Centrofacial angiocentric lymphoma

PERAL-CAGIGAL B, GALDEANO-ARENAS M, CRESPO-PINILLA JI, GARCÍA-CANTERA JM, SÁNCHEZ-CUÉLLAR LA, VERRIER-HERNÁNDEZ A. CENTROFACIAL ANGIOCENTRIC LYMPHOMA. MED ORAL PATOL ORAL CIR BUCAL 2005;10:90-4.

 

SUMMARY

The centrofacial angiocentric lymphoma is a rare lymphoid neoplasm, with an often-difficult diagnosis due to the non-specific clinical picture. On many occasions it is necessary to perform various biopsies to reach the correct diagnosis.
This lymphoma is an aggressive Non-Hodgkin’s (NHL) type, which is normally found in the upper respiratory tract (predominantly in the nasal cavity), and has an ominous prognosis, as the average survival rate is between 12 and 18 months (1). It is predominantly found in subjects of oriental and South American extraction, who are between the ages of 50 and 60 years and with a slight tendency towards males (2:1).
This is the case study of a female Ecuadorian patient who was referred to our department with a hemifacial edema, chocolate-like rhinorrhea and nasal respiratory obstruction, which had been treated with antibiotics and anti-inflammatories for a month without success. After performing a number of diagnostic tests, it was found histologically that the patient had an extranodal T-cell lymphoma of the nasal type (also known as T-cell angiocentric lymphoma).

Key words: Centrofacial angiocentric lymphoma, T-cell nasal lymphoma, lethal midline granuloma.

 

INTRODUCTION

Originally known as lethal midline granuloma, the centrofacial angiocentric lymphoma is not commonly found, although it has received a variety of names including polymorphous reticulosis or lymphomatoid granulomatosis.

It is a very destructive and fast-acting neoplasia which is found in upper airways (especially in the nasal cavity) and palate although there have been extrafacial cases (skin, lung, lymph glands and gastrointestinal tract).

The most common symptoms are fever, weight loss, nasal obstruction, chocolate-like rhinorrhea, nasal edema and/or facial, and ulcero-necrotic lesions in the nasal cavity, gums and palate.

It is predominantly found in subjects of oriental, south American and Mexican extraction. There is an almost constant relationship with the Epstein-Barr virus that is detected by means of the PCR in more than 95% of the cases (2). This virus is also associated with other types of lymphomas such as Burkitt lymphoma, large cell and anaplastic lymphoma or opportunist lymphomas in immuno-depressed patients.

The poor prognosis is due to a very aggressive neoplasia that produces extensive necrosis and angioinvasion which results in an average survival of between 12 and 18 months. The prognosis worsens if the patient is immuno-depressed as in the cases of those with AIDS or tuberculosis.

The standard treatment is polichemotherapy with CHOP (cyclophosphamide, adriamycin, vincristine and prednisone) protocol, although other treatments have been tried such as Radiotherapy.

One common complication that arises in these patients is the hemophagotic syndrome which is associated with various factors including the Epstein-Barr virus to which part of its pathogenesis can be attributed (3).

CASE REPORT

The subject is a 36-year-old Ecuadorian woman, fitted with IUD, who had undergone an operation for right maxillary sinusitis four months previously. She was referred to our Oral and Maxillofacial Surgery Service with a left side hemifacial edema, chocolate-like rhinorrhea and left side nasal respiratory obstruction which had been treated with antibiotics and anti-inflamatories for a month without success.

On examining the patient an important left side hemifacial edema was found together with a large preauricular adenopathy , both of which were very sore to the touch. The rhinoscopy showed an extensive scabby area in the left-hand nasal cavity. The oral examination was found to be normal without signs of ulcero-necrotic lesions.

The neck exam showed a month-old ulcerated lesion of approximately 5 cm in diameter, with erythematosis and hard sides and a weeping bed, in the right supraclavicular area. The patient complained of acute pain. The lesion progressed to central necrotic scab (Fig.1 and 2), that was removed so that the process of granulation and healing could take place (Fig.3). The patient also complained of constitutional symptoms such as fever, asthenia and weight loss in the previous months.

A blood analysis, biochemical and coagulation were carried out and they showed the existence of leucopenia(2600), monocytosis(19.40%), slight normocytic and normochromic anemia and a globular sedimentation rate (ESR) of 42 mm/h.

Various serolgies were performed of which the anti -Toxoplasma IgG, anti HSV and VZV IgG, anti-Chlamydia pneumoniae IgG and anti-Bartonella henselae Ig G proved positive, although none with qualification of infection at the present moment. The rest of the serolgies, those for Chlamydia psittaci and C. trachomatis, Hepatitis B and C, HIV, mononucleosis , leishmaniasis, syphilis and tularemia, proved negative. The Mantoux, the urine culture, of the nasal smear, of the ulcerated supraclavicular lesion, and hemocultures (collected at fever peaks) also proved negative, as did the blood extension looking for parasites.

An orthopantomography, a Waters projection and a simple X-ray of the thorax and abdomen were carried out and no pathologic discovery was found. A cervicofacial CT revealed an enlargement of soft tissue in the nasal septum, that crossed the midline and extended over the soft parts of the face. It also had necrotic adenopathies in the left front jugular chain (Fig.4).

Following this an FNA (fine needle puncture aspiration) of the supraclavicular lesion was performed that was reported as a suggestive atypical primary or metastatic process. After this finding a biopsy of the intranasal tumoration was carried out but only necrotic inflammatory material without signs of malignancy were discovered. Finally the histological diagnosis of T-cell nasal lymphoma was reached by performing a biopsy on the right ulcerated supraclavicular lesion. A dense atypical lymphoid infiltrate, diffused, with angiocentric and angiodestructive increase, with fibrinoid changes on the vessel walls and accompanied by extensive coagulative necrosis was found. The atypical lymphoid proliferation is made up of small- and medium-sized cells with isolated large cells. The immunohistochemistry study showed a T inmunophenotype proliferate lymphoid cluster, with positivity for the CD45RO, CD43, CD3 and CD5, and isolated positivity in some cells for CD30. The cellular proliferation rate measured with the Ki67 marker was higher than 95%.

In the extension study neither adenopathies nor organomegalias on a thoraco-abdominal level were identified. The serum beta 2-microglobulina is used as a tumoral marker, as its presence can be high in this type of lymphomas being its level according to the tumoral size; in this patient the levels were normal.

After finally reaching the diagnosis of a nasal lymphoma, it was decided that the patient should undergo chemotherapy with CHOP (cyclophosphamide, adriamycin, vincristine and prednisone). To date the patient has received 3 cycles of chemotherapy which have proved successful as the hemifacial tumefaction has disappeared, the ulcerated supraclavicular lesion has healed completely and in the new cervicofacial CT the intranasal tumoration does not appear.

DISCUSSION

The angiocentric lymphoma is an NHL included in the REAL (Revised European American Lymphoma) classification as a peripheral T-cell and NK-cell lymphoma. These make up less than 10% of all malignant lymphomas (4). The term “nasal type” refers to the location of the lymphoma in the nasal cavity as typical and more common, as in our case although it can also occur in other facial or extrafacial areas.

The diagnosis is normally difficult as clinically it is non-specific and histologically it is a tumour that produces extensive necrosis, that normally requires various biopsies before reaching a diagnosis (our patient needed two). As a result, the immunohischemistry study proved very helpful.

The most common symptoms are fever, weight loss, chocolate-like rhinorrhea, nasal obstruction and nasal and/or facial edema, all of which were present in our case.

In a similar clinical picture a number of illnesses should be excluded by means of adequate laboratory techniques: these include histoplasmosis, blastomicosis, coccidiomicosis, leprosy, tuberculosis, syphilis, mucocutaneous leishmaniasis, rhinoscleroma and pseudotumour of the orbit (5).

Polichemotherapy with CHOP is regarded as the standard treatment against aggressive NHL, as it is easy to perform and of low toxicity both in limited and advanced pathology (6).

The International Non-Hodgkin’s Lymphoma Prognostic Factors Project has established 4 prognostic groups based on age in a simple and easy-to-reproduce way: the Ann Arbor stage, serum LDH concentration, the general state according to the ECOG (Eastern Cooperative Oncology Group) scale and the number of extraganglionar places affected (7). It is probable that the primary resistance of the lymphoma in being treated is the most unfavorable prognostic factor.

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