Isolated limb perfusion as a treatment option for rare types of tumours.

BACKGROUND: Isolated limb perfusion (ILP) is an established and effective treatment for advanced melanoma and soft tissue sarcomas of the extremities with a high overall response rate. The aim of this study was to describe our experience of ILP for more rare types of tumours. METHODS: Patients with Merkel cell carcinoma (MCC) (n = 4), squamous cell carcinoma (SCC) (n = 2), B-cell lymphoma (n = 1), desmoid tumours (n = 3), pigmented villonodular synovitis (PVNS) (n = 1) and giant cell tumour (n = 1) were treated with ILP and analysed retrospectively. RESULTS: The four patients with in-transit MCC had three complete responses (CR) and one partial response (PR); the two patients with SCC had one CR and one stable disease (SD); the patients with desmoid tumours had two PR and one SD. A CR was also observed for the patient with a giant cell tumour, but the patient with PVNS had a SD. The patient with cutaneous metastases of B-cell lymphoma showed a CR, however with rapid systemic progression. Local toxicity according to Wieberdink was grade II in 10 patients (83%) and grade III in two patients (17%). CONCLUSIONS: These results show that ILP can be used as a treatment option also for more rare disease entities when other treatments have failed.

[Polyps (single or multiple) and juvenile polyposis]. / Polipi (singoli o multipli) e poliposi giovanile.

The authors underline the important aspects of juvenile familial polyposis (JFP), a disease transmitted as an autosomal dominant trait. A case of JFP characterized by the presence of hundreds of polyps in the colo-rectal intestinal tract, is analyzed. The single juvenile polyp, multiple polyps (=/>5 polyps) and the sporadic form are examined. These are mucous hamartomas which can undergo neoplastic transformation (in carcinoma in 68% of untreated cases), a behaviour similar to that of adenomatous polyps. They differ from the later due to the following features: epidemiology (earlier appearance age), anatomopathology (stroma), clinical observation (self-recovery in some cases) and genetics (10q23.3-18q21, genetic mutations in a locus different those of adenomatous polyps). It is also necessary to determine its extension by means of colonoscopy, ileoscopy, gastroscopy and small bowel barium enema. Patients' screening through construction of the genealogical family tree is fundamental. Isolation of possible degenerative aspects of the polyps through biopsy is also fundamental. Single or multiple polyps are treated endoscopically, the juvenile polyposis is treated surgically (colectomy, total colectomy). A rigorous follow-up of the patients and their family members is recommended.

[The efficacy of temporary retention chemotherapy of the treatment of polyps in the remnant rectum of familial polyposis after total colectomy and ileorectostomy--a case report].

A case of familial polyposis is reported. The patient was a 24-year-old woman on whom total colectomy followed by ileorectostomy had been performed. However, there were multiple polyps remaining in the remnant rectum. For the treatment of these residual polyps, a new chemotherapeutic method known as Temporary Retention Chemotherapy (TRC), originally devised by us for the treatment of advanced gastric cancer, was applied. A balloon catheter was inserted and fixed into the rectum via the anus for the treatment of the rectal tumors. Through the catheter, high-dose 5-fluorouracil (5-FU) solution was injected into the rectum and retained there for one hour before being discarded. During the therapy, the patient was asked to change her position of recumbency at certain intervals, so that the 5-FU solution could act evenly on the lesions in question. This therapy was repeated twice a week, and followed by routine endoscopic studies. A total dose of 5,700 mg of 5-FU was necessary for obtaining a complete response of the remnant lesions. 6 months later, 4 polyps were found, and TRC was again applied, resulting in disappearance of the polyps again after the therapy. So far, no side-effect have been noted as a result of this therapy. She is currently still alive without any residual polyps, carcinoma or metastasis 6 years after the operation.