scholarly journals A single institutional experience treating adipocytic tumors: incidence, disease-related outcomes, and the clinical significance of MDM2 analysis

2020 ◽  
Vol 12 (3) ◽  
Author(s):  
Saule Tamkus ◽  
Charles A. Gusho ◽  
Matthew Colman ◽  
Ira Miller ◽  
Steven Gitelis ◽  
...  
Keyword(s):  
Local Recurrence ◽  
High Rate ◽  
World Health ◽  
Low Grade ◽  
Adipocytic Tumors ◽  
Imaging Characteristics ◽  
Institutional Experience ◽  
Atypical Lipomatous Tumors ◽  
Mdm2 Amplification ◽  
Lipomatous Tumors

Adipocytic tumors exist either as a benign or malignant form. The benign variant, lipoma, is composed of normal fat tissue. Lipomas typically develop from superficial fat cells beneath the skin or mucous membranes. Liposarcoma, the malignant counterpart, often develops in deeper tissues and is the most commonly diagnosed Soft Tissue Sarcoma (STS), comprising at least 20% of adult STS. However, malignant tumors of fatty origin exist as a spectrum of diagnoses, each carrying a unique risk of recurrence, metastasis, and longterm survival. The World Health Organization classifies liposarcomas into five categories: i) Atypical Lipomatous Tumors/Well Differentiated (ALT/WD); Ii) Dedifferentiated (ALT/DD); Iii) Myxoid; Iv) Round cell; and v) Pleomorphic. Lipomatous tumors often exhibit different immunohistochemical patterns. Benign lipomas are distinguished by the absence of Murine Double-Minute 2 (MDM2) amplification. Similarly, ALT/WD, classically defined as a low-grade and locally aggressive tumor, demonstrates consistent patterns of MDM2 amplification. Some studies suggest 10% of ALT/WD progress to the highgrade DD form, with others report a dedifferentiation rate of as high as 20% for primary ALT/WD based on location. The ALT/DD subtype is aggressive and has a high capacity to metastasize. While the mechanism of pathogenesis of ALT/DD metastasis is unknown, previous studies suggest that increased MDM2 amplification may play a role. This study sought to evaluate a single institutional experience treating the entire spectrum of lipomatous tumors and describe utilization patterns of MDM2 testing. The group hypothesized: i) Atypical Lipomatous Tumors (ALT), which include ALT/DD and ALT/WD, would exhibit a higher rate of local recurrence than lipomas with no significantly increased incidence of metastases; and ii) at least 50% of our MDM2 testing of ALT would prove positive for the MDM2 overamplification. This study retrospectively reviewed 105 cases (66 lipomas, 27 ALTs, 12 liposarcomas) of patients who underwent lipomatous tumor excision at our institution from 2013 to 2017. Twenty-five tumors (6 lipomas, 18 ALT, 1 liposarcoma) were tested for MDM2 amplification. Three of the tested tumors recurred (2 ALT, 1 liposarcoma), and each exhibited MDM2 overamplification. Five tumors (5 liposarcoma) developed late metastases. These data suggest that although ALT is associated with a higher rate of local recurrence, metastases are quite rare. Additionally, the data demonstrate a high rate of positive MDM2 testing (76%) based on clinical and imaging characteristics of the tumors.

Atypical Lipomatous Tumors: Should They be Treated Like Other Sarcoma or Not? Surgical Consideration from a Bi-Institutional Experience

2014 ◽  
Vol 21 (13) ◽  
pp. 4090-4097 ◽  
Author(s):  
Chiara Erminia Mussi ◽  
Primo Daolio ◽  
Matteo Cimino ◽  
Fabio Giardina ◽  
Rita De Sanctis ◽  
...  
Keyword(s):  
Institutional Experience ◽  
Atypical Lipomatous Tumors ◽  
Lipomatous Tumors

scholarly journals The Utility of Chest Imaging for Surveillance of Atypical Lipomatous Tumors

Sarcoma ◽  
2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Alexander L. Lazarides ◽  
Harrison R. Ferlauto ◽  
Zachary D. C. Burke ◽  
Anthony M. Griffin ◽  
Bruce D. Leckey ◽  
...  
Keyword(s):  
Local Recurrence ◽  
Significant Role ◽  
Soft Tissue Sarcomas ◽  
Standard Of Care ◽  
Chest Imaging ◽  
Surveillance Period ◽  
Term Survival ◽  
Atypical Lipomatous Tumors ◽  
Lipomatous Tumors

Synopsis. For ALTs, the utility of chest surveillance has not been well defined. This study suggests that chest imaging does not have a significant role in the surveillance of ALTs. Advanced local imaging and more intensive chest surveillance may be considered in cases of local recurrence. Background. Unlike other soft tissue sarcomas, atypical lipomatous tumors (ALTs) are thought to have a low propensity for metastasis. Despite this, a standard of care for pulmonary metastasis (PM) surveillance has not been established. This study aimed to evaluate the utility of chest imaging for PM surveillance following ALT excision. Methods. This was a multi-institution, retrospective review of all patients with primary ALTs of the extremities or superficial torso who underwent excision between 2006 and 2018. Minimum follow-up was two years. Long-term survival was evaluated using the Kaplan–Meier method. Results. 190 patients with ALT were included. Average age was 61.7 years and average follow-up was 58.6 months (24 to 180 months). MDM2 testing was positive in 88 patients (46.3%), and 102 (53.7%) did not receive MDM2 testing. 188 patients (98.9%) had marginal excision, and 127 (66.8%) had marginal or positive margins. Patients received an average of 0.9 CT scans and 1.3 chest radiographs over the surveillance period. 10-year metastasis-free survival was 100%, with no documented deaths from disease. Conclusions. This study suggests that chest imaging does not have a significant role in PM surveillance following ALT excision, but advanced local imaging and chest surveillance may be considered in cases of local recurrence or concern for dedifferentiation.

scholarly journals “Deep” vertical Mohs and closure with a tumor-free flap for dermatofibrosarcoma protuberans: A case report

2019 ◽  
Vol 7 ◽  
pp. 2050313X1984778
Author(s):  
Ann M John ◽  
Gina Francisco ◽  
Radhika Srivastava ◽  
Hamza Bhatti ◽  
Babar K Rao
Keyword(s):  
Case Report ◽  
Local Recurrence ◽  
Surgical Technique ◽  
Local Excision ◽  
Free Flap ◽  
Malignant Tumor ◽  
Wide Local Excision ◽  
Dermatofibrosarcoma Protuberans ◽  
High Rate ◽  
Low Grade

Dermatofibrosarcoma protuberans is an uncommon low-grade malignant tumor that can invade locally and rarely metastasize. Dermatofibrosarcoma protuberans has a high rate of local recurrence due to incomplete excision, especially in deep tissues. Morbidity is often related to multiple local recurrences and removal of excessive tissue with large, complex repairs. We present a case of incompletely excised dermatofibrosarcoma protuberans after initial wide local excision. We subsequently employed a “deep” vertical Mohs micrographic surgical technique to remove the remaining tumor while creating a flap with the tumor-free superficial portion to preserve tissue and avoid a complicated repair. The patient is tumor-free for 7 years.

Automated Bright-Field Dual-Color In Situ Hybridization for MDM2: Interobserver Reproducibility and Correlation With Fluorescence In Situ Hybridization in a Series of Soft Tissue Consults

2016 ◽  
Vol 140 (10) ◽  
pp. 1111-1115 ◽  
Author(s):  
Gloria Zhang ◽  
Christopher P. Lanigan ◽  
John R. Goldblum ◽  
Raymond R. Tubbs ◽  
Erinn Downs-Kelly
Keyword(s):  
In Situ Hybridization ◽  
Fluorescence In Situ Hybridization ◽  
Chromosome 12 ◽  
Interobserver Reproducibility ◽  
Bright Field ◽  
Well Differentiated ◽  
Atypical Lipomatous Tumors ◽  
Mdm2 Amplification ◽  
Lipomatous Tumors

Context.—Atypical lipomatous tumors/well-differentiated liposarcomas contain alterations in the 12q13-15 region resulting in amplification of MDM2 and nearby genes. Identifying MDM2 amplification is a useful ancillary test, as the histologic mimics of atypical lipomatous tumors/well-differentiated liposarcomas have consistently shown a lack of MDM2 amplification. Objective.—To assess the interobserver reproducibility of a bright-field assay for MDM2 amplification (dual-color, dual-hapten in situ hybridization [DDISH]) among reviewers with varying degrees of experience with the assay and to assess the concordance of MDM2 DDISH with MDM2 fluorescence in situ hybridization (FISH). Design.—In total, 102 cases were assessed in parallel for MDM2 by FISH and DDISH. MDM2 amplification was defined as an MDM2 to chromosome 12 ratio of 2.0 or greater, whereas an MDM2 to chromosome 12 ratio of less than 2 was nonamplified. Fluorescence in situ hybridization was scored in the routine clinical laboratory and DDISH was evaluated by 3 different pathologists blinded to the final diagnosis and FISH results. Results.—Fluorescence in situ hybridization categorized 27 cases (26%) as MDM2 amplified and 75 cases (74%) as nonamplified; the consensus DDISH diagnosis was 98% concordant with FISH. Agreement between MDM2 DDISH by each reviewer and MDM2 FISH was highly concordant (99%, 98%, and 98%, respectively, for reviewers 1, 2 and 3). The κ agreement of the 3 reviewers scoring DDISH was excellent (κ = 0.949, 0.95, and 0.95, respectively, for reviewers 1, 2, and 3). Conclusions.—This study highlights excellent concordance between DDISH and FISH in MDM2 copy number assessment. Moreover, excellent interobserver reproducibility of the DDISH assay was found among reviewers with varying levels of experience evaluating bright-field assays.

scholarly journals Solid Pseudopapillary Neoplasm of the Pancreas: Unfolding an Intriguing Condition

2021 ◽  
pp. 1-12
Author(s):  
Manuel António Alves Cruz ◽  
Pedro Moutinho-Ribeiro ◽  
Pedro Costa-Moreira ◽  
Guilherme Macedo
Keyword(s):  
Pancreatic Cancer ◽  
Survival Rate ◽  
Malignant Neoplasm ◽  
World Health ◽  
Ductal Adenocarcinoma ◽  
Malignant Neoplasms ◽  
Low Grade ◽  
Solid Pseudopapillary Neoplasm ◽  
Imaging Characteristics ◽  
Health Organization

Pancreatic cancer is one of the most lethal malignant neoplasms, with a 1-year survival rate after diagnosis of 24%, and a 5-year survival rate of only 9%. While this illustrates the behavior of its main histologic type – ductal adenocarcinoma, there are other histologic subtypes of pancreatic cancer that can harbor excellent prognosis. Solid pseudopapillary neoplasm, described as a rare low-grade malignant neoplasm by the World Health Organization, is the best example of that, having an overall 5-year survival rate of about 97%. Not only the prognosis, but everything about this entity is unique: its histogenesis, epidemiology, presentation, imaging characteristics, cytology features, immunohistochemical profile, and treatment. This explains the urge to improve our understanding about this entity and thus our ability to accurately recognize and manage it. Having this in mind, this article aims to summarize the most relevant topics regarding this entity.

scholarly journals Efficacy of Adjuvant Radiotherapy of the Tumor Bed on Local Recurrence of Adrenocortical Carcinoma

2006 ◽  
Vol 91 (11) ◽  
pp. 4501-4504 ◽  
Author(s):  
Martin Fassnacht ◽  
Stefanie Hahner ◽  
Buelent Polat ◽  
Ann-Cathrin Koschker ◽  
Werner Kenn ◽  
...  
Keyword(s):  
Local Recurrence ◽  
Adrenocortical Carcinoma ◽  
Outcome Measure ◽  
Residual Disease ◽  
Disease Free Survival ◽  
High Rate ◽  
World Health ◽  
Main Outcome Measure ◽  
Tumor Bed ◽  
Disease Free

Abstract Context: Local tumor recurrence is common in adrenocortical carcinoma (ACC) and is the most frequent cause for reoperation. Although radiotherapy is often considered ineffective in the treatment of ACC, the limited number of available studies does not support this statement. Objective: The objective of the study was investigation of adjuvant tumor bed irradiation in the treatment of ACC. Design: We performed a retrospective analysis. Patients: The German ACC Registry (n = 285) was screened for patients who had received tumor bed radiotherapy in an adjuvant setting (no macroscopic evidence for residual disease after surgery). Fourteen patients without distant metastases (World Health Organization stage I, one patient; stage II, seven; stage III, three; and stage IV, three) were matched with 14 patients for resection status, adjuvant mitotane treatment, stage, and tumor size. Median follow-up of patients still alive (n = 15) was 37 months. Main Outcome Measure: Survival without local recurrence and disease-free survival was the main outcome measure. Results: Local recurrence was observed in two of 14 patients in the radiotherapy group and in 11 of 14 control patients. The probability to be free of local recurrence 5 yr after surgery differed significantly [79% (95% confidence interval, 53–100) vs. 12% (0–30); P < 0.01]. However, disease-free and overall survival were not significantly different between the two groups. Acute adverse events related to radiotherapy were mostly mild. One patient developed a partial Budd-Chiari syndrome. Conclusion: These data from the largest series of ACC patients treated with adjuvant tumor bed irradiation suggest that radiotherapy is effective in reducing the high rate of local recurrence in ACC. A randomized trial in high-risk patients is needed to further evaluate the efficacy of radiotherapy as an adjuvant treatment option in ACC.

Chondroid Tumors as Incidental Findings and Differential Diagnosis between Enchondromas and Low-grade Chondrosarcomas

2019 ◽  
Vol 23 (01) ◽  
pp. 003-018 ◽  
Author(s):  
Amanda Isaac ◽  
José Villagrán ◽  
P. Afonso
Keyword(s):  
Bone Tumor ◽  
World Health ◽  
Bone Lesions ◽  
Low Grade ◽  
Diagnostic Features ◽  
Primary Bone Tumor ◽  
Imaging Characteristics ◽  
Primary Bone ◽  
Health Organization ◽  
Cartilage Tumor

Chondroid tumors are a heterogeneous group of neoplasms that all share the production of chondroid matrix. This ranges from a fetal type to mature hyaline cartilage and mirrors its imaging characteristics.The benign chondroid tumors represent some of the most encountered incidental bone lesions, with osteochondroma the most frequent benign bone tumor. Enchondroma is mostly asymptomatic, and yet it is probably the second most common primary bone tumor. Similarly, its malignant counterpart, chondrosarcoma, is the second most common malignant primary bone tumor.The 2013 World Health Organization (WHO) updated this group of tumors, and grade 1 chondrosarcoma was renamed “atypical cartilage tumor” and classified as an intermediate type of tumor, not a malignancy, which better describes its clinical behavior.In this article we summarize changes made in the updated 2013 WHO classification and highlight the diagnostic features differentiating an enchondroma from a low-grade chondrosarcoma. We also describe practical imaging aspects of the remaining chondroid tumors.

scholarly journals Radiohistogenomics of pediatric low-grade neuroepithelial tumors

2021 ◽  
Author(s):  
Asim K. Bag ◽  
Jason Chiang ◽  
Zoltan Patay
Keyword(s):  
Holistic Approach ◽  
Genetic Alterations ◽  
Genetic Alteration ◽  
World Health ◽  
Low Grade ◽  
Neuroepithelial Tumors ◽  
Molecular Features ◽  
Standard Clinical Practice ◽  
Imaging Characteristics ◽  
Health Organization

Abstract Purpose In addition to histology, genetic alteration is now required to classify many central nervous system (CNS) tumors according to the most recent World Health Organization CNS tumor classification scheme. Although that is still not the case for classifying pediatric low-grade neuroepithelial tumors (PLGNTs), genetic and molecular features are increasingly being used for making treatment decisions. This approach has become a standard clinical practice in many specialized pediatric cancer centers and will likely be more widely practiced in the near future. This paradigm shift in the management of PLGNTs necessitates better understanding of how genetic alterations influence histology and imaging characteristics of individual PLGNT phenotypes. Methods The complex association of genetic alterations with histology, clinical, and imaging of each phenotype of the extremely heterogeneous PLGNT family has been addressed in a holistic approach in this up-to-date review article. A new imaging stratification scheme has been proposed based on tumor morphology, location, histology, and genetics. Imaging characteristics of each PLGNT entity are also depicted in light of histology and genetics. Conclusion This article reviews the association of specific genetic alteration with location, histology, imaging, and prognosis of a specific tumor of the PLGNT family and how that information can be used for better imaging of these tumors.

scholarly journals Cerebellopontine angle ependymoma presenting as isolated hearing loss in an elderly patient: A case report and literature review

2021 ◽  
Vol 12 ◽  
pp. 572
Author(s):  
Khaled Dibs ◽  
Rahul Neal Prasad ◽  
Kajal Madan ◽  
Kevin Liu ◽  
Will Jiang ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Local Recurrence ◽  
Cerebellopontine Angle ◽  
Case Reports ◽  
Disease Recurrence ◽  
World Health ◽  
Adjuvant Radiation ◽  
Who Grade ◽  
Imaging Characteristics ◽  
Grade Ii

Background: Ependymoma is an uncommon tumor accounting for approximately 1.9% of all adult central nervous system tumors. Ependymomas at the cerebellopontine angle (CPA) are even more rare and only previously described in isolated case reports. Typically, acoustic neuromas and meningiomas represent the bulk of adult CPA tumors. Diagnosis can be challenging, as ependymomas have clinical findings and imaging characteristics that overlap with more common tumor histologies at the CPA. Case Description: We present the case of a 70-year-old male patient with progressive, isolated left-sided hearing loss found to have a World Health Organization (WHO) Grade II CPA ependymoma, representing one of the oldest recorded patients presenting with this primarily pediatric malignancy in this unique location. The patient presentation with isolated hearing loss was particularly unusual. When associated with neurologic deficits, CPA ependymomas more characteristically result in facial nerve impairment with fully preserved hearing, while vestibular schwannomas tend to present with isolated hearing loss. The standard of care for pediatric ependymomas is maximal safe resection with adjuvant radiotherapy, but treatment paradigms in adult CPA ependymoma are not well defined particularly for WHO Grade II disease. After resection, he received adjuvant radiation to decrease the risk of local recurrence. Twenty-nine months after resection, the patient remains free of treatment-related toxicity or disease recurrence. Conclusion: We review this patient’s clinical course in the context of the literature to highlight the challenges associated with timely diagnosis of this rare tumor and the controversial role of adjuvant therapy in preventing local recurrence in these patients.

Myxofibrosarcoma: clinical and prognostic value of MRI features

2020 ◽  
Vol 16 ◽  
Author(s):  
Paolo Spinnato ◽  
Andrea Sambri ◽  
Tomohiro Fujiwara ◽  
Luca Ceccarelli ◽  
Roberta Clinca ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Local Recurrence ◽  
Contrast Enhancement ◽  
Imaging Modality ◽  
Soft Tissue Sarcomas ◽  
The Elderly ◽  
High Rate ◽  
Imaging Features ◽  
High Grade ◽  
Mri Features

: Myxofibrosarcoma is one of the most common soft tissue sarcomas in the elderly. It is characterized by an extremely high rate of local recurrence, higher than other soft tissue tumors, and a relatively low risk of distant metastases.Magnetic resonance imaging (MRI) is the imaging modality of choice for the assessment of myxofibrosarcoma and plays a key role in the preoperative setting of these patients.MRI features associated with high risk of local recurrence are: high myxoid matrix content (water-like appearance of the lesions), high grade of contrast enhancement, presence of an infiltrative pattern (“tail sign”). On the other hand, MRI features associated with worse sarcoma specific survival are: large size of the lesion, deep location, high grade of contrast enhancement. Recognizing the above-mentioned imaging features of myxofibrosarcoma may be helpful to stratify the risk for local recurrence and disease-specific survival. Moreover, the surgical planning should be adjusted according to the MRI features

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