Journal of Surgical Oncology 32:153-155 (1986)

Retroperitoneal Malignant Fibrous Histiocytoma Presenting With Inferior Vena

Caval Obstruction

DEBA P. SARMA, MD AND THOMAS G. WEILBAECHER, MD

From the Department of Pathology, VA Medical Center and Louisiana, State University Medical School, New Orleans, Louisiana

A 54-year-old-man presented with inferior vena caval obstruction. At autopsy, a retroperitoneal malignant fibrous histiocytoma was noted to have invaded the inferior vena cava. Such a clinical presentation is quite unusual for a retroperitoneal sarcoma.

KEY WORDS: retroperitoneal sarcoma

INTRODUCTION At autopsy, a large, ill-defined tumor mass was noted Malignant fibrous histiocytoma is now considered to to involve the retroperitoneal soft tissue with direct ex-

be the most common soft-tissue i1-31. The tu- tension into the inferior vena cava causing complete oc- clusion from the level of renal veins to the right atrial

i2-41. bong the clinical findings described for junction. Tumor also directly extended into multiple he- retropeitoneal sarcomas, findings due to inferior vena patic veins in the liver. There were several metastatic caval obstruction are uncommon. we are describing a tumor nodules in the liver and a single 7-cm metastatic

3) the tumor cells were arranged in a mixture of pleo- was clarified only at autopsy. morphic and storiform patterns. The neoplastic tissue was composed of spindle fibroblast-like cells, plump fi-

A 54-year-old white man presented with a l-week broblasts, histocytelike cells containing bizarre and pleo- history of cramping abdominal pain, and progressive morphic nuclei, and giant cells. There were numerous swelling of the abdomen and lower extremities. The mitoses. Ultrastructurally, some of the tumor cells con- patient had lost 20 pounds of weight Over 2 months. one tained abundant intracytoplasmic filaments, but no defi- month previously, the patient was treated for deep vein nite suggestion of skeletal muscle, smooth muscle, en- thrombosis involving the left and right ileofemoral veins. dothe1ium7 Or lipblast was noted* The was re-

viewed by Dr. Sharon W. Weiss of the Department of There was marked ascites and bilateral pitting edema Soft Tissue Pathology, Armed Forces Institute of Pathol- of both ankles. Aspirated ascitic fluid revealed a large

number of red cells (40,000/~m3) and white cells (4,0001 OgY9 Washington, D.C. , who concurred with our diag- mm’), but no malignant cells. A CT scan (Fig. 1) of the nosis of malignant fibrous histiocytoma. abdomen revealed massive ascites and a dilated inferior vena cava consistent with intravascular obstruction. A

hepatocellular carcinoma or metastases. Inferior vena

vena cava with good collateral flow. The patient’s clinical course rapidly deteriorated, with

rising serum bilirubin and creatinine levels indicating hepatorenal failure. He expired in 4 weeks.

arises in the retroperitoneum in 9-16% of the

case where the cause of inferior vena caval obstruction in the right adrenal gland- Microscopically (Fig*

CASE REPORT

DISCUSSION

toneum in 9-16% of cases, as noted in several large

intra-abdominal pressure, such as abdominal distention,

Accepted for publication February 7, 1985. Address reprint requests to D. Sarma, M.D., 1601 Perdido Street, New Orleans, LA 70146.

patchy infiltrative process of the liver was suggestive of

cavagram (Fig. 2) revealed complete obstruction of the

Malignant fibrous histiocytoma arises in the retroFri-

sMks i2-41- These patients develop signs Of increased

0 1986 Alan R. Liss, Inc.

W SarmaandWeilbaecber

Fig. 1 . CT scan of the abdomen shows ascites and dilated and obstructed inferior vena cava.

Fig. 2. Inferior vena cavagram shows complete obstruction of the vena cava with good collateral flow.

Malignant Fibrous Histiocytoma 155

Fig. 3. Malignant fibrous histiocytoma showing spindle and plump fibroblastic cells and cells with pleomorphic and bizarre nuclei (H&E XSO).

varicocele, and hernia. Other clinical features include fatigue, weakness, weight loss, fever, and gastrointes- tinal symptoms. Direct invasion and obstruction of the inferior vena cava by such a tumor has not been well documented.

Review of the articles studying retroperitoneal sarco- mas [5-71 reveals that the vast majority of such sarcomas are liposarcomas, fibrosarcomas, and leiomyosarcomas. Malignant fibrous histiocytomas have not been recorded as a common tumor in this location. Since the diagnosis of malignant fibrous histiocytoma had remained contro- versial until the publication of the definitive paper by Weiss and Enzinger in 1978 [2], some of the retroperito- neal sarcomas designated by various names probably represented malignant fibrous histiocytomas.

The various tumors that can obstruct inferior vena cava include primary leiomyosarcomas and angiosarcomas of the vena cava as well as vascular invasion by renal cell carcinomas and hepatocellular carcinomas. The retroper- itoneal sarcomas, specifically malignant fibrous histio- cytomas, may rarely invade inferior vena cava, as demonstrated in our case.

ACKNOWLEDGMENTS We thank Ms. Roey Holliday for secretarial assistance.

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