- Email: [email protected]
Solitary lung metastasis from intracranial hemangiopericytoma 18 years after initial resection
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Case Reports / Journal of Clinical Neuroscience 22 (2015) 1210–1212
differential diagnosis and avoid potential diagnostic and therapeutic pitfalls. Conflicts of Interest/Disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. References [1] Sanchez-Mejia RO, Limbo M, Tihan T, et al. Intracranial dermoid cyst mimicking hemorrhage. Case report and review of the literature. J Neurosurg 2006;105:311–4. [2] Brown JY, Morokoff AP, Mitchell PJ, et al. Unusual imaging appearance of an intracranial dermoid cyst. AJNR Am J Neuroradiol 2001;22:1970–2. [3] Smirniotopoulos JG, Chiechi MV. Teratomas, dermoids, and epidermoids of the head and neck. Radiographics 1995;15:1437–55.
[4] Neugroschl C, David P, Sadeghi N, et al. Unusual CT features of dermoid cyst in the posterior fossa. Eur Radiol 2002;12:2726–9. [5] Bucciero A, Del Basso De Caro ML, Carraturo S, et al. Supratentorial dermoid cysts. Presentation and management of five cases. J Neurosurg Sci 1995;39: 7–11. [6] Lunardi P, Missori P. Supratentorial dermoid cysts. J Neurosurg 1991;75: 262–6. [7] Bizzozero L, Talamonti G, D’Angelo VA, et al. Dermoid cyst mimicking hematoma in the posterior fossa. Clin Neurol Neurosurg 1992;94:61–3. [8] Goh GJ, Page RD, Nixon TE. An unusual CT and MR appearance of a posterior fossa dermoid. Eur J Radiol 1995;20:46–7. [9] Wang X, Yu Y, Zhang X, et al. Unusual imaging appearance of a huge intracranial dermoid cyst located across the anterior and middle skull base. J Neurol Surg A Cent Eur Neurosurg 2013;74:e185–7. [10] Smirniotopoulos JG, Rushing EJ, Mena H. Pineal region masses: differential diagnosis. Radiographics 1992;12:577–96. [11] Truwit CL, Barkovich AJ. Pathogenesis of intracranial lipoma: an MR study in 42 patients. AJNR Am J Neuroradiol 1990;11:665–74.
http://dx.doi.org/10.1016/j.jocn.2015.02.004
Solitary lung metastasis from intracranial hemangiopericytoma 18 years after initial resection Erika E. Doxtader, Sanjay Mukhopadhyay, Richard A. Prayson ⇑ Department of Pathology, Cleveland Clinic, Level 25, 9500 Euclid Avenue, Cleveland, OH 44195, USA
a r t i c l e
i n f o
Article history: Received 9 February 2015 Accepted 14 February 2015
Keywords: Hemangiopericytoma Lung Metastasis
a b s t r a c t We report a 29-year-old woman who presented with severe headache, nausea and vomiting. A lesion was found in the left petrous ridge and near-total resection was performed. Pathologic examination showed anaplastic hemangiopericytoma (World Health Organization Grade III). Hemangiopericytoma is an uncommon mesenchymal tumor that rarely occurs in an intracranial location. Prior studies have reported a surprisingly high rate of late recurrence and extracranial metastases from intracranial hemangiopericytomas, including metastases to the lungs. Resection was followed by external beam radiation. The tumor recurred intracranially 6 and 13 years later and was treated with gamma knife stereotactic radiosurgery. At year 14, she noticed a lump in her neck and underwent parotidectomy for a mucoepidermoid carcinoma. This new diagnosis prompted a staging chest CT scan which showed a 4 mm right upper lobe lung nodule along with additional <5 mm indeterminate nodules. Over the next 3 years, the nodule increased to 8 mm. Wedge biopsy of the lung nodule showed metastatic hemangiopericytoma, histologically similar to the intracranial hemangiopericytoma. Both the primary and the lung metastasis were positive for CD34 and STAT-6. To the best of our knowledge, this is the longest reported interval between a resected intracranial hemangiopericytoma and a histologically confirmed solitary metastasis to the lung. Ó 2015 Elsevier Ltd. All rights reserved.
1. Introduction
2. Case report
Hemangiopericytoma is an uncommon mesenchymal tumor of unclear histogenesis that has been reported in nearly every anatomic site. This tumor only rarely occurs in the central nervous system, usually as a supratentorial, parasagittal mass in adults. In this location, it is particularly aggressive with a high rate of late local recurrence and late metastasis. A remarkable feature is the high frequency of extracranial metastases which usually occur as multiple lesions in the bone, liver and lungs. We report a woman with a solitary lung metastasis from a hemangiopericytoma of the petrous ridge found incidentally 18 years after resection of the primary tumor.
A 29-year-old woman presented in 1996 after an episode of severe headache accompanied by nausea and vomiting. A lesion was found in the left petrous ridge and near-total resection was performed. Histologically, the tumor consisted of a cellular proliferation of plump ovoid-to-spindle cells in a collagenous background (Fig. 1). Scattered branching, staghorn-shaped blood vessels were present throughout. The mitotic count was eight per 10 high power fields. There was no necrosis. The tumor cells showed patchy positivity for CD34 (prediluted; Cell Marque, Rocklin, CA, USA) and STAT-6 (1:200 dilution; Abcam, Cambridge, UK). The features were those of anaplastic World Health Organization Grade III hemangiopericytoma. Resection was followed by 54 Gy of external beam radiation and yearly serial MRI studies which showed no evidence of recurrence for 6 years. However, a new, enhancing 1 cm nodule was found in
⇑ Corresponding author. Tel.: +1 216 444 8805; fax: +1 216 445 3707. E-mail address: [email protected] (R.A. Prayson).
Case Reports / Journal of Clinical Neuroscience 22 (2015) 1210–1212
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Fig. 2. (A) Axial chest CT scan from April 2011 demonstrates a 4 mm nodule in the right upper lobe (arrow), along with scattered sub-centimeter indeterminate nodules. (B) The nodule increased in size to 8 mm (arrow) 3 years later. Fig. 1. (A) Intracranial hemangiopericytoma composed of a haphazard arrangement of ovoid-to-spindle cells in a collagenous background (hematoxylin and eosin; original magnification 200). (B) The tumor cells are positive for CD34 on immunohistochemical staining (original magnification 200).
the tumor bed in 2002. The nodule persisted on repeat imaging in 2003 and was felt to represent a local recurrence. The woman underwent gamma knife radiosurgery (GKRS; 24 Gy) and was followed from 2004–2008 without symptoms and with no concerning features on follow-up MRI. In 2009, she had a second recurrence and again underwent GKRS. In 2010, the woman noticed a lump in her neck. A parotidectomy was performed revealing a high grade mucoepidermoid carcinoma (T1N0M0) with negative margins. She underwent post-operative radiation (60 Gy). The diagnosis of mucoepidermoid carcinoma prompted a staging CT scan of the chest which showed a 4 mm right upper lobe lung nodule along with additional <5 mm indeterminate nodules. Over the next 3 years (2011–2014) the lung nodule increased in size to 8 mm (Fig. 2). A wedge biopsy of the lung nodule was performed showing a cellular mesenchymal neoplasm located entirely within the lung parenchyma with no connection to the pleura (Fig. 3). The tumor consisted of a proliferation of short ovoid-to-spindle cells surrounding prominent staghorn-shaped blood vessels. Ropy collagen was not a prominent feature. The mitotic rate was four per 10 high
power fields. There was no necrosis. The neoplastic cells showed diffuse nuclear positivity for STAT-6 and focal positivity for CD34. The histologic appearance was similar to the intracranial hemangiopericytoma. A diagnosis of metastatic hemangiopericytoma was made. 3. Discussion We considered the possibility that the brain and lung lesions in this woman were unrelated, metachronous tumors. Solitary fibrous tumor, considered by many to be synonymous with hemangiopericytoma, classically occurs in the lung (pleura) and is immunohistochemically identical to hemangiopericytoma. However, most solitary fibrous tumors of the lung are pleura-based (intraparenchymal examples are rare) and contain abundant ropy collagen. Malignant solitary fibrous tumors are usually greater than 10 cm in size. In our patient, the presence of malignant histologic features, the tiny size of the lesion, the morphologic similarities to the intracranial primary, the intraparenchymal location in the lung, and the absence of morphologic findings typical of solitary fibrous tumor suggested that the lung nodule was a metastasis from the intracranial primary hemangiopericytoma.
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Case Reports / Journal of Clinical Neuroscience 22 (2015) 1210–1212
Upon reviewing the literature, we were surprised by the high frequency of extraneural metastases reported in central nervous system hemangiopericytomas. A recent review of the literature that pooled data from 15 studies (523 patients) reported an average rate of extraneural metastasis of 22.94% in central nervous system hemangiopericytomas [1]. Extraneural metastases were less common than local recurrences (51.42%) but more common than neural axis metastases (5.99%). The mean time to extraneural metastasis was 93 months. This rate of extraneural metastasis is especially high when compared to the low frequency of extraneural metastases in other brain tumors. Lung metastases from intracranial hemangiopericytoma have been reported as late as 18 years after the initial cranial surgery [2]. Lung metastasis manifesting as a solitary nodule has also been reported [3]. However, to our knowledge, this is the longest reported interval between a resected intracranial hemangiopericytoma and a histologically confirmed solitary metastasis to the lung. We conclude that the possibility of metastatic hemangiopericytoma should be considered in any patient with a lung nodule and a history of intracranial hemangiopericytoma, regardless of the number of lung nodules or the interval between the resection of the primary tumor and biopsy of the lung lesion. Conflicts of Interest/Disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. References [1] Ghose A, Guha G, Kundu R, et al. CNS hemangiopericytoma. A systematic review of 523 patients. Am J Clin Oncol 2014 [Epub ahead of print]. [2] Soyuer S, Chang EL, Selek U, et al. Intracranial meningeal hemangiopericytoma: the role of radiotherapy. Report of 29 cases and review of the literature. Cancer 2004;100:1491–7. [3] Fountas KN, Kapsalaki E, Kassam M, et al. Management of intracranial meningeal hemangiopericytomas: outcome and experience. Neurosurg Rev 2006;29:145–53. http://dx.doi.org/10.1016/j.jocn.2015.02.004
Fig. 3. (A) Wedge biopsy of the lung nodule shows a cellular intraparenchymal nodule without a connection to the pleura (hematoxylin and eosin; original magnification 40). (B) The tumor is similar to the intracranial hemangiopericytoma (see Fig. 1) and is composed of ovoid-to-spindle cells which focally surround staghorn-type blood vessels (hematoxylin and eosin; original magnification 200). (C) The neoplastic cells show strong nuclear positivity for STAT-6 on immunohistochemistry (original magnification 200).
Case Reports / Journal of Clinical Neuroscience 22 (2015) 1210–1212
differential diagnosis and avoid potential diagnostic and therapeutic pitfalls. Conflicts of Interest/Disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. References [1] Sanchez-Mejia RO, Limbo M, Tihan T, et al. Intracranial dermoid cyst mimicking hemorrhage. Case report and review of the literature. J Neurosurg 2006;105:311–4. [2] Brown JY, Morokoff AP, Mitchell PJ, et al. Unusual imaging appearance of an intracranial dermoid cyst. AJNR Am J Neuroradiol 2001;22:1970–2. [3] Smirniotopoulos JG, Chiechi MV. Teratomas, dermoids, and epidermoids of the head and neck. Radiographics 1995;15:1437–55.
[4] Neugroschl C, David P, Sadeghi N, et al. Unusual CT features of dermoid cyst in the posterior fossa. Eur Radiol 2002;12:2726–9. [5] Bucciero A, Del Basso De Caro ML, Carraturo S, et al. Supratentorial dermoid cysts. Presentation and management of five cases. J Neurosurg Sci 1995;39: 7–11. [6] Lunardi P, Missori P. Supratentorial dermoid cysts. J Neurosurg 1991;75: 262–6. [7] Bizzozero L, Talamonti G, D’Angelo VA, et al. Dermoid cyst mimicking hematoma in the posterior fossa. Clin Neurol Neurosurg 1992;94:61–3. [8] Goh GJ, Page RD, Nixon TE. An unusual CT and MR appearance of a posterior fossa dermoid. Eur J Radiol 1995;20:46–7. [9] Wang X, Yu Y, Zhang X, et al. Unusual imaging appearance of a huge intracranial dermoid cyst located across the anterior and middle skull base. J Neurol Surg A Cent Eur Neurosurg 2013;74:e185–7. [10] Smirniotopoulos JG, Rushing EJ, Mena H. Pineal region masses: differential diagnosis. Radiographics 1992;12:577–96. [11] Truwit CL, Barkovich AJ. Pathogenesis of intracranial lipoma: an MR study in 42 patients. AJNR Am J Neuroradiol 1990;11:665–74.
http://dx.doi.org/10.1016/j.jocn.2015.02.004
Solitary lung metastasis from intracranial hemangiopericytoma 18 years after initial resection Erika E. Doxtader, Sanjay Mukhopadhyay, Richard A. Prayson ⇑ Department of Pathology, Cleveland Clinic, Level 25, 9500 Euclid Avenue, Cleveland, OH 44195, USA
a r t i c l e
i n f o
Article history: Received 9 February 2015 Accepted 14 February 2015
Keywords: Hemangiopericytoma Lung Metastasis
a b s t r a c t We report a 29-year-old woman who presented with severe headache, nausea and vomiting. A lesion was found in the left petrous ridge and near-total resection was performed. Pathologic examination showed anaplastic hemangiopericytoma (World Health Organization Grade III). Hemangiopericytoma is an uncommon mesenchymal tumor that rarely occurs in an intracranial location. Prior studies have reported a surprisingly high rate of late recurrence and extracranial metastases from intracranial hemangiopericytomas, including metastases to the lungs. Resection was followed by external beam radiation. The tumor recurred intracranially 6 and 13 years later and was treated with gamma knife stereotactic radiosurgery. At year 14, she noticed a lump in her neck and underwent parotidectomy for a mucoepidermoid carcinoma. This new diagnosis prompted a staging chest CT scan which showed a 4 mm right upper lobe lung nodule along with additional <5 mm indeterminate nodules. Over the next 3 years, the nodule increased to 8 mm. Wedge biopsy of the lung nodule showed metastatic hemangiopericytoma, histologically similar to the intracranial hemangiopericytoma. Both the primary and the lung metastasis were positive for CD34 and STAT-6. To the best of our knowledge, this is the longest reported interval between a resected intracranial hemangiopericytoma and a histologically confirmed solitary metastasis to the lung. Ó 2015 Elsevier Ltd. All rights reserved.
1. Introduction
2. Case report
Hemangiopericytoma is an uncommon mesenchymal tumor of unclear histogenesis that has been reported in nearly every anatomic site. This tumor only rarely occurs in the central nervous system, usually as a supratentorial, parasagittal mass in adults. In this location, it is particularly aggressive with a high rate of late local recurrence and late metastasis. A remarkable feature is the high frequency of extracranial metastases which usually occur as multiple lesions in the bone, liver and lungs. We report a woman with a solitary lung metastasis from a hemangiopericytoma of the petrous ridge found incidentally 18 years after resection of the primary tumor.
A 29-year-old woman presented in 1996 after an episode of severe headache accompanied by nausea and vomiting. A lesion was found in the left petrous ridge and near-total resection was performed. Histologically, the tumor consisted of a cellular proliferation of plump ovoid-to-spindle cells in a collagenous background (Fig. 1). Scattered branching, staghorn-shaped blood vessels were present throughout. The mitotic count was eight per 10 high power fields. There was no necrosis. The tumor cells showed patchy positivity for CD34 (prediluted; Cell Marque, Rocklin, CA, USA) and STAT-6 (1:200 dilution; Abcam, Cambridge, UK). The features were those of anaplastic World Health Organization Grade III hemangiopericytoma. Resection was followed by 54 Gy of external beam radiation and yearly serial MRI studies which showed no evidence of recurrence for 6 years. However, a new, enhancing 1 cm nodule was found in
⇑ Corresponding author. Tel.: +1 216 444 8805; fax: +1 216 445 3707. E-mail address: [email protected] (R.A. Prayson).
Case Reports / Journal of Clinical Neuroscience 22 (2015) 1210–1212
1211
Fig. 2. (A) Axial chest CT scan from April 2011 demonstrates a 4 mm nodule in the right upper lobe (arrow), along with scattered sub-centimeter indeterminate nodules. (B) The nodule increased in size to 8 mm (arrow) 3 years later. Fig. 1. (A) Intracranial hemangiopericytoma composed of a haphazard arrangement of ovoid-to-spindle cells in a collagenous background (hematoxylin and eosin; original magnification 200). (B) The tumor cells are positive for CD34 on immunohistochemical staining (original magnification 200).
the tumor bed in 2002. The nodule persisted on repeat imaging in 2003 and was felt to represent a local recurrence. The woman underwent gamma knife radiosurgery (GKRS; 24 Gy) and was followed from 2004–2008 without symptoms and with no concerning features on follow-up MRI. In 2009, she had a second recurrence and again underwent GKRS. In 2010, the woman noticed a lump in her neck. A parotidectomy was performed revealing a high grade mucoepidermoid carcinoma (T1N0M0) with negative margins. She underwent post-operative radiation (60 Gy). The diagnosis of mucoepidermoid carcinoma prompted a staging CT scan of the chest which showed a 4 mm right upper lobe lung nodule along with additional <5 mm indeterminate nodules. Over the next 3 years (2011–2014) the lung nodule increased in size to 8 mm (Fig. 2). A wedge biopsy of the lung nodule was performed showing a cellular mesenchymal neoplasm located entirely within the lung parenchyma with no connection to the pleura (Fig. 3). The tumor consisted of a proliferation of short ovoid-to-spindle cells surrounding prominent staghorn-shaped blood vessels. Ropy collagen was not a prominent feature. The mitotic rate was four per 10 high
power fields. There was no necrosis. The neoplastic cells showed diffuse nuclear positivity for STAT-6 and focal positivity for CD34. The histologic appearance was similar to the intracranial hemangiopericytoma. A diagnosis of metastatic hemangiopericytoma was made. 3. Discussion We considered the possibility that the brain and lung lesions in this woman were unrelated, metachronous tumors. Solitary fibrous tumor, considered by many to be synonymous with hemangiopericytoma, classically occurs in the lung (pleura) and is immunohistochemically identical to hemangiopericytoma. However, most solitary fibrous tumors of the lung are pleura-based (intraparenchymal examples are rare) and contain abundant ropy collagen. Malignant solitary fibrous tumors are usually greater than 10 cm in size. In our patient, the presence of malignant histologic features, the tiny size of the lesion, the morphologic similarities to the intracranial primary, the intraparenchymal location in the lung, and the absence of morphologic findings typical of solitary fibrous tumor suggested that the lung nodule was a metastasis from the intracranial primary hemangiopericytoma.
1212
Case Reports / Journal of Clinical Neuroscience 22 (2015) 1210–1212
Upon reviewing the literature, we were surprised by the high frequency of extraneural metastases reported in central nervous system hemangiopericytomas. A recent review of the literature that pooled data from 15 studies (523 patients) reported an average rate of extraneural metastasis of 22.94% in central nervous system hemangiopericytomas [1]. Extraneural metastases were less common than local recurrences (51.42%) but more common than neural axis metastases (5.99%). The mean time to extraneural metastasis was 93 months. This rate of extraneural metastasis is especially high when compared to the low frequency of extraneural metastases in other brain tumors. Lung metastases from intracranial hemangiopericytoma have been reported as late as 18 years after the initial cranial surgery [2]. Lung metastasis manifesting as a solitary nodule has also been reported [3]. However, to our knowledge, this is the longest reported interval between a resected intracranial hemangiopericytoma and a histologically confirmed solitary metastasis to the lung. We conclude that the possibility of metastatic hemangiopericytoma should be considered in any patient with a lung nodule and a history of intracranial hemangiopericytoma, regardless of the number of lung nodules or the interval between the resection of the primary tumor and biopsy of the lung lesion. Conflicts of Interest/Disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. References [1] Ghose A, Guha G, Kundu R, et al. CNS hemangiopericytoma. A systematic review of 523 patients. Am J Clin Oncol 2014 [Epub ahead of print]. [2] Soyuer S, Chang EL, Selek U, et al. Intracranial meningeal hemangiopericytoma: the role of radiotherapy. Report of 29 cases and review of the literature. Cancer 2004;100:1491–7. [3] Fountas KN, Kapsalaki E, Kassam M, et al. Management of intracranial meningeal hemangiopericytomas: outcome and experience. Neurosurg Rev 2006;29:145–53. http://dx.doi.org/10.1016/j.jocn.2015.02.004
Fig. 3. (A) Wedge biopsy of the lung nodule shows a cellular intraparenchymal nodule without a connection to the pleura (hematoxylin and eosin; original magnification 40). (B) The tumor is similar to the intracranial hemangiopericytoma (see Fig. 1) and is composed of ovoid-to-spindle cells which focally surround staghorn-type blood vessels (hematoxylin and eosin; original magnification 200). (C) The neoplastic cells show strong nuclear positivity for STAT-6 on immunohistochemistry (original magnification 200).