Primary Merkel cell carcinoma of the parotid gland.

Primary Merkel cell carcinoma (MCC) is a neuroendocrine tumor that typically affects older whites. It usually originates in the dermis, but in rare instances it has arisen in other primary sites, including the oral mucosa and the parotid gland. Explanations for the presence of MCC in these atypical locations have included theories of malignant transformation and neuroendocrine migration. We describe the case of a 35-year-old white woman who had an MCC in the parotid gland and no evidence of any other primary cutaneous lesion. A left superficial parotidectomy with facial nerve preservation was performed, and a histologic diagnosis of MCC was made. Positron-emission tomography was negative for other lesions, and postoperative radiation therapy was administered for local control. To the best of our knowledge, this is only the sixth case of a primary MCC of the parotid gland to be reported in the English-language literature; of these 6 patients, ours was by far the youngest. Otolaryngologists should be familiar with this rare but potentially fatal neoplasm.

Esophageal hamartoma as an unusual cause of neonatal apnea and bradycardia.

Esophageal hamartoma is a rare entity, as only 7 cases have been previously reported in the English-language literature. Common symptoms include dysphagia, weight loss, and vomiting. Life-threatening airway obstruction can also occur. Because of the nonspecific nature of the symptoms, patients with these dangerous polyps are often misdiagnosed as having more common entities such as gastroesophageal reflux, peptic ulcer disease, or achalasia. Most of these tumors are missed on esophagoscopy and radiologic studies, and they can go undiagnosed for years. We diagnosed an esophageal hamartoma in an infant girl who had first presented when she was 8 days old with symptoms of apnea and cyanosis. The patient had undergone a multitude of tests since her birth, and she was eventually diagnosed with episodic bradycardia. When the patient was 6 weeks old, we discovered a polyp on nasopharyngolaryngoscopy, and we removed it by microdirect laryngoscopy and esophagoscopy. This patient was the youngest of the 8 who have now been reported to have been diagnosed with a hamartomatous polyp, and she was the only one to have presented with apnea (secondary to airway obstruction) and bradycardia. We recommend microdirect laryngoscopy and esophagoscopy to remove these pedunculated cervical esophageal lesions. A transcervical approach is warranted for sessile distal esophageal polyps. Esophageal polyps are an interesting entity in view of their rarity and intriguing presentations. Because esophageal obstructions can be life-threatening, further evaluation by laryngoscopy, bronchoscopy, and esophagoscopy is warranted when symptoms of dysphagia, vomiting, intermittent apnea, bradycardia, and weight loss persist despite conventional treatment.