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Alberto Fontana

Publications and source records attributed to Alberto Fontana.

4 recordsLinked to original sources
PubMed2003

Fetal rhabdomyomatous nephroblastoma: report of 14 cases confirming chemotherapy resistance.

The authors retrospectively reviewed their experience with 14 cases of fetal rhabdomyomatous nephroblastoma (15.6% of all renal tumors seen in the 1984-1998 period), analyzing the diagnostic and treatment approach as well outcome. Mean age at presentation was 27 months; 10 (71%) patients were younger than 36 months (5 of these were less than 12 months) at diagnosis. Three patients had bilateral tumors. One case was extrarenal. Nine patients received preoperative chemotherapy after fine-needle aspiration biopsy. The response was null in five children and mild in three; in the remaining one the tumor increased in size. The only patient showing greater than 50% tumor shrinkage was the one treated with radiotherapy. Postoperative treatment according to staging followed the SIOP protocol. In the follow-up (mean 59.5 months) of 12 patients, 6 were in stages 1-2: 4 are alive and free of disease and 2 died (one due to intractable local recurrence and the other in complete clinical remission). All six patients presenting in advanced stages died (four due to disease progression and two due to complications while in complete clinical remission). Patients with fetal rhabdomyomatous nephroblastoma presenting with distant metastatic disease and high stage have unfavorable outcomes, confirming data from the literature indicating that chemotherapy is not as effective as in other variants of nephroblastoma. New protocols are needed to overcome this situation, for which surgery appears the only truly effective option.

Age of Onset↗
PubMedDate not supplied

[Von Recklinghausen's disease and intestinal neurofibromatosis: a case report].

We report the case of a patient with von Recklinghausen's disease, who was admitted with a diagnosis of acute abdomen due to small bowel neurofibromatosis. The patient was submitted to an abdominal CT scan that showed a homogeneous round lesion, with a regular margin, probably belonging to the small bowel and with the appearance of a benign lesion that probably caused an intestinal intussusception. The patient was submitted to a surgical procedure that mainly consisted in multiple small bowel resections. The histopathological examination confirmed the benign nature of the lesions. About one third of patients affected by von Recklinghausen's disease present involvement of the bowel, but only 5% of them are symptomatic. The intestinal tumours are usually neurofibromas and are mainly localized in the jejunum. However, there have also been reports of stromal, nervous and endocrine tumours and even other tumours not belonging to these categories, including adenocarcinoma. The overall incidence of intestinal malignancy in patients with von Reckilnghausen's disease is about 10%. The surgical operation, as well as the histopathological and immunochemical examination of the intestinal lesions are of crucial importance for the treatment of the complications of intestinal neurofibromatosis and for the treatment and diagnosis of malignancy.

Abdomen, Acute↗
PubMedDate not supplied

Endocrine and immunological alterations during cancer processes.

An immunological deficit is often associated with cancer processes. Because cortisol has an important immunosuppressive activity and it is strictly involved in stress responses, we examined its serum levels in colorectal cancer patients and its relationships with peripheral lymphocyte counts. Cortisol circadian rhythm and total and CD4+ lymphocytes were determined in 40 patients. The rate of patients with an altered cortisol circadian rhythm was 28%, and it was significantly more frequent in patients with nodal involvement (P < 0.005) and with metastatic spread (P < 0.005). Patient ratio with total lymphocytes assessment decrease was 30%. There was no correlation between lymphocytopenia and alteration of cortisol circadian rhythm. Data suggest a significant adrenal glands rhythm modification during cancer processes, associated with poor prognostic factors. Cortisol levels and lymphocytopenia seem not to be related. The origins of these alterations are unknown. They may favor or result from cancer spread.

Adrenal Glands↗