Malignant peripheral nerve sheath tumors in childhood.

Malignant peripheral nerve sheath tumor (MPNST) is an uncommon sarcoma in the pediatric population; however, its presence should be considered in a child with an enlarging or painful soft-tissue mass. Diagnosis of this neoplasm depends on either the demonstration of its origin within a peripheral nerve or the association with a contiguous neurofibroma. We have identified 16 cases of MPNST involving children 16 years of age or less, which represent 12.

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.

Neurogenic tumors of the sciatic nerve. A clinicopathologic study of 35 cases.

Thirty-five cases of neurogenic sciatic nerve tumors are described. Pain is the major clinical manifestation but may give rise to diagnostic confusion because of its often prominent site far distant from the neoplasm. Nonpalpable tumors are best identified by computed tomographic scan of the pelvis and thighs.

Pituitary adenomas producing growth hormone, prolactin, and one or more glycoprotein hormones: a histologic, immunohistochemical, and ultrastructural study of four surgically removed tumors.

The morphologic features of four pituitary adenomas, removed from 2 men and 2 women between 31 and 62 years of age, are reported. The tumors contained growth hormone (GH), prolactin (PRL), and one or more glycoprotein hormones--usually thyrotropin (TSH). Three tumors were associated with acromegaly and one with hyperprolactinemia.

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.

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.

Melanotic neuroectodermal tumor of infancy. A review of seven cases.

The melanotic neuroectodermal tumor of infancy (MNTI) is a rare, usually benign, pigmented neuroectodermal tumor which most often involves the maxilla. The authors reviewed seven cases of MNTI, with patient ages of our patients ranged from nine weeks to 18 months; six of the seven were less than six months old at initial diagnosis. Four patients were males, and all were white.

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.

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.

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.

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.

Postirradiation neurofibrosarcoma.

A known risk of radiation therapy is the induction of secondary neoplasms, most commonly osteosarcoma and fibrosarcoma. A recent addition to the list of postirradiation neoplasms is neurofibrosarcoma, a Schwann cell or fibroblastic malignancy arising in peripheral nerves, often associated with von Recklinghausen's disease. In a clinicopathologic review of 109 patients with neurofibrosarcoma seen at the Mayo Clinic from 1912 to 1981, the tumors in 12 cases were found to originate in areas that had previously been irradiated for benign or malignant disease.

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.

Primary neuroendocrine carcinomas of the skin (Merkel cell tumors). A clinical, histologic, and ultrastructural study of thirteen cases.

Neuroendocrine carcinomas of the skin have recently been recognized, and clinicopathologic information on these tumors is accumulating rapidly. We studied 13 such lesions by light and electron microscopy, and eight were subjected to immunohistochemical analysis. The ages of the patients (five women and eight men) ranged from 24 to 84 years.

Melanotic neuroectodermal tumor of infancy: a malignant tumor of the femur.

An 18-month-old white girl had a malignant melanotic neuroectodermal tumor of infancy of the left femur. Light microscopic findings of a biopsy specimen of the femoral lesion demonstrated the characteristic histologic features, that is, nests of small cells with hyperchromatic nuclei and scant cytoplasm lying within cleftlike spaces lined by cuboidal, melanin-producing cells. Electron microscopy confirmed both neuroblastic and melanocytic differentiation.

Thymic neoplasia in two male siblings.

Thymic tumors are uncommon, and malignant variants of such neoplasms are rare. Hence, the association of two siblings with aggressive tumors of the thymus is not likely to be a coincidence. We report on two brothers with thymic epithelial neoplasms, one being a thymic carcinoma and the other an invasive spindle cell thymoma with associated hypogammaglobulinemia.

Primary pulmonary leiomyosarcomas. A light and electron microscopic study.

Fewer than 100 cases of primary leiomyosarcoma of the lung have been reported, few of which have been studied by electron microscopy. We performed clinicopathologic studies, including fine structural analysis, of two such cases. Their ultrastructural features differed in part from those of other smooth-muscle tumors, which may help exclude other malignant pulmonary lesions from the differential diagnosis.

Thrombosed arteriovenous malformations of the brain. An important entity in the differential diagnosis of intractable focal seizure disorders.

Thrombosed arteriovenous malformations (AVM's) in patients with no previous history of hemorrhage are uncommon but benign lesions that present with clinical and radiographic findings which are often indistinguishable from those of other mass lesions, particularly low-grade gliomas. The authors report seven cases of thrombosed AVM's presenting as intractable seizure disorders in which the radiographic studies had suggested a low-grade glioma. All seven patients are now seizure-free 2 to 40 months postoperatively.

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.