Ann Thorac Surg 2004;77:2210-2213
© 2004 The Society of Thoracic Surgeons
Case report
Resection of primary pleural pedunculate hemangiopericytoma
Makoto Takahama, MD, PhD*a,
Keiji Kushibe, MDa,
Michitaka Kimura, MDc,
Takeshi Kawaguchi, MDa,
Yuko Nishimoto, MDb,
Shigeki Taniguchi, MDa
a Department of Thoracic and Cardiovascular Surgery, Kashihara, Nara, Japan
b Department of Radiology, Nara Medical University, Kashihara, Nara, Japan
c Department of Thoracic Surgery, Nara Prefectural Hospital, Nara, Japan
Accepted for publication June 6, 2003.
* Address reprint requests to Dr Takahama, Department of Thoracic and Cardiovascular Surgery, Nara Medical University, 840 Shijo-cho, Kashihara, Nara 634-8522, Japan
e-mail: tmakoto{at}naramed-u.ac.jp
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Abstract
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We are reporting an uncommon clinical case of primary pleural hemangiopericytoma, of which only a few reports have been published in the literature to date. A 40-year-old woman was treated by complete surgical resection of the tumor. No recurrence or metastasis was detected during follow-up at 12 months after the resection. The postoperative pathologic examination confirmed the diagnosis of primary pleural hemangiopericytoma. Details of the clinical and radiographic feature are presented.
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Introduction
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Hemangiopericytoma is a rare mesenchymal neoplasm, accounting for about 1% of vascular tumors [1]. Hemangiopericytoma was first reported by Stout and Murray [2] in 1942, and it generally occurs in the soft tissue of the thigh, pelvis, and retroperitoneum, but rarely in the lung, pleura, or chest wall [3]. We describe herein a case of pleural pedunculate hemangiopericytoma that was resected under video-assisted thoracoscopic surgery.
A 40-year-old woman was referred to our institution with an abnormal shadow in the lower lobe of the right lung, which had been first noted on a routine chest roentgenogram on May 2001. She had no symptoms related to the mass and had no history of smoking or serious illness. Physical examination was essentially within normal limits, and laboratory studies revealed chronic hypochromic anemia, a hemoglobin of 7.9 g/dL, and a hematocrit of 27.8%. The tumor markers alpha-fetoprotein, sialyl Lewis X-1, carcinoembryonic antigen, neuron-specific enolase, and squamous cell carcinoma antigen were within normal limits. A computed tomographic scan of the chest revealed a large solid tumorous mass measuring 12 x 7 x 3 cm located in the right costodiaphragmatic recess, and it showed a lack of homogeneity with the main peripheral enhancement (Fig 1A).
The shadow of the mass moved with changes in the patient's position, indicating that this was a pedunculated tumor (Fig 1B). Magnetic resonance scanning showed that the tumor appeared inhomogeneous, with hypo-intense and hyper-intense signals on T1-weighed images and an inhomogeneous pattern with predominant hyper-intensity on T2-weighed images with a smooth outline. Abdominal computed tomography, gallium-67 scintigraphy, thallium-201 scintigraphy, and head computed tomography were also performed, but no abnormalities were found. A transluminal computed tomographic-guided fine needle aspiration biopsy indicated a probable mesenchymal neoplasm. At preoperative staging, no metastatic lesions were found and a surgical approach was suggested.
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Fig 1. (A and B) Preoperative enhanced computed tomographic scan demonstrated a hypo-intense tumor mass located in the right costodiaphragmatic recess with a smooth outline. The tumor showed contrast enhancement of its margin and compressed adjacent lung tissue. (C and D) Preoperative chest roentgenograms revealed that the tumor changed its location depending on the patient's position. (C) Upright position. (D) Right decubitus position.
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We initially inserted a thoracoscope into the right pleural cavity. The tumor was located in the periphery of the right lower lobe and the overlying pleura was not puckered. The tumor was pedunculated and compressed the surrounding lung parenchyma (Fig 2).
The peduncle of this tumor originated from the visceral pleura of the right lower lobe (segment nine), and its diameter was approximately 15 mm. Wedge resection of the right lower lobe with a sufficient surgical free margin, which was approximately 3 cm away from the base of the peduncle, was performed using a stapler. A small thoracotomy was added to extract the large tumor. The mass measured 12 x 8 x4 cm and weighed 450 g; grossly it was firm and appeared to be encapsulated without invasion into the surrounding pulmonary tissue. Frozen section examination during the surgical procedure indicated a benign spindle cell tumor and wedge resection was considered appropriate for this lesion. The postoperative histologic examination confirmed the completeness of resection. Microscopically, there were numerous capillaries lined with flattened endothelium and surrounded by sheets of tumor cells resembling pericytes. The tumor cells did not invade the pulmonary tissue and had round-to-oval nuclei and moderate amounts of cytoplasm with ill-defined borders; no mitosis was detected in any tumor cells (Fig 3).
Silver impregnation clearly revealed proliferating cells to be present outside the blood vessels. Immunohistochemically, the tumor cells showed positive staining for vimentin, but were negative for desmins, smooth muscle actin, CD34, epithelial membrane antigen, keratin, and S-100. A pathologic diagnosis of primary pleural hemangiopericytoma was made from these findings. The patient was discharged with no supportive therapy 27 days after the operation. To date, 12 months after the operation, the patient remains well with no evidence of recurrence or metastasis.
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Fig 2. The peduncle of this tumor originated from the visceral pleura of the right lower lobe and its diameter was approximately 15 mm. The tumor compressed the surrounding lung parenchyma. (L = right lower lobe; M = right middle lobe; P = peduncle of the tumor; T = tumor; U = right upper lobe.)
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Fig 3. The resected tumor was rich in capillaries and small staghorn-shaped vessels that were haphazardly bounded and surrounded by ovoid and short spindle cells. No mitosis was confirmed in any field. (Hematoxylin & eosin; x200, original magnification.)
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Comment
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Intrathoracic hemangiopericytoma arises from pericytes that surround the basement membrane of capillaries and small venules within the lung parenchyma [4]. This is a slow-growing tumor with high local recurrence, and the long-term prognosis is poor because of its propensity to recur. Our case was an intrathoracic extrapulmonary hemangiopericytoma, which is extremely rare. Only a few isolated case reports are available in the literature, whereas the intrapulmonary variety of the same tumor is relatively more common [3–5].
Hemangiopericytomas are composed of closely-packed spindle cells and prominent vascular channels. The histologic differential diagnosis includes many mesenchymal tumors, such as the solitary fibrous tumor and the synovial sarcoma [4]. Immunohistochemical studies are useful in making the definitive diagnosis of a hemangiopericytoma. In the present case, the final diagnosis was confirmed by the results of immunohistochemical studies, which were positive for vimentin but negative for CD34, epithelial membrane antigen, keratin, and S-100.
Surgical radical excision is the treatment of choice for hemangiopericytomas [4]. Hansen and colleagues [6] stated that it was necessary to consider all hemangiopericytomas as malignant and perform extended surgery; however, the criteria for determining the area of resection has not been established yet. During the resection it is important to look for invasion of tumor cells into the normal surrounding lung tissue and to avoid intrathoracic spread of tumor cells by manual examination [7]. Although adjuvant chemotherapy or radiotherapy may be indicated because of the high risk of recurrence and potential malignancy, it is generally considered to be largely ineffective [4]. Jalal and Jeyasingham [8] reported that preoperative radiotherapy of large hemangiopericytomas on the chest wall significantly reduced the vascularity of the tumor and made complete resection much easier. Due to the small numbers of cases, these preoperative and postoperative treatments have not been appropriately evaluated, and therefore, complete resection is currently the most reliable treatment for hemangiopericytomas. In the present case, in the light of the preoperative radiographic findings, we anticipated that this tumor would be pedunculated and easily resected. Therefore, we initially used the thoracoscopy to resect the tumor with the sufficient surgical margin. Because wide surgical resection is required to treatment hemangiopericytoma, if necessary, a sufficient thoracotomy should be added to prevent the intrathoracic spread of tumor cells.
The 5-year survival of patients with hemangiopericytomas originating in any organ has been reported to be approximately 85% [4], whereas the survival of patients with a tumor of pulmonary origin is comparatively low (approximately 30% to 35%) [6]. It has been demonstrated that recurrent disease usually occurs within 2 years after initial treatment, and recurrences are commonly found in the thorax, either in the pulmonary parenchyma or in the pleura. Thus, long-term follow-up is required.
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References
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- Hart L.L., Weinberg J.B. Metastatic hemangiopericytoma with prolonged survival. Cancer 1987;60:916-920.[Medline]
- Stout A.P., Murray M.R. Hemangiopericytoma: a vascular tumor featuring Zimmerman's pericytes. Ann Surg 1942;116:26-33.[Medline]
- Kusumoto S., Nakamura R., Mizoguchi N., Ono S., Watanabe K. Primary intrathoracic extrapulmonary hemangiopericytoma: CT and MR findings. Clin Imag 1997;21:51-53.[Medline]
- Espat N.J., Lewis J.J., Leung D., et al. Conventional hemangiopericytoma: modern analysis of outcome. Cancer 2002;95:1746-1751.[Medline]
- Cheng Y.L., Yu C.P., Hsu S.H., Lee S.C. Hemangiopericytoma of the pleura causing massive hemothorax. J Formos Med Assoc 2000;99:428-430.[Medline]
- Hansen C.P., Francis D., Bertelsen S. Primary hemangiopericytoma of the lung: case report. Scand J Thorac Cardiovasc Surg 1990;24:89-92.[Medline]
- Van Damme H., Dekoster G., Creemers E., Hermans G., Limet R. Primary pulmonary hemangiopericytoma: early local recurrence after perioperative rupture of the giant tumor mass (two cases). Surgery 1990;108:105-109.[Medline]
- Jalal A., Jeyasingham K. Massive intrathoracic extrapleural haemangiopericytoma: deployment of radiotherapy to reduce vascularity. Eur J Cardiothorac Surg 1999;16:378-381.[Abstract/Free Full Text]
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Abstract
Introduction
