CREST syndrome diagnosed by oral lesions: A case report and review of the literature.

BACKGROUND: Calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST) syndrome is an acronym for the clinical features that are seen. Its etiology is unknown, affecting women three times more than men. CREST syndrome is often diagnosed by systemic symptoms. However, oral manifestations could be helpful in the early diagnosis of the disease. Less than 20 cases of CREST syndrome with oral symptoms were described. OBJECTIVE: To report a case of a 26-year-old female, who was diagnosed with CREST syndrome based on findings of the oral mucosa. CASE REPORT: Clinical examination revealed sclerodactyly and nail alterations. Oral findings were associated with hypochromic and paleness mucosa. The tongue was strongly rigid and reddish areas compatible with telangiectasias were found. The mouth opening was particularly compromised. Histological findings were suggestive of systemic sclerosis in the context of CREST syndrome. Considering the available diagnosis criteria, this case was diagnosed as CREST syndrome based on oral manifestations. CONCLUSIONS: Oral manifestations of CREST syndrome could help in early clinical diagnosis, preventing a delay in the onset of an aggressive form of the disease. Accurate referrals of patients to specialists are needed for a multidisciplinary approach.

Tumor maligno de la vaina nerviosa periférica: reporte de un caso / Malignant peripheral nerve sheath tumor, a case report

Resumen El tumor maligno de la vaina nerviosa periférica (TMVNP) es un sarcoma de alto grado de malignidad. Es poco frecuente, agresivo y generalmente se localiza en tronco y miembros inferiores. Se presenta mayormente en pacientes con neurofibormatosis tipo 1, aunque no siempre se encuentra esta asociación. Este tumorcomparte características histológicas e inmunohistoquímicas con el melanoma, lo que puede dificultar el diagnóstico. Presentamos el caso de un paciente con TMVNP, en el cual los hallazgos histológicos iniciales condujeron a un diagnósticoerróneode melanoma.

Abstract Malignant peripheral nerve sheath tumor (MPNST) is a high-grade sarcoma. It is rare, aggressive and generally located on the trunk and lower limbs. It occurs in a high percentage of patients with neurofibormatosis type 1, although this association is not always found. This tumor shares histological and immunohistochemical characteristics with melanoma, which can make diagnosis difficult. We present the case of a patient with MPNST, in whom the initial histological findings led to an erroneous diagnosis of melanoma.

Surgical resection of a presacral solitary fibrous tumor with extension to iliac vessels using Karakousis's abdominoinguinal approach: Report of a rare case.

INTRODUCTION AND IMPORTANCE: The solitary fibrous tumor (SFT) is a rare tumor of mesenchymal origin, with a reported incidence of 2.8 cases per 100,000 tumors and with distinctive histopathological and immunohistochemical characteristics. It was initially described as a pleural lesion and subsequently, it was found in different organs and tissues. The abdominoinguinal incision described by Karakousis allows a safe and radical approach for lower quadrants abdominopelvic tumors. CASE PRESENTATION: A 47-year-old man was referred to us with a 5-months history of lower backache radiating to the left lower limb. MRI and CT revealed a retroperitoneal mass of 10 cm extending to left iliac vessels. The initial diagnosis corresponded to a sarcomatous retroperitoneal tumor. It was decided to perform an abdominal exploration using Karakousis's approach for surgical resection. The immunohistochemistry and histopathological study revealed neoplasia compatible with a SFT. It was categorized as low risk for developing metastasis and death from disease, according to the new malignancy criteria. Currently, the patient is asymptomatic and disease-free at 19 months after surgery. CLINICAL DISCUSSION: Most patients with SFTs present symptoms derived from the tumor growth and the compression on adjacent structures with clinical manifestations that are frequently insidious and precede the tumor discovery. The diagnosis is based on histopathological studies. Nonetheless, when they present an extrathoracic location, they represent a diagnostic challenge, due to their variable histological characteristics. CONCLUSION: Presacral SFT is a rare entity, with a scant incidence reported regarding this location and long-term treatment. Surgical resection is needed as the immediate treatment.

Phototoxic Effect of Topical Fluoroquinolones Administered Before Corneal Crosslinking in a Murine Model.

PURPOSE: Corneal crosslinking by UV light (UV-CXL) has become a popular treatment for keratoconus and corneal ectasia. Fluoroquinolones (FQs), commonly administered topically before UV-CXL, are known to be phototoxic to the skin and lens. The purpose of this study was to investigate phototoxic effects of topical FQ treatment on murine corneas before UV-CXL, in which the corneal epithelium was kept intact. METHODS: Murine corneas were treated with various antibiotics with or without riboflavin before UV-CXL. At 24 h, the animals were sacrificed, and the corneas were analyzed for histologic evidence of inflammation and apoptosis and for expression of apoptosis markers BAX and caspases 3 and 9 and for expression of matrix metalloproteinase 9 (MMP-9). Spectrofluorometric analysis was performed. RESULTS: Corneas treated with topical FQ with or without riboflavin before UV-CXL showed mild corneal stromal inflammation, apoptosis by both terminal deoxynucleotidyl transferase dUTP nick end labeling staining and increased expression of BAX gene and caspases 3 and 9 by densitometric analysis. Untreated corneas, corneas treated with azithromycin before UV-CXL, and corneas undergoing UV-CXL without any antibiotic or riboflavin pretreatment showed normal histology, no staining for apoptosis, and no increased production of apoptosis markers by polymerase chain reaction. CONCLUSIONS: The phototoxic effects of FQs on the cornea may lead surgeons to consider another antibiotic class for prophylaxis against infectious keratitis in UV-CXL. These effects, along with the known cytotoxic effects of FQs independent of UV radiation, may contribute to some of the complications of corneal UV-CXL. Dosage studies may be warranted.

Protective effect of early placement of nasogastric tube with solid dilator on tissue damage and stricture formation after caustic esophageal burns in rabbits.

BACKGROUND: The ingestion of caustic substances remains an important public health issue worldwide. Children represent 80% of the ingestion injury population globally. Accidental alkaline material accounts for most caustic ingestions. There is no conclusive evidence of tissue damage and stricture protection of a nasogastric-tube with a solid dilator in the literature, therefore it was hypothesized that early intraesophageal tube placement does not cause additional histopathologic damage and prevents strictures. METHODS: An exploratory study on experimental caustic esophageal burns in a rabbit model was designed. In the treated group a silicone tube was placed immediately after causing the burns, while the untreated group followed the natural course of the burn. On the twenty-secondday, an esophagectomy was performed on all animals for microscopic (Histopathologic Damage Score and Stenosis Index) and macroscopic analysis. RESULTS: Forty animals were randomly divided into two groups. The Histopathologic Damage Score was 3.7±1.1 in the treated group versus 3.9±1.2 in the untreated group (p=.9690). The Stenosis Index was 0.6±0.1 in treated rabbits versus 2.3±0.2 in untreated (p<.0001). CONCLUSION: The early placement of an intraesophageal tube with solid dilator prevents stenosis formation and does not produce greater tissue damage.