[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.

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.

[The importance of lymph node dissection in medullary thyroid macrocarcinomas]. / Importance du curage ganglionnaire dans les macrocarcinomes médullaires de la thyroïde.

UNLABELLED: Medullary thyroid carcinoma (MTC) is often regarded as good medium-term forecast. The 5- and 10-years survival rates are, respectively, appraised at 78-85% and 70-78%. These rates take no care, however, of the fact that 50-56% of the patients keep a pathological calcitonine (CT) level giving evidence of an evolutive disease. The treatment is based on the total thyroidectomy and cervical lymphadectomy. This treatment remains often incomplete and the results of reintervention are disappointing. AIM OF THE STUDY: About 48 patients, we wanted to demonstrate the importance of a complete lymph node dissection performed in the neck as soon as possible. PATIENTS AND METHODS: Between 1979 and 2000, 48 patients were treated for macroMTC (size >1 cm). The duration of follow-up was of 1-29 years (mean 9.3 years). The complete (central and lateral) neck dissection was initially made only in 22 cases. The selected criterion to assess the result was the normalization of the basal CT level. RESULTS: The rate of node involvement was 66.6% if the complete lymphadectomies (n = 22), the secondary neck dissections (n = 15), the incomplete (n = 10) and not made lymphadectomies (n = 2) were gathered. In case of primary or secondary complete lymphadectomies, the rate of node involvement was 81%. The 22 primary complete lymphadectomies performed in 13 patients (59%) allowed to normalize the basal CT level and among 17 (77.2%) to decrease this rate over 90%. All the incomplete neck dissection failed in case of positive nodes. CONCLUSION: The frequency of node involvement in macroMTC is about 80%. It does not have a preferential territory and the bilaterality is frequent (28-49%): that justifies a bilateral complete neck dissection. Initial surgical treatment seems essential in regard to the rate of normalization of basal CT level, which is, when a first complete lymphadectomy is done and in case of iterative surgery, respectively 59 and 26.6%. A complete lymphadectomy is still too rarely carried out: 22 times (45.8%) in our own experience and from 14 to 42% in the literature.

[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).

[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.

[Duodenal somatostatinoma. Anatomic/clinical study of 12 operated cases]. / Le somatostatinome duodénal. Etude anatomoclinique de 12 cas opérés.

STUDY AIM: Duodenal somatostatinomas (DS) are very rare neuro-endocrine tumours. The aim of this retrospective and multicentric study was to report the clinical and pathological characteristics of these neoplasms in a series of 12 patients and to compare them with the literature. PATIENTS AND METHODS: From 1987 to 1998, 12 patients were operated for a DS. There were seven women and five men ranging in age from 23 to 72 years (mean age: 56.6 years). Four patients had an associated von Recklinghausen's disease, one of them with multiple endocrine neoplasia (MEN type IIa) and medullary carcinoma of the thyroíd. The surgical procedures were pancreaticoduodenectomy (n = 8), small bowel resection (n = 2), inferior gastrectomy (n = 1) and gastrojejunostomy with hepatic metastases biopsies (n = 1). The tumour was mainly located on the 2nd duodenum (n = 10), with a mean size of 2.7 cm (ranging from 0.4 to 6 cm) and with a pancreatic invasion in three patients. A metastatic disease was present at the time of diagnosis in eight patients. There were, according to Capella's classification, two patients in the groups I and II, and ten patients in group III (83%), respectively. RESULTS: There was one postoperative death after a pancreaticoduodenectomy. Three patients secondarily died from tumoral progression. Eight patients were alive, with a mean follow-up of 84 months (ranging from 5 to 290 months), at the end-point of the study. CONCLUSION: Duodenal somatostatinomas are rare neuroendocrine, generally non-functioning, well-differentiated tumours with a low grade of malignancy. The association with the von Recklinghausen's disease is frequent. The clinical somatostatinoma syndrome with diabetes, diarrhea and biliary lithiasis is rare. The treatment is surgical even with a metastatic disease. The 5-year survival rate is better than those of the pancreatic somatostatinomas or the duodenal gastrinomas.

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.

[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.

Multicentre study of thyroid nodules in patients with Graves' disease.

BACKGROUND: The association of Graves' disease with thyroid nodules and thyroid carcinoma is rarely reported. The incidence seems to be increasing according to recent literature. The aim of this multicentre study was to review patients who had surgery for Graves' disease associated with thyroid nodules, and to evaluate the risk of thyroid carcinoma. METHODS: A retrospective study was made of 557 consecutive patients who underwent operation for Graves' disease between 1991 and 1997 in five endocrine surgery departments. Each patient underwent clinical, biochemical, ultrasonographic and scintigraphic evaluation. None of the patients had had previous radioactive iodine therapy or external irradiation. Surgery consisted of either a subtotal or total thyroidectomy. RESULTS: Nodules were observed before operation in 140 patients (25.1 per cent). Thyroid carcinoma was diagnosed in 21 patients (15.0 per cent), always inside a nodule. The incidence of thyroid carcinoma associated with Graves' disease was 3.8 per cent (21 of 557 patients): 20 papillary and one follicular carcinoma. The carcinoma was multifocal in two patients. Tumour diameter ranged from 2 to 25 mm. A nodule was palpable in four patients. CONCLUSION: This multicentre study of patients having thyroidectomy for Graves' disease showed that 3.8 per cent had a carcinoma; the rate of carcinoma in cold nodules was 15.0 per cent. Surgery should be advised in any patient with Graves' disease and a thyroid nodule; the operation should be total thyroidectomy.

Prognostic and immunohistochemical validation of the capella classification of pancreatic neuroendocrine tumours: an analysis of 82 sporadic cases.

AIMS: To study the clinical outcome of 82 cases of pancreatic neuroendocrine tumours classified according to the recent histological and prognostic classification of Capella. METHODS AND RESULTS: Eighty-two surgical cases of pancreatic neuroendocrine tumours were examined histologically with immunohistochemical staining of paraffin sections using streptavidin-biotin complex and application of antibodies against chromogranin A and 10 hormonal peptides. Classification in four groups correlated with long follow-up and outcome of these cases. Histological examination showed 30 group I, four group II, 41 group III and seven group IV tumours. Twenty-one (70%) of group I tumours were insulinomas, whereas 25% of group III tumours were glucagonomas and 25% were unclassified. Most group IV tumours were unclassified, showing no immunohistochemical staining with any of the 10 hormonal peptides tested. Outcome was clearly correlated with tumour group. Among the 14 patients who died of the disease, four had group IV and 10 group III tumours. Thus, unclassified asymptomatic tumours without immunohistochemical staining had a poorer prognosis than asymptomatic tumours with staining. CONCLUSION: This study validates the Capella classification as easy to apply and useful in predicting clinical outcome.

[Outcome in cervical recurrences of papillary or follicular thyroid cancer]. / Devenir des récidives cervicales de cancer papillaire ou vésiculaire de la thyroïde.

The aim of this study was to evaluate the treatment and outcome of patients with local recurrence (LR) of differentiated thyroid carcinoma. This retrospective study concerned patients treated between 1974 and 1990 for papillary or follicular thyroid cancer. Our patients had at least one LR. LR diagnosed within 6 months after thyroidectomy and patients with increased serum thyroglobulin levels were excluded. Thirty one patients (80% female) aged 15 to 84 years had at least one LR. LR was diagnosed 7 to 200 months after thyroidectomy (mean 63.7). There were 25 papillary and 6 follicular cancers. There were 1.5 LR per patient (range 1-6). LR were treated by radioiodine in 21 cases and by surgery in 22 cases. Among the 22 surgically treated patients, 7 had nodal recurrences, 7 had nodes and tumor, 3 had only tumor, 1 had recurrence in the remnant thyroid. After a mean follow-up of 75.8 months, 11 patients had distant metastases, 11 had died from their thyroid carcinoma (7 after metastases). Three of the 7 patients with nodal recurrence died. In one third of cases, LR announced distant metastases. Node recurrence had a poor prognosis.

Localization of cervical node metastasis of papillary thyroid carcinoma.

Precise localization of cervical node metastasis of papillary thyroid carcinoma is rarely described. The aim of this retrospective study was to map their cervical involvement. Between 1974 and 1996 a series of 119 patients had total thyroidectomy with bilateral cervical lymph node dissection. Patients who had secondary node dissection for a cervical recurrence were excluded. Eight node sites were distinguished (ipsilateral and contralateral): paratracheal, mid-jugular, supraclavicular, subdigastric. All pathologic specimens were reviewed by a single pathologist. Twenty-five patients had lymph node involvement clinically before surgery. Seventy-two (60.5%) had cervical metastasis (N+: node positive patients), with bilateral involvement in 28 cases. In cases of bilateral thyroid tumor localization, ipsilateral dissection designated the side with the largest nodule. The main ipsilateral involved sites were paratracheal (60 patients), mid-jugular (44 patients), and supraclavicular (26 patients). Contralateral paratracheal nodes were involved in 25 patients and mid-jugular nodes in 12. Among the N+ patients, node involvement was absent in 11 cases at paratracheal, 28 jugular, and 46 subclavicular sites. Cervical node metastases concerned 60.5% of the patients, with bilateral involvement in 40.8% of the N+ patients. Ipsilateral paratracheal and jugular sites were most frequently involved. The lateral compartment was sometimes involved independent of the central compartment.

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.

[Prognostic factors of survival without clinical recurrences in medullary thyroid carcinoma: apropos of 52 operated cases]. / Facteurs pronostiques de survie sans récidive clinique dans les cancers médullaires du corps thyroïde: à propos de 52 cas opérés.

The aim of this retrospective study on 52 operated medullary thyroid carcinoma (MTC) was to assess clinical and biochemical factors influencing survival without clinical recidive. There were 52% of familial cases. Mean age was 44 years (3 to 78 years) with 58% of women. The diagnostic was made prostoperatively in 38% of case (nodular specimen), when it was strongly suspected for 50% of patients before intervention (familial context, and/or preoperative Calcitonin (CT) levels, and/or mutation). The medial survival rate was 54 month (3 to 360 month). The absence of postoperative negativation of the CT (between 1 to 3 month) was meeting in 43% of case. Twenty one patients (42%) had presented one or several clinical relapse. At the end of the study, five patients were died in a postoperative time-limit of 22 to 110 month. The different parameters studed were: the sex, the age, the tumoral stage, the familial cases, the tum-oral size, the calcitonin levels normalisation in the three month postoperatively, and the local nodes extent. The 5-year survival rate was 90%, and the 10-year survival was 80%. The familial cases had a better pronostic than the sporadic (no death in the familial group versus 80% of 5-year survival rate in the sporadic cases). In univariate analysis, the good survival-factors without clinical recidive were: the stage I or II (p < 0,0001), the female sex (p = 0,02), the tumoral size under 10 mm (p < 0,02), the postoperative negativation of CT levels (p < 0, 0002), and the absence of cervical node extent (p < 0,0005). In multivariate analysis, only the postoperative negativation of CT-levels was a good survival-factor without clinical relapse (p < 0, 001).

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.

[Percutaneous self-expanding metallic endoprosthesis and malignant biliary stenoses]. / Endoprothèses métalliques auto-expansibles percutanées et sténoses biliaires malignes.

METHOD: Thirty-five patients with malignant obstructive jaundice were given palliative treatment by percutaneous self-expandable metallic stents. Cholangiocarcinoma was the most frequent cause of biliary obstruction. The stricture was located in the hilum in more of 50% of cases. RESULTS: Adequate biliary drainage was achieved in 97% of cases. Median survival was 182 days. 11% of patients have died within 30 days. Early complications occurred in 31% of patients. 25% of patients have shown recurrent jaundice after an average of 180 days. CONCLUSION: Percutaneous self-expandable metallic stents are an efficient means treating malignant biliary strictures, particularly of upper biliary obstructions.