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. 2015:14:40-3.
doi: 10.1016/j.ijscr.2015.06.022. Epub 2015 Jul 15.

Diffuse intra-abdominal low grade fibromyxoid sarcoma with hepatic metastases: Case report and review of the literature

Jana Konecna  1 , Gabriel Liberale  2 , Johnny Haddad  1 , Nicolas de Saint-Aubain  3 , Issam El Nakadi  1
Affiliations

Diffuse intra-abdominal low grade fibromyxoid sarcoma with hepatic metastases: Case report and review of the literature

Jana Konecna et al. Int J Surg Case Rep. 2015.

Abstract

Introduction: Low grade fibromyxoid sarcoma (LGFMS)(1) is a rare soft tissue tumor involving deep soft tissues of the extremities and trunk. Abdominal location is extremely uncommon in which the few cases published in the literature are characterized by slow tumoral progression and long recurrence-free intervals.

Methods: We report the first case of an intra-abdominal LGFMS which was discovered incidentally in a 42-year-old woman presenting diffuse peritoneal nodules and hepatic metastasis on CT and MRI scans.

Results: The patient was successfully treated through conservative measures and remained asymptomatic at the 48 month follow-up.

Conclusions: This is the first reported case of LGFMS with peritoneal and hepatic metastases. Despite the discovery of an advance disease at exploration, the patient who refused a major surgical operation presents an uneventful follow-up and long term survival.

Keywords: Hepatic metastases; Low grade fibromyxoid sarcoma; Peritoneal metastases; Prognosis; Treatment.

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Figures

Fig. 1
Fig. 1
Coronal contrast abdominal CT shows the hepatic metastatic lesions (white arrows) and multiple disseminated peritoneal nodules (black arrows).
Fig. 2
Fig. 2
Coronal Pet/CT image. A: nodules without metabolic activity; B: nodules with a weak hypermetabolic activity.
Fig. 3
Fig. 3
Histopathological image of LGFMS characterized by fibrous and myxoid areas and swirling whorled growth patterns with low to moderate cellularity and bland cells with minimal nuclear pleomorphism.
Fig. 4
Fig. 4
MUC4 immunostaining showing strong and diffuse expression.
See this image and copyright information in PMC

References

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