July 5, 2012 Matthew L. Carlson, MD; Amy M. Saleh, MD; Keith J. Kaplan, MD; Shelagh A. Cofer, MD
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We describe the fifth published report of a mesenchymal hamartoma presenting as a cheek mass. A 5-month-old infant was brought to our institution for evaluation of an enlarging left-sided congenital cheek mass. Over time, the lesion had begun to cause significant facial asymmetry and oral incompetence. Radiologic imaging revealed an approximate 2.5 x 3.5-cm, noncystic lesion located in the left buccal space, separate from the mandible and surrounding the salivary glands. Magnetic resonance imaging (MRI) sequences demonstrated an isointense, T1-weighted lesion with avid gadolinium uptake, and increased intensity of T2-weighted sequences. The patient subsequently underwent biopsy and subtotal resection through a left gingivobuccal incisionwith the goal of improving lip contour and facial symmetry.Histologic examination revealed an admixed arrangement of mature smooth muscle, vascular, adipose, and neural tissue elements within a slightly myxoid stroma, consistent with a mixed mesenchymal hamartoma. An 8-month postoperative MRI demonstrated near-total removal without evidence of regrowth. While rare, hamartomas should be included in the differential diagnosis of a slow-growing pediatric head and neck mass. Gross total resection may provide cure; however, given this lesion’s benign nature, less-than-complete resection should be considered when lesions infiltrate opposing critical structures. Thorough clinical and histologic evaluation is critical to avoid overly aggressive treatment and unnecessary morbidity.
April 30, 2010 Yamileth Nicolau, MD, William C. Faquin, MD, PhD, and Daniel G. Deschler, MD, FACS
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Hamartomas of the parotid gland are very rare. We present the case of a parotid gland hamartoma with oncocytic and sebaceous metaplasia arising as a 3-cm mass in a 70-year-old man. To the best of our knowledge, there has been no previous report of a tumor with the same features. We present this case to create awareness of hamartomas as part of the differential diagnosis of parotid masses.
March 1, 2010 Sidrah M. Ahmad, BS, Fernando Garcia, MD, and Robert T. Sataloff, MD, DMA, FACS
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Cerebellopontine angle (CPA) ganglionic hamartomas are rare. We present a case of a 49-year-old woman who presented with left-sided hearing loss of more than 4 years' duration and vertigo since childhood. Magnetic resonance imaging revealed a mass on the intracanalicular segment of cranial nerve VIII on the left side, consistent with acoustic schwannoma. She opted for observation of the tumor. The patient returned 21 months after the initial evaluation with new-onset left facial weakness. At this time, a working diagnosis of facial nerve neuroma was made. The lesion was excised, and pathology confirmed a diagnosis of left-sided CPA ganglionic hamartoma. Otolaryngologists should be familiar with this uncommon tumor and include it in the differential diagnosis of CPA lesions.
March 1, 2010 Jeffrey Coury, DO, Jonathan Steinfeld, MD, David Zwillenberg, MD, and Seth Zwillenberg, MD
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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.
March 1, 2009 Geva Barzilai, MD, Yaakov Schindler, MD, and Raanan Cohen-Kerem, MD
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Angiomyomatous hamartomas have been found almost exclusively in the inguinal and femoral lymph nodes; few reports of these lesions in the head and neck region have been published. We present a case of angiomyomatous hamartoma in the submandibular area, a site that has not been previously reported in the literature. The mass was initially diagnosed as an enlarged lymph node. When it did not regress following broad-spectrum antibiotic treatment, the patient, a 51-year-old woman, underwent an excisional biopsy. Histopathology identified the mass as an angiomyomatous hamartoma. At follow-up 3 years and 9 months postoperatively, the patient exhibited no evidence of recurrence on physical examination and computed tomography. Even though angiomyomatous hamartoma of the head and neck is rare, we suggest that otolaryngologists include it in the differential diagnosis of head and neck masses.
January 1, 2009 A.A.S. Rifat Mannan, MD, Mehar C. Sharma, MD, Manoj K. Singh, MD, Sudhir Bahadur, MS, and Pradeep Hatimota, MD
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The paranasal sinuses are an extremely unusual location for a vascular hamartoma. As far as we know, only 1 such case has been previously reported in the English-language literature. We report 3 new cases of vascular hamartoma of the paranasal sinuses, which occurred in a 20-year-old woman and in 2 men aged 36 and 45 years. Radiologically, the lesion in the woman was confined to the sinuses, while evidence of intraorbital extension was seen in the 2 men. No intracranial extension was seen in any patient. The diagnosis was confirmed by histopathologic examination of the excised lesions. All 3 patients were alive without recurrence at 12 to 24 months of follow-up.
March 1, 2006 W. Frank Ingram, MD; Michael C. Noone, MD; M. Boyd Gillespie, MD
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Respiratory epithelial adenomatoid (READ) hamartoma is a recently described entity characterized by abnormal glandular formations arising from the epithelium of the nasal cavity. The etiology of the lesion is unclear and may be secondary to either sinonasal inflammation or developmental error. We present a case of a 54-year-old man with a unilateral nasal mass found to be consistent with READ hamartoma upon pathologic review. Although READ hamartomas are thought to be rare, awareness of the lesion is important since it may be confused with sinonasal adenocarcinoma, leading to overly aggressive treatment. Therefore, READ hamartoma should be included in the differential diagnosis of a unilateral nasal mass.