1/99. liver metastases from extremity soft tissue sarcoma. liver involvement by soft tissue sarcoma is an unfavorable prognostic factor for survival. Complete resection of liver metastases can bring improvement in selected patients, but chemotherapy remains the only palliative treatment option for most. Anecdotal long-term survival of patients with unresectable liver metastases treated with systemic chemotherapy has been reported, such as the patient presented here. ( info) |
2/99. Radiological findings in myxoid liposarcoma of the anterior mediastinum. CT and MR findings of a rare myxoid liposarcoma involving the anterior mediastinum are reported. The mass was a low density lesion with calcific septations and some peripheral frond-like enhancement on CT. MRI showed heterogeneous intermediate to high signal intensity on T1 weighted images and high signal intensity on T2 weighted images. The signal of the mass was not suppressed on fat suppressed images. ( info) |
3/99. A rare chimeric TLS/FUS-CHOP transcript in a patient with multiple liposarcomas: a case report. Myxoid liposarcomas harbor a unique and specific t(12;16)(q13,p11) chromosomal translocation. The breakpoint has recently been identified, and involvement of the TLS/FUS gene on chromosome 16 and the CHOP gene on chromosome 12 was demonstrated. We report a case of a 45-year-old woman who developed multiple malignant lipomatous tumors of unknown origin and myxoid/round cell histology at different locations. To examine the diagnostic potential of this translocation and to develop a hypothesis on the origin of the tumors, we used cytogenetic and molecular cytogenetic methods (reverse transcription polymerase chain reaction, RT-PCR). We identified a chimeric rna transcript in the second recurrence in the thigh/groin, as well as in another tumor in the mediastinum, which has an additional sequence of 33 bp, known as fusion transcript type III. cytogenetic analysis of another tumor in retroperitoneal space revealed a rare type of unbalanced translocation der(16)t(12;16). We hypothesize that these tumors are metastases rather than multicentric tumors. The detection of the chimeric message in the present case is not only useful for differential diagnosis, but also for analyzing the origin of multiple neoplasms. ( info) |
4/99. A parapharyngeal myxoid liposarcoma. We present a case of a parapharyngeal space myxoid liposarcoma. This case highlights the importance of wide surgical resection margins, and the difficult histological diagnosis. ( info) |
5/99. Benign recurring lipoblastoma in an adult versus well differentiated subcutaneous myxoid liposarcoma: clinicopathologic, immunohistochemical and molecular analysis of a unique case. Subcutaneous myxoid liposarcoma (ML) is exceedingly rare, with only two or three cases having been reported. lipoblastoma (LB), a rare fatty tumor that arises in infants and children, is unknown after adolescence. In contrast to ML, LB is often superficial. The two tumors can be difficult to distinguish due to many histologic similarities. We examined a 0.9 cm superficial subcutaneous nodule from the dorsal neck of a 48 year old man that had been growing slowly. Three and one half years later, a 0.4 cm palpable recurrent nodule was excised from the scar. The patient is now free of disease at 7.5 years. Because of these unusual features, we performed clinicopathologic, immunohistochemical and molecular analysis of this unusual tumor to decide if this represented a rare cutaneous ML or an unprecedented example of LB in an adult. The primary featured a thick fibrous pseudocapsule with foci of lymphocytes and infiltrating nests of semi-mature fetal-appearing adipocytic tissue. This surrounded a more immature cellular-but-cytologically-bland myxoid tissue featuring stellate cells and signet lipoblasts. There were fibrous sep at the periphery and the vasculature was rather inconspicuous. The 0.4 cm diameter recurrence was distinctly lobular and had minute satellite nodules. It was composed of uniform fetal-appearing bland myxoid lipoblastic tissue featuring signet ring lipoblasts surrounded by a few spindle cells. In both tumors, lipoblasts expressed S-100 protein. In the primary, 5% of the lesional cells were FXIIIa dendritic stromal histiocytes while in the recurrence, 15% of the lesional cells were FXIIIa dendritic cells. CD34 stained only scattered small capillaries. The Ki67 proliferation index was 1% in the primary and 3% in the recurrence. RT-PCR assay for TLS/FUS-CHOP fusion transcripts was negative despite three repeat tests performed on paraffin sections of the primary tumor in the presence of good m-rna internal controls. We reviewed the clinicopathologic and cytogenetic features of ML and LB. Based on this review and on the growth pattern, anatomic features and molecular data from the present case, we conclude that this tumor may represent the first reported case of adult LB. ( info) |
| A 74-year-old woman presented with an acute left hemiplegia. CT demonstrated an irregular mass in the right parietal region of the brain. Two years previously, she had undergone wide local excision of a liposarcoma from her thigh. craniotomy was performed and the lesion removed. Subsequent histology confirmed a liposarcoma. The brain is an exceptionally rare site of first recurrence of a liposarcoma. This case report presents a pathological rarity and briefly reviews the literature relevant to clinicians. ( info) |
7/99. Myxoid liposarcoma with transition to round-cell lesion-cell cycle regulator genes and telomerase activity characterizing tumor progression: a case report. A mixed myxoid/round cell liposarcoma was macrodissected in its 2 histologic components and investigated for genetic differences between its low-grade myxoid and the high-grade round-cell region. For both, we failed to detect p53 gene mutations, loss of heterozygosity at the p53 or Rb genes, and p53 protein expression. The round-cell component showed a high telomerase activity, and an elevated c-myc mRNA and protein expression. The myxoid component was characterized by a lack of telomerase activity and low c-myc mRNA expression, and immunohistochemistry failed to detect the c-myc protein. There was a higher Mib-1 proliferation index in the round-cell portion. The same specific translocation t(12;16) and the fusion transcript type II in both components confirmed the close relationship between myxoid and round-cell liposarcomas. telomerase activity and increased c-myc expression seem to be helpful molecular markers for characterizing tumor progression in myxoid liposarcoma. ( info) |
8/99. Gynaecological presentation of retroperitoneal tumours. OBJECTIVE: To illustrate the problems associated with mistaken pre-operative diagnosis following gynaecological presentation of patients with retroperitoneal tumours. DESIGN: A case series of five referrals. RESULTS: Non-gynaecological tumours were not suspected in each case and hence there was a failure to undertake further pre-operative investigation and referral to a specialised soft tissue sarcoma service. This resulted in four of the patients having an unnecessary laparotomy with an inappropriate transperitoneal biopsy undertaken when the retroperitoneal tumour was discovered. The mistaken diagnosis of ovarian malignancy lead to increased morbidity, compromise of potential for a long disease free interval and/or possibly lessened the chance of cure in each case. CONCLUSIONS: Misinterpretation of clinical signs and an over-reliance on ultrasound diagnosis were the commonest causes of inappropriate management of these patients. Gynaecologists should consider more frequently the other, less common differential diagnoses of a pelvic mass. This is especially true in circumstances with a predominantly solid tumour, where there are clinical signs of vascular or rectal displacement, or where there is ultrasound evidence of ureteric obstruction. The more frequent utilisation of a computerised tomography scan with intravenous and oral contrast with referral before inappropriate transperitoneal biopsy are recommended as complete en bloc surgical excision at the first laparotomy is the treatment of choice in virtually all primary retroperitoneal tumours. ( info) |
9/99. liposarcoma involving the periodontal tissues. A case report. Liposarcomas constitute 15 to 20% of all soft tissue tumors. They are extremely rare in the head and neck and in the oral cavity. A 30-year-old patient was seen for a soft, painless mass in the right palate. Through panoramic radiography it was possible to observe a radiolucent area with sharp margins in the right upper quadrant. The lesion, after an incisional biopsy, was diagnosed as a "myxoid liposarcoma." The patient underwent a wide excision of the lesion with bone laminectomy and he is well at a 4-year follow-up. The differential diagnosis included salivary gland tumors and palatal abscess. ( info) |
10/99. Recurrent liposarcomas of the abdomen and retroperitoneum: three case reports. We report three cases of patients with liposarcomas of the abdomen who had been treated during the last 13 years (1980-1993). Two patients were men, aged 29 and 51 years, with tumors of the retroperitoneal space and the third patient was a woman aged 64 years with a tumor in the peritoneal cavity. Therapeutic treatment was as aggressive as possible excision of the tumor. In the case of the first male patient, the histological examination revealed a retroperitoneal myxoid liposarcoma which recurred 5 times within 13 years. In the second male patient, it revealed a well differentiated retroperitoneal liposarcoma of the sclerosing type which recurred 5 times within 5 years since the first treatment. Finally, the one female patient had 2 recurrences of myxoid liposarcoma of the abdomen 9 years after the first operation, presented with an infected mass and has been well since then. ( info) |