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NR 7-9/2008
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Recurrent benign
pleomorphic adenoma of the lacrimal gland – a case report
Nawrót guza mieszanego
gruczołu łzowego – opis przypadku
Bogusława Orzechowska-Wylęgała1
Piotr Jędrzejewski1, Edward Wylęgała2,
Katarzyna Stęplewska3
1 Department of Maxillofacial Surgery, Medical
University of Silesia, Katowice, Poland
Chairman: Prof. Jan Drugacz MD, PhD
2 Ophthalmology Department of Railway District
Hospital Katowice
Department of Nursing and Social Medical Issues, Medical
University of Silesia, Katowice, Poland
Chairman: Prof. Edward Wylęgała MD, PhD
3 Chair and Department of Pathomorphology in Zabrze,
Medical University of Silesia, Katowice, Poland
Chairman: Daniel Sabat MD |
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| Summary: |
Benign pleomorphic adenoma
(mixed tumor) is the most frequently occurring tumor of
epithelial origin. It occurs in serous glands, most
frequently in parotid glands and rarely in small
palatine glands, cheek glands, palatine tonsils and the
lacrimal gland. This study is a description of the
therapeutic management of a patient with recurrent
pleomorphic adenoma of the lacrimal gland. In 1996, a 35
years old male patient underwent surgery to remove a
tumor of the left orbit. Histopathological examination
revealed pleomorphic adenoma. From 1998, the patient had
observed increasing exophthalmus of the left eyeball. In
1999, vision distortion and headaches had begun. In
2005, from a cut along the upper-outer edge of the left
orbit, an elastic and soft tumor 1 cm in diameter was
removed from soft tissues of the upper eyelid. Then,
with the use of the side orbitotomy technique, the orbit
was opened through incision of the temporal muscle and
removal of the orbital side wall. An eyelid nodule of
0.8 mm in diameter was removed together with an elastic
and hard tumor of size 2.5 × 1 cm. The result of the
histopathological examination of the palpebral tumor was
pseudocystic tumor and of the orbital tumor was
pleomorphic adenoma. Conclusion: patients resected for
pleomorphic adenoma of the lacrimal gland require a long
period of postoperative observation and periodic
ophthalmologic examination. |
| Key words: |
pleomorphic adenoma,
lacrimal gland, diagnosis, treatment. |
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Introduction
Benign pleomorphic adenoma (mixed tumor) is the most frequently
occurring tumor of epithelial origin. It occurs in serous glands,
most frequently in parotid glands and rarely in small palatine
glands, cheek glands, palatine tonsils and the lacrimal gland.
It accounts for approximately 50% of the total lacrimal gland
tumors of epithelial origin and occurs more frequently in males
(60%) than females (40%) between the third and the seventh
decade of life (on average around the age of 40). It occurs
extremely rarely in juveniles (1).
The lacrimal gland consists of two lobes: the palpebral lobe and
the more deeply located orbital lobe, separated by the
aponeurosis of the upper eyelid levator muscle. Although the
eyelid lobe constitutes between one-third and one-half of the
total size of the orbital lobe, the mixed tumor stems mostly
from the deep lobe (2). A characteristic quality of the benign
pleomorphic adenoma is its very slow pain-free growth and its
inclination for local recurrence. As a result of its increasing
weight, the adenoma causes displacement of the eyeball most
often directed downwards and outwards, which leads to
exophthalmus and double vision. A characteristic symptom is also
a slowly progressing ptosis. Most patients suffer from such
symptoms at least a year before starting treatment (3).
Diagnosis of the mixed tumor is based on clinical and
radiological examinations which take the form of computer
tomography of the frontal plane, and in some cases also magnetic
resonance imaging (4).
Treatment of the benign adenoma revolves around radical surgical
resection of the tumor together with pseudocapsule and a margin
of orbital tissues. The tendency for local recurrence even many
years after the extirpation of the tumor may indicate its
malignant transformation. It is characterized by its very rapid
growth with accompanying pain and this quickly leads to
distortion of vision. So far it has not been discovered whether
a tumor resected not radically enough is the cause of its
malignant transformation, or whether it is the cause of a new
tumor of malignant characteristics (5).
The vast majority of authors advocate the avoidance of
aspiration biopsy of the pleomorphic adenoma due to the
possibility of damaging the capsule and scattering the malignant
cells into neighboring tissues (3,4,5,6).
The aim of this study is a description of the therapeutic
management of a patient with recurrent pleomorphic adenoma of
the lacrimal gland.
Case report
In 2005, a 35 years old patient (J.K.) came to the Clinical
Department of Maxillofacial Surgery in Katowice with symptoms of
exophthalmus and vision distortion in the left eye, which made
it difficult for him to work. In 1996, he had undergone surgery
to remove a tumor of the left orbit. The histopathological
examination revealed pleomorphic adenoma. From 1998, the patient
had observed increasing exophthalmus of the left eyeball. By
1999, vision distortion and headaches had begun. The patient did
not exhibit any general disorders and no history of hereditary
disease in the family. The initial examination revealed
exophthalmus of the left eyeball, inclined medially downwards.
In the upper-outer orbital quadrant (within the eyelid), an
elastic tumor was available for palpable examination, movable in
relation to neighboring tissues. No diplopia occurred and
reaction of pupils was regular. An ophthalmological consultation
was carried out and revealed the following symptoms: decreased
vision acuity down to 0.5, diplopia, exophthalmometry: RE 20 mm,
LE 27 mm. The fundus of the left eye: optic disc II of blurred
contours and raised; the retina plicated in the middle.
Computer tomography was carried out using the spiral method,
with 0.5 mm layers with and without contrast medium (Fig. 1).
The postoperative site contained recurrence of the tumor.
Exophthalmus of the left eyeball was recognized. The eye
additionally inclined medially due to the weight of the tumor,
with no infiltration characteristics. There was loss of bone
tissue adjacent to the tumor. The mass of the tumor slightly
strengthened after administering the contrast medium. The right
orbit and its contents remained unchanged.
The decision for surgical treatment was taken. In September
2005, surgery was carried out. From the cut along the
upper-outer edge of the left orbit, an elastic and soft tumor 1
cm in diameter was removed from soft tissues of the upper eyelid.
Then, using the side orbitotomy technique, the orbit was opened
through incision of the temporal muscle and removal of the
orbital side wall. An eyelid nodule of size 0.8 x 1.5 cm was
removed together with an elastic and hard tumor of size
1.5 x 3 cm (Fig. 2).
The result of the histopathological examination:
1. Palpebral tumor
A pseudocystic tumor (0.8 x 1.5 cm) with fibrous pseudocapsule
was sent for examination. Neoplasmatic cells infiltrated the
pseudocapsule of the tumor in some areas (without crossing the
pseudocapsule). The tumor showed epithelial cells forming
tubular structures arranged in an irregularly anastomotic
pattern, lying in a myxoid stroma. The inner layer of the
epithelium secreted mucus or underwent squamous metaplasia.
2. Orbital tumor
An elastic, gray-brown tissue fragment (1.5 x 3.0 cm) was sent
for examination. The histological section showed fragments of
lacrimal gland, fibrous connective tissue, adipose tissue and
fragments of bone trabeculae. The numerous focuses of
pleomorphic adenoma infiltrated these tissues. Not many
epithelial cells lay in a myxoid stroma which predominated in
this tumor (Fig. 3).
The follow-up course was non-complicated. The patient was
referred to the Oncological Institute for consultation but this
did not confirm if the tumor was malignant. Periodic check-ups
were recommended to be carried out at the operating center. The
patient remains under periodic ophthalmological examination.
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Discussion
Pleomorphic adenoma is the most frequent tumor of the lacrimal
glands (50-60%). The tumor most frequently occurs in young
adults (average age 35 years). It occurs twice as often in males
than females. It is a locally malignant tumor which may
infiltrate its own pseudocapsule and the adjacent periosteum. If
not radically resected, it may cause recurrences in soft tissues
or in the bone tissue (3). The pseudocapsule is created as a
result of pressure of the tumor against the surrounding tissues,
but its focal infiltration can nearly always be found. Such a
tumor is characterized by a considerable variability of its
histopathological image, and hence its name. The
histopathological image reveals a myxoid stroma, low cell stroma
which hosts high cell areas composed of epithelial cells,
forming tubes and ducts incorporated into cords and lobes. The
tubes and ducts are lined with two-layer epithelium. The
interior layer of the cells may secrete mucus, or undergo
squamous epithelium metaplasia. On the other hand, its exterior
layer consists of myoepithelial cells which undergo metaplasia
forming myxoid, fibrous or cartilaginous stroma (4).
Both benign and malignant orbital tumors should be
differentiated from other pathological conditions such as
inflammations, vessel malformations, or disorders of the
lymphatic system. Appropriate diagnosis of tumor alterations
facilitates selection of appropriate therapeutic management. The
pleomorphic adenoma is regarded as a benign tumor whose course
is initially very slow and hidden. It progresses without
destruction of the orbital bones, which, if detected, may
indicate the malignant nature of the tumor (1). In our case, in
the computer tomography, we observed loss of the bone adjacent
to the tumor. Yet, bone destruction may be revealed in the case
of pseudotumor or cholesterolic granuloma whose characteristic
feature is bleeding inside the tumor. In some cases, mixed tumor
may develop from additional or ectopic gland tissue. That was
the case with our patient, where under the eyelid there was a
separate small nodule 1 cm in diameter resembling a cyst,
whereas another tumor was present in the orbit. During the
operation, bleeding was of medium intensity, which also bore out
the validity of the original diagnosis (2).
In the MRI examination, it is very difficult to judge if we are
dealing with benign or malignant pleomorphic adenoma. This is of
crucial importance if the appropriate operative technique is to
be adopted effectively. Benign tumors are resected by removal of
the tumor with a margin of adjacent tissues, while malignant
ones require exenteration of the orbit, which is related to
considerable maiming of the patient (4). A solution to the
problem could lie in aspiration biopsy, but the majority of
authors do not recommend this in the case of mixed tumor
(1,3,4,5). The paper by Tse and Folberg contains a description
of a technique which prevents the possibility of scattering the
malignant cells when a diagnostic biopsy is necessary. They
covered the place of injection with a few drops of 2- butyl
cyanoacrylate. Then, they cut out the frozen piece of tumor and
if the diagnosis turned out to be correct, the whole lacrimal
gland was resected (7).
Local recurrences of pleomorphic adenoma turn up at different
time intervals after the first surgery. The majority of authors
consider that approximately 30% of total recurrences are
revealed after over 15 years. Around 10% of the total
pleomorphic adenoma are transformed into malignant tumors after
20 years from the first surgery, and 20% after 30 years (1). Tse
and colleague analyzed the lives of 42 patients treated for
benign pleomorphic adenoma in the period between 0.5 and 17
years after surgery. In total, 34 patients did not suffer from
recurrences or malignant transformations, and 8 had recurrences,
7 of whom underwent multiple surgeries (7).
In the case presented, the recurrence 9 years after the first
surgery could suggest malignant transformation. However, on the
basis of computer tomography, the decision was taken to
implement a sparing procedure, the validity of which was
confirmed by histopathological examination. When pleomorphic
adenoma is present, the entire tumor with its pseudocapsule,
surrounding levator aponeurosis, and conjunctiva must be excised
to avoid recurrences and malignant transformation.
Conclusion
Patients resected for pleomorphic adenoma of the lacrimal gland
require a long period of postoperative observation and periodic
ophthalmologic examination.
References:
1. Sadick H, Riedel F, Naim R, Gossler U, Hormann K, Bergler W:
Benign mixed tumor of the lacrimal gland. ORL J Otorhinolaryngol
Relat Spec 2003, 65, 295-299.
2. Hsu HC: Posttraumatic benign pleomorphic adenoma of the
lacrimal gland. Ophthalmologica 2001, 215, 235-237.
3. Hajda M, Koranyi K, Salomvary B, Bajcsay A: Clinical
presentation, differential diagnosis and treatment of lacrimal
gland tumours. Hungarian Oncology 2005, 49, 65-70.
4. Becelli R, Carboni A, Cassoni A, Renzi G, Iannetti G:
Pleomorphic adenoma of the lacrimal gland: presentation of a
clinical case of relapse. J Craniofac Surg 2002, 13, 49-52.
5. Lakhey M, Thakur SK, Mishra A, Rani S: Pleomorphic adenoma of
lacrimal gland: diagnosis based on fine needle aspiration
cytology. Indian J Patol Microbiol 2001, 44, 333-335.
6. Miyazaki T, Yamasaki T, Moritake K et. al.: Unusual
progression of pleomorphic adenoma of the lacrimal gland – case
report. Neurol Med Chir 2005, 45, 407-410.
7. Tse DT, Folberg R: Technique for incisional biopsy of a
lacrimal gland mass when the diagnosis of benign mixed tumor
cannot be excluded clinically. Ophtalmic Surg 1988, 19, 321-324.
Praca wpłynęła do Redakcji 20.02.2008 r. (1050)
Zakwalifikowano do druku 12.08.2008 r.
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Fig.1. CT scan of the left orbital tumor
Fig.2. Two parts of palpebral and orbital
after tumor removal.
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Fig.3. Myxoid stroma of pleomorphic adenoma
lying in fibrous connective tissue.Magnification ×200. HE.
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