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Table of Contents
CASE REPORT
Year : 2019  |  Volume : 2  |  Issue : 2  |  Page : 74-78

Small-cell carcinoma of nasal cavity and approach to its management: A case report


Department of Radiation Oncology, Gujarat Cancer Research Institute and BJMC, Ahmedabad, Gujarat, India

Date of Web Publication30-Dec-2019

Correspondence Address:
Dr. Amit Kichloo
A 2 Amardeep Apartments, Civil Hospital Road, Ahmedabad, Gujarat.
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jco.jco_19_19

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  Abstract 

Most common site for small-cell neuroendocrine carcinoma (SNEC) is lung. Incidence of extrapulmonary small-cell carcinoma (SCC) is infrequent and very few cases of primary SNEC of head and neck have been reported in the literature. Nasal cavity as a primary site for SCC is extremely rare and thus we report a case of primary SCC of nasal cavity and paranasal sinuses who presented to our institute and approach to its management.

Keywords: Chemoradiation, nasal cavity, small-cell carcinoma


How to cite this article:
Kichloo A, Parikh A, Mankada S, Suryanarayana U. Small-cell carcinoma of nasal cavity and approach to its management: A case report. J Curr Oncol 2019;2:74-8

How to cite this URL:
Kichloo A, Parikh A, Mankada S, Suryanarayana U. Small-cell carcinoma of nasal cavity and approach to its management: A case report. J Curr Oncol [serial online] 2019 [cited 2020 Jan 18];2:74-8. Available from: http://www.journalofcurrentoncology.org/text.asp?2019/2/2/74/274301




  Introduction Top


Primary small-cell neuroendocrine carcinoma (SNEC) of nasal cavity and paranasal sinuses is a rare entity.[1] The prognosis for malignant sinonasal SNEC is poor because of the high incidence of locoregional failure and distant metastasis to lungs, brain, bones, and liver. A multidisciplinary treatment approach of SNEC of nasal cavity is early, aggressive complete surgical excision (if feasible), followed by multiagent chemotherapy and radiotherapy, but there is no definitive treatment and therapeutic recommendations. In this case report, we discuss a patient with SNEC of nasal cavity and paranasal sinuses who presented to our institute. The patient was subsequently treated with four cycles of carboplatin and etoposide (CE) followed by radiation therapy with intensity modulated radiotherapy (IMRT) and was subsequently given two more cycles of chemotherapy (CE). The patient is on regular follow-up and is disease free.


  Case Report Top


In September 2018, a 54-year-old man presented to our institute with swelling over medial aspect of right nasal cavity and nasal obstruction since 1.5 months [Figure 1]. Patient had one episode of epistaxis in the last few days. Personal habit included tobacco chewing since last 20 years.
Figure 1: On presentation, swelling over medial aspect of right nasal cavity

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Physical examination was normal. Nasal examination revealed right-sided nasal mass covering almost whole nasal cavity which bled on touch. Oral cavity examination was normal. Clinical examination revealed multiple firm and mobile palpable nodes over bilateral neck region; largest being 2 cm × 2 cm at right level lb. Chest X-ray (PA view) was normal. Contrast-enhanced computed tomography (CECT) of paranasal sinuses and neck (P + N) revealed multiple round to oval enhancing lymph nodes at bilateral neck region largest of size 2 cm × 1.7 cm at right level IB, 1.3 cm × 8 cm at right level III, 1.4 cm × 9 cm and 1 cm × 0.7 cm at right level V, 1 cm ×9 cm at left level IA, and 1.3 cm × 8 cm at left level II, 1.4 cm ×1 cm at left level III. Large elongated lymph node was seen at retropharyngeal region on left side about 2 cm × 1.2 cm. Polypoidal mucosal wall thickening was seen involving both maxillary sinuses (right > left), which extended from right anterior ethmoidal air cells into nasal cavity. There was no erosion of adjacent sinus wall [Figure 2] and [Figure 3].
Figure 2: Contrast-enhanced computed tomography (CECT) of paranasal sinuses and neck: sagittal view showing primary lesion

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Figure 3: Contrast-enhanced computed tomography (CECT) of paranasal sinuses and neck: axial view showing metastatic nodal involvement

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Fine-needle aspiration from right cervical node showed malignant lesion with possibility of either lymphoma or other round cell tumor. Excision biopsy from right nasal cavity revealed small round atypical cells with little cytoplasm and a high nuclear-cytoplasm ratio, with pleomorphic hyperchromatic nuclei suggesting poorly differentiated carcinoma. Immunohistochemistry revealed cytokeratin AE1, neuron-specific synaptophysin, chromogranin to be positive but leukocyte common antigen (LCA) was negative [Figure 4]. Thus, a final diagnosis of small-cell carcinoma was made. CT thorax did not reveal any abnormal enhancing lesion. Magnetic resonance imaging (MRI) of brain was normal.
Figure 4: Histopathological image of biopsy of primary lesion showing small round atypical cells with little cytoplasm and a high nuclear-cytoplasm ratio, with pleomorphic hyperchromatic nuclei

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After multidisciplinary discussion, patient was treated with induction chemotherapy with four cycles of carboplatin (area under the curve [AUC] 5 on day 1) and etoposide (100mg/m2/day on days 1, 2, and 3) thrice weekly from October 9, 2018 to December 12, 2018, followed by external beam radiation delivered by single daily fraction of 2 Gy for 5 days a week up to a total dose of 6000 cGy in 30 fractions to the primary region and upper cervical lymph node (levels I–III) and 5400 cGy in 30 fractions to lower cervical nodes (level IV) by IMRT planning with 6-MV photons from February 14, 2019 to April 4, 2019 [Figure 5]. Patient further received two cycles of CE as per schedule and complete six cycles. Patient tolerated chemotherapy and radiotherapy well with development of only grade 2 oral mucositis and grade 1 skin reaction over nose and cheek, which was managed with symptomatic treatment. CT PNS + neck of June 28, 2019 showed no abnormally enhancing lesion in nasal cavity [Figure 6] without any lymphadenopathy. He is disease-free since last 6 months.
Figure 5: IMRT planning

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Figure 6: Follow-up contrast-enhanced computed tomography (CECT) of paranasal sinuses and neck: no abnormally enhancing lesion in nasal cavity without any lymphadenopathy

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  Discussion Top


SNEC was first described in the nineteenth century in context to lung cancer and it accounts for 15%–20% of all lung carcinoma. Extrapulmonary SNEC represents 4% of all SNEC. Primary small-cell carcinoma of head and neck was first reported in 1965.[2] Most commonly affected sites in head and neck are larynx, oral cavity, and pharynx with nasal cavity and paranasal sinuses being extremely rare.[3] Small-cell carcinomas show early metastasis but this is not the case with nasal cavity SNEC as the local recurrence rates are much higher associated with this site.[4] Symptoms include nasal blockage, recurrent epistaxis, and discharge, a very similar presentation to that of benign diseases. Thus, these patients usually present late.[5] It is very important to clearly establish the diagnosis of extrapulmonary SNEC; thus, chest radiograph and CT thorax along with MRI brain should be performed to rule out any brain metastasis and any lung primary. In our patient, CECT of chest was normal, so the lesion involving the nose and paranasal sinuses must be considered a primary neoplasm. To differentiate SNEC from malignant lymphoma, the presence of CD56+/CD45 is must. Earlier surgery followed by adjuvant radiation was preferred but it was associated with unfavorable outcomes. Recently combination chemotherapy (etoposide and cisplatin) is considered more effective than use of a single agent because of additive and synergistic effects and radiotherapy has proved better results.[6] The overall prognosis of SNEC is poor. However, the prognosis seems more favorable in the case of localized nasal and paranasal SNEC. Soussi et al.[7] reported that 100% of patients were alive at 5 years, 88% at 7 years, and 77% at 10 years.


  Conclusion Top


Small-cell carcinoma of nasal cavity is extremely rare. They are characterized by aggressive nature and high rate of recurrence and have tendency to metastasize to other sites via lymphatics and blood stream. An aggressive therapeutic approach with combination of chemotherapy and radiotherapy may define a better treatment outcome for this rare entity. Regular, close follow-up is needed to detect any local or distant metastasis. Because of rarity disease, treatment of SNEC of sinonasal tract has not been defined clearly.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Thorup C, Sebbesen L, Danø H, Leetmaa M, Andersen M, Buchwald C, et al. Carcinoma of the nasal cavity and paranasal sinuses in Denmark 1995-2004. Acta Oncol 2010;49:389-94.  Back to cited text no. 1
    
2.
Ibrahim NB, Briggs JC, Corbishley CM. Extrapulmonary oat cell carcinoma. Cancer 1984;54:1645-61.  Back to cited text no. 2
    
3.
Kim SG, Jang HS. Small cell carcinoma of the oral cavity: Report of a case. J Oral Maxillofac Surg 2001;59:680-4.  Back to cited text no. 3
    
4.
Silva EG, Butler JJ, Mackay B, Goepfert H. Neuroblastomas and neuroendocrine carcinomas of the nasal cavity: A proposed new classification. Cancer 1982;50:2388-405.  Back to cited text no. 4
    
5.
Mineta H, Miura K, Takebayashi S, Araki K, Ueda Y, Harada H, et al. Immunohistochemical analysis of tiny cell cancer of the top and neck: A report of 4 patients and review of sixteen patients within the literature with position internal secretion production. Annals of Otology, Rhinology, Laryngology 2001;10.177: 76-82.  Back to cited text no. 5
    
6.
Babin E, Rouleau V, Vedrine PO, Toussaint B, de Raucourt D, Malard O, et al. Small cell neuroendocrine carcinoma of the nasal cavity and paranasal sinuses. J Laryngol Otol 2006;120:289-97.  Back to cited text no. 6
    
7.
Soussi AC, Benghiat A, Holgate CS, Majumdar B. Neuro-endocrine tumours of the head and neck. J Laryngol Otol 1990;104:504-7.  Back to cited text no. 7
    


    Figures

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



 

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