Keywords
cystosarcoma phyllodes - cervical spine - metastasis - vertebrae
Introduction
Phyllodes tumor is a benign fibroepithelial lesion of the breast, which can become
malignant in a minority of cases. Local recurrence and spread is common, but distant
metastasis is rare. Metastases, however, have been known to occur to most organs,
including the brain and thoracolumbar spine.[1]
Case Report
A 32-year-old lady, who had undergone surgery for cystosarcoma phyllodes involving
the right breast 1 year earlier, presented with severe neck pain of 2-month duration.
There was no radiation of pain to the upper limbs and no symptoms suggestive of motor
or sensory deficits. Micturition was normal.
She was fully conscious and oriented. General examination was unremarkable. There
were no focal neurological deficits. Neck movements were restricted, and there was
tenderness of the cervical spine.
X-ray of the cervical spine showed the collapse of C3 vertebra, and MRI of the cervical
spine ([Fig. 1]) revealed a destructive lesion involving the C3 and C4 vertebral bodies, causing
total collapse of C3 body and a large prevertebral soft tissue component at the level
of the epiglottis. A small epidural component was present at the same level causing
thecal compression. There was no compression of the cord. The pedicles were involved
bilaterally at C3 and C4 levels.
Fig. 1 MRI (T2-weighted) showing total destruction of C3 body with preserved disc spaces
and a large prevertebral soft tissue lesion.
She underwent surgical decompression of the soft tissue component. The lesion was
found to be a compressible, rubbery, vascular mass in the prevertebral plane at C3–4
level with extension into the vertebral bodies, causing total destruction of C3 body
and destruction of the upper part of C4 body. The lesion was extending under the C4
body in the extradural space. At the end of the surgery, an expandable cage (Xpand,
Globus Medical Audubon, Pennsylvania, United States) filled with bone graft was placed
in the corpectomy defect anteriorly. Posteriorly pedicle screws were placed on C5
and lamina hooks on C2, and fusion was completed with rods and bone graft.
The biopsy was reported as being consistent with a metastatic high-grade phyllodes
tumor ([Fig. 2]
[3]
[4]). Postoperatively she had complete relief of pain. After the wound had healed, she
was referred to the oncologist for radiation and chemotherapy to control the growth
of the lesion. A repeat imaging of the cervical spine done 6 months later showed evidence
of local recurrence, but there was no spinal compression. A CT scan of the thorax
done at the same time revealed multiple lesions involving both lungs ([Fig. 5]) and hence she was started on chemotherapy. Two years after the surgery, she died
following an episode of uncontrolled hemoptysis. Till the time of her death, she was
ambulant and active, but had pain in her shoulders and neck.
Fig. 2 Spindly tumor cells with oval hyperchromatic nuclei arranged in sheets (hematoxylin
and eosin).
Fig. 3 Spindly cells exhibiting vimentin positivity (immunohistochemistry).
Fig. 4 Spindly cells exhibiting cytokeratin positivity (immunohistochemistry).
Fig. 5 Axial section of the CT scan of the thorax (contrast) showing an enhancing nodule
on the left side (black arrow).
Discussion
Cystosarcoma phyllodes, also known as phyllodes tumors, are rare tumors that involve
breast. First reported by Johannes Muller in 1838, they make up less than 1% of all
breast tumors.[1] The majority of these lesions behave in a completely benign manner, leaving local
recurrence as the only real concern. However, up to 30% of these lesions show histopathological
features of malignancy, though the clinical course may not correlate with the histopathological
grading.[2]
These tumors can occur at any age, but present most commonly in the fifth decade of
life.[3] The mean age of presentation for recurrent lesions is 52 years. The time to metastasis
varies from 3.5 months to 14 years from initial diagnosis in various studies.[4]
[5]
Histopathologically, they are characterized by a combination of hypercellular stroma
and cleft-like or cystic spaces lined by epithelium, into which the stroma classically
projects in a leaf-like fashion. This appearance is in part responsible for the name,
“phyllos” meaning “leaves.” Stromal cellularity and infiltrating tumor margins are
predictors of local recurrence.[6] About 15% of patients with phyllodes tumor develop local recurrence, while 5 to
10% develop metastases.[2] This figure goes up to 20% for patients with malignant phyllodes tumors. The hematogenous
route is usually responsible for the metastatic disease, and lymphatic spread is rare.[6] Most distant metastases may develop without evidence of local recurrence.[7]
Central nervous system (CNS) metastases are uncommon, and most cases reported in the
English literature show a spread to the brain, with dural-based lesions mimicking
meningiomas being a common presentation.[8] Metastases to the sacrum and thoracolumbar spine have also been reported, but are
very rare. Patients with brain metastases have a dismal mean survival of less than
a month, but those with bony metastases, including spine, have a much better prognosis
with 24-month survival being reported. Our patient also survived for 2 years after
initial presentation. Though these tumors are believed to be radiosensitive, the prognosis
depends on the tumor load since most of the patients have multiple metastases by the
time they develop CNS disease.[9]
We believe that this is the first case of cystosarcoma phyllodes with cervical spine
metastasis to be reported. The malignant nature of the disease with multiple metastases
to the lungs is also well demonstrated. The importance of spinal stabilization to
safeguard the quality of life of these patients has to be stressed, since the survival
for patients with spinal metastases is much longer than for those with brain metastases.[9]
