Well-differentiated Retroperitoneal Liposarcoma Research Articles

Huge retroperitoneal liposarcoma with renal involvement requires nephrectomy: A case report and literature review.

A 60-year-old female visited Guihang Guiyang Hospital (Guiyang, China). She presented with abdominal pain in the right side for the previous 2-months, with a touchable mass identified for the previous 1-month. Computed tomography with magnetic resonance imaging revealed a huge mass in the right abdomen. The diagnosis of well-differentiated retroperitoneal liposarcoma with renal involvement was made. During surgery, the tumor was removed, including the fatty renal capsule; however, the kidney was preserved. It is currently debatable whether resection of adjacent organs is required to obtain the negative margins. Conventional viewpoints advise that multi-organ resection is required in order to obtain the negative-margin. However, even if an R0 resection is achieved, the local recurrence rate remains markedly high. Additionally, the complications of organ resection have more impact on patients. Radiotherapy and chemotherapy are an important adjuvant method for these patients. In conclusion, retroperitoneal liposarcoma is a rare disease with a high rate of recurrence. Complete resection is the predominant treatment; however, combined resection of adjacent organs must be considered.

Dedifferentiated Retroperitoneal Liposarcoma Presenting as Right Inguinal Hernia: A Case Report

Liposarcomas are the most common primary soft-tissue malignancies of the retroperitoneum. They are divided into five subtypes: well-differentiated, dedifferentiated, myxoid, pleomorphic, and mixed, according to the 2002 World Health Organization histological classification (1). Well-differentiated retroperitoneal liposarcoma is the most common subtype and is considered a low-grade tumor. The dedifferentiated subtype is a transition from well-differentiated liposarcoma to nonlipogenic sarcoma with variable histological grades. Although several journals have reported retroperitoneal liposarcomas presenting as indirect inguinal hernias, dedifferentiated liposarcoma extending into inguinal canal and seemingly presenting as inguinal hernia has been more scarcely described (2, 3). Here we report multidetector computed tomography (CT) and magnetic resonance imaging (MRI) findings of a dedifferentiated retroperitoneal liposarcoma mimicking a right inguinal hernia.

Retroperitoneal sarcoma with femoral and inguinal herniation: A rare case report

Liposarcoma is a malignant tumorthat arises in fat cells in deep soft tissue, such as that inside the thigh or in the retroperitoneum. They are typically large bulky tumors which tend to have multiple smaller satellites extending beyond the main confines of the tumor.It accounts for approximately 20% of all mesenchymal malignancies.These sarcoma are remarkable because of their frequently large size. We report a case of a large well-differentiated retroperitoneal liposarcoma that weighted 9.5 kilograms with inguinal and femoral herniation of the same mass. The patient underwent surgery and is planned for adjuvant radiotherapy .

English

Liposarcoma is the most common tumor type of retroperitoneal soft tissue sarcomas. Liposarcoma is a malignant tumor of mesenchymal origin in which the bulk of tumor differentiates into adipose tissue. A 50 year old male patient was presented at our clinic with abdominal mass and pain. Abdominal ultrasonography, computed tomography (CT) and magnetic resonance imaging (MRI) showed a huge retroperitoneal mass. Hematologic, biochemistry and tumor markers were normal. Then, he was operated. He was reported as case well-differentiated retroperitoneal liposarcoma that weighted 3.000g.   Key words: Liposarcoma, retroperitoneal mass, sarcoma.

Lymphocyte Composition and Distribution in Inflammatory, Well-differentiated Retroperitoneal Liposarcoma

Lymphocyte Composition and Distribution in Inflammatory, Well-differentiated Retroperitoneal Liposarcoma

Surgical Experience with Retroperitoneal Liposarcoma in a Single Korean Tertiary Medical Center

PurposeThis is a report of the surgical treatment and prognosis of retroperitoneal liposarcoma (RPLS) in Koreans.Materials and MethodsNineteen patients treated for RPLS between July 1, 1984, and March 31, 2009, were included. Patient demographics, histopathologic subtypes, survival rate, disease recurrence rate and interval, and adjuvant therapy were reviewed and analyzed.ResultsOf the 19 patients diagnosed with RPLS, 26.3% presented with well-differentiated RPLS, 10.5% with dedifferentiated RPLS, 15.8% with myxoid/round cell type, and 47.4% with mixed-type liposarcoma. The mean follow-up period was 66.8 months (range, 6 to 165 months). Primary RPLS was treated in 17 patients. Nine patients (52.9%) had recurrent disease, and recurrence developed at a mean of 47.7 months after primary or repeated surgical treatment. The overall survival rate was 84.2% during a mean follow-up of 66.8 months. The 3- and 5-year survival rates were 86.9%, and the 10-year survival rate was 69.5%. The recurrence interval was significantly shorter in recurrent RPLS cases (p=0.023). The mean growth rate of locally recurrent tumors was 0.34 cm per month.ConclusionsThe survival rates reported here were higher than in previous studies. Locally recurrent tumors presented with a low growth rate, which may have contributed to the relatively high survival rate. A high prevalence of mixed-type RPLS was also noted, and its cause and prognosis require further research.

Gastrointestinal: A retroperitoneal liposarcoma that formed a fistula into the descending colon

Liposarcoma frequently occurs in the retroperitoneum and lower extremities, accounting for 9.8–16% of all soft tissue sarcomas. Liposaromas vary by histology and can be classified into four types. Those four types are well differentiated, myxoid/round cell, pleomorphic and dedifferentiated. This classification corresponds to the clinical aspect and prognosis of patients. Dedifferentiated liposarcoma (DDL) has both a well differentiated liposarcoma and a high grade nonlipogenic sarcoma within the tumor. It is difficult to diagnose DDL histologically. DDL can show a variety of histological appearances. The most common phenotype is malignant fibrous histiocytoma. Other phenotypes include leiomyosarcoma, osteosarcoma, rhabdomyosarcoma, and angiosarcoma. For DDL, prognosis is generally poor compared with the other types of liposarcoma. It shows high recurrence rate of 40-83%, metastasis rate of 15–30%, and the overall 5-year survival rate of 20%. DDLs often originate in the retroperitoneum, extremities, trunk, testis, and spermatic cord. A 61-year-old man was admitted to our hospital with 38.8°C of fever and general weakness. He had a history of a 20 × 10 cm well-differentiated retroperitoneal liposarcoma and underwent debulking surgery and intraoperative radiotherapy eight years previously. After surgery, there was no remnant mass visible on the abdominal computed tomography (CT) scan. On the current admission, physical examination revealed a palpable mass in the left mid abdomen. CT scan revealed a 8.5 × 8.3 cm sized large mass abutting the descending colon and left kidney in the left retroperitoneal cavity. The tumor encased a segment of the bowel loop and there was air density suspicious of tumor fistulization into the colonic lumen. Colonoscopy showed a fistula into the descending colon 30 cm from the anal verge (Figure 1). A yellowish mass was seen through the fistula with erythematous and edematous mucosal changes around the fistula. We suspected that the liposarcoma had recurred, and the patient underwent left colon segmental resection. The specimen showed a 9.5 × 8.5 cm sized, well-demarcated, yellowish-gray, lobulated, glistening, and firm mass. Microscopic findings showed a dedifferentiated liposarcoma containing a well-differentiated component with fat lobules and a nonlipogenic hypercellular area. There were scattered atypical lipocytes (Figure 2, upper panel) and pleomorphic spindle cells (Figure 2, lower panel, H&E, orig. mag. ×200) consistent with a subtype of malignant fibrous histiocytoma. Contributed by

Retroperitoneal Giant Liposarcoma

Retroperitoneal liposarcoma is an infrequent, locally aggressive malignancy. We report two cases of huge retroperitoneal liposarcomas. The presence of a palpable abdominal mass was a common symptom of the two patients. Preoperative imaging study showed huge retroperitoneal tumors. Both patients underwent complete surgical resections, and a negative microscopic margin was achieved in both cases. The histopathologic diagnosis was a well-differentiated retroperitoneal liposarcoma. Neither of the two patients developed a recurring tumor during the 1.5 years of follow-up.

Rare case of a giant well-differentiated retroperitoneal liposarcoma

Retroperitoneal liposarcoma is among the most common primary retroperitoneal tumors, along with malignant fibrous histiocytoma and leiomyosarcoma. It is slowgrowing and has the propensity to displace rather than invade adjacent structures. Histologic subtypes include well-differentiated, myxoid (the most common form), and pleomorphic. Liposarcoma is most often seen in the fifth to seventh decades and is more common in men. We report the case of a patient who presented with a well-differentiated giant retroperitoneal liposarcoma.

The prognostic impact of dedifferentiation in retroperitoneal liposarcoma

A series of patients with well differentiated (WD)/dedifferentiated (DD) retroperitoneal liposarcoma (RLS) was studied to evaluate the prognostic value of the presence, extension, and grade of the DD component. Among 148 patients with RLS who underwent surgery over 20 years, the authors retrieved data on patients who had localized WD/DD RLS. For the current analysis, patients were included only if they had primary disease or a first recurrence at their initial presentation. The DD component, when present, was graded according to National Federation of Centers in the Fight Against Cancer (FNCLCC) criteria, and the extension of the DD component was described as a percentage. Univariate and multivariate analyses were carried out for local recurrence-free survival (LRFS), event-free survival (EFS), and disease-specific survival (DSS). Of 93 patients who were identified, 36 patients (39%) had WD RLS, and 57 patients (61%) had DD RLS. The median follow-up was 71 months (range, 28-132 months). Seven patients (7.5%) developed distant metastases, including 5 patients who had DD RLS. The 5-year DSS rate was 42% in patients with DD RLS and 71.6% in patients with WD RLS (P = .018). The corresponding rates for LRFS were 22% and 43.3%, respectively (P = .007). The presence of the DD component and its FNCLCC grade were independent prognostic factors for DSS and LRFS. The administration of radiation therapy was associated independently with better LRFS. Patients with high-grade DD RLS had a worse prognosis in terms of both DSS and LRFS. The extension of the DD component and its mitotic index were relevant for EFS. The results indicated that radiation therapy may improve LRFS. These data may help stratify the risk of recurrence for patients with RLS. Clinical studies on new multimodality approaches are warranted.

Case Report: Laparoscopic Removal of 10-cm Retroperitoneal Liposarcoma

Liposarcomas are the second most frequent tumors in the retroperitoneal space and classically present as a large mass with minimal symptoms and a high local recurrence rate. The gold standard of treatment remains surgical excision. We describe the first successful removal of a well-differentiated retroperitoneal liposarcoma using a laparoscopic approach. An asymptomatic patient presented a large fatty mass in the retroperitoneal space on CT imaging. A transperitoneal laparoscopic approach was planned. A well-capsulated mass was exposed under Gerota's fascia that was dissected easily and removed completely without complications. Histologic examination revealed a well-differentiated liposarcoma. No recurrence was found at 12 months' followup. Resecting the mass completely remains the most important predictor of local recurrence and overall survival in liposarcoma. Thus, the success of radical surgical treatment is very important. The laparoscopic approach is technically safe and successful in maintaining oncologic principles and avoiding recurrences in retroperitoneal liposarcomas, representing a feasible alternative to open surgery.

骨形成を伴う後腹膜高分化型脂肪肉腫の１例

症例は68歳の男性で, 腹部膨満感を主訴に当科を受診した. 右側腹部に弾性硬の小児頭大の腫瘤を触知し, CTでは右腎下極に接した低吸収腫瘤を認め, 内部に粗大な石灰化を認めた.MRIではCTの石灰化した部位に一致し, 腫瘍の中心に, 低信号の結節を認めたが, 辺縁では, 脂肪成分と等しく, T1で低信号, T2で高信号を呈した. 右腎動脈造影と腰動脈造影検査で腫瘍濃染像を認めた. 10月中旬に開腹したところ, 右後腹膜に小児頭大の巨大腫瘍を認めた. 腫瘍は右腎下極と強固に癒着しており, 右腎周囲脂肪から発生したと判断し, 腫瘍と右腎をenblockに摘出した. 腫瘍径は24.5×20.5×11.5cmで重量は1,600gであった. 病理組織学的検査所見は著明な骨形成を伴う高分化型脂肪肉腫と診断された. 骨形成を伴った後腹膜高分化型脂肪肉腫は極めてまれであり, 自験例を含め6 例のみであった. 本症例は病理組織学的にも興味深く, 若干の文献的考察を加えて報告する.

Inflammatory variant of a well-differentiated retroperitoneal liposarcoma: Case report of a rare giant variety

Inflammatory liposarcoma is a rare variant of a well-differentiated liposarcoma (WDLPS). We present a case of a 37 years old male who had a giant variety of this inflammatory WDLPS. CT scan revealed a large abdomino-pelvic mass abutting the left kidney and pushing the IVC, Aorta and the left ureter across the midline. CT guided FNAC did not reveal any malignant cells. A large 9-kg fibro-fatty mass, which appeared irregular, congested and bosillated was excised. Microscopic picture revealed foci of fibrosis with mature adipose tissue. Lymphocyte and plasma cell infiltrate was abundant along with multi-nucleate giant cells and few lipoblasts. There are no case reports of a giant inflammatory variant of WD-LPS in world literature and this is the first of its kind from the Indian sub-continent. We present a case report of this rare giant variant of inflammatory WDLPS and discuss the review of literature.

CT Findings of Pelvic Lipomatosis of Nerve

Torigian DA and Siegelman ES(AQ1) ipomatosis of nerve, also known as fibrolipomatous hamartoma or fibrofatty proliferation of nerve, is a rare benign tumorlike lesion that most often affects the median nerve or its branches. Rarely, involvement of the nerves of the upper and lower extremities and of the face have been reported [1–7]. Frequently, no symptoms or signs are present, although over 50% of patients are symptomatic because of mass sensation, focal compressive neuropathy, or associated macrodactyly [1, 2, 7–9]. We report the CT findings of a woman with presumed asymptomatic pelvic lipomatosis of nerve involving the right sciatic nerve and lumbosacral plexus, as this lesion may potentially be mistaken for the more common retroperitoneal well-differentiated liposarcoma.

Retroperitoneal Liposarcoma and Li-Fraumeni Syndrome: A Case Report and Review of Management Options

A 44 year-old female, known to have Li-Fraumeni syndrome, was discovered to have a retroperitoneal mass on surveillance ultrasound. Computerized tomography revealed a 2.0 cm non-cystic, enhancing soft tissue mass abutting the postero-superior margin of the right kidney. She underwent radical nephrectomy, adrenalectomy and en bloc resection of the tumor. Pathology showed a grade I, well-differentiated retroperitoneal liposarcoma with negative surgical margins. She recovered well from surgery and continues to remain recurrence free at 12 months based on serial CT imaging.

Primary retroperitoneal liposarcoma

A retroperitoneal liposarcoma that marginally involved the right kidney and ovary was presented clinically as an adnexial mass in a 61-year-old woman. At laparatomy a large retroperitoneal mass in addition to the right kidney and ovary was removed totally. A well-differentiated retroperitoneal liposarcoma was detected in histological speciment. Adjuvant irradiation was given. The 5-year survival rate of retroperitoneal liposarcoma is low. Curative resection remains the main treatment for primary and recurrent liposarcomas.

A CASE OF RETROPERITONEAL LIPOSARCOMA

Liposarcoma occurring in the retroperitoneum is relatively rare and is often found as a giant mass. In this paper, we reported a case of well-differentiated retroperitoneal liposarcoma. A 43-year-old man was admitted to the hospital because of feeling of abdominal fullness and lumbago. An infant-head-sized tumor was palpable in the left upper abdomen. Scout film of the abdomen revealed a mass shadow in the left upper abdomen. In an abdominal ultrasonic examination, the tumor mass showed an irregular pattern. Abdominal CT scan visualized a low density area with a clear boundary, and with enhancing, a low density ara filled with heterogenous reticular shadow was observed. From the above findings, a diagnosis of well-differentiated retroperitoneal liposarcoma was entertained. Celiotomy was performed with confirmation of the preoperative diagnosis by microscopic examinations. Postoperative course was satisfactory and he was discharged on the 12th postoperative day. Excision of a retroperitoneal liposar-coma is relatively easy due to poor blood supply and its prognosis is favorable in well-differentiated type. For a preoperative diagnosis, abdminal CT scan is useful.