For citation purposes: Hadchiti W, Nasseh I, Hayek E, Mora F, Bouchard P. Prevalence, location and orientation of maxillary sinus septa. Annals of Oral & Maxillofacial Surgery 2014 May 04;2(1):9.

Research study

 
Implantology

Prevalence, location and orientation of maxillary sinus septa.

W Hadchiti1*, I Nasseh2, E Hayek2, F Mora1, P Bouchard 1
 

Authors affiliations

(1) Department of Periodontology, Service of Odontology, Rothschild Hospital, AP-HP; U.F.R. of Odontology, Paris 7- Denis Diderot University, Paris, France,

(2) Department of Dentomaxillofacial Radiology and Imaging, School of Dentistry, Lebanese University, Lebanon.

* Corresponding author Email: Wahib_hadchity@hotmail.com

Abstract

Introduction

Primary Sinonasal Small Cell Carcinomas, Neuroendocrine type (SCCNET) are rare tumours. Few cases have been reported in the head and neck region of adult patients. They have a fair response to multimodality therapy; but the prognosis remains poor.

Case Report

We report here a case of SCCNET in a 5 year old child. He presented with swelling in his right eye with diminution of vision and right sided neck swelling. Cervical lymph node biopsy revealed poorly differentiated metastatic neuroendocrine carcinoma; confirmed by immunohistochemistry. 18 FDG- PET CT scan revealed a nasopharyngeal mass extending to other paranasal sinuses and base of frontal lobe with bilateral extensive cervical lymphadenopathies. He was treated with neoadjuvant platinum based combination chemotherapy followed by radical radiotherapy. At 17 months post diagnosis he is alive but on palliative care.

Conclusion

SCCNETs have an unfavourable outcome. Further improvements in our understanding of the pathology and treatment strategies are needed to combat the disease.

Introduction

Primary Sinonasal Small Cell Carcinomas, Neuroendocrine type (SCCNET) are rare tumours which occur more commonly in the lungs and gastrointestinal tract [1]. Few cases have been reported in the head and neck region of adult patients, mainly in the paranasal sinuses, salivary glands, nasal cavity, tonsil, larynx, and tongue [2,3,4,5]. The cell origin of the head and neck small cell carcinomas was initially thought to be from amine-precursor uptake and decarboxylase (APUD or Kulchitsky) cells; however, the current opinion favours their origin from multipotential stem cells [6,7]. SCCNETs have a fair response to chemotherapy; chemo-radiotherapy but these are aggressive tumours and ultimately the prognosis remains poor [8,9,10]. There are few reported cases of SCCNETs in the sinonasal region, all in the adult population.[11,12,13]. To the best of our knowledge this is the first reported case of SCCNET in the paediatric age group. He was treated with neoadjuvant chemotherapy to which he had a partial response; was followed up with radical radiotherapy. At 17 post diagnosis he is alive and on palliative care.

Case report

We report a case of a 5 year old child who presented to the paediatric oncology outpatient department of our centre in April 2012, with complaints of gradual increase in swelling of the right neck region for the past 1 month and swelling with diminution in vision of the right eye for 2 weeks.

On examination he was found to have bilateral cervical lymphadenopathies, right eye proptosis with no perception of light in the right eye, and no pupillary reaction. The patient was advised for neck node biopsy.

Right cervical lymph node biopsy revealed metastatic neuroendocrine carcinoma, poorly differentiated. Cells had increased nuclear cytoplasmic ratio, scanty cytoplasm, monotonous round or oval mitotically active nuclei with powdery chromatin. Immunohistochemical stains done in the pathology department of our centre revealed the neoplastic cells to be strongly positive for Neuron specific enolase (NSE), CD56, and Thyroid transcription factor-1 (TTF-1) (Figure 1). Cells were also positive for CD57(Figure 1), and showed patchy positivity for synaptophysin and chromogranin (Figure 2); many of the cells showed peri- nuclear dot like positivity for AE1-AE3 (Cytokeratin) (Figure 2). Neoplastic cells were negative for CD45, CD20, CD3, CD2, Desmin, GFAP, Tdt, S100, CD99, and HMB-45. Ki proliferation index was about 75%. Further work up was done to complete staging. CT scan of the orbit revealed a large heterogeneous sinonasal mass of about 5 cm in largest diameter with extension into the ethmoid, sphenoid and maxillary sinus, intracranial extension into the anterior cranial fossa. CT scan of the neck revealed bilateral level IB, II, III, and V cervical lymphadenopathies. An 18-FDG PET scan showed a nasopharyngeal mass lesion of 4.2x5.5x4.1cm (SUV max 6.4) involving nasal cavity, anterior and posterior ethmoid air cells and sphenoid sinuses bilaterally. Lesion involved extraconal space in both orbits, not separable from the medial rectus muscle. Destruction of lamina papyracea, cribriform plate was present with intracranial extension to the base of the frontal lobe. FDG avid lateral retropharyngeal node (SUV max 4.1), bilateral conglomerated cervical nodal mass involving level II & III, abutting carotid vessels was also noted. Staged as T4N3M0, the patient was started on neo-adjuvant chemotherapy comprising cisplatin and etoposide.

Axial slice used to localize the antero-posterior position of the septum

Coronal slice used to localize the infero-superior position of the septum

After completion of 3 cycles of chemotherapy, interim CT scan of orbit was done to assess response. It revealed enhancing lesion in the sphenoid sinus measuring 22x12mm, mucosal thickening in maxillary, ethmoid and frontal sinuses. Small enhancing intracranial lesion was seen at the base of the anterior cranial fossa measuring 7x8mm (Figure 3). As there was partial response with chemotherapy, multi-disciplinary tumour board (MDT) of the institution decided to continue with chemotherapy for a further 3 cycles. At the end of 6 cycles of chemotherapy, CT scan face reported a small increase in size of the lesion at the base of the anterior cranial fossa measuring 24x18mm; bilateral cervical nodes were seen in level II with the largest measuring 15x12mm. There was no radiological residual in the nasal cavity and para-nasal sinuses.

Axial slice used to calculate the angle between the septum and median palatine suture

He was then planned for external beam radiotherapy with image-guided intensity modulated radiotherapy on Helical Tomotherapy to a 30 fraction schedule over 6 weeks. The gross residual disease was planned for 63Gy. The entire pre-treatment volume covering the sinonasal region and bilateral level I-III+V lymph nodes received 60Gy. The frontal brain and level IV nodes were planned for 54Gy. Concurrent chemotherapy was not planned in view of the fact that the child had received a full course of cisplatin and etoposide (6 cycles). He completed the treatment successfully without treatment breaks, but with manageable Grade 3 mucositis and dysphagia.

He remained on close follow-up. In the first 2 months his acute radiation reactions had subsided and he did not have any symptoms attributable to disease. However, he presented with recurrent headache at 3 months. An MRI of the neck and brain suggested a large progressive lesion in the frontal lobe with haemorrhage. There was no radiological evidence of disease in the rest of the head and neck region. The lesion was not operable. A further multidisciplinary discussion recommended best supportive care only. At 17 months following diagnosis he is moribund but alive receiving palliative treatment.

Discussion

To the best of our knowledge this is the first immunohistochemically confirmed case of primary sinonasal small cell carcinoma, neuroendocrine type being reported in a child. SCCNET of the sinonasal region are rare. In a review of literature by Han et al. in 2012 [16], 55 prior reported cases have been compiled. All were positive for CD56, Synaptophysin and chromogranin A. The largest reported series of 16 cases by Babin et al. is from a consortium of 8 French hospitals [15]. Other large series include those by Perez-Ordonez and Rosenthal [14,17]. Table 1 summarizes a few of the larger series. In all the reports, the patients were adults. The mean age of all the previously reported cases summarized by Ho et al. was 51.3 years. We believe that this is the first report of this histology in a child.

Table 1

study population

Initial clinical differentials included lymphoma but the pathology was confirmed from a lymph node biopsy. The closest histopathological differential diagnosis considered was Olfactory Neuroblastoma. Presence of diffuse perinuclear dot like positivity for AE1-AE3 (Cytokeratin), strong and diffuse TTF-1 positivity and absence of S100 positive sustentacular cells were in favour of SCCNET. S100, CD99, and Desmin, were negative excluding Melanoma, Ewing/PNET and Rhabdomyosarcoma respectively. Neoplastic cells were immunonegative for CD45, CD3 and CD2 which ruled out Lymphomas including NK/T cell lymphomas (Table 2 below gives the differential diagnosis for SNEC). Therefore our case was diagnosed as a case of Primary Sinonasal Small Cell Carcinoma, Neuroendocrine type.

Table 2

distribution of septa

The clinical presentation of cervical lymphadenopathies, sinonasal symptoms and unilateral blindness was in keeping with the usual presentation of sinonasal cancers. The treatments of sinonasal small cell carcinomas have been varied, with reports of chemotherapy, radiotherapy and surgery being used. The use of cisplatin based chemotherapy is common. Radiotherapy is also very commonly used either with concurrent chemotherapy or alone in sequence with chemotherapy. Our patient being a young child and considering his advanced disease presentation was taken up for neo – adjuvant combination chemotherapy followed by radical radiotherapy. Despite completing his planned treatment he developed early large-volume local failure in the brain that could not be salvaged.

Conclusion:

The reported literature also shows an unfavourable outcome with this histology and a short disease-free and overall survival using commonly used treatment protocols. Further improvements in our understanding of the pathology and treatment strategies are needed to combat this disease.

Consent

Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Conflict of interests

None declared.

Competing interests

None declared

References

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11.Lee LY, Chang KP, Hsu CL, et al. Small-cell neuroendocrine carcinoma of the nasopharynx: Report of a rare case lacking association with Epstein-Barr virus. Int J Surg Pathol. 2011 Apr; 19(2):199-202

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    Licensee to OAPL (UK) 2014. Creative Commons Attribution License (CC-BY)

    study population

    Age

    Male

    Female

    0-20

    17

    24

    21-40

    53

    73

    41-60

    63

    79

    61-85

    26

    13

    TOTAL

    159

    189

    Total

    348

     

     

    distribution of septa

    Location of septum

    patients

     

    Nb of septum

    Nb of Right septum

    Nb of Left septum

    1 Septum in right sinus

    39

    39

    39

    0

    1 Septum in left sinus

    39

    39

    0

    39

    2 Septum in right sinus

    4

    8

    8

    0

    2 Septum in left sinus

    2

    4

    0

    4

    1 in right sinus + 1 in left sinus

    31

    62

    31

    31

    1 in right sinus + 2 in left sinus

    5

    15

    5

    10

    2 in right sinus + 1 in left sinus

    7

    21

    14

    7

    2 in right sinus + 2 in left sinus

    0

    0

    0

    0

    3 in right sinus + 2 in left sinus

    1

    5

    3

    2

    3 in right sinus + 1 in left sinus

    1

    4

    3

    1

    Total

    129

    197

    103

    94

     

    Keywords