Lymphocyte-predominant Hodgkin disease manifested as a subcutaneous arm mass.
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Adult↗
Biomedical subjects
E E Lack
Publications and source records attributed to E E Lack.
Adenomatoid tumor of the adrenal gland with ultrastructural and immunohistochemical demonstration of a mesothelial origin.
We describe an adenomatoid tumor arising in the left adrenal gland of a patient who underwent bilateral adrenalectomy for the ectopic adrenocorticotropic hormone syndrome. The mesothelial origin of the tumor was confirmed by electron microscopy and positive immunohistochemical staining for keratin. The tumor had a prominent cystic component raising the question whether adrenal cysts are derived from mesothelial inclusions. It is proposed that consideration be given to adding a new category of "mesothelial cysts" to the classification of adrenal gland cysts.
Adrenal Gland Neoplasms↗
Periurethral colonic-type polyp simulating urethral caruncle. A case report.
A 50-year-old, black woman presented with a 1-cm, polypoid lesion on the posterior edge of the urethral meatus that had the gross appearance of a urethral caruncle. The histologic features, however, revealed a superficially ulcerated lesion composed of colonic-type mucosal glands with focal regenerative atypia in response to inflammation. A similar histologic pattern is also found in so-called solitary rectal ulcer syndrome and inflammatory cloacogenic polyps arising in the anorectal area. Based upon an English-language literature review, this case appears to be the second reported one of an intestinal-type polyp in this location. The patient had no further problems after excisional biopsy.
Choristoma↗
Gingival granular cell tumors of the newborn. An ultrastructural and immunohistochemical study.
Gingival granular cell tumor of the newborn, or congenital epulis, is a rare congenital lesion of uncertain histogenesis located exclusively on the alveolar ridge with marked predilection for female infants. Although histologically similar to the more ubiquitous granular cell tumor or myoblastoma, ultrastructural and immunohistochemical studies support separate histogenetic pathways for the two lesions. A newborn female infant with three gingival granular cell tumors is described herein along with immunohistochemical and ultrastructural observations. There was positive immunostaining for vimentin but staining for S100 protein was uniformly negative. Immunocytochemical assay for estrogen and progesterone receptors was also negative. The findings indicate a mesenchymal origin and the demonstration of intermediate filaments with fusiform electron densities suggests that some of the granular cells have morphologic attributes of myofibroblasts.
Female↗
Extremity soft tissue sarcomas: analysis of prognostic variables in 300 cases and evaluation of tumor necrosis as a factor in stratifying higher-grade sarcomas.
From July 1975 to February 1985, 300 patients with extremity soft tissue sarcomas (STS) were entered into treatment protocols at the National Cancer Institute. Using the Cox proportional hazards model, the STS were analyzed for independent prognostic variables that had significant association with disease-free and overall survival. The effects of adjuvant treatment were also included in the analysis. Percent tumor necrosis was the basis for separating intermediate and high-grade STS (less than 15% necrosis = grade 2; greater than 15% necrosis = grade 3). The most important prognostic variable was histologic grade, which was highly associated with relative risk of metastasis and fatal outcome (P less than .0001). Histologic type of STS was not a significant prognostic variable in predicting metastases. Patients with tumors located on the proximal or middle part of the extremity had a fivefold increased risk of death (P = .02) compared with patients who had STS located more distally. Mitotic activity (P = .0005) and vascular invasion (P = .0034) were also associated with malignant behavior, but histologic grade was the most significant predictor of outcome. Histologic grade of malignancy was based upon a combination of tumor typing and visual estimation of extent of tumor necrosis; using these two features, one can reliably separate low-grade (i.e., grade 1) from high-grade STS and also stratify the more aggressive (i.e., grade 2 and 3) tumors. Over the 9.5-year period of study, the tumor-related mortality was as follows: grade 1 (1/38), 3%; grade 2 (19/142), 13%; and grade 3 (45/120), 38%.
Adult↗
Case report 557: Solitary intramuscular myxoma in a patient with polyostotic fibrous dysplasia.
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Adult↗
Case report 560: Parosteal osteoma of tibia.
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Aged↗
Microanatomy of vagal body paraganglia in infancy including victims of sudden infant death syndrome.
The microanatomy of vagal body paraganglia (VBP) in the cephalic segment of both vagus nerves was evaluated in an autopsy study of 32 infants one year of age or younger. The study group included 14 victims of sudden infant death syndrome (SIDS) and 18 non-SIDS cases. VBP in both groups were located at or below the lower border of the ganglion nodosum, and were histologically identical to carotid body chemoreceptors, although spatially dispersed and much smaller in size. Using a combined step and serial sectioning technique, there were no significant differences between the two groups (i.e. SIDS vs. non-SIDS) with regard to microanatomy, number, distribution and size of VBP. The proportion of chief and sustentacular cells was similar to carotid body paraganglia. For the study group as a whole, VBP were present in 89% of vagus nerves, and were typically multiple with good correlation between the number of separate paraganglia on the two sides. Small collections of ectopic parathyroid chief cells were identified in 6% of nerve segments, and histologically should be distinguished from VBP. Although the microanatomy of VBP in the SIDS group was identical to that of non-SIDS, one cannot exclude an underlying functional abnormality with autonomic malregulation.
Female↗
Lung biopsy interpretation in the acquired immunodeficiency syndrome: experience of the National Institutes of Health with literature review.
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Acquired Immunodeficiency Syndrome↗
Primary tumors of the exocrine pancreas. Classification, overview, and recent contributions by immunohistochemistry and electron microscopy.
Histologic classification and subtyping of primary tumors of the exocrine pancreas have great prognostic value, although most tumors--such as duct cell adenocarcinomas--are consistently high grade with respect to biologic behavior. The mortality rate for these tumors is nearly equal to the incidence rate, and prognosis does not appear to be significantly influenced by histologic grade of the primary tumor. Other tumors, such as solid and papillary epithelial neoplasms, have intermediate to low malignant potential, while microcystic adenomas have proved to be essentially benign. Recent application of immunohistochemistry, along with electron microscopy, has helped to elucidate the histogenesis of some tumor subtypes. An intestinal phenotype has been demonstrated for mucinous cystic neoplasms with goblet cells, Paneth cells, and a variety of endocrine cells. Divergent tumor differentiation has been seen in a rare group of mixed tumors with variable composition of duct, acinar, or endocrine cells. Some infantile pancreatic tumors, known as pancreatoblastomas, may also show mixed-cell composition. Immunohistologic studies of solid and papillary epithelial neoplasms have not yet identified a consistent or reliable phenotype or marker, and ultrastructural studies have reached disparate conclusions regarding histogenesis. The rarity of other tumors--e.g., those with osteoclasttype giant cells--has hampered complete characterization.
Aged↗
Localized amyloidosis of seminal vesicles: report of three cases in surgically obtained material.
Localized amyloidosis of the seminal vesicles (ASV) is reported as an incidental finding in surgical specimens from three elderly men. In two cases, the amyloid deposits were bilateral, subepithelial, and clinically inapparent, features similar to other cases in the literature. In one case, the diagnosis was made on a transrectal prostatic needle biopsy that included a small portion of seminal vesicle; to our knowledge, this has not been previously reported. Electron microscopy in one case demonstrated nonbranching fibrils characteristic of amyloid, and pretreatment of tissue sections using the permanganate method in two cases showed almost complete ablation of congophilia. Evidence suggests that ASV is a permanganate-sensitive, non-AA (amyloid, protein A) type of amyloid that may be different from all other types of amyloid previously characterized.
Aged↗
Vitamin B12 malabsorption in patients with acquired immunodeficiency syndrome.
We have examined 11 patients with the acquired immunodeficiency syndrome (AIDS) for evidence of subclinical vitamin B12 malabsorption. Three subjects (27%) had low levels of vitamin B12. Eight subjects (73%), including these 3 subjects plus 5 others with normal vitamin B12 levels, had abnormal Schilling test results. In addition, 15% of an unselected population of 121 patients with AIDS and 7% of 27 patients without AIDS who were seropositive for human immunodeficiency virus type 1 (HIV-1) had low serum vitamin B12 levels. Stool cultures from the 8 subjects with abnormal Schilling test results revealed no pathogens. Intestinal involvement by Kaposi's sarcoma was found in only 1 patient. Biopsy specimens from 5 of 6 patients with vitamin B12 malabsorption, however, contained mononuclear cells harboring HIV-1, as indicated by in situ hybridization studies. Our observations suggest that vitamin B12 malabsorption is common in patients with AIDS and may be a very early manifestation of infection with HIV-1.
Acquired Immunodeficiency Syndrome↗
Histopathologic review of salivary gland tumors in childhood.
During the 58-year period from 1928 through 1986, 80 patients 18 years of age or younger were treated or seen in consultation at Children's Hospital, Boston, for an epithelial or nonepithelial tumor of salivary gland origin. Using established criteria, ten (40%) of the 25 epithelial tumors were pleomorphic adenomas and the remaining tumors were classified as histologically malignant growths including mucoepidermoid carcinoma (n = 6), acinic cell carcinoma (n = 5), and adenocarcinoma most likely of duct cell origin (n = 2). Two other carcinomas presented as congenital parotid salivary gland tumors in newborns and were considered to have biphasic composition including both epithelial and myoepithelial cells, with the latter type predominating. There was only one tumor-related death due to metastases among the 15 children with malignant epithelial tumors (7%); four other patients experienced a local recurrence of tumor (27%). Of the 55 nonepithelial tumors, capillary hemangioma was the most common and showed distinct predilection for female infants with localization in the left parotid gland. The histologic spectrum of primary salivary gland tumors in infancy and childhood is distinctive and merits careful correlation with therapeutic strategies.
Adenocarcinoma↗
Decreased expression of neuropeptides in malignant paragangliomas: an immunohistochemical study.
Paraffin-embedded sections of 99 human adrenal and extraadrenal paragangliomas were analyzed by the indirect immunoperoxidase technique for the presence of neuron-specific enolase (NSE) and 10 neuropeptides. Each showed diffuse staining for NSE. Most tumors were positive for [Leu5]-enkephalin (76 per cent), [Met5]-enkephalin (75 per cent), somatostatin (67 per cent), and pancreatic polypeptide (51 per cent), followed by vasoactive intestinal polypeptide (VIP) (43 per cent), substance P (31 per cent), ACTH (28 per cent), calcitonin (23 per cent), bombesin (15 per cent), and neurotensin (12 per cent). The neuropeptides paralleled to a large extent those normally found in the sympathetic nervous system. Clinically malignant paragangliomas (n = 25) with proven regional or distant metastases expressed considerably fewer neuropeptides, although the spectrum of those seen remained similar. Malignant paragangliomas contained an average of two neuropeptides per tumor, in contrast to five for the benign tumors (P less than 0.05). Logistic regression analysis of staining results revealed that the paucity of enkephalins, somatostatin, pancreatic polypeptide, and VIP along with the patient's sex was predictive of clinical malignancy. Our results show a definite relationship between expression of neuropeptides and the biologic behavior of these paragangliomas.
Adolescent↗
Adjuvant chemotherapy for patients with high-grade soft-tissue sarcomas of the extremity.
We have previously reported the results of a randomized trial that demonstrated the survival benefit of adjuvant chemotherapy in the treatment of patients with high-grade extremity sarcomas compared with no chemotherapy. This regimen included doxorubicin, cyclophosphamide, and methotrexate. This report updates and extends our experience. The median follow-up of this trial is now 7.1 years and reveals a 5-year disease-free survival of 75% and 54% for chemotherapy and no chemotherapy groups, respectively (two-sided P [P2] = .037). The 5-year overall survival for patients in this trial was 83% and 60% for the chemotherapy and no chemotherapy groups, respectively, with a trend towards improved survival in the chemotherapy arm (P2 = .124). Because of doxorubicin-induced cardiomyopathy we performed a subsequent randomized trial comparing this high-dose regimen to reduced cumulative doses of doxorubicin and cyclophosphamide without methotrexate. Eighty-eight patients were entered into this trial which has a median follow-up of 4.4 years. The 5-year disease-free and overall survival for patients treated with the reduced doses of chemotherapy was 72% and 75%, respectively, and was not significantly different from the high-dose regimen. No patients developed congestive heart failure on this study. We conclude that adjuvant chemotherapy improves disease-free survival in patients with extremity soft-tissue sarcomas. The overall survival advantage in patients receiving adjuvant chemotherapy in our initial randomized high-dose chemotherapy trial has diminished though it continues to favor the chemotherapy group. A reduced-dose chemotherapy regimen was found to be comparable to the high-dose regimen.
Antineoplastic Combined Chemotherapy Protocols↗
Ectopic parathyroid tissue within the vagus nerve. Incidence and possible clinical significance.
A postmortem study of 64 vagus nerves from 32 children up to 1 year of age was done to determine the incidence of ectopic parathyroid tissue. The segments of nerve (average length, 2.6 cm), including the entire ganglion nodosum, were examined using a combination of step and serial sectioning. Discrete solitary collections of ectopic parathyroid chief cells were seen in 6% of vagus nerves and ranged in diameter from 162 to 360 micron. Confirmation of the nature of the cells was based on the presence of abundant glycogen and positive immunoreactivity for chromogranin and parathormone. The possible significance of intravagal parathyroid tissue is briefly discussed.
Choristoma↗
Trimetrexate for the treatment of Pneumocystis carinii pneumonia in patients with the acquired immunodeficiency syndrome.
Preclinical studies have demonstrated that trimetrexate is a potent inhibitor of dihydrofolate reductase from Pneumocystis carinii. On the basis of this evidence, this lipid-soluble antifolate was used as an antipneumocystis agent in 49 patients with the acquired immunodeficiency syndrome (AIDS) and pneumocystis pneumonia. Simultaneous treatment with the reduced folate leucovorin was used as a specific antidote to protect host tissues from the toxic effects of the antifolate without affecting the antipneumocystis action of trimetrexate. Patients were assigned to three groups and treated for 21 days: in Group I, trimetrexate with leucovorin was used as salvage therapy in patients in whom standard treatments (both pentamidine isethionate and trimethoprim-sulfamethoxazole) could not be tolerated or had failed (16 patients); in Group II, trimetrexate with leucovorin was used as initial therapy in patients with a history of sulfonamide inefficacy or intolerance (16 patients); and in Group III, trimetrexate with leucovorin plus sulfadiazine was used as initial therapy (17 patients). The response and survival rates were, respectively, 69 percent and 69 percent in Group I; 63 percent and 88 percent in Group II; and 71 percent and 77 percent in Group III. Trimetrexate therapy had minimal toxicity; transient neutropenia or thrombocytopenia occurred in 12 patients and mild elevation of serum aminotransferases in 4. We conclude that the combination of trimetrexate and leucovorin is safe and effective for the initial treatment of pneumocystis pneumonia in patients with AIDS and for the treatment of patients with intolerance or lack of response to standard therapies.
Acquired Immunodeficiency Syndrome↗
Seventeen year follow-up and autopsy findings in a case of massive osteolysis.
Clinical, radiologic, and pathologic findings in an unusual case of massive osteolysis are described. Peculiar clinical episodes of a foul body odor with fever and erythematous skin lesions, presence of radiographic findings such as acro-osteolysis and periarticular erosions, and the absence of angiomatous or lymphangiomatous dysplasias at autopsy, indicate that this is a new form of massive osteolysis rather than a variant of Gorham disease.
Bone and Bones↗