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CASE REPORT
Year : 2019  |  Volume : 12  |  Issue : 3  |  Page : 262-266  

A case report of sinonasal undifferentiated carcinoma in a 60-year-old female in clinical practice


1 Department of Radiology, Trauma Surgical Centre, Ondo City, Ondo State, Nigeria
2 Department of Radiology, Obafemi Awolowo University Teaching Hospital, Ile-Ife, Osun State, Nigeria
3 Department of Hematology, University of Medical Sciences, Ondo City, Ondo State, Nigeria
4 Department of Pharmacology and Therapeutics, University of Medical Sciences, Ondo City, Ondo State, Nigeria

Date of Submission28-Jun-2018
Date of Acceptance12-Nov-2018
Date of Web Publication15-May-2019

Correspondence Address:
Salewa Evelyn Osho
Department of Radiology, Trauma Surgical Centre and Faculty of Clinical Sciences, University of Medical Sciences, Ondo City, Ondo State
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/mjdrdypu.mjdrdypu_104_18

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  Abstract 


This was a rare case of sinonasal undifferentiated carcinoma (SNUC) in a 60-year-old female who presented with complaints of progressive right cheek swelling, associated headache, nasal obstruction, and mucopurulent nasal discharge. Conventional plane radiograph of the cranial paranasal air sinuses revealed opacification of the right maxillary sinus with erosion of the floor of the orbit. The cranial computed tomographic (CT) scan images revealed opacification of the right maxillary sinus, both ethmoidal sinuses and the entire right and medial half of the left frontal sinus. There was erosion of the floor of the anterior cranial fossa on the right side close to the midline. Furthermore, there was extensive and expansile mixed density mass lesion filling the entire right maxillary sinus, both ethmoidal sinuses, right sphenoidal sinus, frontal sinus, and both nasal cavities with medial displacement and infiltration of the right medial rectus muscle. A radiological assessment of extensive, locally advanced right antronasal mass was made on cranial CT scan. At surgery, a cheesy and friable tumor involving the roof, medial wall, and part of the floor of the maxillary sinus extending into the ethmoidal sinuses was seen with associated mucocele of the frontal sinus. Biopsy, histology, and immunohistochemistry of the mass showed SNUC.

Keywords: Paranasal sinuses, radiological features, sinonasal undifferentiated carcinoma


How to cite this article:
Osho SE, Ibitoye BO, Osho PO, Fasipe OJ, Ibiyemi-Fasipe OB. A case report of sinonasal undifferentiated carcinoma in a 60-year-old female in clinical practice. Med J DY Patil Vidyapeeth 2019;12:262-6

How to cite this URL:
Osho SE, Ibitoye BO, Osho PO, Fasipe OJ, Ibiyemi-Fasipe OB. A case report of sinonasal undifferentiated carcinoma in a 60-year-old female in clinical practice. Med J DY Patil Vidyapeeth [serial online] 2019 [cited 2019 May 25];12:262-6. Available from: http://www.mjdrdypv.org/text.asp?2019/12/3/262/258193




  Introduction Top


Malignant sinonasal tumors comprise <1% of all neoplasms and 3% of those of upper aerodigestive tracts. They comprise a wide variety of epithelial, lymphoid, and mesenchymal tumors.[1]

Sinonasal tract malignancies most commonly affect the maxillary sinus (about 60%), followed by the nasal cavity (about 22%), ethmoidal sinus (15%), and frontal and sphenoidal sinuses (<3%). These tumors are diverse, with majority being squamous cell carcinoma and its variants (55%), followed by nonepithelial neoplasm (20%), glandular tumors (15%), undifferentiated carcinoma (7%), and miscellaneous tumors (3%).[2],[3],[4] The treatment of choice are surgical resection with chemotherapy ± radiotherapy.

This was a case presentation of rare sinonasal undifferentiated carcinoma (SNUC) in clinical practice.


  Case Report Top


ACE is a 60-year-old female farmer and petty trader, who presented in April 2018 at the Trauma Surgical Centre, Ondo City, Ondo State, Nigeria, on account of a 6-month history of progressive swelling over her right cheek.

The patient was well until 6 months before presentation when she noticed a swelling on her right cheek. The swelling initially increased slowly in size, but later grew rapidly over the last 5 months. There was associated headache, nasal obstruction, and purulent nasal discharge. There was no history of epistaxis, difficulty with swallowing, or cough.

Examination of the head and neck revealed a mass in the right cheek, measuring 5 cm × 6 cm. The mass was firm, tender with smooth surface, and not attached to the underlying skin. No clinically significant peripheral lymphadenopathy was palpable around the head, neck, and other body regions.

Examination of the other system was not contributory.

A clinical diagnosis of the right maxillary antral tumor was made.

Full blood count, electrolyte, and urea and clotting profile were essentially normal. The packed cell volume was 41%.

Conventional plane radiograph of the cranial paranasal air sinuses revealed opacification of the right maxillary sinus with erosion of the floor of the orbit [Figure 1].
Figure 1: In this skull radiograph, the occipitomental view of the skull showed some haziness over the right maxillary sinus along with destruction of the inferior orbital wall (black arrow)

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The cranial computed tomographic (CT) scan images on the coronal slices [Figure 2]a and [Figure 2]b, axial slice [Figure 3], sagittal slice [Figure 4], and bone window slices [Figure 5] and [Figure 6] revealed opacification of the right maxillary sinus, both ethmoidal sinuses, as well as the entire right and medial half of the left frontal sinus. There was erosion of the floor of the anterior cranial fossa on the right side close to the midline. Furthermore, there was extensive and expansile mixed density mass lesion filling the entire right maxillary sinus, both ethmoidal sinuses, right sphenoidal sinus, frontal sinus, and both nasal cavities. Punctate areas of hyperdensity were present within the superior half of the mass. The mass was also seen to extend superiorly into the anterior cranial fossa (but limited by the dura) and laterally into the medial aspect of the right orbit displacing the globe. There was medial displacement and infiltration of the right medial rectus muscle with destruction of the walls of the right maxillary antrum, roof of the orbit, and floor of the anterior cranial fossa. A radiological assessment of extensive, locally advanced right antronasal mass was made on cranial CT scan.
Figure 2: (a) Computed tomographic precontrast coronal slice showing right maxillary anthral mass which was seen to erode on the inferior orbital wall (black arrow). (b) Computed tomographic postcontrast coronal slice showing right maxillary anthral mass which was seen to erode on the inferior orbital wall (black arrow)

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Figure 3: Computed tomographic postcontrast axial slice showing extent of soft tissues and bony involvement

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Figure 4: Computed tomographic postcontrast sagittal slice showing extent of soft tissues and bony involvement

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Figure 5: Computed tomographic axial bone window slice showing extent of bony involvement and destruction by the tumor

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Figure 6: Computed tomographic sagittal bone window slice showing extent of bony involvement and destruction by the tumor

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At surgery, a cheesy and friable tumor involving the roof, medial wall, and part of the floor of the maxillary sinus extending into the ethmoidal sinuses was seen with associated mucocele of the frontal sinus. Biopsy, histology, and immunohistochemistry of the mass showed SNUC. The patient is currently undergoing postoperative radiotherapy and chemotherapy sessions.


  Discussion Top


SNUC is a rare and highly aggressive malignancy of the paranasal sinuses. SNUC which was first described by Frierson et al.[5] in 1986 must be distinguished from other small-to-medium-sized sinonasal neoplasms because of its aggressive behavior and often fulminant clinical course.[6] The tumor is characterized by rapid growth as seen in the case presented, propensity for locoregional recurrence, distal metastasis to the lung and bone, and poor prognosis.

The etiology of SNUC is unknown, although those who have survived radiation treatment for retinoblastoma[7] as well as those who smoke appear to be at risk. But this patient case has neither history of previous irradiation exposure nor smoking habits. Epstein–Barr virus has been suggested to be an etiological factor,[8] especially in Asian patients,[9] but recent studies in the USA[10] and Europe,[11] and in an Epstein–Barr endemic area[12] failed to demonstrate the presence of SNUC. It is, therefore, widely accepted that the involvement of Epstein–Barr virus in the pathogenesis is unlikely and that the presence of Epstein–Barr virus excluded a diagnosis of SNUC.

The median age of the presentation is the sixth decade, with men more commonly affected than women. The male-to-female ratio is 2.3:1.[13],[14] The case presented is a female aged 60 years.

Majority of patients present with locally advanced disease. The most common symptoms are nasal obstruction, proptosis, cranial nerve palsy, periorbital swelling, diplopia, epistaxis, and periorbital pain. The duration of symptoms varies from a few weeks to months. The index case presented with a 6-month history of swelling over the right cheek, which was associated with nasal congestion, nasal obstruction, mucopurulent nasal discharge, and headache.[15]

Grossly, the tumors are large (>4 cm) with bony invasion and poorly defined margins.[16]

SNUC is believed to originate from Schneiderian epithelium or from nasal ectoderm of the paranasal sinuses.[5] It is a member of the neuroendocrine group of sinonasal malignancies that also includes esthesioneuroblastoma, neuroendocrine carcinoma, and small cell carcinoma. The malignancies are divided into two groups: esthesioneuroblastoma and nonesthesioneuroblastoma based on the achievement of locoregional and distant control of esthesioneuroblastoma by local treatment alone, unlike SNUC which requires aggressive multimodal therapy. SNUC is chemically composed of small-to-medium-sized undifferentiated cells, characterized by high mitotic rates, significant cellular pleomorphism, polychromasia, high nuclear-to-cytoplasmic ratios, necrosis, and lymphovascular invasion.

Immunohistochemical analysis showed that SNUC reacts with keratin (especially CK7, CK8, and CK19). In addition, SNUC may stain positive to neuron-specific enolase (NSE), and P53 chromogranin and synaptophysin are rarely positive. It is nonreactive to S-100 protein or CD-45.

Radiologically, on CT scan, SNUC usually appears as an expansile lesion of soft-tissue attenuation with significant bony erosion and invasion of adjacent structures including the anterior cranial fossa, adjacent paranasal sinuses, and the orbits. Obstruction of the adjacent sinuses is also noted. The tumors are usually noncalcified and they demonstrate contrast enhancement. CT scan of the case presented showed expansile, mixed density mass filling the right maxillary sinus and ethmoidal, sphenoidal, and frontal sinuses with extension into the anterior cranial fossa. There was associated invasion of the orbital muscles and bony destruction. Unusual finding of calcification was seen within the mass.

On magnetic resonance imaging (MRI), the lesions are usually isointense to skeletal muscle on T1-weighted MR images and iso- to hyperintense to muscle on proton density-weighted and T2-weighted MR images. Heterogeneous enhancement to gadolinunm is the rule. MRI is also able to distinguish between tumor and postobstructive secretions. MRI was not done for this patient due to financial constrain.

The differential diagnosis of undifferentiated tumor from the sinonasal region is wide and includes poorly differentiated squamous cell carcinoma, nasopharyngeal undifferentiated carcinoma (NPUC), neuroendocrine carcinoma, olfactory neuroblastoma, melanoma, and lymphoma. Distinguishing these processes is essentially impossible with imaging criteria alone, and ultimately, a diagnosis based on histopathological and immunochemical findings is essential. At times, immunochemical and ultrastructural analysis is needed to distinguish these entities, especially when a preliminary histological determination of poorly differentiated or undifferentiated carcinoma is reached. The important feature for the diagnosis of SNUC lies in its histological and immunochemical distinction from more indolent neoplasm such as esthesioneuroblastoma, lymphoepithelioma, or neuroendocrine carcinoma.

NPUC is composed of cells with indistinct borders and vesicular nuclei with inconspicuous nucleus and often has a marked lymphoplasmacytic infiltrate (lymphoepithelioma). Cytological atypia is less prominent in NPUC and necrosis is not usually seen. Nasopharyngeal carcinomas are positive for Epstein–Barr virus.

Separation of SNUC from olfactory neuroblastoma may be difficult. Olfactory neuroblastoma typically presents as a polyploid mass protruding from the roof of nasal cavity and consists of lobules of small cells with eosinophilic fibrillary intercellular material in a highly vascular stroma. A dumbbell mass extending across the cribriform plate is one of the characteristic radiological findings. MRI with or without contrast will delineate the extent of the disease with T1-weighted images, showing marked enhancement with gadolinum. Olfactory neuroblastomas are positive for neuroendocrine markers (NSE, synaptophysin, and chromogranin), with S-100 protein found at the periphery of the tumor lobules.

Poorly differentiated squamous cell carcinomas show small areas of obvious differentiation or keratinization and have a different cytokeratin pattern. Radiologically, they show little destruction and spread.

Melanoma and lymphoma are early differentiated on the basis of immunohistochemistry.

Multimodal approach to treatment of patients with SNUC is necessary. These include surgical resection, chemotherapy, and radiotherapy. The case presented had surgical resection and is currently undergoing postoperative radiotherapy and chemotherapy sessions.

The prognosis of SNUC is poor. The medial survival ranges from 12.3 months to 40.5 months. The optimal treatment is yet to be determined. The overall survival is about 20% at 5 years. There is frequent recurrence with metastasis to lymph nodes and distant sites.


  Conclusion Top


This was a rare case of SNUC in a 60-year-old female who presented with complaints of progressive right cheek swelling, associated headache, nasal obstruction, and mucopurulent nasal discharge. Surgery had been done with the patient currently undergoing postoperative radiotherapy and chemotherapy sessions.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that the name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Pilch BZ, Bouquot J, Thompson LD. Squamous cell carcinoma. In: Bernes EL, Everson JW, Reichart P, Sidransky D, editors. Pathology and Genetics of Head and Neck Tumours (Kleihuer P, Sobin LH, series editors. World Health Organisation Classification of Tumours). Lyon, France: IARC Press; 2005. p. 15-7.  Back to cited text no. 1
    
2.
McNicoll W, Hopkin N, Dalley VM, Shaw HJ. Cancer of the paranasal sinuses and nasal cavities. Part II. Results of treatment. J Laryngol Otol 1984;98:707-18.  Back to cited text no. 2
    
3.
Hopkin N, McNicoll W, Dalley VM, Shaw HJ. Cancer of the paranasal sinuses and nasal cavities. Part I. Clinical features. J Laryngol Otol 1984;98:585-95.  Back to cited text no. 3
    
4.
Jackson RT, Fitz-Hugh GS, Constable WC. Malignant neoplasms of the nasal cavities and paranasal sinuses: (a retrospective study). Laryngoscope 1977;87:726-36.  Back to cited text no. 4
    
5.
Frierson HF Jr., Mills SE, Fechner RE, Taxy JB, Levine PA. Sinonasal undifferentiated carcinoma. An aggressive neoplasm derived from schneiderian epithelium and distinct from olfactory neuroblastoma. Am J Surg Pathol 1986;10:771-9.  Back to cited text no. 5
    
6.
Levine PA, Frierson HF Jr., Stewart FM, Mills SE, Fechner RE, Cantrell RW, et al. Sinonasal undifferentiated carcinoma: A distinctive and highly aggressive neoplasm. Laryngoscope 1987;97:905-8.  Back to cited text no. 6
    
7.
Greger V, Schirmacher P, Bohl J, Bornemann A, Hürter T, Passarge E, et al. Possible involvement of the retinoblastoma gene in undifferentiated sinonasal carcinoma. Cancer 1990;66:1954-9.  Back to cited text no. 7
    
8.
Gallo O, Di Lollo S, Graziani P, Gallina E, Baroni G. Detection of epstein-barr virus genome in sinonasal undifferentiated carcinoma by use of in situ hybridization. Otolaryngol Head Neck Surg 1995;112:659-64.  Back to cited text no. 8
    
9.
Lopategui JR, Gaffey MJ, Frierson HF Jr., Chan JK, Mills SE, Chang KL, et al. Detection of epstein-barr viral RNA in sinonasal undifferentiated carcinoma from Western and Asian patients. Am J Surg Pathol 1994;18:391-8.  Back to cited text no. 9
    
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Cerilli LA, Holst VA, Brandwein MS, Stoler MH, Mills SE. Sinonasal undifferentiated carcinoma: Immunohistochemical profile and lack of EBV association. Am J Surg Pathol 2001;25:156-63.  Back to cited text no. 10
    
11.
Franchi A, Moroni M, Massi D, Paglierani M, Santucci M. Sinonasal undifferentiated carcinoma, nasopharyngeal-type undifferentiated carcinoma, and keratinizing and nonkeratinizing squamous cell carcinoma express different cytokeratin patterns. Am J Surg Pathol 2002;26:1597-604.  Back to cited text no. 11
    
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Jeng YM, Sung MT, Fang CL, Huang HY, Mao TL, Cheng W, et al. Sinonasal undifferentiated carcinoma and nasopharyngeal-type undifferentiated carcinoma: Two clinically, biologically, and histopathologically distinct entities. Am J Surg Pathol 2002;26:371-6.  Back to cited text no. 12
    
13.
Frierson HF Jr. Sinonasal undifferentiated carcinoma. In: Barnes L, Everson JW, Reichart P, Sidransky D, editors. World Health Organisation Classification of Tumors Pathology and Genetics of Head and Neck Tumors. Lyon, France: IARC Press; 2005.  Back to cited text no. 13
    
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Smith SR, Som P, Fahmy A, Lawson W, Sacks S, Brandwein M, et al. A clinicopathological study of sinonasal neuroendocrine carcinoma and sinonasal undifferentiated carcinoma. Laryngoscope 2000;110:1617-22.  Back to cited text no. 14
    
15.
Gallo O, Graziani P, Fini-Storchi O. Undifferentiated carcinoma of the nasal and paranasal sinuses. Am immunohistochemical and clinical study. ENT J 1994;72:588-93.  Back to cited text no. 15
    
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Phillips CD, Futterer SF, Lipper MH, Levine PA. Sinonasal undifferentiated carcinoma: CT and MR imaging of an uncommon neoplasm of the nasal cavity. Radiology 1997;202:477-80.  Back to cited text no. 16
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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