Hereditary paraganglioma (PGL) is characterized by the development of benign, vascularized tumors in the head and neck. The most common tumor site is the carotid body (CB), a chemoreceptive organ that senses oxygen levels in the blood. Analysis of families carrying the PGL1 gene, described here, revealed germ line mutations in the SDHD gene on chromosome 11q23. SDHD encodes a mitochondrial respiratory chain protein-the small subunit of cytochrome b in succinate-ubiquinone oxidoreductase (cybS). In contrast to expectations based on the inheritance pattern of PGL, the SDHD gene showed no evidence of imprinting. These findings indicate that mitochondria play an important role in the pathogenesis of certain tumors and that cybS plays a role in normal CB physiology.
Tumors originating in the parapharyngeal space are rare. During the period of January 1977 to July 1989, 51 patients underwent surgery for parapharyngeal space tumors at the University of Pittsburgh's Eye and Ear Hospital. Eighty percent of the parapharyngeal space neoplasms were benign; 20% were malignant. Fifty-seven percent (31/54) were of neurogenic origin, 30% (16/54) were of salivary origin, and 13% (7/54) were of miscellaneous origin. The use of computed tomography scan and magnetic resonance imaging, and selective use of angiography, allowed us to ascertain the location, size, vascularity, and relation of parapharyngeal space tumors to surrounding anatomical structures. Imaging techniques established the site of origin of these tumors with 96% accuracy. This information was essential in planning surgical approaches and predicting prognoses. Details of the surgery, morbidity, and outcome of these patients are presented.
Perineural invasion of small nerves is associated with an increased risk of local recurrence and cervical metastasis and is, independent of extracapsular spread, a predictor of survival for patients with SCC of the upper aerodigestive tract.
We have built a digital nuclear atlas of the newly hatched, first larval stage (L1) of the wild type hermaphrodite of C. elegans at single cell resolution from confocal image stacks of 15 individuals. The atlas quantifies the stereotypy of the locations and provides for other statistics on the spatial patterns of the 357 nuclei that could be faithfully segmented and annotated of the 558 present at this developmental stage. Given this atlas we then developed an automated approach to assign cell names to each nucleus in a 3D image of an L1 worm. We achieve 86% accuracy in identifying the 357 nuclei automatically. This computational method is essential for high-throughput single cell analyses of the worm at post-embryonic stages, such as determining the expression of every gene in every cell during development from the L1 onward, or ablating or stimulating cells under computer control in a high-throughput functional screen.
This paper updates a 1981 report on the management of inverted papilloma. In that report, routine lateral rhinotomy with en bloc resection of the lateral nasal wall, including the entire schneiderian membrane, was recommended. This report emphasizes the use of computed tomography scanning in management planning. Of 33 patients with inverted papilloma treated between 1969 and 1987, 7 had associated squamous cell carcinoma. Twenty-two patients with IP alone were treated with medial maxillectomy; however, 3 patients have been successfully treated with either a Caldwell-Luc/ethmoidectomy or an external ethmoidectomy for localized disease. The use of computed tomography capable of higher resolution allowed preoperative determination of the extent of disease, enabling the surgeon to plan the surgery more precisely. Patients were followed for an average of 9.6 years. Recurrent disease occurred in one patient (4%) following a lateral rhinotomy/medial maxillectomy approach. The principles set forth in this series of patients remain the same as those previously described due to the unique characteristics of this tumor: its ability to destroy bone, its tendency to recur, and its association with malignancy. The technique of lateral rhinotomy and en bloc excision of the lateral nasal wall, followed by meticulous removal of all mucosa in the ipsilateral paranasal sinuses, remains the standard therapy.
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