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HEREDITARY HEMORRHAGIC TELANGIECTASIA: A CASE REPORT
OOOO Volume 129, Number 1 covered by healthy oral mucosa. Cone beam computed tomography showed coalescent and well-defined hypodense areas at the periapical region of teeth 11, 12, and 13, measuring 12 mm, with buccal cortical bone discontinuity. The diagnosis hypothesis of an odontogenic cyst was confirmed by microscopic examination after incisional biopsy. The lesion was enucleated and tooth 12 was removed. The surgical specimen analysis revealed multiple cystic spaces lined by a thin, nonkeratinized epithelium with plaquelike thickenings. Some clear cells were noticed. The cystic wall was of a fibrous connective tissue. The final diagnosis was of botryoid odontogenic cyst. After 9 months of follow-up, there are no clinical or radiographic signs of recurrence. SUPPORT: FAPEMIG.
HEREDITARY HEMORRHAGIC TELANGIECTASIA: A CASE REPORT. MARINA FERNANDES BINIMELIZ, DANIEL FALHEIRO, BRUNO TURELI, RICART GIL MACEDO, LUCIENE SILVEIRA and, CESAR W. NOCE Hereditary hemorrhagic telangiectasia (HHT) is a rare mucocutaneous disease, inherited by autosomal dominant trait. Its diagnosis can be established by the identification of 3 of the following criteria: (1) frequent episodes of epistaxis; (2) telangiectasias of the skin and mucosa; (3) arteriovenous malformations involving lungs, liver, or central nervous system; and (4) family history of HHT. The purpose of this study is to perform a case report of HHT, attended at a stomatology service. The patient was a 70-year-old female, with numerous vascular lesions affecting the oral mucosa, hands, and feet. Endoscopic analysis also showed telangiectasias in the gastric mucosa. The patient had anemia and frequent episodes of epistaxis, and also reported a family history of these manifestations, involving her father, sister, and children. The diagnosis was defined as HHT. The dentist should be aware of the different manifestations of HHT and consider it as a possible diagnosis for patients with various vascular lesions.
WHITE SPONGE NEVUS: CASE REPORT WITHOUT A FAMILY HISTORY. SABRINA OLIVEIRA VARELA, JOSEPH COTTA VIANA, ^ e VELLOSO, PATRICIA RONCON TANIA REGINA GRAO ^ BIANCHI, MARTHA ALAYDE ALCANTARA SALIM VENAN, DANIELLE RESENDE CAMISASCA and, LILIANA APARECIDA PIMENTA DE BARROS White sponge nevus (WSN) is a rare autosomal dominant disorder with variable expressivity and high penetrance. Oral mucosa is the most affected region. Usually, this entity occurs at birth or later in childhood or adolescence. A 42-year-old man complained of white, asymptomatic lesions, which were present for more than 3 years. No family history of similar lesions was reported. Intraoral examination revealed nonremovable, symmetric, diffuse white plaques, all over the buccal mucosa, bilaterally, with a corrugated surface. An incisional biopsy was performed and histopathologic analysis revealed stratified squamous epithelium, with hyperparakeratosis, an extensive area with acanthosis and keratotinocytes with perinuclear eosinophilic condensation. Based on clinical and microscopic aspects, the diagnosis made was WSN. Further material was collected for cytopathologic examination in order to confirm the diagnosis. Treatment is not necessary due to the benign nature of WSN. Correct diagnosis is
ABSTRACTS
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important to exclude other more serious lesions with similar characteristics.
BURKITT LYMPHOMA IN A PEDIATRIC PATIENT: A CASE REPORT. JOSE ALEXANDRE DA ROCHA CURVELO, DEBORAH SUTTER AYRES PEREIRA, JOSIANNE PEREIRA DE OLIVEIRA, ADRIANA MARTINS DE SOUSA, FABIO RIBEIRO GUEDES, MARCIA GRILLO CABRAL and, MARIA ELISA RANGEL JANINI Burkitt lymphoma (BL) is a very rare non-Hodgkin lymphoma with an extremely short doubling time that tends to affect children in the jaw. Here we report the case of a 6-year-old boy who visited the emergency service complaining of jaw pain, jaw swelling, and dental mobility for 20 days. Biopsy was performed and the diagnosis of BL was established. The patient was immediately admitted at the oncohematology center of a pediatric institute for treatment. Admission examination revealed generalized lymphadenopathy, sagittal sinus thrombosis, and tumor infiltration in the sphenoidal and maxillary sinuses, liver, lung, both kidneys, pancreas, and spinal canal, curving the spinal cord backwards. Treatment started immediately according to the nonHodgkin lymphoma Berlin-Frankfurt-Munster-95 (NHL-BFM95) protocol and currently, the patient is in complete remission without signs of disease. This report demonstrates that rapid and precise diagnosis with immediate treatment was essential for therapeutic effectiveness and should be considered for improved BL prognosis.
LOW-GRADE FIBROMYXOID SARCOMA OF THE MAXILLA. REPORT OF A CASE. RENE MARTINEZ FLORES, BERNARDO VENEGAS ROJAS, VICTOR MORAGA GOMEZ and, CARLO LOZANO BURGOS Low-grade fibromyxoid sarcoma (LGFS) is a variant of fibrosarcoma that commonly arises in the deep soft tissues of the lower extremities in young or middle-aged adults. It is characterized by high rates of local recurrence and late metastases, despite its deceptively benign image. We report the case of a 45-yearold man referred for a swelling of his left maxilla for 15 years’ duration. An incisional biopsy showed a proliferation of bland spindle and stellate-shaped cells, deposited in an alternating myxoid and fibrous stroma. Diagnosis of odontogenic myxoma and LGFS were proposed. A computed tomography showed an expansive and well-defined tumor in the left maxillary sinus and maxilla, without regional lymph node involvement or metastases. A complete resection of the tumor was performed and the diagnosis of LGFS was confirmed. Adjuvant treatment was not required and after 1 year follow-up, the patient remains free of disease.
EPSTEIN-BARR VIRUS POSITIVE MUCOCUTANEOUS ULCER IN A PATIENT PRESENTING WITH MONOCLONAL GAMMOPATHY. PATRICIA MARIA FERNANDES, FELIPE D’ALMEIDA, FRED LOUVEIRA AYRES, MARCIO AJUDARTE LOPES and, FABIO ABREU ALVES A 74-year-old man presenting with a gingival ulcer of 2 months’ duration was referred for evaluation. His medical history
HEREDITARY HEMORRHAGIC TELANGIECTASIA: A CASE REPORT. MARINA FERNANDES BINIMELIZ, DANIEL FALHEIRO, BRUNO TURELI, RICART GIL MACEDO, LUCIENE SILVEIRA and, CESAR W. NOCE Hereditary hemorrhagic telangiectasia (HHT) is a rare mucocutaneous disease, inherited by autosomal dominant trait. Its diagnosis can be established by the identification of 3 of the following criteria: (1) frequent episodes of epistaxis; (2) telangiectasias of the skin and mucosa; (3) arteriovenous malformations involving lungs, liver, or central nervous system; and (4) family history of HHT. The purpose of this study is to perform a case report of HHT, attended at a stomatology service. The patient was a 70-year-old female, with numerous vascular lesions affecting the oral mucosa, hands, and feet. Endoscopic analysis also showed telangiectasias in the gastric mucosa. The patient had anemia and frequent episodes of epistaxis, and also reported a family history of these manifestations, involving her father, sister, and children. The diagnosis was defined as HHT. The dentist should be aware of the different manifestations of HHT and consider it as a possible diagnosis for patients with various vascular lesions.
WHITE SPONGE NEVUS: CASE REPORT WITHOUT A FAMILY HISTORY. SABRINA OLIVEIRA VARELA, JOSEPH COTTA VIANA, ^ e VELLOSO, PATRICIA RONCON TANIA REGINA GRAO ^ BIANCHI, MARTHA ALAYDE ALCANTARA SALIM VENAN, DANIELLE RESENDE CAMISASCA and, LILIANA APARECIDA PIMENTA DE BARROS White sponge nevus (WSN) is a rare autosomal dominant disorder with variable expressivity and high penetrance. Oral mucosa is the most affected region. Usually, this entity occurs at birth or later in childhood or adolescence. A 42-year-old man complained of white, asymptomatic lesions, which were present for more than 3 years. No family history of similar lesions was reported. Intraoral examination revealed nonremovable, symmetric, diffuse white plaques, all over the buccal mucosa, bilaterally, with a corrugated surface. An incisional biopsy was performed and histopathologic analysis revealed stratified squamous epithelium, with hyperparakeratosis, an extensive area with acanthosis and keratotinocytes with perinuclear eosinophilic condensation. Based on clinical and microscopic aspects, the diagnosis made was WSN. Further material was collected for cytopathologic examination in order to confirm the diagnosis. Treatment is not necessary due to the benign nature of WSN. Correct diagnosis is
ABSTRACTS
e33
important to exclude other more serious lesions with similar characteristics.
BURKITT LYMPHOMA IN A PEDIATRIC PATIENT: A CASE REPORT. JOSE ALEXANDRE DA ROCHA CURVELO, DEBORAH SUTTER AYRES PEREIRA, JOSIANNE PEREIRA DE OLIVEIRA, ADRIANA MARTINS DE SOUSA, FABIO RIBEIRO GUEDES, MARCIA GRILLO CABRAL and, MARIA ELISA RANGEL JANINI Burkitt lymphoma (BL) is a very rare non-Hodgkin lymphoma with an extremely short doubling time that tends to affect children in the jaw. Here we report the case of a 6-year-old boy who visited the emergency service complaining of jaw pain, jaw swelling, and dental mobility for 20 days. Biopsy was performed and the diagnosis of BL was established. The patient was immediately admitted at the oncohematology center of a pediatric institute for treatment. Admission examination revealed generalized lymphadenopathy, sagittal sinus thrombosis, and tumor infiltration in the sphenoidal and maxillary sinuses, liver, lung, both kidneys, pancreas, and spinal canal, curving the spinal cord backwards. Treatment started immediately according to the nonHodgkin lymphoma Berlin-Frankfurt-Munster-95 (NHL-BFM95) protocol and currently, the patient is in complete remission without signs of disease. This report demonstrates that rapid and precise diagnosis with immediate treatment was essential for therapeutic effectiveness and should be considered for improved BL prognosis.
LOW-GRADE FIBROMYXOID SARCOMA OF THE MAXILLA. REPORT OF A CASE. RENE MARTINEZ FLORES, BERNARDO VENEGAS ROJAS, VICTOR MORAGA GOMEZ and, CARLO LOZANO BURGOS Low-grade fibromyxoid sarcoma (LGFS) is a variant of fibrosarcoma that commonly arises in the deep soft tissues of the lower extremities in young or middle-aged adults. It is characterized by high rates of local recurrence and late metastases, despite its deceptively benign image. We report the case of a 45-yearold man referred for a swelling of his left maxilla for 15 years’ duration. An incisional biopsy showed a proliferation of bland spindle and stellate-shaped cells, deposited in an alternating myxoid and fibrous stroma. Diagnosis of odontogenic myxoma and LGFS were proposed. A computed tomography showed an expansive and well-defined tumor in the left maxillary sinus and maxilla, without regional lymph node involvement or metastases. A complete resection of the tumor was performed and the diagnosis of LGFS was confirmed. Adjuvant treatment was not required and after 1 year follow-up, the patient remains free of disease.
EPSTEIN-BARR VIRUS POSITIVE MUCOCUTANEOUS ULCER IN A PATIENT PRESENTING WITH MONOCLONAL GAMMOPATHY. PATRICIA MARIA FERNANDES, FELIPE D’ALMEIDA, FRED LOUVEIRA AYRES, MARCIO AJUDARTE LOPES and, FABIO ABREU ALVES A 74-year-old man presenting with a gingival ulcer of 2 months’ duration was referred for evaluation. His medical history