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|LETTERS TO EDITOR
|Year : 2015 | Volume
| Issue : 6 | Page : 963-965
Malignant phyllodes tumor of the breast with isolated intracranial metastases: A report
Sukhdeep Singh Jhawar1, Shekhar Upadhyay2, Amit Mahajan2, Sarvpreet S Grewal1
1 Department of Neurosurgery, Christian Medical College and Hospital, Ludhiana, Punjab, India
2 Department of Surgery, Christian Medical College and Hospital, Ludhiana, Punjab, India
|Date of Web Publication||20-Nov-2015|
Sukhdeep Singh Jhawar
Department of Neurosurgery, Christian Medical College and Hospital, Ludhiana, Punjab
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Jhawar SS, Upadhyay S, Mahajan A, Grewal SS. Malignant phyllodes tumor of the breast with isolated intracranial metastases: A report. Neurol India 2015;63:963-5
Phyllodes tumor (PT), first described by Johannes Muller in 1838, is a rare fibroepithelial neoplasm of the breast having an unpredictable clinical course. Metastases in PT may occur either at the time of primary presentation or may be as late as 12 years after the diagnosis., The incidence of distant metastases in patients with PT has ranged from 25% to 40% according to various studies.,,, The lungs, pleura, and bone have been the most common sites of metastatic involvement. Intracranial metastases from this tumor are, however, extremely rare. Local recurrence or metastases to other organs is almost invariably present by the time the central nervous system (CNS) is involved by the PT. In this report, we describe a rare case of malignant PT with isolated CNS involvement without any evidence of systemic dissemination elsewhere at the time of presentation.
A 63-year-old lady presented with a lump in her left breast, which she first noticed around 3 years ago. For 4–5 months prior to her presentation, she noticed a rapid increase in the size of the lump. On examination, there was a large lobulated mass involving the left breast. The overlying skin was stretched and shiny. The patient underwent a simple mastectomy followed by split thickness skin grafting of the defect. Grossly, the specimen was measuring 36 × 26 × 20 cm [Figure 1]. Serial cut sections showed large nodular solid and cystic areas occupying whole of the mastectomy specimen. The microscopic examination showed a biphasic tumor comprising both epithelial and stromal elements. There was an overgrowth of the stromal elements with heterogeneous round- to spindle-shaped cells [Figure 2]a. Thus, a diagnosis of malignant PT was made. An week after surgery, the patient started complaining of headache, associated with vomitings. Magnetic resonance imaging of the brain showed two well-defined, dural-based, ring-enhancing lesions involving the right occipital lobe and ipsilateral right cerebellar hemisphere [Figure 3]. A thorough systemic workup did not reveal any other organ involvement. The patient underwent simultaneous suboccipital craniotomy and right occipital craniotomy followed by a gross total excision of both the lesions. Histopathological examination of the excised cranial mass confirmed the diagnosis of metastases from malignant PT [Figure 2]b. The patient was doing fine after excision of the metastases without any evidence of recurrence at a 6-month follow-up.
|Figure 1: Gross total excision of the breast lump involving whole of breast with nipple|
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|Figure 2: Photomicrograph of both lesions. (a) Malignant phylloides tumor in the breast showing benign glands and pleomorphic spindle cell stroma (H and E, ×200). (b) Cerebellar metastases showing pleomorphic spindle cells (H and E, ×200)|
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|Figure 3: Magnetic resonance image of brain. (a) Contrast-enhanced axial image showing a ring-enhancing, dural-based lesion involving the right cerebellar hemisphere. (b) Contrast-enhanced axial image showing the dural-based enhancing lesion involving right occipital lobe. (c) Contrast-enhanced sagittal image showing both the lesions. (d) Contrast-enhanced coronal image showing both supra- and infratentorial lesions in a single frame|
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PT is an uncommon tumor of the breast, accounting for only 0.4%–1% of all breast tumors. It is a fibroepithelial breast tumor arising from intralobular stroma of the breast and is morphologically characterized by stromal overgrowth with an epithelial covering that often resembles a leaf-like structure. The terms "benign" and "malignant" have been traditionally used to categorize PT in an attempt to better predict prognosis; however, there are reported cases of even benign PT that can metastasize. Furthermore, malignant PT metastasize in only a small proportion of cases, thus putting a question mark on these tumors being categorized into these two types. Only invasive local recurrence or a documented metastases are the unequivocal proofs of malignancy in PT. However, upon histopathologic analysis, malignant phylloides tumors typically exhibit nuclear atypia, a higher degree of stromal cellularity and epithelial overgrowth, high mitotic activity, and infiltrative borders.
Distant metastases from PT is not very common. However, those who develop distant disease share a few aggressive pathologic features such as large tumor size, infiltrative tumour borders, marked stromal overgrowth with increased cellularity, high mitotic rate, and necrosis. Distant metastases usually occur through the hematogeneous route and the most frequent sites of metastases are the lungs, soft tissue, bone, and pleura.,, In a comprehensive review, Kessinger et al., analyzed distant spread of 67 patients with metastatic PT. The most common sites of metastases were the lungs (in 66% of the patients), bones (28%), heart (9%), and liver (6%). CNS involvement by PT is very rare and usually occurs late in the course of the disease. In almost all the previously reported cases, except one, CNS metastases occurred in the setting of multiorgan involvement. , CNS metastases from PT tends to be dural based like most other intracranial metastases., In the present case also, both the lesions were adherent to the overlying dura.
In the treatment of PT, the effectiveness of radiation therapy, chemotherapy, and axillary node excision is unclear. Local control of PT can be achieved by means of surgical excision with at least a 1 cm clear margin. Brain metastases in PT usually occur late in the course of the disease and respond poorly to treatment (mean survival of 29 days, in contrast to 24 months at other sites). Isolated involvement of the central nervous system by PT is very rare. Hence, whenever encountered, a histological diagnosis is always warranted. If possible, a total excision is desirable as these are unresponsive to adjuvant therapies like radiation, chemotherapy or hormonal manipulations. Patients with malignant PT having an isolated CNS involvement without metastasis elsewhere in the body may have a favourable outcome following surgery, as seen in the present case.
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[Figure 1], [Figure 2], [Figure 3]