bluuebunny/crossref_metadata_2025 huggingface dataset:
Cystic Fibrosis
Authors: Grody Wayne W.
Published: January 2021

Objective.—To review the current status of scientific knowledge and opinion regarding molecular genetic testing of mutations in the CFTR gene for purposes of diagnosis and population carrier screening of cystic fibrosis (CF).

Data Sources.—Published research findings on the nature of the CFTR gene, pilot population screening studies in the United States and Europe, and ongoing deliberations of professional and governmental agencies considering implementation of widespread testing.

Study Selection.—Findings relevant to the molecular heterogeneity of CFTR mutations and its implications for population carrier screening were considered.

Data Extraction.—Information was extracted from studies published by us and others, as made available to recent consensus panels and professional committees.

Data Synthesis.—These data were reevaluated in light of recent movements in professional and public policy regarding acceptability and desirability of widespread CF mutation testing. Effects to date of such testing on patient outcomes is reported.

Conclusions.—The ability to test for CFTR mutations at the molecular level has already improved the diagnosis of symptomatic patients and expanded the reproductive options of family members of CF patients. The same technology also holds promise of identifying asymptomatic carriers and at-risk couples without family history in the general population so that they too might be offered prenatal diagnosis or other options. However, a number of key questions remain to be worked out before a widespread national screening program can be put into practice. These include the target population to be offered testing (the entire population vs high-risk ethnic groups), the size and nature of the mutation test panel (universal vs ethnic specific), the inclusion or exclusion of CFTR variants that do not cause classical CF, the optimal testing technology, appropriate standards for laboratory quality assurance, and the development of sufficient educational materials and genetic counseling resources for test delivery, reporting, and interpretation. The answers to these questions will be relevant not only to CF testing but also to many other large-scale molecular genetic screening programs being considered in the future.
Utility of RET Mutation Analysis in Multiple Endocrine Neoplasia Type 2
Authors: Noll Walter W.
Published: January 2021

Objective.—To review the role of RET mutation analysis in the diagnosis of multiple endocrine neoplasia type 2 (MEN 2) and in presymptomatic screening for this disorder.

Data Sources.—Review of the medical literature and current clinical practice.

Conclusions.—RET mutation analysis is a sensitive and specific test for MEN 2. It plays a pivotal role in the diagnosis and management of patients and families with MEN 2 and in the individual who presents with an apparently sporadic medullary thyroid carcinoma or pheochromocytoma. These disorders may first come to the attention of either the anatomic or clinical pathologist, who has the opportunity to see that appropriate testing is done. As with any familial disease, professional genetic counseling is an important part of the care of these patients.
Efforts to Regulate the Collection and Use of Genetic Information
Authors: Reilly Philip R.
Published: January 2021

Public fascination with and support for genetic medicine is complicated by a deeply held fear that genetic information will be used by third parties (eg, insurers, employers, school systems) in ways that will harm the individuals from whom it was derived. Since the mid-1990s there has been much state and some federal legislative activity to address 2 closely related issues: the maintenance of genetic privacy and the prevention of genetic discrimination. These laws have had to confront several challenging questions such as what constitutes a genetic test, is genetic information qualitatively different from other medical information, and is there a means to distinguish between the two. In general the state laws are not well crafted. I will argue that a far more preferable policy is to draft a global, comprehensive medical records privacy law and to develop a model statute that defines the role of predictive genetic information in insurance underwriting. Concerns over misuse of genetic information also pose major issues for the conduct of genomic research. Among those I discuss are ownership of the DNA sample, significant changes in the scope of consent that must precede the decision to volunteer as a subject in genomic research, the reuse of long-archived samples, the challenges to intellectual property rights that flow from research, and the rise of the doctrine of community consent.
Interobserver Variability in Subclassification of Squamous Intraepithelial Lesions
Authors: Woodhouse Sherry L. , Stastny Janet F. , Styer Patricia E. , Kennedy Mary , Praestgaard Amy H. , Davey Diane D.
Published: January 2021

Objective.—To determine whether, on a national cytology proficiency test, a competent cytologist can consistently distinguish grades of squamous intraepithelial lesions.

Design.—Results for low- and high-grade squamous intraepithelial lesion referenced slides from the College of American Pathologists Interlaboratory Comparison Program in Cervicovaginal Cytology for 1996 and 1997 were analyzed including educational, nongraded vs graded validated slides.

Results.—The discrepant rate between low- and high- grade lesions ranged from 9.8% to 15% for cytotechnologist, pathologist, laboratory, and all responses. There was a statistically significant difference in performance on graded, validated slides vs educational slides with better performance on validated slides.

Conclusion.—This significant interobserver variability in subclassification of squamous lesions should be considered in management guidelines for abnormal Papanicolaou test results and implementation of national cytology proficiency testing.
Prostate-Specific Antigen Expression and Lipochrome Pigment Granules in the Differential Diagnosis of Prostatic Adenocarcinoma Versus Seminal Vesicle–Ejaculatory Duct Epithelium
Authors: Shidham Vinod B. , Lindholm Paul F. , Kajdacsy-Balla André , Basir Zainab , George Varghese , Garcia Fernando U.
Published: January 2021

Background.—Lipochrome pigment granules (LPGs) and prostate-specific antigen (PSA) localization have been cited as helpful adjuncts in differentiating atypical histologic patterns of seminal vesicle–ejaculatory duct (SVED) from prostatic adenocarcinoma. However, LPGs have been described in both benign and neoplastic prostatic acini, and PSA expression within the intraprostatic SVED has not been fully explored.

Design.—Fifty radical prostatectomy specimens were studied for LPGs and 9 cases for PSA expression.

Results.—Two morphologic types of LPGs (type 1 and type 2) were observed. The reproducibility in classifying LPGs was evaluated by κ statistics, which demonstrated a strong agreement between 4 observers. Type 1 was restricted to SVED in all 50 specimens. Type 2 was subclassified into 2A and 2B. Type 2 LPGs were observed in prostatic acini of different zones, high-grade prostatic intraepithelial neoplasia, prostatic adenocarcinoma, and occasionally with type 1 LPG in SVED. Focal reactivity for PSA in the distal portion of SVED near urethra was noted in 1 of 9 cases.

Conclusion.—Awareness about morphologic differences between the 2 types of LPGs could help to avoid a potential diagnostic pitfall of misinterpreting SVED epithelium for adenocarcinoma. Caution is recommended in interpreting PSA expression, since rare focal PSA reactivity was observed in the distal SVED.
Evaluation of the Enhanced Amplified Mycobacterium Tuberculosis Direct Test for Direct Detection of Mycobacterium tuberculosis Complex in Respiratory Specimens
Authors: Smith Michael B. , Bergmann John S. , Onoroto Michelle , Mathews Greg , Woods Gail L.
Published: January 2021

Objective.—To evaluate the performance of the enhanced Mycobacterium Tuberculosis Direct Test (E-MTD), for the direct detection of M tuberculosis complex (MTBC) in respiratory specimens.

Design.—Two hundred seventy-four respiratory specimens from 151 patients in respiratory isolation were tested with the E-MTD, and the results were compared with the results of mycobacterial smear, culture, and the earlier form of the test, MTD-1.

Results.—Forty-one specimens were culture positive for mycobacteria (20 MTBC and 21 nontuberculous mycobacteria), 23 of which were smear positive (16 MTBC, 7 nontuberculous mycobacteria). Twenty-four specimens were positive by E-MTD, and 21 were positive by MTD-1. Of the 20 MTBC culture-positive specimens, 19 were positive by the E-MTD and 19 were positive by the MTD-1. The remaining specimens were MTBC negative by all methods. After resolution of discrepancies, the sensitivity, specificity, and positive and negative predictive values were 95.2%, 100%, 100%, 99.6% for the MTD-1 and 95.2%, 98.8%, 87.0%, and 99.6%, for the E-MTD. For the E-MTD smear-positive and smear-negative specimens, these same values were 93.8%, 100%, 100%, and 87.5% and 100%, 98.8%, 62.5%, and 100%, respectively.

Conclusion.—The results suggest that the E-MTD is a reliable method for the direct detection of MTBC in smear-positive respiratory specimens.
Outpatient Order Accuracy
Authors: Valenstein Paul , Meier Frederick
Published: January 2021

Context.—Laboratory test order entry errors potentially delay diagnosis, consume resources, and cause patient inconvenience.

Objective.—To evaluate the frequency and causes of computer order entry errors in outpatients.

Design.—Cross-sectional survey and prospective sample of errors. Participants answered questions about their test order entry policies and practices. They then examined a sample of outpatient requisitions and compared information on the requisition with information entered into the laboratory computer system. Order entry errors were divided into 4 types: tests ordered on the requisition, but not in the computer; tests performed but not ordered on the requisition; physician name discrepancies; and test priority errors.

Participants.—Six hundred sixty laboratories enrolled in the College of American Pathologists Q-Probes program.

Main Outcome Measure.—Overall order entry error rate.

Results.—A total of 5514 (4.8%) of 114 934 outpatient requisitions were associated with at least 1 order entry error. The median participant reported 1 or more order errors on 6.0% of requisitions; 10% of institutions reported errors with at least 18% of requisitions. Of the 4 specific error types, physician name discrepancies had the highest error rate, and test priority errors the lowest error rate. Four institutional factors were significantly associated with higher overall error rates: orders verbally communicated to the laboratory; no policy requiring laboratory staff to compare a printout or display of ordered tests with the laboratory requisitions to confirm that orders had been entered correctly; failure to monitor the accuracy of outpatient order entry on a regular basis; and a higher percentage of occupied beds (ie, a busier hospital).

Conclusions.—Computer order entry errors are common, involving 5% of outpatient requisitions. Laboratories may be able to decrease error rates by regularly monitoring the accuracy of order entry, substituting written and facsimile orders for verbal orders, and instituting a policy in which orders entered into computer systems are routinely rechecked against orders on requisitions.
Protocol for the Examination of Specimens From Patients With Carcinomas and Malignant Melanomas of the Vulva
Authors: Wilkinson Edward J.
Published: January 2021

No Abstract Available
Practice Parameter for the Recognition, Management, and Prevention of Adverse Consequences of Blood Transfusion
Authors: Sazama Kathleen , DeChristopher Phillip J. , Dodd Roger , Harrison Chantal R. , Shulman Ira A. , Cooper E. Shannon , Labotka Richard J. , Oberman Harold A. , Zahn Christopher M. , Greenburg A. Gerson , Stehling Linda , Lauenstein Karla J. , Price Thomas H. , Williams Lee K.
Published: January 2021

No Abstract Available
Criteria for Biopsy Diagnosis of Minimal Volume Prostatic Adenocarcinoma
Authors: Iczkowski Kenneth A. , Bostwick David G.
Published: January 2021

Context.—Minimal volume prostatic adenocarcinoma (defined as involving less than 5% of biopsy tissue) is diagnosed increasingly today because of successful cancer screening. We previously described the diagnostic category called atypical small acinar proliferation (ASAP), suspicious for but not diagnostic of malignancy, present in about 2.5% of routine prostatic needle biopsy specimens.

Objective.—To establish the criteria enabling a distinction between ASAP and cancer.

Design.—We prospectively evaluated clinical and histologic findings from all 319 patients consecutively diagnosed as having ASAP or minimal cancer by prostatic needle biopsy in a consultation service. Seventeen histopathologic features were assessed.

Results.—Fifty-six patients (18%) had ASAP, and 100 (31%) had minimal cancer; the remaining 163 (51%) had benign diagnoses, high-grade prostatic intraepithelial neoplasia, or larger amounts of cancer. The mean age of patients with ASAP did not differ from that of patients with minimal cancer (64.2 years vs 63.3 years; P = .65). In 10 of 17 histopathologic findings, ASAP differed significantly from minimal cancer. Among architectural findings, ASAP foci averaged 0.4 vs 0.8 mm (P < .0001) and comprised a mean of 11 vs 17 acini (P < .0001). Infiltrative growth occurred in 75% of ASAP foci and 100% of minimal cancers (P < .0001). Among cytologic findings, ASAP was significantly less likely than cancer to have mitotic figures (0% vs 10%, respectively; P < .01) or prominent nucleoli in at least 10% of cells (55% vs 100%, respectively; P < .0001) and showed more frequent nuclear hyperchromasia (44% vs 9%, respectively; P < .0001) and less nuclear enlargement (P = .0002). Luminal blue mucin secretions were less common in ASAP than cancer (6% vs 33%, respectively; P < .0001), but eosinophilic granular secretions and crystalloids were about equally frequent. Concomitant high-grade prostatic intraepithelial neoplasia was present in 23% of ASAP cases and 57% of cancers (P < .0001). Moderate-to-severe atrophy confounded 59% of cases with ASAP and 35% of cancers (P = .003); both ASAP foci and cancer were associated with inflammation in about a quarter of cases. In each case with ASAP, we stratified our level of suspicion among 3 categories (favor benign, uncertain, and favor carcinoma). As suspicion increased so did the mean nuclear enlargement and percentage of cases with infiltrative growth and nuclear hyperchromasia (all P < .05).

Conclusions.—These criteria, which differ significantly between ASAP and minimal volume cancer, can help to separate patients for whom a second biopsy is recommended from candidates for prostatectomy or other therapy.
Practical Value of Ki-67 and p53 Labeling Indexes in Stereotactic Biopsies of Diffuse andPilocytic Astrocytomas
Authors: Tihan Tarik , Davis Raphael , Elowitz Eric , DiCostanzo Damian , Moll Ute
Published: January 2021

Objective.—Stereotactic biopsies are increasingly being used for the diagnosis and grading of astrocytomas, and there is a growing need to obtain maximum information from these tissue samples. In everyday practice, p53 protein and Ki-67 immunohistochemical analyses are the most frequently used ancillary studies to aid in diagnosis and grading, but their exact role is not clearly established. This study was undertaken to evaluate the practical value of these markers in stereotactic biopsy samples from diffuse astrocytomas as well as pilocytic astrocytomas.

Methods/Results.—We analyzed the Ki-67 (MIB-1) and p53 labeling indexes in the stereotactic biopsy specimens from 11 pilocytic astrocytomas; 8 grade 2, 15 grade 3, and 16 grade 4 diffuse astrocytomas. Pilocytic astrocytomas and diffuse astrocytomas were evaluated as 2 separate groups. There was a strong correlation with poor outcome when both labeling indexes were higher than 15% in the same tumor for diffuse astrocytomas (P < 0.01). The indexes did not correlate with outcome in pilocytic astrocytomas.

Conclusion.—Combined Ki-67 and p53 labeling indexes higher than 15% indicated a worse outcome than suggested by the histologic grading. The analysis aided or improved histologic evaluation of stereotactic biopsies in our patients. We believe that a realistic prognostic upgrading of diffuse astrocytomas should be made only when labeling indexes for both markers are greater than 15%.
Mixed Ductal-Endocrine Carcinoma of the Pancreas
Authors: Leteurtre Emmanuelle , Brami Fabrice , Kerr-Conte Julie , Quandalle Pierre , Lecomte-Houcke Martine
Published: January 2021

We describe herein a case of a mixed ductal-endocrine pancreatic carcinoma. Rare cases of mixed pancreatic tumors have been described, with endocrine and exocrine components each making up a significant proportion of the neoplasm; to our knowledge, only one case has been reported with a mixed liver metastasis. In our case, ductal and endocrine cells were intimately admixed in the primary tumor and in a peripancreatic lymph node metastasis, diagnosed by standard light microscopy and double immunostaining for cytokeratin 19 and synaptophysin. The endocrine component was immunoreactive for somatostatin. Tumors with admixed endocrine and exocrine components support the hypothesis of a common endodermal histogenesis for the ductal and endocrine cells in the human pancreas.
Aggressive Natural Killer Cell Lymphoma Presenting as an Anterior Mediastinal Mass in a Patient With Acquired Immunodeficiency Syndrome
Authors: Tao Jianguo , Savargaonkar Pratima , Vallejo Christina , Cesarman Ethel , Fuchs Alexander
Published: January 2021

We report a case of aggressive natural killer cell lymphoma presenting as an anterior mediastinal mass in an African-American man with acquired immunodeficiency syndrome. Histologically, the anterior mediastinal mass showed a diffuse dense infiltrate of atypical intermediate-sized and large lymphoid cells, as well as scattered immunoblasts with angiocentric and angiodestructive growth and extensive zonal necrosis. Similar lymphoid infiltrates were present in the patient's lungs, spleen, and bone marrow, accompanied by extensive lymphophagocytosis. Electron microscopic and cytologic examinations showed the presence of dense cytoplasmic granules. Immunophenotyping by flow cytometry and by immunohistochemistry yielded surface markers consistent with a natural killer cell lymphoma. The Epstein-Barr virus genome and monoclonality were detected by in situ hybridization and Southern blot analysis. Polymerase chain reaction confirmed the presence of type A Epstein-Barr virus. T-cell receptor gene rearrangement could not be identified by Southern blot analysis or polymerase chain reaction. To the best of our knowledge, this is the first reported case of designated natural killer cell lymphoma from the mediastinum, as well as the first reported case of natural killer cell lymphoma in a patient with acquired immunodeficiency syndrome. This tumor disseminated early and pursued a highly aggressive course. Epstein-Barr virus may play a role in the pathogenesis of this disease.
Coccidioidomycosis Meningitis With Massive Dural and Cerebral Venous Thrombosis and Tissue Arthroconidia
Authors: Kleinschmidt-DeMasters B. K. , Mazowiecki Michael , Bonds Lian A. , Cohn David L. , Wilson Michael L.
Published: January 2021

To our knowledge we report the first case of meningitis from Coccidioides immitis associated with massive dural and cerebral venous thrombosis and with mycelial forms of the organism in brain tissue. The patient was a 43-year-old man with late-stage acquired immunodeficiency syndrome (AIDS) whose premortem and postmortem cultures confirmed C immitis as the only central nervous system pathogenic organism. Death was attributable to multiple hemorrhagic venous infarctions with cerebral edema and herniation. Although phlebitis has been noted parenthetically to occur in C immitis meningitis in the past, it has been overshadowed by the arteritic complications of the disease. This patient's severe C immitis ventriculitis with adjacent venulitis appeared to be the cause of the widespread venous thrombosis. AIDS-related coagulation defects may have contributed to his thrombotic tendency.
Prophylactic Mastectomy
Authors: Khurana Kamal K. , Loosmann Anne , Numann Patricia J. , Khan Seema A.
Published: January 2021

Background.—According to recently published data, prophylactic mastectomy (PM) appears to prevent about 90% of the expected malignant neoplasms in women with a family history of breast cancer.

Objectives.—To identify the frequency of high-risk lesions in PM specimens and to determine occurrence of any new primary breast cancer following PM.

Design.—We performed a retrospective study of women undergoing unilateral or bilateral PM. Medical charts and pathologic findings of 35 patients who underwent bilateral mastectomies at University Hospital, Syracuse, NY, from 1989 to 1996 were reviewed. Patients with biopsy-proven bilateral breast cancer were excluded. Patients were divided into 3 groups: (A) positive family history and no known breast cancer (n = 9), (B) positive family history and contralateral neoplasia (n = 13), and (C) negative family history and contralateral neoplasia (n = 13). These findings were compared with those found in reduction mammoplasty specimens from 10 women at standard risk of breast cancer.

Results.—The mean age of the control group of women undergoing reduction mammoplasty was 38 years. The pathologic specimens demonstrated no significant pathologic findings in 9 and fibrocystic change in 1. In group A, the mean number of affected relatives was 3.1, and the mean age was 38 years. Two of these 9 women had atypical duct hyperplasia and 1 had atypical lobular hyperplasia in their breasts (ie, 33% with high-risk pathologic findings). Of the 13 group B women (mean age, 46.6 years; mean of 2.5 affected relatives and unilateral breast cancer), the contralateral PM specimen contained duct carcinoma in situ in one and invasive ductal cancer in a second (15% with occult malignant neoplasms). In 13 group C patients (mean age, 47.1 years), 3 (23.1%) of the contralateral PM specimens displayed atypical duct hyperplasia or atypical lobular hyperplasia. At a mean follow-up of 4.8 years, there have been no new breast malignant neoplasms in these 45 women.

Conclusions.—The occurrence of unilateral cancer in patients with family history of breast cancer is associated with a 15.4% probability of simultaneous occult malignant neoplasms in the contralateral breast. Patients with a strong family history but no evidence of breast cancer have a substantially similar rate of proliferative disease in their PM specimens as those women who have unilateral cancer but no significant family history.
Pagetoid Bowen Disease
Authors: Williamson John D. , Colome Maria I. , Sahin Aysegul , Ayala Alberto G. , Medeiros L. Jeffrey
Published: January 2021

Bowen disease is a variant of squamous cell carcinoma in situ. In some cases a pagetoid growth pattern can be observed with cytologically atypical clear cells arranged singly and in nests. The differential diagnosis of pagetoid Bowen disease includes primarily Paget disease and malignant melanoma in situ, as well as other less common entities. Two cases of pagetoid Bowen disease are described, one in a 65-year-old man with a thigh lesion and the other in a 25-year-old man with a lesion in the penile/scrotal region. Neither patient had clinical evidence of an internal malignant neoplasm. In both cases, the neoplastic cells were positive for cytokeratin (CK) 7 and CK 19 and were negative for CK 18, CK 20, carcinoembryonic antigen, GCDFP-15, c-erbB2, S100, and HMB-45. In aggregate, these findings support the diagnosis of pagetoid Bowen disease. Previously, others have shown that CK 7 is an almost invariable marker of Paget disease. Thus, we report these two cases to illustrate that CK 7 can be expressed by pagetoid Bowen disease and should not be a cause of confusion in the differential diagnosis.
Metastatic Female Adnexal Tumor of Probable Wolffian Origin
Authors: Sheyn Irena , Mira Jose L. , Bejarano Pablo A. , Husseinzadeh Nader
Published: January 2021

Female adnexal tumor of probable wolffian origin is a rare neoplasm that can present diagnostic difficulties. We report herein a case of a 60-year-old woman with female adnexal tumor of probable wolffian origin arising within the leaves of a broad ligament and, 5 years later, presenting with metastasis to the liver. The morphologic, immunohistochemical, ultrastructural, and DNA ploidy findings of the original and metastatic tumor, differential diagnoses, and the results of the English-language literature review are presented.
Calcifying Fibrous Pseudotumor of the Neck
Authors: Hoffmann Heather , Beaver Mary Es , Maillard Alberto A. J.
Published: January 2021

Calcifying fibrous pseudotumor is a rare lesion of uncertain histogenesis that has a unique histologic appearance. We report herein a case of a 24-year-old woman with a mass on the right posterior side of the neck. Magnetic resonance imaging with contrast showed a well-circumscribed mass between the right splenius and semispinalis cervicis muscles; the study suggested high collagen content. Simple excision was performed. The histologic findings were diagnostic of calcifying fibrous pseudotumor. Our review of 19 previously reported cases suggests that a good outcome is expected when a diagnosis of calcifying fibrous pseudotumor is made.
Evaluating Laboratory Performance on Quality Indicators With the Six Sigma Scale
Authors: Nevalainen David , Berte Lucia , Kraft Cheryl , Leigh Elizabeth , Picaso Lisa , Morgan Timothy
Published: January 2021

Context.—Laboratory quality indicator data, most often presented and reported as a percentage of variance, may be misleading, inasmuch as variances, and therefore percentages, appear to be low.

Method.—Current data from laboratory quality indicators and national data derived from several years of College of American Pathologists Q-Probes studies were normalized to parts-per-million defects, as commonly practiced in the manufacturing and service industries for benchmarking performance.

Results.—Laboratory data in parts-per-million defects demonstrated opportunities for significant improvements in laboratory performance across the total testing process.

Conclusions.—Historical quality assurance programs do not appear to be significantly improving the total testing process. Manufacturing and service industries are using quality systems strategies, such as ISO 9000 and the Baldridge Award Criteria, to effect improvements in both productivity and cost. Quality system solutions for performance improvement may provide a systematic approach to improving laboratory performance.
Surgical Pathologic Findings of Extratemporal-Based Intractable Epilepsy
Authors: Frater John L. , Prayson Richard A. , Morris Harold H. , Bingaman William E.
Published: January 2021

Background.—Surgical management of intractable epilepsy continues to be important in select cases to achieve seizure control.

Design.—This study retrospectively reviews the pathologic findings in 133 consecutive cases of extratemporal lobe epilepsy experienced during a 17-year period.

Results.—The study group consists of 133 patients (78 males) who underwent extratemporal lobe resection for epilepsy at a mean age of 21.1 years (range, 3 months to 57 years). In 50 patients (37.6%), cortical dysplasia (neuronal migration abnormalities) was identified. The most common patterns of dysplasia observed included diffuse architectural disorganization in 46 cases, neuronal cytomegaly in 30 cases, increased numbers of molecular layer neurons in 30 cases, and balloon cells in 18 cases. Tumors were identified in 37 cases (27.8%) and included 13 astrocytomas, 7 gangliogliomas, 6 dysembryoplastic neuroepithelial tumors, 6 glioneuronal hamartomas, 4 oligodendrogliomas, and 1 oligoastrocytoma (mixed glioma). Twenty-four resections (18%) showed evidence of remote ischemic damage or infarct. Neuronal heterotopia was identified in 59 resection specimens (44.4%). Other less common findings included vascular malformations in 4 patients (3.0%), Sturge-Weber malformations in 3 patients (2.3%), and Rasmussen encephalitis in 2 patients (1.5%). Two patients were known to have tuberous sclerosis. In 23 resection specimens (17.3%), no significant pathologic finding was identified. Coexistent cortical dysplasia and tumor were seen in 10 cases and coexistent dysplasia and infarct or remote ischemic damage in 11 cases.

Conclusion.—This series demonstrates that most patients with extratemporal lobe epilepsy have significant histopathologic findings, which most frequently include cortical dysplasia, tumor, or evidence of remote ischemic damage or infarct. Coexistent pathologic findings were present in a significant minority of cases (16.5%).
The Autopsy Lexicon
Authors: Hanzlick Randy L.
Published: January 2021

Context.—Although standard autopsy texts and other publications discuss the general content of autopsy reports, and some provide examples of autopsy report formats, no publication to date has recommended specific headings for autopsy report organization. The College of American Pathologists Autopsy Committee decided it would be helpful to provide suggestions for autopsy report headings to foster more standardized autopsy reporting, to facilitate review of reports by third parties, and to facilitate searches of electronically stored autopsy reports.

Objectives.—To create a model document (named the Autopsy Lexicon), which defines standard categories of information that are useful to include in autopsy reports; to offer specific wording for the headings of various sections of the report; and to explain the rationale for including the various items of information and headings.

Participants and Methods.—The members of the Autopsy Committee of the College of American Pathologists prepared the document by reviewing various examples of autopsy report formats, identifying specific categories of information usually contained in such reports, and developing wording for various sections of the autopsy report that would contain specific information. A draft was submitted to 45 members (including 12 forensic pathologists) of the College of American Pathologists for review, comment, and reality testing. Reviewers included pathologists from both community and academic settings. Comments of reviewers were incorporated to the extent possible.

Results.—The Autopsy Lexicon was prepared and is a model document for autopsy pathologists who wish to define an autopsy template of headings for consistent organization of autopsy reports.

Conclusions.—The Autopsy Lexicon is available to foster more uniform reporting of autopsy information, which may facilitate review of autopsy reports and retrieval of information from electronically stored autopsy reports.
Malignant Adenomyoepithelioma of the Breast With Malignant Proliferation of Epithelial and Myoepithelial Elements
Authors: Ahmed Atif A. , Heller Debra S.
Published: January 2021

Malignant adenomyoepithelioma of the breast is a rare lesion characterized by malignant proliferation of epithelial and myoepithelial cells that show characteristic histologic and immunohistochemical features. Eleven cases have been reported, 4 of which showed evidence of distant metastasis. The authors report a case of malignant adenomyoepithelioma in the axillary tail of a 71-year-old woman, one of the oldest patients described so far, and review the literature. Malignancy in the current case was evidenced by the presence of local invasion, high mitotic rate, and severe cytologic atypia. The tumor was associated with adenosis and lobular adenomyoepithelial hyperplasia. Malignant adenomyoepithelioma is a rare neoplasm, diagnosable by light microscopy and immunohistochemistry. To date, it has only been reported in women, who ranged in age from 26 to 76 years. Metastases have only been documented in tumors 2.0 cm in diameter or larger.
Follow-up of Abnormal Gynecologic Cytology
Authors: Jones Bruce A. , Novis David A.
Published: January 2021

Objectives.—To measure the percentage of women with abnormal gynecologic cytology who have follow-up within 1 year and to identify patient and laboratory characteristics associated with higher percentages of follow-up.

Design and Setting.—Retrospective identification of patients with abnormal cervicovaginal cytology and identification of the initial clinical follow-up activity during the 12 months following the cytologic diagnosis.

Main Outcome Measure.—Percentage of women receiving follow-up.

Results.—Three hundred six laboratories reported follow-up information on 16 132 patients with gynecologic cytology diagnoses of carcinoma, high-grade squamous intraepithelial lesion, low-grade squamous intraepithelial lesion, or glandular intraepithelial lesion. The following percentages of women received follow-up within 1 year: 85.6% of patients with cytologic diagnoses of carcinoma, 87.2% with diagnoses of high-grade squamous intraepithelial lesion, 82.7% with diagnoses of low-grade squamous intraepithelial lesion, and 84.9% with diagnoses of glandular intraepithelial lesion. Within 6 months, 82.2% of patients with cytologic diagnoses of carcinoma, 82.4% with diagnoses of high-grade squamous intraepithelial lesion, 71.9% with diagnoses of low-grade squamous intraepithelial lesion, and 74.7% with diagnoses of glandular intra-epithelial lesion received follow-up. Overall, 90.8% of patients who received follow-up within the 1-year time frame of this study had their follow-up completed within 6 months. Specific follow-up activities and their frequencies are listed for each diagnostic category. Patients 30 years old or younger and pregnant patients had lower follow-up percentages.

Conclusions.—With less than 83% of patients with high-grade squamous intraepithelial lesion or carcinoma cytology findings having available documentation of follow-up within 6 months, and less than 88% within 1 year, there is room for improvement in this area of health care. Monitoring and critical analysis of the follow-up process is a starting point for improvement.
Synchronous Occurrence of Epithelial and Stromal Tumors in the Stomach
Authors: Maiorana Antonio , Fante Rossella , Cesinaro Anna Maria , Fano Rita Adriana
Published: January 2021

Objective.—The synchronous development of epithelial and stromal tumors in the stomach has been reported rarely in the literature. A series of 6 such cases is described in this article.

Methods.—Clinical and pathologic data were recorded and the literature was reviewed.

Results.—Five cases featured the simultaneous occurrence of stromal tumors (1 benign, 3 borderline, 1 malignant) and adenocarcinomas, whereas the stromal tumor in the sixth case was found in association with a carcinoid. No collision tumors were observed. In 2 cases, tumors arose from the same site and were closely juxtaposed, but in 4 patients they developed from different areas of the stomach. A preoperative histologic diagnosis of both tumors was not achieved in any case. Two patients harbored occult infiltrative epithelial lesions (1 diffuse-type adenocarcinoma, 1 carcinoid), which were detected only at pathologic examination of the gastric mucosa adjacent to the stromal tumor.

Conclusions.—The simultaneous occurrence of epithelial and stromal tumors in the stomach can be less rare than usually expected. Coincidence alone could account for such an association, particularly in areas with high incidence rates of gastric cancer. The hypothesis that a single carcinogenic agent might interact with two neighboring tissues in the stomach inducing the development of tumors of different histotype cannot be theoretically discarded.
Non-Hodgkin Malignant Lymphoma With Tissue Neutrophilia
Authors: Badve Sunil , Blumstein Andrea , Wiernik Peter , Ratech Howard
Published: January 2021

Context.—Neutrophils, in the absence of necrosis, are uncommon in non-Hodgkin malignant lymphoma. Recently, a neutrophil-rich type of Ki-1 (CD30)-positive, anaplastic large cell lymphoma was described.

Objectives.—We report 3 cases of nonanaplastic large cell lymphoma with an abundance of tissue neutrophils; 2 cases were associated with breast carcinoma and possible infection.

Results.—Peripheral blood neutrophilia was noted in only 1 of these 3 patients. Neutrophilia in the lymph nodes occurred in either a sinusoidal or interstitial pattern. Multiple biopsies were available for review in 2 patients; however, tissue neutrophilia was present in only 1 biopsy each.

Conclusion.—These findings suggest that nonanaplastic large cell lymphoma–related tissue neutrophilia is a transient phenomenon.
Declining Autopsy Rate in a French Hospital
Authors: Chariot Patrick , Witt Karine , Pautot Vivien , Porcher Raphaël , Thomas Guy , Zafrani Elie Serge , Lemaire François
Published: January 2021

Context.—Autopsy rates have been declining throughout the world, although preservation of the autopsy is considered a fundamental principle of medical care. In France, the 1994 bioethics law requires physicians to inform relatives before performing an autopsy.

Objective.—To analyze the following factors that potentially influence hospital autopsy rates: legal constraints, autopsy reporting times, opinions of physicians requesting autopsies and pathologists regarding the usefulness of autopsy in patient care, and use of autopsy material in research publications.

Design.—Record of the annual numbers of deaths and autopsies during a 10-year period (1988–1997). Record of the delays for transmission of final autopsy report to the requesting physician. Questionnaire analyzing the possible factors influencing autopsy rate. Categorization of articles published by pathologists according to the use of autopsy material.

Setting.—A 1000-bed, university teaching hospital in the Paris, France, area.

Participants.—Questionnaire addressed to physicians, head nurses, and mortuary staff.

Results.—A total of 1454 autopsies were reviewed. The autopsy rate declined from 15.4% in 1988 to 3.7% in 1997. This decline was marked after 1994 and tended to be slower for neurologic indications than for other indications. The final report had not been communicated within 180 days in 620 (42.6%) of 1454 autopsies. Fifty-five of 105 respondents considered that the bioethics law was one cause of the recent decrease of autopsy rate. Considering the contribution of autopsy to medical research, 94 (81%) of 116 articles dealing with central nervous system but only 28 (6%) of 464 articles dealing with other organs used autopsy-derived material.

Conclusions.—The 1994 bioethics law seems to contribute to the decline of autopsy. Inadequate delays for communicating autopsy results are frequent. Except for neuropathologists, autopsy is a minor source of research material.
Unilocular Retroperitoneal Cyst of Mesothelial Origin Presenting as a Renal Mass
Authors: Smith Vincent C. , Edwards Robert A. , Jorgensen Jeffrey L. , Goldfarb Richard A. , Kadmon Dov , Cagle Philip , Truong Luan D.
Published: January 2021

We report the first 2 cases, to our knowledge, of retroperitoneal cysts with features of mesothelial differentiation that clinically mimic renal masses. The first lesion occurred in a 71-year-old man who presented with flank pain. Ultrasound and magnetic resonance imaging studies showed a unilocular cystic structure arising from the upper pole of the left kidney. The second lesion was in a 44-year-old woman who presented with left flank pain. Imaging studies revealed an 8-cm hemorrhagic cyst at the lower pole of the left kidney. Histologic examination of the nephrectomy specimens in each case revealed a unilocular cyst with intracystic and pericystic hemorrhage. In each case, the cyst was lined by a single layer of cells with ample eosinophilic cytoplasm and benign nuclear features without mucinous or müllerian differentiation. Histochemical staining showed Alcian blue positivity on the cell surface, which was sensitive to hyaluronidase digestion. Intracytoplasmic mucin, however, was not detected. Immunostaining showed that the cyst lining cells were positive for keratin, vimentin, HBME-1, WT1, and thrombomodulin but negative for carcinoembryonic antigen, B72.3, Leu-M1, and BerEP4. The first case was positive for calretinin, whereas the second was negative. These findings support the mesothelial nature of the cysts.
Prognostic Factors in Prostate Cancer
Authors: Bostwick David G. , Grignon David J. , Hammond M. Elizabeth H. , Amin Mahul B. , Cohen Michael , Crawford David , Gospadarowicz Mary , Kaplan Richard S. , Miller Daniel S. , Montironi Rodolfo , Pajak Thomas F. , Pollack Alan , Srigley John R. , Yarbro John W.
Published: January 2021

Background.—Under the auspices of the College of American Pathologists, a multidisciplinary group of clinicians, pathologists, and statisticians considered prognostic and predictive factors in prostate cancer and stratified them into categories reflecting the strength of published evidence and taking into account the expert opinions of the Prostate Working Group members.

Materials and Methods.—Factors were ranked according to the previous College of American Pathologists categorical rankings: category I, factors proven to be of prognostic importance and useful in clinical patient management; category II, factors that have been extensively studied biologically and clinically but whose importance remains to be validated in statistically robust studies; and category III, all other factors not sufficiently studied to demonstrate their prognostic value. Factors in categories I and II were considered with respect to variations in methods of analysis, interpretation of findings, reporting of data, and statistical evaluation. For each factor, detailed recommendations for improvement were made. Recommendations were based on the following aims: (1) increasing uniformity and completeness of pathologic evaluation of tumor specimens, (2) enhancing the quality of data collected pertaining to existing prognostic factors, and (3) improving patient care.

Results and Conclusions.—Factors ranked in category I included preoperative serum prostate-specific antigen level, TNM stage grouping, histologic grade as Gleason score, and surgical margin status. Category II factors included tumor volume, histologic type, and DNA ploidy. Factors in category III included perineural invasion, neuroendocrine differentiation, microvessel density, nuclear roundness, chromatin texture, other karyometric factors, proliferation markers, prostate-specific antigen derivatives, and other factors (oncogenes, tumor suppressor genes, apoptosis genes, etc).
The Oncologic Pathology Report
Authors: Zarbo Richard J.
Published: January 2021

Objective.—To review tenets of basic quality assurance and identify opportunities for quality improvement in the laboratory assessment of prognostic factors.

Design.—College of American Pathologists Q-Probes data obtained from hundreds of American laboratories throughout the 1990s are used to illustrate laboratory performance and practice opportunities in the preanalytic, analytic, and postanalytic phases of the total testing process.

Results.—A wide range of performance deficiencies are documented, most in the preanalytic and postanalytic phases, and contributed by individuals outside and not under the control of the laboratory. In the analytic phase, the effectiveness of checklists on the content and completeness of reported diagnostic and prognostic data for breast and colon cancers is proven.

Conclusions.—In the preanalytic phase, marked enhancements in quality would result from improvements in (1) patient and specimen identification, (2) adherence to patient-sampling and specimen-handling requirements, and (3) communication to the laboratory of the pertinent clinical context of the individual test. Once basic analytic quality control and laboratory quality assurance issues are resolved, a focus on standardization and enhancement of preanalytic and postanalytic communications and satisfaction of clinical expectations becomes the source of improved laboratory performance.
Clinicians Are From Mars and Pathologists Are From Venus
Authors: Powsner Seth M. , Costa José , Homer Robert J.
Published: January 2021

Context.—Text reports convey critical medical information from pathologists, radiologists, and subspecialty consultants. These reports must be clear and comprehensible to avoid medical errors. Pathologists have paid much attention to report completeness but have ignored the corresponding issue of report comprehension. This situation presents an increasingly serious potential problem. As laboratories are consolidated and as reports are disseminated in new ways (eg, via the World Wide Web), the target audience becomes more diverse and less likely to have any contact with pathologists beyond the written reports themselves.

Objective.—To compare clinician comprehension with pathologist intent in written pathology reports.

Methods.—Typical surgical pathology reports relevant to surgeons and covering a range of specimen complexity were taken from our hospital files. Questionnaires based on these cases were administered open-book-examination style to surgical attending physicians and trainees during surgical conferences at an academic medical center.

Main Outcome Measures.—Scores from questionnaires.

Results.—Surgeons misunderstood pathologists' reports 30% of the time. Surgical experience reduced but did not eliminate the problem. Streamlined report formatting exacerbated the problem.

Conclusions.—A communication gap exists between pathologists and surgeons. Familiarity with report format and clinical experience help reduce this gap. Paradoxically, stylistic improvements to report formatting can interfere with comprehension and increase the number of misunderstandings. Further investigation is required to reduce the number of misunderstandings and, thus, medical errors.
Policies and Procedures Related to Weak D Phenotype Testing and Rh Immune Globulin Administration
Authors: Domen Ronald E.
Published: January 2021

Objective.—To determine and evaluate policies and procedures related to weak D phenotype testing and terminology and the administration of Rh immune globulin in selected clinical situations.

Design, Setting, and Participants.—Institutions participating in the College of American Pathologists 1999 J-A Comprehensive Transfusion Medicine Survey program were asked to respond to a series of supplementary questions related to weak D phenotype testing and Rh immune globulin administration. More than 3500 institutions and transfusion services participated.

Results.—Most supplementary questions elicited more than 3000 responses. Despite no clinical or regulatory mandate, 58.2% of transfusion services routinely perform an antiglobulin test for the weak D phenotype in patients who test negative with anti-D reagents. Significant differences were found concerning the transfusion of blood components to patients with the weak D phenotype and the administration of Rh immune globulin to these individuals. At least one patient with the weak D phenotype with anti-D alloantibody formation was observed during a 12-month period by 31.8% of transfusion services.

Conclusions.—Significant variability concerning policies and procedures related to weak D typing and terminology was found in this survey. Transfusion of blood components to patients with the weak D phenotype and the administration of Rh immune globulin also demonstrated variations. Anti-D alloantibody formation by patients with the weak D phenotype may not be as rare as previously thought. Additional study related to the clinical significance of these results is warranted.