Aggressive intracranial fibromatosis: case report

Abstract

-Fibromatosis is a locally aggressive, proliferative fibroblastic lesion affecting musculoaponeurotic stru c t u res, most often in the limbs and trunk. Intracranial fibromatosis is extremely rare and re q u i re s a g g ressive treatment to prevent re c u rrence. Case Description -We present the case of a 20 year old woman with aggressive skull base fibromatosis. The lesion extended through the sphenoid, ethmoid sinus and nasal cavity, destroying the right roof of the orbit and penetrating in anterior skull base. A combined anterior craniofacial approach was perf o rmed; complete resection with surgical margin was impossible due to the localization of the tumor and relation to important neurovascular stru c t u res. Complete resection with s u rgical margin is often impossible because of its widely infiltrative nature. Radiotherapy and chemotherapy are often required to improve local control of the lesion.KEY WORDS: intracranial fibromatosis, skull base, craniofacial approach. Fibromatose agressiva Intracraniana: relato de casoRESUMO -Fibromatose é lesão fibroblástica proliferativa com agressividade localizada, afetando as estruturas músculo-aponeuróticas e acometendo, mais freqüentemente, os membros e tronco. A fibro m a t o s e intracraniana é rara e requer tratamento agressivo para evitar recidiva. Descrevemos uma paciente de 20 anos portadora de fibromatose agressiva de base de crânio. A lesão se estendia através da cavidade nasal, seios esfenoidal e etmoidal, destruindo o teto da órbita direita e invadindo a porção anterior da base do crânio. A paciente foi submetida a uma via craniofacial anterior combinada, não sendo possível uma ressecção completa devido ao envolvimento de estruturas neuro v a s c u l a res. A ressecção completa é, freqüentemente, impossível devido sua natureza infiltrativa. Radioterapia e quimioterapia são indicadas para melhorar o controle da lesão. PALAVRAS-CHAVE: fibromatose intracraniana, base do crânio, via craniofacial.

Gastric Perforations from Abdominal Trauma

“…Complementary diagnostic tests range from plain-film chest radiographs (signs of pneumoperitoneum or diaphragmatic hernia) or plain-film abdominal radiographs (signs of pneumoperitoneum), abdominal US (intra-abdominal free fluid or solid organ injury), CT (localization of gastric rupture, solid organ injury, intra-abdominal free fluid, active bleeding, pneumoperitoneum), diagnostic peritoneal lavage (turbid fluid or gastric contents), laparoscopy (direct visualization of the gastric perforation) or exploratory laparotomy (surgical findings) [3, 7, 8, 14,17,18,19,20]. …”

“…As far as treatment is concerned, in the first place, general life support and resuscitation measures must be employed on the multiple trauma patient [8]. The surgical treatment of gastric rupture runs from simple suturing after debriding the margins of the wound in the case of small or single lesions to partial gastrectomy in cases with extensive damage to the stomach [1, 6, 7].…”

“…Morbidity and mortality in patients with gastric rupture is related to associated lesions, predominantly respiratory and infectious complications [1, 3, 7, 8]. The mortality associated to gastric rupture ranges from 0 to 66% [1, 2, 6, 7].…”

“…Diverse factors may be involved, including fullness of the stomach from a recent meal, localization of the trauma site in the left abdomen and inappropriate use of safety belts [5,6,7]. Morbidity and mortality in this condition have been related to delayed diagnosis, abdominal contamination and associated lesions [3, 5, 6, 8, 9]. Mortality in the different series published ranges from 0 to 66%, however this depends more on the associated lesions than on gastric rupture itself [1, 6, 7].…”

Infantile Intracranial Aggressive Fibromatosis: Report of Two Cases with a Review of the Literature

“…Because of the absence of a capsule, intracranial AF may infiltrate into adjacent tissues, with some cases appearing well circumscribed on imaging studies [5,6,36]. When the periosteum is involved, bony erosions always occur.…”