PMC4705745_01
a Whole body bone scintigraphy 99mTc-MDP. Moderate uptake in the right shoulder, on both sides of the ribs due to osteoblastic lesions. Reduces radiotracer uptake in right femoral head (long arrow). b 99mTc-MDP SPECT/CT of the pelvis. Reduced radiotracer uptake in right femur head, corresponding sclerotic lesion on CT (long arrow) (year 2011)
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Fig1
Whole body bone scintigraphy with 99mTc-MDP 550 MBq showed increased focal uptake of a radiotracer in the left shoulder and in the ribs on both sides due to osteoblastic process and reduced uptake in right femoral on SPEC/CT sclerotic bone lesion without tumour at that time or Morbus Paget specificity (Fig. 1).
a The posture before the right hip endoprosthesis operation and tumour extirpation. The patient has given the written consent for his medical findings and photos to be published. b 99mTc-Tektrotyd whole body scintigraphy. Intensive, focal radiotracer uptake in right femur head ( red markers around) (year 2014)
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Fig2
During the time period of almost 4 years, the clinical condition continued to worsen with a loss of height for 15 cm and weight for 20 kg from year 2011 now standing for 165 cm height and 53 kg weight (BMI = 23.1 kg/m2), permanent pain in chest and hips, progressing breathing discomfort, muscular weakness, inability to bend below knees and posture change (Fig. 2, photo taken in October 2014).
a 99mTc-Tektrotyd SPECT /CT of a pelvis. Focal, intensive radio tracer uptake in right femoral head (long arrow) and b corresponding sclerotic lesion on CT (long arrow) (year 2014)
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Fig3
During the time period of almost 4 years, the clinical condition continued to worsen with a loss of height for 15 cm and weight for 20 kg from year 2011 now standing for 165 cm height and 53 kg weight (BMI = 23.1 kg/m2), permanent pain in chest and hips, progressing breathing discomfort, muscular weakness, inability to bend below knees and posture change (Fig. 2, photo taken in October 2014). The whole body scintigraphy with somatostatin analogues 99mTc-tekrotyd 605 MBq revealed intensive uptake of radiotracer in the right femoral head, and SPECT/CT demonstrated uptake in sclerotic bone lesion in the right femoral head:tumour intensively expressing somatostatin receptors (Fig. 3).
Osteosclerotic tumour in the right femur head revealed during the whole body computerised tomography (year 2014)
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Fig4
During the time period of almost 4 years, the clinical condition continued to worsen with a loss of height for 15 cm and weight for 20 kg from year 2011 now standing for 165 cm height and 53 kg weight (BMI = 23.1 kg/m2), permanent pain in chest and hips, progressing breathing discomfort, muscular weakness, inability to bend below knees and posture change (Fig. 2, photo taken in October 2014). CT indicated deformations in skeletal bones, ribbons, thoracic vertebrae and pelvic bone due to osteomalacia or stress fractures, and no signs of healing were observed. CT also showed compressive fractures in Th3-Th11 with pathologic kyphosis, stress fractures in both femoral heads and osteosclerotic tumour in right femur head (Fig. 4).
Histologic examination of the specimen revealed a hypercellular tumour composed of spindle-shaped cells in fibromyxoid matrix, with a hemangiopericytoma-like pattern (a). Tumour cells had clear to eosinophilic cytoplasm, nuclei were oval or elongated, of monomorphic appearance, with no signs of atypia. Mitotic figures were rarely seen. Surrounding bone trabeculae were irregular in size and shape with wide osteoid seams and foci of hyaline cartilage, showing enchondral ossification (b, c). The tumour cells were strongly and diffusely positive with vimentin (d) but exhibited no reactivity with PanCK, SMA, CD34, CD10 and D2-40 (year 2014)
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Fig5
During the time period of almost 4 years, the clinical condition continued to worsen with a loss of height for 15 cm and weight for 20 kg from year 2011 now standing for 165 cm height and 53 kg weight (BMI = 23.1 kg/m2), permanent pain in chest and hips, progressing breathing discomfort, muscular weakness, inability to bend below knees and posture change (Fig. 2, photo taken in October 2014). The tumour cells were strongly and diffusely positive with vimentin but exhibited no reactivity with PanCK, SMA, CD34, CD10 and D2-40 (Fig. 5). 
Conventional radiography of the spine after 1 year after tumour resection showed permanent and nonreversible spine deformation due to the old compressions from Th3 to Th11 with no evidence of new compression fractures (year 2015)
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Fig6
During the time period of almost 4 years, the clinical condition continued to worsen with a loss of height for 15 cm and weight for 20 kg from year 2011 now standing for 165 cm height and 53 kg weight (BMI = 23.1 kg/m2), permanent pain in chest and hips, progressing breathing discomfort, muscular weakness, inability to bend below knees and posture change (Fig. 2, photo taken in October 2014). Conventional radiography of the spine after 1 year showed permanent and nonreversible spine deformation due to the old compressions from Th3 to Th11 with no evidence of new compression fractures (Fig. 6).