Leiomyosarcoma of the urinary bladder: a clinicopathological study of 34 cases

2010 ◽  
Vol 63 (8) ◽  
pp. 708-713 ◽  
Author(s):  
Matthew R Lindberg ◽  
Cyril Fisher ◽  
Khin Thway ◽  
Dengfeng Cao ◽  
John C Cheville ◽  
...  

BackgroundLeiomyosarcomas of the urinary bladder (LMS-UB) are rare, usually aggressive neoplasms. Owing to their rarity, only a limited number of cases with clinical follow-up information have been published. There is no current consensus on LMS-UB grading, and it is unknown whether the widely accepted Fédération Nationale des Centres de Lutte Contre le Cancer (FNCLCC) and National Cancer Institute (NCI) grading systems of soft-tissue sarcomas are applicable to LMS-UB.MethodsThe authors studied 34 well-characterised LMS-UB and compared the prognostic power of the FNCLCC and NCI systems with that of one published grading scheme for LMS-UB (Mayo). All available slides from 34 LMS-UB were retrieved and evaluated with regards to degree of differentiation, mitotic rate/10 high-powered fields (HPF), and % necrosis. Cases were graded using published criteria for the FNCLCC, NCI and Mayo schemes. Follow-up information was obtained.ResultsThe tumours occurred in 17 females and 17 males, ranging from 31 to 91 years (median 65), and measured 2–12 cm in size. One tumour was well differentiated, 17 tumours were moderately differentiated, and 16 tumours were poorly differentiated. Mitotic rates ranged from 1 to >30/10 HPF (median 12/10 HPF), and tumours showed 0–60% necrosis (median 25%). FNCLCC grades were 1 (3), 2 (12) and 3 (19). NCI grades were 1 (2), 2 (11) and 3 (21). Mayo grades were low (7) and high (27). FNCLCC and NCI grades were identical in 23/34 cases (68%). Four cases were FNCLCC/NCI grade 2 or 3 and Mayo low-grade. Clinical follow-up was available for 25 of 34 patients (74%). Clinical follow-up of ≥12 months was available for 17 of these 25 cases (68%) with a median follow-up duration of 52 months (range 12–120 months). Adverse outcome was seen in nine of these 17 patients (53%). Seven of the eight cases (88%) with a clinical follow-up duration of <12 months died of their disease. Overall, adverse outcome was documented in 16 of 25 (64%) cases. Metastatic disease was seen in 13 of 25 (52%) cases, with the lungs being the most common site of metastasis (62%). Adverse outcome was noted in 15 of 23 (65%) of FNCLCC grade 2 or 3 LMS-UB, as compared with zero of two (0%) FNCLCC grade 1 tumours (p=0.15), in 15 of 23 (65%) NCI grade 2 or 3 LMS-UB, versus zero of two (0%) NCI grade 1 sarcomas (p=0.17) and in 13 of 20 (65%) Mayo high grade LMS-UB, as opposed to two of five (40%) low-grade lesions (all results not statistically significant).ConclusionsThe authors conclude that LMS-UB occurs in older adults of either sex and is characterised by aggressive behaviour, with adverse outcome in >60% of cases. Certain advantages of the FNCLCC system may support its more widespread adoption for future studies.

2017 ◽  
Vol 2017 ◽  
pp. 1-6
Author(s):  
Nicolas Macagno ◽  
Stéphane Fuentes ◽  
Gonzague de Pinieux ◽  
André Maues de Paula ◽  
Sébastien Salas ◽  
...  

Despite being one of the most frequent soft-tissue sarcomas, well-differentiated liposarcoma has never been reported near the spine. The authors present the case of a 67-year-old man with progressive history of back pain. Physical examination revealed a mass located within the right paravertebral muscles. MR and CT imaging showed a heavily ossified central mass surrounded by a peripheral fatty component. No connection with the underlying bone was detected on imagery and during surgery. After surgical resection, histopathological examination revealed a tumor harboring combined features of well-differentiated liposarcoma and low-grade osteosarcoma. Tumor cells displayed overexpression of MDM2, CDK4, and P16 by immunohistochemistry and CGH revealed amplification of 12q13-15 as the only genetic imbalance. MDM2 FISH analysis was performed but was inconclusive. The pathological, immunohistochemical, and genetic features, the differential diagnoses, and the therapeutic management of this unusual tumor are discussed. No complementary treatment was performed initially. Following first treatment, two recurrences occurred 6 and 9 years later, both displaying histological features similar to the first occurrence. Radiotherapy was started after the second recurrence. Follow-up shows no evidence of disease 11 years after initial diagnosis. This case was unusual due to the paravertebral location of the tumor and its divergent differentiation.


2013 ◽  
Vol 31 (15_suppl) ◽  
pp. e14673-e14673
Author(s):  
Raymond Elsoueidi ◽  
Sabrina Tom ◽  
Eyob Adane ◽  
Mark Dignan

e14673 Background: Colorectal cancer is the third most common cancer worldwide. Smoking has been increasingly reported as a risk factor. Poorly differentiated adenocarcinoma in colorectal cancer has been reported to have a worse outcome. Poorly and moderately differentiated adenocarcinomas in Non Small Cell Lung cancer were more strongly correlated with smoking than well differentiated adenocarcinoma. This study was designed to evaluate the association between cigarette smoking and tumor grade in colorectal cancer. Methods: The medical records of patients diagnosed with colorectal cancer between 2001 and 2011 at the Appalachian Regional Healthcare in Hazard, KY were retrospectively reviewed. Age, gender, pathology and smoking history were obtained. The association between smoking and tumor grade was evaluated by the χ2 test. Results: Three hundred seventeen patients were diagnosed with colorectal cancer between 2001 and 2011. The percentage of male and female patients was 53.3% and 46.7%, respectively. Among these patients, 49.5% (n=157) were smokers and 38.8% were nonsmokers (n=138), while for 11.7% (n=35) of patients smoking history was unknown. In smokers, 17.1% had high grade adenocarcinoma (poorly differentiated) and 82.9% had low grade adenocarcinoma (moderately differentiated + well differentiated) whereas in non-smokers, 13.8% had high grade adenocarcinoma and 86.2% had low grade adenocarcinoma. Although the percentage of high grade adenocarcinoma was higher among smokers this was not statistically significantly (p=0.55). Moreover, tumor grade was not significantly associated with age and gender. Conclusions: Based on these results, there was no association between degree of differentiation of tumor in colorectal cancer and smoking history, age and gender. Although to be noted that the pathologic grading may vary from one pathologist to another.


2016 ◽  
Vol 9 (1) ◽  
pp. 85-89
Author(s):  
Svetlana A. Mateva ◽  
Margarita R. Nikolova ◽  
Alexandar V. Valkov ◽  
Margarita R. Nikolova

Summary Liposarcoma is one of the most common soft tissue sarcomas in adults with a relative incidence amongst other sarcomas ranging from 9.8% to 16%. It usually locates in the limbs and retroperitoneum. Primary liposarcomas of the larynx and hypopharynx are rare, comprising less than 20% of all head and neck liposarcomas. According to World Health Organization, these tumors are divided into four histologic types, and well-differentiated liposarcoma is the most common one. It is a tumor of low-grade malignancy that may recur locally, but does not metastasize. We present a case of laryngopharyngeal well- differentiated liposarcoma in an old patient with two previous removals. We also discuss recently published cases with this unusual location of liposarcoma.


2013 ◽  
Vol 137 (10) ◽  
pp. 1337-1341 ◽  
Author(s):  
Melissa L. Stanton ◽  
Li Xiao ◽  
Bogdan A. Czerniak ◽  
Charles C. Guo

Context.—Urothelial tumors are rare in young patients. Because of their rarity, the natural history of the disease in young patients remains poorly understood. Objective.—To understand the pathologic and clinical features of urothelial tumors of the urinary bladder in young patients. Design.—We identified 59 young patients with urothelial tumors of the urinary bladder treated at our institution and analyzed the tumors' pathologic features and the patients' clinical outcomes. Results.—All patients were 30 years or younger, with a mean age of 23.5 years (range, 4–30). Thirty-eight patients (64%) were male, and 21 (36%) were female. Most tumors were noninvasive, papillary urothelial tumors (49 of 59; 83%), including papillary urothelial neoplasms of low malignant potential (7 of 49; 14%), low-grade papillary urothelial carcinomas (38 of 49; 78%), and high-grade papillary urothelial carcinomas (4 of 49; 8%). Only a few (n = 10) of the urothelial tumors were invasive, invading the lamina propria (n = 5; 50%), muscularis propria (n = 4; 40%), or perivesical soft tissue (n = 1; 10%). Clinical follow-up information was available for 41 patients (69%), with a mean follow-up time of 77 months. Of 31 patients with noninvasive papillary urothelial tumors, only 1 patient (3%) later developed an invasive urothelial carcinoma and died of the disease, and 30 of these patients (97%) were alive at the end of follow-up, although 10 (32%) had local tumor recurrences. In the 10 patients with invasive urothelial carcinomas, 3 patients (30%) died of the disease and 5 others (50%) were alive with metastases (the other 2 [20%] were alive with no recurrence). Conclusion.—Urothelial tumors in young patients are mostly noninvasive, papillary carcinomas and have an excellent prognosis; however, a small subset of patients may present with high-grade invasive urothelial carcinomas that result in poor clinical outcomes.


2020 ◽  
Vol 153 (6) ◽  
pp. 811-820 ◽  
Author(s):  
Kelsey E McHugh ◽  
Sanjay Mukhopadhyay ◽  
Erika E Doxtader ◽  
Christopher Lanigan ◽  
Daniela S Allende

Abstract Objectives INSM1 has been described as a sensitive and specific neuroendocrine marker. This study aims to compare INSM1 with traditional neuroendocrine markers in gastrointestinal neuroendocrine neoplasms. Methods Retrospective review (2008-2018) was used to retrieve paraffin-embedded tissue from 110 gastrointestinal neuroendocrine neoplasms and controls that was subsequently stained with INSM1, synaptophysin, chromogranin, CD56, and Ki-67. Results INSM1 was positive in 16 of 17 (94.1%) gastric, 17 of 18 (94.4%) pancreatic, 13 of 18 (72.2%) small bowel, 17 of 21 (81.0%) colonic, and 26 of 36 (72.2%) appendiceal tumors. INSM1 was positive in 58 of 70 (82.9%) well-differentiated neuroendocrine tumors, 17 of 20 (85.0%) poorly differentiated neuroendocrine carcinomas, 8 of 11 (72.7%) low-grade goblet cell adenocarcinomas (grade 1), and 6 of 9 (66.7%) high-grade goblet cell adenocarcinomas (grade 2/3). INSM1 sensitivity for neuroendocrine neoplasms (80.9%) was less than that of synaptophysin (99.1%), chromogranin (88%), and CD56 (95.3%); specificity was higher (95.7% vs 86.0%, 87.3%, and 86.0%, respectively). Conclusions INSM1 is a useful marker of neuroendocrine differentiation in gastrointestinal neuroendocrine and mixed neuroendocrine neoplasms. Compared with traditional neuroendocrine markers, INSM1 is less sensitive but more specific.


Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3892-3892
Author(s):  
Andres O. Soriano ◽  
Michael Thompson ◽  
Jorge Romaguera ◽  
Alma Rodriguez ◽  
Fredrick B. Hagemeister ◽  
...  

Abstract Objectives: To describe the clinical characteristics, pathologic features, immunophenotype, treatment and outcome of patients with FDCS seen at MD Anderson Cancer Center in the past 10 years. Background: FDCS is grouped in the WHO classification of tumors with the histiocytic and dendritic cell neoplasms, this group also includes histiocytic sarcoma, Langerhans cell tumors and interdigitating dendritic cell tumors. Data on this disease is based on case reports and case series. Methods: After IRB approval, cases were identified from the files of the lymphoma and pathology departments at MD Anderson Cancer Center from 1995 to 2005. Results: Fourteen patients were identified. Median age was 48 years old (25–69). Three patients presented with cervical lymphadenopathy, five had abdominal lymphadenopathy, three had mediastinal adenopathy, two had nasopharyngeal disease and one had pleural involvement. Extranodal disease included liver, spleen, pancreas and pleura. Constitutional symptoms were reported in 2 patients. Median performance status was 1 (0–2). Histologically, five cases showed low grade cytology with proliferation of spindled cells, growth patterns included whorled, storiform, fasicular and nodular. Three cases showed low grade features with focal high grade cytology and a diffuse growth pattern. Five cases showed high grade cytology that also included areas of necrosis in 2 cases and a high mitotic rate (>20/10HPF) in one case. CD21, CD23 and CD35 were positive in 83%, 90% and 44% of the cases respectively. Epidermal growth factor receptor (EGFR) was strongly positive in 12/13 cases tested (92%). The case that tested negative had high grade features and high mitotic rate. One patient had coexistent Castleman’s disease. Information on initial treatment was available in 11 patients which included surgery alone in 1 patient, surgery and radiation in 2 patients, surgery and chemotherapy in 1, chemotherapy alone in 3, chemotherapy and radiation in 1, surgery followed by radiation and chemotherapy in 3 patients. The initial chemotherapy regimen was CHOP in 8 patients. Complete remission (CR) was achieved in 7/11 patients (63%), 3 patients had disease progression and 1 had stable disease. Relapse occurred in all the patients who had a CR. Salvage treatment included surgery in 2 patients and chemotherapy in 7 patients (including the 2 patients who progressed on initial treatment). Two patients underwent allogenic hematopoietic stem cell transplantation after salvage therapy. CR was achieved in 5 patients, partial remission in 2, progression in 1 and stable disease in 1. Information on disease status at last follow up was available in 13 patients. Ten patients were alive at a median follow up of 22 months, 3/13 patients (23%) had no evidence of disease and 7/13 patients (53%) were alive with disease. Conclusions: The pathologic characteristics and immunophenotype found in our series were similar to those previously reported. EGFR was strongly positive in all but one of the cases tested. Consistent with previous reports Castleman’s disease was found in one of the cases. Although most of the patients initially responded to treatment, all of them eventually relapsed, which is in contrast to previously reported relapse rates of 16–36%. A better understanding of the biology of FDCS could guide our efforts in the development of new treatment modalities for this rare disease.


2009 ◽  
Vol 27 (15_suppl) ◽  
pp. 1061-1061
Author(s):  
R. Santana-Davila ◽  
D. W. Visscher ◽  
C. M. Vachon ◽  
M. Frost ◽  
R. A. Vierkant ◽  
...  

1061 Background: Benign breast disease (BBD) is a significant risk factor for breast cancer (BC); however little is known about the aggressiveness of the BCs these patients develop. Shared features between select atypias and low-grade DCIS have led some to speculate that a history of BBD portends the development of well-differentiated BCs. Methods: The Mayo BBD cohort includes 9,376 women who had benign breast biopsies from 1967–1991. Data on BC events were obtained from medical records and questionnaires. Tissue from the BCs was reviewed by a single breast pathologist (DWV). Results: Our cohort included 6,244 patients with nonproliferative disease (NP), 2,801 women with proliferative disease without atypia (PDWA), and 331 with atypical hyperplasias (AH). With a median of 18 years of follow-up, 799 patients with BBD have developed breast cancer, 416 initially had NP, 313 had PDWA, and 70 AH. BC tissue was available for 703 of the women. The cancers were invasive ductal in 76.1% (n = 535), invasive lobular in 9.4% (n = 66), and DCIS alone in 14.5% (n = 102) patients. Grade is currently available for 500 women, and is well differentiated in 29.4% (n = 150), moderately differentiated in 42.7% (n = 218) and poorly differentiated in 27.8% (n = 142). In 537 cases the malignant tissue had a concurrent benign component consisting of NP in 24% (n = 129), PDWA in 25.1% (n = 135), and AH in 50.8% (n = 273). In regards to tumor size, 69.6% had T1 tumors, 24.9% T2 tumors, and 5.5% T3 disease. Metastasis to lymph nodes occurred in 25% of patients. Median time to BC was 12.3 years and did not differ across the different benign entities. Breast cancer developed within 5 years from the initial BBD in 163 women (20%). In this group, tumor size was greater (p = 0.02), and there were more poorly differentiated tumors (p = 0.006) than in those who later developed BC. Conclusions: In the BCs that have developed in our BBD cohort, we do not see a preponderance of well-differentiated, good-risk BCs. Patients diagnosed within 5 years of BBD appear to have BCs with more aggressive features. No significant financial relationships to disclose.


Neurosurgery ◽  
2004 ◽  
Vol 54 (4) ◽  
pp. 832-839 ◽  
Author(s):  
Michael Kerin Morgan ◽  
Andrew Michael Rochford ◽  
Antonio Tsahtsarlis ◽  
Nicholas Little ◽  
Kenneth Charles Faulder

Abstract OBJECTIVE Grade I and II arteriovenous malformations (AVMs) have been considered safe to resect. However, unoperated low-grade AVMs have not been considered in previously reported series. The aim of this study was to examine all cases, both operated and unoperated, to identify any characteristics of low-grade AVMs that comprise a subgroup that might pose a relatively higher risk. METHODS A prospectively enrolled AVM database included 237 patients in Spetzler-Martin Grade I or II. These patients were analyzed on the basis of demographic characteristics, angiographic and magnetic resonance imaging features, clinical presentation, method of treatment, and outcome. RESULTS Surgery was performed in 220 patients in Spetzler-Martin Grade I or II. Seventeen patients did not undergo treatment because of poor neurological condition (six patients), patient refusal (nine patients), and perceived surgical difficulty (AVM size approaching 3 cm adjacent to Broca's area) (two patients). The overall surgical morbidity rate was 0.9%, and the mortality rate was 0.5%. Adverse outcomes occurred in 1 (0.6%) of 180 patients with AVMs located away from eloquent cortex and in 2 (5%) of 40 patients with AVMs adjacent to eloquent cortex. None of 28 surgical patients with deep venous drainage had an adverse outcome. All 219 patients who survived surgery underwent postoperative angiography that confirmed cure. No postoperative hemorrhage has occurred in 1143 patient-years of follow-up (mean follow-up, 5.3 yr). CONCLUSION When considering adverse outcome in the surgical series of Grade I and II AVMs alone, no statistical difference between non-eloquently located AVMs (0.6%) and eloquently located AVMs (5% adverse outcome) can be detected. However, consideration of all Grade I and II AVMs, both surgical and nonsurgical, may prove that a difference in outcome exists between these two groups masked by case selection. Generalization of the chances of adverse outcomes to all Grade I and II AVMs (both operated and unoperated) suggests that the risk of performing surgery on noneloquent brain in our series was 0.6% and that in eloquent brain could have been as high as 9.5%, had all such patients undergone surgery.


2002 ◽  
Vol 39 (4) ◽  
pp. 452-457 ◽  
Author(s):  
T. O. Johnson ◽  
F. Y. Schulman ◽  
T. P. Lipscomb ◽  
L. D. Yantis

Most feline cutaneous mast cell tumors (CMCT) are behaviorally benign; however, there is a subset of these tumors with marked pleomorphism (previously termed poorly differentiated) that have been reported to be more aggressive. In this study, pleomorphic CMCT from 15 cats were identified from surgical biopsy submissions, and follow-up clinical data were obtained for 14 of these cats. Pleomorphic CMCT were discrete dermal nodules composed of sheets of pleomorphic round cells. Tumors from all 15 cats contained markedly cytomegalic and karyomegalic cells; 9/15 tumors (60%) contained multinucleated tumor giant cells. Typical mast cell granules were easily identified in sections stained with hematoxylin and eosin and with metachromatic stains and based on ultrastructural evaluation in cytomegalic as well as smaller tumor cells, indicating that the tumors were not poorly differentiated. The mitotic rate was very low (<1 mitosis per 10 high-power fields [hpf]) in 14 of 15 tumors (93%). Affected cats were 6–19 years old (mean age = 11.5 years), and there was no breed or sex predilection. Two cats had local recurrence. The only cat that had a pleomorphic CMCT with a high mitotic rate (1–2 mitoses/hpf) subsequently developed numerous other dermal neoplasms and was euthanatized. In this study, the large majority of feline pleomorphic CMCT were behaviorally benign. Mitotic rate is likely an important prognostic indicator of CMCT behavior.


2007 ◽  
Vol 25 (35) ◽  
pp. 5609-5615 ◽  
Author(s):  
Cristina R. Ferrone ◽  
Laura H. Tang ◽  
James Tomlinson ◽  
Mithat Gonen ◽  
Steven N. Hochwald ◽  
...  

Purpose The WHO classification for well-differentiated pancreatic endocrine neoplasms (PENs) incorporates both stage and grade. This study compares the prognostic value of a simplified staging and grading system with the WHO system in a large single-institution study. Patients and Methods A prospective database (1982 to 2005) identified 183 patients who underwent operative treatment for PENs. Tumors were staged (< 2 cm primary, ≥ 2 cm primary, or metastases) and graded (low grade: no necrosis and < two mitoses/50 high-powered fields [HPF]; or intermediate grade: necrosis and/or ≥ two mitoses/50 HPF) with a simplified schema. Influence of stage and grade on recurrence and disease-specific survival (DSS) was determined. Prognostic strength was assessed with the concordance index (CI). Results Median age of the 183 patients was 56 years, and 53% were women. Median follow-up time was 44 months (range, 1 to 226 months). Classification identified 28 patients (15%) with WHO 1.1 disease, 74 (41%) with 1.2 disease, and 81 (44%) with 2.0 disease. Classification by stage identified 35 patients (19%) with tumors less than 2 cm, 96 (52%) with tumors ≥ 2 cm, and 52 (29%) with nodal or distant metastases. Tumors were low grade in 102 patients (56%). Earlier stage tumors were more likely to be low grade (< 2 cm, 83%; ≥ 2 cm, 61%; metastases, 28%; P < .001). The WHO classification, tumor stage, and grade were associated with 5-year DSS (P < .001). Tumors ≥ 2 cm or metastases are stratified by grade (5-year DSS rate for low v intermediate grade: ≥ 2 cm, 97% v 80%, respectively; P < .001; metastases, 93% v 62%, respectively; P = .05). The CI was 0.72 for WHO, 0.71 for stage, 0.66 for grade, and 0.76 for stage combined with grade. Conclusion Accurate prognostic information can be obtained by combining tumor size and metastases with simple grading information based on necrosis and mitotic rate.


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