Clinical Findings

The clinical features of the cases involved in this study are summarized in Table 1 for the pleomorphic adenomas and Table 2 for the adenoid cystic carcinomas. Among the cases of pleomorphic adenoma, three were male and four were female ( Figure 1 , top left). The mean age at presentation was 47 years. Five patients presented with an upper eyelid mass, two had mild proptosis (2 mm), and one had lateral upper eyelid ptosis. The mean duration of symptoms was 39 months. Radiologic imaging showed a well-defined, rounded mass; one lesion displayed intralesional foci of calcification but without major bone changes ( Figure 1 , top right). Another tumor occurred as an enlargement of the palpebral lobe of the lacrimal gland, which was readily visible on physical examination with eyelid eversion. The first surgery in all cases was an attempt at complete excision, and an incisional biopsy was studiously avoided. No patient has experienced a recurrence, with an average follow-up of 15 months. However, only one of the seven cases had a follow-up of longer than 3 years (37 months).

Clinical, radiographic, and histopathologic features of lacrimal gland epithelial tumors. Top left: A 55-year-old man with 20/20 vision OU developed 2 mm of relative left proptosis without any pain, epibulbar injection or congestion, or diplopia. (Courtesy of Dr. Aaron Fay). Top right: Sagittal computed tomographic view of a pleomorphic adenoma. The lesion is well circumscribed without causing any bone erosion or destruction. There are foci of intratumoral calcification (arrow). Middle left: A 53-year-old woman developed 4 mm of right proptosis over 1 month. Visual acuity was 20/20 OU. Double vision, pain, and pressure behind the globe were reported. (Courtesy of Dr. Francis Sutula). Middle right: Coronal computed tomographic view of an adenoid cystic carcinoma. There is some elevation of the superolateral orbital bone (arrow) without destruction. Bottom left: Ductules of a pleomorphic adenoma are shown on the left. They are lined by a double row of cells. On the right there is a bluish myxoid stroma populated by delaminating outer myoepithelial cells of the ductules. Bottom right: A focus within a pleomorphic adenoma of densely eosinophilic, sclerotic islands of collagen (C) placed among variably wide tracts of blue staining ovoid- to spindle-shaped myoepithelial cells devoid of lumen formation. (Hematoxylin and eosin: bottom left, ×200; bottom right, ×100)

Five adenoid cystic carcinomas arose in the major lacrimal gland, one in an accessory lacrimal gland of Wolfring in the lower eyelid, and one in the maxillary sinus with secondary orbital extension. The American Joint Committee on Cancer (AJCC) TNM (tumor, node, metastasis) classification for each tumor is given in Table 2 . Four patients were male and three were female, with a mean age at presentation of 46 years ( Figure 1 , middle left). Five patients manifested proptosis (2 to 7mm), three complained of pain, and two had diplopia. The mean duration of symptoms was 7 months. Radiologic imaging showed bony destruction, erosion or remodeling of the orbital roof in four of the five lesions arising in the lacrimal gland ( Figure 1 , middle right). The orbital floor and medial wall in the primary maxillary tumor invading the orbit were partially destroyed. There were no bony changes in the tumor arising in the accessory gland of Wolfring. One tumor contained intralesional calcification without adjacent bony changes. The patient with the adenoid cystic carcinoma primarily in the maxillary sinus with orbital extension was the only patient who had regional lymph node involvement at the time of initial presentation. In all cases, a malignancy was strongly suspected based on the clinical and radiologic findings, and the widest excision possible was undertaken in the face of infiltrative margins. Average follow-up has been 71 months. Tumor recurrence has been found in four patients. Four patients are alive with disease and three have died as the result of their disease.

Histopathologic Findings

The diameters of the pleomorphic adenomas ranged from 1.2 cm to 2.6 cm, with a mean of 1.6 cm. All were invested with a variably thick pseudocapsule and all but one of the lesions were composed of a mixture of double-layered, epithelium-lined glandular structures with small to patulous lumens ( Figure 1 , bottom left) and spindly outer myoepithelial cells. The lumens commonly contained amorphous, eosinophilic secretory material that was positive for periodic acid Schiff (PAS) and mucicarmine. Basophilic mucoid stromal material surrounded the ductlike units. The outer myoepithelial cells of the ductular structures delaminated into the stroma. One myoepitheliomatous tumor predominantly displayed non-lumen–forming oval and spindle-shaped cells. This variant manifested stromal regions of compacted collagen ( Figure 1 , bottom right). One lesion was remarkable because of its scattered, atypical, hyperchromatic spindle cells ( Figure 2 , top left) and atypical low cuboidal cells, which created micro-acinar units ( Figure 2 , top right and inset). Another tumor exhibited foci of dystrophic calcification.

Histopathology of lacrimal gland pleomorphic adenoma and adenoid cystic carcinoma. Top left: An atypical pleomorphic adenoma displays spindle cells with atypical hyperchromatic nuclei. Mitotic figures were not observed. Top right: Small glandular (microacinar) units, in atypical pleomorphic adenoma, also shown in inset, are composed of low cuboidal cells with scattered large hyperchromatic nuclei possessing prominent nucleoli. Middle left: Classical sievelike, Swiss cheese or cribriform pattern of adenoid cystic carcinoma. The inset demonstrates perineural invasion. Middle right: The adenoidal spaces are not true lumens because they are rimmed by periodic acid Schiff–positive basement membrane material (arrows) and filled with a delicate extracellular fibrillar matrix and not mucin. Bottom left: Left panel. Hyaline stroma has compressed the cribriform lobules into smaller cellular clusters that still manifest small pseudoglandular formations. Right panel. Advanced hyalinization has reduced the cellular component to narrow curvilinear strands. Bottom right: Tubular variant of an adenoid cystic carcinoma with eosinophilic secretory material in the lumens, shown in the inset to be intensely periodic acid Schiff–positive. Note that the outer cells of the glandular units do not splay off into the nonmyxoid stroma, unlike the feathery ablumenal cellular pattern in pleomorphic adenoma. (Hematoxylin and eosin: top left, ×200; top right, ×200; inset, ×600; middle left, ×200; inset, ×400; Periodic acid Schiff: middle right, ×400; Hematoxylin and eosin: bottom left, left panel ×100; bottom left, right panel ×100; bottom right, ×100; periodic acid Schiff: inset, ×600).

Of the five adenoid cystic carcinomas arising primarily in the main lacrimal gland, the widest diameters of the lesions ranged from 1.4 cm to 3.1 cm, with a mean of 2 cm. The lesions were removed only exceptionally as single specimens and more typically fragmentarily. The outer perimeters of some lesional fragments possessed only thin partial pseudocapsules that were commonly infiltrated by small islands of tumor cells. Five tumors had a combination of cribriform ( Figure 2 , middle left and middle right) and tubular patterns; one cribriform lesion was reduced to small tumor cell clusters with pseudolumens or curvilinear strands by a prominent enveloping and compressive hyaline stroma ( Figure 2 , bottom left, left and right panels); one lesion in the sinus had a trabecular pattern occasionally punctuated by minute lumens; one had a predominantly tubular pattern ( Figure 2 , bottom right); and one had a predominantly solid/basaloid pattern ( Figure 3 , top left and top right). A final tumor manifested a mixture of the most unusual features: subconjunctival multinodularity due to its origin in an accessory gland of Wolfring in the lower lid tarsus ( Figure 3 , middle left); small lumen-forming cellular clusters suggestive of a pleomorphic adenoma ( Figure 3 , middle right); tightly packed tubular structures ( Figure 3 , bottom left); and interanastomosing cords of small, banal, basaloid cells encountered more typically in extremely rare monomorphic adenomas ( Figure 3 , bottom right). In lesions with a tubular pattern, the true lumens focally contained PAS and mucicarmine-positive eosinophilic secretions ( Figure 2 , bottom right inset). Evidence of perineural invasion was seen in three cases ( Figure 2 , middle left inset).

Histopathology of varieties of lacrimal gland adenoid cystic carcinoma. Top left: Aggregates of small blue cells, representing the solid/basaloid variant of adenoid cystic carcinoma, are separated by a collagenous stroma. The bottom left inset reveals the regular, bland, round character of the tumor cell nuclei. A small pseudolumen with fibrillar material is shown in the bottom right inset. Top right: Solid/basaloid lobules of tumor display bone invasion (arrow). There are scattered cribriform formations within the small lobules in the upper left portion of the field. Middle left: Multiple, suspicious, different-sized nodules (arrows) are present subconjunctivally, having originated in an accessory tarsal gland of Wolfring. Some pathologists thought that the lesion was an “atypical” pleomorphic adenoma. Middle right: The arrangement of the cells with small lumens in a loose stroma looks deceptively like a pleomorphic adenoma. The inset shows a classical cribriform lobule at a deeper level of sectioning. Bottom left: Myriad back-to-back tubules with secretory material were also discovered. Bottom right: Cords of small basaloid cells are imitative of a monomorphic adenoma in this field. (Hematoxylin and eosin: top left, ×100; bottom left inset, ×600; bottom right inset, ×400; top right, ×200; middle left, ×40; middle right, ×200; inset, ×100; bottom left, ×200; bottom right, ×100.)

Immunohistochemical Findings

The immunohistochemistry results of nuclear staining with the four probes are summarized in Table 3 . The Ki-67 proliferation index for the pleomorphic adenomas ( Figure 4 , top left) was relatively low with an average of 3.8% (range of 2.6% to 5.4%). The average Ki-67 proliferation index for adenoid cystic carcinomas ( Figure 4 , top right) was 29.1% (range of 23.8% to 42.5%). The basaloid variant had the highest Ki-67 positivity of 42.5% ( Figure 4 , top right inset). p53 in the pleomorphic adenomas ( Figure 4 , middle left) displayed an average positivity of 18.5% (range, 11.9% to 28.9%). The average p53 positivity in the adenoid cystic carcinomas ( Figure 4 , middle right) was 39% (range, 24.2% to 50.3%). Two cases (the solid/basaloid adenoid cystic carcinoma and the adenoid cystic carcinoma originating in the maxillary sinus) were totally negative for p53, possibly an artifactual finding. In all specimens of pleomorphic adenoma, PLAG1 stained strongly positive in the outer ductular myoepithelial cells and spindled stromal cells ( Figure 4 , bottom left and bottom right). The adenoid cystic carcinomas were all negative for PLAG1 ( Figure 5 , top left). None of the pleomorphic adenomas stained strongly positive for MYB: two cases stained focally and weakly positive for MYB, and five cases were totally negative, including the myoepitheliomatous variant ( Figure 5 , top right and middle left). In six cases of adenoid cystic carcinoma, tumor cells stained strongly positive for MYB ( Figure 5 , middle right, bottom left and bottom right), whereas one case was negative. Positive staining was seen in both the outer myoepithelial cells and the inner luminal cells in cases with mixed tubular-cribriform and predominantly tubular patterns. MYB staining was diffuse and uniform within the solid lobules of cells of the basaloid tumor ( Figure 5 , bottom right).

Table 3

Immunohistochemical Results for Lacrimal Gland Pleomorphic Adenoma and Adenoid Cystic Carcinoma of the Ocular Adnexa

	Ki-67 Proliferation Index (Mean [Range])	P53 Positivity (Mean [Range])	PLAG1 (Positive/Negative)	MYB (Positive/Negative)
Pleomorphic Adenoma (n = 7)	3.8% [2.6 − 5.4%]	18.5% [11.9 – 28.9%]	7 b /0	2 c /5
Adenoid Cystic Carcinoma (n = 7)	29.1% [23.8 – 42.5%]	39% [24.2 – 50.3%] a	0/7 b	6 b /1

Only gold members can continue reading. Log In or Register to continue

Share this:

• Click to share on Twitter (Opens in new window)
• Click to share on Facebook (Opens in new window)

Related

Related posts:

1. Systemic Diagnostic Testing in Patients With Apparently Isolated Uveal Coloboma
2. Effects of Choroidal Vascular Hyperpermeability on Anti–Vascular Endothelial Growth Factor Treatment for Polypoidal Choroidal Vasculopathy
3. Conjunctival Mini-flap Operation for Restrictive Strabismus After Periocular Surgery
4. Surgical Results of a Muscle Transposition Procedure for Abducens Palsy Without Tenotomy and Muscle Splitting

Stay updated, free articles. Join our Telegram channel

Join