Small-cell neuroendocrine carcinoma originating

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Issue Date: November 2008, Posted On: 11/1/2008

Small-cell neuroendocrine carcinoma originating from the lateral nasopharyngeal wall Abstract

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A 40-year-old man presented with right-sided nasal blockage and epistaxis of 18 months' duration. Examination revealed a pinkish mass in the right nasal cavity and nasopharynx. Initial biopsy was suggestive of angiofibroma. Complete excision through a lateral rhinotomy approach was performed. The postoperative histopathologic report was suggestive of sinonasal neuroendocrine carcinoma, which was confirmed by immunohistochemistry. To the best of our knowledge, this is the first reported case of sinonasal neuroendocrine carcinoma arising from the lateral wall of the nasopharynx in the English literature. We describe its clinical presentation and management and provide a brief review of the literature.

by Shetty Deviprasad, MS (ENT), DLO, Aroor Rajeshwari, MS (ENT), Mohammed Tahir, MS (ENT), T.V. Adarsha, DLO, DNB (ENT), and Somayaji Gangadhara, MS (ENT) Poll Question:

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Introduction Primary neuroendocrine carcinomas in the head and neck region are rare. Fewer than 50 cases of sinonasal neuroendocrine carcinoma (SNEC) have been reported in the world literature. Some have been reported to extend into the nasopharynx,1-4 but we found only 1 case of SNEC primarily originating from the nasopharynx.5 However, the exact subsite of origin in the nasopharynx was not mentioned in that case. To the best of our knowledge, therefore, ours is the first reported case of SNEC originating from the lateral wall of the nasopharynx. The first neuroendocrine tumor was reported by Oberndorfer in 1907.6 The first neuroendocrine carcinoma of the larynx was reported in 1969 by Goldman et al.7 This tumor is the most common nonsquamous malignancy in the larynx.8 The first case of small-cell carcinoma of the paranasal sinuses was reported in the literature in 1965 by Raychowdhuri.9 Since then, very few cases of SNEC occurring in the head and neck region have been reported. Therefore, because of their rarity, morphologic nature, spectrum, classification, and staging, their prognosis and management are confusing and controversial.

Case report A 40-year-old man was referred to the ENT outpatient clinic of our hospital with a history of right-sided nasal obstruction and epistaxis of 18 months' duration. On examination, a pinkish mass was seen filling the right nasal cavity; the mass could be probed on all sides. It was not sensitive to touch, and there was minimal bleeding when it was probed. The postnasal examination showed a pinkish globular mass in the nasopharynx.

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Findings of a biopsy of the mass, done at an outside laboratory, were suggestive of nasopharyngeal angiofibroma. The patient underwent routine blood and urine analyses, which were normal. Computed tomography showed that the right nasal cavity and right nasopharynx were filled with a contrastenhancing mass, without any bone erosion and with retained secretions in the sinuses (figure 1). The patient was scheduled for lateral rhinotomy and medial maxillectomy. Axial CT shows the mass in the right nasal cavity and the right side of the nasopharynx

The mass was removed en bloc (figure 2) and was found to be arising from an area near the sphenopalatine foramen on the lateral wall of the nasopharynx, above and behind the posterior end of the middle turbinate. The sphenopalatine artery was ligated to control the bleeding.

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A: Intraoperative photograph shows the mass in the right nasal cavity. B: Photograph shows the resected mass. The small arrow indicates the nasal portion of the mass, and the large arrow indicates the sphenopalatine portion

The patient's postoperative period was uneventful. The histopathologic examination suggested SNEC. Immunohistochemistry was positive for chromogranin (figure 3) and negative for neuron-specific enolase. The patient was asymptomatic for 6 months, after which he returned with a complaint of spontaneous epistaxis. On examination, he was found to have local recurrence and was advised to undergo chemotherapy. He refused treatment and died 11 months after the initial surgery. Immunohistochemistry reveals chromogranin positivity (original magnification x40)

Discussion According to Mills, neuroendocrine tumors can be divided into two groups: group I, which show primarily epithelial differentiation, and group II, which are of a nonepithelial type.8 Mills contends that the group I tumors, which were once known as carcinoid, atypical carcinoid, and small-cell carcinoma, should be called well-differentiated, moderately differentiated, and poorly differentiated neuroendocrine carcinomas and states that poorly differentiated neuroendocrine carcinomas should be subdivided into small-cell and large-cell variants.8 Sinonasal undifferentiated carcinoma is an example of the large-cell subtype of poorly differentiated neuroendocrine carcinoma.8 Group 2 neuroendocrine tumors include olfactory neuroblastoma, malignant melanoma, and Ewing sarcoma.8 The cell of origin of neuroendocrine carcinomas is not clearly known. Some believe they arise from the neuroendocrine cells of the dispersed neuroendocrine system, and others state that the origin is pluripotent stem cells that are capable of dual epithelial and endocrine differentiation.4,10 Most neuroendocrine malignancies in the head and neck region arise from the larynx. The second most common site is the salivary gland.8 Few cases have been reported in the sinonasal tract, petrous apex, and tongue.6,11,12 Histologically, SNEC manifests cellular pleomorphism, nuclear atypia, frequent mitosis, and necrosis. Optical microscopy using hematoxylin and eosin staining reveals sheets, cords, or ribbons of small cells with little cytoplasm. The nuclei are large and pleomorphic, varying in appearance from densely hyperchromatic to a punctuate chromatic distribution.4,12 Scattered areas of necrosis with typical crust/artifact of neoplastic cells are observed. Because of the poorly differentiated nature of the tumor, immunohistochemical stains are of limited value,8 and therefore multiple markers are tested. These include carcinoma markers containing cytokeratin, and neuroendocrine markers containing chromogranin, synaptophysin, and neuron-specific enolase.12 Antibodies against protein convertase have also been used as endocrine cell markers. The clinical presentations of SNEC are nonspecific. They include rhinologic syndrome (nasal obstruction, rhinorrhea, and epistaxis); ophthalmic signs (exopthalmos, reduced visual acuity, and restricted eye movements); locoregional invasion; and metastatic neck nodes. Paraneoplastic syndrome is also observed in SNEC. Abnormal secretion of antidiuretic hormone is the most frequent endocrine manifestation. Other hormones secreted include adrenocorticotropin hormone, calcitonin, parathormone, glucagon and somatotropin-releasing hormone.12 Our patient did not have paraneoplastic syndrome. For neuroendocrine tumors of the sinonasal tract, Kadish classifications are often used for staging. In group A, the tumor is limited to the nasal cavity. In group B, the tumor is confined to the nasal cavity and paranasal sinuses. In group C, the tumor extends beyond the nasal cavity and paranasal sinuses.13 At present, no standard staging system exists for SNEC. Furthermore, because of the small number of patients treated at each institution, there is no accepted standard treatment for this disease. Three therapeutic modalities currently used for SNEC are surgery, radiotherapy, and chemotherapy. In the late 1990s, the combination of chemotherapy and radiotherapy, with or without surgery, produced encouraging results,12 but according to Austin et al, surgery is the primary treatment modality, and chemotherapy and radiotherapy may not be required.13



In conclusion, sinonasal SNECs are aggressive tumors with a high rate of recurrence and a very poor prognosis. Locoregional invasion may lead to death. The 5-year survival rate is only 13% in nonpulmonary SNEC, and the survival duration is 13 months in head and neck SNEC.12 No standard treatment modality has been established for these tumors. From the Department of ENT and Head and Neck Surgery, Yenepoya Medical College, Deralakatte, Mangalore, Karnataka, India. Dr. Rajeshwari Aroor, SHARADA VASUDEVA SADANA, No. 3-3/88, 3rd main road, Prashanth nagar layout, Mangalore, Karnataka, India, 575006. Phone: 91-8242204-668/669/671/672, ext. 608; email: [email protected]

References 1. Galera-Ruiz H, Villar-Rodriguez JL, Sanchez-Calzado JA, et al. Sinonasal neuroendocrine carcinoma presenting as a nasopharyngeal mass. Otolaryngol Head Neck Surg 2001; 124 (4) 56. 2. Perez-Ordonez B, Caruana SM, Huvos AG, Shah JP. Small cell neuroendocrine carcinoma of the nasal cavity and paranasal sinuses. Hum Pathol 1998; 29 (8) 826-32. 3. Kameya T, Shimosato Y, Adachi I, et al. Neuroendocrine carcinoma of the paranasal sinus: A morphological and endocrinological study. Cancer 1980; 45 (2): 330-9. 4. Kanamalla US, Kesava PP, McGuff HS. Imaging of nonlaryngeal neuroendocrine carcinoma. AJNR Am J Neuroradiol 2000; 21 (4); 775-8. 5. Lin IH, Hwang CF, Huang HY, Chien CY. Small cell carcinoma of the nasopharynx. Acta Otolaryngol 2007; 127 (2): 206-8. 6. Kovac L, Gjurić M, Branica S, et al. Small cell neuroendocrine carcinoma in the petrous apex. J Laryngol Otol 2006; 120 (1): 74-6. 7. Goldman NC, Hood CI, Singleton GT. Carcinoid of the larynx. Arch Otolaryngol 1969; 90 (1): 647. 8. Mills SE. Neuroectodermal neoplasms of the head and neck with emphasis on neuroendocrine carcinomas. Mod Pathol 2002; 15 (3); 264-78. 9. Raychowdhuri RN. Oat-cell carcinoma and paranasal sinuses. J Laryngol Otol 1965; 79:253-5. 10. Wenig BM, Gnepp DR. The spectrum of neuroendocrine carcinomas of the larynx. Semin Diagn Pathol 1989; 6 (4): 329-50. 11. Soussi AC, Benghiat A, Holgate CS, Majumdar B. Neuro-endocrine tumours of the head and neck. J Laryngol Otol 1990; 104 (6); 504-7. 12. Babin E, Rouleau V, Vedrine PO, et al. Small cell neuroendocrine carcinoma of the nasal cavity and paranasal sinuses. J Laryngol Otol 2006; 120 (4): 289-97. 13. Austin JR, Cebrun H, Kershisnik MM, et al. Olfactory neuroblastoma and neuroendocrine carcinoma of the anterior skull base. Treatment results at the M.D. Anderson Cancer Center. Skull Base Surg 1996; 6 (1): 1-8. Ear Nose Throat J. 2008 November;87(11):E1

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