Right ventricular myxoma

We recently treated a patient with right ventricular myxoma and a clinical picture of pulmonary embolism and tricuspid valve obstruction who had successful removal of the tumor through the tricuspid valve via a right atriotomy.     

The results of various modalities of treatment of well differentiated thyroid carcinomas: a retrospective review of 1599 patients.

This study analyzed the impact of prognostic variables of age, sex, histopathological diagnosis, extent of disease at diagnosis, and surgical intervention on well differentiated thyroid carcinoma and how surgical treatment, radioactive iodine, and radiotherapy influence the patients' outcomes. There have been 1599 patients with well differentiated thyroid cancer treated and followed at the University of Texas M.D. Anderson Cancer Center from 1948 to 1989. The median follow-up for all patients was 11.0 yr, with the maximum follow-up being 43 yr and the minimum follow-up being 1 yr. The patients were predominantly female (2.3:1), with papillary (81%) and intrathyroidal carcinomas (42%) at the time of diagnosis. Sixty-six percent of the patients had a total thyroidectomy, 7% received external radiotherapy, and 46% had radioactive iodine as part of the treatment of the original disease; the overall recurrence rate was 23%, and the death rate was 11%. This study showed that treatment with radioactive iodine was the single most powerful prognostic indicator for increased disease-free interval (P less than 0.001) and that its use significantly increased survival as well. No benefit was obtained from treatment with external radiotherapy. Children had the best overall survival, but of the adult patients, females who had intrathyroidal papillary disease treated with total thyroidectomy, who had been given radioactive iodine, and whose disease had been diagnosed between 20-59 yr of age had the best prognosis.     

Detection of metastatic adrenal carcinoma using 131I-6-beta-iodomethyl-19-norcholesterol total body scans.

Adrenal and total body scintigraphs with 131I-6-beta-iodomethyl-19-norcholesterol were obtained in 5 patients who had had prior resection of adrenal cortical carcinoma. The results were compared with roentgenographic findings and liver, bone, and total body gallium-67 citrate scintigraphs. Metastatic lesions were detected with radiolabeled cholesterol in 4 of 5 patients, including 3 liver metastases, 2 bone metastases, and 1 lung metastasis. These lesions were also demonstrated by one or more of the other diagnostic modalities. All initial findings were negative in a fifth patient, who developed brain metastases within two months. The 6-methyl-analog of iodocholesterol makes it possible to detect metastatic adrenocortical carcinoma with total body scans. Whether or not this agent is "tumor specific" and will be of significant clinical utility will have to be determined more fully in a larger series of patients.     

Adrenal cortical carcinoma

The records of 110 patients (58 females and 52 males) with histologically proven adrenal cortical carcinoma were studied. Fifty-six of 110 (50.9%) patients had abdominal symptoms and 33 (30.0%) had a palpable abdominal mass. Surgery for localized and regional disease was associated with a disease-free survival time of at least 2 years in 28 of the 50 (56%) patients. Abdominal radiotherapy, systemic chemotherapy, and 1,1 dichloro-2 (O-chlorophenyl)-2 (P-chlorophenyl) ethane (OPDDD) were effective in three of 19 (15.8%) patients, three of 31 (9.7%) patients, and 21 of 72 (29.2%) patients, respectively. Combined radiotherapy and OPDDD did not confer any additional benefit over OPDDD alone. Six of seven patients who received OPDDD as adjuvant treatment are still alive at 1 to 4 years. Distant metastases occurred most commonly in the lungs, liver, peritoneum, lymph nodes, and bones. Only 23% of the patients survived 5 years or more. We conclude that early diagnosis and complete surgical excision offer the best prospects for long-term survival and that the efficacy of adjuvant OPDDD needs to be evaluated further.     

Multiple endocrine syndrome type I. Clinical, laboratory findings, and management in five families

The clinical features of 20 patients from five families with multiple endocrine neoplasia syndrome type I (MEN-I) were studied. Nineteen patients (95%) had hyperparathyroidism. Five patients who had a diagnosis during surgery of adenoma and who had fewer than 3.5 glands removed had recurrence of hypercalcemia after surgery. Fourteen patients (70%) had pancreatic islet cell tumors. All had one or more elevated serum polypeptide hormones, and six had symptoms related to the hormones produced. Multiple pancreatic tumors were identified in the nine patients who underwent surgery. Three patients who died had a mean survival of 6.3 +/- 2.9 years. Eight patients had pituitary tumors; seven had macroadenomas. Of the eight patients with pituitary tumors, seven had high serum prolactin and responded to bromocriptine therapy, whereas the eighth patient had acromegaly treated with radiotherapy. It was concluded that hypercalcemia due to hyperparathyroidism in MEN-I syndrome patients should be managed by a resection of four glands and transplantation of one half gland into the forearm because none of the patients has shown evidence of a recurrence, and serum calcium levels have been normal. Pancreatic tumors, which are usually multiple, may be asymptomatic. Patients with these tumors usually have long survival rates, even with distant metastasis. Total pancreatectomy may be the method of choice, especially in patients with gastrinoma caused by the diffuse nature of the disease. Long-term follow-up is needed, however, with more patients. Pituitary tumors are primarily prolactin-producing tumors, and medical treatment is the method of choice.