Robert C. Hickey

Anaplastic carcinoma of the thyroid: A clinicopathologic study of 121 cases

One hundred twenty‐one cases of anaplastic carcinoma of the thyroid treated at M. D. Anderson Cancer Center, Houston, were reviewed. Anaplastic carcinoma is a rapidly growing neoplasm with a dismal prognosis. The mean survival of our patients was 7.2 ± 10 months. A significant percentage of our patients (35%) had areas of well‐differentiated thyroid carcinoma elsewhere, supporting the hypothesis that anaplastic thyroid carcinoma arises from preexisting well‐differentiated thyroid carcinoma. Twenty‐four of 30 tumors analyzed (84%) stained for keratin, 28 (93.3%) stained for vimentin, and ten (33%) stained for epithelial membrane antigen. Younger patients lived longer than older patients, and patients whose disease was earlier‐stage at presentation responded better than patients with metastases at presentation. Radical surgery alone did not significantly increase survival duration over less radical surgery. The role of multimodality therapy needs further evaluation.

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.

Cortical-sparing adrenalectomy for patients with bilateral pheochromocytoma

BACKGROUNDnBilateral pheochromocytomas are common in patients with multiple endocrine neoplasia type 2 (MEN 2) and von Hippel-Lindau disease (VHL). In an effort to avoid long-term steroid dependence and Addisonian crisis, we have performed cortical-sparing adrenalectomy in this patient population.nnnMETHODSnRetrospective chart review was completed for patients with MEN 2- or VHL-related pheochromocytomas who underwent laparotomy at our institution for intended cortical-sparing adrenalectomy between June 1965 and March 1995.nnnRESULTSnFifteen patients (MEN 2A [10], MEN 2B [2], VHL [3]) underwent laparotomy for cortical-sparing adrenalectomy. None of the tumors were malignant. Cortical-sparing adrenalectomy was possible in 14 (93%). Thirteen of these 14 patients (93%) had normal postoperative plasma cortisol measurements and did not require steroid hormone supplementation. At a median follow-up of 138 months, two patients had died of metastatic medullary thyroid cancer, no patient had suffered Addisonian crisis, and three patients (21%) had recurrent pheochromocytomas (at 118, 176, and 324 months after operation). The remaining nine patients were alive without pheochromocytomas.nnnCONCLUSIONSnCortical-sparing adrenalectomy can be performed successfully in MEN 2 or VHL patients with bilateral pheochromocytomas, avoiding chronic steroid hormone replacement and the risk of Addisonian crisis in most patients. Long-term follow-up is necessary because recurrence may develop many years after operation.

Surgical management, DNA content, and patient survival in adrenal cortical carcinoma

BACKGROUNDnSurgical resection is the only potentially curative treatment for adrenal cortical carcinoma, yet the value of extended resection, palliative resection, and tumor DNA analysis remains unclear.nnnMETHODSnThe records of 23 patients with adrenal cortical carcinoma who underwent primary surgical resection at our institution were retrospectively reviewed. Flow cytometric DNA analysis was performed on primary tumor tissue from 14 patients.nnnRESULTSnSixteen of 23 patients underwent complete resection. For these 16 patients the median follow-up was 43 months, the actuarial median survival was 46 months, and the actuarial 5-year survival rate was 46%. The seven patients who underwent incomplete resection all died of disease with a median survival of 8.5 months. Isolated local recurrence as the first site of failure occurred in two patients. Only completeness of resection (p = 0.004) and stage at presentation (p = 0.006) were significant prognostic indicators. None of the following predicted a poor prognosis in patients who underwent complete resection: (1) need for extended resection, (2) presence of renal vein or inferior vena cava tumor thrombus, or (3) tumor aneuploidy (14 of 14 tumors were aneuploid).nnnCONCLUSIONSnLong-term survival is possible in patients with adrenal cortical carcinoma if complete, margin-negative tumor resection can be achieved. Isolated local recurrence is uncommon after complete resection. Because adrenal cortical carcinomas are consistently aneuploid, tumor DNA content is not a useful prognostic factor.

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.

Immunoperoxidase study of uncommon parathyroid tumors. Report of two cases of nonfunctioning parathyroid carcinoma and one intrathyroid parathyroid tumor-producing amyloid

Nonfunctioning carcinomas of the parathyroid gland are rare and difficult to diagnose. They are often confused with thyroid tumors or with metastasis from other sites. We report two cases of nonfunctioning parathyroid carcinomas; one was originally diagnosed as follicular carcinoma of the thyroid gland. The immuno-histochemical demonstration of parathormone in the tumor cells established the parathyroid origin of these neoplasms. An intrathyroid parathyroid tumor, associated with large amounts of interstitial amyloid, mimicking medullary carcinoma of the thyroid, in a patient with primary hyperparathyroidism is also reported. Positive immunoreaction in the tumor cells for parathormone, negative staining for calcitonin, and the return of patients serum calcium levels to normal after tumor resection, confirmed the parathyroid nature of this neoplasm. Immunohistochemistry studies proved to be extremely helpful in establishing the diagnoses of these unusual parathyroid tumors.

Hyperparathyroidism and Carcinoid Tumor

Three patients with carcinoid tumor associated with hyperparathyroidism are described. All patients showed a high circulating immunoreactive calcitonin level with no differential increase in the neck venous catheterization specimens, suggesting that the high concentrations of circulating immunoreactive calcitonin may have come from the carcinoid tumor. We think that the hyperparathyroidism in these patients was primary and that the association with carcinoid tumor represents another form of multiple endocrine tumor formation. Hyperparathyroidism should be investigated in patients with carcinoid tumor.

An Unusual Skin Rash Associated with a Pancreatic Polypeptide-Producing Tumor of the Pancreas

Excerpt Pancreatic polypeptide is a newly identified secretory product from pancreatic islet cell tumors (1, 2). This peptide has been seen with increasing frequency in different islet cell tumors ...

Extended en bloc resection of a primary mediastinal parathyroid carcinoma

Parathyroid carcinoma is a rare endocrine tumor infrequently seen in the mediastinum. This report describes a patient who underwent en bloc resection of a primary mediastinal parathyroid carcinoma. The tumor originated from the thymus and extended from the aortic arch to the thyroid; local invasion suggested malignancy. En bloc resection of this carcinoma with all surrounding tissue provided local control of the tumor and relief of symptomatic hypercalcemia.

Diagnosis of thyroid cancer by bone marrow biopsy in a patient with lymphoma and goiter

Metastatic follicular thyroid carcinoma was diagnosed by bone marrow biopsy performed during the staging evaluation in a patient with large-cell lymphoma and diffuse goiter who showed lung and bone lesions upon radiologic examination. After thyroidectomy, both sites concentrated radioactive iodine, confirming their thyroid origin and allowing for the appropriate treatment.