Ann Thorac Surg 1997;64:841-843
© 1997 The Society of Thoracic Surgeons
Case Report
Primary Pulmonary Malignant Hemangiopericytoma Associated With Coagulopathy
Yu-Chung Wu, MD,
Liang-Shun Wang, MD,
Wenby Chen, MD,
Huei-Jyh Fahn, MD,
Min-Hsiung Huang, MD,
Jacqueline Whang-Peng, PhD
Division of Thoracic Surgery, Department of Surgery, and Department of Pathology, Veterans General Hospital-Taipei, and Institute of Biomedical Sciences, Academia Sinica, Taiwan, Republic of China
Accepted for publication April 18, 1997.
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Abstract
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A 34-year-old woman with a massive pulmonary malignant hemangiopericytoma and coagulopathy as a paraneoplastic syndrome is reported. Although coagulopathy may appear as a paraneoplastic syndrome and cause treatment to be more complicated and difficult, it can also be a useful marker to monitor the results of operation and tumor recurrence. This unusual case shows that primary pulmonary malignant hemangiopericytoma deserves aggressive surgical intervention with complete resection even under the circumstances of coagulation abnormality.
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Introduction
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Hemangiopericytoma is an uncommon, potentially malignant tumor originating from pericytes in the small vessels. It generally occurs in the soft tissue of the thigh, pelvis, and retroperitoneum, but rarely in the lungs [1]. Fewer than 100 patients with pulmonary hemangiopericytoma have been reported in the literature. The tumor has no uniform clinicoroentgenologic features, usually affects older individuals, and mostly presents as an asymptomatic, noncalcified, solitary mass on the chest radiogram. No single clinical or histologic feature including histologic type or DNA ploidy allows prediction of biologic aggressiveness [2]. Malignant hemangiopericytoma is recognized by its increased mitotic rate, tumor size, and foci of hemorrhage and necrosis. Surgical resection of the tumor is the treatment of choice because of its malignant potential, unpredictable behavior, radioresistance, and tendency to local recurrence.
Recently, we successfully treated a young female patient for a giant pulmonary malignant hemangiopericytoma with coagulopathy as a paraneoplastic syndrome. We present the unusual clinical picture and treatment course, and provide our experience in the management of this case.
A 34-year-old young woman presented with right chest discomfort after 38 weeks of pregnancy in February 1992. A chest radiogram was taken and showed a large tumor mass over the right chest (Fig 1
). She was referred to our hospital in April 1992. The laboratory studies revealed 1.2 times prolonged prothrombin time, 1.2 times prolonged activated partial thromboplastin time, decreased platelet count (13,000/µL), decreased fibrinogen level (180 mg/dL), and increased levels of fibrin degradation products (10 µg/mL) and D-dimer (1.0 µg/mL). A video-assisted thoracoscopic biopsy was performed and the result of histopathologic examination implied a spindle cell sarcoma.
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Fig 1. . Malignant hemangiopericytoma. Radiogram of the chest computed tomographic scan showed a huge tumor in the right lower field 1 month before the initial admission.
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Two courses of a combination chemotherapy regimen were administered initially due to the high surgical risk. The combination regimen consisted of cisplatin (30 mg • m-2 • day-1 x 3 days), dacarbazine (200 mg • m-2 • day-1 x 3 days), and ifosfamide (2.4 g/m2 x 1 day). However, coagulopathy were sustained (prothrombin time, 1.3 times; activated partial thromboplastin time, 1.8 times) and worsened while an episode of deep vein thrombosis developed. Ultimately, right thoracotomy with tumor debulking was performed 3 weeks after chemotherapy. Uncontrolled bleeding and active oozing from the chest wall and rough surface of the tumor mass occurred during the dissection, so a total of 20,000 mL of fresh whole blood was eventually transfused for maintenance of blood pressure. The thoracic space was temporarily packed with gauze to control bleeding. On the next day, a second-look operation was carried out and the gauze was removed after the bleeding had been properly controlled. The laboratory coagulation abnormalities recovered on the tenth postoperative day. No more adjuvant therapy was administered.
Unfortunately, 3 months after the initial tumor resection, signs of bleeding tendency such as gum bleeding, cutaneous ecchymosis, hematuria, and hemoptysis were noted. Recurrence of the tumor concomitant with prolonged prothrombin time (1.1 times) and activated partial thromboplastin time (1.3 times), decreased fibrinogen (150 mg/dl), and increased levels of fibrin degradation products (20 µg/mL) and D-dimer (1.0 µg/mL) were also detected. Therefore, the patient underwent right pneumonectomy and total removal of the tumor combined with a resection of the chest wall after preoperative fresh frozen plasma and perioperative fresh whole blood transfusion. The operation was smoothly performed and the postoperative course was uneventful. The signs of bleeding tendency disappeared and all coagulation abnormalities returned to normal soon after the operation. She was regularly followed up at the outpatient clinic and there has been no tumor recurrence up to the present (3
years).
Grossly, the resected specimen was a tan friable tumor with hemorrhage and central necrosis measuring 14 x 12 x 9 cm, and 3.5 cm from the bronchial cut end. Microscopic examination of the resected tumor showed sheets or bundles of tightly packed spindle cells surrounded by thin-walled, endothelium-lined vascular channels; a typical "staghorn" arrangement was observed in most of the tumor part (Fig 2). The tumor cells possessed round, oval, or spindle-shaped nuclei and moderate amounts of cytoplasm; mitotic features was more than 5/10 high-power fields, and necrotic foci were frequently seen. Tumor cell infiltration of the parietal pleura and diaphragm was also noted. Immunohistochemical examinations for desmin, HHF-35 (anti-human muscle actin, Dako), vimentin, S-100, cytokeratin, synaptophysin, endomysial antibody, neuron-specific enolase, and HMB-45 (anti-human melanoma, Dako) were performed and all revealed negative reactions. The final pathologic diagnosis was malignant hemangiopericytoma of the right lower lobe of the lungs with pleural and diaphragm invasion.
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Fig 2. . Malignant hemangiopericytoma. Histologically, the resected tumor was a sheet of tightly packed spindle cells surrounded by thin-walled, endothelium-lined vascular channels with staghorn configuration. (Hematoxylin and eosin, x10 before 30% reduction.)
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Comment
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Although hemangiopericytoma usually presents as an asymptomatic mass lesion, paraneoplastic syndromes have been reported on rare occasions, including hypoglycemia [3], hypertension [4], and pulmonary osteoarthropathy [5]. Our patient had an association with coagulopathy. In the present case, the coagulopathy was subsymptomatic in the initial diagnosis and was detectable only by laboratory evaluations (prolonged prothrombin time and activated partial thromboplastin time, depressed platelet and fibrinogen levels, and elevated levels of fibrin degradation products and D-dimer). The chemotherapy failed to diminish the tumor growth, which became symptomatic and initiated a hypercoagulable status (deep vein thrombosis). Similarly, when the remaining tumor mass grew during the follow-up period after the initial debulking operation, the same laboratory abnormalities appeared again and an episode of acute disseminated intravascular coagulation followed. So, like other solid tumor, the most possible pathogenetic mechanism for a hemangiopericytoma associated with coagulopathy may be the tumor burden, which markedly alters the coagulation pathways and initiates coagulopathy. In this case, these clinical and laboratory coagulation abnormalities apparently correlate with tumor burden, which makes them useful for predicting the effects of surgical resection and tumor recurrence.
Depressed or low-normal fibrinogen levels and significant titers of fibrin degradation products imply the presence of chronic coagulopathy, which would raise the risk of perioperative and postoperative bleeding. Furthermore, malignant hemangiopericytomas are highly vascular tumors that will be associated with life-threatening hemorrhage if dissection is carried through the tumor. In our case, it became a disaster when both situations were encountered in the initial debulking operation. Eventually, complete resection of tumor mass led to the successful resolution of paraneoplastic coagulopathy and avoidance of inappropriate dissection.
Reviewing the literature, we find that complete surgical resection for pulmonary malignant hemangiopericytoma is the treatment of choice. Some authors have proposed an innovative approach of treatment, which includes complete surgical resection along with intraoperative and postoperative radiotherapy [6], whereas others have recommended that radiotherapy be used palliatively for local tumor recurrence or superior vena cava obstruction [7, 8]. Chemotherapy has been reserved for metastatic lesions or those in which surgical resection is not feasible. However, our results have demonstrated that a combination chemotherapy regimen failed to diminish tumor growth and associated coagulopathy. Thus, for patients with a huge pulmonary malignant hemangiopericytoma, particularly for those with coagulopathy as a neoplastic syndrome, complete surgical excision of the tumor, as early as possible, is preferred.
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Footnotes
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Address reprint requests to Dr Wang, Division of Thoracic Surgery, Department of Surgery, Veterans General Hospital Taipei, No. 201, Sec. 2, Shih-pai Road, Shih-pai, Taipei, Taiwan, 11217, Republic of China.
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References
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Abstract
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