Biomedical subjects

R A Prayson

Publications and source records attributed to R A Prayson.

At least 109 records · Page 6Linked to original sources

PubMed1995

Symptomatic Mycobacterium avium complex infection of the central nervous system. A case report and review of the literature.

Mycobacterium avium complex infections rarely involve the central nervous system, even in patients with widely disseminated disease. We report a case of disseminated M. avium complex infection presenting with central nervous system symptoms in a patient with Hodgkin's disease. Neurologic findings included disorientation, nuchal rigidity, seizures, an extensor plantar response, cerebrospinal fluid studies consistent with meningitis, and eventually, coma and death. Organisms of the M. avium complex were cultured from several sites. Histopathologically, the central nervous system lesions consisted of small aggregates of lymphocytes and macrophages containing acid-fast organisms present in a predominantly perivascular location. In view of the life-threatening nature and potential treatability of this uncommon presentation of M. avium complex infection, we suggest that these organisms be sought in immunocompromised patients presenting with unexplained central nervous system symptoms.

Adult↗

PubMed1995

The neuropathology of cardiac allograft transplantation. An autopsy series of 18 patients.

OBJECTIVE: To examine the neuropathologic findings associated with cardiac transplantation and to assess the role, if any, that the neuropathology had in the patient's death. DESIGN: Retrospective autopsy series of 18 patients. SETTING: Tertiary referral center with a high volume of cardiac transplantation. PATIENTS: Cardiac transplant patients who died and in whom a complete autopsy was performed. RESULTS: Eighteen patients, including 11 men and seven women, comprised the study group. Patients ranged in age from 27 to 59 years (mean 48 years). Postoperative survival ranged from 1 to 1517 days (mean 334 days). Autopsies in 15 (83%) patients showed neuropathology; in only one patient, however, was cause of death attributed to these findings. The most common neuropathology was related to anoxia or infarction: ischemia or focal neuronal necrosis (n = 9), diffuse anoxic encephalopathy (n = 3), infarction (n = 6). Other findings included infection with Nocardia, Aspergillus, and Staphylococcus (n = 3) and hemorrhage (n = 3). Of the seven patients who received transplants for ischemic heart disease, five had evidence of cerebral ischemia or infarction. Systemic pathology encountered included infection in 13 patients, ischemia and/or infarction in 11 patients, and hemorrhage in six patients. Cause of death was attributed to infection in nine patients, cardiac rejection in five patients, disseminated intravascular coagulation/hemorrhage in three patients, and systemic amyloidosis in one patient. CONCLUSIONS: The most common neuropathologic findings are related to ischemia and infarction. Neuropathologic findings were only rarely the main cause of death, but they were a significant cause of morbidity in patients who have undergone cardiac allograft transplantation.

Adult↗

PubMed1995

Pathologic considerations of uterine smooth muscle tumors.

Smooth muscle tumors are the most common type of uterine neoplasm. The vast majority of such tumors are benign leiomyomas. Leiomyosarcomas fortunately are relatively infrequent. A variety of gross and microscopic features may be found in both benign and malignant tumors and great care must be taken not to mistake leiomyomas with atypical or unusual features of leiomyosarcomas. Individual features, such as hypercellularity, necrosis, nuclear atypia, mitotic figures, and intravascular growth, are ominous but must be interpreted with caution because variants of benign leiomyoma may contain such changes.

Adult↗

PubMed1994

Pseudomyxoma peritonei. A clinicopathologic study of 19 cases with emphasis on site of origin and nature of associated ovarian tumors.

Nineteen patients (10 men, 9 women) with pseudomyxoma peritonei were studied to determine the site of origin of the disease and the nature of associated ovarian tumors. A primary appendiceal mucinous neoplasm was found in 16 of the 17 patients with an evaluable appendix. One woman's appendix had intramural and serosal mucinous deposits without neoplastic epithelial cells. The appendiceal tumors were classified as mucin-producing adenoma ("mucinous cystadenoma") in 13 patients (seven women, six men), intramucosal adenocarcinoma associated with mucin-producing adenoma in one man, and invasive adenocarcinoma associated with mucin-producing adenoma in one man and one woman. Five of the nine women had cystic mucinous ovarian neoplasms; each also had an appendiceal mucinous neoplasm (adenoma in four, invasive adenocarcinoma with adenoma in one). The ovarian neoplasms had histologic features resembling a borderline (low malignant potential) mucinous tumor in four and a mucinous cystadenoma in one; all five ovarian tumors also had features of pseudomyxoma ovarii. Mucinous implants were also on the ovarian surface of the contralateral ovary in four of the five women with ovarian tumors and in the other four women without ovarian tumors. The intraperitoneal mucus deposits contained neoplastic mucinous epithelial cells in 16 patients (eight men, eight women) and were acellular in three (two women, one man). Of 17 patients with more than 6 months of follow-up, 12 (seven women, five men) were alive after postoperative intervals of 7 to 147 months, including three with known residual disease. Five (three men, two women) died of disease 16 to 60 months after initial operation, including two patients with appendiceal carcinoma. Acellular intraperitoneal mucus appeared to be a favorable prognostic feature. We conclude that (a) the appendix is the primary site of origin of pseudomyxoma in the vast majority of cases in both men and women, and (b) the associated mucinous ovarian tumors are most likely secondary neoplasms resulting from incorporation of implanted mucus and neoplastic mucinous epithelial cells of the pseudomyxoma peritonei.

Adult↗

PubMed1993

Anaplastic astrocytoma with granular cell differentiation: case report and review of the literature.

In contrast to the relatively common soft tissue form of granular cell tumor (GCT), intercerebral GCTs are rare neoplasms. The Schwann cell is the accepted cell of origin for soft tissue GCTs. However, the origin of intracerebral tumors is controversial. We report a case of a GCT intimately associated with an anaplastic astrocytoma. Immunohistochemical staining with glial fibrillary acidic protein demonstrated focal positive staining within the granular cells. Six GCTs from other body sites were stained with glial fibrillary acidic protein for comparison and all were negative. The granular cell component was diffusely positive for S-100 and negative for epithelial membrane antigen and cytokeratin. Ultrastructurally, filaments characteristic of astrocytic cells were demonstrated within some granular cells. Based on our light microscopic, electron microscopic, and immunohistochemical findings, the granular cell component of this anaplastic astrocytoma is likely astrocytic in origin. We propose that these tumors be designated astrocytic neoplasms with granular cell differentiation and their prognoses dictated by the grade of the glial component.

Astrocytoma↗

PubMed1993

Meningeal myofibroblastoma.

Myofibroblastoma is a recently described benign mesenchymal tumor. Only one case has been reported previously in the CNS. We report a second case of myofibroblastoma arising in the meninges in a 70-year-old woman who presented with visual changes. The histologic appearance was characterized by alternating areas of spindled and rounded cells separated by collagen and amianthoid fibers. Immunostaining demonstrated strong vimentin and focal smooth-muscle actin positivity; staining for epithelial membrane antigen, cytokeratin, S-100, desmin, myosin, glial fibrillary acidic protein, and factor VIII-related antigen was negative. Ultrastructurally, the myofibroblasts had features of both smooth-muscle cells and fibroblasts. Differentiating the benign myofibroblastoma from more aggressive meningeal sarcomas and meningeal meningiomas is important. The tumor most likely arises from myofibroblasts that probably reside in the meninges.

Aged↗

PubMed1993

Stereotactic brain biopsy for diagnosis of progressive multifocal leukoencephalopathy.

We describe three patients in whom the diagnosis of progressive multifocal leukoencephalopathy (PML) was made by stereotactic biopsy. All three patients had a rapidly progressive clinical course pathologically characterized by demyelination, Alzheimer I astrocytes, and basophilic intranuclear inclusions in oligodendrocytes. In each case, the diagnosis was confirmed with immunohistochemical staining of oligodendrocytes for the JC virus and in one case by electron microscopy. Determination of the adequacy of tissue received at the time of frozen section is critical and can direct the appropriate submission of tissue to maximize available diagnostic procedures.

Adult↗

PubMed1992

Mitotically active leiomyomas of the uterus.

A series of 15 uterine smooth muscle tumors with histologic features of typical leiomyomas, except for the presence of mitotic activity exceeding 4 mitotic figures per 10 high-power fields (HPF), were studied. The patients' ages ranged from 25 to 50 years (median, 37 years). At least 60% of the tumors were submucosal. The maximum dimension of the tumors ranged from 1.3 to 8.0 cm (median, 3.8 cm). On gross examination, the tumors appeared to be generally unremarkable. By definition, none had cytologic atypia. Mitosis counts were performed in the most active areas by two methods. Counts ranged from 5 to 15 mitotic figures per 10 HPFs when the highest count in any single set of 10 HPFs was recorded (method A) and from 4.2 to 10.2 mitotic figures per 10 HPFs when the average count from 50 consecutive HPFs was determined (method B). Treatment included hysterectomy in eight patients, myomectomy followed by hysterectomy in one, and myomectomy only in six. All but one patient with evaluable endometria were in the secretory phase of the menstrual cycle. Follow-up periods ranged from 6 months to 10.5 years (mean, 2.5 years; median, 2.1 years). None developed local recurrences or metastases. The benign clinical behavior of such tumors warrants the designation of mitotically active leiomyoma rather than smooth muscle tumor of uncertain malignant potential or low-grade leiomyosarcoma.

Adult↗

PubMed1992

Dysembryoplastic neuroepithelial tumor.

Dysembryoplastic neuroepithelial tumor is a recently described but rare tumor that occurs in children and characterized by long-standing, intractable partial complex seizures. Due to a paucity of literature on this condition and its heterogeneous cellular composition, dysembryoplastic neuroepithelial tumors can present difficulties in diagnosis. The authors describe two cases of dysembryoplastic neuroepithelial tumor occurring in young patients (ages 8 and 19 years). Both tumors were located in the temporal lobe. Temporal lobectomy with excision of mesial structures resulted in resolution of the seizures. Differential diagnosis includes oligodendrogliomas, mixed gliomas, and gangliogliomas. Features of the dysembryoplastic neuroepithelial tumor that are useful in making the distinction include a multinodular and multicystic appearance, the presence of both neuronal and glial (oligodendrocytic and astrocytic) components with little if any cytologic atypia, the presence of accompanying cortical dysplasia, and the lack of an arcuate vascular pattern. Because dysembryoplastic neuroepithelial tumors are curable by excision, the recognition and correct diagnosis of this tumor is important.

Adult↗

PubMed1992

Primary smooth-muscle tumors of the ovary. A clinicopathologic study of four leiomyomas and two mitotically active leiomyomas.

A series of six primary ovarian smooth-muscle tumors were studied. Four were ordinary leiomyomas and two were neoplasms with prominent mitotic activity, which we designated mitotically active leiomyomas. The patients' ages ranged from 42 to 76 years (median, 50.5 years). In five of six patients, synchronous leiomyomas of the uterus were also present. In three patients, the ovarian tumor was an incidental finding. The maximum dimension of the ovarian tumors ranged from 0.3 to 20.0 cm (median, 3.0 cm). Three of the tumors originated in the hilar region, one on the cortical surface, and in two the exact location within the ovary could not be determined. Follow-up was available on all six patients. None developed local recurrence or metastasis from their ovarian tumor, including the two patients with mitotically active leiomyoma whose postoperative follow-up intervals were 3.8 and 7.7 years.

Female↗

PubMed1992

Recurrent cervical squamous cell carcinoma presenting with cardiac tamponade. Recurrent cervical carcinoma-tamponade.

Cervical squamous cell carcinoma rarely metastasizes to the heart, and cardiac tamponade secondary to pericardial involvement has been only rarely reported. We describe a case of recurrent cervical squamous cells carcinoma presenting with cardiac tamponade secondary to extensive pericardial metastases. The patient, a 38-year old woman, initially presented with Stage IIIB cervical squamous cell carcinoma. She responded well to radiation and chemotherapy, there was no clinical or radiographic evidence of persistent disease after the initial therapy. Sixteen months after presentation, she developed shortness of breath and chest pain. The patient received additional chemotherapy; however, she died 17 months after her initial presentation. At autopsy, metastatic keratinizing squamous cell carcinoma extensively involved the pericardium and superficial myocardium. This case illustrates the unusual occurrence of recurrent cervical squamous carcinoma presenting with cardiac dysfunction secondary to pericardial metastases.

Adult↗

PubMed1991

Angiotropic large-cell lymphoma in a patient with adrenal insufficiency.

Angiotropic large-cell lymphoma is a rare disorder characterized by a proliferation of malignant lymphoid cells within the lumina of small vessels. The skin and central nervous system are typically affected; however, involvement of other organs has been described. We document an unusual case of this disorder in a patient who suffered clinically significant adrenal insufficiency and subsequently died. Autopsy disclosed angiotropic large-cell lymphoma involving both adrenal glands.

Adrenal Gland Neoplasms↗

PubMed1990

Two unusual congenital anomalies of the tricuspid valve.

We describe two unusual congenital anomalies of the tricuspid valve discovered incidentally at autopsy. One is an abnormal attachment of the tricuspid septal leaflet to one of two posterior papillary muscles with a concomitant fusion of the right ventricular septal and anterior papillary muscles in a patient with ectodermal dysplasia. The other involves a fenestration defect of the septal leaflet of the tricuspid valve in a patient with aortic stenosis, coronary artery atherosclerosis, and cardiac amyloidosis.

Adolescent↗

PubMed1990

An analysis of outcome following percutaneous transluminal coronary artery angioplasty. An autopsy series.

We analyzed autopsy findings on 26 patients who died following percutaneous transluminal coronary angioplasty (PTCA). Twenty-one patients died within 3 weeks of undergoing PTCA; demonstrable cardiac complications were found in 19 patients: platelet-fibrin thrombi (10 patients [48%]), coronary artery dissections (17 patients [81%]), thromboemboli (13 patients [62%]), atheroemboli (seven patients [33%]), and myocardial infarcts (17 patients [81%]). An increased incidence of coronary platelet-fibrin thrombi was noted when compared with a non-PTCA cardiac autopsy population (five of 53 patients). Apparently there was an increased incidence of coronary atheroemboli and thromboemboli in the patients with coronary platelet-fibrin thrombi (eight patients) when compared with patients who did not have platelet-fibrin thrombi (five patients), although this was not statistically significant. There was no evidence of a systemic hypercoagulable state or of disseminated intravascular coagulation. The pathogenesis of this is unclear; however, vasospasm and a disruption of the endothelial surface induced by PTCA with subsequent platelet activation are possible causes. Although not statistically significant, there was a proponderance of female subjects (seven patients) and an increased incidence of diabetes mellitus (six patients) and hypertension (13 patients) when compared with a control population of all patients undergoing PTCA at The Cleveland (Ohio) Clinic Foundation in 1987, suggesting that diabetes mellitus, hypertension, and female sex may be clinical risk factors for fatal complications following PTCA.

Adult↗

PubMedDate not supplied

Coexistence of neoplasia and cortical dysplasia in patients presenting with seizures.

Tumors and cortical dysplasia are associated with epilepsy, but few studies have examined the coexistence of neoplasia and dysplasia in these patients. We studied 13 patients (age 4-29 years) with recurrent seizures of 1 month to 21-year' duration (median 72 months). Ten patients were aged < 21 years. Imaging studies localized the lesion to the temporal lobe (10 patients), parietal lobe (2 patients), and frontal lobe (1 patient). Tumors included ganglioglioma (8 patients), dysembryoplastic neuroepithelial tumor (DNT) (3 patients), and low-grade astrocytoma (2 patients). Cortical dysplasia, including atypical aggregates of neurons (6 patients), multifocal loss of the cortical laminar architecture (7 patients), and neurons in the molecular layer of the cortex (3 patients) were observed near but separate from the tumor. Coexistence of certain tumors with cortical dysplasia, most frequently observed in the pediatric population, suggests a hamartomatous/dysplastic nature of the neoplasms.

Adolescent↗

PubMedDate not supplied

The search for diagnostic criteria in Alzheimer's disease: an update.

BACKGROUND: Although the pathologic findings in Alzheimer's disease are well documented, definitive diagnostic criteria are lacking. OBJECTIVE: To review the histopathologic findings in Alzheimer's disease. SUMMARY: In Alzheimer's disease, the brain may be normal in size or atrophic. There is selective neuronal loss associated with neurofibrillary tangles and senile plaques with amyloid deposits. Amyloid protein may also be deposited within arterioles. There may be granulovacuolar degeneration and Hirano bodies in the hippocampus. Two diagnostic schemes have been proposed based on the presence of senile plaques, but neither is entirely satisfactory. CONCLUSIONS: The pathologic diagnosis is usually made in conjunction with the clinical history. Probably, neither definitive diagnostic criteria nor effective treatment for Alzheimer's disease will be possible until we understand more about its etiology. Clinicians should attempt to rule out other, potentially curable causes of dementia in elderly patients such as trauma, depression, metabolic abnormalities, infection, vascular disease, and other central nervous system diseases.

Alzheimer Disease↗

PubMedDate not supplied

Bcl-2, bcl-x, and bax expression in dysembryoplastic neuroepithelial tumors.

BACKGROUND: Bcl-2, bcl-x and bax are regulatory proteins which are variably expressed in brain tissue and are known to be involved in the regulation of apoptosis; bcl-2 and bcl-x inhibit apoptosis and bax generally promotes apoptosis. This study is a retrospective clinicopathologic and immunohistochemical review of 18 dysembryoplastic neuroepithelial tumors (DNTs), specifically looking for evidence of aberrant expression of apoptosis regulatory proteins which may promote the survival of one or more of the cellular constituents of the tumor. MATERIALS AND METHODS: Eighteen patients (11 males) with DNTs comprise the study group. Patients at the time of surgery ranged in age from 2.1-52 years (mean 16.1 years). Mean seizure duration prior to surgery was 6.4 years (n = 16 patients). Sixteen patients were alive with markedly reduced or no seizures at a mean postsurgical follow-up interval of 63 months; 2 patients were lost to follow-up. RESULTS: All tumors were characterized by an admixture of oligodendroglial cells, neurons and astrocytic cells, focally arranged against a microcystic background. Coexistent cortical dysplasia was noted in 14 evaluable cases. Mitotic figures were rarely noted in 2 tumors. MIB-labeling indices ranged from 0-0.6 (mean 0.2). Astrocytes which were part of the tumor stained with all three antibodies in all cases. The oligodendroglial-like cells of DNT stained positively for bcl-2 in 2/17 tumors, bcl-x in 10/17 tumors, and bax in 12/17 tumors. The neuronal cell component of the DNT stained positively with bcl-2 in 15/17 tumors, bcl-x in 5/17 tumors, and bax in 8/17 tumors. CONCLUSION: Aberrant expression of apoptosis-associated proteins, similar to what has been previously described in gangliogliomas (another epilepsy-related, dysplasia-associated tumor), may play a role in the pathogenesis of DNT.

Adolescent↗

PubMedDate not supplied

Shah-Waardenburg syndrome and Dandy-Walker malformation: an autopsy report.

Autopsy reports of individuals with Shah-Waardenburg syndrome are rare, and to the best of our knowledge, have never been reported in association with Dandy-Walker malformation. This report documents the autopsy findings of a 2.5-year-old boy with Shah-Waardenburg syndrome (Type IV Waardenburg syndrome) and a Dandy-Walker malformation. The patient had a past medical history of congenital deafness, skin and hair pigmentary disturbances, Hirschsprun disease and hydrocephalus. At autopsy, multiple patches of dermal and hair hypopigmentation were observed over the face, trunk, eyelashes and eyebrows. The brain had a small, underdeveloped anterior cerebellar vermis, bilateral atrophic cerebelli, a markedly dilated ventricular system and a large incomplete midline cerebellar cyst. The entire colon and much of the small bowel had been previously excised secondary to the Hirschsprung disease. This case expands the spectrum of pathologic findings in Shah-Waardenburg syndrome to include the Dandy-Walker malformation.

Autopsy↗