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Liposarcoma is the most commonly diagnosed soft tissue sarcoma in adults and occurs predominantly in the lower limbs and retroperitoneum. Primary mediastinal liposarcomas are rare.They are often asymptomatic and when growing to large size the presenting symptoms are related to direct invasion or compression of other thoracic organs such as the hear...
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Lipomas, one of the commonest soft tissue tumours encountered in surgical OPD’s and liposarcomas are malignant tumours which may mimic the former. Here we present a case report of a 55 years old female who presented with a slow and progressively growing tumour over her right upper limb which was compressible and provided no pain or discomfort mimic...
Introduction:
Primary mediastinal dedifferentiated liposarcoma is an extremely rare malignant mesenchymal neoplasm composed of lipogenic tissue. It can be huge enough to compress heart and surrounding organs leading to clinical symptoms.
Presentation of case:
We present a case of huge primary mediastinal dedifferentiated liposarcoma in a 54-year...
Background
Cardiac Lipoma is a rare entity constituting approximately 10-19 % of primary tumors of the heart and pericardium. To our best knowledge, such a large cardiac lipoma with septal enhancement in our case has never been reported before.
Case presentation
Here we present a rare case of a 65-year-old symptomatic female with an unusual giant...
Citations
... These masses usually have an insidious growth course and only become symptomatic once they exert a mass effect on local structures [3]. They are often diagnosed incidentally as part of a workup of cardiopulmonary symptoms [5]. Ultimate management is dependent on specific tumor characteristics, subtype classification, and the extent to which surrounding structures are involved [6]. ...
... Since there is limited data available on these tumors, there is no set standard of care, and cases should be evaluated on a multidisciplinary platform. While some studies involving mediastinal masses suggest surgical resection as the best treatment modality [5], others have evaluated pre-and postoperative radiotherapy and chemotherapy; results have failed to show efficacy [7]. Of note, most evidence-based recommendations regarding radiotherapy use have been principally grounded on prospective trials involving extremity soft tissue sarcomas, which generally respond well to function-preserving surgery combined with radiotherapy [8]. ...
... Furthermore, the tumor was anatomically favorable for resection despite its involvement of major structures. In circumstances of extensive surrounding tissue involvement where complete resection may not be technically feasible, en bloc debulking has been suggested as the best option [5]. Therefore, the notion that surgical resection alone may be sufficient is supported. ...
A 64-year-old patient presented with shortness of breath and chest pressure. The initial examination was unremarkable, and a chest X-ray revealed a large mediastinal mass. Computed tomography (CT) scan demonstrated a lobulated mediastinal mass involving the great vessels and mass effect on the trachea, esophagus, and heart. A CT-guided biopsy showed a monotonous, evenly spaced population of mature, normal-appearing adipocytes consistent with a well-differentiated lipoma-like liposarcoma/atypical lipomatous tumor. The patient underwent a median sternotomy with en bloc tumor resection without adjuvant chemoradiation. Three-year follow-up CT imaging shows no evidence of tumor recurrence.
... Although ALT and WDL are morphologically and genetically identical, the term WDL is used for tumors of the retroperitoneum, mediastinum and deep pelvis (in which the chance of reaching negative margins is reduced and local recurrence is increased), while the term ALT includes tumors of the extremities and superficial regions (in which complete excision is possible and curative) (1). ALT/WDL occurs most frequently in the 5th and 7th decades, and predominantly in adult males (6,7). Although surgical excision is the optimal treatment option for mediastinal liposarcomas, postoperative recurrence rates range from 50-90% (8). ...
... The laxity and mobility of tissues and the mediastinal state of these structures allow them to gradually adapt and accommodate slow-growing tumors. Tumors can thus reach large sizes, and subsequently, to press on neighboring structures causing such symptoms as superior vena cava syndrome, Horner's syndrome, dysphagia, dyspnea, cough, spinal nerve palsy and tachycardia (3,7). In our case, the patient presented to us with acute cough. ...
... Histological typing is very important in determining the type and extent of treatment. Due to differences in the treatment approaches, prognosis and long-term followup, it is important to preoperatively differentiate between simple lipoma and well-differentiated liposarcoma (4) as the survival of patients with differentiated or pleomorphic liposarcoma is significantly shorter than those with myxoid or well-differentiated liposarcoma (7). The most important factor in prognosis is anatomical location. ...
... Soft tissue sarcomas are rare tumors, accounting for less than 1% of all malignancies, while in adults liposarcoma is the most common among them (1). This type of tumor can be found anywhere in the body, but the most common localization is on the extremities and retroperitoneum (2). More common it occurs in men (1.43:1), most often in the population aged between 40 and 70 years (3,4). ...
Retroperitoneal liposarcoma is a rare type of tumor characterized by slow growth and nonspecific symptoms, and is usually diagnosed at an advanced stage. Patients with huge retroperitoneal liposarcoma have a high risk of developing perioperative complications, and require special preoperative preparation and a carefully planned anesthetic approach. We present the case of a 57-year-old man, who was diagnosed with a huge retroperitoneal liposarcoma, 70 cm in diameter, weighing 30.4 kg and planned for surgical resection of the tumor under general anesthesia. Perioperative treatment and anesthesia for this patient were a great challenge for the anesthesiologist. However, due to preoperative preparation, monitoring and fluid replacement, hemodynamic and respiratory stability of the patient was maintained perioperatively.
... The well-differentiated LS is a well-circumscribed mesenchymal tumor that arises from adipose tissue [2]. Primary mediastinal LS are extremely rare, accounting for less than 1% of mediastinal tumors and 2% of LS [3] [4]; thus, only a small number of cases have been reported [1]. ...
... LS compromises approximately 1% of all malignancies [4], and it is the most common soft tissue sarcoma, accounting for 20% -30% of soft tissue sarcomas in adults [7]. Mediastinal liposarcoma is a very rare primary malignant tumor; it accounts for only 0.1% to 0.75% of all mediastinal tumors [2] [4]. ...
... LS compromises approximately 1% of all malignancies [4], and it is the most common soft tissue sarcoma, accounting for 20% -30% of soft tissue sarcomas in adults [7]. Mediastinal liposarcoma is a very rare primary malignant tumor; it accounts for only 0.1% to 0.75% of all mediastinal tumors [2] [4]. This tumor's behavior in the mediastinum resembles to its behavior in the lower extremities and the retro-peritoneum, the most common sites, respectively [4] [7]. ...
... This research focuses on the under-studied rare cancer liposarcoma. Liposarcoma is a malignant mesenchymal tumor [7], accounting for almost 20% percent of adult mesenchymal tumors [8]. It can be found anywhere in the body, but most commonly in the extremities and retroperitoneum [8]. ...
... Liposarcoma is a malignant mesenchymal tumor [7], accounting for almost 20% percent of adult mesenchymal tumors [8]. It can be found anywhere in the body, but most commonly in the extremities and retroperitoneum [8]. ...
Rare cancers, affecting 1 in 5 cancer patients, disproportionally contribute to cancer mortality. This research focuses on liposarcoma, an understudied rare cancer with unknown risk factors and limited treatment options. Liposarcoma incident cases were identified from the U.S. Surveillance, Epidemiology, and End Result (SEER) program and the combined SEER-National Program of Cancer Registries (CNPCR) between 2001–2016. Incidence rates (age-adjusted and age-specific), 5-year survival, and the time trends were determined using SEER*stat software. Three-dimensional visualization of age–time curves was conducted for males and females. SEER liposarcoma cases represented ~30% (n = 11,162) of the nationwide pool (N = 37,499). Both sources of data showed males accounting for ~60% of the cases; 82%–86% cases were identified among whites. Age-adjusted incidence was greater among males vs. females and whites vs. blacks, whereas survival did not differ by sex and race. The dedifferentiated (57.2%), pleomorphic (64.1%), and retroperitoneal (63.9%) tumors had the worse survival. Nationwide, liposarcoma rates increased by 19%, with the annual percent increase (APC) of 1.43% (95% confidence interval (CI): 1.12–1.74). The APC was greater for males vs. females (1.67% vs. 0.89%) and retroperitoneal vs. extremity tumors (1.94% vs. 0.58%). Thus, incidence increased faster in the high-risk subgroup (males), and for retroperitoneal tumors, the low-survival subtype. The SEER generally over-estimated the rates and time trends compared to nationwide data but under-estimated time trends for retroperitoneal tumors. The time trends suggest an interaction between genetic and non-genetic modifiable risk factors may play a role in the etiology of this malignancy. Differences between SEER and CNCPR findings emphasize the need for nationwide cancer surveillance.
... At CT, thymolipomas appear fatty tissue mixed with soft tissue attenuation that represent thymic tissue with sharp border, well-defined capsule and no invasion of surrounding structures (1,2). CT findings have an important role in the diagnosis of thymolipomas (1)(2)(3). The thymolipoma in CT seems a large mixed fat and soft tissue density mass adjacent to the mediastinum and conform to the shape of adjacent mediastinal structures and it is connected to thymus ( Fig. 1, Fig. 2). ...
... Mediastinal liposarcoma may infiltrate the heart, early metastasis to the lungs and frequent local recurrence after surgical resection. The presenting symptoms are related to size and direct invasion of pericardium or superior vena cava (3)(4)(5)(6). Dyspnea, vague chest discomfort, cough, or constitutional symptoms can be seen. On conventional chest radiography widened mediastinum and deviation of the trachea and vessels may be apparent. ...
... Mediastinal liposarcoma (ML) is an extremely rare entity, accounting for about 1% of all mediastinal tumors, predominantly in posterior part, and less than 2% of all liposarcomas [1]. Dedifferentiated liposarcoma is the least common subtype of liposarcoma, particularly found in male over the fourth decades [2] and appear to be challenging to diagnose. ...
... Most patients present with dyspnea, chest discomfort or cough. Patients may also present with superior vena cava syndrome [2] Most similar reported cases were successfully treated by complete surgical excision alone [1,2], but in this case, after removal of the giant tumor, it recurred 6 months later and leading for a need of chemotherapy. * Correspondence author. ...
... Most patients present with dyspnea, chest discomfort or cough. Patients may also present with superior vena cava syndrome [2] Most similar reported cases were successfully treated by complete surgical excision alone [1,2], but in this case, after removal of the giant tumor, it recurred 6 months later and leading for a need of chemotherapy. * Correspondence author. ...
Introduction:
Primary mediastinal dedifferentiated liposarcoma is an extremely rare malignant mesenchymal neoplasm composed of lipogenic tissue. It can be huge enough to compress heart and surrounding organs leading to clinical symptoms.
Presentation of case:
We present a case of huge primary mediastinal dedifferentiated liposarcoma in a 54-year-old man, confirmed by immunohistochemistry staining, who received surgical removal. However, six months later, the tumor recurred, and the patient underwent chemotherapy for 6 cycles resulting in stable disease. Six months after the last visit, the tumor showed no sign of recurrence anymore.
Discussion:
Among the various subtypes, the dedifferentiated mediastinal liposarcoma is the least found type of liposarcoma and often leads to misdiagnosis. Challenges occurred not only in diagnosis but also in treatment since it frequently grows back and chemotherapy may be needed after surgery.
Conclusion:
This is a highly rare case of huge mediastinal liposarcoma that recurred after surgery and gave a satisfying outcome after chemotherapy.
... Less differentiated tumours are more aggressive and associated with a poor prognosis. 5 A solid calcified de-differentiated liposarcoma located within the anterior-mid mediastinum has not been described before. Complete absence of macroscopic fat obstructed diagnostic differentiation. ...
... Common locations include deep soft tissue of the lower limbs and retroperitoneum. [1] Primary anterior mediastinal liposarcoma is an extremely rare clinical entity, constituting <1% of all reported mediastinal tumors. [2] The presenting signs and symptoms are related to tumor size and the direct invasion of contiguous structures. ...
Liposarcomas are extremely rare in the mediastinum. Patients usually present late due to the compressive effect of the tumor on the adjacent structures. Severity of the symptoms depend mainly on the size of the tumor and the structure it infiltrates. Well differentiated slow growing liposarcomas are the most common ones in the mediastinum followed by dedifferentiated and poorly differentiated ones. These tumors have bad prognosis because of incomplete surgical excision due to its inaccessible location. Hence these patients should be kept under close follow up because of high recurrent rates. Here we are presenting a rare case of anterior mediastinal sclerosing liposarcoma in a 77 year old male.
... Primary mediastinal liposarcoma is an uncommon neoplasm of intrathoracic origin [Barbetakis 2007]. In 1951 Kozonis et al published that in the English language only four cases of liposarcomas originating in the mediastinum had been described [Kozonis 1951]. ...
... The predominant finding of mediastinal liposarcoma on conventional chest radiography is usually a widened mediastinum. On CT, the appearance of mediastinal liposarcomas varies from a predominantly fat-containing mass to a solid mass [Barbetakis 2007;Eisenstat 2000]. ...
Liposarcoma is the name given to a group of soft tissue sarcomas (STSs) with adipocytic differentiation. As a group, liposarcomas are the second most common STSs in adults. In 1951 Kozonis et al published that in the English language only four cases of liposarcomas originating in the mediastinum had been described. Primary mediastinal liposarcoma is an uncommon neoplasm of intrathoracic origin. We present the case of a 47-year-old woman diagnosed with a large mediastinal mass with intrapericardial invasion and massive pericardial effusion; biopsies showed a mediastinal liposarcoma.
