Perianal mucinous adenocarcinoma arising from a chronic fistula-in-ano.

Mucinous adenocarcinoma arising in a long-standing anorectal fistula is rare in clinical practice. Evidenced-based guidelines for treatment are lacking due to the rarity of this tumour. We present the clinical case of an advanced perianal mucinous adenocarcinoma associated with a longstanding perianal fistula, successfully treated by extended -abdomino-perineal resection, reconstructed by bilateral myocutaneous gluteus flaps, without any adjuvant therapy.

Appendiceal diverticulitis: a rare cause of right iliac fossa pain syndrome.

Appendiceal diverticulitis is a rare pathologic entity that usually simulates acute appendicitis. However, it can present as a separate clinical entity that should be part of the differential diagnosis of right lower quadrant abdominal pain. Pre-operative diagnosis is rarely described but is important in preventing perforation and subsequent abdominal sepsis. We present a case of an atypical right iliac fossa pain syndrome caused by histologically proven appendiceal diverticulitis, pre-operatively diagnosed by computed tomography.

Gardner syndrome and desmoid tumors.

Desmoid tumours present difficult management problems in patients with Gardner's syndrome. We recently studied two patients with Gardner's syndrome, who developed a desmoid tumour arising of the abdominal wall and mesenteric root. One patient had a total resection of the mesenteric desmoid tumour followed by postoperative radiotherapy. No recurrence occurred in the last three years. The other patient had an incomplete resection and refused postoperative radiotherapy. Abdominal CT scan revealed tumour expansion 6 months postoperatively. From our experience and with respect to current literature, we suggest that complete surgical excision combined with radiotherapy (4.000-6.000 rads) could diminish the recurrence rate of desmoid tumours. When resection is incomplete or technically impossible, radiotherapy remains the second choice of treatment.

Treatment of high-output gastric fistulas with omeprazole.

Two patients with high-output gastrocutaneous fistulas were treated with total parenteral nutrition and gastric antisecretory drugs. IV administration of omeprazole decreased acid output in one patient in a rapid and significant way, resulting in the spontaneous healing of the fistula after eight days of treatment. In the second patient, omeprazole caused a marked reduction in the acidity of the fluid in the fistula, which also closed without surgical operation. The long-standing decrease in acid output induced by intravenous omeprazole may be very useful for promoting spontaneous closure of high-output gastrocutaneous fistula.

Restoration of colorectal continuity reverses atrophy in human rectal mucosa.

Cell kinetic activity and adaptive response of rectal mucosa from patients with Hartmann's procedure were studied before and after restoration of colorectal continuity. Patients without colostomy and with normal rectal mucosa were used as controls. Autoradiography of in vitro labeled mucosal samples with [3H]thymidine was used. The proliferative activity in the rectal crypts was estimated by measuring labeling and mitotic indices, total DNA of isolated crypts, and total crypt cell numbers. One hundred forty days after creating a proximal end colostomy, labeling index (P less than 0.05), mitotic index (P less than 0.01), DNA content per crypt (P less than 0.05), and number of cells per crypt (P less than 0.05) decreased significantly compared to control values. Restoration of colorectal continuity resulted in a significant increase of the labeling index (P less than 0.05), the mitotic index (P less than 0.01), the DNA content per crypt (P less than 0.05), and the cell number per crypt (P less than 0.05). There were no significant differences between the postclosure values and the controls. These data indicated that excluding the human rectal mucosa from fecal stream determined a mucosal atrophy that could be reversed by restoration of colorectal continuity.

Surgical treatment of a retroperitoneal neurofibroma in von Recklinghausen's disease.

The rare occurrence of a retroperitoneal neurofibroma in a patient with von Recklinghausen's disease is presented. Malignant degeneration of a benign neurofibroma is a common threat in neurofibromatosis and cannot be definitely excluded by actual means. Therefore complete surgical removal of the retroperitoneal mass, even in asymptomatic patients, is advocated.

An unexpected cause of pathologic hip fracture. Malignant fibrous histiocytoma.

We describe a case of a 68-year-old patient presenting with pathological hip fracture and multiple pulmonary metastases, who has been operated without a prior histological diagnosis. The hip lesion was thought to be a metastasis of an unknown primary tumor. The proximal part of the femur was resected and replaced by a Müller mega-prosthesis. Histological analysis of the resected bone revealed a malignant fibrous histiocytoma, a rare but very aggressive bone tumor. The patient died three weeks after operation of widespread metastases. By presenting this case report, we want to stress the importance of pretreatment histological diagnosis of osteolytic bone lesions in older patients with metastases.

Femorofemoral bypass grafting in high-risk patients.

A total of 37 extra-anatomic femorofemoral bypasses were inserted for severe unilateral iliac artery stenosis or a thrombosed graft limb of an aorto-bifemoral bifurcation graft. All patients were severely debilitated and at high risk for direct aorto-iliac reconstruction. 30% suffered severe claudication; 70% had ischemic restpain or trophic lesions. 43% died during a mean follow-up period of 17 months. This study demonstrates that the extra-anatomic femoro-femoral bypass procedure is an effective alternative therapeutic modality for high-risk patients with an acceptable operative mortality (5.4%) and morbidity to improve the quality of life.

Agarose electrophoresis and inhibitor tests for isoamylase determination can give complementary clinical information.

We report the presence of an extremely high proportion of "aged" amylase in the serum and cyst fluid of a patient with a pancreatic pseudocyst. A salivary amylase inhibitor test helped us to differentiate these "aged" pancreatic amylases from salivary fractions having a similar electrophoretic mobility.