Unusual Gigantiform Angiomatosis of Jawbone.

ABSTRACT: The purpose of this paper is to highlight rare highly infiltrative massive Angiomatosis and depict surgical outcome of a rare case series of gigantic lesions in the maxillofacial region. Data were recorded from the medical records of patients. Predictor variables were drawn from demographics, age, gender, site, evaluation of surgical treatment. The outcome variables were the challenges encountered and related complications. Out of 6 patients, the youngest was 10 and the oldest was 26 years old. Soft tissue and jawbone involvement were seen in 3 patients each with a single massive lesion involving both maxilla and mandible. All 6 patients had wide excision of the lesion with one patient having 2 stage procedures. No complications or recurrence was seen at 1-year follow-up. Angiomatosis is a rare and benign lesion in the head and neck region. its diffuse infiltrating nature may give a false malignant picture. it clinically mimics Hemangioma or Arteriovenous (AV) malformation thus requires thorough evaluation and its surgical intervention is challenging as described in this case series because of its magnitude and infiltrative nature.

Diffuse Reactive Angioendotheliomatosis Secondary to the Administration of Trabectedin and Pegfilgrastim.

Diffuse dermal angiomatosis is a rare benign condition considered a variant of reactive angioendotheliomatosis, usually related to vascular disease such as arteriovenous fistula or severe peripheral vascular disease. The most frequent clinical manifestations range from a solitary erythematous patch to an indurated plaque that may ulcerate. A clinical case of a 60-year-old woman who developed generalized livedoid lesions 2 days after the administration of intravenous trabectedin and subcutaneous pegfilgrastim for a recidivant myxoid liposarcoma has been reported. A biopsy of the skin lesions showed a pronounced proliferation of vessels in the upper dermis that was diagnosed as diffuse dermal angiomatosis.

Massive craniofacial intraosseous vascular malformation resembling cystic angiomatosis: report of 2 cases and review of the literature.

Cystic angiomatosis of bone is a rare condition of multifocal angiomas of the skeleton. The condition is believed to be congenital, grows slowly and starts in first decades of life. Two cases of progressive bimaxillary enlargement, presented here with a history of slowly enlargement of facial bones when they were 9 and 6 year old, respectively. Radiographic evaluation of the craniofacial bones revealed aggressive hypertrophy with severe displacement of the teeth. The histopathological evaluation of the gross specimen showed vital bone containing capillary and cavernous spaces with endothelial lining. Aggressive cystic angiomatosis of the facial bones was described here as the most probable diagnosis.

Cutaneomeningospinal Angiomatosis (Cobb Syndrome) in a Young Patient.

Cobb Syndrome (Spinal Arteriovenous Metameric Syndrome 1-31 (SAMS 1-31)) is a rare, non-hereditary disorder. Approximately 100 cases of CS have been described to date. The actual incidence may be much higher since only symptomatic patients were documented. In particular, post mortem studies suggest a possibly higher incidence of this syndrome. The main clinical manifestations of this syndrome include skin stains of vascular nature on the torso, in combination with spinal vascular malformations localized in one and the same metameric or spinal segment. A rare diagnosis of this syndrome in patients over 18 is probably related to the fact that the disease may be asymptomatic throughout a long period of time, while patients may tend to disregard the skin lesions. As a result, most publications on this pathology are based on separate case reports. Significant variability of clinical manifestations as well as prolonged progress of the disease often cause errors in diagnosis. What follows is a case report of a young patient with Cobb Syndrome, who was admitted to a regional vascular centre with a misdiagnosis of stroke. 20 patients of young age (from 20 to 35 years old), with a diagnosis of stroke, who were admitted to a University Clinic (of the Russian National Research Medical University Named After Pirogov N.I., Moscow). Among this group of patients, a patient with Cobb syndrome was identified. Patient P., of 22 years, presented with acute, intensive cervical spinal pain, predominantly on the right, numbness and weakness in the arms and legs. About 3 weeks before admission to the hospital, the patient had ARVI with a fever of up to 37.5°C: two weeks before the onset of symptoms, he had undergone extirpation of 2 teeth, for which reason he spent over 2 hours in a forced position with his head thrown back (prolonged overextension in the cervical spine). Multiple skin angiomas on the chest spreading to the shoulder and scapula region. Tetraparesis up to 4 points: tetraparesis in hands with low muscle tone, low reflexes, tetraparesis in legs with high muscle tone, high reflexes, foot clonus when causing Achilles reflexes, tremor in the extremities and no plantar reflex pathology were detected, sensitivity disorders in the hands "the high gloves" and no pelvic disorders were detected. Given the presence and exacerbation of neurological symptoms and cutaneous angiomas, MRI with a contrast agent of the cervical spine was recommended. MR-image of an advanced arteriovenous malformation (AVM) of the cervical spinal cord with signs of gliosis and spinal cord oedema at the C2-C7 level. Endovascular embolization of the AVM in the cervical spinal cord was performed. The treatment led to the complete reversal of neurological symptoms. In the presence of skin lesions, the diagnosis of CS does not present particular difficulties, so in children and young patients with skin angiomatosis, it is advisable to conduct a comprehensive examination using selective spinal angiography or MR angiography to exclude arteriovenous malformations in the spinal cord.

Telangiectasia and Pulmonary Arterial Hypertension Following Treatment With Trastuzumab Emtansine: A Case Report.

Trastuzumab emtansine (T-DM1) is a Food and Drug Administration-approved novel agent for the treatment of HER-2 positive advanced breast cancer. We report a case of pulmonary arterial hypertension (PAH) that we attribute to the use of T-DM1. A 43-year-old woman with stage IV breast cancer presented with dyspnea on exertion. After excluding other secondary causes of pulmonary hypertension, a diagnosis of moderately severe PAH was made based on right heart catheterization. History revealed that the patient had been on T-DM1 before presentation. During T-DM1 treatment, the patient experienced hereditary hemorrhagic telangiectasia-like symptoms consisting of spider angiomata-skin lesions, epistaxis, and hematochezia, which resolved with discontinuation of T-DM1. Temporal associations of T-DM1 use with the development of PAH in the patient, and the reported association between hereditary hemorrhagic telangiectasia and PAH via genetic linkage, led us to suspect T-DM1 as the cause of PAH.

Dental care challenges in Sturge-Weber syndrome: a case report / Desafios do tratamento odontológico na síndrome de Sturge-Weber: relato de caso

Introduction: Sturge-Weber syndrome (SWS) is a rare condition characterized by facial capillary malformation, involves ocular, neurological, and cutaneous alterations. Associated with unilateral characteristic port-wine stains, gingival growth and purple-red coloration. Aim: his case aims to report dental treatment challenges in patients with SWS and importance of oral health maintenance in these individuals. Case report: a 20-year-old woman with an established diagnosis of SWS, presented bad breath and spontaneous gingival bleeding, with gingival growth and reddish-purple spots spread to labial and alveolar mucosa, tongue, and palate. Conditioning of the patient's oral environment by supra and subgingival scraping, dental unit extraction was performed. A conservative treatment plan was adopted for management adequacy of oral environment owing to possible complications inherent to the condition. Conclusion: it is important to emphasize the importance of dental surgeon's performance in relation to a multidisciplinary health team, as well as cooperation of patient, to obtain better results from the proposed therapy.

Introdução: a síndrome de Sturge-Weber (SSW) é uma condição rara caracterizada por malformação capilar facial, envolve alterações oculares, neurológicas e cutâneas. Associada a manchas unilaterais características do vinho do porto, crescimento gengival e coloração vermelho-púrpura. Objetivo: este caso tem como objetivo relatar desafios do tratamento odontológico em pacientes com SSW e a importância da manutenção da saúde bucal nesses indivíduos. Relato de caso: paciente do sexo feminino, 20 anos, com diagnóstico estabelecido de SSW, apresentou mau hálito e sangramento gengival espontâneo, com crescimento gengival e manchas roxas avermelhadas espalhadas pela mucosa labial e alveolar, língua e palato. Condicionamento do ambiente oral do paciente por raspagem supra e subgengival, foi realizada extração da unidade dental. Foi adotado um plano de tratamento conservador para adequação do manejo do ambiente bucal devido a possíveis complicações inerentes à condição. Conclusão: é importante enfatizar a importância do desempenho do cirurgião-dentista em relação a uma equipe multidisciplinar de saúde, bem como a cooperação do paciente, para obter melhores resultados com a terapia proposta.

Cobb's syndrome and its treatment with embolization. Case report.

A case of Cobb's syndrome, which is characterized by spinal and vertebral angiomas and a skin nevus at the same metameric level, is reported. The spinal angiomas were treated with liquid embolization using ethylene vinyl alcohol.

Cystic angiomatosis of bone: a case report.

Diffuse skeletal cystic angiomatosis is an extremely rare disorder that has not previously been reported in detail in the dental literature. This case report deals with the oral manifestations in a 10-year-old boy. Clinical, radiographic, and computed tomographic examinations showed hyperplasia of the right side of the face. The computed tomographic imaging studies revealed the extent of the jawbone lesions and the adjacent hyperplastic soft tissues. The histologic appearance of the lesions, the course of the disease, and the differential diagnosis are discussed.

Management of a dental infection in a patient with Sturge-Weber disease.

A patient with Sturge-Weber disease was treated for a facial infection. Particular attention is given to oral histopathologic changes, and the special care needed for patients with this disease is outlined.

Angiomatous malformation of the floor of the mouth associated with fluctuating macroglossia in an adult: an unusual case report.

We report an unusual case of a large vascular lesion of the floor of the mouth of rapid onset in a 50-year-old man who presented with an external swelling in the submental region and a history of fluctuating swelling of the tongue. The clinical features, investigations, treatment and course of this rare condition are discussed.

Epithelioid angiomatosis affecting the oral cavity as a first sign of HIV infection.

Oral lesions are frequently the first manifestation of HIV infection and accurate diagnosis may be important in determining the management of individual patients. Lesions may be relatively common but non-specific, such as candidosis, or may be indicative of AIDS, such as Kaposi's sarcoma. Epithelioid angiomatosis is a recently described vascular lesion which may be clinically and histologically similar to Kaposi's sarcoma, but which is infective in origin. Usually it is a manifestation of AIDS and presents as multiple cutaneous lesions but has rarely been reported in the mouth. In this report a patient presented with lesions of epithelioid angiomatosis which were confined to the oral mucosa and which were associated with HIV infection, but not with AIDS. Differentiation from Kaposi's sarcoma is important, since epithelioid angiomatosis may be treated with antibiotics and a mistaken diagnosis of Kaposi's sarcoma may wrongly categorise an individual as having AIDS.

[Vascular neoplasms of the larynx, pharynx, and mouth cavity in children]. / Sosudistye opukholi gortani, glotki i polosti rta u detei.

41 children with vascular tumors of the larynx, pharynx and mouth cavity were examined in the hospital. Hemangiomas were found in 29, lymphangiomas in 11 cases, lymphemangioma in one child. The tumors had multiple location and in 22 patients were combined with dermal angiomatosis. The chief techniques of removing the above vascular tumors were cryosurgery and CO2-laser coagulation. Radiotherapy was applied in cases of extensive dermal angiomatosis. A good response was achieved in 37 cases, tumor growth persisted in one patient. One child died as a result of the disease progression, and one more child of an intercurrent disease.