A case of retroperitoneal liposarcoma

A case of retroperitoneal liposarcoma is reported. A 42-year-old woman was admitted to our hospital with the complaint of a palpable mass in her left abdomen. Intravenous pyelography, barium enema and an ultrasonogram revealed a huge left abdominal tumor. At operation, a huge yellow-brownish tumor found in the retroperitoneal space, was completely removed. The tumor was 32 x 24 x 12 cm and weighed 4,075 g. Histologically, it was a myxoid liposarcoma. Three courses of adjuvant chemotherapy (cis-platin) were administered. The postoperative course was uneventful and the patient has been free of disease for 16 months.     

A case of metachronous, multifocal development of liposarcoma in the back of left thigh, the left subclavicular region and the right perirenal tissue

A 46-year-old woman had received surgery to remove a mass arising from the back of the left thigh 13 years before and on the left subclavicular region 3 years before. Histological diagnosis of both masses was myxoid type liposarcoma. She was admitted to our hospital because of right abdominal pain. Abdominal computed tomography (CT) and magnetic resonance imaging (MRI) revealed the presence of a large retroperitoneal tumor. The removed specimen weighed 1,450 g and measured 24 x 13 x 9 cm in size. Histological diagnosis was myxoid type liposarcoma. She has been free of any recurrence for 4 months postoperatively without adjuvant therapy.     

Laparoscopic Resection of a Retroperitoneal Liposarcoma: A Case Report and Review of the Literature

Here, we describe a case of a retroperitoneal liposarcoma successfully managed by laparoscopic surgery. A 72-year-old man underwent abnormal hypertension screening using computed tomography (CT), which revealed a low-density mass measuring 7 cm in diameter in the retroperitoneal space. We diagnosed the mass as a liposarcoma before the operation. The mass was resected laparoscopically. No perioperative complications were encountered, and the patient was discharged on the fourth postoperative day. The pathologic diagnosis was well-differentiated liposarcoma, indicating complete surgical resection. Thus, we conclude that a laparoscopic approach for the patients with retroperitoneal tumors, including liposarcomas, is safe, feasible, and minimally invasive, even if there is a possibility of the tumor being malignant.     

Multifocal liposarcoma showing rapid growth in a short term: a case report

We report a case of multifocal liposarcoma that showed rapid growth in a short term. A 65-year-old male was referred from a local doctor to our clinic with the chief complaint of abdominal fullness. Abdominal CT shows two huge tumors in the intraperitoneal and retroperitoneal space, and we diagnosed it as multifocal liposarcoma. Both tumors were resected. The surgical specimens of intraperitoneal and retroperitoneal masses were 20 x 10 cm and 12 x 8 cm in size, and 3.5 kg and 2.8 kg in weight, respectively. Histological diagnosis of both tumors was myxoid liposarcoma. However, the tumor recurred 4 months later, and two additional operations were required because of rapid growth. The patient died at 7 months after the first admission. The clinical outcome of myxoid liposarcoma is known to be better than other histological types, but it is very poor when the tumor appears multifocally.     

Retroperitoneal liposarcoma: a report of 2 cases and review of 163 cases in Japan

Two cases of retroperitoneal liposarcoma are reported. Case I was a 71-year-old-male presenting with a mass in the left abdomen. On X-ray examination, a large extrarenal tumor was found in the left retroperitoneal space. The tumor appeared to invade the psoas muscle and the aorta. Exploratory laparotomy was carried out. The histological diagnosis of the tumor was myxoid-type liposarcoma. He died of the disease. Case 2 was a 44-year-old-male who presented with a mass in the left abdomen. On X-ray examination, a large tumor was found in the left retroperitoneal space, and left kidney and the descending colon were markedly displaced by the tumor. Excisional surgery was carried out. The tumor was almost completely removed. The histological diagnosis was myxoid-type liposarcoma. A combination chemotherapy (actinomycin D, vincristine, cyclophosphamide) was started. but soon switched to radiation therapy because of liver dysfunction. Four months following the operation, there is no evidence of residual disease. One hundred sixty three cases of retroperitoneal liposarcoma reported in Japan are reviewed.     

Therapeutic experience with primary liposarcoma from the sigmoid mesocolon accompanied with well-differentiated liposarcomas in the pelvis

Multifocal liposarcoma with different histological types is rare, especially that originating from the mesentery. We herein report a case of primary myxoid liposarcoma from the sigmoid mesocolon that was accompanied with well-differentiated liposarcomas in the pelvis. The location, the fat signal and the relationship with adjacent organs of the liposarcomas were well shown in the axial, coronal and sagittal dimensions of MRI, giving a comprehensive and specific image before surgery. To alleviate the patient’s symptoms and mental stress, a laparotomy was performed. The tumors were all completely excised with macroscopic free margins. The final histopathological report showed that the tumor in the sigmoid mesocolon was a pure myxoid liposarcoma, while the pelvic tumors were spindle cell liposarcomas, a special type of well-differentiated liposarcoma. According to the AJCC staging system, they were all stage IIB. No further adjuvant therapy was performed. Close follow-up after the surgery has been performed, and the patient has remained healthy without any evidence of recurrence or metastasis for 17 months after the surgery.     

Surgical removal of retroperitoneal liposarcoma after transarterial embolization: a case report]

A case of retroperitoneal liposarcoma that was removed after transarterial embolization is reported. A 62-year-old man was admitted with body weight loss and general fatigue. Computed tomography revealed an extrarenal tumor, 27 x 17 x 11 cm in size, in the left retroperitoneal space. Arteriography revealed that the hypervascular tumor was fed from the left renal artery, the left adrenal artery and the left lumber arteries (L1-L4). At first the patient underwent transarterial embolization of the left renal artery and the left lumbar arteries (L1, L3, L4). Twenty-two days later he underwent surgical excision of the tumor with combined resection of the left kidney and the descending colon. The resected tissue weighed 2,500 g. Histological examination revealed liposarcoma, pleomorphic type. His postoperative course was uneventful, and he has remained free of disease for 15 months.     

Two cases of retroperitoneal liposarcoma arisen from perirenal fat tissue, which could not be diagnosed preoperatively

We report two cases of retroperitoneal liposarcoma arisen from the perirenal fat tissue, which could not be diagnosed preoperatively. Case 1 is a 58-year-old male. He complained of left flank tumor. Computed tomography and magnetic resonance image showed a mass over 10 cm that contained fat components in the retroperitoneal space. The tumor was resected with left nephrectomy and histological examination revealed well differentiated liposarcoma. As adjuvant therapy, he received chemotherapy and 30 months has passed uneventfully. Case 2 is a 70-year-old male. Screening ultrasonography revealed incidental retroperitoneal tumor. With clinical diagnosis as non-functioning adrenal tumor, he received left nephrectomy. The pathological diagnosis was well differentiated liposarcoma, sclerosing type. No adjuvant therapy was performed. He has stopped visiting our clinic due to aggravation of heart disease. The characteristics of the images of the two cases were different despite the histological resemblance. This difference was considered to be due to the difference in the distribution of lipomatous tissue in each patient.     

Giant recurrent retroperitoneal liposarcoma initially presenting as inguinal hernia: Review of literature

INTRODUCTIONLiposarcomas comprise around 15% of soft tissue tumors. These tumors of mesodermal origin arise as single tumors, present one histologic type and diverse locations (including the retroperitoneum). Diagnosis of liposarcomas of retroperitoneum is difficult because of this unspecific presentation and in 50–100% of the cases there is recurrence from residual tissue.PRESENTATION OF CASEAn 86 year old male patient was admitted in 1996 due to a right and voluminous inguinal hernia. During the herniaplasty, a right paratesticular tumor was isolated and removed. The histologic exam revealed a well-differentiated liposarcoma. A CT scan was performed and a large abdominal mass was detected. The patient underwent a laparotomy and an incomplete resection of the tumor was achieved. After the surgery the patient remained asymptomatic during a long period. Nine years later, the patient underwent another laparotomy with partial removal of the giant recurrent retroperitoneal liposarcoma.CONCLUSIONThe purpose of this publication is to report the recurrence of giant retroperitoneal liposarcoma, which is an unusual presentation in surgery today. Furthermore, we would like to emphasize the long-term survival of this patient despite partial resection and the possibility of performing a re-resection in this type of cases.     

Intussusception secondary to metastasis from a low-grade retroperitoneal liposarcoma

Liposarcomas are the most prevalent soft tissue sarcomas in adults. Low-grade liposarcomas are the most frequent and least aggressive and are noted as having a low risk of metastasis. We present a case of low-grade myxoid liposarcoma that metastasized to the small bowel resulting in intussusception. This case involves a 44-year-old woman with a recurrent retroperitoneal liposarcoma. Approximately 30 months after initial diagnosis, the patient was found to have a solitary metastasis to the small bowel that presented as a jejunal intussusception. Interestingly, there was no indication of a round cell component in either the metastasis or recurrent tumor, although the original tumor did contain a small round cell component. This is the first report in the English literature of liposarcoma metastasizing to the small bowel. We suggest that atypical presentations of liposarcoma metastases should always be considered in the management of patients with a history of liposarcoma.     

Intra-abdominal and retroperitoneal liposarcomas.

Nine primary intra-abdominal or retroperitoneal liposarcomas, of which eight were recurrent tumors, were surgically resected and enrolled in this study. Histopathological examination of primary tumors revealed that the number of well-differentiated, pleomorphic and myxoid type was two, four and three, respectively. In two recurrent cases, histological differentiation changed from well-differentiated type into myxoid or pleomorphic types. Prognoses of patients with large tumors (a 20 cm) were significantly poorer than for patients with small tumors (< 20 cm). Labelling index of Ki-67 of recurrent tumors increased in two cases. The tumor size affected prognoses of patients with intra-abdominal or retroperitoneal liposarcoma. Combined resection was important to get a tumor-free margin.     

Long-term results of aggressive surgical treatment of primary and recurrent retroperitoneal liposarcomas

The aim of this study was to establish the role of surgery in the treatment of retroperitoneal liposarcomas. Data concerning 28 patients submitted to surgery for retroperitoneal liposarcoma in our department over the period from 1972 to 1999 were reviewed retrospectively and analysed. Seventy-four operations were performed; in 54% of the operations it was necessary to resect contiguous organs (kidney 60%, colon 50%, adrenal gland 35%). In 89%, grossly curative resection was achieved at the first operation; 20 patients had at least one local recurrence after first operation (median time interval: 22 months). The mean follow-up was 80 months; median survival time was 51 months and 5-year actuarial survival time 51%. Patients with low-grade liposarcoma showed a statistically significant improvement (P < 0.001) in median survival (153 months) versus those with medium- (37 months) and high-grade sarcomas (8 months). At present surgery is still the treatment of choice in the treatment of primary and recurrent liposarcoma; in the case of low-grade liposarcomas especially, an aggressive surgical approach can result in long-term survival.     

Dedifferentiated liposarcoma of spermatic cord: degeneration of lipoma previously resected

Spermatic cord sarcomas are rare tumours. Dedifferentiated liposarcoma accounts for only 10% of all spermatic cord sarcomas. These are usually large-sized tumours histologically characterised for being well-differentiated liposarcomas with some high grade sarcoma areas. Volume, location, mass homogeneity as well as presence of pelvic and retroperitoneal adenopathies are reported by CT and ultrasound techniques. These are useful for post-treatment follow-up. This paper presents one spermatic cord, dedifferentiated liposarcoma from which lipomas from the same spermatic cord had been previously removed in three occasions. We believe this is a degeneration of the earlier resected lipoma. Management is by extended inguinal radical orchiectomy. Value of adjuvant radio- and chemotherapy is uncertain. Post-surgery local relapses are common, and haematogenous and pelvic nodes metastasis likely. Survival at 5 and 10 years is 75% and 63% respectively.     

Retroperitoneal malignant peripheral nerve sheath tumor (MPNST) complicated with von Recklinghausen's disease: a case report

A retroperitoneal malignant peripheral nerve sheath tumor (MPNST) in a patient with von Recklinghausen's disease is reported. A 55-year-old woman was admitted with a left side abdominal mass. Physical examination showed numerous cafe-au-lait-spots, subcutaneous masses, scoliosis, and a baby's head-sized fixed mass in the left abdomen. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a 9 x 9 cm retroperitoneal mass. Two other tumors were also found. One on the left side of the T2-T3 thoracic spine, and the other posterior to the right hip joint. The retroperitoneal tumor was resected en bloc. The tumor was a solid yellow mass. Macroscopically it has a pseudocapsule of fibrous tissue, weighed 1,120 g and measured 9 x 9 x 15 cm. The histopathological diagnosis was malignant peripheral nerve sheath tumor (MPNST). Since the responsiveness of these tumors to chemotherapy and radiation therapy is poor, we did not administer adjuvant therapy. The patient is alive with no evidence of recurrence more than 6 months after surgey.     

Retroperitoneal germ cell tumors with high level of serum and urine hCG in an adult: a case report

A case of retroperitoneal germ cell tumors in a 32-year-old male is reported. He was admitted to our hospital with complaints of lumbago and abdominal mass. He was diagnosed with primary retroperitoneal malignant tumor and underwent resection of the tumor with retroperitoneal lymphadenectomy. The final pathological diagnosis revealed germ cell tumors of more than one histological type in the retroperitoneal space. Serum human chorionic gonadotropin (hCG) and hCG-beta levels were very high preoperatively (4,8000 mIU/ml, 57 ng/ml, respectively) and they decreased to normal after vinblastine-bleomycin cisplatin combination chemotherapy three times postoperatively. The patient later developed a solitary lung metastasis and died in another hospital 7 months after the operation. Retroperitoneal germ cell tumors in adults are rare. One question continually arises: are they extragonadal occurrences or germ cell tumors or do they represent metastatic lesions from occult testicular tumors?     

Giant retroperitoneal liposarcoma--case report

Retroperitoneal liposarcomas are the most frequent soft tissue sarcomas and the second most frequent retroperitoneal tumours. They represent less then 0.1% of all human malignancies. Hereby we describe the treatment of our patient where of a 15-kg giant retroperitoneal liposarcoma was successfully removed and a local recurrence two years later was operated on as well. This was the 3rd largest retroperitoneal malignant tumor that was successfully removed according to the available literature of the last five decades. These typically symptom-free tumors usually grow extreme size before diagnosed. The "gold-standard" of treatment remains surgical total excision, but the high local recurrence rate (50-60%) hopefully can be reduced by adjuvant radio- and chemotherapy. Multidisciplinary treatment and long-time follow-up can provide as high as 40% 5-year survival rate. We summarize the recent clinical, diagnostic and therapeutic methods of this rare condition.     

c-myc、MDM2及p53基因在原发性腹膜后脂肪肉瘤中的表达及临床意义

目的:探讨原发性腹膜后脂肪肉瘤中c-myc、MDM2及p53基因蛋白表达及三者与肿瘤发生、发展的关系。方法:应用免疫组化PV-6000两步法对原发性腹膜后脂肪肉瘤及脂肪瘤组织中c-myc、MDM2及p53基因蛋白的表达进行检测,并进行统计学分析。结果:c-myc、MDM2及p53蛋白在原发性腹膜后脂肪肉瘤中表达阳性率分别为58.3%(28/48)、64.6%(31/48)、50.0%(24/48),三种蛋白在脂肪肉瘤与脂肪瘤组织中的阳性率差异有显著性(P0.05)。三种蛋白均与患者的年龄、性别及肿瘤大小无相关性。不同类型脂肪肉瘤分化较好者阳性率明显低于分化较差者。原发性腹膜后脂肪肉瘤中c-myc、MDM2及p53蛋白表达呈正相关(P0.05),c-myc、MDM2及p53蛋白表达在原发者与复发者之间的差异均无统计学意义(P0.05)。结论:c-myc、MDM2及p53蛋白与原发性腹膜后脂肪肉瘤的形成、分化及恶性程度有关,它们可作为判断原发性腹膜后脂肪肉瘤分化程度及恶性程度参考指标之一,但与肿瘤复发无关。在脂肪肉瘤的发生、发展中三者可能起协同作用。     

A case of a large retroperitoneal liposarcoma presenting as an incarcerated inguinal hernia

We report a rare case of retroperitoneal liposarcoma developing within an incarcerated inguinal hernia. A 53-year-old man presented to our hospital with left inguinal mass. Preoperative computed tomography revealed an intraabdominal huge mass, and a clinical diagnosis of liposarcoma. Physical findings except for the inguinal mass and laboratory examination, including tumor markers (CEA, CA19-9), were within normal range. Intraoperatively, the mass was incarcerated in the inguinal canal and involved the left testis. We performed a radical tumor resection, including a left orchiectomy. The resected specimen measured 45 × 30 cm and weighed 7,510 g. Histopathologically, the tumor was diagnosed as a well-differentiated liposarcoma, and originated from retroperitoneum. We did not undergo adjuvant therapy in consideration of histopathological subtype and curable surgical treatment. The patient is well and shows no evidence of recurrence four months after the operation.     

Three cases of retroperitoneal soft tissue sarcoma

Soft tissue sarcomas (STS) are rare tumors that comprise only 1% of all malignancies. The retroperitoneum is an uncommon site of origin for them, accounting for about 10% of all STS. In case 1, a 30-year-old man complaining of chest pain was found to have a retroperitoneal tumor around the left kidney by computed tomography (CT). The tumor was removed along with the left kidney. Histologically, it was fibrosarcoma 3,000 g in weight. In case 2, a 31-year-old woman complaining of abdominal distension was found to have a retroperitoneal tumor by CT. The tumor was removed in the same way. It was undifferentiated sarcoma 2,000 g in weight. In case 3, a 73-year-old woman, who had no complaint was found to have mass in the left lateral region during follow up for cardiac failure. She was referred to our department for treatment of retroperitoneal tumor revealed by ultrasound and CT. The tumor was dedifferentiated liposarcoma 2,420 g in weight. All three patients had local recurrences, and two of them underwent a second surgical procedure.