HEMANGIOPERICYTOMA: A CASE REPORT AND RATIONALE FOR AGGRESSIVE THERAPY

CHAKLES COOK, MD,* GERARD S. KAKOS, M D , ~ AND STUART ROBERTS, MD$

Hemangiopericytomas are infrequently encountered neoplasms which exhibit growth and recurrence potentials distinct from other vascular tumors. Late recurrence (greater than 5 years) is not uncommon, and the over-all metastasis rate approximates 50%. An aggressive approach is indicated for these tumors, as both radiation therapy and chemotherapy seem to be of little benefit. A case in which the primary lesion arose in the meninges, followed by pulmonary and hepatic metastasis, resected 11 and 16 years, respectively, after the primary tumor is presented. The patient is currently alive and asymptomatic without evidence of additional tumor.

Cancer 34:1830-1833, 1974.

EMANGIOPERICYTOMAS ARE INFREQUENTLY H seen neoplasms of vascular origin, de- scribed as a separate entity in 1942 by Stout and Murray.5 Since receiving this classifica- tion, approximately 225 of these unusual tu- mors have been reported, the majority occur- ring in the musculoskeletal system.l.4 While various therapeutic modalities have been em- ployed, operative removal of the primary le- sion and, infrequently, of recurrent or meta- static tumors has yielded the best survival cl a t a

This communication reports a case in which extended right hepatectomy was employed to control metastasis from a primary hemangio- pericytoma removed from the meninges 17 years earlier. The aggressive approach appears justified by the growth pattern and late re- currence rate associated with these tumors, a5

is exemplified both here and in previous re- ports in the l i t e r a t ~ r e . ~ ~ ~ ~ ~

CASE REPORT

A 36-year-old Caucasian woman was ad- mitted to the University Hospital in 1971 for evaluation of a 3-month history of progressive, severe, right upper quadrant abdominal pain,

From the Department of Surgery, Thc Ohio State

Supported in part by NIGMS Grant 5TIGM-1539. * Clinical Cancer Fellow. t Assistant Professor. X Clinical Professor of Surgery. Address for reprints: G. S. Kakos, MD, Dcpt. of

Surgery, The Ohio State llniversity, College of- Medi- cine, 410 W. 10th Ave., Columbus, OH 43210.

Received for publication January 24, 1974.

[Jniversity, College of Medicine, Columbus, OH.

associated with a palpable abdominal mass. In 1955, she liad undergone a right craniotomy for removal of a “vascular meningioma” and had done well until 1966, when a persistent cough with hemoptysis prompted her first ad- mission to our hospital. At that time, a mass lesion in the left upper lobe of the lung was removed by lobectomy. Histologic examina- tion demonstrated this to be a “benign, highly vascular mesenchymal tumor, possibly a he- mangiopericytoma.” She had remained asymp- tomatic until the present admission. The pa- tient’s physical examination was within normal limits with the exception of a firm, palpable mass in the right lobe of the liver which ex- tended 12 cm below the right costal margin. Laboratory data, including the hemoglobin, hematocrit, white blood cell count, serum elec- trolytes, blood urea nitrogen, creatinine, uri- nalysis, van der Bergh, prothrombin time, al- kaline phosphatase, a-2 fetal globulin, lactic dehytlrogenase, and serum glutamic oxalacetic and pyruvic transaminases were within nor- mal limits. Roentgenograms of the skull, chest, and long bones, and contrast studies of the kidneys and upper and lower gastrointestinal tracts demonstrated no evidence of neoplasm. Technetium liver scan showed two large le- sions involving the right and medial portions of the liver (Fig. 1A). This was confirmed by hepatic angiography, which further delineated the blood supply to these lesions and to the remainder of the hepatic tissue (Fig. 2).

At exploration, two large lesions confined to the right lobe and medial segment of the left lobe of the liver were found (Fig. 3). An extended right hepatectomy, involving ap- proximately 85% of the liver, was carried out. Reoperation to control a persistent, slow blood loss from the liver surface was required in 24

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No. 5 AGGRESSIVE THERAPY I N HEMANCIOPERICYTOMA * Cook et al.

FIG. 1A. (left). Initial technetium liver scan; two lesions measuring 6 cm and 7 cm in diameter. B (center). Liver scan 6 weeks post operatively; significant “regeneration” has oc- curred. C (r ight) . Scan 18 months postoperatively; liver size is normal.

FIG. 2A. (top). Selective celiac arteriogram demonstrating two lesions in right lobe and medial segment of left lobe, early and late phase. B (bot tom). Superior mcsenteric arteriogram delineating additional anomalous right hepatic artery supplying the tumor and right lobe of liver.

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1832 CANCER November 1974 Vol. 34

hours, but the remainder of the hospital course was essentially uneventful. At followup over a 24-month period, serial hepatic scans have shown almost complete regeneration of the resected hepatic tissue, with no evidence of tumor (Fig. IB, C). Hepatic enzymes and other laboratory data, plus chest roentgenograms, have remained normal since discharge from the hospital.

Histologic examination of the hepatic tu- mors confirmed the diagnosis of hemangioperi- cytoma (Fig. 4). Sections of the previous tumors removed from the meninges and lung were acquired and proved to be identical to the hepatic lesion.

DISCUSSION

Although infrequently encountered, heman- giopericytoma demonstrates well a form of malignant tumor which characteristically pro- duces late recurrence or metastasis amenable to operative intervention.’,* An 11% recur- rence or metastasis rate described by Stout6 has been updated to 52% in an exhaustive, recent review by Buckwinkel and Diddamsl which encompassed a prolonged followup pe- riod. They further confirmed that radiation and/or chemotherapy were ineffective in con- trolling these lesions, while surgery provided a 53y0 5-year (or greater) survival without recurrence.

As previously stated, the majority of heman-

FIG. 3. Resected specimen with obvious tumor in medial seg- ment, left lobe, and intraparen- chymal tumor partially hidden by gallbladder.

giopericytomas arises in the musculoskeletal system (5070).1v4 The remainder have occurred throughout the body, including 15 cases in the brain.1 The prognosis in the latter was quite poor, as 12 patients exhibited local or distant recurrence within 5 years. Primary in- volvement of the liver has not been reported, but multifocal involvement, either from metas- tasis or de novo, is possible in view of the pericytes in every organ. Attempts to charac- terize these tumors as benign or malignant have been unrewarding, again because of the propensity for late recurrence. The case pre- sented here demonstrated this point firmly, as the first two lesions removed were considered “benign.” The hepatic tumors, however, did show local invasion into normal hepatic tissue, although they grossly appeared en- capsulated.

The histologic characteristics of heman- giopericytomas have been well described5 and include the following necessary criteria: 1) normal endothelial cells distinctly separated from the proliferating pericytes, and 2) the tumor cell per se must be a pericyte, i.e. out- side the fibrous sheet surrounding the vascular endothelial cells. The importance of accu- rately differentiating these tumors from other neoplasms of vascular origin, such as he- mangioendotheliomas, is obviously important, both in therapeutic approach and ultimate prognosis.

No. 5 AGGRESSIVE THERAPY I N HEMANCIOPERICYTOMA Cook et al. 1833

FIG. 4. Histologic section confirmed hemangiopericytoma (210, reduced from x240).

The case reported in this communication The patient’s 18-year survival justifies the latter, even though an accurate diagnosis was not confirmed on the initial meningeal tumor at the time of its removal.

supported the necessity for accurate histologic diagnosis, prolonged followup (greater than 5 years), and an aggressive surgical approach.

REFERENCES

1 . Buckwinkel, K . D., and Diddams, J. A.: Heman- gioprricytoma-Report of a case and comprehensive review of the literature. Cancer 252396-901, 1970.

4. O’Brien, P., and Brasfield, R . D.: Hcmangioperi-

5 . Stout, A. P., and Murray, M. R.: Hemangiopcri- cytoma. Cancer 18:249-252, 1965.

cytoma-A vascular tumor featuring Zimmcrmann’s pericytcs, Ann, surg, 116:2633, 1942.

6. Stout, A. P.: Hemangiopericytoma-A study of twenty-five new cases. Cancer 2:1027-1054, 1949.

2. Forrcstcr,J. S., and Houston, R. A.: Hemangio- pericytoma with metastasis-Report of a case with autopsy. Arch. Pathol., 51:651-653, 1951.

3. McCormack, L. J., and Gallivan, W. F.: Hemangio- 7. Wise, R. A,: Hemangiopericytoma-Surgical con- pericytoma. Cancer 7:595-601, 1954. siderations. Arch. Surg. 65:201-210, 1952.