Solitary Cardiac Low-Grade Neuroendocrine Tumor: A Case Report and Review of the Literature

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S47-S47
Author(s):  
M Kavesh ◽  
P Drew ◽  
B Stewart
Keyword(s):  
Neuroendocrine Tumor ◽  
Frozen Section ◽  
Mitotic Rate ◽  
Complete Resection ◽  
Low Grade ◽  
Primary Tumors ◽  
Radiotracer Uptake ◽  
Pet Ct ◽  
The Right

Abstract Introduction/Objective Primary tumors of the heart are uncommon; even rarer are primary cardiac neuroendocrine tumors. To our knowledge, only two cases have been described to date, both being high-grade tumors. We report a solitary low-grade neuroendocrine tumor of the heart, unexpectedly discovered on the wall of the right ventricle in a 44-year-old man during aortic valve repair for infectious bacterial endocarditis. Results Frozen section was sent intraoperatively and showed a plasmacytoid neoplasm. Final pathology of the biopsies showed a tumor composed of both cohesive and discohesive plasmacytoid cells separated by a vascular network and strands of fibrosis. Neither necrosis nor a mitotic rate greater than 2 mitoses per 2 mm2 were seen. The tumor showed strong reactivity for AE1/3, synaptophysin and CDX2 with focal reactivity for chromogranin-A and CD56, confirming the diagnosis of a low-grade neuroendocrine tumor. Both an esophagogastroduodenoscopy and colonoscopy were performed and showed no evidence of a primary gastrointestinal tumor. PET-CT DOTATATE performed after complete resection of the tumor showed no abnormal radiotracer uptake. The possibility of an intestinal neuroendocrine tumor undetected by DOTATATE PET-CT with a single known metastasis to the heart was considered. This too, on the basis of the literature, was considered exceedingly rare. Complete resection of this patient’s tumor was considered sufficient treatment; the patient was advised to follow up annually with medical oncology. Conclusion The presentation and management of a solitary cardiac low-grade neuroendocrine tumor has not previously been described in the literature.

scholarly journals Searching for primaries in patients with neuroendocrine tumors (NET) of unknown primary and clinically suspected NET: Evaluation of Ga-68 DOTATOC PET/CT and In-111 DTPA octreotide SPECT/CT

2014 ◽  
Vol 48 (4) ◽  
pp. 339-347 ◽  
Author(s):  
Nils Friedemann Schreiter ◽  
Ann-Mirja Bartels ◽  
Vera Froeling ◽  
Ingo Steffen ◽  
Ulrich-Frank Pape ◽  
...  
Keyword(s):  
Neuroendocrine Tumor ◽  
Neuroendocrine Tumors ◽  
Clinical Efficacy ◽  
Unknown Primary ◽  
Primary Tumors ◽  
Pet Ct ◽  
Clinical Verification

Abstract Background. To evaluate the clinical efficacy of In-111 DTPA octreotide SPECT/CT and Ga-68 DOTATOC PET/CT for detection of primary tumors in patients with either neuroendocrine tumor of unknown primary (NETUP) or clinically suspected primary NET (SNET). Patients and methods. A total of 123 patients were included from 2006 to 2009, 52 received Ga-68 DOTATOC PET/CT (NETUP, 33; SNET, 19) and 71 underwent In-111 DTPA octreotide SPECT/CT (50; 21). The standard of reference included histopathology or clinical verification based on follow-up examinations. Results. In the NETUP group Ga-68 DOTATOC detected primaries in 15 patients (45.5%) and In-111 DTPA octreotide in 4 patients (8%) (p < 0.001); in the SNET group, only 2 primaries could be detected, all by Ga-68 DOTATOC. In patients with NETUP, primary tumors could be found significantly more often than in patients with SNET (p = 0.01). Out of these 21 patients 14 patients were operated. Conclusion. Ga-68 DOTATOC PET/CT is preferable to In-111 DTPA octreotide SPECT/CT when searching for primary NETs in patients with NETUP but should be used with caution in patients with SNET.

scholarly journals Evaluation of the Role of Early Second Look Cystoscopy after Complete Resection of Non-Muscle Invasive Bladder Tumors

QJM ◽  
2020 ◽  
Vol 113 (Supplement_1) ◽  
Author(s):  
K A Taema ◽  
M Shabayek ◽  
A M Tawfeek ◽  
A M Emam ◽  
M M S Khafaga
Keyword(s):  
Tnm Classification ◽  
Transitional Cell ◽  
Complete Resection ◽  
Prospective Clinical Study ◽  
Low Grade ◽  
Bladder Tumors ◽  
Primary Tumors ◽  
Multiple Tumors ◽  
Second Look ◽  
Muscle Invasive

Abstract Background most of the bladder cancers are non-muscle invasion at the time of diagnosis. Non-Muscle invasive tumors confined to the mucosa and invading the lamina propria are classified as stage Ta and T1, respectively, according to the Tumor, Node, Metastasis (TNM) classification system. Aim of the Study to determine the efficacy of early second look cystoscopy after complete resection of non-muscle invasive urinary bladder tumors and its impact on subsequent treatment policy. Patients and Methods this is a prospective clinical study that was done at Urology Department, Faculty of Medicine, Ain Shams University and Damanhur Oncology Centre. A total of 40 consecutive patients with newly diagnosed non-muscle invasive bladder cancer will be enrolled in this prospective study for second-look cystoscopy with TURBT in two to six weeks after initial TURBT and follow them up in one year. Results Single tumor was found in 22 cases (55%), while multiple tumors were recognized in 18 cases (45%) with average sizes ranging from 12mm to 70mm. Low-grade transitional cell carcinoma (TCC) was found in 26 cases (65%), whereas high-grade TCC was found in 14 cases (35%). Stage PTa tumors was found in 12 cases (30%) while 28 cases (70%) have had tumors of PT1 stage. All cases underwent an early second TURBT after (2-6) weeks which has reviled the following data. The majority of cases (28 cases) representing 70% of whole study population were free with no detection of any tumors either residual or recurrence. Residual tumors were detected in six cases (15%) all of which detected in patient with large primary tumor size exceeding 50mm or patients with multiple tumors. Whole detected residual tumors were found at edges of primary tumors ranging 2-5 mm in size which were completely resected and they were of the same stage and grade as the initial tumor. Conclusion Our findings in this study support the notion that second-look cystoscopy with or without TURBT is a prerequisite in patients with superficial bladder cancers except for patients with solitary, small low-grade pTa tumors. It is particularly highly recommended for patients with extensive and multiple tumors at the first TURBT

Atypical pediatric ganglioglioma is common and associated with a less favorable clinical course

2016 ◽  
Vol 17 (1) ◽  
pp. 41-48 ◽  
Author(s):  
Mohana Rao Patibandla ◽  
Thomas Ridder ◽  
Kathleen Dorris ◽  
Michelle R. Torok ◽  
Arthur K. Liu ◽  
...  
Keyword(s):  
Temporal Lobe ◽  
Clinical Course ◽  
Oncological Outcome ◽  
Tumor Location ◽  
Complete Resection ◽  
Low Grade ◽  
Imaging Features ◽  
Total Resection ◽  
Brainstem Death

OBJECT Ganglioglioma (GG) is commonly recognized as a low-grade tumor located in the temporal lobe, often presenting with seizures. Most are amenable to complete resection and are associated with excellent oncological outcome. The authors encountered several GGs in various locations, which seem to have a less favorable clinical course than GGs in the temporal lobe. METHODS The authors performed a single-center retrospective review of all children with a histological diagnosis of GG who were treated at Children’s Hospital Colorado between 1997 and 2013. Each tumor was categorized by 2 pediatric neuroradiologists as typical or atypical based on preoperative MRI appearance. Typical lesions were cortically based, within a single cerebral lobe, well-circumscribed, and solid or mixed solid/cystic. The treatment and clinical course of each patient was analyzed. RESULTS Thirty-seven children were identified, with a median age at presentation of 8.2 years and median follow-up of 38.0 months. Eighteen tumors (48.6%) were typical and 19 (51.4%) were atypical. All typical lesions presented with seizures, whereas no atypical lesions did so. Sixteen (88.9%) typical lesions were located in the temporal lobe. In the atypical group, tumor location was variable, including 11 (57.9%) in the brainstem. Death during follow-up was statistically more common in the atypical group (31.6% vs 0%, p = 0.02). Gross-total resection (GTR) was achieved for 15 of 16 typical tumors (93.8%), compared with 3 atypical tumors (15.8%, p < 0.0001). Presentation with seizure or non-brainstem location were each associated with survival (p = 0.02 and 0.004, respectively). The presence of mutation in BRAF exon 15 did not differ between the 2 groups. CONCLUSIONS Pediatric GG with typical imaging features is associated with excellent rates of GTR and overall survival. Atypical GG is commonly encountered, less amenable to GTR, and associated with a worse outcome. This may relate to anatomical or biological characteristics and merits further investigation.

Follicular Dendritic Cell Sarcoma (FDCS): A Rare and Incurable Disease.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3892-3892
Author(s):  
Andres O. Soriano ◽  
Michael Thompson ◽  
Jorge Romaguera ◽  
Alma Rodriguez ◽  
Fredrick B. Hagemeister ◽  
...  
Keyword(s):  
Dendritic Cell ◽  
Stable Disease ◽  
Initial Treatment ◽  
Castleman’S Disease ◽  
Mitotic Rate ◽  
Cancer Center ◽  
Low Grade ◽  
High Grade ◽  
Md Anderson

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.

PET/CT a New Marker of Response in Waldenstrom Macroglobulinemia (WM).

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 1937-1937
Author(s):  
Xavier Leleu ◽  
Renee Leduc ◽  
Meghan Rourke ◽  
Brianna Harris ◽  
Aldo M. Roccaro ◽  
...  
Keyword(s):  
Predictive Value ◽  
Fdg Pet ◽  
Low Grade ◽  
Minor Response ◽  
Female Ratio ◽  
Probability Of Survival ◽  
Pet Ct ◽  
Before And After ◽  
Pet Scans

Abstract Abstract 1937 Poster Board I-960 Background. Waldenstrom's macroglobulinemia is a rare B cell neoplasm characterized by the production of a monoclonal IgM protein and a lymphoplasmacytic infiltrate in the bone marrow. The clinical manifestations related to tumor infiltration include hepatomegaly (20%), splenomegaly (15%) and lymphadenopathy (15%). Organomegaly was associated with adverse prognosis in a large series of WM. More sensitive tools of tumor burden and prognosis are needed in these patients. The use of FDG-PET has not been previously studied in WM but has proved an effective diagnostic and prognostic tool in other in low-grade lymphomas. Therefore the objective of this study was to determine whether FDG-PET was an effective tool in evaluating pts with WM. Methods. We prospectively studied PET/CT in 39 WM patients homogeneously treated with bortezomib-rituximab (given IV bortezomib 1.6mg/m2 at days 1, 8, 15 q 28 days × 6 cycles and rituxan 375 mg/m2 at days 1, 8, 15, 22 on cycles 1 and 4) on a phase II clinical trial, at diagnosis (N=12) and with relapsed/refractory disease (N=27). All pts underwent staging evaluation by FDG-PET in combination with CT scan before and after therapy. PET results were analyzed as positive or negative. Response (ORR) was assessed after cycle 3, confirmed with 2 consecutive values and included minor response or better. Overall (OS) and treatment free survivals (TFS) were calculated from start of treatment to date of last follow up and time of next treatment, respectively. Results. The median age of the population was 62 years (range, 43-78), Male/Female ratio 1.64, WM-International staging score breakdown was 46% low, 23% intermediate, 31% high. Serum M-spike was 2.5g/L (0.41-4.62) with 8% patients >= 4g/L. The overall response rate was 89.7% with minor response in 13 pts and major response in 22 pts. With a median (+/-se) follow-up of 15 months (+/-1.22), death occurred in 2 patients, and the median OS was not reached with a 3-year probability of survival of 89%. The median TFS was 21 months (+/-2.09). Twenty-five (64.1%) and 13 (37.1%) patients had a positive PET before and after treatment, respectively. 11 (45.8%) patients had a negative post treatment PET which was positive before treatment, 1 (4.8%) had a positive PET after treatment while initially negative and all other patients had no change. Patients with positive PET before treatment had no clinical-biological difference (age, gender, hemoglobin level, serum beta 2-microglobulin value, platelet count, IgM spike and ISS-WM score) with other patients. A positive PET before treatment had no influence on either OS or TFS or ORR or MR. However, a normal PET after treatment, including a negative PET after treatment which initially was positive before treatment, correlated with response (p=0.04). The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) for a normal PET after treatment and for a negative PET after treatment while initially positive before treatment in predicting ORR were 68.7%, 100%, 100%, 23% and 52.4%, 100%, 100%, 23.1%, respectively. Although the number of deaths is low in this series, a positive PET after treatment was an adverse prognostic factor for OS. The median survival and the 2-year probability of survival was not reached and 100% for patients with a normal (negative) PET after treatment (number of death/number of pts in the group, O/N=0/22) while it was 20 months and 46% for patients with a positive PET after treatment (O/N=2/13 ), respectively (p=0.019). Conclusion: Over 60% of WM pts demonstrated FDG-avid disease when using FDG-PET scans with the majority showing negative imaging after therapy. PET positive scans after therapy correlated with poor prognosis. FDG-PET scans may prove an effective tool in the diagnosis and prognosis in WM. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Paravertebral Well-Differentiated Liposarcoma with Low-Grade Osteosarcomatous Component: Case Report with 11-Year Follow-Up, Radiological, Pathological, and Genetic Data, and Literature Review

2017 ◽  
Vol 2017 ◽  
pp. 1-6
Author(s):  
Nicolas Macagno ◽  
Stéphane Fuentes ◽  
Gonzague de Pinieux ◽  
André Maues de Paula ◽  
Sébastien Salas ◽  
...  
Keyword(s):  
Histopathological Examination ◽  
Soft Tissue Sarcomas ◽  
Fish Analysis ◽  
Low Grade ◽  
Central Mass ◽  
Divergent Differentiation ◽  
Paravertebral Muscles ◽  
Well Differentiated ◽  
The Right

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.

scholarly journals Evaluation of SUV normalized by lean body mass (SUL) in 68Ga-PSMA11 PET/CT: a bi-centric analysis

2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Andrei Gafita ◽  
Jeremie Calais ◽  
Charlott Franz ◽  
Isabel Rauscher ◽  
Hui Wang ◽  
...  
Keyword(s):  
Body Weight ◽  
Lean Body Mass ◽  
Body Mass ◽  
Standardized Uptake Value ◽  
Ct Scans ◽  
Volume Of Interest ◽  
Pet Ct ◽  
Positive Correlations ◽  
The Right

Abstract Introduction The aim of this analysis was to investigate whether the standardized uptake value (SUV) normalized by lean body mass (SUL) is a more appropriate quantitative parameter compared to the commonly used SUV normalized by patient’s weight in 68Ga-PSMA11 PET/CT. Material and methods 68Ga-PSMA11 PET/CT scans of 121 patients with prostate cancer from two institutions were evaluated. Liver SUV was measured within a 3-cm volume-of-interest (VOI) in the right hepatic lobe and corrected for lean body mass using the Janmahasatian formula. SUV and SUL repeatability between baseline and follow-up scans of the same patients were assessed. Results SUV was significantly positively correlated with body weight (r = 0.35, p = 0.02). In contrast, SUL was not correlated with body weight (r = 0.23, p = 0.07). No significant differences were found between baseline and follow-up scan (p = 0.52). Conclusion The Janmahasatian formula annuls the positive correlations between SUV and body weight, suggesting that SUL is preferable to SUV for quantitative analyses of 68Ga-PSMA11 PET/CT scans.

scholarly journals Cardiac myxoma: The influence of preoperative clinical presentation and surgical technique on late outcome

2007 ◽  
Vol 135 (7-8) ◽  
pp. 401-406
Author(s):  
Aleksandar Mikic ◽  
Biljana Obrenovic-Kircanski ◽  
Mladen Kocica ◽  
Mile Vranes ◽  
Vesna Lackovic ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Clinical Presentation ◽  
Cardiac Myxoma ◽  
Surgical Techniques ◽  
Right Atrial ◽  
Functional Improvement ◽  
Primary Tumors ◽  
Cardiac Myxomas ◽  
The Right

Introduction Cardiac myxomas are the most frequent primary tumors of the heart in adults, and they can be found in each of four cardiac chambers. Although biologically benign, due to their unfavorable localization, myxomas are considered "functionally malignant" tumors. Diagnosis of cardiac myxoma necessitates surgical treatment. Objective To analyze: 1) the influence of localization, size and consistency of cardiac myxomas on preoperative symptomatology; 2) the influence of different surgical techniques (left, right, biatrial approach, tumor basis solving) on early, and late outcomes. Method From 1982 to 2000, at the Institute for Cardiovascular Diseases, Clinical Center of Serbia, there were 46 patients with cardiac myxomas operated on, 67.4% of them women, mean age 47.1?16.3 years. The diagnosis was made according to clinical presentation, electrocardiographic and echocardiographic examinations and cardiac catheterization. Follow-up period was 4-18 (mean 7.8) years. Results In 41 (89.1%) patients, myxoma was localized in the left, while in 5 (10.9%), it was found in the right atrium. Average size was 5.8?3.8 cm (range: 1?1 cm to 9?8 cm) and 6?4 cm (range: 3?2 cm to 9?5 cm) for the left and right atrial myxomas, respectively. A racemous form predominated in the left (82.6%) and globous in the right (80%) atrium. Fatigue was the most common general (84.8%) and dyspnoea the most common cardiologic symptom (73.9%). Preoperative embolic events were present in 8 patients (4 pulmonary, 4 systemic). In our series: 1) different localization, size and consistency had no influence on the preoperative symptomatology; 2) surgical treatment applied, regardless of different approaches and basis solving, resulted in excellent functional improvements (63.1% patients in NYHA III and IV class preoperatively vs. 6.7% patients postoperatively) and had no influence on new postoperative rhythm disturbances (8.7% patients preoperatively vs. 24.4% patients postoperatively); 3) early (97.8%), and late survival rates (91.3%) were excellent; 4) there were no relapses during the follow-up period. Conclusion Localization, size and consistency had no influence on the preoperative symptomatology. Excellent survival rate with significant functional improvement, rare postoperative complications and no recurrences, justify the applied strategies of surgical approach and tumor basis solving in our series.

Intramural Leiomyosarcoma of the Dorsal Pedal Vein: A Clinical Mimicry of Ganglion

1994 ◽  
Vol 15 (1) ◽  
pp. 48-51 ◽  
Author(s):  
Louis R. Bégin ◽  
Pierre Guy ◽  
Benjamin Mitmaker
Keyword(s):  
Smooth Muscle ◽  
Low Grade ◽  
Neoplastic Growth ◽  
Dorsal Aspect ◽  
Smooth Muscle Proliferation ◽  
The Right

A 75-year-old woman presented with a ganglion-like nodule on the dorsal aspect of the right foot. A 2.5 × 1.5 cm, saccular and malleable tumor, that was in continuity with the dorsal venous arch, was completely resected. It was characterized by a diffuse intramural and circumferential, low grade, malignant, smooth muscle proliferation with an aneurysmal-like luminal space. No endoluminal or periadventitial invasive neoplastic component was present. The patient had no evidence of disease at 58-month follow-up. This is the first reported case of venous leiomyosarcoma in the foot. Furthermore, the intramural confinement of neoplastic growth is a unique observation.

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