PMC9330136_01
The timeline of the disease.
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F1
A 46-year-old male patient complained a painless mass with slow progreesion at the right ankle over 6 years (Figure 1).
(A-E) The pathological findings of the primary and metastatic tumors obtained from five surgeries (HE staining, x200 magnification and x400 magnification). The panels exhibit a characteristic cellular round cell tumor with a background of abundant myxoid matrix comprised with numerous lipoblasts. (F) NR4A3 rearrangement by break-apart FISH showing signals detected in the form of split signals (centromere, orange; telomere, green), in contrast to normal fused signals. DAPI x 1000.
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F2
However, the pathological exmation revealed the diagnosis of extraskeletal myxoid chondrosarcoma (Figure 2A). Tomography (SPECT) only presented diffusely increased activity in the right foot without distant metastasis (Figure 3C). Subsequently, a incisional biopsy of the lesion at the right foot and right tibial was performed. Pathological finding showed a characteristic cellular round cell tumor with a background of abundant myxoid matrix comprised with numerous lipoblasts (Figure 2B). The FISH test identified the translocation of NR4A3, that confirmed the diagnosis of extraskeletal myxoid chondrosarcoma (Figure 2F). Nonetheless, the patient refused further surgical resection, and the chemotherapy consist of doxorubicin and ifosfamideand was administrated for five cycles after Multi-Disciplinary Treatment. The pathological result confirmed the metastase of EMC in inguinal lymph node (Figures 2C,D). CT showed multiple bone metastases including ribs, vertebral body and ilium (Figures 5A,B). Considering the involvement of vascular and nerves, the patients was treated with right hip disarticulation to control the lesion and improve the quality of life (Figure 2E).
(A-B) Radiograph showed a mass on the back of the right foot and a low intensity at the middle of the tibia (red arrow). (C) The SPECT displayed a hypermetabolism foci on the right foot.
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F3
The radiograph showed a mass on the back of the right foot, and a low intensity at the middle of the tibia (Figures 3A,B). Tomography (SPECT) only presented diffusely increased activity in the right foot without distant metastasis (Figure 3C). Subsequently, a incisional biopsy of the lesion at the right foot and right tibial was performed. Pathological finding showed a characteristic cellular round cell tumor with a background of abundant myxoid matrix comprised with numerous lipoblasts (Figure 2B). The FISH test identified the translocation of
(A) The radiograph showed an increase in size compared with Figure 1A and implication of the tarsus and talus; the radiograph revealed that pathological fracture in the middle of the right femur with a heterogeneous low signal (red arrow) in the medullary cavity. (B) T2-weighted MRI showed a heterogeneous high homogeneous signal in T2WI with a clear border. (C) and (D) the X-ray and MRI revealed the metastasis in the tibia and fibula. Consecutive bone destruction was seen in the middle and lower segment of the tibia and the distal fibula. (E) and (F) The MRI (coronal and transverse sections) displayed the metastasis in right inguinal lymph nodes (G) CT scan of the chest showed the pulmonary metastasis. (H) CT showed the left axillary lymph node metastasis.
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F4
After five cycles chemotherapy, the local recurrent lesion progressed and involved the right foot, tibia, and fibula with a complete loss of right ankle function (Figures 4A-D). In addition, CT and magnetic resonance imaging (MRI) also revealed multiple suspicious metastases in the right inguinal lymph node (Figures 4E,F), pulmnoary (Figure 4G) and the left axillary lymph nodes (Figure 4H).
(A) Pathological fracture of the right femur. (B) Metastases at the ribs, vertebral and ilium on CT.
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F5
CT showed multiple bone metastases including ribs, vertebral body and ilium (Figures 5A,B). Considering the involvement of vascular and nerves, the patients was treated with right hip disarticulation to control the lesion and improve the quality of life (Figure 2E).