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R Giuli

Publications and source records attributed to R Giuli.

At least 19 recordsLinked to original sources
PubMed2001

Familial medullary thyroid carcinoma (FMTC). Study of one family (treatment criteria).

AIM: The nosology of familial medullary thyroid carcinoma (FMTC) has been described as a distinct pathology, genetically determined and with autosomal dominant transmission with a gene penetrance of almost 100%. The diagnosis of this morbid condition can be made if at least four members of the same family are affected by calcitonin-secreting C-cell carcinoma. METHODS AND RESULTS: We report the analysis of a family in which FMTC was diagnosed between 1993 and 1998. Of the five patients we confirmed as being affected by FMTC, we were able to perform a prophylactic thyroidectomy in only one case. The high possibility of lymph-node metastasis at the time of clinical diagnosis (52-75%), and the high morbidity and radio-chemo-resistance to adjuvant therapies, indicate total thyroidectomy with central lymph-node dissection. CONCLUSION: It appears that preventive lymphadenectomy does not substantially improve survival, while pre-clinical diagnosis is of greater importance than surgery in improving survival and preventing recurrence. Total preventive thyroidectomy has been recommended in all carriers of ret genetic defects, even in families at risk with mutations of the 618 or 620 codon, because the penetrance of FMTC approaches 100%, and a 100% accordance between presence of the disease and gene carrier status is reported. This procedure would therefore represent the only possibility of achieving a 100% cure in subjects affected by familial medullary thyroid carcinoma.

Adolescent↗
PubMed1992

[Endo-brachy-esophagus, Jean-Louis Lortat-Jacob and Barrett's esophagus. The history of a modern disease].

The term-endo-brachy-oesophagus (EBO) became part of the specialised vocabulary of all those interested in the pathology of the esophagus at the beginning of the 1950s, when J. L. Lortat-Jacob identified the existence, in certain patients suffering from reflux, of the endo-esophageal ascent of a mucosa of gastric type, without any change in the gross topography of the gastro-esophageal junction. A similar lesion had been described at this same time in Great Britain by N. R. Barrett. Since then, numerous clinical and experimental arguments have been advanced in support of the acquired origin of this disorder, in the context of progressive gastro-esophageal reflux disease. However, the possibility of its regression after the treatment of reflux and its histologic presentation, though of major importance, remain unsettled. It is now generally acknowledged that EBO is one of the digestive lesions most prone to malignant degeneration, to the extent that it is now generally classified as precancerous, and all the most sophisticated procedures have been currently suggested and exploited in the attempt to detect EBO of potential malignancy. Within this field, ultramicroscopic study, enzyme assays and the techniques of flow cytometry are being used to better define this problem and to specify the point after which it is legitimate to abandon the medical treatment of reflux in favor of more aggressive methods, even surgical resection of the esophagus. The OESO Group, created in 1978, is currently conducting an international multidisciplinary and multicenter study to deal with the epidemiologic and therapeutic problems of this disorder.

Barrett Esophagus↗
PubMed1986

Diagnostic, therapeutic, and prognostic features of cancers of the esophagus: results of the international prospective study conducted by the OESO group (790 patients).

The prospective study conducted by the Organisation internationale d'Etudes Statistiques pour les maladies de l'Oesophage (OESO) (International Organization for Statistical Studies of Esophageal Diseases) created in 1979 concerns 790 patients operated on for a tumor of the esophagus or the mouth of the esophagus. Among the preoperative investigations, endoscopic examinations were associated with a high error rate (20%). Respiratory function tests cannot constitute a formal contraindication to surgery. The value of extended surgical excision is confirmed by the results, although the need for extensive lymphatic resection has not been clearly demonstrated. The incidence of fistula has decreased, but it still remains an important element of operative deaths. However, the complications of esophagectomy are essentially pulmonary and the problem of preoperative identification of high-risk patients has not yet been resolved. The influence of various modalities of postoperative ventilation techniques were evaluated. The overall mortality rate was 14.7%, and a much broader definition of this term is required. Because of a number of contradictions in prognostic factors, the OESO group is currently testing a new clinical and histologic classification.

Combined Modality Therapy↗
PubMed1981

[Thymic seminomas (author's transl)].

The authors report on three cases of thymic seminoma treated between 1971 and 1981. These tumours, first described by Friedman in 1981. These tumours, first described by Friedman in 1951, belong to the group of extra-gonadal germinal tumours. They constitute about 2.5% of all thymic masses. The most probable pathogenic theory is abnormal migration of germinal cells from the vitelline sac to the embryonic thymus. Thymic seminomas are usually found in young men and are asymptomatic in 30% of the cases. Macroscopically, they present as solid tumours capable of invading the surrounding structures. Histologically, they resemble gonadal seminomas but are sometimes difficult to identify, which is unfortunate since treatment is dependent upon an accurate histological diagnosis. The authors suggest that the tumour should be biopsied under mediastinal fluoroscopy, so that an accurate histological diagnosis can be made. Treatment consists of surgical excision, which should be restricted and on no account should destroy important structures, completed by mediastinal radiotherapy. The mean survival time is 6.3 years; the 5-year survival rate is 75%.

Adult↗
PubMed1980

Treatment of carcinoma of the esophagus. Retrospective study of 2,400 patients.

A multicenter retrospective statistical study of 2,400 patients with tumors of the esophagus and cardia was undertaken. Study of individual sites revealed the operability of certain carcinomas of the upper third of the esophagus with a reasonable five-year survival rate despite macroscopic invasion seen in more than half the patients. For the middle third of the esophagus, intrathoracic colonic esophagoplasty with esophageal resection extending as high as possible appeared to offer the best long-term results, particularly if the anastomosis was performed in the neck. Tumors of the lower third of the esophagus were also associated with better results when the esophageal anastomosis was made at a level above the aortic arch, resulting in an improved survival rate for patients undergoing intrathoracic colonic esophagoplasties. For carcinomas of the cardia, use of total gastrectomy was superior to the use of upper polar gastrectomy, but the results were better when gastric excision was also associated with esophageal excision. The finding of normal lymph nodes did not preclude recurrence of the tumor in approximately one-fourth of the patients. Esophageal sections at a distance from the tumor was not necessarily synonymous with section in a healthy area, since the sites of sections studied were either invaded (29%) or areas of neoplastic repermeation (40%). Existence of a histologically normal esophageal section site did not preclude recurrences in 27% of patients with more than one-third in the esophagus. Undifferentiated or poorly differentiated squamous cell carcinomas paradoxically appeared to have a somewhat better long-term prognosis than well differentiated forms, but the increased number of metastases associated with them confirms their unfavorable prognosis. The importance of the T/N classification was confirmed for tumors in classes T1 and T2. From Stage T3, the N criterion was not important. Incidence of postoperative mortality from fistulas appeared to decrease progressively, chiefly due to appropriate medical treatment. Cervical fistulas were associated with a mortality rate of 21%. After esophageal anastomosis above the aortic arch was performed, more than 10% of the recurrences were seen in the neck, indicating the need for extension of the incision as high as possible. Metastases to the bone were present in 15% of the patients. Preoperative radiotherapy did not lessen the number of lymph nodes found to be invaded at the time of excision; the tumors considered to be histologically "sterilized" by irradiation were nevertheless associated with a high incidence of lymph node involvement (approximately one-third) and with more than a 40% rate of distant metastases. Excisions considered to be "palliative" by the surgeon nevertheless were of definite value. The mean survival rate at five years was 12%, and one-third of these patients showed no recurrence of neoplasm.

Adult↗