Pseudomyxoma peritonei is a rare clinical condition that is characterized by the presence of mucinous ascitis. It is believed to originate predominately from a mucinous neoplasm of the appendix including a heterogeneous group of tumours ranging from indolent to malignant. It was first described in the late 19th century. Pseudomyxoma retroperitonei is extremely rare with only 33 cases having been reported since the first description in the middle of the 20th century. We report two additional cases of pseudomyxoma retroperitonei and present a review of the literature.

Familial adenomatous polyposis (FAP) is a rare syndrome characterized by the presence of hundreds to thousands of colorectal adenomas and is responsible for less than 1% of all colorectal cancers. The syndrome is also characterized by extra-colorectal features including amongst others upper gastrointestinal tract polyps and desmoid tumors. The syndrome is inherited by an autosomal dominant gene, the adenomatous polyposis coli (APC) gene. We present the physical history, clinical presentation, diagnosis and treatment of a patient with a novel germline APC mutation, the W421X mutation, which resulted in FAP presenting with about a hundred colorectal polyps, gastric hyperplastic polyps and multiple aggressive intra-abdominal and extra-abdominal desmoid tumors.

Peutz-Jeghers syndrome is a rare hereditary autosomal dominant disease caused by a mutation of the tumor suppressor gene serine/threonine kinase 11 located in chromosome 19p13.3. It is characterized by the presence of extensive mucocutaneous pigmentation, especially of the lips and the occurrence of hamartomatous polyps throughout the gastrointestinal tract. Gastrointestinal hamartomas occur predominantly in the small intestine and can become symptomatic leading usually to intestinal obstruction and abdominal pain. We present a case of recurrent intestinal obstruction caused by small bowel intussusception treated by reduction, enterotomy and polypectomy and followed by intraoperative enteroscopy and endoscopic polypectomy.

Geralmente se define o bócio mergulhante como o bócio em que a massa da tireoide descende ao plano da cavidade torácica ou mais do que 50% da massa tireoidiana se localiza abaixo da cavidade tireoidiana. O bócio mergulhante pode ser assintomático ou apresentar sintomas resultantes da compressão de órgãos adjacentes. A insuficiência respiratória aguda é rara nos casos de bócio mergulhante. Nos casos de bócio mergulhante sintomático, o tratamento indicado é cirúrgico, por tireoidectomia. Nesse relato, descreveu-se um caso raro de bócio atóxico mergulhante gigante negligenciado que resultou em insuficiência respiratória aguda e foi tratado com tireoidectomia de emergência com incisão em T.

Substernal goiter is usually defined as a goiter in which the thyroid mass has descended the plane of the thoracic inlet or if more than 50% of the thyroid mass is located below the thoracic inlet. Substernal goiters may be asymptomatic or may present with symptoms caused by compression of adjacent organs. Acute respiratory failure is rare in cases of substernal goiter. In cases of symptomatic substernal goiter the treatment is surgical by thyroidectomy. We present a rare case of a giant substernal nontoxic goiter which caused acute respiratory failure which was treated by urgent thyroidectomy through a T-incision.