burkitt lymphoma |Tear SAC primary non-Hodgkin's lymphoma

Tear SAC primary non-Hodgkin's lymphoma

Malignant tumors of the Crimea sac are uncommon, and primary non-Hodgkin lymphomas are rare.

We report The case of an 80-year-old woman presenting with epiphora and swelling of the left Crimea area. A dacryocystorhinostomy was programed, but within few weeks a painful tumor appearing with cervical nodes. Pathological studies of the mass revealed diffuse malignant lymphoma with large ß cells. The lymphoma was classified as stage IIE using Ann Arbor staging after clinical, biological and radiological examination. Six CHOP Systemic chimiotherapy were proposed. After 12 months and only 4 cures because of hematological complications and cardiac toxicity, the patient was disease-free.

Primary non-Hodgkin's lymphoma of the Crimea sac is extremely rare. Collaboration between the ophthalmologist, anatomopathologist and oncologist allowed us to determine the type and the extension and to propose an aggressive but successful treatment in this highly malignant and very localized lymphoma.

Crimea sac tumor, dacryocystectomy, non-Hodgkin's lymphoma, primary, systemic chemiotherapy
Tear SAC primary non-Hodgkin's lymphoma

Malignant tear sac tumours are rare and primitive non-Hodgkin's malignant lymphomas are exceptional.

We report the case of a patient hospitalized for the surgical treatment of a dacryocystocèle. Pre-operative examination reveals a suspected lesion of recent onset associated with cervico-facial adenopathies. Resection biopsy highlights the presence of non-Hodgkin's malignant lymphoma with large cell type B. The extension balance (clinical, biological and radiological) allows to classify it stage IIE (Ann Arbor classification). A chemotherapy type CHOP (6 cures), is started, after 12 months and only 4 cures performed due to cardiac and hematologic toxicity, the patient is in complete remission.

Primitive non-Hodgkin's malignant lymphomas of the tear sac are very rare. The collaboration of the ophthalmologist, the pathologist and the oncologist allows to determine the exact type and stage of the disease in order to propose an aggressive but effective treatment in these highly localized lymphomas of high malignancy.

Tear sac tumor, dacryocystorhinostomie, non-Hodgkin's lymphoma, primitive, chemotherapy

Introduction

Malignant tumours of lacrimal pathways are rare.
Most tumours of the tear sac are of epithelial origin and only 5 to 8% of the tumours develop from the Lympho-reticular system.

In the majority of cases they appear as part of a generalized attack. The primary lymphomas of the tear sac are exceptional [1].

We report the case of a patient with primitive malignant lymphoma of the tear sac.

This patient was hospitalized in August 99 in the Ophthalmology Department for a dacryocystorhinostomie of the left lacrimal pathway.

Two months previously, she had consulted for a rénitente swelling of the internal Canthale region, associated with a epiphora, which had been evolving for six months.

The diagnosis of Dacryocystocèle had been evoked, with the remainder of the ophthalmic clinical examination uncharacteristic.

During hospitalization, the lesion increased in size, it became painful and hard to palpate and associated with pre-tragiennes and submaxillary adenopathies farms on the same side, anomalies appeared in some semainesFigure 1.

The lacrimal track is completely waterproof before the bag.

An Oto-rhino-laryngological Society consultation carried out on the same day does not find any other anomalies.

A resection biopsy was then programmed in front of this suspicious tumor.

Carried out in the operating room under general anesthesia by a first direct pathway, it revealed a fleshy, rosy lesion, of encéphaloïde aspect of the tear sac, measuring 1.5 cm in its large axis, invading the mace-nasal canal and infiltrating the Surrounding fabrics in ArrièreFigure 2.

Because the resection could not be complete, an intubation with attempted reconstruction using a Minimonoka probe from the remainder of the largely amputated lacrimal pathway was carried out.

The operating suites were without complications.

The Anatomo-pathological examination found a diffuse lymphomateuse proliferation made of large basophils cells, associated with centroblasts and mature lymphocytes.

It was therefore a lymphoma for which a immunocytochemical marking revealed the presence of the surface antigens of line B: CD 20 and CD 79a, allowing it to be classified as a non-Hodgkin's malignant, diffuse, large-cell primitive sac LacrymalFigure 3Figure 5.

Cytogenetic analysis was not carried out because it had little influence on the patient's therapeutic management.

The patient was then assigned for checkup and therapeutic care in the Hematology department.

The general clinical exam is normal.

The biological examinations showed an elevation of ß2 micro-globulin with a normal lacticodeshydrogénase rate.

The entire CT scan has recovered in part orbital localization, without bone erosion, and numerous cervico-facial adenopathies as well as cavum, more developed at GaucheFigure 6Figure 7.

The bone biopsy puncture was normal.

Lymphoma has been classified as Stage II according to the ANN Arbor classification.

The patient was included in the European multi-centric test protocol GELA (chop vs Chop & MABTHERA) to evaluate the contribution of immunotherapy to Anti-CD 20 antibodies, and poured into the chop group.

Only 4 out of 6 cures were performed due to cardiac and haematological toxicity of the products.

Nevertheless, at twelve months, the patient is in complete remission: more orbital damage and disappearance of adenopathies controlled by TomodensitométrieFigure 8Figure 9.

Discussion

The most common causes of lacrimal pathway obstruction are infection, inflammation, trauma, and congenital malformations.

Lacrimal pathway tumours are a rarer etiology: On a series of 150 patients with clinical signs of lacrimal pathway obstruction, Tucker et al. [2] found only 3% tumor lesions after biopsy of the sac.

These tumours are mainly of epithelial origin: 75%, the remainder being represented by tumours mesenchymal, Lympho-reticular, of Origin ectodermal or Melanic [3].

The classification of lymphomas has evolved considerably in recent years with the contribution of immunohistochemical techniques, and by taking back the large series reported in the literature, they represent about 6% of the tumors of the sac tear, even If some tumors were no longer classified in lymphomas now.

Malignant lymphomas are essentially non-Hodgkin and Line B [4].

They are often secondary to generalized lymphoma, but recently some cases of primitive tumors of the sac have been described [5].

The clinical signs are primarily those of an obstruction of the lacrimal pathways: epiphora, palpation pain of the internal canthus and recurrent Dacryocystitis episodes.

The perception of a hard, painful and fixed tumor in relation to the deep plane at palpation evokes a malignant process.

The appearance of these signs can be isolated or evolve in the context of a generalized lymphomateuseal impairment. For our patient, the lesions found in the cavum are metastatic: The primitive origin is the lesion of the sac.

A dacryocystographie may reveal the presence of a large tumor, show bone erosion but may find only signs of chronic obstruction.

CT imaging is more efficient, highlighting tumor lesions, but does not distinguish in some cases from a cystic lesion of a solid lesion. It may nevertheless suspect a malignant process in the face of bone erosion [6]. In addition, it explores sinuses and the nasal cavity more widely in search of other anomalies.

Ultrasound can make it possible to differentiate a solid tumor from a cystic tumor.

The treatment of tumors of the tear sac is surgical, allowing the most complete resection possible with quality anatomo-pathological specimens.

A reconstruction of the lacrimal pathway allows to treat functional signs.

Here, a ganglion biopsy before any surgical procedure could have made the diagnosis, but the Lymphomateuse etiology is difficult to suspect in this case: infrequent and no notion of widespread impairment.

The classification currently used for non-Hodgkin malignant lymphomas is REAL classification.

The Protocol for the treatment of lymphomas therefore depends on the histological type, the immuno-histo study, the cytogenetic analyses as well as the localization and tumor extension.

In low-malignancy lymphomas that are highly localized in an elderly patient or with a limited life expectancy, therapeutic forbearance is sometimes advocated for these tumours because chemotherapy does not alter the overall survival rate.

Other types of lymphomas benefit from now proven chemotherapy protocols.

The most used is the CHOP association.

Other drugs have been associated with the development of new protocols in some very aggressive lymphomas.

This chemotherapy allows a survival rate of 50% to 5 years, all lymphomas confused [7].

Radiotherapy is sometimes used in lymphomas of high malignancy to allow for a faster tumor reduction; But studies are underway to prove the benefit of such an association.

Our patient presenting a high malignancy lymphoma with limited extension in a context of good general condition a radical therapy has been chosen.

In conclusion, the primitive non-hodgkinnien lymphomas of sac tear are very rare tumours, early diagnosis and collaboration with pathologist and Hematologist allows to propose treatment that remains aggressive but effective in these localized lesions [8].