Case Report Urol Int 1992;48:457-459

G. Ziincl B. Kreienbiihl D. Gobet D. Hauri

Retroperitoneal Malignant Fibrous Histiocytoma

Department of Urology, University Hospital of Zürich. Switzerland

Report of Two Cases

Key Words Malignant fibrous histiocytoma Retropcritoneum Sarcoma

Abstract We present 2 cases of malignant fibrous histiocytoma (MFH) of the retroperitoncum. Only 12-14% of all MFH occur in the retropcritoneum. Both patients were operated with the diagnosis of a kidney tumor. The surgical exposure showed a tumor arising from the retropcritoneum, infiltrating or surrounding the kidney. We would like to emphasize that large tumors of the retroperitoneum which resemble kidney tumors can also arise from mesen­ chymal tissue.

Introduction Malignant fibrous histiocytoma (MFH) is a primitive mesenchymal tumor first described by O'Brien and Stout [1] in 1964. It is regarded as the most common soft tissue sarcoma of late adult life [2]. About 70% of the tumors arise from the soft tissue of the extremities and only 1214% of the tumors occur in the retroperitoneum [2, 3], The clinical characteristics of this tumor consist of an abdominal mass, tendency to local recurrence, paraneo­ plastic syndrome and a poor prognosis [4], We present 2 cases of retroperitoneal MFH.

Case Reports

abdomen showed a large tumor in the upper left quadrant of the abdomen, resembling a large kidney tumor (fig. 1). The tumor appeared inhomogeneous with areas of hematomas. The patient underwent surgery'. Surgical exposure showed a large tumor, arising from the left retroperitoneum infiltrating the left psoas, diaphragm, stomach and surrounding the left kidney. Only an incomplete tumor extirpation with nephrectomy on the left side could be done. The histology showed a malignant fibrous histiocytoma from the pleomorphic-storiform type. The postoperative course was complicated by respiratory insufficiency due to pneumonia. Three months after the operation the patient underwent a second operation to remove the rest of the tumor. A wide surgical excision with resection of the diaphragm, splenectomy, caudal pancreatectomy, removal of the jejunum, colon resection on the left side and tumorectomy in the retroperitoneum were performed. Again the postoperative course was complicated by a necrosis of the colon sigmoideum. A resection of the sigma and a terminal colostomy had to be done. Five weeks after the third operation the patient is doing well and an adjuvant chemotherapy is planned.

Case I

Accepted: June 27. 1991

Case 2

A 53-ycar-old white man was seen in the emergency room with nausea, vomiting and acute abdominal pain in the upper left quad­ rant. There was a 5-month history of fatigue, perspiration, backache and weight loss. There was a palpable mass in the left upper quadrant of the abdomen. A CAT scan of the abdomen showed a 22 X 15 X

Beat Kreienbiihl. MI) Department o f Urology University Hospital of Zurich CH-8091 Zürich (Switzerland)

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A 56-year-old white man was seen at the emergency station with pain in his upper left quadrant of the abdomen, which began 3 h ear­ lier. There was no history of trauma. An ECG was performed to rule out myocardial infarction. The past medical and surgical history was uneventful. On physical exam there was a large tender mass in the upper left quadrant of the abdomen. Sonography and CAT of the

Fig. 1. Large tumor in the upper left quadrant of the abdomen resembling a large kidney tumor (case 1).

Fig. 2. A 22 X 15 X 20 cm inhomogeneous retroperitoneal tumor with displacement of the stomach (case 2).

20 cm retroperitoneal tumor originating most likely from the left kid­ ney with displacement of the stomach (fig. 2) and invasion to the left renal and inferior caval vein (fig. 3). A wide tumorectomy with nephrectomy on the left side and splenectomy were performed. The tumor was penetrating the omental bursa and the posterior wall of stomach. Only an incomplete tumor extirpation could be performed. The tumor thrombus in the inferior caval vein was removed through the left kidney vein by a Fogarty catheter. The histology revealed a malignant fibrous histiocytoma from the pleomorphic-storiform type. The postoperative course was complicated by an abscess which had to be drained. Three months after the operation the patient died of an uncontrolled stomach bleeding. The postmortem examination showed a tumor spread to the peritoneum, a tumor infiltration into the stomach, the left lower lobe of the lung and a tumor embolism into the right lower lobe of the lung.

Discussion

458

Fig. 3. Tumor invasion to the left renal and inferior caval vein

(case 2).

series that report a palpable abdominal mass in 85% of their cases [3]. Both patients were men and older than 50 years. Male patients are more affected than female [2-4], MFH can occur at any age, but it is more common in late adult life [3]. The most helpful diagnostic tool is the abdominal CT scan [2], The CAT of the abdomen in our 2 patients showed a large tumor mass in the left upper retroperitone-

Ziind/Kreienbiihl/Gobet/Hauri

Retroperitoneal Malignant Fibrous Histiocytoma

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Malignant Fibrous histiocytoma is regarded as the most common soft tissue sarcoma of late adult life [2], Most MFH arise within the soft tissue of the trunc or the extremities, and only 14% occur initially in the retroperitoneum [3]. In 1990, 2 cases of retroperitoneal MFH were operated on at the Department of Urology at the Univer­ sity Hospital of Zürich. The main complaint of both patients was severe pain in the upper left quadrant of the abdomen. In case 1, symptoms were of such sudden onset that myocardial infarction had to be ruled out. In con­ trast, Kearny et al. [3] reported tumor-related pain in only 20% of their patients. Both of our patients had an abdom­ inal mass which is in accordance with the Mayo Clinic

um. The tumor was assumed to originate from the left kidney with infiltration of adjacent structures (fig. 1,2). Only during the operation, the situs revealed a mass aris­ ing from the retroperitoneum, surrounding or infiltrating the left kidney. Tumor invasion in the left renal vein and the inferior cava! vein (fig. 3), as in case 2, was a further feature misleading to the diagnosis of a kidney tumor. The histology in both cases showed a mixture of pleo­ morphic and storiform pattern of MFH. According to the literature, this is the most common histological subtype of MFH [2, 3, 6]. Using the criteria of Kyriakos and Kempson [7] and Weiss and Enzinger [2], where tumors are subclassified as fibrous, giant cell, myxoid or inflammatory variants, both tumors in our cases were from the fibrous variant. In both patients only an incomplete tumorectomy with nephrectomy on the left side was first possible. Unfortu­

nately the complete excision is rarely possible at the time of diagnosis. Death occurs in the majority by complica­ tion of local invasion [3]. Our second patient died of an uncontrolled stomach bleeding 3 months after the opera­ tion because of tumor invasion of the posterior stomach wall. A 4-year survival rate of only 14% is noted for retro­ peritoneal MFH [3]. Capanna et al. [8] reported a some­ what better 3-year survival rate using a combined therapy with wide surgical excision and adjuvant chemotherapy. The first patient underwent a second operation with the goal of total excision of the rest of the tumor. The patient is now doing well 5 weeks after the last operation and adjuvant chemotherapy is planned. Finally we would like to emphasize again that a large retroperitoneal tumor, resemblingly arising from the kid­ ney, can be a mesenchymal tumor like MFH, even if inva­ sion of the renal or inferior caval vein is present.

References 5

6

Meoli FG. Cerone A: Malignant fibrous histio­ cytoma: Report of two cases and review of the literature. J Am Osteopath Assoc 1984:84:184190. Cherin P. Piette JC. Blctry O, Le Charpentier Y, Judet T. Richard F, Gatfosse M. Aubert L, Mornet M. Wechsler B. Godeau P: Histiocytome fibreux malin des tissus mous: presenta­ tions atypiques. Rev Med Interne 1990:11: 121-128.

7

8

Kyr.akos M. Kempson RL: Inflammatory fi­ brous histiocytoma: An aggressive and lethal lesion. Cancer 1976:37:1584-1606. Capanna R. Bertoni F, Bacchini P. Bacci G. Guerra A, Camnacci M: Malignant fibrous his­ tiocytoma of the bone. Cancer 1984:54:177— 187.

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1 O'Brien JS. Stout AP: Malignant fibrous xan­ thomas. Cancer 1964; 17:1445—1455. 2 Weiss SW. Enzinger FM: Malignant fibrous histiocytoma. Cancer 1978:41:2250-2266. 3 Kearny MM. Soule EH. Ivins JC: Malignant fibrous histiocytoma: A retrospective study of 167 cases. Cancer 1980:45:167-178. 4 Vera-Donoso CD. Llopis B. Froufe A. Alonso M. Boronat F. Oliver F. Jimenez-Cruz JF: Ret­ roperitoneal malignant fibrous histiocytoma. Eur Urol 1988:15:302-305.

Retroperitoneal malignant fibrous histiocytoma. Report of two cases.

We present 2 cases of malignant fibrous histiocytoma (MFH) of the retroperitoneum. Only 12-14% of all MFH occur in the retroperitoneum. Both patients ...
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