Otolaryngology: Open Access
Open Access

This is a hematological malignancy characterized by monoclonal B cell proliferation, it is a non-Hodgkins lymphoma (NHL) high degree of malignancy. Its former name is the Lymphatic Reticulo-Sarcoma. This retrospective study is descriptive and aims to report data from 10 years of experience in Senegal. The purpose of the study is mainly to show the decrease of cases of LB in maxillofacial location in Senegal. This retrospective study cannot point to etiologies or discoveries that have clinical and therapeutic implications. It tries to find an explanation for the progressive disappearance of maxillofacial localization.

The study is retrospective covering a period 10 years (1994 to 2004), during which all patients with LB files (20 files) were investigated and 11 were selected. Epidemiological, clinical, paraclinical, therapeutic and evolutionary are presented. Ethical considerations were done with informed consent.

Epidemiological aspects

The series consisted of 11 cases over a period of 10 years so a frequency of 1 case per year. The average age was 9 years old. Sex ratio was 1. The average time of consultation was 4 and half months.

In terms of geographical origin, the wolof ethnic group was the most affected with 6 cases.

The remaining was: (Mandingo: 1 case, Diola: 1 case, Serere: 1 case, Peul: 1 case, Tukulor: 1 case)

Past history

We observed tooth extraction (1 case), facial trauma (1 case), inbreeding (2 cases) were recognized.

Clinical aspects

Reasons for consultation: The majority of patients consulted for a facial mass in 10 cases either (90%), dental signs in 2 cases (18.2%), impaired general condition in 1 case (9%) and neck lymphadenopathy in 1 case (9%).

Distribution of LB’s site: Facial and extra facial location.

Maxilla was preferred initial tumor location of Burkitt lymphoma: 6 cases or 54%; then it touched the mandible in 3 cases or 27.2% and finally the two bones (maxilla and mandible) in 2 cases or 18.8%. (Figures 1 and 2). Extra-facial sites were in 2 cases the spleen, liver and skeleton in 1 case.

Figure 1: A 4 year old girl with LB Location of the maxilla. Classic look “Facies of Batracien”.

Figure 2: A 6 year old girl with a LB mandibular localization.

Distribution of nodal sites: Cervical lymph nodes enlargement was found in 3 children: 27% and in one case (9%) it was a extra cervical lymphadenopathy.

Clinical investigations

Hypochromic anemia was found in 6 patients (54.5%).

Panoramic radiography objectified images of osteolysis 1 times.

Two (2) patients had an abdominal ultrasound: 18.2%.

Anatomical pathology results were obtained through a needle aspiration biopsy in 2 patients (18.2%) and tumor biopsy in 9 patients (81.8%). Cytology found the “cell Burkitt” and in biopsy specimens: A “starry sky” look (Figure 3).

Figure 3: Histology of a Burkitt lymphoma.

Distribution Murphy classification: Therapeutic data

A chemotherapy was instituted in 8 children cyclophosphamide, oncovin, Natulan and others). Adjuvant 2 where TS. An emergency tracheotomy was done in 2 children (Table 1).

Table 1: Distribution of cases of Burkitt lymphoma of the series as Murphy classification.

Scalable data or follow data: In the short term, 1 Death has been reported. Survival has been reported in 4 children (36%). 4 Four patients had a transfer in Pediatrics. The long-term complete remission was reported in 4 patients 4 cases (36%) and 2 patients were lost to follow (18%) (Table 3).

Table 3: Etiology aspect evoked in litterature.

Epidemiology

The geographical distribution of LB is well known. There are three areas:

• High incidence area (endemic forms LB): African belt

• Low incidence area (sporadic) (Europe, USA)

• Intermediate impact area (North Africa, South America).

In Africa, in “Burkitt tumor belt,” There are areas where the LB rampant than in others like Nigeria where Ajayi et al. [1] reported a rate of 38.3% of LB on a set of 47 orofacia l malignancies in children and in Papua New Guinea 64% of LB are reported of 67 tumors [2]. In Libya, the incidence seems to be lower [3].

Burkitt and O’Conor [4] reported in the 1960s that LB was 40 to 70% of childhood cancer in Africa. The Senegal belongs to the “Burkitt tumor belt” between Southern Africa and North Africa and occasionally a work devoted to head and neck cancers of the child, Diop [5] was estimated at 100% LB over 5 years.

Comparisons with others studies

Most retrospective series in black Africa range from 1990 to 2000 and reported that the LB is the most common tumor in children (Table 2).

Table 2: LB series in some African countries.

To believe the numbers, there was a marked decrease in cases, two decades after in Senegal, in fact, the current series (1994-2004) found 11 cases in 10 years: 1 case per year; compared to the DIOP series [5]. Then, since 2004 until 2015, the ENT department of Dakar recorded only 2 cases of LB in Head and Neck. Locations were exceptional at the mastoid and in the nasopharyngeal space. In Senegal, LBs with maxillofacial location were the subject of very few publications before 1990. M DIOP reporting 33 cases in 8 years [5] it is the most important work published in Senegal. After a review of the more recent literature, there are 15 cases of maxillofacial LB followed by haematoclinics diagnosed before 1998 [6,7]. The report is also made by the onco-pediatricians of the important center of Aristide Le Dantec, the abdominal forms take over. Unfortunately no comparative study is published at home.

What causes to the disappearance of the head and neck LB in our country? What are the factors affecting the epidemiology of childhood ENT cancer? Several studies showed that LB would be linked to the virus Ebstein Barr [8]. In Madagascar [8] and Uganda [9-11], seasonal predisposing factors were responsible. For South African series, Madagascar and Malawi, there would be more of LB during the rainy season, period of highest transmission (EBV vectors) [11]. While Nigerian [1] and Ugandan [11] studies show more than LB during the dry season. Thus, the explanation is the conjunction, at this age, of malnutrition, frequent parasitic infections and multiple and repeated immune stimulation in an immunologically immature and fragile terrain [8].

Senegal has known for 10 years more increased drought, -this would be one reason for the decline in the LB in our country. Among other causes, this decrease would it be a sign of:

• Better health coverage

• -Fight against malaria vectors

• Better life (given that it is children from disadvantaged social layer with low social economic level that are most affected)

• Climate change.

Table 2 shows that the distribution of LB within the “belt”, in the last decade for other African countries remains disparate. There is a singularity of the panorama of the pediatric head and neck oncology in Senegal with the resurgence of squamous cell carcinoma in children (cancers of the hypopharynx [12]), at the expense of lymphomas.

Clinical stages

The average age of onset of Burkitt lymphoma varies according to studies, it is 4 years in a Cameroonian study [7] and 8 years in Togo [10]. The predominance of clinical stages III of Murphy in our series is observed in other African series [7]. It is explained by the late period before the medical visit: 4½ months in our series and 15 days to 3 and a half months in Madagascar [8], allowing time for the disease to develop. All studies report a male predominance [7].

The African form is characterized by a maxillofacial swelling; our series reports a preferential involvement of the upper jaw. The overlying skin is stretched, shiny. There is almost never ulceration. Clinical manifestations depend on the location of LB with “swelling cheek and upper jaw involvement”. Sometimes nasal obstruction in a child may be the only first event [13,14]. Other unusual locations such as the tonsil or parapharyngeal space, parotid space or sphenoid sinuses are reported in the literature. Orbital Extensions (rarely primitive) and the nasal passages are also published [15-17].

Apart pathological anatomy, others common diagnostic tests are hemogram, renal balance sheet, uric acid and EBV serology and HIV. Imaging including: x-ray bone (bone, chest), lumbar puncture and finally a myelogram ultrasound (liver kidneys, spleen, ovaries, adrenal glands, testicles).

Treatment protocols vary: VMCA, CMA, CM, CM strengthened. Ivory coast teams were awarded with the CMA, 76% remission. [9] While Moroccan series received LMB89 protocol (high doses of cyclophosphamide, vincristine, doxorubicin and prednisone) 68% of remission [13].

In Senegal, the assessment of treatment outcomes is difficult because of the large number of lost to follow the cure of attack and the lack of feedback from patients transferred in pediatrics. For a long time, accessibility problems to treatment have lowered the prognosis, Senegal today have a pediatric oncology unit belonging to the Franco- African Pediatric Oncology Group using cyclophosphamide alone in LB of Children in Senegal [18].

Changing epidemiology of head and neck cancers of the child in Senegal is noted, the LB in head and neck presentation seems to disappear into tumors of the head and neck for ten years. The situation in other African countries LB would be interesting to know, to see if the differences in the incidence of LB linked to geographical distribution are still relevant.

Key points

• Epidemiology of neck cancer of the child in Senegal is changing.

• As this study demonstrates such decrease of the incidence of the Burkitt’s lymphoma comparing with previous studies and compared to other African countries.

• Reasons of this decline may have socioeconomic explanations.