1/31. endosonography in the diagnosis of "blue rubber bleb nevus syndrome": an uncommon cause of gastrointestinal tract bleeding.Blue rubber bleb nevus syndrome is a rare condition characterized by the presence of multiple angiomatic lesions of the skin. These are associated with similar lesions in other organs, namely in the gastrointestinal tract, causing anemia through chronic bleeding. We describe the case of a 72-year-old woman with microcytic anemia. A barium study revealed irregular lacunae in the distal esophagus. A subsequent endoscopy showed blue nodular lesions similar to angiomas of the esophagus and stomach fundus. endosonography confirmed its angiomatic nature. Exploration of other organs, using magnetic resonance and cranial computed tomography, did not reveal the presence of this type of lesion. In physical examination, two angiomatic lesions were observed on the face and lips, respectively. These were blue in color and compressible, leaving an empty wrinkled sac that rapidly refilled, typical of angiomas.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/31. Prosthetic rehabilitation of large midfacial defects.Advanced tumors of the midfacial region often require removal of the nose, upper lip, portions of the maxilla, and other adjacent structures. When the resultant defects do not lend themselves to surgical reconstruction, prosthetic appliances may be used successfully to restore the functions of speech and swallowing to near-normal levels. The degree of success depends upon the nature of the previous treatment, the existing surgical defect, and the adaptability of the patient. The most important anatomical consideration is the form and amount of the remaining maxilla. Recent advances in the development of the polyurethanes have resulted in lighter, more flexible, and, therefore, better tolerated prostheses. Close cooperation between the surgeon and the prosthodontist is necessary if successful rehabilitation of these patients is to be achieved.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/31. Resurfacing of facial angiofibromas in tuberous sclerosis patients using CO2 laser with flashscanner.BACKGROUND: Angiofibromas are a common presentation of tuberous sclerosis. They cause considerable cosmetic and hygienic morbidity for patients. Treatments of angiofibromas have included curettage, cryosurgery, chemical peel, dermabrasion, shave excision, and 13-cis retinoic acid. Results from these modalities in many cases were not satisfactory from a cosmetic standpoint. copper vapor, argon, pulsed dye, and CO2 lasers have been used with success in isolated cases. OBJECTIVE: The purpose of this study was to evaluate the efficacy of CO2 laser resurfacing with flashscanner in the treatment of facial angiofibromas. methods: Two patients with angiofibromas on the face were treated with a CO2 laser with flashscanner. The cheek and nose were treated in one patient, and entire face was done in the other. RESULTS: Both patients showed remarkable cosmetic improvements without scarring. Mild hyperpigmentation was found in both patients, which disappeared in 1 month with the application of topical agents. Facial erythema persisted about 2 months in both patients. CONCLUSION: A CO2 laser equipped with flashscanner causes less residual thermal damage than conventional CO2 lasers and enables controlled depth vaporization for more precise and regular removal of angiofibromas. In whole-face resurfacing, more cosmetically acceptable results are possible because localized treatment leaves marginal prominences. Laser resurfacing is an effective alternative in the treatment of multiple protuberant angiofibromas even though we cannot permanently clear these lesions due to their nature.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/31. forehead lipoblastoma mimicking a hemangioma.A case of forehead lipoblastoma simulating a hemangioma in a male infant is reported, to alert pediatricians to this rare tumor and to increase the index of suspicion in atypical hemangiomas. A 2-month-old male infant developed a protruding forehead mass with increased vascularity. It demonstrated progressive and accelerated growth over the subsequent 6 months, unresponsive to steroid therapy. A magnetic resonance imaging scan supported the diagnosis of hemangioma because of the hypervascular nature of the lesion. Surgical excision was performed because of visual obstruction. Pathologic examination of the specimen was consistent with a very primitive lipoblastoma. This tumor is a rare, benign lesion of immature fat cells that is found almost exclusively in the pediatric population. Lipoblastomas are more common in males than females and frequently present as asymptomatic, rapidly enlarging, soft lobular masses on the extremities. Complete surgical excision is the definitive treatment. In the vast majority of reported cases, however, the preoperative diagnosis was incorrect, underscoring the diagnostic dilemma presented by these rare tumors.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/31. Management issues in massive pediatric facial plexiform neurofibroma with neurofibromatosis type 1.BACKGROUND: Plexiform neurofibroma is a relatively common but potentially devastating manifestation of neurofibromatosis type 1 (NF1). Surgical management is the mainstay of therapy, but within the head and neck region it is limited by the infiltrating nature of these tumors, inherent operative morbidity, and high rate of regrowth. METHOD: We describe a case of a 7-year-old girl with neurofibromatosis type 1 and a massive facial plexiform neurofibroma with the aim of emphasizing the treatment and timing issues involved in the management of this difficult problem. A medline search (1966 through December 2000) was carried out, and pertinent literature on the subject was reviewed. RESULT: The patient described in this case report was carefully observed for a period of 6 years from diagnosis before surgical excision of the tumor was undertaken with an uneventful recovery. CONCLUSION: Surgical management remains the mainstay of treatment for these locally invasive tumors, but functional disturbances are almost inevitable in resecting substantial tumors involving the head and neck region. The indication and timing of surgery in pediatric patients therefore needs to be carefully weighed against the physical and psychologic consequences of treatment.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/31. epidermodysplasia verruciformis: 14 members of a pedigree with an intriguing squamous cell carcinoma transformation.A 38-year-old farmer was apparently well until 5 years of age. At this age, the patient's mother found mildly itchy, raised eruptions over the scalp during combing of the scalp hair. Since then, the eruptions have progressed insidiously to involve the face and other parts of the body, with a preponderance over the face, upper back, and chest, including the palms. The soles, oral cavity, and genital tract were unaffected. The lesions were persistent in nature and showed no remission or exacerbation. Mild to moderate pruritus/discomfort was experienced following sunlight exposure. A prominent, raised eruption appeared on the right side of the forehead 9 years ago, 25 years after the initial eruptions, which in due course ulcerated. It was progressive in nature and acquired a large size. Two years later, it was diagnosed as squamous cell carcinoma, for which liquid nitrogen cryosurgery was performed. There was a recurrence of the lesion at the site of surgery, which was excised 4 months later. Subsequently, there was a sudden flare up at the same site. It was badly infected with maggot infestation. The relentless course of the disease was evident by the appearance of two similar lesions, one on the right half of the nose and the other on the left preauricular region. A short while ago, fatigue and loss of weight were also recorded. Bladder and bowel functions were normal and there was no loss of appetite, hoarseness of voice, or breathlessness. Four of the patient's six children (three sons and one daughter) were reported to have similar lesions all over the body. In addition, nine other relatives were also affected. Accordingly, a total of 14 (12 males and two females) family members were found to be affected from amongst 41 individuals (26 males and 15 females) spread across several generations (Fig. 1). There was a second-degree consanguinity of marriage, with the patient's grandmother and wife's father being brother and sister. skin surface examination was marked by multiple, discrete, flat-topped, scaly, brownish-black papules of diverse morphology, from hypopigmented macules to verrucous lesions, with a few coalescing to form plaques. The scales were brown, dry, and adherent (Fig. 2a). The lesions were conspicuous by their presence over the face, neck, and front and back of the chest. The extremities were also affected. Nevertheless, the soles and genitalia were spared. The oral mucosae, hair, and nails were normal. Koebner's phenomenon was explicit, whereas Auspitz's sign was not demonstrable. In addition, a perceptible nodulo-ulcerative lesion (size, 3 in x 2 in) with indurated, raised, averted margins was encountered on the right side of the forehead. The ulcer was tender and had a fetid discharge. Necrotic slough was prominent over its base. Similar lesions were located on the left preauricular region and right half of the nose. hematoxylin and eosin-stained sections prepared from biopsies taken from representative lesions of epidermodysplasia verruciformis and squamous cell carcinoma revealed their diagnostic features. epidermodysplasia verruciformis showed features of hyperkeratosis and acanthosis with hardly any papillomatosis. Marginal elongation of the rete ridges was present. Extensive vacuolization was a remarkable feature, and was largely confined to the upper stratum Malpighian and the granular cell layer. Some of the vacuolated cells were fairly large; their nuclei were located in the center and had variable pyknosis. The granular cell layer was uniformly thickened, whereas the stratum corneum had a loosely felted (basket-weave) appearance. The dermis was apparently normal (Fig. 2b,c). The other microsection(s) from squamous cell carcinoma were conspicuous by the presence of hyperkeratosis, acanthosis, and irregular masses of epidermal cells, proliferating downwards and invading the dermis. Varying proportions of differentiated squamous cells formed their bulk. These cells were atypical, characterized by variations in size and shape, hyperplasia and hyperchromasia of the nuclei, absence of prickles, chas, characterization of individual cells, and the presence of both mitotic and atypical mitotic figures (Fig. 3b).- - - - - - - - - - ranking = 2keywords = nature (Clic here for more details about this article) |
7/31. Encephalofacial angiomatosis sparing the occipital lobe and without facial nevus: on the spectrum of sturge-weber syndrome variants?We report two cases of leptomeningeal angiomatosis in atypical frontoparietotemporal locations without an associated facial port-wine stain. Evidence of a leptomeningeal angioma was found in each when they were evaluated for headaches and seizures. The diagnosis of a leptomeningeal angioma was suggested by calcifications noted on computed tomographic scan of the head and confirmed with contrast-enhanced magnetic resonance images of the brain. We hypothesize that given the lack of occipital involvement with the angioma, and therefore the noncontiguous nature of this lesion with the developing upper facial ectoderm, the failure to develop a facial angioma would be expected. We found that the useof an anticonvulsant along with a migraine prophylactic medication appeared to have the greatest efficacy in these two cases, whereas anticonvulsants alone were less helpful. This diagnosis should be considered in any child presenting with seizures or complicated migraines and intracranial calcifications.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/31. Non-malignant perineural spread of epithelial tissue in the orofacial region.A 48 year-old male had two operations because of severe neuralgic pains in the region of the right infraorbital nerve. The first operative specimen consisted of connective tissue, striated muscle fibers and two myelinated nerve bundles, surrounded by a ring of well differentiated squamous epithelium. Initially the lesion was assumed to show perineural spread of cancer in this region. As the pain was not alleviated, a second operation was performed. The framework of the second specimen also consisted of connective tissue and muscle. It contained in addition an atheromalike cyst and some nerve bundles, ensheathed by well differentiated epithelial cells partly in a ring-, partly in a horseshoe-like pattern. The perineural epithelial sheaths could be traced to a rupture of the cyst, and to benign proliferation of its epithelial lining in the connective tissue and along the nerve bundles. Following the second operation the patient was relieved of his complaints. During the last five years he had repeated control examinations but no sign of malignant disease could be found; his state of health was perfect. Our finding of benign perineural spread of squamous epithelium has not been described previously. The nature of the so-called perineural lymph spaces is also discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/31. Multiple subclinical syringomatous proliferations encountered during mohs surgery for basal cell carcinoma.BACKGROUND: Examination of mohs surgery frozen sections may lead to discovery of incidental benign neoplasms such as syringomas. OBJECTIVE: An unusual occurrence of numerous subclinical syringomatous proliferations discovered during mohs surgery for a basal cell carcinoma that posed a diagnostic and management dilemma is reported. methods: Clinical records and histologic sections are examined. RESULT: Multiple syringomatous proliferations were noted around a basal cell carcinoma during mohs surgery. A few foci were atypical appearing and focally extending into the deep dermis causing a diagnostic dilemma. All atypical syringomatous proliferations were excised. Evaluation of final Mohs margins by permanent sections and biopsies of normal-appearing facial skin showed multiple benign syringomas. CONCLUSION: Although solitary subclinical syringomas have been described in association with basal cell carcinomas, the occurrence of multiple syringomas and syringomatous proliferations has not been previously reported. Dermatologists should be aware of the existence of this phenomenon and consider permanent section evaluation to better determine nature of unusual incidental tumors identified in frozen sections during mohs surgery.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/31. Microcystic adnexal carcinoma involving a large portion of the face: when is surgery not reasonable?BACKGROUND: We report a case of microcystic adnexal carcinoma (MAC) involving a large portion of the face, one of the largest of any MAC reported thus far in this area, and review the literature regarding the nature of the tumor and available treatments. We also review all of the reported cases of metastases and the possible role of radiation in the etiopathogenesis of this tumor. OBJECTIVE: To review the literature about what is known about therapy for MAC and what options are available to patients who have this disease. MATERIALS AND methods: Case report and review of the literature. RESULTS: Of the 274 cases of MAC thus far reported, there are 6 cases of metastases, only 1 of which resulted in death. CONCLUSION: mohs surgery should be the treatment of choice for this tumor; however, when extirpation entails sufficiently large morbidity, given the low rate of metastases and mortality, observation is a reasonable alternative.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
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