Primary retroperitoneal myxoid/round cell liposarcoma is a nonexisting disease: an immunohistochemical and molecular biological analysis

Vreeze, Ronald S. A. de; Jong, Daphne de; Tielen, Ivon H. G.; Ruijter, Henrique J.; Nederlof, Petra M.; Haas, Rick L.; Coevorden, Frits van

doi:10.1038/modpathol.2008.164

Cited by 74 publications

(50 citation statements)

References 24 publications

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“…Myxoid liposarcoma is rarely found in the mediastinum or in the retroperitoneum; therefore, metastatic spread from other sites has to be excluded first in order to make this diagnosis. 30 The behavior of these tumors will be dependent on their histology; conventional myxoid liposarcoma behaves as a low-grade malignancy, whereas cases characterized by increased cellularity and solid sheets of cells (that is, round cell liposarcoma) can display a highly aggressive behavior. In our study, all three cases of myxoid liposarcoma contained a prominent hypercellular round cell component and displayed aggressive behavior, in two cases leading to death due to tumor with widespread metastases.…”

Section: Discussionmentioning

confidence: 99%

Liposarcomas of the posterior mediastinum: clinicopathologic study of 18 cases

et al. 2015

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Sarcomas of the posterior mediastinum are rare and correspond mostly to neurogenic tumors. We studied 18 cases of liposarcoma presenting in the posterior mediastinum; because of their unusual location, some of these tumors posed difficulties for diagnosis. There were 11 men and 7 women aged 29-87 years (mean: 57). The tumors were large lobulated masses ranging from 6 to 30 cm in greatest diameter (median: 15 cm). Symptoms included cough, dysphagia, and chest pain. Four patients were asymptomatic and the tumors were discovered incidentally on chest X-rays. Histologically, 10/18 (55%) cases were atypical lipomatous tumor/ well-differentiated liposarcoma, one of which harbored a smooth muscle component (lipoleiomyosarcoma); 3/18 (16%) were de-differentiated liposarcoma, one of which also harbored a smooth muscle component; 3/18 (16%) were myxoid/round cell liposarcoma; and 2/18 (11%) were pleomorphic liposarcoma. The cases of well-differentiated liposarcoma were mostly of the sclerosing type; however, five of them also showed prominent myxoid stroma closely resembling myxoid liposarcoma. Immunohistochemistry was performed in selected cases; 4/8 cases tested showed focal positivity for S-100 protein and 5/8 cases showed nuclear positivity for MDM-2. The three cases of myxoid liposarcoma were all negative for MDM2. Both cases of lipoleiomyosarcoma showed positivity for SMA and desmin in the smooth muscle component. FISH was performed in two cases of well-differentiated liposarcoma and high levels of amplification of MDM2 at 12q13-15 were observed; the CHOP translocation at 12q13.1-q13.2 was absent in both cases. Complete surgical excision was performed in 11 cases; however, negative surgical margins were achieved only in four. Clinical follow-up ranging from 1 to 192 months (median 28 months) was available for 13 patients. Two patients with myxoid/round cell liposarcoma died of tumor after 4 months and 3 years, respectively. Both had widely disseminated metastatic disease at the time of death. Six patients (6/10) with well-differentiated liposarcoma were alive and well with no evidence of disease (at 4, 7, 12, 15, and 25 months) and three (3/10) were alive with disease (at 3, 4, and 6 months). One patient with well-differentiated liposarcoma had multiple recurrences and a liver metastasis after 14 years; however, the patient was alive and well at 16 years. Five patients were lost to follow-up. In general, the biologic behavior of liposarcomas in the posterior mediastinum seems to correlate well with the histologic subtype and mirrors that of their counterpart in the retroperitoneum.

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Section: Discussionmentioning

confidence: 99%

Liposarcomas of the posterior mediastinum: clinicopathologic study of 18 cases

et al. 2015

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“…The recognition that WDLS may display prominent myxoid features has led to the understanding that a significant number of (if not all) cases that previously were labeled as mixed well differentiated and myxoid liposarcomas, in fact represents heavily myxoid WDLSs [18,19]. Moreover, awareness of this should help to avoid making an erroneous diagnosis of a myxoid liposarcoma and if in doubt perform immunohistochemical and/or molecular genetic studies for over expression/amplification of mdm2-MDM2/cdk4-CDK4 and CHOP-FUS rearrangements, respectively.…”

Section: Discussionmentioning

confidence: 99%

Dedifferentiated Liposarcoma of the Deep (Paralaryngeal) Soft Tissue: Lessons Learnt from a Case with a Partly Deceptively Benign Appearing Dedifferentiated Component

Petersson

Murugasu

2013

Head and Neck Pathol

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We present a case (female, 61 years of age) of dedifferentiated liposarcoma of the deep, cervical (paralaryngeal) soft tissue with a significant myxoid component and characteristic immunohistochemical (strong and diffuse expression of p16, mdm2 and cdk4 in both the well differentiated liposarcomatous and dedifferentiated components) and molecular genetic findings (MDM2-gene amplification on fluorescence in situ hybridization). The myxoid component which was present in the well differentiated liposarcomatous component gave the tumor atypical radiological features. The case presented initial diagnostic difficulties, mainly because of the bland histomorphological appearance of the limited biopsy material from the sampled non-lipogenic, dedifferentiated component. The dedifferentiated part of the tumor turned out to harbor significant heterogeneity with regards to cellularity, cytomorphology and proliferative activity.

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“…46 A diagnosis of primary retroperitoneal myxoid liposarcoma should made with caution, because such cases represent either metastatic myxoid liposarcoma or well-differentiated/dedifferentiated liposarcoma with myxoid stromal change. 43,63 Round cell liposarcoma is not an independent subtype but rather a high-grade spectrum of myxoid liposarcoma. Round cell liposarcoma shares the same molecular change as myxoid liposarcoma but is morphologically composed of blue round tumor cells.…”

Section: Other Subtypes Of Liposarcomamentioning

confidence: 99%

“…7,9,11,14,16 Myxoid/round cell liposarcoma as a primary malignancy is rare in the retroperitoneum (< 0.9% of all retroperitoneal sarcoma subtypes), and physical examination and imaging of the extremities to rule out a primary site in an extremity with distant retroperitoneal metastasis are indicated. 6,43 Primitive neuroectodermal tumors, Ewing sarcoma, and alveolar/embryonal rhabdomyosarcoma are radiosensitive and chemosensitive, so they should not be considered for first-line surgery. Because primary chemotherapy is always given, the strategy for these tumors significantly differs from that undertaken for adult-type soft-tissue sarcoma.…”

Section: Dedifferentiated Liposarcomamentioning

confidence: 99%