Not Your Typical Tonsil: Metastatic Merkel Cell Carcinoma or Primary Disease?

Merkel cell carcinoma (MCC) is a rare, aggressive neuroendocrine tumor that almost always presents as a cutaneous lesion in the sun-exposed areas on the bodies of elderly white males. Metastasis to lymph nodes in the presence or absence of a known primary site and occurrence of these tumors in non-sun-exposed sites have also been described; however, an incidence of recurrent disease arising in the palatine tonsil in the absence of any detectable primary lesion has never been reported in the literature. In this report, we discuss a case of a 72-year-old female who was found to have a single axillary lymph node, which was resected and proved to be positive forMCC of unknown primary (MCCUP). Since there was no evidence of additional disease, the patient elected not to pursue adjuvant therapies. Six and a half months later, she presented with a complaint of dysphagia and a right-sided exophytic tonsillar mass. Tonsillectomy revealed MCC with no detectable primary cutaneous lesion. She received adjuvant therapy with avelumab and demonstrated a complete response after one year of bi-weekly treatments. Seven months following cessation of adjuvant treatments, surveillance positron emission tomography (PET) revealed enlarged retroperitoneal, pretracheal, periaortic, and left axillary lymph nodes concerning for recurrence. She elected to forgo additional biopsies and restarted avelumab the following month. She continues to be followed up on a monthly basis.

Prophylactic Flexible Bronchoscopy Immediately following Open Airway Reconstruction in Children.

OBJECTIVES: Prophylactic flexible bronchoscopy immediately following open airway reconstruction allows for directed clearance of the distal airways, potentially reducing the rate of certain postoperative respiratory complications. In this investigation, we sought to determine if prophylactic flexible bronchoscopy at the conclusion of pediatric open airway reconstruction has any benefit over blind flexible suctioning of the trachea. METHODS: A retrospective, single-center study at an urban tertiary care hospital was completed. From January 2010 to April 2013, patients underwent open airway reconstruction, immediately followed by blind flexible suctioning of the trachea for distal airway clearance. From May 2013 through December 2016, sequential patients underwent prophylactic flexible bronchoscopy immediately following airway reconstruction. RESULTS: A total of 29 patients (age: 3.6 months-6.2 years) met inclusion criteria. Sixteen sequential patients underwent simple blind flexible suctioning and 13 sequential patients underwent directed, prophylactic flexible bronchoscopy. Demographics and comorbidities between the groups were equivalent other than slightly older age in the prophylactic bronchoscopy group. All clinical outcomes analyzed were equivalent other than faster time to room air (P < .002) and a decrease in the number of chest physical therapy sessions (P < .02) in a subset of patients who did not undergo prophylactic bronchoscopy. CONCLUSION: This investigation suggests that the use of prophylactic flexible bronchoscopy immediately following open airway reconstruction may not be superior to blind flexible suctioning of the trachea in limiting postoperative pulmonary complications. Further studies of greater power are needed to better elucidate any small differences that may exist between these two interventions.

Breast Cancer Metastases to the Paranasal Sinuses Mimicking Inflammatory Sinus Disease.

INTRODUCTION: Breast cancer is one of the most common cancers in women. Metastatic disease is a leading cause of morbidity and mortality. It frequently metastasizes to bone, lungs, regional lymph nodes, liver and brain. Metastasis to the orbit and paranasal sinuses is uncommon. Patients presenting with sinus pain, nasal congestion, or visual disturbance can be misdiagnosed with an infectious process. METHODS: The authors describe 2 patients with metastatic breast cancer to the paranasal sinuses presenting with signs and symptoms of sinusitis and orbit pathology unresponsive to antibiotics. The authors discuss diagnostic strategy and perform a literature review. Both patients had biopsy-proven metastatic breast adenocarcinoma lesions, and subsequently underwent various treatment options. RESULTS: A literature review reveals that metastatic breast adenocarcinoma lesions to the paranasal sinuses are a rare entity that commonly denotes a very poor prognosis. These lesions can significantly affect one's quality of life, and can cause blurry vision, diplopia, proptosis, sinus pressure and pain, nasal congestion, mandibular misalignment or difficulty with mastication. However, early diagnosis and treatment of these lesions can lead to prolonged survival and improved quality of life. If the lesion is surgically resectable, endoscopic sinus surgery is generally considered to be the optimal treatment. However, radiotherapy, chemotherapy, and immunotherapy can potentially play a role in controlling the symptomatology. CONCLUSION: Metastatic lesions to the paranasal sinuses are a rare entity that should be considered in the appropriate clinical setting in order to expedite the proper treatment modality for improved morbidity and mortality.

Squamous cell carcinoma presenting with trigeminal anesthesia: An uncommon presentation of head & neck cancer with unknown primary.

BACKGROUND: The differential diagnosis of facial anesthesia is vast. This may be secondary to trauma, neoplasm, both intracranial and extracranial, infection, and neurologic disease. When evaluating a patient with isolated facial anesthesia, the head and neck surgeon often thinks of adenoid cystic carcinoma, which has a propensity for perineural invasion and spread. When one thinks of head and neck squamous cell carcinoma with or without unknown primary, the typical presentation involves dysphagia, odynophagia, weight loss, hoarseness, or more commonly, a neck mass. Squamous cell carcinoma presenting as facial anesthesia and perineural spread, with no primary site is quite rare. METHODS: Case presentations and review of the literature. CONCLUSIONS: Trigeminal anesthesia is an uncommon presentation of head and neck squamous cell carcinoma with unknown primary. We present two interesting cases of invasive squamous cell carcinoma of the trigeminal nerve, with no primary site identified. We will also review the literature of head and neck malignancies with perineural spread and the management techniques for the two different cases presented.

Metastatic breast carcinoma of the jugular foramen: a rare case of Villaret syndrome.

BACKGROUND: The differential diagnosis of skull base masses is diverse and includes benign and malignant neoplasms, vascular anomalies, congenital lesions, as well as infectious and inflammatory processes. Metastatic masses of the skull base are a rare manifestation of systemic malignancies. Breast cancer is the most common cause of skull base metastases. Villaret syndrome refers to cranial nerves IX, X, XI, and XII and sympathetic chain neuropathies. It is a clinical subtype of jugular foramen syndromes. METHODS AND RESULTS: A 62-year-old woman with a history of breast carcinoma presented with hoarseness dating to shortly after her mastectomy years earlier. CT angiography showed enhancing tissue just outside the right jugular foramen, and biopsy confirmed metastatic adenocarcinoma consistent with breast cancer. CONCLUSION: Villaret syndrome caused by breast cancer metastases has not been previously described. We present a case of Villaret syndrome caused by metastasis of invasive breast adenocarcinoma and a review of the literature of metastases of breast cancer to the skull base.

Oral squamous cell carcinoma in post-transplant patients.

OBJECTIVE: Patients on immunosuppressant therapy after transplantation have an increased risk of developing cutaneous squamous cell carcinomas. The risk of developing solid tumors of the upper aerodigestive tract in this population has been less defined. We present five patients that subsequently developed oral squamous cell carcinoma after transplantation. STUDY DESIGN: Retrospective chart review and literature review. RESULTS: Three bone marrow and two heart transplant patients were subsequently diagnosed with oral (oral cavity or oropharynx) carcinoma. The timing of diagnosis of oral cancer after transplant ranged from 18 months to 17 years post-transplantation. CONCLUSIONS: Patients with a history of transplantation should be routinely assessed for the potential development of oral neoplastic lesions. Oral squamous cell carcinoma in transplant patients can be more aggressive and clinically mistaken for chronic graft versus host disease. It is therefore reasonable to consider early biopsy in these patients to guide the need for intervention.

Management of a postradiation esophageal web in the setting of a coexisting Zenker's diverticulum.

We present a case of postradiation obstructive esophageal web, seen in the setting of a coexisting Zenker's diverticulum, that was treated with combined anterograde and retrograde esophagoscopy puncture and dilation. We also review the relevant literature. Obstructing esophageal webs and stenoses are well-documented complications seen in patients with head and neck cancer treated with radiation therapy. For thin webs, combined anterograde and retrograde esophagoscopy along with puncture and dilation can be a relatively safe treatment option for selected patients. The presence of a coexisting Zenker's diverticulum may predispose the patient to the development of postradiation esophageal complications and make subsequent assessment more difficult to perform.