A giant mesentery malignant solitary fibrous tumor recurring as dedifferentiated liposarcoma- a report of a very rare case and literature review.

[dedifferentiated liposarcoma]

We report a case of a 59 -year -old woman with a very rare giant mesentery malignant solitary fibrous tumor that recurred as dedifferentiated liposarcoma . The woman was admitted to the hospital because of low abdominal pain . Radiological and biopsy findings revealed a multi-lobulated giant malignant solitary fibrous tumor that had invaded the inferior vena cava , abdominal aorta , and superior mesentery vessels . The tumor was completely removed during the first cytoreductive surgery . Histopathologically , tumor had a heterogeneous cell population , composed of spindle cells with fibrous collagen proliferation . The spindle cells were not arranged in a specific pattern . Immunohistochemistry revealed that the tumor cells were positive for CD 34 , CD 99 , Bcl-2 , and smooth muscle actin ( SMA ) and negative for CD 117 , epithelial membrane antigen (EMA ), CAM 5 .7 , S 100 , desmin , and caldesmon . The tumor recurred 9 months after surgery , and another cytoreductive surgery was then performed . The postoperative histopathological appearance of the invaded area indicated a well-differentiated liposarcoma . Formation of tumorous bone was also noted in the same area , in addition to atypical mesenchymal cells and multi-vacuolated lipoblasts in the area of the well-differentiated liposarcoma . Proliferated spindle cells arranged in a storiform pattern were found in the area adjacent to the tumor . Immunohistochemical analysis revealed that the tumors cells were positive for SMA , HHF-35 , and caldesmon and negative for CD 117 , CD 34 , and S 100 . A diagnosis of dedifferentiated liposarcoma was made .

Diseases
Validation

Diseases presenting "abdominal pain" symptom

22q11.2 deletion syndrome

adrenal incidentaloma

alpha-thalassemia

benign recurrent intrahepatic cholestasis

carcinoma of the gallbladder

child syndrome

cholangiocarcinoma

congenital diaphragmatic hernia

cushing syndrome

cutaneous mastocytosis

cystinuria

dedifferentiated liposarcoma

erdheim-chester disease

erythropoietic protoporphyria

fabry disease

familial hypocalciuric hypercalcemia

familial mediterranean fever

focal myositis

liposarcoma

lymphangioleiomyomatosis

malignant atrophic papulosis

pleomorphic liposarcoma

primary effusion lymphoma

primary hyperoxaluria type 1

proteus syndrome

scrub typhus

sneddon syndrome

systemic capillary leak syndrome

typhoid

von hippel-lindau disease

well-differentiated liposarcoma

This symptom has already been validated