PMC7160461_01
(A) Plain X-ray images of bilateral knee joints of the patient at 11 yr of age: arrow
heads indicate the rachitic changes. (B) Hematoxylin-eosin staining of the primary
tumor: The histopathology of the tumor shows proliferation of spindle to oval-shaped
cells with mild nuclear atypia and eosinophilic cytoplasm arranged in fascicular
pattern with an absence of mitosis. (C) Immunohistochemical staining of fibroblast
growth factor 23 (FGF23) shows immunopositive reactivity of the tumor cells for FGF23.
(D, E) 68Ga-DOTATOC-PET CT imaging revealed multiple tumors in the lung
fields, right forearm, right thigh and left tibia (arrows).
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An X-ray film of the limbs revealed irregular widening of the metaphyseal regions (Fig. 1-A), suggesting the presence of rickets. The tumor cells were immunohistochemically positive for FGF23 (Fig. 1-B, C). Chest X-ray revealed multiple small nodules in the lung all positive for 68Ga-DOTATOC-PET CT (Fig. 1-D, E).
Sequential levels of serum fibroblast growth factor 23 (FGF23) levels during the
observation. FGF23 levels were plotted from the sampled sites of upper extremity ( ),
right upper extremity (), left upper extremity (), right lower extremity (x), and
left lower extremity (+). Serum FGF23 levels increased to 3100 pg/ml despite the
resection of lesions in the trapezius muscle, right thigh and left tibia, and
decreased to 1180 pg/ml prior to amputation of her left leg. Values higher than 800
pg/ml were measured after dilution of the samples; hatched area indicates the standard
range of FGF23 in healthy subjects (16-69 pg/ml). Surgical intervention has been
indicated in the upper panel of the figure, and clinical events have been shown below
the horizontal axis.
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FGF23 (Fig. 2). Four months after the intervention, non-tender massive lesions emerged in the right thigh, left tibia, and right forearm. Serum intact FGF23 levels were found to have increased to 3,100 pg/ml (Fig. 2), suggesting an effect of the residual tumors.
(A) The swollen left leg (arrow head) shows (B) histopathology of the tumor in the
leg at 17 yr of age. Tumor shows a diffuse sheet-like growth pattern with oval to
spindle-shaped or polygonal cells possessing hyperchromatic and pleomorphic nuclei and
occasional multinucleated giant cells. Numerous mitotic figures have been identified.
(C) CT scan image of the abdomen shows a large mass at the head of pancreas (arrow
heads). (D) PET CT scan image of body shows multiple positive lesions in the left
lung, head of pancreas, right ovary and left inguinal lymph nodes (arrows). (E) CT
scan image of the brain shows a mass measuring 35 mm x 29 mm, suggesting intracranial
metastasis (arrow heads).
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Two months after the beginning of swelling of the leg, the patient was re-hospitalized due to difficulty in walking (Fig. 3-A). They were accompanied by bizarre giant cells, multinucleated osteoclast-like giant cells, osseous formations, hemosiderin deposition and massive necrosis with florid mitotic findings (Fig. 3-B) indicating pleomorphic sarcoma. A CT scan was performed, which detected a mass at the head of the pancreas (Fig. 3-C). PET CT showed an increased number of lung lesions as well as new lesions emerging in the inguinal lymph nodes and the right ovary (Fig. 3-D). One month later, the patient was brought into the emergency room because of convulsions, and the CT scan revealed a brain metastasis measuring 35 mm x 29 mm (Fig. 3-E).