of more than three months from the time they were initially seen by an outside ophthalmic service until referral or definitive tissue diagnosis, or both. The average interval was 41.4 months. For those patients who had resectable tumors, the interval was only two months. The nonepithelial lesions in the review included acute dacryoadenitis, idiopathic inflammatory pseudotumor, and lymphomatous lesions. These lesions represented a broad and varied spectrum of clinical appearance. Ages of the patients ranged from the first through the eighth decades. Generally symptoms had been present less than six months. Inflammatory signs and discomfort. were relatively common. Roentgenographic abnormalities were unusual. Table 1 summarizes the ages, sex, duration of symptoms, presence of pain, and radiologic findings in those patients with benign and malignant epithelial lacrimal gland neoplasms. DISCUSSION
Although the clinical appearance of cases with benign mixed lacrimal gland tumors has been included in the excellent reviews by Zimmerman,1 Forrest, 4 Davies, 5 Reese, 6 and Henderson/ lr 409 l further emphasis must be placed on improving clinical diagnosis.
OPHTH AAO
Benign mixed lacrimal gland tumors tend to occur in patients aged from late 20s to early 50s, with a slowly progressive, painless upper lid swelling or proptosis, or both, without inflammatory symptoms or signs. A careful, detailed history will reveal a duration of symptoms in excess of 12 months. Examination of old photographs will sometimes reveal a ptosis or displacement of the globe that will help date the onset of symptoms. This pattern of presentation, when compared with the other lacrimal fossa lesions, is the most useful diagnostic feature. When considered with the roentgenographic findings, this long duration is virtually pathognomonic. These slow-growing tumors displace the normal lacrimal gland elements and often cause a pressure enlargement of the lacrimal fossa without destruction of overlying bone. 8 1° Careful tomographic study may reveal this characteristic change when plain reontgenograms are negative. (This occurred in one patient in our series.) Although described as a rare entity by Duke-Elder, 11 acute dacryoadenitis occurs with sufficient frequency to be considered in the differential diagnosis of lacrimal fossa lesions. 12 • 13 Furthermore, suspicion of this lesion may cause delays in biopsy by therapeutic trials and periods of observation. While bacterial and viral dacryoadenitis is more commonly seen in children and in younger persons, 11 • 12 epithelial lacrimal gland tumors are extremely rare in children.l 4 Acute dacryoadenitis has a short history measured in days rather than months, may be related to trauma, and is associated with local tenderness, erythema, lid swelling, conjunctival chemosis, discharge or
889
SYMPOSIUM ON ORBITAL DISEASES
VOLUME 86 MAY 1979
TABLE
1
SUMMARY OF PATIENTS WITH BENIGN AND MALIGNANT EPITHELIAL LACRIMAL GLAND NEOPLASMS MALIGNANT EPITHELIAL TUMORS BENIGN MIXED CELL LACRIMAL GLAND TUMORS AGE (YR)
0-20
3 4 5 2
20-30 30-40 40-50 50-60
MALIGNANT OTHER CARCINOMAS CARCINOMAS
~!XED CELL ADENOCYSTIC
TUMORS
1 1
2 1 2
1 1
1 2 4
2
3 4
5 3
1
3
3 2 2 1
60-70 Over 70 SEX
DURATION OF SYMPTOMS (MO)
7 7
M
w 0-3 3-6 6-9
Over PAIN
18
Present Not Present
ROENTGENO-
2 1
1 2
9-12 12-18
Norma]
GRAPHIC FINDINGS Pressure . (PLAIN AND DestructiOn TOMOGRAPHIC)
11
1
1
1 13
1 1
6 1
5 3
2
1 5 1
5 2
1
13
suppuration, enlarged preauricular and cervical nodes, and systemic findings such as pyrexia, malaise, upper respiratory involvement, and abnormal blood studies. 11 •12 Radiologic studies, m the absence of osteomyelitis or associated fracture, would be expected to be normal. Acute dacryoadenitis is usually unilateral and tends to resolve rapidly. 12 Secondary dacryoadenitis related to adjacent infections, mumps, infectious mononucleosis, or herpes zoster should be associated with characteristic systemic abnormalities. Chronic dacryoadenitis may
1
follow acute inflammation or be caused by thyroid ophthalmopathy, trachoma, syphilis, tuberculosis, or sarcoidosis.U Usually the diagnosis can be made on the basis of local or systemic findings; however, biopsy, particularly in chronic cases, may be necessary. Malignant tumors compose about one half of epithelial lacrimal gland masses. Epithelial lacrimal gland neoplasms (benign and malignant) account for about 7% of lesions in surveys of proptosis and orbital tu-
STEW ART ET AL
890
mors. Of these, approximately one half are benign mixed cell tumors, one third are adenacystic carcinomas, and the remainder are malignant mixed and other carcinomas. In general, patients with these carcinomas have a poor prognosis.L 4.7
