Recurrent retroperitoneal liposarcoma in a patient with neurofibromatosis type I.

[dedifferentiated liposarcoma]

Liposarcoma complicating neurofibromatosis is very rare . Only a few cases have been described until now . We present a case of recurrent dedifferentiated retroperitoneal liposarcoma in a patient with neurofibromatosis type I (NF-1 ).A 47 -year -old Caucasian woman with NF-1 presented to the hospital initially complaining of left lumbar pain irradiating to the anterior thigh and knee . Physical examination showed atrophy of the lower extremities bilaterally and decreased motor strength on the left lower extremity . Radiological studies demonstrated an enhancing lesion in the left paraspinal region , suggesting malignancy . The patient underwent local resection of tumour with safety margins . Pathological examination was consistent with dedifferentiated liposarcoma (DDLS ) with positivity for MDM 2 and CDK 4 markers . No evidence of metastasis was noted on the radiological studies . The final diagnosis was DDLS , high -grade (G 3 ), pT 2 bN 0 M 0 , stage III . After 6 weeks post-tumour resection , the patient experienced recurrence of malignancy . Chemotherapy with cisplatin and doxorubicin was initiated in the patient .Liposarcoma in the context of neurofibromatosis is very rare . To the best of our knowledge , only six cases have been reported until now in the literature . We are presenting this case to underline the possibility of recurrence in the case of retroperitoneal DDLSs despite local tumour resection . Also , although the role of chemotherapy is controversial we decided to start treatment with cisplatin and doxorubicin given the success of chemotherapy in similar case presentations .

Diseases
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Diseases presenting "liposarcoma in the context" symptom

dedifferentiated liposarcoma

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