2012
DOI: 10.1136/bcr-2012-006310
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Recurrent retroperitoneal liposarcoma in a patient with neurofibromatosis type I

Abstract: 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.

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Cited by 7 publications
(2 citation statements)
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“…WD/DD liposarcoma is characterized by 12q13-15 amplification, a hallmark genetic feature in this disease. While there are no known genetic cancer syndromes associated with 12q13-15 amplification, liposarcoma has been infrequently reported in select patients with Li-Fraumeni syndrome and neurofibromatosis (9,(21)(22)(23). There are no other defined genetic cancer syndromes associated with a higher risk of liposarcoma to our knowledge.…”
Section: Discussionmentioning
confidence: 72%
“…WD/DD liposarcoma is characterized by 12q13-15 amplification, a hallmark genetic feature in this disease. While there are no known genetic cancer syndromes associated with 12q13-15 amplification, liposarcoma has been infrequently reported in select patients with Li-Fraumeni syndrome and neurofibromatosis (9,(21)(22)(23). There are no other defined genetic cancer syndromes associated with a higher risk of liposarcoma to our knowledge.…”
Section: Discussionmentioning
confidence: 72%
“…WD tumours are low grade and generally indolent, without the potential for metastasis; in contrast, tumours that have a DD component are high grade, with an approximately 20%–30% distant metastatic rate, most commonly to the lungs 2. While there are hereditary syndromes associated with colon adenocarcinoma, except for isolated case reports (eg, neurofibromatosis),3–5 there are no defined hereditary syndromes associated with WD/DD liposarcoma. In this case report, we describe a patient who had concurrent colon adenocarcinoma and DD liposarcoma, treated to no evidence of disease, followed by synchronous recurrence of both cancers.…”
Section: Introductionmentioning
confidence: 99%