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Case Reports / Journal of Clinical Neuroscience 22 (2015) 226–227
References [1] Al Hinai QS, Pawar SJ, Sharma RR, et al. Subgaleal migration of a ventriculoperitoneal shunt. J Clin Neurosci 2006;13:666–9. [2] Kloss BT, Hart DM, Secreti L. Subgaleal coiling of the proximal and distal components of a ventriculoperitoneal shunt. Int J Emerg Med 2012;5:15.
[3] Dominguez CJ, Tyagi A, Hall G, et al. Sub-galeal coiling of the proximal and distal components of a ventriculo-peritoneal shunt. An unusual complication and proposed mechanism. Childs Nerv Syst 2000;16:493–5. [4] Shahsavaran S, Kermani HR, Keikhosravi E, et al. Ventriculoperitoneal shunt migration and coiling: a report of two cases. J Pediatr Neurosci 2012;7:114–6.
http://dx.doi.org/10.1016/j.jocn.2014.09.004
Metastatic malignant phyllodes tumor involving the cerebellum J. Jordi Rowe, Richard A. Prayson ⇑ Department of Pathology, L25, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USA
a r t i c l e
i n f o
Article history: Received 4 September 2014 Accepted 13 September 2014
Keywords: Brain tumor Cystosarcoma phyllodes Metastasis
a b s t r a c t Brain metastases from malignant phyllodes tumors of the breast are a rare occurrence. We report a patient with a malignant phyllodes tumor of the right breast which subsequently metastasized to the right lower lobe of the lung 1 year after initial presentation, and to the right cerebellar hemisphere 2 years after diagnosis of her breast mass. After both chemotherapy and whole brain radiotherapy the patient is tumor free at most recent follow-up, 116 months after the breast tumor diagnosis was made. The literature is briefly reviewed and the differential diagnosis of malignant spindle cell brain tumors is discussed. Ó 2014 Elsevier Ltd. All rights reserved.
1. Introduction Malignant phyllodes accounts for less than 1% of all breast neoplasms [1]. When tumors metastasize, which has been reported in 6.2–25% of cases, the most frequently involved sites include the lung, bone and liver [1,2]. Only rare cases of brain metastasis have been reported in the literature [1,2], often in the setting of other metastases. We report a patient with metastatic malignant phyllodes tumor of the breast involving the lung and cerebellum and discuss histopathologic differential diagnostic considerations for the brain metastasis. 2. Case report The patient is a 44-year-old woman who presented 2 years prior with a right breast mass. An excisional biopsy of the 5.5 4.5 4.5 cm mass diagnosed a malignant phyllodes tumor. Features of phyllodes tumor, including a leaf-like architecture, stromal overgrowth (greater than one 4 field without epithelial elements), stromal atypia, infiltrative borders, and a mitotic count of greater than 10 per 10 high power fields were seen (Fig. 1). She underwent a right modified radical mastectomy with transverse rectus abdominis myocutaneous flap reconstruction. All 16 lymph nodes examined at the time showed no evidence of metastatic disease. One year later, she presented with right upper chest pain and a 5 cm right lower lobe lung mass. She underwent a right videoassisted thoracoscopic resection of the mass and a diagnosis of metastatic malignant phyllodes tumor was rendered. She received chemotherapy (doxorubicin and ifosfamide). Two years after diagnosis of the breast mass, she presented with occipital headaches with no diurnal variation, and blurred vision. CT imaging showed a 3 3 cm right cerebellar hemispheric, homogenously enhancing, mass with no surrounding edema. There was ⇑ Corresponding author. Tel.: +1 216 444 8806; fax: +1 216 445 6967. E-mail address: [email protected] (R.A. Prayson).
compression of the fourth ventricle and tonsillar herniation. There was no evidence of tumor on CT scans of the chest, abdomen and pelvis and a bone scan was negative for metastasis. The cerebellar mass was resected and she underwent a course of whole brain radiation (3750 cGy). On most recent follow-up, 116 months after the brain tumor resection, there is no evidence of tumor. Histologic sections of the brain tumor showed a markedly cellular, spindle cell neoplasm (Fig. 2, 3). There was moderate nuclear pleomorphism and up to 34 mitotic figures per 10 high power fields. Necrosis was not identified. Epithelial or metaplastic elements or heterologous differentiation were not seen. The findings were interpreted as consistent with a metastasis from the phyllodes tumor of the breast. 3. Discussion Most of the literature related to brain metastases from phyllodes tumors has been case reports. One literature review of 11 cases noted that sufficient detail for analysis was present in only six cases [1]. In this review, the mean reported age was 52 years, with the interval between initial diagnosis and brain involvement being 5 years (range 3.5 months to 14 years). Hlavin et al. [1] noted a particularly poor prognosis associated with parenchymal brain lesions (mean survival of 29 days) in contrast to the current case in which the patient is alive, with no evidence of disease, 116 months after initial breast surgery. Sites reported for brain metastases have been variable and have included the skull, dura, brain parenchyma and spinal roots. Most metastases are disseminated hematogenously and are composed of only the sarcomatous component, as in the current patient. The histopathology of the current case raises the difficulty of differential diagnosis of malignant spindle cell lesions in the brain. Either primary or metastatic sarcomas should be considered. Many primary sarcomas arise in the meninges, with spindle cell tumors including fibrosarcomas, malignant fibrous histiocytomas, and leiomyosarcomas having all been rarely described. Many other
Case Reports / Journal of Clinical Neuroscience 22 (2015) 226–227
Fig. 1. Malignant phyllodes tumor of the breast marked by stromal overgrowth and cytologic atypia (hematoxylin and eosin, original magnification 200).
227
Fig. 3. High power view of the metastatic phyllodes tumor in the brain showing numerous mitotic figures in a single high power field (hematoxylin and eosin, original magnification 400).
rhabdomyosarcomas). Hemangiopericytomas, one of the more common primary mesenchymal tumors of the brain, typically demonstrate a staghorn vascular pattern and stain with vascular markers such as CD34. Rare cases of anaplastic or malignant meningioma may be marked by a spindle cell proliferation and high mitotic activity, resembling sarcoma; the clue to diagnosis often rests in identifying lower grade appearing areas. Gliomas, such as gliosarcoma and the rare gliofibroma, may be marked by a pronounced spindle cell component. Gliosarcomas can present a particular challenge; these tumors consist of areas resembling sarcoma admixed with a glioblastoma component. The key to recognizing the lesion lies in recognizing the glioblastoma component of the tumor. Conflicts of Interest/Disclosures
Fig. 2. Low power view of the brain metastasis showing a markedly cellular neoplasm comprised of spindle cells (hematoxylin and eosin, original magnification 100).
sarcoma types readily demonstrate morphologic evidence of differentiation (for example, osteosarcomas, chondrosarcomas, and http://dx.doi.org/10.1016/j.jocn.2014.09.004
The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. References [1] Hlavin ML, Kaminski HJ, Cohen M, et al. Central nervous system complications of cystosarcoma phyllodes. Cancer 1993;72:126–30. [2] Al-Zoubaidi M, Qiu S, Bonnen M, et al. Malignant phyllodes tumor of the breast: a case report. Open Breast Cancer J 2011;3:45–8.
Case Reports / Journal of Clinical Neuroscience 22 (2015) 226–227
References [1] Al Hinai QS, Pawar SJ, Sharma RR, et al. Subgaleal migration of a ventriculoperitoneal shunt. J Clin Neurosci 2006;13:666–9. [2] Kloss BT, Hart DM, Secreti L. Subgaleal coiling of the proximal and distal components of a ventriculoperitoneal shunt. Int J Emerg Med 2012;5:15.
[3] Dominguez CJ, Tyagi A, Hall G, et al. Sub-galeal coiling of the proximal and distal components of a ventriculo-peritoneal shunt. An unusual complication and proposed mechanism. Childs Nerv Syst 2000;16:493–5. [4] Shahsavaran S, Kermani HR, Keikhosravi E, et al. Ventriculoperitoneal shunt migration and coiling: a report of two cases. J Pediatr Neurosci 2012;7:114–6.
http://dx.doi.org/10.1016/j.jocn.2014.09.004
Metastatic malignant phyllodes tumor involving the cerebellum J. Jordi Rowe, Richard A. Prayson ⇑ Department of Pathology, L25, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USA
a r t i c l e
i n f o
Article history: Received 4 September 2014 Accepted 13 September 2014
Keywords: Brain tumor Cystosarcoma phyllodes Metastasis
a b s t r a c t Brain metastases from malignant phyllodes tumors of the breast are a rare occurrence. We report a patient with a malignant phyllodes tumor of the right breast which subsequently metastasized to the right lower lobe of the lung 1 year after initial presentation, and to the right cerebellar hemisphere 2 years after diagnosis of her breast mass. After both chemotherapy and whole brain radiotherapy the patient is tumor free at most recent follow-up, 116 months after the breast tumor diagnosis was made. The literature is briefly reviewed and the differential diagnosis of malignant spindle cell brain tumors is discussed. Ó 2014 Elsevier Ltd. All rights reserved.
1. Introduction Malignant phyllodes accounts for less than 1% of all breast neoplasms [1]. When tumors metastasize, which has been reported in 6.2–25% of cases, the most frequently involved sites include the lung, bone and liver [1,2]. Only rare cases of brain metastasis have been reported in the literature [1,2], often in the setting of other metastases. We report a patient with metastatic malignant phyllodes tumor of the breast involving the lung and cerebellum and discuss histopathologic differential diagnostic considerations for the brain metastasis. 2. Case report The patient is a 44-year-old woman who presented 2 years prior with a right breast mass. An excisional biopsy of the 5.5 4.5 4.5 cm mass diagnosed a malignant phyllodes tumor. Features of phyllodes tumor, including a leaf-like architecture, stromal overgrowth (greater than one 4 field without epithelial elements), stromal atypia, infiltrative borders, and a mitotic count of greater than 10 per 10 high power fields were seen (Fig. 1). She underwent a right modified radical mastectomy with transverse rectus abdominis myocutaneous flap reconstruction. All 16 lymph nodes examined at the time showed no evidence of metastatic disease. One year later, she presented with right upper chest pain and a 5 cm right lower lobe lung mass. She underwent a right videoassisted thoracoscopic resection of the mass and a diagnosis of metastatic malignant phyllodes tumor was rendered. She received chemotherapy (doxorubicin and ifosfamide). Two years after diagnosis of the breast mass, she presented with occipital headaches with no diurnal variation, and blurred vision. CT imaging showed a 3 3 cm right cerebellar hemispheric, homogenously enhancing, mass with no surrounding edema. There was ⇑ Corresponding author. Tel.: +1 216 444 8806; fax: +1 216 445 6967. E-mail address: [email protected] (R.A. Prayson).
compression of the fourth ventricle and tonsillar herniation. There was no evidence of tumor on CT scans of the chest, abdomen and pelvis and a bone scan was negative for metastasis. The cerebellar mass was resected and she underwent a course of whole brain radiation (3750 cGy). On most recent follow-up, 116 months after the brain tumor resection, there is no evidence of tumor. Histologic sections of the brain tumor showed a markedly cellular, spindle cell neoplasm (Fig. 2, 3). There was moderate nuclear pleomorphism and up to 34 mitotic figures per 10 high power fields. Necrosis was not identified. Epithelial or metaplastic elements or heterologous differentiation were not seen. The findings were interpreted as consistent with a metastasis from the phyllodes tumor of the breast. 3. Discussion Most of the literature related to brain metastases from phyllodes tumors has been case reports. One literature review of 11 cases noted that sufficient detail for analysis was present in only six cases [1]. In this review, the mean reported age was 52 years, with the interval between initial diagnosis and brain involvement being 5 years (range 3.5 months to 14 years). Hlavin et al. [1] noted a particularly poor prognosis associated with parenchymal brain lesions (mean survival of 29 days) in contrast to the current case in which the patient is alive, with no evidence of disease, 116 months after initial breast surgery. Sites reported for brain metastases have been variable and have included the skull, dura, brain parenchyma and spinal roots. Most metastases are disseminated hematogenously and are composed of only the sarcomatous component, as in the current patient. The histopathology of the current case raises the difficulty of differential diagnosis of malignant spindle cell lesions in the brain. Either primary or metastatic sarcomas should be considered. Many primary sarcomas arise in the meninges, with spindle cell tumors including fibrosarcomas, malignant fibrous histiocytomas, and leiomyosarcomas having all been rarely described. Many other
Case Reports / Journal of Clinical Neuroscience 22 (2015) 226–227
Fig. 1. Malignant phyllodes tumor of the breast marked by stromal overgrowth and cytologic atypia (hematoxylin and eosin, original magnification 200).
227
Fig. 3. High power view of the metastatic phyllodes tumor in the brain showing numerous mitotic figures in a single high power field (hematoxylin and eosin, original magnification 400).
rhabdomyosarcomas). Hemangiopericytomas, one of the more common primary mesenchymal tumors of the brain, typically demonstrate a staghorn vascular pattern and stain with vascular markers such as CD34. Rare cases of anaplastic or malignant meningioma may be marked by a spindle cell proliferation and high mitotic activity, resembling sarcoma; the clue to diagnosis often rests in identifying lower grade appearing areas. Gliomas, such as gliosarcoma and the rare gliofibroma, may be marked by a pronounced spindle cell component. Gliosarcomas can present a particular challenge; these tumors consist of areas resembling sarcoma admixed with a glioblastoma component. The key to recognizing the lesion lies in recognizing the glioblastoma component of the tumor. Conflicts of Interest/Disclosures
Fig. 2. Low power view of the brain metastasis showing a markedly cellular neoplasm comprised of spindle cells (hematoxylin and eosin, original magnification 100).
sarcoma types readily demonstrate morphologic evidence of differentiation (for example, osteosarcomas, chondrosarcomas, and http://dx.doi.org/10.1016/j.jocn.2014.09.004
The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. References [1] Hlavin ML, Kaminski HJ, Cohen M, et al. Central nervous system complications of cystosarcoma phyllodes. Cancer 1993;72:126–30. [2] Al-Zoubaidi M, Qiu S, Bonnen M, et al. Malignant phyllodes tumor of the breast: a case report. Open Breast Cancer J 2011;3:45–8.
