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Surgical Challenges in a Case of a Recurrent Giant Pleomorphic Liposarcoma of Neck And Mediastinum in a Child: A Rare Occurrence.

ABSTRACT: Liposarcomas are rare in childhood, representing < 1% of all childhood soft tissue sarcomas. The most frequent site of involvement are the extremities with the involvement of head and neck being an absolute rarity. Pleomorphic subtype of liposarcoma is pretty aggressive and hence portends a poor prognosis. Here, we present the case of a child who presented with a recurrence of liposarcoma in the neck and mediastinum within 3 months of the index surgery.

Supplementary information

The online version contains supplementary material available at 10.1007/s12070-021-02837-w.

SUBMITTER: Mishra AK

PROVIDER: S-EPMC8396137 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:BackgroundPleomorphic liposarcoma is the least common but most aggressive subtype of liposarcoma. Very few studies have presented data on pleomorphic liposarcoma specifically, often including a limited number of cases and short-term follow-up. As a result, the survivorship and prognostic characteristics of this tumor remain incompletely identified.Study design and settingCross-sectional analysis of the Surveillance Epidemiology and End Results database (1996-2015).ResultsOverall survival for the entire series was 54% (95% confidence interval [CI], 49-58%) and 40% (95% CI, 35-45%) at 5 and 10 years, respectively. Disease-specific survival for the entire series was 60% (95% CI, 56-65%) and 53% (95% CI, 48-58%) at 5 and 10 years, respectively. Patients who survived 10 years or more were more likely to die of events unrelated to pleomorphic liposarcoma. Univariate and multivariate analysis demonstrated that not receiving cancer-directed surgery was an independent poor prognostic factor. Older age (≥ 65 years old) was associated with worse overall survival but not disease-specific survival. Tumor stage and radiotherapy showed different impact on survival depending on tumor size. In comparison to localized staged tumors, regional stage only predicts poor survival in patients with tumor size less than 5 cm, while distant stage is an independent worse prognosis factor. Radiotherapy only benefits patients with tumor size larger than 10 cm. These results were confirmed in competing risk analysis.ConclusionSurvival rates of patients with pleomorphic liposarcoma has not changed over the past 20 years. Patients with distant stage have poor prognosis; regional stage indicates worse survival in patients with tumor size less than 5 cm. Receiving surgery could prolong the survival, while radiotherapy only benefits patients with large tumor size (> 10 cm). Older age is associated with poor overall survival but not disease-specific survival. Routine patient surveillance following initial diagnosis should at least be 10 years for pleomorphic liposarcoma.

Dataset Information

Surgical Challenges in a Case of a Recurrent Giant Pleomorphic Liposarcoma of Neck And Mediastinum in a Child: A Rare Occurrence.

Supplementary information

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