scholarly journals Primary Pericardial Synovial Sarcoma: A Case Report and Literature Review

Diagnostics ◽  
2022 ◽  
Vol 12 (1) ◽  
pp. 158
Author(s):  
Simona Manole ◽  
Roxana Pintican ◽  
Emanuel Palade ◽  
Maria Magdalena Duma ◽  
Alexandra Dadarlat-Pop ◽  
...  

We report a case of a 52-year-old woman who was referred to our institution with a superior vena cava syndrome and was investigated through echocardiography, CT and MRI revealing a well-defined, encapsulated pericardial mass. The pathology, correlated with the immunohistochemical analysis, concluded it was an extremely rare primary pericardial synovial sarcoma. The patient underwent surgery and chemotherapy with a 16-month disease-free survival and passed away after a contralateral aggressive relapse. Moreover, we discuss the role of each imaging modality together with their pericardial synovial sarcoma reported features.

2016 ◽  
Vol 119 ◽  
pp. S667
Author(s):  
M.S. Mariaquila Santoro ◽  
M.L. Marianna Lacaria ◽  
M.A.M. Maria Angela Molinaro ◽  
L.R.F. Lucia Rachele Fabiano ◽  
A.D. Anna Destito ◽  
...  

1984 ◽  
Vol 2 (4) ◽  
pp. 260-266 ◽  
Author(s):  
R Perez-Soler ◽  
P McLaughlin ◽  
W S Velasquez ◽  
F B Hagemeister ◽  
J Zornoza ◽  
...  

Thirty-six of 915 patients with non-Hodgkin's lymphoma presented with superior vena cava syndrome (SVCS). The histologic types associated with SVCS were diffuse large cell in 23 patients, lymphoblastic in 12, and follicular large cell in one patient. Radiotherapy alone appeared equal to chemotherapy alone or in combination with radiotherapy in achieving relief of SVCS symptoms. Chemotherapy alone or in combination with radiotherapy was superior to radiotherapy alone in prolonging relapse-free survival and overall survival. No differences in relapse-free survival and survival were found between the patients treated with chemotherapy alone and those treated with chemotherapy and radiotherapy, but the addition of radiotherapy appeared to prevent local relapses in the group with large-cell lymphoma. The presence of symptoms of involvement of other mediastinal structures such as dysphagia, hoarseness, or stridor (DHS), a higher grade of intensity, and a shorter duration of symptoms (less than or equal to 2 weeks) appeared to adversely influence relapse-free survival and survival. The following conclusions were made: (1) a histologic diagnosis before the onset of treatment is desirable and feasible in patients presenting with SVCS except in those with severe respiratory distress, (2) both chemotherapy and radiotherapy are equally effective in alleviating the symptoms of SVCS, and (3) combined modality treatment with chemotherapy and radiotherapy results in a lower frequency of local relapses compared to chemotherapy alone but survival was similar in both groups.


Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 4697-4697 ◽  
Author(s):  
Maurizio Martelli ◽  
Vittorio Stefoni ◽  
Giuseppina Cabras ◽  
Sergio Cortellazzo ◽  
Ercole Brusamolino ◽  
...  

Abstract Background: Third generation regimens such as MACOP-B or VACOP-B (M/VACOP-B) in combination with involved-field radiotherapy (IFRT) seem to improve lymphoma-free survival of PMLBCL. The superiority of R-CHOP over CHOP-like regimens has been recently demonstrated in younger low risk DLBCL. Recently, the addition of Rituximab to CHOP has also improved survival in PMBLCL. Aims: To evaluate the effectiveness and safety of Rituximab added to the standard M/VACOP-B regimens (R-M/VACOP-B) +/− IFRT in PMLBCL. Patients and Methods: A total of 40 patients with PMLBCL has so far been treated in the six participating centers between February 2002 and July 2005. The median age was 38 years (range 17–54); 21/19 (53%) were females; 30 patients had stage II and 10 stage IV; 38 (95%) presented a bulky disease; LDH was increased in 26 (65%) and 21(53%) had a superior vena cava syndrome. According to the age-adjusted IPI score, 24 patients had an IPI = 0–1 and 16 an IPI = 2–3. All patients were treated with standard MACOP-B (30 patients) or VACOP-B (10 patients) regimens plus six cycles of Rituximab (375mg/m2) given at weeks 3, 5, 7, 9, 11 and 13. Twenty-six patients (65%) received mediastinal IFRT at a median dose of 36 Gy. The response was evaluated in all patients after six cycles of chemo-immunotherapy, at the end of the planned chemotherapy and after IFRT. Results: The response rate after six cycles of the scheduled R-M/VACOP-B regimen was CR/CRu = 20 (50%), PR = 19 (47%) and NR = 1 (3%). Eight of the 40 patients received a second line therapy followed by HDT-ASCT (6/8 patients) because considered as low responders (PR = 7 and NR = 1). At the end of the chemo-immunotherapy program, 28 patients witnessed a CR/CRu (70%) and 12 a PR (30%). Seven of the 12 PR patients obtained a CR/CRu following IFRT for an overall CR/CRu rate of 87% (35/40). After a median follow-up of 13 months, the 2-year OS and PFS are 75% and 78%, respectively. No additional toxicities other than those related to chemotherapy were observed during and after Rituximab infusion. Conclusions: R-M/VACOP-B are active therapeutic regimens devoid of severe toxicity for the management of patients with PMLBCL. The addition of Rituximab to M/VACOP-B regimens does not seem to improve the lymphoma-free survival of this group of patients. An update of these results on a broader number of patients will be presented at the meeting.


2001 ◽  
Vol 7 (2) ◽  
pp. 47-53 ◽  
Author(s):  
F. H. W. Hermens ◽  
F. J. Visser ◽  
A. Termeer ◽  
W. B. Barendregt ◽  
J. P. Janssen

Question of the Study In this study, safety and feasibility of thoracoscopic fenestration of pleuropericardial cysts under local and general anaesthesia is evaluated. Besides, a rare case of a pleural cyst, causing a superior vena cava syndrome, is described.Materials, Patients and Methods In a retrospective study, the results of thoracoscopic treatment of pleuropericardial cysts in three patients are presented. We performed videothoracoscopic fenestration of pleuropericardial cysts. One of these was performed under local anaesthesia. The two other cases were performed under general anaesthesia. After fenestration, talc poudrage of the inner lining of the cysts was performed in one case.Results Thoracoscopic fenestration appeared to be safe and effective. No recurrence was observed. One patient was lost to follow-up.Answer to the Question Thoracoscopic fenestration of pleuropericardial cysts is safe and effective. This procedure can be performed under local anaesthesia in selected cases. The role of talc poudrage of the cysts is unclear and needs further investigation.


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