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Case Reports
. 2016 Feb 24;6:2.
doi: 10.1186/s13569-016-0042-6. eCollection 2016.

Metastatic Mesenteric Dedifferentiated Leiomyosarcoma: A Case Report and a Review of Literature

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Free PMC article
Case Reports

Metastatic Mesenteric Dedifferentiated Leiomyosarcoma: A Case Report and a Review of Literature

Mercy Varghese et al. Clin Sarcoma Res. .
Free PMC article

Abstract

Background: Abdominal leiomyosarcoma arising from the mesentery is a rare malignancy. It is an aggressive entity with an overall 5 year survival rate between 20 and 30 %. Surgical resection is the cornerstone of primary treatment and may be curative for localized disease. However, patients often develop intra-abdominal relapse and/or metastatic disease. If surgical resection is not feasible, palliative chemotherapy is the treatment of choice. However, there are no clear guidelines regarding chemotherapy; neither in the adjuvant nor advanced setting.

Case presentation: We present a 40 year-old woman, with a mesenteric leiomyosarcoma, who underwent radical tumor resection and did not receive adjuvant oncological therapy. Three months postoperatively, she developed metastatic disease to the lungs and liver. After multidisciplinary assessment she received an unconventional histological-subtype-tailored chemotherapy comprising 3-4 regimens. Initially, there was a decrease both in number and size of metastases. Ultimately, an almost complete radiological response was seen. Subsequent surgical resection and radiofrequency ablation of residual metastatic foci in the liver and lung brought her into complete clinical remission. She is presently tumor free, 36 months following diagnosis of metastatic disease.

Conclusions: To our knowledge, this is the first report of a patient with metastatic mesenteric leiomyosarcoma who is in complete clinical and radiological long-term remission following very aggressive multimodal treatment; including intense poly-drug chemotherapy and without any demonstrable long-term side effects. Given the rarity of mesenteric leiomyosarcoma and lack of guidelines regarding oncological therapy, we suggest that multimodal therapy including aggressive chemotherapy, guided by a multidisciplinary team, is essential to achieve an optimal outcome.

Keywords: Abdominal; Chemotherapy; Histological-subtype; Leiomyosarcoma; Mesenteric; Metastases; Multimodal.

Figures

Fig. 1
Fig. 1
CT of the primary LMS. CT of the abdomen and pelvis showing an intraperitoneal tumor in the left quadrant measuring 8 × 7 × 7 cm (a coronal section). The tumor is located in the mesentery and is in close relation to both the small bowel and the sigmoid colon (b axial section). The low-density region within the tumor indicates necrosis
Fig. 2
Fig. 2
Histopathology of tumor. Histopathological analysis of primary tumor revealed pleomorphic, spindle cells (a) and pleomorphic cells (b) with eosinophilic cytoplasm consistent with a high grade, pleomorphic leiomyosarcoma. Immunohistochemical examination showed distinct, focal positivity for SMA (c), desmin (d) and H-caldesmon (e), markers that are characteristic for LMS
Fig. 3
Fig. 3
CT of metastatic disease. Axial CT of the abdomen and thorax. Multiple contrast-enhancing lesions in the liver with irregular borders typical for metastases (a). Histopathological analysis of a liver metastasis revealed only large, pleomorphic cells (a inset) consistent with a highly malignant dedifferentiated pleomorphic sarcoma. CT of thorax showing multiple round, well-circumscribed lung lesions consistent with metastases. The ground-glass opacity around the lesions may be caused by hemorrhage (b)
Fig. 4
Fig. 4
CT showing radiological response. Axial CT of the thorax and abdomen showing almost complete radiological response after histological subtype-specific chemotherapy. A small metastatic lesion measuring 5 × 6 mm (a, arrow) and no other visible metastatic foci in the lungs or the liver (b) are seen after chemotherapy
Fig. 5
Fig. 5
A timeline of events and chemotherapy schedules. Diagnosis of metastatic disease designated as time point zero and denoted as 0 months (0 mo). Milestones within response evaluation and interventional therapy denoted as running months from zero time point. Abbreviations of chemotherapeutic drugs: Doxorubicin and Ifosfamide (Doxo + Ifo), Low-dose Doxorubicin (LD Doxo), Gemcitabine and Docetaxel (Gem + Doce), High-dose Ifosfamide (HD Ifo)

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