Ann Thorac Surg 2001;72:283-285
© 2001 The Society of Thoracic Surgeons
Case report
Angiomatoid fibrous histiocytoma in mediastinum
Shoji Asakura, MDa,
Noriaki Tezuka, MDa,
Shuhei Inoue, MDa,
Naoki Kihara, MDa,
Shozo Fujino, MDa
a Second Department of Surgery, Shiga University of Medical Science, Otsu, Shiga, Japan
Accepted for publication May 22, 2000.
Address reprints requests to Dr Asakura, Department of Thoracic Surgery, National Minami-Kyoto Hospital, Nakaashihara 11, Joyo, Kyoto, 610-0113 Japan
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Abstract
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Angiomatoid fibrous histiocytoma (AFH) is a rare tumor of soft tissue with low-grade malignancy that occurs most commonly in the soft tissues of the extremities or trunk. We present a case of AFH of the mediastinum, which is a very unusual site for this tumor. The patient has survived with no recurrence of the disease for 60 months after surgery and adjuvant radiotherapy.
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Introduction
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Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor with low-grade malignancy, occurring chiefly in children or young adults [1, 2]. Most of the tumors develop as a slow-growing mass of soft tissues of the extremities. We present a case of AFH occurring on the mediastinum, which is a very unusual site for AFH.
A 39-year-old man presented with an abnormal shadow on a chest roentgenogram film from a routine medical examination. He was admitted to our hospital on December 13, 1994. He had no clinical symptoms and findings on physical examination were unremarkable. A 4.5 x 3 cm well-demarcated mass shadow was detected adjacent to the left main pulmonary artery (PA) and descending aorta on chest computed tomography (CT), and the mass revealed heterogenous intense enhancement (Fig 1). Differential diagnosis was neurogenic tumor, hemangioma, or other malignant mediastinal tumor.
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Fig 1. Computed tomographic scan demonstrating the left mediastinal mass with heterogenous intense enhancement.
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Thoracotomy with a left posterior incision was performed on December 22, 1994. A 4.7 x 3.5 x 2.5 cm dark red well-circumscribed extrapleural mass was located adjacent to the left main PA, descending aorta, and left vagal nerve. The tumor did not invade the neighboring organs, and it was easily dissociated from the PA and vagal nerve. The tumor bled easily and several feeding arteries from the aorta were identified. It was removed by blunt dissection and the pathologic diagnosis from frozen section was sarcoma of unknown origin. We excised 4 cm of the vagal nerve and sampled mediastinal lymph nodes. The first diagnosis from evaluation of permanent sections was epithelioid hemangioendothelioma. Lymph node metastases were not detected. Radiation therapy of 50 Gy was given to the middle and upper mediastinum postoperatively.
The tumor was sent to Dr Thomas V. Colby, Mayo Clinic Scottsdale and Dr Antonio G. Nascimento, Mayo Clinic Rochester, for pathologic consultation. The immunohistochemical stains performed at the Mayo Clinic gave the following results: keratin = negative, desmin = negative, Factor VIII-related antigen = negative, CD31 = negative, CD34 = negative, Vimentin = positive and CD68 = positive. Based on the findings of H-E stains (Fig 2A, B) and immunohistochemical stains, this tumor was interpreted as angiomatoid fibrous histiocytoma.
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Fig 2. (A) Histologic finding demonstrating hemorrhagic spaces that simulate vascular spaces. (Hematoxylin and eosin stain; x40.) (B) Vascular spaces were lined by histiocyte-like cells characteristic of AFH (x400).
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The patient has had no local recurrence and no distant metastasis for 5 years after the operation.
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Comment
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The soft tissue tumor previously termed angiomatoid malignant fibrous histiocytoma was recently renamed angiomatoid fibrous histiocytoma (AFH), because of its low-grade malignancy [2]. AFH differs from malignant fibrous histiocytoma; AFH occurs primarily in individuals under the age of 40 years and has a relatively good prognosis [2].
Three microscopic findings are characteristic of AFH: (1) solid arrays or nests of histiocyte-like cells, (2) hemorrhagic cyst-like spaces, and (3) aggregates of chronic inflammatory cells. Multifocal recent and old hemorrhages are a striking feature in this tumor. These spaces resemble vascular spaces, but they are not lined by endothelium. Inflammatory cells present include lymphocytes and plasma cells. A thick pseudocapsule and occasional germinal centers give this tumor a resemblance to a lymph node.
Immunohistochemical studies are helpful in differential diagnosis of AFH [3]. AFH is characteristically negative for factor VIII-related antigen and CD 34, which are often positive in vascular tumors [4]. Smith and associates reported that the histiocytic marker CD68 was positive in 9 (47.4%) of 19 cases and desmin was positive in 3 of 6 cases [5]. Enzinger presumed that AFH was a tumor of fibrohistiocytic origin according to the decidedly histiocytic appearance of the cells. Phagocytosis of hemosiderin and positivity for CD68 supports this conclusion. In our case, CD68 was positive. Fletcher has reported that focal positivity for desmin was shown in 5 of 6 cases [6]. He considered that this result provided good evidence for some sort of myogenic differentiation in AFH and suggested that angiomatoid myosarcoma might be a more appropriate name for this tumor [6]. The results of immunohistochemical study in our case were consistent with histiocytic differentiation, and desmin stain was negative.
In a study of more than 100 patients with AFH, local recurrence developed in 12% of patients and local metastasis developed in 4%. Only one patient developed distant metastasis, and died from disease. Recurrence and metastasis were related to infiltrative tumor margins and deep location. Complete surgical excision without adjuvant therapy was considered appropriate treatment for AFH [2]. However, radiotherapy after postsurgical recurrences was reported to be successful in a patient who had no evidence of tumor recurrence 7 years after adjuvant radiotherapy [7].
AFH occurs most commonly on the extremities (65%), followed by the trunk (28%) and head and neck (7%) [8]. The mediastinum is a very unusual site for AFH, with no cases reported in the literature. Recently, Fanburg-Smith and Miettinen reported that 66% of 158 cases of angiomatoid "malignant" fibrous histiocytoma occurred in areas of normal lymphoid tissue, and they suggested that some of these lesions might arise from lymphoid tissue. The hypothesis of lymph node origin of these tumors might explain the mediastinal location of our case [9].
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Acknowledgments
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We acknowledge Thomas V. Colby, MD, Mayo Clinc Scottsdale, and Antonio G. Nascimento, MD, Mayo Clinic Rochester, for analysis of the pathology in this patient.
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References
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Enzinger F.M. Angiomatoid malignant fibrous histiocytoma: a distinct fibrohistiocytic tumor of children and young adults simulating a vascular neoplasm. Cancer 1979;44:2147-2157.[Medline]
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Enzinger F.M., Weiss S.W. Fibrohistiocytic tumors of intermediate malignancy. In: Enzinger F.M., Weiss S.W., eds. Soft tissue tumors. St. Louis: Mosby-Year Book, 1995:325-349.
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Wegman W., Heitz U. Angiomatoid malignant fibrous histiocytoma: Evidence for the histiocytic origin of tumor cells. Virchows Arch [Pathol Anat] 1985;406:59-66.
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Grossman L.D., White R.R., IV, Arber D.A. Angiomatoid fibrous histiocytoma. Ann Plast Surg 1996;36:649-651.[Medline]
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Smith M.E.F., Costa M.J., Weiss S.W. Evaluation of CD68 and other histiocytic antigens in angiomatoid malignant fibrous histiocytoma. Am J Surg Pathol 1991;15:757-763.[Medline]
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Fletcher C.D.M. Angiomatoid "malignant fibrous histiocytoma": An immunohistochemical study indicative of myoid differentiation. Hum Pathol 1991;22:563-568.[Medline]
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Costa M.A., Silva I., Carvalhido L., et al. Angiomatoid fibrous histiocytoma of the arm treated by radiotherapy for local recurrence—case report. Med Pediatr Oncol 1997;28:373-376.[Medline]
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Costa M.J., Weiss S.W. Angiomatoid malignant fibrous histiocytoma: A follow-up study of 108 cases with evaluation of possible histologic predictors of outcome. Am J Surg Pathol 1990;14:1126-1132.[Medline]
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Fanburg-Smith J.C., Miettinen M. Angiomatoid "malignant" fibrous histiocytoma: a clinicopathologic study of 158 cases and further exploration of the myoid phenotype. Hum Pathol 1999;30:1336-1343.[Medline]
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Abstract
Introduction
