The language of surgical pathology--a precis for the head and neck surgeon.
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Biomedical subjects
Publications and source records attributed to Alessandra Rinaldo.
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Paragangliomas are neuroendocrine tumors derived from the extra-adrenal paraganglia of the autonomic nervous system. Within the head and neck, they are generally defined and named according to their site of origin, and may be found frequently neighboring vascular structures. Physiologic activity is rare in these neoplasms and they may exhibit patterns of inheritance which predispose their occurrence in families, often with multicentricity. These tumors generally exhibit a slow rate of growth, most often presenting asymptomatically as a space occupying mass lesion noted clinically or radiographically. The most common paraganglioma of the head and neck is the carotid body tumor followed by the jugulo-tympanic and vagal varieties. Other rare sites where this tumor may occur include; the larynx, sinonasal chambers and orbit. Diagnosis is generally made through a combination of clinical findings and radiographic studies. Magnetic resonance represents the most important imaging modality for the evaluation and characterization of suspected head and neck paraganglioma. Definitive management for these lesions should be carefully considered in relation to both tumor and patient-oriented factors, especially in regard to the potential morbidity of treatment. Surgery and radiation therapy represent the main treatment modalities for paraganglioma. The selection of treatment depends on the size, location, and biologic activity of the tumor as well as the overall fitness of the patient. Although radiotherapy may be effective in arresting growth of these tumors, rarely is the neoplasm eliminated without surgical resection. Surgery may be associated with significant morbidity, primarily as a consequence of incurring major cranial nerve injury. Patient selection (relative to age and medical condition) should be carefully considered prior to recommending aggressive surgery for paragangliomas of head and neck, especially in those patients at risk for disabling surgical morbidity.
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Merkel cell carcinoma (MCC) is a rare neuroendocrine neoplasm of the skin. The tumor most frequently affects elderly patients, with a preference for the head and neck. Incidence rates increase with sun exposure and after immunosuppression and organ transplantation. A significant proportion of MCC have been reported to occur in intimate association with malignant epithelial neoplasms. The genetic mechanisms underlying the development and tumor progression of MCC are poorly understood, sharing pathogenetic mechanisms with other neoplasms of neural crest derivation. MCC has a propensity for spreading to regional lymph nodes, either at presentation or as a first site of relapse. Sentinel lymph node positivity is helpful in predicting the risk of recurrence or metastasis in patients with MCC. Complete surgical resection is the mainstay of treatment of the primary tumor. Tumor resections are recommended to include a 2-3-cm tumor-free margin around the primary lesion when possible, but this is often difficult to achieve in the head and neck, where Mohs micrographic surgery has proved to be effective. The role of adjuvant radiation therapy is controversial. The role of adjuvant chemotherapy in diminishing the risk of subsequent systemic recurrence in patients with positive nodes remains undefined. Overall response rates to combination chemotherapy for surgically unresectable distant metastatic disease are generally high, although responses are transient. Overall survival of head and neck MCC at 5 years postoperatively ranks between 40% and 68%. MCC has a high incidence of locoregional recurrences, but even after a locoregional failure, a substantial proportion of patients achieve long-term disease-free survival. Finally, factors generally associated with survival are the stage of disease at presentation, distant recurrence usually being the most adverse predictor of survival.
The reported success rate of microvascular free flap reconstruction ranges between 95% and 97%. However when complications occur, they must be identified early and managed efficiently because there is a narrow window of opportunity to salvage potential flap failure. While technical advances in instrumentation and magnification have improved overall success rates, the rare complication may prove devastating for the patient, his/her hospital stay, and the optimum rehabilitation. Complications of microvascular free tissue transfer may occur at the recipient site or at the donor site. Complications occurring at the recipient site are largely a result of vessel thrombosis while complications occurring at the donor site may result from many causes, ranging from infection to those related to the harvesting of the flap. Irrespective of the site of the complication, it is essential that complications be recognized and addressed early in their course to prevent or minimize devastating consequences.
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The majority of tumors of the sublingual gland are malignant, with adenoid cystic carcinoma and mucoepidermoid carcinoma being the most frequent. Many other malignant tumor types have also been reported. The sublingual gland anatomically is not a unit organ and while it is described anatomically as being confined to the anterior floor of the mouth, salivary tissue may be located laterally along the submandibular duct and posterior floor of the mouth. Diagnosis should be suspected when any thickening or raised lesion presents in this area and a biopsy performed to confirm malignancy before planning further treatment. Surgery is the treatment of choice, and should include an en-block resection of the anterior floor of mouth as a minimum, and may include a portion of mandible, as well as a supraomohyoid neck dissection. Adjuvant radiotherapy should be considered in most of the patients after surgical excision.
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Open supraglottic laryngectomy is an oncologically sound surgical procedure for selected cases of laryngeal cancer which maintains physiologic speech and swallowing without the need for a permanent tracheostoma. The oncologic principles, techniques for resection and reconstruction, postoperative management and complications are discussed.
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Crohn's disease may present to the head and neck surgeon with symptoms and signs attributed to the disease. Many clinical presentations, both specific and non-specific, have been recorded in the oral cavity, nose and larynx. Some of these clinical manifestations have been found to be consistent with Crohn's disease, but most have been attributed to Crohn's disease without histologic confirmation of the presence of non-caseating granulomas, which is pathognomonic of Crohn's disease. Lesions, when present without an associated diagnosis of Crohn's disease, should be ascribed the diagnosis of orofacial granulomatosis, until a diagnosis of intestinal Crohn's disease has been confirmed.
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