PMC5745994_01
Axial contrast enhanced T1-weighted MRI. Pathologic tissue in the hypothenar eminence of the right hand, involving the flexor tendons and the spaces between 3rd, 4th and 5th metacarpal bones with intense and homogeneous contrast enhancement (a). Progressive size and contrast enhancement reduction after ILP (b), and chemotherapy (c)
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Fig1
The treatment was well tolerated and achieved an excellent clinical and radiological response, i.e. partial response (PR) according to RECIST (Fig. 1b). We observed a PR both on the relapsed primary tumor and on the lung metastases after three and six cycles (Figs. 1c, 2b, 4c, d).
Picture of the patient's hand. At presentation a the tumor involved widely the hypothenar eminence and medial aspect of the wrist. Seven months after ILP b the nodule in the wrist was hardly palpable while the nodule in the hand was smaller and softer
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Fig2
We observed a PR both on the relapsed primary tumor and on the lung metastases after three and six cycles (Figs. 1c, 2b, 4c, d).
a Malignant OFMT pre-ILP biopsy, (HE, 40x). Densely packed cords of monomorphic cell with small round nuclei, high mitotic index. b Fluorescent in situ hybridization showing tetraploid cells showing unbalanced PHF1 gene translocation (green 5'). c Malignant OFMT post-ILP and chemotherapy, vital component (HE, 20x). The neoplasm is less densely cellulated and exhibits a variable morphology ranging from more rounded to spindle cell morphology. Mitotic activity is considerably reduced. d Malignant OFMT post-ILP and chemotherapy, non-vital component (HE, 20x). At the bottom of the image, a dense collagen sclerosis with haemosiderin deposition, ghost cells at the upper part, in between cell debris and isolated neoplastic cells with nuclei similarly to the viable component
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Fig3
The mitotic count was 14/10 HPF, necrosis was present (Fig. 3a). The diagnosis of malignant OFMT was confirmed by FISH test positive for PHF gene rearrangement (Fig. 3b).  Viable tumor was mostly located proximally, characterized by a heterogeneous morphology (Fig. 3c, d).
Coronal contrast enhanced CT scan. A right lung lower lobe metastases (arrow) at baseline (a), increasing after ILP (b), and progressively reducing after 3 (b) and 6 (d) cycles of chemotherapy with epirubicin and ifosfamide
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Fig4
In September 2016, when a new CT scan showed further progression of the lung metastases (Fig. 4b), and missing any evidence regarding the effectiveness of systemic treatment in this histotype, the patient underwent sarcoma-oriented chemotherapy with Epirubicin 105 mg/sqm and Ifosfamide 9000 mg/sqm + MESNA in 3 days every 3 weeks. We observed a PR both on the relapsed primary tumor and on the lung metastases after three and six cycles (Figs. 1c, 2b, 4c, d).