Dedifferentiated Liposarcoma of the Transverse Colon

2021 ◽  
pp. 000313482110545
Author(s):  
Katie Fitzgerald ◽  
Eliza M. Slama ◽  
Irina Bernescu

While liposarcoma is one of the most common soft tissue sarcomas, it is rarely seen within the gastrointestinal tract, and even less frequently seen within the colon. Dedifferentiated liposarcoma is a subtype of liposarcoma, which along with the pleomorphic subtype is considered a high-grade, aggressive tumor; both possess the ability to metastasize and are associated with decreased survival. Despite complete resection, recurrence is common. While surgical excision is the cornerstone of treatment for liposarcoma of the colon, there is no consensus on adjuvant therapies. We present the case of a 66-year-old woman who presented with abdominal pain with rectal bleeding and was found on colonoscopy to have a high-grade dedifferentiated liposarcoma of the transverse colon. She underwent robotic segmental colectomy. Due to absence of nodal involvement or distal metastasis, adjuvant therapy was not administered. On 1-year follow-up, the patient remains disease free.

2019 ◽  
Vol 180 (2) ◽  
pp. 127-134 ◽  
Author(s):  
S Asioli ◽  
A Righi ◽  
M Iommi ◽  
C Baldovini ◽  
F Ambrosi ◽  
...  

Objective and design A clinicopathological score has been proposed by Trouillas et al. to predict the evolution of pituitary adenomas. Aim of our study was to perform an independent external validation of this score and identify other potential predictor of post-surgical outcome. Methods The study sample included 566 patients with pituitary adenomas, specifically 253 FSH/LH-secreting, 147 GH-secreting, 85 PRL-secreting, 72 ACTH-secreting and 9 TSH-secreting tumours with at least 3-year post-surgical follow-up. Results In 437 cases, pituitary adenomas were non-invasive, with low (grade 1a: 378 cases) or high (grade 1b: 59 cases) proliferative activity. In 129 cases, tumours were invasive, with low (grade 2a: 87 cases) or high (grade 2b: 42 cases) proliferative activity. During the follow-up (mean: 5.8 years), 60 patients developed disease recurrence or progression, with a total of 130 patients with pituitary disease at last follow-up. Univariate analysis demonstrated a significantly higher risk of disease persistence and recurrence/progression in patients with PRL-, ACTH- and FSH/LH-secreting tumours as compared to those with somatotroph tumours, and in those with high proliferative activity (grade 1b and 2b) or >1 cm diameter. Multivariate analysis confirmed tumour type and grade to be independent predictors of disease-free-survival. Tumour invasion, Ki-67 and tumour type were the only independent prognostic factors of disease-free survival. Conclusions Our data confirmed the validity of Trouillas’ score, being tumour type and grade independent predictors of disease evolution. Therefore, we recommend to always consider both features, together with tumour histological subtype, in the clinical setting to early identify patients at higher risk of recurrence.


1985 ◽  
Vol 3 (5) ◽  
pp. 698-709 ◽  
Author(s):  
S A Rosenberg ◽  
C A Seipp ◽  
D E White ◽  
R Wesley

One hundred fifty-six patients with high-grade soft-tissue sarcomas of the extremities treated on prospective randomized trials were analyzed to determine the impact of perioperative blood transfusions on tumor recurrence and patient survival. A significant increase in the incidence of tumor recurrence and decrease in survival were associated with the receipt of blood transfusions at the time of definitive surgical therapy of the sarcoma. Actuarial 5-year continuous disease-free survival was 70% in patients who had not been transfused compared to 48% in patients who received one or more transfusions (P = .007). Overall 5-year survival was also substantially decreased in patients receiving transfusions (85% compared to 63%; P = .0035). A direct relationship existed between the number of transfusions administered and the decrease in disease-free and overall survival; the larger the number of transfusions the worse the prognosis (P less than .0001 and P = .0001, respectively). A large number of other prognostic factors were included in the analysis including the age, sex, race of the patient, histology of the primary lesion, anatomic site of the primary lesion, final surgical margins, size of the tumor, type of surgery required, the use of chemotherapy, actual time in the operating room under anesthesia, the exact anesthetic agent used, and the individual surgeon who performed the operation. Accounting for all of these factors a strong association continued to exist between the receipt of blood transfusion and poor patient prognosis. We have previously shown that adjuvant chemotherapy is of benefit to patients with high-grade extremity sarcomas, and 132 (84.6%) of 156 patients in this series received chemotherapy. In patients receiving chemotherapy, blood transfusions were associated with increased recurrence (P less than .0001) and decreased survival (P = .0001). The only other significant independent prognostic variable in these patients was the size of the primary tumor. An analysis of all patients, stratified for tumor size, revealed an impact of transfusions on increasing recurrence (P = .007) and decreasing survival (P = .016). An analysis of the subpopulation of patients with large tumors (greater than 150 mL) gave the same results (P = .03 and .015, respectively). It thus appears that the receipt of blood transfusions is associated with increased tumor recurrence and decreased survival in patients with high-grade soft-tissue sarcomas of the extremities.(ABSTRACT TRUNCATED AT 400 WORDS)


1988 ◽  
Vol 6 (9) ◽  
pp. 1491-1500 ◽  
Author(s):  
A E Chang ◽  
T Kinsella ◽  
E Glatstein ◽  
A R Baker ◽  
W F Sindelar ◽  
...  

We have previously reported the results of a randomized trial that demonstrated the survival benefit of adjuvant chemotherapy in the treatment of patients with high-grade extremity sarcomas compared with no chemotherapy. This regimen included doxorubicin, cyclophosphamide, and methotrexate. This report updates and extends our experience. The median follow-up of this trial is now 7.1 years and reveals a 5-year disease-free survival of 75% and 54% for chemotherapy and no chemotherapy groups, respectively (two-sided P [P2] = .037). The 5-year overall survival for patients in this trial was 83% and 60% for the chemotherapy and no chemotherapy groups, respectively, with a trend towards improved survival in the chemotherapy arm (P2 = .124). Because of doxorubicin-induced cardiomyopathy we performed a subsequent randomized trial comparing this high-dose regimen to reduced cumulative doses of doxorubicin and cyclophosphamide without methotrexate. Eighty-eight patients were entered into this trial which has a median follow-up of 4.4 years. The 5-year disease-free and overall survival for patients treated with the reduced doses of chemotherapy was 72% and 75%, respectively, and was not significantly different from the high-dose regimen. No patients developed congestive heart failure on this study. We conclude that adjuvant chemotherapy improves disease-free survival in patients with extremity soft-tissue sarcomas. The overall survival advantage in patients receiving adjuvant chemotherapy in our initial randomized high-dose chemotherapy trial has diminished though it continues to favor the chemotherapy group. A reduced-dose chemotherapy regimen was found to be comparable to the high-dose regimen.


2018 ◽  
Vol 127 ◽  
pp. S876
Author(s):  
A. Cortesi ◽  
A. Galuppi ◽  
A. Romeo ◽  
G. Ghigi ◽  
D.M. Donati ◽  
...  

2019 ◽  
Vol 37 (15_suppl) ◽  
pp. e22528-e22528
Author(s):  
Sara Albagoush ◽  
Jonathan Gootee ◽  
Kevin Nguyen ◽  
Sarah J Aurit ◽  
Christina Curtin ◽  
...  

e22528 Background: Among the types of soft tissue sarcomas (STS), liposarcoma is the most common malignant STS. Considering therapeutic options, surgical resection is the most utilized therapeutic option. In this study, we aim to explore the effects of varying degrees of surgical margins on survival in patients with dedifferentiated liposarcoma. Methods: The National Cancer Database (NCDB) was used to select patients with dedifferentiated liposarcoma to determine if surgical margins and other variables were associated with worse overall survival after accounting for age, gender, race, Charlson-Deyo score, anatomic site, treatment approach, tumor size, tumor grade, and presence of metastases through multivariable analysis. Results: Of the 1,004 patients, 64.4% were male, 87.0% were white, and the median age was 63 years. Approximately 95% had no metastases at the time of diagnosis, and 91.5% had high grade liposarcoma. For the status of surgical margins, 50.8% had no residual tumors, 26.1% had microscopic residual tumors, and 4.3% had macroscopic residual tumors. In general, the risk of death was higher for older males (25.8% increased risk of mortality) and those with metastases (312.9% increased risk of mortality) as well as patients with high grade liposarcoma (112.4% increased risk of mortality). When compared to no residual tumor after surgery, patients with macroscopic residual tumors, had a 96.7% increased risk of death (HR 95% CI:1.24 to 3.13; p= 0.004). Conclusions: Older age, presence of metastasis, male patients, retroperitoneal/abdomen primary site, high grade tumors, and macroscopic or residual tumor present after surgery led to an increased risk of mortality.


Rare Tumors ◽  
2016 ◽  
Vol 8 (4) ◽  
pp. 201-202 ◽  
Author(s):  
Christopher L. Brett ◽  
Daniel H. Miller ◽  
Liuyan Jiang ◽  
Herbert C. Wolfsen ◽  
Steven Attia ◽  
...  

Soft tissue sarcomas of the esophagus represent an extremely rare cause of esophageal masses, and an even smaller proportion of these tumors represent dedifferentiated liposarcomas. We present a case of a 75-year-old gentleman presenting with dysphagia found to have a 5 cm pedunculated mass in the cervical esophagus, originating at the cricopharyngeus. This was found to have involvement limited to the superficial mucosa by endoscopic ultrasound, and the lesion was subsequently resected endoscopically. Pathology demonstrated an undifferentiated pleomorphic sarcoma later determined to represent dedifferentiated liposarcoma after fluorescence in situ hybridization analysis. The patient received no additional adjuvant therapy and remains disease free 20 months from the procedure. While treatment experience is limited, our case demonstrates that in selected patients, sustained local control can be obtained without radical resection.


2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Ali Bourgi ◽  
Elias Ayoub ◽  
Sleiman Merhej

Purpose. In this retrospective case review we analyze the outcomes of patients treated for intradiverticular bladder tumors (IDT).Materials and Methods. A retrospective case review was done between January 2002 and May 2014 in Hotel-Dieu de France hospital. The series included 17 patients diagnosed with IDT, all males with a mean age of 49.8 years.Results. One patient was treated with tumor resection and adjuvant BCG instillation with no recurrence on follow-up cystoscopies and urine cytologies. 64% of patients were treated by diverticulectomy. Mean follow-up time was 38.7 months. At the end of the follow-up, 81% were disease-free. One patient had a radical cystectomy 6 months after diverticulectomy for recurrent high grade tumor; another one had a nodal metastasis 10 months after diverticulectomy and was managed with chemotherapy. 29% of patients were treated with radical cystectomy. Mean follow-up time was 28.4 months. No recurrence was documented on annual CT scans.Conclusions. Our data support a conservative approach for tumors confined to the bladder diverticulum, even in high grade or in the presence of CIS provided complete removal is feasible and close follow-up is ensured.


Blood ◽  
1991 ◽  
Vol 77 (7) ◽  
pp. 1593-1598
Author(s):  
PJ Carey ◽  
SJ Proctor ◽  
P Taylor ◽  
PJ Hamilton

We report the safety and efficacy of 34 consecutive autologous bone marrow transplant (ABMT) procedures performed in adult patients with high-grade lymphoid malignancy after remission induction therapy. Fifteen patients with acute lymphoblastic leukemia (ALL) and six with high-grade non-Hodgkin's lymphoma (NHL) received pretransplant conditioning with intravenous (IV) melphalan and fractionated total body irradiation (TBI). Thirteen other patients with NHL were conditioned with melphalan alone, having previously received local involved field radiotherapy. Unmanipulated noncryopreserved autologous marrow was reinfused within 48 hours of harvesting. Engraftment occurred in all patients with medians of 10 days of neutropenia (neutrophils less than 0.5 x 10(9)/L), 4-day platelet transfusion requirement, 3 U packed RBC transfusion, and 18 days in hospital posttransplant. There were no procedure-related deaths. Actuarial disease-free survival in the 13 patients with ALL receiving autotransplant early in first remission is 48% with a median follow-up of 3 years. Two other ALL patients who had autotransplants after a period of maintenance therapy also remain in complete remission (CR). These results compare favorably with our 34% disease-free survival (DFS) in 15 allogeneic ALL transplant patients and 21% DFS in 19 patients on standard maintenance after a common induction schedule. No relapses have occurred in the 17 NHL patients transplanted in remission (median follow-up 2 years), but the two NHL patients who developed recurrent disease before ABMT died of progressive disease after temporary responses. We conclude that this method of ABMT results in rapid reengraftment with lack of toxicity and that the conditioning treatment used shows good efficacy against disease. It is applicable in high-grade lymphoid malignancy in first remission, and our results call into question the need for marrow purging in ALL and NHL patients transplanted in first remission.


2019 ◽  
Vol 111 (3) ◽  
pp. 143-161
Author(s):  
Matías H. Loccisano ◽  
◽  
María F. Montesinos ◽  
Pedro A. Brégoli ◽  
Eugenia Paradeda ◽  
...  

Background: Soft-tissue sarcomas (STS) are rare mesenchymal tumors with several histologic subtypes and different clinical patterns. Objective: The aim of this study was to describe the clinical and pathological characteristics and surgical outcomes of a series of patients with STS. Material and methods: The clinical records of 2403 undergoing surgery between October 2014 and April 2018 were retrospectively reviewed. Twenty-two patients (0.91%) presented STS. Results: Mean age was 52 years (range: 19-92) and 13 (59%) were women. The tumors were located in the lower extremities in 12 cases, head and neck in five, trunk in three and upper extremities in two. Fourteen cases (63.6%) were high-grade tumors. Pleomorphic sarcoma was the most common histologic type (32%) followed by synovial sarcoma (18%), liposarcoma (14%), and other types (36%). All the tumors were completely resected and five patients (35.7%) required amputation, four in the lower extremity and on in the upper extremity. Different reconstructive procedures were performed accor- ding to tumor size and location, including three free flaps. Those patients with high-grade sarcomas or with positive margins received postoperative radiotherapy. After a mean follow-up of 16 months, six patients presented local recurrences and four patients had synchronous metastatic disease in the lungs; all these patients had high-grade tumors. Overall survival was 86.4%. Conclusion: STS are rare and invasive neoplasms, widely distributed, requiring aggressive and occa- sionally complex surgical procedures. It is necessary to consider adjuvant treatments in selected cases and to maintain regular follow-up due to the high rate of recurrences and distant metastases.


1988 ◽  
Vol 68 (6) ◽  
pp. 880-883 ◽  
Author(s):  
Chung W. Chow ◽  
Geoffrey L. Klug ◽  
Elizabeth A. Lewis

✓ The authors describe five cases of subependymal giant-cell astrocytoma in children in which many clinical, histological, immunohistochemical, and ultrastructural features typical of this tumor were present. However, prominent focal necrosis and mitoses, features usually associated with high-grade tumors, were seen in all cases. Despite the presence of necrosis and mitoses, clinical follow-up studies have revealed a lack of aggressive tumor behavior after surgery alone. The discrepancy between the histological and clinical features in these cases is emphasized so that excessive treatment of a basically low-grade tumor may be avoided. Mast cells were seen in all five cases, often in large numbers.


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