[Malignant hereditary paraganglioma: problems raised by non-functional forms management]. / Paragangliomes malins héréditaires: problèmes liés à la prise en charge des formes non sécrétantes.

Non-functional paraganglioma have not clinical or biological characteristics, so that the diagnostic is most of the time delayed and made on the occasion of advanced abdominal tumor or symptomatic metastasis management. Hereditary forms, notably those with SDHB mutation, seem to have a poor prognosis. On the other hand, and on the oposite to sporadic forms, they are the only ones to benefit from genetic testing which make possible, if positive, an earlier diagnostic, before apparition of symptoms, recurrence or metastasis. We report a case of non-functional malignant hereditary paraganglioma diagnosed belatedly and we will consider management problems raised by non-functional forms.

Predictive factors for node involvement in papillary thyroid carcinoma. Univariate and multivariate analyses.

For patients with papillary thyroid carcinoma, lymph node involvement is a common complication, resulting in node dissection and its resulting morbidity. To determine means of limiting lymph node dissections, we attempted to define intra-operative criteria predictive of node metastasis and so identify the patients likely to benefit from this procedure. This retrospective study concerned 158 patients (118 female) treated between 1974 and 1996 for papillary thyroid carcinoma by total thyroidectomy associated with bilateral (central and lateral) (n = 119) or unilateral (n = 39) dissection. The following criteria were used to study the predictive value of node involvement: age, sex, tumour size, tumour site, uni- or multifocality, existence or not of a tumour capsule, existence or not of perithyroid involvement and presence or not of vascular invasion. 99 patients (63%) had node involvement. Four factors showed predictive value for node involvement in univariate analysis: vascular invasion (P = 0.02), male sex (P = 0.008), absence of a tumour capsule (P < 0.0001) and perithyroid involvement (P < 0.0001). Two factors were predictive in multivariate analysis: absence of a tumour capsule and perithyroid involvement. Our results enabled us to calculate the risk of node involvement for each patient as a function of the existence of a peritumoral capsule and/or perithyroid involvement and to determine the indication for dissection. When neither of these factors was present, the risk of node involvement was 38.3% and dissection was not considered essential. If both risk factors were found, the risk was 87.1% and dissection was considered necessary.

Percutaneous cholecystostomy for acute cholecystitis in critically ill patients.

BACKGROUND: Cholecystectomy remains the best treatment for acute cholecystitis but may cause high morbidity or mortality in critically ill or elderly patients. METHODS: We report a retrospective study of ultrasonography-guided percutaneous cholecystostomy (USGPC) performed between 1988 and 1994 in 41 patients (mean age, 77.8 years; range, 42-95 years) as an alternative to surgery. RESULTS: Five patients (12.2%) died in the hospital, four (9.8%) subsequently underwent operation without complications, six (15%) had a recurrence of cholecystitis between 3 and 24 months after withdrawal of drainage, and 26 patients are cured without recurrence after a mean follow-up of 33 months (range, 3-67 months). CONCLUSIONS: USGPC appears to be the treatment of choice for high-risk patients, especially those with postoperative cholecystitis, severe acute calculous pancreatitis, or total parenteral nutrition.

Percutaneous self-expandable metallic stents and malignant biliary strictures.

Thirty-five patients with malignant obstructive jaundice received palliative treatment using percutaneous self-expandable metallic stents. Cholangiocarcinoma was the most frequent cause of the biliary obstruction. In more than 50% of cases, the stricture was located in the hilum. Adequate biliary drainage was achieved in 97% of cases. Median survival was 182 days, and 11% of patients died within 30 days. Early complications occurred in 31% of patients, and 25% of patients showed recurrent jaundice after an average of 180 days. Percutaneous self-expandable metallic stents are an efficient means of palliatively treating malignant biliary strictures, particularly high biliary obstructions.

Thyroglobulin monitoring after treatment of well-differentiated thyroid cancer.

AIMS: The prognosis for well-differentiated thyroid carcinomas is favourable after treatment, but the rate of recurrence is around 20%. Cervical ultrasonography, radio-iodine scans, and monitoring of serum thyroglobulin (Tg) levels allow these recurrences to be diagnosed. The management of patients with isolated elevated Tg levels is controversial in the presence of negative radio-iodine scans. METHODS: The records of 57 patients diagnosed with recurrence of well-differentiated thyroid cancer were reviewed. Serum Tg was not evaluated in 31 of these patients (group 1) and measured in the other 26 cases (group 2). RESULTS: Forty-three recurrence sites were found; four deposits in the thyroid bed and 39 cervical metastatic nodes, with an average of five nodes per patient. The radio-iodine scan was accurate in detecting 10/24 of cases, radiology in 9/17, and elevated Tg levels in 20/25. Thirteen patients with recurrences diagnosed on the basis of Tg levels had negative radio-iodine scans. After surgery, Tg levels were normal in 10 patients from group 1 and 16 patients from group 2 (p=0.0078). CONCLUSIONS: Elevated Tg levels are indicative of disease progression or recurrence in patients who have previously been operated on for well-differentiated thyroid cancer. Even when the radiological study or radio-iodine scan is normal, surgical re-exploration of the neck, with total thyroidectomy and lymphadenectomy, is advisable.

Hepatic resections for non-colorectal metastases: forty resections in 35 patients.

BACKGROUND/AIMS: Hepatic resection, though now an accepted practice for colorectal primary tumors, is poorly documented for non-colorectal metastases. However, the few series reported suggest that this approach may lead to a significant increase in survival. METHODOLOGY: Study of 40 cases of resection in 35 patients with non-colorectal hepatic metastasis to define the role of hepatic resection between 1986 and 1997. RESULTS: Resection was performed for 5 metastases of ovarian and fallopian tube carcinoma, 8 gastrointestinal tract adenocarcinomas, 8 endocrine tumors, 8 sarcomas and 6 miscellaneous metastases, involving 17 lobectomies, 3 trisegmentectomies, 5 lateral segmentectomies and 15 non-anatomical local resections. Survival at 1, 2 and 5 years was 54 +/- 8, 42 +/- 8 and 27 +/- 8%, respectively. Hepatic metastases of gastrointestinal tract adenocarcinomas were found to have the poorest prognosis (median time: 13 months), and genital tract adenocarcinomas the best (27 months). CONCLUSIONS: Some carefully selected patients may benefit from liver resection for non-colorectal metastases.

Feasibility of radioimmunoguided surgery of colorectal carcinoma using indium 111 CEA specific antibody and simulation with a phantom using 2 steps targetting with bispecific antibody.

The study was undertaken to define the potential use of radiolabelled (Indium 111 or Technetium 99 m) carcinoembryonic antigen specific antibody (CEA f(ab')2) for the radioimmunodetection of colorectal cancer using an intraoperative hand-held gamma probe. A clinical study performed with ten patients showed that tumor with good uptake of CEA specific antibody could be detected with sufficient contrast only in two patients. Results of a biodistribution study performed with tumor fragment and normal tissue countings in a gamma counter showed high tumor uptake in five patients. There was no correlation between tumor uptake and the count rates measured intraoperatively. To increase the signal/background of the gamma probe, a simulation study with a peritoneal cavity phantom was performed. We determined the efficiency of a two steps targetting method compared to the direct method. We simulated different tumor sizes with plexiglas balls (0.5, 1, 2, 5 ml) and tested two scintillators (NaI, BgO). Experiments were performed with 111 In and 99 m Tc. The two steps targetting method was better than direct method. The results of simulation with direct method radiolabelled with 111 in confirmed our clinical study: no efficiency of a gamma probe for the surgeon to detect a tumor. However the two steps targetting method (indirect labelling method) was very encouraging to detect tumors (size 1 and 2 ml) and definitively convincing with 99 m Tc.

Laparoscopic hepatic surgery. Report of a clinical series of 11 patients.

BACKGROUND: Hepatic laparoscopic surgery is now possible, though its feasibility has not been demonstrated. METHODS: Eleven patients with benign hepatic lesions underwent laparoscopic surgery for different reasons (deroofing or excision of cysts, non-anatomical and anatomical resections of benign tumours). RESULTS: No deaths occurred, and morbidity was low. Conversion to laparotomy was not required, and none of the patients needed homologous blood transfusion. CONCLUSIONS: Hepatic laparoscopic surgery appears to be a safe and valid procedure for benign lesions but may not be suitable for all types of malignancy.

Sigmoid perforation, an exceptional late complication of peritoneal prosthesis for treatment of inguinal hernia.

Two cases of sigmoid perforation and fistula occurred as late complications after insertion of a nonresorbable prosthesis by the open preperitoneal inguinal route. These infrequent complications are favoured by peritoneal defects and use of materials which can cause extensive sclerous reactions. Indications for this type of mesh are increasingly common with the intraperitoneal laparoscopic approach, so that careful peritoneal dissection and closure are required.

Solid tumors of the liver. Diagnostic and therapeutic problems.

The diagnostic and therapeutic problems involved in 29 cases of isolated hepatic tumors have been presented. In nine cases the hepatoma was discovered during emergency treatment for complications. Selective arteriography was the most accurate method of establishing the etiologic diagnosis. Suprahepatic phlebography indicated the possibilities for surgical treatment. These possibilities are not influenced by the volume of the tumor, but are closely dependent on damage to the suprahepatic vascular system. Effective surgery was performed in 22 cases, of which 14 were excisions.

Study of survival and prognostic factors in patients undergoing resection for gastric linitis plastica: a review of 86 cases.

A retrospective study was conducted in a series of 86 patients (51 men and 35 women; mean age 63.4 years) treated from 1979 to 1995 for linitis plastica of the stomach (LP). The mean interval between the first manifestations and surgery was 3.5 months. The most frequent clinical sign was epigastric pain which occurred in 80.4% of cases. Biopsies were positive in 75.6% of cases. Typical features of LP were found in only 46% of esogastric barium enemas and 11.8% of upper gastrofiberscopic examinations. Seventy-four patients had surgical excision (51 total and 23 partial gastrectomies). There were 6 (7%) postoperative deaths and 10 (11.6%) surgical complications. Node involvement was found in 54 (72.9%) patients. Overall actuarial survival (n = 86) was 50% at 12 months, 40% at 18 months and 7.5% at 84 months. Survival did not depend on the delay in diagnosis, histological analysis of the extremities of the excised piece, associated tissue differentiation, node involvement or the type of surgical excision. The prognosis differed according to tumor height (P<0.01) and involvement of the deep stomach wall (P<0.001). No independent prognostic factor was found in multivariate analysis. Surgery remains the sole possibility for curative therapy in these patients.

[Clinical characteristics and evolution of permanent digestive ostomies. Results of a nation-wide retrospective survey]. / Caractéristiques cliniques et évolution des stomies digestives définitives. Résultats d'une enquête rétrospective nationale.

Factors influencing the achievement and the evolution of permanent stomas were determined during a National Inquiry including 1,082 stomas (978 colostomies and 104 ileostomies). This study, although not exhaustive, was representative, giving its statistical value. The principal concepts are well known with regard to the indications of colostomies (cancer) and ileostomies (inflammatory bowel diseases), to age (older in patients with colostomies: 63.8 vs 41.7 years, P < 0.001) and to the unequal geographical distribution of ileostomies (most frequent in northern provinces: P = 0.02). Frequency of complications (25%) after definitive stoma have decreased since 1985 (P = 0.01) but reoperation was necessary in 50% of cases. Most patients were informed of their stoma before the operation, but 23% were not. Among the informed patients, one fourth considered themselves insufficiently acquainted with their stoma.

[Hormone immunocytochemical studies of 46 endocrine tumors of the pancreas in 24 patients]. / Etudes immunocytochimiques hormonales de quarante-six tumeurs endocrines du pancréas chez 24 malades.

The aim of this retrospective study was to correlate the results of hormonal immunocytochemistry of 46 endocrine tumors to the corresponding clinical syndromes in 24 patients. They were divided as following: 14 cases of insulinoma, 3 cases of Zollinger-Ellison syndrome, 1 case of glucagonoma, 1 case of carcinoid syndrome and 5 cases without any obvious endocrine manifestations. Each tumor was tested with anti-insulin, anti-glucagon, anti-pancreatic polypeptide, anti-vasoactive intestinal peptide, anti-gastrin immune sera according to the peroxidase-antiperoxidase method. The presence of insulin was proved in 13 of 14 cases of insulinomas and the presence of gastrin in 2 of 3 cases of Zollinger-Ellison syndrome. Among the 5 asymptomatic cases, a somatostatinoma and a vipoma were individualized. More than 50 p. 100 of the tumors showed plurihormonal secretion with one predominantly secreted hormone responsible for the clinical syndrome. This study demonstrated the diversity of the hormonal secretion by some tumors and their metastasis in the same patient. Malignant insulinomas correspond either to poorly secreting tumors or to plurihormonal tumors secreting gastrin and glucagon as well.

["Mixed' (follicular and parafollicular) carcinomas of the thyroid. Histological and immunocytological study of 5 cases]. / Les carcinomes "mixtes" (vésiculaires et paravésiculaires) de la thyroïde. Etude histologique et immunocytochimique de 5 cas.

We studied five cases of poorly differentiated follicular or papillary thyroid carcinomas. Immunohistochemical study revealed numerous ACE positive cells, also positive for calcitonin, ACTH, somatostatin or several of these peptides. These tumors containing both vesicular component and parafollicular cells are endocrine tumors of "mixed" or "intermediate" type. The diagnosis must be confirmed by immunohistochemistry but can be suggested by histological findings: abundant fibrous stroma, trabeculovesicular pattern, and swelled moderately acidophilic cells neighbouring vesicular cells. These facts argue in favor of a common-embryological origin of vesicular and parafollicular cells from ultimobranchial undifferentiated cells. Nevertheless such tumors must take place in thyroid neoplasia's classifications and an appropriate terminology remains to be precised.

[Pathologic study with immunohistochemistry of 61 pancreatic endocrine tumors in 16 patients suffering from multiple endocrine neoplasia type I (MEN I). Review of the literature]. / Etude anatomopathologique avec immunohistochimie de 61 tumeurs endocrines pancréatiques chez 16 patients atteints de néoplasies endocriniennes multiples de type I (NEM I). Revue de la littérature.

The Multiple Endocrine Neoplasia (MEN I) or Wermer's syndrome is an uncommon disease which is most often inherited and affects mainly parathyroid glands, pancreatic islets and pituitary gland. The aim of this study concerning 61 pancreatic tumors in 16 patients suffering from MEN I was to define the macroscopic, histological and immunohistochemical characteristics of these tumors. The pancreatic endocrine tumors as part of the MEN I syndrome concern multiple tumors of small size, localized most often to the pancreas's tail. In 79% of cases, these tumors have a different predominating peptidic hormonal secretion in a same patient though most of them have plurihormonal secretions. The pancreatic polyendocrinopathy detection imposes a family investigation to look for a type I polyendocrinopathy.

[Locoregional recurrence of differentiated thyroid cancers: diagnosis-treatment]. / Récidives locorégionales des cancers différenciés de la thyroïde: diagnostic-traitement.

AIM OF THE STUDY: To evaluate the improvement of the diagnosis and the treatment of local recurrences (LR) in patients with differentiated thyroid carcinoma. MATERIAL AND METHOD: Among a total of 57 patients, two groups were compared: group I: 31 patients operated on from 1974 to 1990; group II: 26 patients operated on from 1991 to 2000. In the group I, the diagnosis of the cervical recurrence was supported by imaging study (ultrasonography, tomodensitometry), in the group II by radioiodinescan and serum thyroglobuline (Tg) measurement. The main difference was the consideration of Tg measurement to detect the recurrence in the group II. A high level of Tg was the only abnormality for 9 patients of the group II. RESULTS: A nodal recurrence was respectively present in the group I and II in 88.8% and 92% of the cases. Re-operation consisting in thyroid totalisation and bilateral lymphadenectomy was respectively performed in 71% and 100% of the cases. Surgery associated with iodine 131 therapy was respectively the treatment for 45.1% and 88.4% of the cases. After a median follow up of 66.2 months; results of the group I were as follow: normal or undetectable Tg: 10 (33.3%), second or more cervical recurrences: 7, distant metastases: 11, death in relation to thyroid cancer: 11. After a median follow up of 36.3 months, results of the group II were as follow: normal or undetectable Tg: 17 (65.4%), second or more cervical recurrences: 6, distant metastasis: 5, death in relation to thyroid cancer: 1. The best results concerned patients with an isolated elevated Tg without anatomical location of the first LR. CONCLUSION: LR diagnosis is difficult and needs imaging study, radioiodine-scan and serum Tg determination together. Re-operation associated with radioiodine-therapy is the treatment of choice. Elevated serum Tg is suffisant to indicate re-operation even if no anatomical substrate is found. Iodine-radiotherapy alone is generally unable to obtain undetectable serum Tg.

[Hepatic resections for non-colorectal, non-neuroendocrine metastases. Results of 32 hepatectomies in 27 patients]. / Résections hépatiques pour métastases non colorectales, non neuroendocrines. Résultats de 32 hépatectomies chez 27 patients.

STUDY AIM: Liver resections for metastases are commonly performed in colorectal primary tumors and poorly documented in non colorectal tumors. The aim of this study was to report a series of 32 liver resections in 27 patients for different types of non colorectal, non neuroendocrine liver metastases. PATIENTS AND METHOD: From 1986 to 1997, 27 patients (20 women and 7 men, mean age: 56.8 years) were operated on in the same center for liver metastases. Initial cancer was female genital tract (ovarian and fallopian tube) adenocarcinomas (n = 5), gastrointestinal tract adenocarcinomas (n = 8), sarcomas (n = 8), and miscellaneous cancers (n = 6). Liver resections included atypical resections (n = 9), right hepatectomies (n = 11), extended right hepatectomies (n = 2), left hepatectomies (n = 4) and resections of 2 or 3 segments (n = 6). RESULTS: There was no perioperative death. Postoperative morbidity included 8 complications in seven patients, requiring reintervention in three patients. Follow-up was complete for all patients. Survival rate at one, two and five years was 59, 44 and 29% respectively. The longest median survival time was observed in genital tract adenocarcinomas (27 months), whereas the other types of malignancies had a 13- to 17-month mean survival rate. CONCLUSION: These results are almost similar to those observed in liver resections for colorectal metastases. Some carefully selected patients may benefit from liver resection for non colorectal, non neuro-endocrine metastases.

[Parathyroid cyst. Report of ten cases]. / Les kystes parathyroïdiens. A propos de dix observations.

AIM OF THE STUDY: To evaluate the characteristics of the parathyroid cysts (PC). PATIENTS AND METHOD: Ten patients with PC were included in this retrospective study. The PC were discovered as follows: cervical mass (n = 3), hyperparathyroidism (n = 3), incidentally during thyroid surgery (n = 3) and screening for obesity (n = 1). Intracystic parathormone determination was performed after fine needle aspiration in 2 cases. RESULTS: Mean cyst measurements were 27 mm (ext: 5-70 mm) to 22 mm (5-45 mm). Nine cysts were cervical (resection by cervicotomy), and one was mediastinal (resection by sternotomy). In addition to the resection of the PC, 3 adenomas, 1 hyperplasia of the parathyroid glands and 3 benign thyroid diseases were recognized and treated during the cervicotomies. CONCLUSION: The diagnosis of PC is not common and must be based primarily on the study of the cyst liquid obtained by percutaneous puncture (intracystic parathormone measurement).

[Leiomyosarcomas of the rectum. Amputation of the rectum or local resection?]. / Les léiomyosarcomes du rectum. Amputation du rectum ou exérèse locale?

Two cases of high grade leiomyosarcoma of the rectum treated by local excision are reported. The first patient presented a local recurrence associated with liver and pulmonary metastases and eventually died, respectively 11 and 22 months after resection. The second patient's tumor recurred 3 months after excision and was treated with pelvic exenteration and CYVADIC regimen as chemotherapy and died 3 years later from local recurrence. A review of the literature indicates that local surgical excision in comparison to a more radical surgical approach, such as abdominoperineal resection, is as effective in improving survival. Lesions less than 2.5 cm in diameter, confined to the bowel can be treated by local surgical excision. To date no adjuvant therapy is effective in terms of survival or local control.

[Stomach leiomyoblastoma: contribution of cytology. Apropos of a case]. / Léiomyoblastome gastrique: apport de la cytologie. A propos d'un cas.

The authors report the case of a 51-year-old man with gastric leiomyoblastoma, presenting with a history of gastrointestinal bleeding and hemoperitoneum. CAT scan showed a mass measuring 10 x 10 cm arising from the stomach. At laparotomy the mass extended into the gastrosplenic omentum. A splenopancreatectomy was performed and the mass was excised for biopsy. The cytologic findings suggested a mesenchymal tumor; histology demonstrated the diagnosis of leiomyoblastoma. The postoperative course was uneventful. In the literature there is no evidence that intraperitoneal bledding from these tumors worsens survival. The cytopathologic features of leiomyoblastoma are discussed.