ABSTRACT
Cicatricial pemphigoid (CP) is a rare, chronic, vesiculobullous disease characteristically affecting the mucous membranes and healing with cicatrization. Laryngeal involvement is rare and leads to airway stenosis. We present a 74-year-old Caucasian woman with CP, affecting the oral cavity, esophagus, lower eyelids, and larynx. Regardless of regular treatment with hydrocortisone and azathioprine, she developed bilateral cicatrization of the aryepiglottic folds and ovoid stenosis of the laryngeal introitus, leading to dyspnea. To avoid tracheostomy, we were able to utilize infraglottic high-frequency jet ventilation under total intravenous anesthesia to perform a CO2 laser supraglottoplasty with sectioning of the aryepiglottic folds. Post-operatively, her dyspnea at rest resolved; there was no progression at the six- and 12-month follow-up, and she was satisfied with the result.
ABSTRACT
Thornwaldt cyst is a rare cystic formation, located along the midline of the nasopharynx. We present the case of a 60-year-old man with impaired nasal breathing and a several months-long history of serous otitis media. His only concomitant disease was arterial hypertension. The diagnostic imaging tests revealed a well-rounded cystic formation involving the upper part of the nasopharynx, characteristic of Thornwaldt cyst. Following, endoscopic transnasal marsupialization was performed and the benign cystic nature was confirmed on histopathology. The patient responded to the administered treatment and reported no persistence or emergence of new symptoms. The current case presents a symptomatic Thornwaldt cyst successfully treated by endoscopic transnasal marsupialization.
ABSTRACT
Pleomorphic adenomas (PAs) are benign tumors of the salivary glands. Rarely, they arise in the sinonasal cavity, presenting as well-defined, homogeneous soft tissue masses, causing expansive bony changes. The significance of PAs is the possibility of giving rise to malignant carcinoma - "carcinoma ex-pleomorphic adenoma" (CXPA).Here, we present the case of a 64-year-old female complaining of progressive unilateral congestion and external nose deformation, mostly along the left contour of the radix, with epiphora of the ipsilateral eye. Eventually, a tumor began protruding from the left naris. The computed tomography excluded osteolysis, while the surgical procedure discovered the inferior turbinate as the origin of the tumor. In addition, the ipsilateral maxillary sinus was found to have developed secondary sinusitis. After complete surgical excision, the histological result was sinonasal melanoma, but following no progression of the disease, a second pathologist with additional immunohistochemical markers (HMB-45 (human melanoma black 45) negative, Melan-A (melanoma antigen recognized by T-cells 1) negative, S100 (protein soluble in 100% ammonium sulfate at neutral pH) positive, panCK AE1/AE3 (pan cytokeratin antibodies AE1 and AE3) negative, p63 (tumor protein 63) negative, Ki-67 (marker of proliferation Kiel 67) 10%, CD68 (cluster of differentiation 68) negative, CK7 (cytokeratin 7) negative, and CDX2 (caudal-type homeobox 2) negative) placed the definitive diagnosis of PA.PA of the inferior turbinate is an extremely rare finding, with the clinical symptoms being unspecific. Sometimes, SOX-10 (SRY-box transcription factor 10) positivity can mislead to malignant melanoma, as in our case, which is why a broad panel of immunohistochemical markers is critical for the definitive diagnosis.
ABSTRACT
INTRODUCTION: Gastrointestinal stromal tumors (GISTs) usually present as an exophytic mass localized in stomach. Years ago, conventional surgery was the only option. Laparoscopic and robotic procedures have risen to prominence in recent decades as technology and surgical techniques have advanced.
Subject(s)
Gastrointestinal Stromal Tumors , Laparoscopy , Robotic Surgical Procedures , HumansABSTRACT
OBJECTIVES: To examine and characterize the agenesis of the corpus calosum (ACC) in an epidemiological study of fetal autopsies, as well as, to analyze the associated anomalies and to emphasize the importance of the clinical examination of ACC. METHODS: The subjects of observation are 20 fetuses from a total of 2238 autopsies carried out during a period of three years (2006-2009) in Tunis. RESULTS: The associated abnormalities are hydrocephalus, cerebellar hypoplasia, agenesis of vermis cerebelli, polymicrogyria and lissencephaly. Sixteen of the cases (80%) are syndromic: Trisomy 13,18,21 (5,1,2 fetuses respectively) and Thanatophoric dysplasia, Fetal akinesia syndrome, Dandy-Walker Malformation and the Association VACTERL are represented by two cases each. CONCLUSION: The prenatal diagnosis of ACC must be the result of a multidisciplinary approach. The phenotype of the XLAG syndrome creates an interest to study asymptomatic patients with ACC, especially when the anomaly is detected prenatally.