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Perivascular epithelioid cell tumors are rare mesenchymal neoplasms composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. The perivascular epithelioid cell tumor family includes angiomyolipoma, clear cell sugar tumor of the lung, lymphangioleiomyomatosis, clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres, and rare clear cell tumors of other anatomic sites. Perivascular epithelioid cell tumors have been reported previously in various sites, but to our knowledge not in the gallbladder. We report here, for the first time, a malignant perivascular epithelioid cell tumor arising in the gallbladder.

Although the link between the BRCA1 tumour–suppressor gene and hereditary breast and ovarian cancer is established1,2,3,4,5, the role, if any, of BRCA1 in non–familial cancers is unclear. BRCA1 mutations are rare in sporadic cancers6,7,8, but loss of BRCA1 resulting from reduced expression or incorrect subcellular localization9,10 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 nuclear11,12,13,14,15, to conditionally nuclear10, to the ER/golgi16, to cytoplasmic invaginations into the nucleus17. 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.

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.

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. ( Chest 1991; 99:8–13 )

A 4-year-old child with AIDS developed encephalitis 2 months prior to death. Adenovirus was cultured from the cerebrospinal fluid. At autopsy, sections of the brain revealed complete sloughing of the intracranial ependyma, with marked gliosis and edema of the periventricular white matter. Cells with large, dark, smudgy intranuclear inclusions, consistent with adenovirus inclusions, were also seen in subependymal locations. In situ hybridization studies confirmed the presence of adenovirus DNA in these cells. This represents a case of morphologically proven adenovirus encephalitis in a pediatric AIDS patient.

Animal studies suggest that dietary polyunsaturated fatty acids (PUFAs) of the n-6 class, found in corn and safflower oils, may be precursors of intermediates involved in the development of mammary tumors, whereas long-chain (LC) n-3 PUFAs, found in fish oil, can inhibit these effects. This case-control study was designed to examine the relationship between the PUFA composition of breast adipose tissue and the risk of breast cancer. Using fatty acid levels in breast adipose tissue as a biomarker of past qualitative dietary intake of fatty acids, we examined the hypothesis that breast cancer risk is negatively associated with specific LC n-3 PUFAs (eicosapentaenoic acid and docosahexaenoic acid) and positively associated with n-6 PUFAs (linoleic acid and arachidonic acid). Breast adipose tissue was collected from 73 breast cancer patients and 74 controls with macromastia. The fatty acid levels were determined by gas-liquid chromatography. A logistic regression model was used to obtain odds ratio estimates while adjusting for age. The age-adjusted n-6 PUFA (linoleic acid and arachidonic acid) content was significantly higher in cases than in controls (P = 0.02). There was a trend in the age-adjusted data suggesting that, at a given level of n-6 PUFA, LC n-3 PUFAs (eicosapentaenoic acid and docosahexaenoic acid) may have a protective effect (P = 0.06). A similar inverse relationship was observed with LC n-3-to-n-6 ratio when the data were adjusted for age (P = 0.09). We conclude that total n-6 PUFAs may be contributing to the high risk of breast cancer in the United States and that LC n-3 PUFAs, derived from fish oils, may have a protective effect.

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.

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 (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.

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.

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.

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

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 >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.

Clinicopathological features of infection of the nervous system by cytomegalovirus (CMV) in 31 patients with the acquired immune deficiency syndrome (AIDS) are reviewed. Neuropathology was variable, ranging from rare isolated CMV inclusions in brain without associated inflammation or necrosis, to severe necrotizing ependymitis and meningoencephalitis. In 1 patient, CMV had produced a necrotizing meningoradiculitis which presented clinically as ascending paralysis. In the brains and spinal cords of 6 patients, evidence of human immunodeficiency virus (HIV) infection of neural parenchyma was seen in close proximity to CMV infection. Both viruses individually or together were associated with low grade (microglial nodule) encephalitis. In retrospect, the diagnosis of CMV had been a difficult one to make clinically in neurologically impaired patients with AIDS. The results suggest that CMV may also localize in the nervous system without significant clinical sequelae. Imaging studies and analysis of cerebrospinal fluid revealed abnormalities in many patients, but none of them (short of culture of CMV itself in two cases) appeared to be specific to this neurological complication of the immunodeficiency.

Clinical data and histologic sections of the liver, including immunohistochemical studies for hepatitis B surface and core antigens, were reviewed in 42 autopsy cases of the acquired immune deficiency syndrome (AIDS). Hepatomegaly, elevation of serum transaminases, and mild elevation of alkaline phosphatase were commonly observed clinical and biochemical abnormalities. Mildly elevated alkaline phosphatase and normal bilirubin levels were present in patients with Mycobacterium avium-intracellulare (MAI) infection, cytomegalovirus (CMV) infection, and Kaposi's sarcoma (KS). Histologic sections demonstrated liver involvement by MAI in eight cases; KS in six cases; cryptococcus in three cases; and CMV in two cases. One case of MAI infection was associated with marked central vein sclerosis, a finding previously unreported. Thirty-two (76%) of 42 cases had serologic or pathologic evidence of hepatitis exposure. Two patients had histologic evidence of chronic active hepatitis. The pathologic processes involving the liver appeared to be secondary to the infections and neoplasms for which this population is susceptible and did not significantly contribute to morbidity or mortality. No findings specific or pathognomic for AIDS were identified in the liver.

Adrenal pathology was examined in 41 autopsied patients with the acquired immune deficiency syndrome. This represents the largest series and the first study with quantitation of adrenal cortical necrosis. In 32 cases clinical data were analyzed for features of adrenal insufficiency. Common clinical findings included vomiting, diarrhea, fever, hypotension, and hyponatremia. None of the 32 patients showed characteristic skin hyperpigmentation. Two patients were suspected premortem to have adrenal insufficiency. In one of these patients, adrenocorticotrophic hormone (ACTH) stimulation resulted in an adequate rise in plasma cortisol values. In the other patient, the baseline plasma cortisol value was elevated and failed to rise significantly after ACTH stimulation. Pathologic findings included widespread lipid depletion, infection by cryptococcus, and acid-fast organisms consistent with Mycobacterium avium-intracellulare, involvement by Kaposi's sarcoma, and necrotizing adrenalitis due to cytomegalovirus (CMV). A point-counting method was used to quantitate adrenal cortical and medullary necrosis. Necrosis due to CMV was greater in the medulla than the cortex. The maximum amount of adrenal cortical necrosis in any case was 70%. The degree of cortical necrosis was less than that usually associated with adrenal insufficiency.

RECENT CLINICAL, MORPHOLOGIC, immunohistochemical, and molecular hybridization studies have drastically changed and improved our understanding of human papillomavirus (HPV) infections, especially in the female genital tract. Of particular importance is the realization that genital HPV infection (1) is one of the most common sexually transmitted diseases (STDs),1–3 (2) manifests a wide spectrum of morphologic changes2–10 that may be caused by different types of HPV,11–15 and (3) has a strong relationship to the development of squamous neoplasms.4,8,12–16 Previous epidemiologic studies are largely based on populations with the exophytic variant of genital warts (condylomata acuminata).17–19 Only a few studies have included other types of genital warts.20

Excerpt To the editor: Montilla and coworkers (1) lay claim to reporting the first example of lymphomatoid granulomatosis in a patient with the acquired immunodeficiency syndrome (AIDS). Claims of ...