Case history



“A 10 y/o Male with a Left Chest Mass”

California Tumor Tissue Registry’s

Case of the Month

CTTR COTM Vol (9), June, 2006

A ten y/o boy presenting with dyspnea was found to have a 4.5 x 2.5 x 3.0 cm mass involving the left lung. Upon excision, the neoplasm was found to be composed of solid sheets and nests of histiocyte-like cells surrounded by a thick pseudocapsule of inflammatory cells, mainly lymphocytes (Fig. 1). These inflammatory cells included lymphocytes and plasma cells, aggregating around and interspersed between the histiocytes to form follicular patterns (Figs 2,3). Although the lymphoplasmacytic "cuff" mimicked a lymph node, there were no subcapsular sinuses allowing the reaction to be interpreted as a "host response" rather than as a reactive lymph node.

Diagnosis: “Angiomatoid Fibrous Histocytoma”

Wafaa Elatre, M.D., Fouad Abdelhalim, M.D., Donald R. Chase, M.D,

Department of Pathology, Loma Linda University and Medical Center

California Tumor Tissue Registry

Angiomatoid Fibrous Histiocytoma (AFH) is a rare low grade tumor of soft tissue that occurs most commonly in the soft tissues of the extremities or trunk. It usually occurs in superficial tissues and may accompany symptoms such as anemia, pyrexia and weight loss. Pain and tenderness are uncommon [1,2].

AFH is usually a slowly growing multinodular or cystic mass which is typically firm, well-circumscribed, and grey-tan to red brown. It may contain irregular blood-filled spaces that are sometimes seen to be similar to those of a hematoma, hemangioma, or a thrombosed vessel.

Microscopically AFH is characterized by:

1. Irregular solid masses of histiocytes like cells,

2. Cystic areas of hemorrhage, and

3. Chronic inflammation.

The “vascular” spaces are not lined by true endothelial cells but rather by compressed, flattened tumor cells. An inflammatory cell rim around the periphery the tumor is characteristic and consists of a mixture of lymphocytes and plasma cells.

AFH can mimic systemic vasculitis. Cabral emphasizes that AFH needs to be considered in the differential diagnosis of children with vasculitis especially in those who failure to respond to conventional treatment [3]

Originally described as a sarcoma (“angiomatoid MFH)”), these tumors have recently been reclassified by the WHO as being non-malignant, albeit of “intermediate” malignant potential. The tumor is most noteworthy in that it may mimic metastatic carcinoma to a lymph node. The lack of sub-capsular sinuses, however, is most helpful as is the lack of cytokeratin positivity. In fact immunohistochemical studies show the tumor to also be negative for factor VIII-related antigen and CD 34. Smith and associates have reported that the histiocytic marker CD68 was positive in 9 (47.4%) of 19 cases and that desmin was positive in 3 of 6 cases [10]

The lineage of AFH continues to be debated. Enzinger felt that that AFH was a tumor of fibrohistiocytic origin largely based upon the decidedly histiocytic appearance of the cells. Phagocytosis of hemosiderin and positivity for CD68 tends to support his impression. Fletcher has reported that focal positivity for desmin was shown in 5 of 6 cases [6], and he considers this as evidence for some sort of myogenic differentiation in AFH. He has also suggested that angiomatoid myosarcoma might be a more appropriate name for this tumor [11].

So far, local recurrence has developed in 12% of patients and local metastasis 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 is generally considered appropriate treatment for AFH [[12]. However, radiotherapy after post-surgical recurrence has been successfully reported in a patient who had no evidence of tumor recurrence 7 years after adjuvant radiotherapy [12]

In summary, AFH occurs most commonly in the extremities (65%), followed by the trunk (28%) and head and neck (7%) [13] Recently, Fanburg-Smith and Miettinen reported that 66% of 158 cases of angiomatoid "malignant" fibrous histiocytoma occurred in areas of normal lymphoid tissue, and suggested that some of these lesions might arise from lymphoid tissue. The hypothesis of lymphoid origin of these tumors might explain the mediastinal location of our case [14]

References & Suggested Reading:

1. Enzinger FM. Angiomatoid malignant fibrous histiocytoma. A distinct fibrohistiocytic tumor of children and young adults simulating a vascular neoplasm. Cancer;44:2147–5, 1979.

Polyarteritis nodosa as a presenting feature of angiomatoid fibrous histiocytoma D. Hothi, P. A. Brogan, E. Davis, A. Ramsay1 and M. J. Dillon Rheumatology (Oxford). Feb;43(2):245-6, 2004.

2. Cabral DA, Tucker LB. Malignancies in children who initially present with rheumatic complaints. J Pediatr;134:53–7, 1999.

3. Fletcher C.D.M. Angiomatoid "malignant fibrous histiocytoma": An immunohistochemical study indicative of myoid differentiation. Hum Pathol;22:563-568, 1991.

4. 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 28:373-376, 1997.

Angiomatoid fibrous histiocytoma in mediastinum: Shoji Asakura, Noriaki Tezuka, Shuhei Inoue, Naoki Kihara, Shozo Fujino Ann Thorac Surg;72:283-285, 2001.

5. 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,:325-349, 1995.

6. Wegman W., Heitz U. Angiomatoid malignant fibrous histiocytoma: Evidence for the histiocytic origin of tumor cells. Virchows Arch [Pathol Anat];406:59-66, 1985.

7. Grossman L.D., White R.R., IV, Arber D.A. Angiomatoid fibrous histiocytoma. Ann Plast Surg;36:649-651, 1996.

8. 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;15:757-763, 1991.

9. Fletcher C.D.M. Angiomatoid "malignant fibrous histiocytoma": An immunohistochemical study indicative of myoid differentiation. Hum Pathol;22:563-568, 1991.

10. 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;28:373-376, 1997.

11. 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;14:1126-1132, 1990.

12. 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 30:1336-1343, 1999.

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