Medullary carcinoma of the thyroid--therapeutic strategy derived from fifteen years of experience.

BACKGROUND: The purpose of this investigation was to analyze our experience with patients treated for medullary carcinoma of the thyroid to identify prognostic factors and to develop a stage-related treatment strategy that might improve cure rates. METHODS: Between 1970 and 1985 a total of 40 patients with medullary thyroid carcinoma were treated (21 women, 19 men; mean age +/- SEM; 40 +/- 3 years; mean follow-up, 82 +/- 12 months). Initial operation involved total thyroidectomy (28 patients), subtotal resection (11 patients), and a lobectomy (1 patient). The initial lymph node dissection generally consisted of a selective removal of enlarged nodes. Unilateral neck dissection was performed in six cases. Secondary operation for recurrent disease was necessary in 26 patients. RESULTS: At the end of the follow-up period 10 patients were tumor free, 12 patients were scheduled for further treatment, six patients suffered from persistent but clinically occult disease, and 12 patients had died (mean survival time, 68 +/- 7 months). The paramount prognostic factor was the absence or presence of lymph node involvement at the time of primary operation (p = 0.011). Patients with distant metastases died within 2 years of diagnosis. Women, patients younger than 40 years of age, and those elicited by familial screening programs exhibited increased survival times. CONCLUSIONS: Because of the prognostic and therapeutic importance we recommend the total thyroidectomy with a complete dissection of the central lymph node compartment as primary treatment. Patients with lymph node involvement or elevated serum calcitonin levels should subsequently be treated by a modified radical neck dissection of the lateral compartments.

Pedal reconstructions for limb salvage.

If only one pedal artery is patent the vascular surgeon has to decide between primary amputation and a bypass. Between 1980 and 1990, 460 femoro-distal bypasses were performed in our clinic and 8% (39 cases) of these received a pedal reconstruction. At discharge from the hospital 85% of these grafts were patent. The primary goal of treatment, to maintain life and salvage the limb on the basis of a functioning bypass, was obtained in 82% of patients. After 1 year the secondary patency of our pedal reconstructions was 68%. Taking the mortality of amputations into account, vascular surgery to the foot arteries is preferable to amputation, whenever possible.

Immunohistochemical reclassification of anaplastic carcinoma reveals small and giant cell lymphoma.

Thirty-one cases of thyroid malignancies which were originally classified as anaplastic carcinoma were reexamined immunohistochemically using PAP methods (peroxidase:antiperoxidase) for IgM, IgG, IgA, cytokeratin, calcitonin, lysozyme and alpha-1-antitrypsin. The reclassification results were compared with patient data such as clinical symptoms, treatment modalities, and clinical outcome. Postoperative radiotherapy was carried out in more than 80% of cases, chemotherapy in none. Seven of 31 tumors showed a positive staining for IgM (n = 4), IgG (n = 2), and IgA (n = 1) antibodies. All of these cases were negative for epithelial markers. Surprisingly, not only all small cell tumors (n = 3) but also 4 tissues with predominantly giant cell areas were among those reclassified as primary malignant lymphoma.

[Status of tracheotomy in treatment of the respiratory emergency in anaplastic thyroid cancer]. / Stellenwert der Tracheotomie in der Behandlung des respiratorischen Notfalls beim anaplastischen Schilddrüsenkarzinom.

A review is presented on the 69 patients requiring intraoperative tracheotomy amongst 170 patients with locally infiltrating carcinoma of the thyroid operated on over a 30-year period. 55% of these patients underwent this procedure for absolute indications; in 45% of cases it was performed as a prophylactic procedure to prevent later respiratory complications due to further infiltration by the anaplastic tumour. The follow-up showed that there was no benefit for patients with anaplastic neoplasms from this additional procedure. Most of these patients suffered from local wound healing complications so that obligatory postoperative external radiation could not be administered. The overall prognosis was very poor. Survival was significantly lower still in the group of patients in whom tracheotomy had been performed.

Quantification and classification of echographic findings in the thyroid gland by computerized B-mode texture analysis.

Computerized B-mode image analysis was investigated in a pilot study to establish quantitative criteria for thyroid echography and to demonstrate the diagnostic accuracy of the procedure in thyroid disease. The ten per cent percentile of grey level distribution and entropy proved to be the most efficient statistical parameters. Computerized ultrasound detected all nodular lesions with an overall accuracy of about 85%. Standard diagnostic evaluation in nodular thyroid disease includes conventional ultrasound followed by needle biopsy to confirm the final diagnosis. However, in many cases, single needle biopsy is not sufficient to exclude malignant disease. Computer-aided ultrasound proved to be helpful for the identification of typical echographic characteristics in thyroid lesions with a high risk of malignancy. We suggest that needle biopsies be repeated in cases in which computerized analysis is positive, even when initial needle biopsy is negative.

Postoperative pentagastrin-stimulated serum calcitonin concentrations in patients with medullary thyroid carcinoma: reoperations in patients with concentrations bordering the detection limit.

The case reports on two patients with medullary thyroid carcinoma show that even postoperatively stimulated serum calcitonin (CT) concentrations near the detection limit (using a polyclonal antibody against synthetic CT) can demonstrate persistent disease. Stimulated CT concentrations can be lowered to nondetectable levels by a second and third operation if a meticulous technique is used for dissection of the lymph compartments. The patient can then be assumed to be cured. Diagnostic accuracy at very low CT concentrations can be improved by selective venous catheterization with blood sampling for CT after stimulation.

[Femoro-crural bypass--orthotopic]. / Der femoro-crurale Bypass--orthotop.

From 1980 to 1988 248 femoro-distal bypass procedures were performed in anatomical position for limb salvage due to peripheral artery disease. Bypass material strongly influenced bypass patency rate. The analysis showed a highly significant difference between venous and prosthetic grafts over a period of 3 years (p less than 0.0001). Use of the composite jump craft technique increased patency rates after 3 years to 58%, which is close to that of vein bypasses (63%). Use of prosthetic bypass material alone achieved a patency rate of only 38%. It is concluded that technically more difficult procedures such as the composite jump graft technique are superior to prosthetic bypasses.

[Problems of tracheotomy in locally invasive anaplastic thyroid cancer]. / Probleme der Tracheotomie beim organüberschreitenden anaplastischen Schilddrüsencarcinom.

Between 1955-1985 515 patients with carcinoma of the thyroid have been operated. Among 170 cases with a local infiltration 69 tracheostomies were performed in 55% for lesions of both laryngeal nerves or intratracheal bleeding (absolute indication), in 45% as a prophylactic intraoperative procedure to avoid later death from asphyxiation (relative indication). In a retrospective study clinical results were analysed with special reference to the question whether patients profit by the tracheostomy. Follow-up examinations showed that tracheostomy did not influence the outcome of patients with a differentiated thyroid carcinoma while there was a remarkable difference in survival of patients with anaplastic tumors where those with a tracheostomy had a worse survival. In this group postoperative external radiotherapy often could not be administered or was delayed due to local complications of the tracheostoma.

[The value of clinical aspects, laboratory and circulatory parameters in blunt abdominal trauma]. / Die Aussagekraft von Klinik, Labor und Kreislauf beim stumpfen Bauchtrauma.

Within seven years 506 patients with blunt abdominal trauma were included into a prospective trial. The aim of the study was checking of the validity of clinical parameters, routinely performed laboratory examinations and of the initial circulatory situation in relation to an abdominal organ lesion. Three groups were separated out of the total collective: Group 1: Patients without abdominal lesion (N = 274). Group 2: Patients with abdominal lesion, verified by operation, sonography or CAT scan (N = 232). Group 3: Patients with rupture of the spleen (N = 107) (subgroup of 2). Among the clinical parameters: spontaneous abdominal pain, contusions marks, abdominal tenderness, shoulder pain, and abdominal palpation, the latter does have a high validity (92%). However, in group 1, more than half of the cases also had palpation pain. Shoulder pain has a high sensitivity. Of the laboratory parameters: hemoglobin, hematocrit and leucocytes, only the leucocyte count provided a certain importance: 83% of group 2 had values above 10,000. The circulatory parameters blood pressure and pulse as initial spot picture are of minor validity. Continuous registration of these values at clinical observation has much higher relevance indicating trends towards improvement or deterioration.

Pheochromocytoma in multiple endocrine neoplasia.

Three patients with a surgically confirmed and 1 with suspected pheochromocytoma as part of the syndrome of multiple endocrine neoplasia (MEN) type II are presented. Pheochromocytoma and medullary thyroid carcinoma (MTC) as well as other tumors are part of this heritable entity. Three cases were members of a MEN IIa family and 1 patient has suffered from a sporadic form of MEN IIb. Pheochromocytoma was diagnosed by screening procedures (catecholamine screening, computerized tomography, sonography or selective venous catheterization). In 2 of the patients there was no clinical manifestation of the pheochromocytoma. On three occasions MTC was diagnosed first, the thyroid tumor occurred prior to the pheochromocytoma by 7, 6, and 1/2 years. Pheochromocytoma occurred bilaterally in 2 patients, in 1 it is suspected in both adrenals.

Importance of early diagnosis and follow-up in multiple endocrine neoplasia (MEN II B).

Three patients with a sporadic form of multiple endocrine neoplasia (MEN) IIb are presented. MEN IIb is the association of medullary carcinoma of the thyroid (MTC), pheochromocytoma and multiple mucosal neuromata. The age at diagnosis and primary therapy of MTC was relatively late (8, 16, 18 years) although thyroid enlargement (two cases), typical marfanoid habitus (two cases) or multiple mucosal neuromata (one case) had been evident since early childhood. The diagnosis was confirmed by measurements of tumour markers like calcitonin (CT) and carcinoembryonic antigen (CEA) for MTC, measurements of vanillylmandelic acid for pheochromocytoma, evaluation of the thyroid gland by sonography, scintigraphy and fine needle biopsy, and investigation of the adrenals by computer tomography and meta-(I-131) iodobenzylguanidine (I-131-MIBG) scintigraphy. After surgical treatment determinations of tumour markers confirmed relapses of MTC in all cases. Early diagnosis of MEN IIb, which could be made by recognition of the typical clinical appearance, is of special importance because of the poor prognosis of MTC.

[The C-cell-tumours of the thyroid gland in childhood (author's transl)]. / Die C-Zell-Tumoren der Schilddrüse im Kindesalter.

C-cell tumours are rare in children. They must be classified as hereditary types of the C-cell carcinomas. C-cell hyperplasias are considered as obligatory precanceroses of this malignoma. The article reports on two cases of C-cell hyperplasia discovered within the framework of a family screening study. Attention is drawn to the importance of screening for diagnosis of early tumour stages resulting in improved prognosis.

[Early recognition of C-cell carcinoma by family screening (author's transl)]. / Früherkennung des C-Zell-Karzinoms durch Familien-Screening.

All available family members of five patients with C-cell carcinoma (medullary carcinoma of the thyroid) were tested for their basal serum-calcitonin level and after pentagastrin stimulation. In two females, aged 12 and 25 years, from two different families, serum-calcitonin was elevated, but only after repeated tests or after pentagastrin stimulation, to definitely abnormal levels. Neither had palpable thyroid nodules, lymph-adenopathy or diarrhoea. The thyroid scan was normal and there were no storage defects. But because of the family history and the elevated serum-calcitonin levels total thyroidectomy was performed in both. The specimens revealed multicentric, bilateral C-cell carcinomas. The serum-calcitonin concentration became completely normal postoperatively, so that one may justifiably speak of a curative effect of the thyroidectomy in these two instances of early diagnosis.