PMC4765132_01
CT of the primary LMS. CT of the abdomen and pelvis showing an intraperitoneal tumor in the left quadrant measuring 8 x 7 x 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
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Fig1
Computerized tomography (CT) of the abdomen revealed an intraperitoneal tumor in the left quadrant measuring 8 x 7 cm axially and 7 cm craniocaudally (Fig. 1a). The tumor was closely related to the descending colon and small intestine and revealed heterogenous contrast enhancement with necrotic areas (Fig. 1b).
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
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Fig2
Up to 14 mitoses per 10 high power fields (1734 mm2) were found (French malignancy grade 3) (Fig. 2a, b). Immunohistochemical examination showed distinct, focal positivity for caldesmon, SMA and desmin, supporting the diagnosis of LMS (Fig. 2 c-e).
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)
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Fig3
CT of the abdomen, but multiple contrast enhancing lesions, typical for metastases, were seen in the liver, the largest measuring close to 3 cm (Fig. 3a). Chest CT revealed multiple round, well-circumscribed lesions in both lungs consistent with metastases (Fig. 3b). Biopsy of a liver lesion confirmed metastatic disease and showed only large, pleomorphic cells (Fig. 3a insert) consistent with a highly malignant pleomorphic sarcoma.
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 x 6 mm (a, arrow) and no other visible metastatic foci in the lungs or the liver (b) are seen after chemotherapy
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Fig4
The patient showed a radiological response, exhibiting partial remission with a reduction in size and number of liver and lung metastases (Fig. 4). Subsequent radiological assessment confirmed an almost complete radiological response to this intense schedule of chemotherapies (Fig. 4).
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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Fig5
A complete outline and scheduling of the various chemotherapies are displayed in Fig. 5.