scholarly journals Clinicopathologic Characteristics and Outcome Descriptors of Metaplastic Breast Carcinoma

Author(s):  
Evi Abada ◽  
Fayez Daaboul ◽  
Kingsley Ebare ◽  
Hyejeong Jang ◽  
Ziad Fehmi ◽  
...  

Context.— Metaplastic breast carcinoma is an aggressive form of breast cancer that accounts for 0.5% to 3% of all breast cancers. Objective.— To study the clinicopathologic characteristics and outcomes of this rare disease. Design.— Retrospective study of patients with a diagnosis of metaplastic breast carcinoma between 2000 and 2019. Hematoxylin-eosin–stained slides were reviewed and additional clinical data were obtained from electronic medical records. Univariable and multivariable Cox proportional hazard regression analyses were used to determine associations between overall survival and several clinicopathologic variables. Results.— Of the 125 patients with metaplastic breast carcinoma identified, only patients with high-grade disease (N = 115) were included in the data analysis. A total of 38 participants (33%) were white, 66 (57%) were African American, and 11 (10%) belonged to other ethnicities. The median age at diagnosis was 57 years. The median tumor size was 3 cm. Heterologous histology was seen in 30% of cases. Multivariable analyses showed that patients with a larger tumor size had worse overall survival (hazard ratio [HR], 1.25; 95% CI, 1.10–1.44; P < .001). Distant metastatic disease was also associated with worse overall survival on multivariable analysis (HR, 10.27; 95% CI, 2.03–55.54; P = .005). In addition to treatment with either partial or complete mastectomies, 84 patients (73%) received chemotherapy. Multivariable analyses showed that chemotherapy had no effect on overall survival (HR, 0.53; 95% CI, 0.09–6.05; P = .55). Conclusions.— A larger tumor size and distant metastatic disease are associated with worse overall survival in patients with metaplastic breast carcinoma. Additional studies are needed to further characterize our findings.

Author(s):  
Vithya Sanmugasiva ◽  
Marlina Tanty Ramli Hamid ◽  
Farhana Fadzli ◽  
Nazimah Ab Mumin ◽  
Kartini Rahmat

Introduction: Metaplastic breast carcinoma is an uncommon malignancy that constitutes < 5% of all breast cancers. There are 5 subtypes which are spindle cell, squamous cell, carcinosarcoma, matrix-producing and metaplastic with osteoclastic giant cells. Spindle cell carcinoma represents approximately <0.3% of invasive breast carcinomas. It is typically a triple-negative cancer with distinct pathological characteristics, but relatively a non-conclusive imaging findings. Case report: An elderly lady presented with an enlarging painful left breast lump for 1 year. Palpable left breast lump noted on clinical examination. Mammography demonstrated a high density, oval lesion with a partially indistinct margin. Corresponding ultrasound showed a large irregular heterogeneous lesion with solid-cystic areas. Histopathology showed atypical spindle-shaped cells which stained positive for cytokeratins and negative for hormone and human epidermal growth factor receptors, which favours spindle cell metaplastic carcinoma. Left mastectomy and axillary dissection were performed, and the final diagnosis was consistent with metaplastic spindle cell carcinoma. Conclusion: Spindle cell carcinoma of the breast is a rare aggressive histological type of carcinoma which may present with benign features on imaging. Tissue diagnosis is essential for prompt diagnosis with multidisciplinary team discussion to guide management and improve patient’s outcome.


2021 ◽  
Vol 39 (6_suppl) ◽  
pp. 361-361
Author(s):  
Nandini Sharrel Menon ◽  
Devanshi Kalra ◽  
Kumar Prabhash ◽  
Vanita Noronha ◽  
Santosh Menon ◽  
...  

361 Background: Primitive neuroectodermal tumours (PNET) of the kidney are rare tumours with aggressive behaviour. This study was conducted to review the diagnosis and management of patients with renal PNET at our centre. Methods: This was a retrospective study conducted at a tertiary cancer care centre in Mumbai, India. The demographic and clinical data of 17 patients treated by the uro-oncology services were retrieved from electronic medical records. Descriptive analysis was performed for baseline characteristics.Overall & progression-free survival was determined using the Kaplan Meier method. Cox regression was used for multivariate analysis. Results: There were 12 male and 5 female patients in this cohort with a median age of 27 years. At diagnosis 2 patients had metastatic disease and 15 patients had non-metastatic disease. Median follow up in this cohort was 22 months (range 2-30 months). Presenting complaints were hematuria, abdominal pain, flank pain, fever, bone pain, and incidentally detected renal mass. All patients were Mic -2 positive and 13 were FLI-1 positive on immunohistochemistry. Fourteen patients underwent radical nephrectomy. One (5.9%) patient received both neoadjuvant and adjuvant chemotherapy, 8 (47.1%) received adjuvant and 2 (11.8%) received palliative chemotherapy upfront. Eight patients received adjuvant radiation to the renal bed.There was disease progression in12 patients,10 of 15 patients with non metastatic disease at diagnosis eventually developed metastasis.The median progression free survival (PFS) was 10.55 months.The pathological feature that was associated with a shorter PFS was tumor size ⩾10 cm(p = 0.044).The median overall survival was 20.04 months (95% CI 9.49 -not reached). The presence of metastasis and treatment received significantly impacted overall survival (OS). Median OS in patients with non-metastatic disease was not reached versus 14.1 months in those with metastatic disease (p = .019).The median OS in patients treated with multimodality approach was 20.11 months. Patients did not undergo surgery had a median OS of 5.45 months (p < .001) and those who did not receive any chemotherapy had a median OS of 4.57 months (p = .024).Thus, patients who received multimodality treatment had better outcomes. Conclusions: PNET kidney is an aggressive tumor which should be treated with a multimodality approach. Tumor size ⩾10 cm was an adverse prognostic factor.


Sarcoma ◽  
2020 ◽  
Vol 2020 ◽  
pp. 1-8
Author(s):  
Jules Lansu ◽  
Winan J. Van Houdt ◽  
Michael Schaapveld ◽  
Iris Walraven ◽  
Michiel A. J. Van de Sande ◽  
...  

Background. The purpose of this study was to evaluate the overall survival (OS) and associated characteristics for patients with Myxoid Liposarcoma (MLS) over time in The Netherlands. Methods. A population-based study was performed of patients with primary localized (n = 851) and metastatic (n = 50) MLS diagnosed in The Netherlands between 1989 and 2016, based on data from the National Cancer Registry. Results. The median age of the MLS patients was 49 years, and approximately two-thirds was located in the lower limb. An association was revealed between age and the risk of having a Round Cell (RC) tumor. OS rates for primary localized MLS were 93%, 83%, 78%, and 66% after 1, 3, 5, and 10 years, respectively. The median OS for patients with metastatic disease at diagnosis was 10 months. Increasing age (Hazard Ratio (HR) 1.05, p=0.00), a tumor size >5 cm (HR 2.18; p=0.00), and tumor location (trunk HR 1.29; p=0.09, upper limb HR 0.83; p=0.55, and “other” locations HR 2.73; p=0.00, as compared to lower limb) were independent prognostic factors for OS. The percentage of patients treated with radiotherapy (RT) increased over time, and preoperative RT gradually replaced postoperative RT. In contrast to patients with localized disease, significant improvement of OS was observed in patients with metastatic disease over time. Conclusions. In this large nationwide cohort, tumor size and tumor location were independent prognostic factors for OS. Furthermore, a higher probability of an RC tumor with increasing age was suggested. An increased use of RT over the years did not translate into improved OS for localized MLS.


2020 ◽  
Vol 22 (11) ◽  
pp. 1647-1657 ◽  
Author(s):  
James L Leach ◽  
James Roebker ◽  
Austin Schafer ◽  
Joshua Baugh ◽  
Brooklyn Chaney ◽  
...  

Abtract Background This study describes imaging features of diffuse intrinsic pontine glioma (DIPG) and correlates with overall survival (OS) and histone mutation status in the International DIPG Registry (IDIPGR). Methods Four hundred cases submitted to the IDIPGR with a local diagnosis of DIPG and baseline MRI were evaluated by consensus review of 2 neuroradiologists; 43 cases were excluded (inadequate imaging or alternative diagnoses). Agreement between reviewers, association with histone status, and univariable and multivariable analyses relative to OS were assessed. Results On univariable analysis imaging features significantly associated with worse OS included: extrapontine extension, larger size, enhancement, necrosis, diffusion restriction, and distant disease. On central review, 9.5% of patients were considered not to have DIPG. There was moderate mean agreement of MRI features between reviewers. On multivariable analysis, chemotherapy, age, and distant disease were predictors of OS. There was no difference in OS between wild-type and H3 mutated cases. The only imaging feature associated with histone status was the presence of ill-defined signal infiltrating pontine fibers. Conclusions Baseline imaging features are assessed in the IDIPGR. There was a 9.5% discordance in DIPG diagnosis between local and central review, demonstrating need for central imaging confirmation for prospective trials. Although several imaging features were significantly associated with OS (univariable), only age and distant disease were significant on multivariable analyses. There was limited association of imaging features with histone mutation status, although numbers are small and evaluation exploratory.


Author(s):  
Benjamin L. Franc ◽  
Christi DeLemos ◽  
Christopher Jones

AbstractIntroductionCombined modality treatment regimens have provided modest gains in locoregional control rates of cancers of the head and neck (HNC), and intensity-modulated radiation therapy (IMRT) has gained widespread use. The methodology for determining contours of the gross tumour volume (GTV) in the radiation treatment plan is often based on combined anatomic and metabolic data from positron emission tomography–computed tomography (PET-CT). This study aimed to retrospectively evaluate the overall survival and disease-free survival outcomes of patients with HNC who received definitive IMRT with or without chemotherapy, planned with PET-CT.Materials and MethodsA total of 1,200 patients underwent treatment for HNC during the study period, from 1 January 2002 to 31 December 2010. Of those, 261 cases had evaluable data that met the inclusion criteria for the study. The incidence and timing of locoregional recurrence, distant metastatic disease, new primary malignancies and death were evaluated retrospectively. Overall and disease-free survival (survival to time of first recurrence) were determined by the life table method. Incidence of distance metastatic disease and additional cancers were also studied.ResultsMedian follow-up from treatment initiation was 26·4 months (range 1·2–84·7 months). Overall survival and disease-free survival rates were 0·883 and 0·791, respectively, at 1 year; 0·793 and 0·688, respectively, at 2 years; and 0·732 and 0·619, respectively, at 3 years. The cumulative risk of recurrence was 22·6% at 3 years following definitive IMRT and the median time to recurrence was 345 days. There was an overall low incidence of distant metastatic disease (3·07%) and additional cancers (8·05%).ConclusionOverall and disease-free survival outcomes of a large cohort of HNC patients treated with definitive IMRT radiotherapy following treatment planning with PET-CT shows a similar high level of disease control and mortality rate as previously published outcome studies of shorter terms and/or smaller numbers of patients.


2019 ◽  
Author(s):  
Tiffany C. Chang ◽  
Margarite D. Matossian ◽  
Steven Elliott ◽  
Hope E. Burks ◽  
Rachel A. Sabol ◽  
...  

AbstractMetaplastic breast carcinoma (MBC) is a clinically aggressive and rare subtype of breast cancer, with similar features to basal-like breast cancers. Due rapid growth rates and characteristic heterogeneity, MBC is often unresponsive to standard chemotherapies; and novel targeted therapeutic discovery is urgently needed. Histone deacetylase inhibitors (DACi) suppress tumor growth and metastasis through regulation of the epithelial-to-mesenchymal transition axis in various cancers, including basal-like breast cancers.We utilized a new MBC patient-derived xenograft (PDX) to examine the effect of DACi therapy on MBC. Cell morphology, cell cycle-associated gene expressions, transwell migration, and metastasis were evaluated in patient-derived cells and tumors after treatment with romidepsin and panobinostat. Derivations of our PDX model, including cells, spheres, organoids, explants, and in vivo implanted tumors were treated. Finally, we tested the effects of combining DACi with approved chemotherapeutics on relative cell biomass.DACi significantly suppressed the total number of lung metastasis in vivo using our PDX model, suggesting a role for DACi in preventing circulating tumor cells from seeding distal tissue sites. These data were supported by our findings that DACi reduced cell migration, populations, and expression of mesenchymal-associated genes. While DACi treatment did affect cell cycle-regulating genes in vitro, tumor growth was not affected compared to controls. Importantly, gene expression results varied depending on the cellular or tumor system used, emphasizing the importance of using multiple derivations of cancer models in preclinical therapeutic discovery research. Furthermore, DACi sensitized and produced a synergistic effect with approved oncology therapeutics on inherently resistant MBC.This study introduced a role for DACi in suppressing the migratory and mesenchymal phenotype of MBC cells through regulation of the epithelial-mesenchymal transition axis and suppression of the CTC population. Preliminary evidence that DACi treatment in combination with MEK1/2 inhibitors exerts a synergistic effect on MBC cells was also demonstrated.


2019 ◽  
Vol 13 (1) ◽  
Author(s):  
Matthew W. McGee ◽  
Sarag A. Boukhar ◽  
Varun Monga ◽  
Ronald Weigel ◽  
Sneha D. Phadke

Abstract Background Dermatofibrosarcoma protuberans is a rare soft tissue malignancy that, if left untreated, can be locally destructive and life-threatening. Dermatofibrosarcoma protuberans is uncommon in the breast, and the similarity of its morphologic features with other spindle cell malignancies can make correct identification difficult. Immunohistochemistry and molecular testing can aid in the correct diagnosis when there is diagnostic uncertainty. Imatinib, a selective tyrosine kinase inhibitor, has been used for adjuvant treatment of dermatofibrosarcoma protuberans following surgical resection. When used as a neoadjuvant treatment, imatinib offers the opportunity to decrease tumor size prior to surgery to lessen the chance for disfigurement. Case presentation We present the case of a Caucasian woman who was 46-year-old when she first noted a mass in her right breast in 2015; she was initially diagnosed as having metaplastic breast carcinoma. Mastectomy and systemic chemotherapy were planned; however, after review of pathology at a referral center, the diagnosis was changed to dermatofibrosarcoma protuberans. She was treated with 4 months of neoadjuvant imatinib with adequate tumor shrinkage to perform breast conservation. Conclusion This patient’s case stresses the importance of correctly diagnosing this rare breast tumor through the histopathologic appearance of dermatofibrosarcoma protuberans, molecular pathogenesis, and immunohistochemistry. These techniques can help differentiate dermatofibrosarcoma protuberans from metaplastic breast carcinoma and other spindle cell lesions of the breast. This is critical, as the treatment options for metaplastic breast carcinoma significantly differ from treatment options for dermatofibrosarcoma protuberans. This case describes the use of imatinib as a neoadjuvant option to reduce preoperative tumor size and improve surgical outcomes.


2021 ◽  
Vol 39 (15_suppl) ◽  
pp. 4108-4108
Author(s):  
Diana Hsu ◽  
Sidney Le ◽  
Alex Chang ◽  
Austin Spitzer ◽  
George Kazantsev ◽  
...  

4108 Background: Pancreatic neuroendocrine tumors (PNET) are a heterogeneous group of tumors that represent 1-2% of all pancreatic neoplasms. Their biologic behaviors are unpredictable with high grade, nodal metastasis, or liver metastasis lending an unfavorable prognosis. Current guidelines recommend resection for functioning tumors and those 2 cm or larger but are less straightforward regarding tumors < 2 cm in size. Previous data show that observation for nonfunctioning tumors < 2 cm can be safe and feasible; however, a significant portion of these patients may have nodal involvement or metastatic disease. Methods: A retrospective review was undertaken to identify patients with pancreatic neuroendocrine tumors treated at Northern California Kaiser Permanente (KP-NCAL) between February 2010 and December 2018. Univariate and multivariate analyses were performed with the log-rank test and Cox regression. Chi-squared test of relevant clinicopathologic factors determined which factors were predictive for overall survival (OS). Results: Mean age was 61 years in our cohort of 354 patients, with 29% over the age of 70. Mean tumor size was 3.43 cm; 32% of tumors were 2 cm or smaller. 51% of the patients had localized disease; 32% of the patients presented with metastatic disease. The pancreatic tail was the most common tumor location (38%), followed by the head of the pancreas (24%). On multivariate survival analysis, stage, location of the tumor, and surgical resection were statistically significant in terms of overall survival ( p<.001). Mean OS for patients with localized and metastatic disease was 93 months versus 37 months ( p<.001). Surgery was utilized in 8.9% of patients with metastatic disease ( p<.001). All patients with PNET smaller than 1 cm in our study group had localized disease only. However, in patients with tumor size between 1 and 2 cm, 11% had nodal or metastatic spread. Conclusions: PNETs are indolent but have malignant potential at any size. In our retrospective study, all of the patients with tumor size < 1 cm had localized disease. For those with PNETs 1-2 cm in size, 11% had nodal or metastatic spread. Based on our findings, we suggest a more aggressive surgical resection size criteria of 1 cm.[Table: see text]


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