bluuebunny/crossref_metadata_2025_split huggingface dataset:
Intramuscular Juvenile Xanthogranuloma in an Adult
Authors: Kuo Fang-Ying , Eng Hock-Liew , Chen Shih-Hao , Huang Hsuan-Ying 
Published: February 2021

Abstract
Juvenile xanthogranuloma (JXG) is a self-limited cutaneous lesion that most often occurs in infancy. Approximately 10% to 30% of JXGs occur in adult patients, where most cases present as cutaneous papulonodular lesions, with only rare cases involving extracutaneous sites. Intramuscular JXG is extremely rare and has received little attention. On review of the literature, all of the 6 previously reported intramuscular JXGs were noted in the pediatric population. The authors hereby describe a case of adult intramuscular JXG that occurred in a woman who initially had a dermal JXG in the nasal skin at the age of 48 years and then developed a slow-growing painless intramuscular JXG in the right forearm 4 years later. Both the dermal and intramuscular lesions revealed similar histologic features and consisted of diffuse infiltrates of histiocytes with eosinophilic and foamy cytoplasm, lymphocytes, eosinophils, and Touton giant cells in varying proportions. However, central fibrosis and a focal storiform arrangement of spindled fibroblast-like cells in the intramuscular lesion resulted in a histologic pattern reminiscent of a fibrous histiocytoma. Immunohistochemically, the intramuscular JXG was positive for CD68, factor XIIIa, CD31, and vimentin. This case underscores the fact that intramuscular JXG can also involve adult patients and its morphologic variation is more likely to be time dependent rather than site specific or age related.
Fulminant Multisystem Non–Langerhans Cell Histiocytic Proliferation With Hemophagocytosis
Authors: Rao R. Nagarjun , Chang Chung-che , Uysal Nevin , Presberg Kenneth , Shidham Vinod B. , Tomashefski Joseph F. 
Published: February 2021

Abstract
Hemophagocytosis (HP), a feature seen in malignant histiocytosis and infection- and lymphoma-associated disorders, has not been previously emphasized in Erdheim-Chester disease (ECD). Generally, ECD is recognized as a rare, systemic, non–Langerhans cell histiocytosis with a variable clinical course. Herein, we describe a unique case of multisystem non–Langerhans cell histiocytic proliferation with a fulminant clinical course (death occurred within 3 months of presentation) that showed prominent HP and extensive involvement of multiple organs, including the lungs, resulting in respiratory failure. Hemophagocytosis led to severe anemia that required transfusion and thrombocytopenia. Antemortem lung and bone marrow biopsy specimens revealed involvement by a histiocytic infiltrate with features highly suggestive of ECD and HP. Furthermore, the autopsy documented the presence of HP and the histiocytic infiltrate in multiple other organs. This case is best categorized as a variant form of ECD. Recognizing this variant has the following important implications: (1) HP may be a marker for fulminant clinical course in ECD, (2) the presence of HP does not exclude a diagnosis of ECD, and (3) ECD should be considered in the differential diagnosis of HP.
Rhabdoid Tumor of the Thyroid Gland: A Variant of Anaplastic Carcinoma
Authors: Lai Maria Letizia , Faa Gavino , Serra Stefano , Senes Giancarlo , Daniele Giovanni M. , Boi Francesco , Mariotti Stefano , Beauchemin Michel , Asa Sylvia L. 
Published: February 2021

Abstract
Rhabdoid tumor of the thyroid gland is a very rare neoplasm, characterized by significant metastatic potential. All of the 6 cases reported in the recent literature had poor outcomes. We report an additional case involving, to our knowledge, the oldest patient reported so far. A 67-year-old woman had a nodular goiter for all of her adult life and presented with a rapidly growing mass in the right lobe. Histologic examination showed a highly cellular neoplasm with a solid infiltrative growth pattern. Extracapsular invasion was evident. Rhabdoid cells were large, with abundant cytoplasm, eosinophilic inclusions, and eccentric nuclei containing distinct nucleoli. Immunohistochemistry identified vimentin, sarcomeric actin, myoglobin, and cytokeratin expression in the tumor cells; they were negative for desmin, thyroglobulin, and calcitonin. Scattered follicles with nuclear features of papillary thyroid carcinoma were detected; these cells were immunoreactive for thyroglobulin and TTF-1. Reverse transcriptase polymerase chain reaction using specific primers for RET/PTC1 and RET/PTC3 fusion genes identified a RET/PTC3 gene rearrangement in the rhabdoid tumor. Despite radiotherapy, the neoplasm rapidly progressed, with massive local and mediastinal metastasis leading to death 5 months after presentation. The hypothesis that rhabdoid tumor is a variant of anaplastic thyroid carcinoma is supported by the identification of a RET/PTC gene rearrangement, a feature of carcinomas of follicular cell derivation.
Renal Failure and Nephrocalcinosis Associated With Oral Sodium Phosphate Bowel Cleansing: Clinical Patterns and Renal Biopsy Findings
Authors: Gonlusen Gulfiliz , Akgun Hulya , Ertan Atilla , Olivero Juan , Truong Luan D. 
Published: February 2021

Abstract
Acute renal failure (ARF) is rarely reported after bowel preparation with sodium phosphate. We report a patient with mild Crohn disease (in remission), without history of renal disease, and with normal baseline renal function, who developed ARF 14 days after bowel preparation for colonoscopy with oral sodium phosphate. A renal biopsy showed multifocal calcium phosphate deposition in the renal tubules against a background of diffuse chronic tubulointerstitial injury. Review of the literature suggested 2 distinct patterns of ARF in the context of sodium phosphate bowel cleansing. One pattern is characterized by ARF, which develops a few hours or days after sodium phosphate administration, as a component of a systemic syndrome associated with severe hyperphosphatemia and hypocalcemia. Correction of these electrolyte abnormalities was frequently associated with rapid recovery of renal function. The cause of ARF in this context was not clear because the favorable outcome negated the need for renal biopsy. In the second pattern, exemplified by the current patient, ARF was identified incidentally. These patients did not have any features of an acute syndrome immediately after sodium phosphate administration and presented much later (usually weeks) with mild, nonspecific symptoms. At the time of presentation, the serum calcium and phosphate levels were normal. The renal biopsies in each of these patients showed nephrocalcinosis as the possible cause of ARF. The renal failure improved at least partially in most of these patients, but persisted in rare cases.
Interobserver Agreement in Hepatitis C Grading and Staging and in the Banff Grading Schema for Acute Cellular Rejection: The “Hepatitis C 3” Multi-Institutional Trial Experience
Authors: Netto George J. , Watkins David L. , Williams James W. , Colby Thomas V. , dePetris Giovanni , Sharkey Francis E. , Corless Christopher L. , Lewin David , Petrovic Lydia , Sharma Shobha , Kanel Gary , Theise Neil , West A. Brian , Koehler Alison , Jhala Nirag C. , Lefkowitch Jay , Lezzoni Julia , Jennings Linda W. , Tillery G. Weldon , Klintmalm Goran B. 
Published: February 2021

Abstract
Context.—Establishing adequate interobserver agreement is crucial not only for standardization of patient care but also to ensure validity of findings in multi-institutional trials.
Objective.—To evaluate interobserver agreement in assessing chronic hepatitis C (HCV) and acute cellular rejection (ACR) among 17 hepatopathologists involved in the “Hepatitis C 3” trial.
Design.—The trial is a randomized multicenter (17 institutions) study involving 312 patients undergoing transplantation for HCV. Patients are randomized to 3 treatment arms. For final data analysis, all biopsy specimens are reviewed by a central pathologist (G.J.N.). Recurrence of HCV is evaluated according to the Batts and Ludwig schema. The 1997 Banff schema is used to evaluate ACR. To assess interobserver agreement, hematoxylin-eosin–stained sections from 11 liver biopsy specimens (6 HCV and 5 ACR) were sent by the central pathologist to 16 local pathologists from 13 institutions. Statistical analysis was performed on raw ACR/HCV data as well as data grouped according to clinically significant primary endpoint cutoffs.
Results.—Statistically significant agreement was found among all participating pathologists (P &lt; .001). On κ analysis, the degree of agreement was rated “moderate” for HCV grade and stage and ACR global grading (κ = 0.30, 0.33, and 0.37, respectively). Interobserver agreement was weaker for rejection activity index scoring of ACR (κ = 0.15). A stronger degree of agreement was found when scores were grouped based on endpoint cutoffs (κ = 0.76 “almost perfect” for HCV and 0.62 “substantial” for ACR).
Conclusions.—An overall statistically significant interobserver agreement was found among 17 pathologists using the 1997 Banff schema and the Batts and Ludwig schema.
Histopathologic Findings of Malformations of Cortical Development in an Epilepsy Surgery Cohort
Authors: Morris E. Brannon , Parisi Joseph E. , Buchhalter Jeffrey R. 
Published: February 2021

Abstract
Context.—Malformations of cortical development (MCDs) are an important cause of pharmacoresistent epilepsy and are frequently diagnosed in surgical pathology. The lack of uniform tissue processing and standard histopathologic nomenclature to describe MCDs has resulted in diagnostic ambiguity.
Objective.—To describe the immunohistochemical findings of MCDs from a relatively large surgical epilepsy cohort and incorporate terminology that more adequately reflects the histopathologic findings into a contemporary classification of MCD.
Design.—Utilizing the Mayo Clinic Rochester Surgical Pathology Database and patient records, 53 patients with previous intractable epilepsy and a known malformation of cortical development were identified. All of the cohort's paraffin embedded surgical specimens were resectioned and stained with hematoxylin-eosin, Luxol fast blue/cresyl violet, neurofilament protein, and glial fibrillary acidic protein. Each specimen was reviewed histologically and categorized according to a proposed focal MCD classification scheme that substitutes cytoarchitectural dysmorphism for cortical dysplasia and architectural disorganization for microdysgenesis.
Results.—An MCD was recognized in 49 patients and grouped into 1 of the following 4 categories: (1) cytoarchitectural dysmorphism with balloon cells (n = 19), (2) cytoarchitectural dysmorphism without balloon cells (n = 12), (3) architectural disorganization (n = 8), or (4) polymicrogyria (n = 9).
Conclusions.—The histopathologic features of focal MCD in a large epilepsy surgical cohort by using practical immunohistochemistry and a contemporary MCD classification scheme are described. It is proposed that the term focal cortical dysplasia be renamed as focal malformations of cortical development.
Allelic Loss of 3 Different Tumor Suppressor Gene Loci in Benign and Malignant Endothelial Tumors of the Head and Neck
Authors: Domfeh Akosua B. , Fichera Michelle , Hunt Jennifer L. 
Published: February 2021

Abstract
Context.—Angiosarcomas are rare malignancies that commonly arise in the head and neck. No definitive precursor lesion or etiological link between hemangiomas and angiosarcoma has been postulated. Evidence at the cell culture level suggests that loss of heterozygosity of 13q might be involved in tumorigenesis of endothelial cells. Although overexpression of p53 and WT-1 has been found in angiosarcoma, little is known about the molecular changes involved.
Objective.—This study compared the molecular profile of angiosarcoma with that in some benign vascular lesions.
Design.—Specimens from 6 cases of angiosarcoma, 5 of hemangioma, and 5 of granulation tissue were microdissected. DNA extraction and polymerase chain reaction amplification for 11 microsatellite markers on chromosomes 11p13, 13q14, and 17p13 (WT-1, RB, p53) were performed. Loss of heterozygosity of amplification products was assessed by capillary electrophoresis. Allelic loss and fractional allelic loss were calculated.
Results.—The mean fractional allelic loss was 43% for angiosarcomas and 29% for hemangiomas. Eighty-three percent of angiosarcomas had allelic loss at 17p13, 66% at 13q14, and 50% at 11p13. Allelic loss was seen in 60% of hemangiomas at 13q14 and 17p13, but only in 20% at 11p13. Two cases of hemangioma and all the granulation tissue cases had no allelic losses.
Conclusions.—In our study, 11p13, 13q14, and 17p13 allelic losses were present in both hemangiomas and angiosarcomas. This supports the cell culture–based theories that tumorigenesis in endothelial cells likely involves these chromosomes and provides some insight into the potential pathogenesis of angiosarcomas and benign hemangiomas.
Mesenchymal Hamartoma of the Liver With an Interstitial Deletion Involving Chromosome Band 19q13.4: A Theory as to Pathogenesis?
Authors: Talmon Geoffrey A. , Cohen Samuel M. 
Published: February 2021

AbstractMesenchymal hamartoma of the liver is the third most common liver tumor of infancy. The lesion is primarily mesenchymal in origin, believed to arise from a developmental abnormality in the formation of ductal plates during late embryogenesis. Several studies have revealed a recurrent translocation in mesenchymal hamartomas, all involving a breakpoint at chromosome band 19q13.4. We present a case with a deletion involving this locus. A 10-month-old girl presented with an asymptomatic abdominal mass. The 21.0-cm tumor arose from the surface of the liver. The cut surfaces displayed multiple cysts with intervening loose fibrous tissue. Microscopically, the tumor was mostly composed of edematous connective tissue with intervening cysts, islands of hepatocytes, and haphazard branching bile ducts. Cytogenetic analysis revealed an interstitial deletion on the long arm of chromosome 19(q13.1q13.4). The patient was discharged on postoperative day 4 without complications. The cytogenetic findings support the involvement of an alteration of a gene at chromosome band 19q13.4 in the pathogenesis of mesenchymal hamartoma of the liver.
Medical Examiners, Coroners, and Public Health: A Review and Update
Authors: Hanzlick Randy 
Published: February 2021

AbstractContext.—Traditionally, the emphasis of work done by medical examiners, coroners, and the death investigation community has been viewed as serving the criminal justice system. During the last several decades, however, an important role for these 3 groups has emerged within public health.Objective.—To provide important background information on death investigation systems, the evolution and framework of public health entities that rely on information gathered by medical examiners and coroners, and the role of medical examiners and coroners in epidemiologic research, surveillance, and existing public health programs and activities.Data Sources.—Previous articles on epidemiologic aspects of forensic pathology and the role of medical examiners and coroners in epidemiologic research and surveillance; a review of the Web sites of public health and safety agencies, organizations, and programs that rely on medical examiner and coroner data collected during medicolegal investigations; and a review of recent public health reports and other publications of relevance to medical examiner and coroner activities.Conclusions.—The role of medical examiners and coroners has evolved from a criminal justice service focus to a broader involvement that now significantly benefits the public safety, medical, and public health communities. It is foreseeable that the public health role of medical examiners and coroners may continue to grow and that, perhaps in the not-too-distant future, public health impact will surpass criminal justice as the major focus of medicolegal death investigation in the United States.
Upper Aerodigestive Tract Squamous Dysplasia: Correlation With p16, p53, pRb, and Ki-67 Expression
Authors: Wayne Sigrid , Robinson Robert A. 
Published: February 2021

Abstract
Context.—Differential expression of cell cycle–associated proteins may correlate with human papillomavirus status and may help delineate degree of dysplasia in upper aerodigestive tract squamous lesions.
Objective.—To determine intraepithelial height of immunohistochemical staining for p16, p53, pRb, and Ki-67 in upper aerodigestive tract lesions with reference to degree of dysplasia. Human papillomavirus status was ascertained to correlate with p16 expression.
Design.—Biopsy specimens of 53 squamous dysplastic and 13 keratotic/hyperplastic lesions were immunohistochemically stained for p16, p53, pRb, and Ki-67. Mean height and proportion of positive staining were quantified and compared for keratotic/hyperplastic, mild, moderate, and severe dysplasia/carcinoma in situ. DNA extracted from paraffin-embedded blocks was evaluated for human papillomavirus by polymerase chain reaction amplification using consensus primers for mucosal viral types.
Results.—Height of Ki-67 staining within the epithelium increased significantly between mild and moderate and between mild and severe dysplasia, with a trend toward increasing height between moderate and severe dysplasia. The percentage of cases with p16 staining decreased significantly with increasing degree of dysplasia; however, no significant trend in staining height or proportion was observed with p16, p53, or pRb in relation to degree of dysplasia, or between dysplastic and keratotic/hyperplastic lesions. Only 1 of 53 dysplastic lesions was positive for human papillomavirus.
Conclusions.—This series of upper airway dysplastic mucosal lesions demonstrated a positive correlation between intraepithelial Ki-67 staining height and degree of dysplasia, offering a diagnostic aid in delineating degree of dysplasia in difficult squamous lesions.
Morphologic Examination of Sequential Bone Marrow Biopsies after Nonmyeloablative Stem Cell Transplantation Complements Molecular Studies of Donor Engraftment
Authors: Lagoo Anand S. , Gong Jerald Z. , Stenzel Timothy T. , Goodman Barbara K. , Buckley Patrick J. , Chao Nelson J. , Gasparetto Cristina , Long Gwynn D. , Rizzieri David A. 
Published: February 2021

Abstract
Context.—Nonmyeloablative stem cell transplantation (NMSCT) is a mode of immunotherapy increasingly employed in treating hematologic, lymphoid, and solid tumors. Patients are monitored principally by molecular analysis of donor engraftment.
Objective.—To determine the role of morphologic examination of bone marrow after NMSCT.
Design.—Seventy-three patients undergoing NMSCT under the Campath 1H (humanized anti-CD52 antibody) protocol were studied. Pretransplant and sequential posttransplant bone marrow specimens were evaluated and the findings were correlated with corresponding engraftment data.
Results.—Pretransplant bone marrow specimens from 43% of the patients were involved by disease, and these marrow specimens were significantly more cellular than those that were free of disease. Morphologically detectable disease was still present in day 14 posttransplant marrow specimens in more than one half of these patients, but there was no difference in engraftment in those with or without marrow disease. Early posttransplant marrow in nearly one half of the patients showed myeloid hyperplasia and atypical localization of immature myeloid precursors. Marrow cellularity for the first 2 months after NMSCT was significantly lower in those patients receiving stem cells mismatched at 1 to 3 loci as compared with those who received fully matched grafts (mean cellularity, 38.1% vs 54.1% at day 14). Marrow failure without recurrent disease at 3 to 6 months after transplant was detected by engraftment study in only approximately 15% of cases. Similarly, early recurrence of disease was detected first by morphologic examination in 4 of 13 cases before a decline in donor engraftment occurred.
Conclusion.—Morphologic examination of bone marrow provides additional information that is complementary to donor engraftment analysis for optimal management after NMSCT.
Evaluation of C4d Staining in Liver and Small Intestine Allografts
Authors: Troxell Megan L. , Higgins John P. , Kambham Neeraja 
Published: February 2021

Abstract
Context.—Antibody-mediated humoral rejection in kidney and heart allografts is well recognized and is often associated with poor outcome. C4d deposition in allograft biopsy specimens occurs at sites of antibody-mediated complement activation and has become one of the histopathologic criteria for diagnosis of humoral rejection in the kidney and the heart.
Objective.—To study immunohistochemical C4d staining as a potential diagnostic marker in liver and small intestine allograft biopsy specimens.
Design.—Thirty-six small intestine and 71 liver specimens, including native specimens, allografts with and without histologic features of acute cellular rejection, and explants, were stained with antisera to C4d using an immunohistochemical method on formalin-fixed, paraffin-embedded tissue.
Results.—In small intestine, C4d labeled capillaries in 27% of cases with no evidence of rejection, 36% of cases with evidence of acute rejection, and 2 (28%) of 7 specimens of native normal small intestine. In liver allograft biopsy specimens, C4d stained endothelium of veins, arteries, and/or sinusoids in 2 (8%) of 25 cases of acute rejection with central vein involvement; C4d staining was negative in biopsy specimens with no evidence of rejection. C4d stained the endothelium in a subset of explanted liver allografts with ductopenic rejection or chronic vascular rejection and strongly stained 1 explant with features of hyperacute rejection.
Conclusions.—The clinical utility of C4d staining in solid organ transplantation may vary by organ. Our data show C4d is unlikely to have utility in small intestine allograft biopsy specimens; however, further study in liver allografts, in conjunction with donor-specific antibody testing, is warranted.
Fetiform Teratoma (Homunculus)
Authors: Weiss Jason R. , Burgess Jeanette R. , Kaplan Keith J. 
Published: February 2021

Abstract
Fetiform teratoma (homunculus) is a term that has been given to a rare form of ovarian teratoma that resembles a malformed fetus. There are very few reported cases of this entity in the English language literature. In this report, we document a case of fetiform teratoma in a 23-year-old woman, gravida 0, who initially presented with a chief complaint of dyspareunia. The clinical and pathologic aspects of this rare entity are presented here, with a review of the English literature. Differentiating fetiform teratoma from the more highly developed fetus-in-fetu and ectopic pregnancy is also discussed.