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We report the case of a patient presenting with eyelid hernias who required bilateral upper and lower blepharoplasty; histological examination of the excised fat revealed B-cell non-Hodgkin's lymphoma. At diagnosis, the disease was already systemically advanced, but the patient was asymptomatic. No sign of disease had been detected in the preoperative tests. The bilateral orbital presentation of a systemic lymphoma is very rare, and is usually accompanied and revealed by exophthalmos, increased tear secretion, diplopia and decreased visual acuity. To our knowledge, this is the first case in which lid hernias were the first and only clinical sign of such a systemic disease.
In October 2001, a 48-year-old man presented to our department for treatment of bilateral upper and lower eyelid hernias. He complained about the senile appearance of his orbital region and the sense of effort required to open the lids, particularly in the evening. He had suffered from glaucoma and conjunctivitis for many years, and had undergone two ophthalmic examinations, which had ruled out any contraindication to surgery of the lids.
The patient was a heavy smoker but had no other relevant history.
Physical examination revealed asymmetry of the face and of the two orbital regions. The orbital fat was herniated, causing bilateral swelling of the upper and lower lids, especially on the right. The overlying skin was flaccid, particularly at the lateral canthus, decreasing the palpebral fissure. Mild redness of the conjunctiva was present (Fig. 1) .
Figure 1Preoperative appearance of the patient. The bilateral upper and lower lid hernias are evident. Exophthalmos is not present.
Preoperative laboratory tests showed a slight increase in white blood cells. Blood and urine analysis were normal. An electrocardiogram and chest radiograph were also normal.
Under sedation, we performed a standard upper and lower blepharoplasty, excising the redundant skin and the fat bulging through the orbital septum, which appeared to be attenuated. The excised fat was red and hard, very different from the common soft yellow orbital fat (Fig. 2) .
Figure 2The fat herniated from the orbit was reddish and hard, very different from that commonly found during a blepharoplasty.
Because of the unusual nature of the fat, it was examined histologically. This showed a neoplasm composed of monoclonal B lymphocytes. The high proliferative index and the uniform centrocytic-like morphology were suggestive of a mantle-cell lymphoma, and this diagnosis was confirmed by the cyclin D1 overexpression observed on immunohistochemistry. Bone-marrow biopsy showed a massive infiltration (>75%) of lymphomatous cells.
NMR showed extensive bilateral involvement of the orbits (Fig. 3) . A total-body CT scan showed systemic disease involving the mediastinal and abdominal nodes. The postoperative course was normal, and the stitches were removed 7 days after surgery.
Figure 3NMR shows lymphomatoid tissue invading the orbital fat, particularly on the left.
The orbit is a rare site of secondary dissemination for systemic non-Hodgkin's lymphoma, being involved in only 5.3% (10/187) of cases, according to Bairey et al.
Bairey et al report only one patient (0.5%), 71 years old, with bilateral involvement, which became clinically evident about 46 months after the diagnosis of systemic lymphoma.
Lazzarino et al studied the characteristics of non-Hodgkin's lymphoma presenting in the orbit.
They report that the incidence of this clinical presentation is 2.5% (8/325 patients), and in their series 5/325 patients (1.5%) had clinically evident bilateral involvement, always accompanied by exophthalmos or conjunctival masses.
In our case, the patient was completely asymptomatic, and no exophthalmos, lymphadenopathy, cutaneous nodules or abdominal masses were detected during the preoperative physical examination. No haematological abnormalities were found in the preoperative tests. The appearance of the orbital regions caused by lid hernias that both the patient and the surgeon attributed to facial aging and which were corrected by a blepharoplasty, was the first and only clinical sign of widespread B-cell lymphoma.
We found unusual histological features, compatible with mantle-cell lymphoma. The most frequent type of lymphoma with orbital involvement is the lymphoplasmocytoid subtype, as reported by Lazzarino et al.
Their eighth patient had an aggressive lymphoma with a diffuse centrocytic-like morphology, very similar to that in our case, which would probably, today, be classified as a mantle-cell lymphoma. It affected only the left orbit, while in our case the involvement was bilateral and synchronous.
References
Bairey O
Kremer I
Rakowsky E
Hadar H
Shaklai M
Orbital and adnexal involvement in non-Hodgkin's lymphoma.
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