Article Note: Conflict of interest: the authors declare that they have no conflicts of interest. Byline: D. Mintoff, N. P. Pace, V. Mercieca, P. Bauer, I. Borg
Keywords: Laryngology; Early glottic cancer; Laryngeal cancer/vocal fold dysplasia; Shrinkage Purpose The margin status after CO.sub.2 laser cordectomy for glottic carcinoma may influence prognosis. ...There are no studies assessing the possible bias due to anatomic changes of the specimens for shrinkage. The authors evaluated the intraoperative shrinkage of specimens immediately after transoral CO.sub.2 laser microsurgery (CO.sub.2 TOLMS) to improve the understanding and the interpretation of surgical margins. Methods This is a prospective study involving a consecutive cohort of 23 patients (19 males, 4 females, mean age 69.9 years, range 42--83 years) with early glottic carcinoma treated by CO.sub.2 TOLMS from February 2017 to April 2019. The anteroposterior shrinkage of the specimen, of the tumor, and of the anterior and posterior margins was measured intraoperatively with a cross table reticle glass micrometer ruler, inserted into the eyepiece of the surgical microscope. Results The mean shrinkage of the mucosal specimen from intralaryngeal measurement to post-resection measurement was 3.8+ or -0.3 mm, resulting in an average loss of 29% of the anteroposterior length (p<0.01). The anteroposterior length of both the tumor and the margins after resection significantly decreased, but the shrinkage of the anterior and posterior margins was significantly greater than the shrinkage of the tumor (49% versus 20% and 45% versus 20%, p<0.01). Conclusion The present study demonstrates significant shrinkage of specimens after CO.sub.2 TOLMS, especially in the anteroposterior length of the vocal cords, and justifies the good oncological results for specimens with close and single positive superficial margins. Follow-up versus a second surgical look policy could be safely suggested in cases of close superficial and single positive superficial margins. Author Affiliation: (1) Unit of Otorhinolaryngology, Department of Surgery, Azienda Ospedaliero-Universitaria di Cagliari, University of Cagliari, 09100, Cagliari, Italy (2) Unit of Pathology, Department of Surgery, Azienda Ospedaliero-Universitaria di Cagliari, University of Cagliari, Cagliari, Italy (b) filippocarta@unica.it Article History: Registration Date: 01/15/2021 Received Date: 10/20/2020 Accepted Date: 01/15/2021 Online Date: 01/30/2021 Byline:
Fibrous dysplasia (FD) is an uncommon and debilitating skeletal disorder resulting in fractures, deformity, functional impairment, and pain. It arises from post-zygotic somatic activating mutations ...in
GNAS
, in the cAMP-regulating transcript α-subunit, G
s
α. Constitutive G
s
signaling results in activation of adenylyl cyclase and dysregulated cAMP production. In the skeleton, this leads to the development of FD lesions with abnormal bone matrix, trabeculae, and collagen, produced by undifferentiated mesenchymal cells. FD may occur in isolation or in combination with extraskeletal manifestations, including hyperfunctioning endocrinopathies and café-au-lait macules, termed McCune-Albright syndrome (MAS). This review summarizes current clinical and translational perspectives in FD/MAS, with an emphasis on FD pathogenesis, natural history, pre-clinical and clinical investigation, and future directions.
Arrhythmogenic right ventricular cardiomyopathy is a heritable heart-muscle disorder that causes progressive replacement of right ventricular myocardium by fibrofatty tissue. Mutations in genes ...encoding desmosomal proteins play a key role in the pathogenesis of the disease.
Arrhythmogenic right ventricular cardiomyopathy (ARVC), also known as arrhythmogenic right ventricular dysplasia, is a heritable heart-muscle disorder that predominantly affects the right ventricle. Progressive loss of right ventricular myocardium and its replacement by fibrofatty tissue is the pathological hallmark of the disease.
1
ARVC is one of the leading causes of arrhythmic cardiac arrest in young people and athletes. Since the original report by Marcus and colleagues was published in 1982, describing 24 affected patients,
2
there have been substantial advances in our understanding of the pathogenesis, clinical manifestations, and long-term outcome of the disorder. The disease was initially designated as a . . .
Purpose of Review
This study aims to review diagnosis, potential complications, and clinical management in craniofacial fibrous dysplasia.
Recent Findings
Fibrous dysplasia (FD) is a rare mosaic ...disorder in which normal bone and marrow are replaced with expansile fibro-osseous lesions. Disease presents along a broad spectrum and may be associated with extraskeletal features as part of McCune-Albright syndrome (MAS). The craniofacial skeleton is one of the most commonly impacted areas in FD, and its functional and anatomical complexities create unique challenges for diagnosis and management.
Summary
This review summarizes current approaches to diagnosis and management in FD/MAS, with emphasis on the clinical and therapeutic implications for the craniofacial skeleton.
PDF
