Undifferentiated Embryonal Sarcoma of the Liver With Rhabdoid Morphology Mimicking Carcinoma: Expanding the Morphologic Spectrum or a Distinct Variant?

2021 ◽  
pp. 109352662110189
Author(s):  
David J Papke ◽  
Adam S Fisch ◽  
Sarangarajan Ranganathan ◽  
Allison O’Neill ◽  
Micheál Breen ◽  
...  
Keyword(s):  
Tp53 Mutation ◽  
Mesenchymal Hamartoma ◽  
High Grade ◽  
Micro Rnas ◽  
Cytogenetic Testing ◽  
Poorly Differentiated ◽  
Chromosome 19Q13 ◽  
Rhabdoid Features ◽  
Embryonal Sarcoma ◽  
Specific Cluster

Undifferentiated embryonal sarcoma of the liver (UESL) is a rare aggressive neoplasm that occurs predominantly in children. Like mesenchymal hamartoma of the liver (MHL), UESL harbors recurrent rearrangements involving 19q13.3 and 19q13.4, a region of the genome that contains a primate-specific cluster of micro-RNAs. Here, we present a case of a high-grade neoplasm that arose in the left hepatic lobe of a 5-year-old male and gave rise to widespread lymph node, visceral, and soft tissue metastases. The tumor was composed of sheets, tubules, and papillae of epithelioid cells with rhabdoid morphology. INI1 and BRG1 expression were retained. Tumor cells diffusely expressed epithelial markers, including multiple keratins. While the morphologic and immunophenotypic features were suggestive of poorly differentiated carcinoma with rhabdoid features, the tumor was found to harbor the t(11;19)(q13;q13.3) translocation characteristic of UESL, as well as a TP53 mutation. Given the clinical presentation, imaging, clinical course, the tumor was classified as UESL with unusual, carcinoma-like histopathologic features. In the context of an unclassified high-grade hepatic tumor in a young child, molecular or cytogenetic testing for chromosome 19q13 alterations should be considered.

High-grade urothelial carcinoma of the bladder with rhabdoid features: cytological and histological report of two cases

Cytopathology ◽  
2014 ◽  
Vol 26 (1) ◽  
pp. 54-56 ◽  
Author(s):  
A. Kagotani ◽  
M. Ishida ◽  
K. Yoshida ◽  
M. Iwai ◽  
H. Okabe
Keyword(s):  
Urothelial Carcinoma ◽  
High Grade ◽  
Carcinoma Of The Bladder ◽  
Rhabdoid Features

CDX2 Is a Useful Marker of Intestinal-Type Differentiation: A Tissue Microarray–Based Study of 629 Tumors From Various Sites

2005 ◽  
Vol 129 (9) ◽  
pp. 1100-1105 ◽  
Author(s):  
Lindsey B. De Lott ◽  
Carl Morrison ◽  
Saul Suster ◽  
David E. Cohn ◽  
Wendy L. Frankel
Keyword(s):  
Squamous Cell ◽  
Signet Ring Cell ◽  
Squamous Cell Carcinomas ◽  
Intestinal Type ◽  
High Grade ◽  
Poorly Differentiated Neuroendocrine Carcinoma ◽  
Poorly Differentiated ◽  
Lung Adenocarcinomas ◽  
Colorectal Adenocarcinomas ◽  
Neuroendocrine Carcinomas

Abstract Context.—CDX2, a critical nuclear transcription factor for intestinal development, is expressed in intestinal epithelium and adenocarcinomas. Objectives.—To determine if CDX2 is a useful marker for intestinal-type differentiation and to correlate tumor histology with CDX2 staining in colorectal adenocarcinomas. Design.—Tissue microarrays from 71 colorectal adenocarcinomas, 31 hepatocellular carcinomas, 47 lung adenocarcinomas, 55 squamous cell carcinomas of the lung, 69 neuroendocrine carcinomas of the lung and 43 of the pancreas, 57 pancreatic adenocarcinomas, and 256 endometrial adenocarcinomas were stained with antibody against CDX2. Results.—CDX2 staining was positive in 51 (71.8%) of 71 colorectal cancers, including 38 (74.5%) of 51 well- or moderately differentiated tumors and 13 (65.0%) of 20 high-grade tumors. Of the high-grade tumors, 5 (71.4%) of 7 mucinous, 3 (100%) of 3 signet ring cell, and 5 (50.0%) of 10 poorly differentiated tumors were positive. Other tumors showing occasional CDX2 staining included 1 of 30 well- or moderately differentiated neuroendocrine carcinomas of the lung and 2 of 43 from the pancreas, 1 of 47 lung adenocarcinomas, 3 of 57 pancreatic adenocarcinomas, and 15 of 256 endometrial carcinomas. Hepatocellular, poorly differentiated neuroendocrine carcinoma of the lung and squamous cell carcinomas of the lung were not immunoreactive for CDX2. Conclusions.—CDX2 is a useful marker for intestinal-type differentiation, is rarely seen in tumors from the other sites evaluated, and may be useful in determining the site of origin for some metastatic tumors. However, CDX2 is not a sensitive marker for poorly differentiated colorectal carcinoma.

Undifferentiated Embryonal Sarcoma of the Liver is Associated with Mesenchymal Hamartoma and Multiple Chromosomal Abnormalities: A Review of Eleven Cases

2011 ◽  
Vol 14 (2) ◽  
pp. 111-116 ◽  
Author(s):  
Bahig M. Shehata ◽  
Nitika A. Gupta ◽  
Howard M. Katzenstein ◽  
Charlotte K. Steelman ◽  
Mark L. Wulkan ◽  
...  
Keyword(s):  
Chromosomal Abnormalities ◽  
Mesenchymal Hamartoma ◽  
Embryonal Sarcoma

scholarly journals Pannus Is the New Prepuce? Penile Cancer in a Buried Phallus

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Jared Manwaring ◽  
Srinivas Vourganti ◽  
Dmitriy Nikolavsky ◽  
Alfredo L. Valente ◽  
Timothy Byler
Keyword(s):  
Squamous Cell Carcinoma ◽  
Early Detection ◽  
Cell Carcinoma ◽  
Chronic Inflammation ◽  
Penile Cancer ◽  
High Grade ◽  
Fat Pad ◽  
Poorly Differentiated ◽  
Suspicious Lesions ◽  
Buried Penis

Two males presented to our urology department with complaints of bleeding and malodor from buried phallus within a suprapubic fat pad. Although both men had neonatal circumcisions, advanced penile carcinoma was found in both men. Formal penectomies showed high grade, poorly differentiated squamous cell carcinoma invading the corporal bodies and urethra. Buried penis represents a difficulty in early detection of suspicious lesions but may also provide an environment susceptible to poor hygiene and subsequent chronic inflammation. Patients with buried penis may be at a higher risk for development of invasive penile cancer and may benefit from regular and thorough genital exams.

scholarly journals Hepatic mesenchymal hamartoma and undifferentiated embryonal sarcoma of the liver: a pathologic review

2020 ◽  
Vol 7 (2) ◽  
pp. HEP19 ◽  
Author(s):  
Sebastiao N Martins-Filho ◽  
Juan Putra
Keyword(s):  
Chromosomal Aberrations ◽  
Benign Lesion ◽  
Differential Diagnoses ◽  
Mesenchymal Hamartoma ◽  
Clinicopathologic Characteristics ◽  
Radiological Features ◽  
Histologic Evaluation ◽  
Embryonal Sarcoma

This review highlights two rare entities that are predominantly seen in children: hepatic mesenchymal hamartoma (HMH) and undifferentiated embryonal sarcoma of the liver (UESL). HMH is a benign lesion predominantly seen in the first 2 years of life, while UESL is malignant and usually identified in patients between 6 and 10 years of age. UESL may arise in the background of HMH, and the association has been supported by similar chromosomal aberrations (19q13.4). The diagnosis of both lesions is primarily based on histologic evaluation, as the clinical and radiological features are not always typical. The clinicopathologic characteristics, pathogenesis, differential diagnoses and treatment for both lesions are discussed.

Poorly differentiated thyroid carcinomas: application of the Turin proposal provides prognostic results similar to those from the assessment of high-grade features

2013 ◽  
Vol 64 (2) ◽  
pp. 263-273 ◽  
Author(s):  
Viviane Gnemmi ◽  
Florence Renaud ◽  
Christine Do Cao ◽  
Julia Salleron ◽  
Georges Lion ◽  
...  
Keyword(s):  
High Grade ◽  
Thyroid Carcinomas ◽  
Poorly Differentiated

First-Line Irinotecan Combined with 5-Fluorouracil and Leucovorin for High-Grade Metastatic Gastrointestinal Neuroendocrine Carcinoma

2013 ◽  
Vol 99 (1) ◽  
pp. 57-60 ◽  
Author(s):  
Zedong Du ◽  
Yi Wang ◽  
Yi Zhou ◽  
Feng Wen ◽  
Qiu Li
Keyword(s):  
Neuroendocrine Carcinoma ◽  
Progression Free Survival ◽  
Disease Stage ◽  
Neuroendocrine Neoplasms ◽  
High Grade ◽  
First Line ◽  
Laboratory Findings ◽  
Poorly Differentiated ◽  
Prospective Trials ◽  
Line Setting

Aim and background High-grade gastrointestinal neuroendocrine neoplasms, ie, poorly differentiated neuroendocrine carcinomas, with no effective therapeutic approaches, have a high ability to metastasize. Methods A review of the hospital information system was performed. Patients with histologically proven gastrointestinal neuroendocrine carcinoma who were treated with irinotecan combined with 5-fluorouracil and leucovorin in a first-line setting were eligible for analysis. We extracted information on age, sex, disease stage, laboratory findings, radiological findings, pathological findings, chemotherapy, effectiveness and adverse events of therapy, and outcomes. Results Eleven patients were included in the study. Partial response was observed in 7 patients. Median progression-free survival and overall survival were 6.5 (95% CI, 5.1–7.9) and 13.0 (95% CI, 9.8–16.2) months, respectively. No treatment-related deaths occurred. Conclusions The results demonstrated that irinotecan combined with 5-fluorouracil and leucovorin is an active regimen with acceptable toxicity for patients with metastatic high-grade gastointestinal neuroendocrine carcinoma that merits further investigation in prospective trials.

The association of very low PSA with increased cancer-specific death in men with high-grade prostate cancer.

2015 ◽  
Vol 33 (7_suppl) ◽  
pp. 62-62
Author(s):  
Brandon Arvin Virgil Mahal ◽  
Ayal Aaron Aizer ◽  
Jason Alexander Efstathiou ◽  
Paul Linh Nguyen
Keyword(s):  
Prostate Cancer ◽  
Reference Group ◽  
High Grade ◽  
Specific Mortality ◽  
National Cohort ◽  
Poorly Differentiated ◽  
Cancer Specific Mortality ◽  
Prostate Cancers ◽  
Very High

62 Background: It has been hypothesized that very low PSAs in men with high-grade prostate cancer could reflect dedifferentiation and a poorer prognosis, but clinical evidence to support this is limited. We sought to determine whether a very low-presenting PSA was associated with greater prostate cancer-specific mortality (PCSM) among men with Gleason score (GS) 8-10 disease. Methods: The Surveillance, Epidemiology and End Results Program was used to identify a national cohort of 328,904 men diagnosed with cT1-4N0M0 prostate cancer between 2004 and 2010. Multivariable Fine-Gray competing-risks regression analysis was used to determine PCSM as a function of PSA level (<2.5 ng/mL, 2.6-4 ng/mL, 4.1-10 ng/mL, 10.1-20 ng/mL, 20.1-40 ng/mL, or >40ng/mL) and GS (8-10 vs. <=7). Results: Median follow-up was 38 months. Among men with GS 8-10 disease, using PSA 4.1-10 as the reference group, the Adjusted HR (AHR) for PCSM for men with PSA level <2.5 was 1.86 (95% CI 1.51-2.29; P<0.001), PSA 2.6-4 was1.44 (1.17-1.78; P<0.001), PSA 10.1-20 was 1.58 (1.39-1.78; P<0.001), PSA 20.1-40 was 2.04 (1.78-2.33; P<0.001), and PSA>40 was 3.19 (2.83-3.59; P<0.001), suggesting a U-shaped distribution. There was a significant interaction between PSA level and GS (Pinteraction<0.001) such that PSA <2.5 only significantly predicted for poorer PCSM among patients with high grade GS 8-10 disease. Conclusions: Among patients with high grade GS 8-10 disease, patients with PSA <2.5 and 2.6-4 appear to have a higher risk for cancer-specific death compared to patients with a 10.1-20 PSA level, supporting the notion that low PSA in GS 8-10 disease may be a sign of underlying aggressive and extremely poorly differentiated or anaplastic low PSA-producing tumors. Patients with low PSA GS 8-10 disease should be considered for clinical trials studying the use of chemotherapy and other novel agents in very-high risk prostate cancers.

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