Primary carcinoid of the testis 25 years after contralateral testicular seminoma.

We report a case of primary testicular carcinoid occurring coincidentally 25 years after contralateral radical orchiectomy for seminoma, which has not been previously reported.

Intraosseous glomus tumor of the fibula.

Glomus tumor is a rare, benign vascular tumor and intraosseous glomus tumor, which arises primarily within bone, is even rarer. Fewer than 20 cases have been reported in the literature. We present the case of a 34-year-old woman with glomus tumor primarily in the midshaft of the fibula that radiologically mimicked chondromyxoid fibroma, aneurysmal bone cyst or adamantinoma, together with a review of other reported cases.

A study of an educational intervention to decrease inappropriate preoperative autologous blood donation: its effectiveness and the effect on subsequent transfusion rates in elective hysterectomy.

BACKGROUND: Decreasing the overcollection of preoperative autologous blood is difficult to achieve. The purpose of this study was to determine whether an educational intervention designed to outline the risks of preoperative autologous collection can decrease such donations and, if so, to determine how this decrease will affect subsequent transfusion rates. STUDY DESIGN AND METHODS: An educational intervention consisting of a didactic presentation to the Department of Gynecology staff about the risks and benefits of autologous blood was implemented. Written material with similar information was given to patients. Subsequently, the percentage of patients donating autologous blood, the number of autologous units donated, and the rates of transfusion in patients eligible for autologous donation who were admitted for elective abdominal or vaginal hysterectomy were measured. These rates were compared to those in similar patients admitted in the 2 years before the educational intervention. RESULTS: After an educational intervention, the proportion of patients donating autologous blood decreased from 53 percent to 26 percent (p<0.01), and the number of units collected per patient decreased from 0.86 to 0.31 (p<0.01); this resulted in a savings of 80 autologous donations per year. Despite no difference in estimated blood loss (p = 0.46), the overall transfusion rate decreased from 10 percent to 3.7 percent (p = 0.03), while the allogeneic transfusion rate demonstrated no significant change (1.1% vs. 2.2%; p = 0.40). CONCLUSIONS: Unnecessary preoperative autologous donations by elective hysterectomy patients can be decreased by educating physicians and patients about the risks of preoperative autologous blood donation. Decreasing such unnecessary donations can decrease the subsequent autologous transfusion rate, with its attendant risks, without increasing the risk of allogeneic transfusion.

Localization of human BRCA1 and its loss in high-grade, non-inherited breast carcinomas.

Although the link between the BRCA1 tumour-suppressor gene and hereditary breast and ovarian cancer is established, the role, if any, of BRCA1 in non-familial cancers is unclear. BRCA1 mutations are rare in sporadic cancers, but loss of BRCA1 resulting from reduced expression or incorrect subcellular localization is postulated to be important in non-familial breast and ovarian cancers. Epigenetic loss, however, has not received general acceptance due to controversy regarding the subcellular localization of BRCA1 proteins, reports of which have ranged from exclusively nuclear, to conditionally nuclear, to the ER/golgi, to cytoplasmic invaginations into the nucleus. In an attempt to resolve this issue, we have comprehensively characterized 19 anti-BRCA1 antibodies. These reagents detect a 220-kD protein localized in discrete nuclear foci in all epithelial cell lines, including those derived from breast malignancies. Immunohistochemical staining of human breast specimens also revealed BRCA1 nuclear foci in benign breast, invasive lobular cancers and low-grade ductal carcinomas. Conversely, BRCA1 expression was reduced or undetectable in the majority of high-grade, ductal carcinomas, suggesting that absence of BRCA1 may contribute to the pathogenesis of a significant percentage of sporadic breast cancers.

Fatal embolization of ball portion of Starr-Edwards aortic valve prosthesis.

We report an unusual case of a Starr-Edwards aortic valve malfunction with fatal dislodgment and embolization of its ball portion to the distal abdominal aorta, just above the bifurcation, leading to sudden death. This case is unusual in that it is, to date, the longest time elapsed between valve placement and ball dislodgment.

Aspergillosis of the testis in a renal transplant recipient.

Aspergillus is ubiquitous worldwide. It is found primarily in soil, dust, vegetation, and decaying matter. While disseminated aspergillosis affects the testis only 1% of the time, testicular aspergillosis in the absence of disseminated disease is exceedingly rare, as demonstrated in the present case report.

Giant cell arteritis in association with cerebral amyloid angiopathy: immunohistochemical and molecular studies.

Giant cell arteritis (GCA) usually manifests as a transmural vascular infiltrate of mononuclear and multinucleated giant cells (MNGC). We describe six patients with GCA associated with severe cerebral amyloid angiopathy (CAA), all with cerebral hemorrhage or varying degrees of cerebral infarct, and histological evidence of Alzheimer's disease (cortical CAA often predominating over senile plaques and neurofibrillary tangles). One case showed mostly cortical involvement (with old microhemorrhages), and the others were primarily leptomeningeal (with involvement of the underlying cortex and extensive encephalomalacia of adjacent brain). Many vessels with CAA exhibited a pronounced adventitial and perivascular infiltrate of lymphocytes, histiocytes, and MNGC. Immunohistochemical staining showed deposition of beta/A4 peptide primarily in the thickened media of CAA vessels, and within the cytoplasm of MNGC--suggesting phagocytosis of insoluble peptide. Cystatin C antibody stained vascular amyloid and diffusely highlighted astrocytic and MNGC cytoplasm. HAM56-positive macrophages were frequently seen around amyloid-laden vessels. Anti-smooth muscle actin immunohistochemistry suggests the occurrence of medial destruction by amyloid, with relative preservation of intimal cells. Ultrastructural studies performed in one case confirmed the presence of intracytoplasmic amyloid in MNGC. The GCA seen in these cases of CAA most likely represents a foreign body response to amyloid proteins, causing secondary destruction of the vessel wall. DNA from brain tissues of five affected patients was examined to assess whether mutations were present in exon 17 of the APP gene or exon 2 of the cystatin C gene, a finding that might explain the foreign body giant cell response to amyloid proteins in these cases. However, restriction fragment mapping of amplified gene segments showed that previously described mutations were not present in these cases.

Preoperative autologous blood donations before elective hysterectomy.

OBJECTIVE: To determine whether preoperative autologous blood donation is justified for patients undergoing elective abdominal or vaginal hysterectomy. DESIGN: Retrospective cohort study. PATIENTS AND SETTING: A total of 263 consecutive patients admitted for elective abdominal or vaginal hysterectomy to a community health maintenance organization hospital during 1993 and 1994. MAIN OUTCOME MEASURES: Evaluation of transfusion rates for patients who did and did not donate autologous blood; determination of any risk factors that would predict the need for transfusion; and evaluation of the need for transfusion based on chart review. RESULTS: Of 263 patients, 26 received a blood transfusion. The major risk factor identified for transfusion was the donation of autologous blood. Of 140 patients who donated autologous blood, 25 were transfused, whereas just 1 patient of 123 who did not donate autologous blood was transfused (P<.001). Patients who donated autologous blood had significantly lower mean admission hemoglobin level than patients who did not donate (119 g/L vs 132 g/L; P<.05); logistic regression showed that autologous donation was an independent risk factor for transfusion. CONCLUSION: For hysterectomy patients, donation of autologous blood causes anemia and is associated with a more liberal transfusion policy. These 2 factors result in a markedly increased incidence of transfusion with its associated risks. Elimination of preoperative autologous donation for these patients should not result in frequent exposure to allogeneic blood.

Neurilemoma of the kidney.

Neurilemomas are neoplasms that originate from Schwann cells of the central and peripheral nervous systems. Thirteen case reports of neurilemomas involving the kidney have been described in the English literature. We report the fifth case of a neurilemoma within the renal sinus and review the previous cases of renal neurilemoma. This neoplasm is usually found incidentally. Presenting signs are nonspecific and radiographic features are highly variable. These factors make a preoperative diagnosis extremely difficult. Accurate identification relies heavily on detailed pathologic evaluation. Wide local excision is the treatment of choice, since malignancy is found in up to 30% of cases and there is a tendency for local recurrence and distant metastasis.

Benign melanocytic nevus cells in axillary lymph nodes. A prospective incidence and immunohistochemical study with literature review.

Benign nevus cell aggregates (NCAs) in lymph nodes usually present as intracapsular or trabecular collections of small, uniform melanocytes that resemble those seen in intradermal melanocytic nevi. The surgical pathologist must be aware of nodal NCAs because they can mimic micrometastasis of carcinoma. Although not uncommon, the frequency with which NCAs occur is controversial. Two previous studies attempted to determine the case incidence of NCAs in axillary lymphadenectomies; widely different results were reported, ranging from .33% to 6.2%. In this study, the authors examined prospectively 300 axillary lymph node dissections containing 5186 lymph nodes, using S-100 protein immunohistochemistry as a supplemental evaluation measure, to determine the incidence of NCAs. Twenty-eight NCA-positive lymph nodes from 22 cases were found, for a 7.3% case incidence and a .54% nodal incidence; these figures were higher than those previously reported. The possible pathogenesis of this phenomenon is discussed, with a review of the literature.

Oat cell carcinoma of the gallbladder. A rare and highly lethal neoplasm.

Oat cell carcinomas arising in the gallbladder are an extremely rare and aggressive form of gallbladder cancer. These neoplasms are morphologically identical to their pulmonary counterparts, contain neurosecretory granules on ultramicroscopic examination, and demonstrate immunohistochemical staining for cytokeratin, neuron-specific enolase, and other neuroendocrine markers. Oat cell carcinomas typically occur in elderly women with cholelithiasis, and pursue a fulminant course with extensive metastases, especially to the liver. The mean survival is less than 7 months. We present a well-documented case of oat cell carcinoma of the gallbladder and review the literature on this unusual entity.

Multifocal necrotizing leukoencephalopathy with pontine predilection in immunosuppressed patients: a clinicopathologic review of 16 cases.

Multifocal necrotizing leukoencephalopathy (MNL) is characterized by multiple, usually microscopic, foci of necrosis confined primarily to the white matter of the basis pontis, but sometimes found elsewhere in the central nervous system. All 16 patients in whom we identified MNL had underlying immunosuppression, either naturally occurring or iatrogenic, including the acquired immunodeficiency syndrome (seven patients), acute leukemias (four patients), and lymphoma (one patient). One patient each had severe combined immunodeficiency, orthotopic liver transplantation, Shwachman-Diamond syndrome, and idiopathic thrombocytopenic purpura treated with high-dose steroids. Histologically, lesions of MNL show demyelination, spongiosis, axonal swelling, minimal histiocytic infiltrates, and frequent axonal calcification. Multifocal necrotizing leukoencephalopathy lesions were found in the pons of all patients, most commonly in the crossing pontocerebellar fibers, but often in the descending white matter tracts and rarely in the tegmentum. Three cases also showed nonpontine foci of MNL involving the white matter of the medulla, cerebellum, and cerebral hemispheres. Multifocal necrotizing leukoencephalopathy is a distinct entity usually localized to the basis pontis and is most consistently associated with immunosuppression, but as yet lacks other clearly defined clinical correlates.

Human prion diseases.

The Council on Scientific Affairs of the California Medical Association presents the following inventory of items of progress in pathology. Each item, in the judgment of a panel of knowledgeable physicians, has recently become reasonably firmly established, both as to scientific fact and important clinical significance. The items are presented in simple epitome, and an authoritative reference, both to the item itself and to the subject as a whole, is generally given for those who may be unfamiliar with a particular item. The purpose is to assist busy practitioners, students, researchers, and scholars to stay abreast of these items of progress in pathology that have recently achieved a substantial degree of authoritative acceptance, whether in their own field of special interest or another. The items of progress listed below were selected by the Advisory Panel to the Section on Pathology of the California Medical Association, and the summaries were prepared under its direction.

Testicular plasmacytoma. A case report with immunohistochemical studies and literature review.

Plasmacytomas of the testes are extremely rare tumors, especially when occurring in the absence of a previous or concurrent diagnosis of multiple myeloma. We present a case of solitary testicular plasmacytoma in a 71-year-old man who had no clinical evidence of myeloma. Immunohistochemical stains showed monoclonal cytoplasmic production of IgA-kappa within tumor cells. A summary of the other reported cases of testicular plasmacytoma is presented. Plasmacytoma of the testes, while occasionally manifesting as a solitary, primary lesion, most likely is a precocious, highly unusual manifestation of systemic multiple myeloma.

Acute eosinophilic pulmonary disease associated with the ingestion of L-tryptophan-containing products.

A series of four patients with pulmonary infiltrates, pleural effusions, hypoxemia, peripheral eosinophilia, and symptoms of dyspnea, fatigue, and weakness is reported. Lung tissue obtained in three patients revealed interstitial pneumonitis, small-to-medium-vessel mixed-cell vasculitis, and alveolar exudate of histiocytes and eosinophils. All patients reported ingestion of L-tryptophan-containing products at a time when an association between L-tryptophan and the eosinophilia-myalgia syndrome was established. This clinical pattern of pulmonary involvement may be part of the continuum of the eosinophilia-myalgia syndrome. The pathophysiology of this syndrome and the relationship with the ingestion of L-tryptophan-containing products have not yet been identified.

The many faces and phases of borreliosis II.

Borrelia burgdorferi, the etiologic agent of Lyme disease, has also been associated with other cutaneous conditions. Acrodermatitis chronica atrophicans and lymphadenosis benigna cutis are also caused by B. burgdorferi. Recent evidence links some cases of progressive facial hemiatrophy of Parry-Romberg, benign lymphocytic infiltrate of the skin (Jessner-Kanof), lichen sclerosus et atrophicus, morphea, and Shulman syndrome with borreliae. This article reviews the manifestations of the diseases definitely linked to borreliosis and the evidence linking borreliae to progressive facial hemiatrophy, benign lymphocytic infiltrate, lichen sclerosus et atrophicus, morphea, and Shulman syndrome.

The many faces and phases of borreliosis. I. Lyme disease.

Lyme disease is increasingly being reported throughout the United States and many parts of the world. Borrelia burgdorferi, the etiologic agent of Lyme disease, is a spirochete that, not unlike the treponema of syphilis, can cause a spectrum of disease from the initial skin lesion, through widely varied symptoms and signs, to chronic neurologic and arthritic disability. The borrelial spirochete and Lyme disease are the subject of this review. A subsequent article will review other definite and possible cutaneous manifestations of borreliosis.