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Biomedical subjects

L E Tisell

Publications and source records attributed to L E Tisell.

At least 19 recordsLinked to original sources
PubMed1992

Metastasizing gastric epithelioid leiomyosarcomas (leiomyoblastomas) in young individuals with long-term survival.

RESULTS. Four patients with metastasizing epithelioid leiomyosarcoma of the stomach, three females and one male (15, 20, 22, and 25 years of age, respectively), are reported. Two patients had recurrent tumors in the gastric remnant. Liver metastases occurred in all patients, three of whom had lymph node metastases; two had peritoneal metastases. The patients are alive at 17, 19, 27 and 48 years after the diagnosis was made and 17-27 years after the first demonstration of metastases. One woman had multiple chondromatous hamartomas of the lung. Operations were performed when the patients had symptoms and during periods of no symptoms, as directed by the second-look principle. One patient had 10 operations. None of the patients received adjuvant therapy. All three women have given birth to healthy children after metastases were diagnosed. Three of the primary tumors were large (10-20 cm) and multinodular, features that have been associated with unfavorable prognosis. The four primary tumors had a similar light microscopic appearance, characterized by moderate cell and nuclear pleomorphism and low mitotic activity, 0.03-0.1/mm2. Ultrastructurally, a network of intermediate filaments was found within the cytoplasm of the tumor cells corresponding to the immunohistochemical positivity for vimentin. The immunohistochemical findings (negative immunoreaction for desmin and alpha smooth-muscle actin) and the ultrastructural analysis produced no evidence of the production of smooth-muscle cell myofilaments. However, there were tumor cells with an abundance of mitochondria and a paucity of filaments, features that may be characteristic of epithelioid leiomyomatous tumors. In addition, immunohistochemical negativity for cytokeratins, epithelial membrane antigen, S-100 protein, neuron-specific enolase, and chromogranin militate against an epithelial or neuroectodermal cell differentiation. CONCLUSIONS. An awareness of this type of gastric leiomyosarcoma in children and young adults is of importance in making correct assessments of prognosis and choosing an active therapeutic approach. The biologic background to the clinical behavior of these metastasizing tumors remains an enigma. Additional studies are needed to elucidate the biology of these tumors.

Adolescent↗
PubMed1991

Management of disseminated midgut carcinoid tumours.

Forty-one patients with disseminated midgut carcinoid tumours were treated over a 6-year period according to a strict programme including primary surgical treatment. In 10 patients, a total remission of the disease was obtained. Patients with bilobar hepatic disease had ischaemic treatment of their liver metastases by hepatic arterial embolisation after primary surgical and medical treatment (low dose octreotide). Thus, by combining surgical, radiological and medical treatment modalities, we wanted to offer these patients optimal palliation. This treatment programme resulted in good symptomatic relief in all patients accompanied by a marked reduction in 5-hydroxyindoleacetic acid (5-HIAA) levels. At recurrence of symptoms in combination with rising 5-HIAA levels, embolisation was repeated. Ten of the treated patients have deceased during the observation period, but only 5 from their carcinoid disease.

Adult↗
PubMed1991

Muscle structure and function before and after surgery for primary hyperparathyroidism.

In 23 unselected cases of primary hyperparathyroidism (pHPT), muscle strength, morphology and enzymatic activities were studied and electromyography (EMG) performed before and 6 months after surgical treatment. Hypercalcemia was mostly mild or moderate. Nine women undergoing surgery for benign thyroid conditions served as controls regarding muscle strength, while muscle morphologic and enzymatic data were compared with findings in healthy persons of similar age. Only three pHPT patients reported muscle weakness preoperatively, and two were subjectively improved after surgery. Muscle strength did not differ significantly before or after operation between patients and controls: After surgery both groups showed increased isokinetic muscle strength at higher angular velocities. Nor did muscle morphology differ between pHPT patients and controls. No conclusive EMG changes were found before or after surgery for pHPT but postoperatively the pHPT patients showed significant increase in glycolytic but not oxidative muscle enzymes, possibly reflecting early effect of pHPT on especially type II fibers, which previously were shown to be most extensively involved in pHPT. Otherwise no measurable negative effects of mild or moderate pHPT were found on muscle strength or function.

Adult↗
PubMed1991

Disodium pamidronate in the preoperative treatment of hypercalcemia in patients with primary hyperparathyroidism.

Nine patients (median age, 81 years) with primary hyperparathyroidism were treated with intravenous infusions of disodium pamidronate (APD), which is a bisphosphonate drug. Six patients had severe hypercalcemia (serum calcium concentration, greater than 3 mmol/L) persisting after rehydration with saline and treatment with furosemide; three patients had moderate hypercalcemia with pronounced symptoms (serum calcium concentration 2.8 to 2.9 mmol/L). Three of the patients were considered to have hypercalcemic crises. In all patients, the raised serum calcium levels were lowered by the disodium pamidronate infusions. One week after a single infusion of 15 to 60 mg disodium pamidronate, six of the nine patients had serum calcium concentrations within the normal reference interval and two patients had slightly raised values. Transient asymptomatic hypocalcemia was noted in one patient. All patients tolerated the infusions well, and no side effects were noted. In the patients with verified parathyroid adenomas, a temporary increase in parathyroid hormone levels were observed concomitant with the drop in serum calcium level. The patient with parathyroid cancer displayed no such effect indicating an autonomous parathyroid hormone secretion from the parathyroid carcinoma tumor. The good effect of treatment with the osteoclast inhibitor disodium pamidronate on hypercalcemia caused by primary hyperparathyroidism suggests that this hypercalcemia is mainly due to an increased osteoclast activity. The number of patients in this series is yet too small to allow general conclusions. But the case histories in this series show that disodium pamidronate promises to be of value in different clinical situations for the treatment of severe hypercalcemia in patients with hyperparathyroidism. It can be used (1) preoperatively to investigate whether the patient's symptoms are related to the hypercalcemia, (2) in the treatment of hypercalcemic crises when "forced diuresis" has failed to normalize the serum calcium, (3) after unsuccessful parathyroid surgery when it can be used as a long-term treatment before reoperation, giving time for localization studies and healing of the scar reaction, and (4) in aged and fragile patients where it can be tried as an alternative to surgery.

Adolescent↗
PubMed1989

Concomitant occurrence of an adrenal ganglioneuroma and a contralateral pheochromocytoma in a patient with von Recklinghausen's neurofibromatosis. An immunocytochemical study.

This report presents concomitant occurrence of an adrenal ganglioneuroma and a contralateral pheochromocytoma in a patient with von Recklinghausen's disease. The patient's daughter also has cutaneous neurofibromatosis and an adrenal medullary tumor indicating that the observed "three component disease" may represent an inherited neurocristopathy. Immunocytochemically the ganglioneuroma showed a positive reaction with a tyrosinhydroxylase antiserum, but a negative reaction with a dopamine-beta-hydroxylase antiserum, suggesting the capacity of dopamine synthesis. Frequent ganglion cells were immunopositive against neuropeptide Y, but occasional ganglion cells were also positive against enkephalin and substance P. Adrenergic nerve fibers were abundant in the Schwann cell portion of the tumor, but peptide containing nerve cell processes were also demonstrated.

Adrenal Gland Neoplasms↗
PubMed1989

Surgical treatment of patients with the carcinoid syndrome.

During the last 5-year period aggressive surgical debulking was performed in 19 of 21 consecutive patients with the midgut carcinoid syndrome. Two patients were excluded due to severe cardiac disease. Fourteen of the patients had hepatic metastases. Carcinoid symptoms can occur also in patients without proven liver disease with sole lymph node metastases or retroperitoneal tumours. In 4 patients all metastatic tumour tissue could be removed resulting in biochemical cure and no symptoms. In patients with multifocal hepatic metastases surgical debulking was followed by transarterial embolisations of the hepatic arteries. All patients had good symptomatic relief of the combined surgical and radiological treatment. Five patients had currently stable disease anatomically and biochemically, 2 slowly progressive disease and one patient has a more rapid progression. Three patients have just undergone debulking and are waiting for further treatment. During the observation period 4 patients have died. Of these deaths one was related to the surgical procedures. After the introduction of somatostatin analogues major surgery can be performed safely in these patients with little risk of carcinoid crises. The role of additional drug therapy in these rare patients must be evaluated critically in randomized multicenter trials using a common surgical primary treatment.

Aged↗
PubMed1989

Treatment of the pancreatic disease of multiple endocrine neoplasia type 1 (MEN 1).

This review comprises an analysis of the treatment alternatives for pancreatic tumours of the MEN 1 syndrome presently in use at the Surgical Endocrine Unit of Sahlgrenska Hospital, Göteborg, Sweden. Patients with pancreatic tumours localized by imaging studies are offered surgical excision. Otherwise the therapy is individualized. Some patients with Zollinger-Ellison syndromes are managed with medical antiulcer treatment, others are treated with total gastrectomy. MEN 1 patients with other syndromes, e.g. insulinomas, glucagonomas and VIPomas, are offered surgery with the aim of tumour excision. Even noncurative excision might benefit the management of these cases and possibly prolong life. Asymptomatic patients with raised hormonal tumour markers in whom imaging studies have failed to reveal any tumours, are continuously followed without treatment. The malignant behaviour of the pancreatic tumours varies between different MEN 1 families. MEN 1 patients are considered for total pancreatectomy if they have multiple malignant pancreatic tumours and if the pancreatic disease has caused high morbidity and mortality in the family.

Adenoma, Islet Cell↗
PubMed1989

Surgical treatment of medullary thyroid carcinoma in a thirteen-year-old girl with MEN 2B.

Patients with palpable medullary thyroid carcinoma (MTC) have lymph node metastases in 90% of cases. In most series such patients continue to have elevated serum calcitonin (CT) levels after surgery indicating residual tumor tissue. We attempted a microdissection technique for the treatment of MTC. "Micro" refers not to a small incision or a limited exploration but to a more safe operation associated with good lighting and magnification and minimal bleeding. This technique was used in a 13-year-old girl with multiple endocrine neoplasia type 2B (MEN 2B). The prognosis for MTC in MEN 2B is worse than for sporadic MTC and the MTC of MEN 2A. Every possible effort should be made to remove all tumor tissue in MEN 2B patients with MTC. In this case the pathologist found bilateral MTC and metastases in eight of 129 lymph nodes removed. The preoperative stimulated CT levels, which were markedly elevated, decreased to near normal postoperatively.

Adolescent↗
PubMed1989

Surgical treatment of medullary carcinoma of the thyroid.

In our series the prognosis of medullary carcinoma of thyroid (MCT) has improved during recent years due to earlier diagnosis and more systematic treatment. MCT remains confined to the neck for long periods of time. The surgical strategy should be based on this fact and on the occurrence and distribution of lymph node metastases. Lymph node metastases occurred in 90% of patients with palpable tumors. In those patients with hereditary MCT who had palpable tumors the lymph node metastases were distributed bilaterally in 88%. Hereditary cases with non-palpable thyroid tumors had lymph node metastases in 27%. During the last 15 years none of the patients with hereditary but 15% of those with sporadic disease died from MCT. During the same period 12 of 22 patients with lymph node metastases had their provoked calcitonin levels normalized by surgery.

Carcinoma↗
PubMed1988

Recent results of reoperative surgery in medullary carcinoma of the thyroid.

Reoperative surgery of medullary carcinoma of the thyroid can be avoided by meticulous primary surgery. Reoperative surgery can succeed even if the tumour and its metastases are bilateral, as is often the case in hereditary cases. Even patients with numerous macroscopic metastases can show normaligation of their calcitonin (CT) levels in response to reoperative surgery. Patients with normal postoperative CT values after the combined short time Ca++ and pentagastrin test continued to show normal results when followed up for up to eight years.

Calcitonin↗
PubMed1988

Total pancreatectomy in the MEN-1 syndrome.

A family with the multiple endocrine neoplasia Type 1 (MEN-1) syndrome, followed over three generations, is presented. In the first generation, one family member died after a history indicating Zollinger-Ellison syndrome. In the second generation endocrine pancreatic tumours were diagnosed or suspected in three out of five members. In the third generation, five out of seven members were investigated and four of these had positive tests at hormonal screening. Of particular diagnostic value in this family was the determination of peripheral serum levels of proinsulin and C-peptide, which were used as tumour markers both for diagnosis and in the postoperative follow-up. Two members in the third generation have been successfully managed by total pancreatectomy. A MEN-1 patient with malignant pancreatic tumours should be considered for total pancreatectomy if the pancreatic disease has caused high morbidity and mortality within the family. This is especially pertinent if the patient has multiple malignant tumours.

Adult↗
PubMed1988

The pentagastrin test as an indicator of a cure in surgically treated patients with advanced carcinoid disease.

A unique case of midgut carcinoid with advanced disease, engaging lymph nodes and liver but still accessible to curative surgery, is reported. Besides elevated urinary levels of 5-hydroxyindoleacetic acid, the diagnosis was based on a positive provocation test with pentagastrin, i.e., basally elevated levels of serotonin in peripheral whole blood increased twofold 3 min postinjection related to the appearance of carcinoid symptoms. After removal of primary and secondary lesions in a two-step procedure carcinoid symptoms disappeared completely. Biochemically, urinary excretion of 5-hydroxyindoleacetic acid and serotonin levels in peripheral blood before and during provocation with pentagastrin were both normalized after surgery and have remained so during 27 months of follow-up. Computed tomographies of the abdomen at regular intervals do not indicate recurrent disease. Histologically, the carcinoid lesions displayed a mixed growth pattern and signs of local aggressiveness. Immunocytochemically, the majority of the tumour cells (positive against neuron specific enolase) showed a positive reaction with serotonin antisera. Substance P-like immunoreactivity was also found in a large proportion of these cells.

Carcinoid Tumor↗