Search PubMed⌕ Search

Biomedical subjects

B W Scheithauer

Publications and source records attributed to B W Scheithauer.

At least 397 records · Page 22Linked to original sources
PubMed1983

Neuron-specific enolase in neuroendocrine tumors of the thymus, bronchus, and skin.

In order to better define the use of neuron-specific enolase (NSE) as a marker for neuroendocrine neoplasms, we studied 11 thymic carcinoid tumors, three bronchial small-cell carcinomas (all with cutaneous metastases), and 10 trabecular carcinomas of the skin for its presence, using the peroxidase-antiperoxidase (PAP) technic with an antiserum directed at NSE. All 11 carcinoid tumors stained positively, as did two of the bronchial small-cell carcinomas and seven of the trabecular carcinomas. We conclude that PAP staining for NSE content may be a useful adjunct to morphologic analysis in diagnostically identifying the tumors we studied and that our results support the concept of a functionally unified APUD system, as reflected in the tumors originating from it. Nevertheless, because of the vagaries of the PAP method, exemplified by the results in our small series, it cannot be relied upon as a sole indicator that a tumor contains NSE and is therefore neuroendocrine. Also, since it is hypothesized that NSE is present in all tumors of this type, staining for its presence would seem to be of little benefit in distinguishing primary from secondary neuroendocrine tumors or in identifying the origin of metastatic lesions that have a neuroendocrine histologic appearance.

Bronchial Neoplasms↗
PubMed1983

Giant-cell tumor of the sphenoid bone. Review of 10 cases.

Giant-cell tumor rarely affects the sphenoid bone. Ten cases of this tumor seen at the Mayo Clinic are reviewed, bringing the number of cases in the world literature to 31. These tumors, which present in the second and third decades of life and are slightly more frequent in women than in men, may present with various symptoms, including headache, visual field defects, blindness, and diplopia. The symptom complex and roentgenographic findings are seldom, if ever, sufficiently characteristic to allow preoperative diagnosis. The microscopic differential diagnosis of giant-cell tumor of the sphenoid region includes giant-cell reparative granuloma, aneurysmal bone cyst, fibrous dysplasia, and "brown tumor" of hyperparathyroidism. Interesting features of this series include the presence of multifocal giant-cell tumor in one case, and the absence of Paget's disease of bone in every case. Complete follow-up review in these cases allowed a discussion of therapy and prognosis; it appears that subtotal excision with postoperative radiation therapy is the treatment of choice. While the histological appearance of giant-cell tumor is characteristic, caution must be exercised in interpreting small samples, and various reparative and metabolic disorders must be considered in the differential diagnosis.

Adolescent↗
PubMed1983

The pituitary gland in untreated Addison's disease. A histologic and immunocytologic study of 18 adenohypophyses.

The pituitary glands of 18 patients with untreated Addison's disease were studied by histologic and immunocytochemical methods. Adrenal destruction was caused by tuberculosis (13 cases) or autoimmune adrenalitis (five cases), and the duration of the adrenal insufficiency ranged from one to 16 years. Both diffuse and nodular hyperplasia of corticotropic cells were evident in each case, and the extent of hyperplasia correlated with the duration of disease. In five cases, nodular proliferations with morphologic features between those of hyperplasia and those of adenoma, termed tumorlets, were identified, as were two microadenomas, only one of which was available for study. In all instances, the proliferating corticotrophs stained positively with PAS and were immunoreactive for adrenocorticotropic hormone and beta-endorphin. We conclude that diffuse and nodular corticotroph hyperplasia are common in untreated Addison's disease, although frank adenoma formation seems to be rare. The latter may be related to the short duration of disease or may imply the absence of additional, unknown factors that are required for adenoma growth.

Addison Disease↗
PubMed1983

Malignant bone tumors associated with neurofibromatosis.

Neurofibromatosis (von Recklinghausen's disease), a phakomatosis inherited as an autosomal-dominant trait, predisposes to multiorgan neoplasia as well as to skeletal abnormalities. In an attempt to define more clearly the nature of osseous malignant lesions associated with von Recklinghausen's disease, we reviewed three cases of coexistent neurofibromatosis and malignant tumors of bone. None of the tumors was considered neurogenic. One patient had a fibrosarcoma, and two patients had malignant fibrous histiocytomas. No gross relationship to nerves was demonstrated, and special studies for axons were negative. Primary neurogenic sarcomas of bone are extremely rare; the diagnosis should be considered only when the tumor originates from an intraosseous nerve or contiguous neurofibroma. The occurrence of primary bone sarcomas in patients with neurofibromatosis seems to be a sporadic event.

Adult↗
PubMed1983

Hypothalamic neuronal hamartoma and adenohypophyseal neuronal choristoma: their association with growth hormone adenoma of the pituitary gland.

Hypothalamic neuronal hamartomas and neuronal choristomas of the anterior pituitary are rare lesions; either may be associated with endocrinopathy. We describe a case of each with associated growth hormone-producing pituitary adenomas and clinical acromegaly, both well documented and studied by immunocytochemistry and electron microscopy. That a functional relationship exists between the neuronal malformation and the pituitary neoplasm remains speculative. We suggest that a growth hormone-releasing factor-like substance may have been elaborated by hypothalamic-type neurons, which, by a trophic effect, may have resulted in the production of an adenohypophyseal neoplasm. Our study supports the concept that secretory neurons, either outside or within the sella, may induce adenomas.

Acromegaly↗
PubMed1983

Transsphenoidal microsurgical treatment of prolactin-producing pituitary adenomas. Results in 100 patients.

One hundred patients, 84 females and 16 males, with presumed prolactin-producing pituitary adenomas were treated with transsphenoidal microsurgery. Criteria for success of the operation were return of serum prolactin values to normal and reversal of the endocrine effects of hyperprolactinemia, eradication of the adenoma, and preservation of anterior pituitary function. Of the 84 females, 54 had a microadenoma (a noninvasive adenoma 10 mm or less in diameter), and of the 32 with preoperative values of serum prolactin of less than 100 ng/ml, 28 (88%) had a successful operative result, whereas 11 of 22 (50%) with preoperative values of 100 ng/ml or more had a successful result. Five of 10 patients (50%) with a diffuse adenoma (a noninvasive adenoma more than 10 mm in diameter) and 5 of 20 (25%) with an invasive adenoma had a successful result. Of the 16 males, 3 of 7 (43%) with diffuse adenoma and 3 of 16 (19%) with invasive adenoma had a successful result. There were no males with microadenoma. The largest success rate (88%) was among female patients with microadenoma and preoperative serum prolactin values of less than 100 ng/ml.

Adenoma↗
PubMed1983

Multifocal Merkel's cell tumors associated with a cutaneous dysplasia syndrome.

Multifocal Merkel's cell tumors developed in a 24-year-old patient with an unusual cutaneous dysplasia syndrome. The neoplasms behaved in an aggressive fashion, with the development of multiple lymph node metastases. In this case, the dermatologic syndrome had features of both dyshidrotic ectodermal dysplasia and the basal cell nevus syndrome. In addition to Merkel's cell tumors, basal cell carcinomas and actinic elastosis were seen in sun-exposed skin sites. These findings, in conjunction with a known tendency for Merkel's cell tumors to arise in solar-damaged areas, suggest a pathogenetic relationship between these neoplasms and the underlying cutaneous syndrome in this patient.

Adenocarcinoma↗
PubMed1983

Coexistent primary empty sella syndrome and hyperprolactinemia. Report of 11 cases.

The series involved 11 women with concurrent hyperprolactinemia and primary empty sella syndrome. Eight had amenorrhea and six had galactorrhea. All 11 patients had intact hypothalamic-pituitary function, except for having elevated prolactin levels that ranged from 33 to 498 ng/mL. One patient had primary hypothyroidism. Radiologic investigations included sellar polytomography in eight cases, computed tomography of the head in eight cases, bilateral carotid angiography in six cases, and pneumoencephalography in three cases. Of eight patients undergoing transsphenoidal exploratory surgery, one had a pituitary microadenoma and an empty sella while seven had only an empty sella with a flattened pituitary gland. Conventional histologic methods (seven cases) and immunocytologic studies (three cases) of the pituitary gland showed no abnormalities. The cause of this syndrome is unknown. It should be recognized that hyperprolactinemia, with or without galactorrhea-amenorrhea, may occur in association with an empty sella in the absence of an associated pituitary tumor.

Adult↗
PubMed1982

Oat-cell carcinoma of the thymus.

Oat-cell carcinoma of the thymus gland is exceedingly rare as a primary lesion, and only a few cases have been reported. We report a case in which a neoplasm interpreted as an oat-cell carcinoma was intimately associated with and arose in transition from a carcinoid tumor of the thymus. Ultrastructural features of this lesion were unusual compared with previous descriptions of thymus neuroendocrine neoplasms.

Adult↗
PubMed1982

Adamantinoma of bone. An electron microscopic and immunohistochemical study.

Adamantinoma of bone is a rare tumor, and fine structural analysis has been done in only a few cases. We report four cases studied by electron microscopy and immunohistochemical methods. Ultrastructural evaluation revealed a characteristic constellation of features, including intracellular bundles of type I microfilaments, moderate numbers of evenly dispersed mitochondria, scattered profiles of rough endoplasmic reticulum, occasional Golgi bodies and lysosomes, and scattered glycogen particles. Microvillous processes and desmosomes were identified in all tumors. Well-formed basement membranes enveloped cell clusters but did not surround individual cells. Intercellular basement membrane-like material also was found focally in pools. Ultrastructural features of endothelial differentiation, including Weibel-Palade bodies, micropinocytotic vesicles, and tight junctions, were not identified. Immunoperoxidase stains for coagulation factor VIII (von Willebrand factor) and blood group antigens were negative, whereas similar stains for keratin were positive. Our findings strongly suggest that adamantinoma is a neoplasm expressing definite epithelial, rather than endothelial, characteristics.

Adolescent↗
PubMed1982

Primary nonlymphoreticular malignant neoplasms of the spleen.

Primary sarcomas of the spleen are exceedingly uncommon neoplasms, approximately 90 substantiated cases having been reported. We report on six cases of primary splenic angiosarcoma and three cases of primary malignant fibrous histiocytoma of the spleen which have been seen at the Mayo Clinic during the last 51 years. In one of the cases of splenic angiosarcoma, the lesion was manifested by spontaneous rupture and hemoperitoneum; interestingly, two cases of splenic malignant fibrous histiocytoma were of the inflammatory type and showed corresponding clinical features of a systemic nature. Five patients with angiosarcoma of the spleen died of metastatic tumor within 3 years after diagnosis; one patient with splenic malignant fibrous histiocytoma is alive with metastasis 11/2 years after splenectomy. One case of splenic angiosarcoma and two of malignant fibrous histiocytoma of the spleen have been diagnosed only recently. All three patients are alive and currently tumor-free, from 3 to 7 months after diagnosis.

Adolescent↗
PubMed1982

Primary thymic carcinomas.

Within a 75-year period, 20 patients with primary nonteratomatous carcinomas of the thymus were seen at the Mayo Clinic. Fourteen were males and six were females, with a mean age of 48 years. Thirteen of the lesions were classified as poorly differentiated or spindling squamous cell carcinomas. One neoplasm was a sarcomatoid carcinoma, and two showed both squamous cell and neuroendocrine ultrastructural features. The remaining four tumors had both light- and electron-microscopic features of neuroendocrine carcinomas. In all 20 patients, careful clinical and pathologic examinations, including autopsy in fatal cases, excluded malignant lymphomas and metastasis to the thymus from epithelial tumors in other anatomic sites. Thirteen patients underwent surgery, with or without postoperative radiotherapy; of these, one was alive and apparently disease-free 43 months after diagnosis. There were two operative deaths. The remaining patients received primary radiotherapy or chemotherapy, or both. The mean survival of the 16 patients who died (excluding operative deaths) was 18.7 months for the 14 patients with tumors of squamous cell differentiation and 36.0 months for the two patients with pure neuroendocrine carcinomas. Among patients with squamous cell carcinoma, 11 developed metastases to lungs, liver, bones, adrenal glands, or extrathoracic lymph nodes; nine of these died of massive local growth within the mediastinum. Among patients with neuroendocrine carcinomas, two of four patients died of local growth within the mediastinum; one of these also had metastases to the liver and adrenal glands. One patient with oat cell carcinoma was alive with residual thoracic tumor and cervical lymph node metastasis 18 months after diagnosis.

Adolescent↗
PubMed1982

Gaucher's disease and glioblastoma multiforme in two siblings: a clinicopathologic study.

The clinical and pathologic features of two brothers with the adult form of Gaucher's disease, both of whom developed cerebral glioblastoma multiforme, are presented. Neither patient had a long-standing neurologic disorder, and morphologic evidence of nervous system glucocerebroside storage was not seen. Despite the known increased incidence of malignancy in Gaucher's disease, a review of the literature and of sixty-two Mayo Clinic cases of adult onset Gaucher's disease revealed only one other instance of the association with glioma. The significance of the relationship is unknown.

Brain Neoplasms↗
PubMed1982

Clinical and pathological study of meningiomas of the first two decades of life.

The clinical features and pathological materials of 51 cases of histologically verified meningiomas in patients under 21 years of age were reviewed. The age range was 7 to 20 years, with the majority of cases being clustered in the second decade of life. There was virtually a 1:1 sex ratio, with 26 females and 25 males. Thirty-eight patients had intracranial meningiomas, three had intraorbital tumors, and 10 had intraspinal tumors. Twelve patients (24%) had neurofibromatosis. Twenty patients (39%) had tumor recurrence. The cases were subdivided into five histological categories: meningotheliomatous, fibrous, transitional, psammomatous, and papillary. In each case, the clinical course was correlated with histological subtype. The 15-year survival rate in patients with intracranial meningiomas of all types was 68%. Factors adversely affecting survival included infratentorial location, papillary histology, and evidence of brain invasion.

Adolescent↗