man presented with 2-year history of bump and pain in his left chest. In 1995,
the soft tissue tumor of abdominal wall was excised. In 2000, the recurrent tumor
was excised again. In January of 2007, he was referred to our hospital. There
was tenderness in the left chest. Radiological examination revealed a lesion
with bone destruction in the left 6th rib (). CT revealed a pleural
lesion. Bone scintigraphy and positron emission tomography (PET) showed
increased uptake in the bilateral humeral shaft and left rib (). Plain
X-ray films demonstrated an osteolytic lesion in the humeral shaft ().
The cortex was thinned. MRI revealed the lesion in the left humeral shaft with
low signal intensities on T1 weighted images and iso to high signal intensities
on T2 weighted images. The mass was enhanced after gadolinium injection ().
Figure 1 (a) Plain radiogram showing bone destruction of the rib (arrow). (b) Bone scintigram showing increased spots in the bilateral humerus and the left rib (arrow). (c) Plain radiogram of the left humerus showing an osteolytic lesion (arrow). (d) T1 weighted (more ...)
examination of the specimen removed in 1995 revealed that the lesion was composed
of many spindle cells admixed with abundant collagen matrix (). Epithelioid
cells arranged in an alveolar pattern (). Mitosis was conspicuous. It
was consistent with SEF.
the specimen of the abdominal tumor excised in 1995 showing abundant collagen proliferation (a) and alveolar pattern (b).
The tumor removed from abdominal
wall in 2000. There was richness in abundant collagen. The features of the
tumor were similar to those of the previous surgery specimen. However, this
tumor showed increased cellularity. In this specimen, epithelioid cells were more dominant than spindle
cells, and the epithelioid cells were arranged in alveolar pattern ().
These cells showed nuclear polymorphism, and increased frequency of mitosis 15
cells per 10 high-power fields (HPF). Necrosis was found in the specimen. This
area revealed ghost-like figures. These features well corresponded to SEF showing
higher-grade malignancy compared to the primary lesion.
The specimen of the abdominal tumor excised in 2000 showing epithelioid cells arranged in an alveolar pattern, too.
To confirm the diagnosis, excisional
biopsy of the 6th rib was performed. Macroscopically, the bone marrow was occupied
by a white solid tumor. Microscopically, the specimen was composed of dysplastic
cells in the hyalinized stroma. These cells were arranged in epithelioid and
alveolar patterns too. In contrast to the primary lesion, this lesion was
characterized by much higher cellularity, high nuclear/cell rate, and more prominent
nuclear polymorphism (). Mitosis was more prominent with 30 to 40 cells
per 10 HPF. Necrosis was frequently found in the lesion. Ghost-like figures were
also present too.
The specimen of the rib excised in 2007 showing cellular atypia and prominent nuclear pleomorphism.
revealed vimentin-positive cells. Although it was focal, tumor cells were
positive for HHF-35. CD99 had a weak immunostaining pattern. Tumor cells were
negative for α-SMA, desmin, CD34, S-100
protein, cytokeratin (AE1/AE3), and epithelial membrane antigen (EMA). All
three specimens showed same features by immunohistochemistry.
Ki-67 labeling indices were 7~8%, 20%, and 60%
in the tumor excised in 1995, in 2000, and in 2007, respectively, (Figures , ,
and ). In conclusion, the primary lesion was SEF and that the lesion of the
rib was bone metastasis from SEF in the abdomen.
Ki-67 staining of the specimen resected in 1995. There are few Ki-67 positive cells. Ki-67 labeling index was 7 to 8%.
The specimen resected in 2000. There were more Ki-67 positive cells than . Ki-67 labeling index was 20%.
The specimen resected in 2007. More cells were stained by Ki-67. Ki-67 labeling index was 60%.
In February 2007, pathological
fracture occurred in the left humeral shaft. Since it was a metastatic lesion, chemotherapy
using ifosfamide and adriamycin was performed. However, this chemotherapy
was discontinued because of acute myocardial infarction. Since the tumor size
is increasing, the patient was treated by radiation therapy to the left
humerus. After that, he hoped the second opinion, so he was referred to another