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Journal of Clinical Oncology, Vol 23, No 9 (March 20), 2005: pp. 2094-2096 © 2005 American Society of Clinical Oncology. DOI: 10.1200/JCO.2005.02.130
Unusual Sites of MalignanciesCASE 1. Small-Cell Carcinoma of the HypopharynxAlbert Einstein Cancer Center, Montefiore Medical Center, Bronx, NY A 65-year-old man with a history of excessive alcohol consumption and remote history of tobacco use presented with a 4-month history of severe dysphagia to solid food, odynophagia, dry cough, hoarseness, dyspnea, nighttime snoring, and weight loss of 9 lbs (4.1 kg) secondary to altered food intake. Direct laryngoscopy revealed an exophytic mass of the pyriform sinus (hypopharynx) overhanging the airway, and an immobile left vocal cord (Fig 1A). A contrast-enhanced computed tomography scan of the neck revealed a large left pyriform sinus mass (Fig 1B, solid arrow) and a 1.5-cm level III ipsilateral cervical lymph node (Fig 1B, dotted arrow); no pulmonary or other distant metastatic disease was noted. He underwent laser debulking at the time of biopsy to avoid further airway compromise. Pathologic evaluation revealed poorly differentiated small-cell carcinoma (Fig 2A). Imunohistochemical staining demonstrated the expression of the neuroendocrine markers neuron-specific enolase, CD56, chromogranin, and synaptophysin (Fig 2B), consistent with the diagnosis. The patient was treated with concurrent platinum-based chemotherapy and neck irradiation to 69 Gy of a planned hyperfractionated course of 74.4 Gy to the primary tumor. At 24 Gy, the patient had a clinical complete response, and the remaining radiotherapy was continued at single daily fractions at the patient's request. Following the completion of definitive chemoradiotherapy, a computed tomography scan and direct laryngoscopy revealed no evidence of disease. Consolidative systemic chemotherapy consisting of two cycles of irinotecan and carboplatin was administered. Prophylactic cranial irradiation was offered but was refused. Post-treatment evaluation, including a computed tomography scan, 18F-2-deoxy-2-fluoro-d-glucose positron emission tomography scan, and direct laryngoscopy, were all negative for residual disease. The patient remains free of disease with an excellent performance status at more than 2 years after diagnosis.
By definition, patients with extrapulmonary small-cell carcinoma have a histologic diagnosis of small-cell carcinoma; a normal plain radiograph and computed tomography scan of the chest; and normal sputum cytology or preferably, a negative bronchoscopic examination.1 The first reports of primary small-cell carcinomas of the head and neck appeared in the literature as early as 1949.2 The larynx is the most prevalent site of disease.3 Other sites where these tumors have been reported are in the minor salivary glands,4 paranasal sinus,5,6 parotid,7,8 hypopharynx,3 and trachea.2 The majority of patients with small-cell carcinoma of the head and neck are male, and as is the case with lung primaries, there seems to be a strong association with cigarette smoking.9,10 Frequent metastatic sites include lymph nodes, lung, liver, and bone.3,4,11 Unlike pulmonary small-cell carcinomas, in which 17% to 47% of patients have bone marrow metastases, marrow involvement for patients with small-cell cancers of the head and neck is unusual.12 Patients are staged in a similar fashion as those with small-cell carcinoma of the lung. In limited disease, tumor is confined to a localized region (encompassing one radiation portal) with or without regional lymphatic involvement. Spread of disease beyond locoregional boundaries is considered extensive disease and is incurable. The majority of cases of localized small-cell cancer of the head and neck presented in the published literature have been treated with local modalities of surgery, radiation, or both in the absence of systemic chemotherapy. Tumors of the major salivary glands are associated with a better prognosis than small-cell carcinomas of the lung or nonsalivary gland sites in the head and neck region, with 2- and 5-year survival rates of 70% and 46%, respectively.8 Survival approaching 2 years or more has been reported in three patients with small-cell carcinoma metastatic to cervical lymph nodes, without an identifiable primary tumor.9,13 However, the median survival of patients with primary small-cell cancers of the larynx, hypopharynx, and trachea is between 7 and 11 months7,11,14; these tumors seem to demonstrate a more aggressive clinical course. Although fewer in number, more recent case reports and series support the present use of concurrent chemoradiotherapy regimens for limited-stage disease1,6,15 and offer potential for long-term survival. However, the role of prophylactic brain irradiation for these patients is unknown. Authors' Disclosures of Potential Conflicts of Interest The authors indicated no potential conflicts of interest.
REFERENCES 1. Galanis E, Frytak S, Lloyd RV: Extrapulmonary small cell carcinoma. Cancer 79: 1729-1736, 1997[CrossRef][Medline] 2. Pantridge JF: Primary carcinoma of the trachea. Br J Surg 37: 48-49, 1949[Medline] 3. Olofsson J, Van Nostrand AW: Anaplastic small cell carcinoma of larynx. Case report. Ann Otol Rhinol Laryngol 81: 284-287, 1972[Medline] 4. Koss LG, Spiro RH, Hajdu S: Small cell (oat cell) carcinoma of the minor salivary gland origin. Cancer 30: 737-741, 1972[Medline] 5. Kameya T, Shimosato Y, Adachi I, et al: Neuroendocrine carcinoma of the paranasal sinus: A morphological and endocrinological study. Cancer 45: 330-339, 1980[Medline] 6. Noguchi K, Urade M, Sakurai K, et al: Small cell neuroendocrine carcinoma of the maxillary sinus: A case report and nude mouse transplantable model. Head Neck 24: 491-496, 2002[Medline] 7. Levenson RM, Ihde DC, Matthews MJ, et al: Small cell carcinoma presenting as an extrapulmonary neoplasm: Sites of origin and response to chemotherapy. J Natl Cancer Inst 67: 607-612, 1981 8. Gnepp DR, Corio RL, Brannon RB: Small cell carcinoma of the major salivary glands. Cancer 58: 705-714, 1986[Medline] 9. Remick SC, Hafez GR, Carbone PP: Extrapulmonary small-cell carcinoma: A review of the literature with emphasis on therapy and outcome. Medicine 66: 457-471, 1987[Medline] 10. Remick SC, Ruckdeschel JC: Extrapulmonary and pulmonary small-cell carcinoma: Tumor biology, therapy and outcome. Med Pediatr Oncol 20: 89-99, 1992[Medline] 11. Gnepp DR, Ferlito A, Hyams V: Primary anaplastic small cell (oat cell) carcinoma of the larynx: Review of the literature and report of 18 cases. Cancer 51: 1731-1745, 1983[Medline] 12. Pierce ST, Cibull ML, Metcalfe MS, et al: Bone marrow metastases from small cell cancer of the head and neck. Head Neck 16: 266-271, 1994[Medline] 13. Kasimis BS, Wuerker RB, Malefatto JP, et al: Prolonged survival of patients with extrapulmonary small cell carcinoma arising in the neck. Med Pediatr Oncol 11: 27-32, 1983[Medline] 14. Mills SE, Cooper PH, Garland TA, et al: Small cell undifferentiated carcinoma of the larynx: Report of two patients and review of 13 additional cases. Cancer 51: 116-120, 1983[CrossRef][Medline] 15. Barker JL Jr, Glisson BS, Garden AS, et al: Management of nonsinonasal neuroendocrine carcinomas of the head and neck. Cancer 98: 2322-2328, 2003[Medline] This article has been cited by other articles:
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Copyright © 2005 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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