Pelvic hemangiopericytomas: A report of five cases and literature review

GYNECOLOGIC

ONCOLOGY

36, 380-382 (1990)

Pelvic Hemangiopericytomas: A Report of Five Cases and Literature Review ALAN K. Mufioz,

M.D.,

JONATHANS. BEREK, M.D.,

YAO S. Fu, M.D.,

AND PETER A. M. HEINTZ,

M.D.

Division of Gynecologic Oncology, Departments of Obstetrics and Gynecology, and Pathology, UCLA School of Medicine, Jonsson Comprehensive Cancer Center, Los Angeles, California Received April 11, 1989

Five casesof pelvic hemangiopericytomas are reported. One of thesetumors arose from the uterus, and four patients had extrauterine, pelvic hemangiopericytomas.The patient with a primary uterinehemangiopericytomas had only simpleexcision,and, after 6 years, is alive and free of disease.All four patients with extrauterine, pelvic hemangiopericytomashad incompleteresection of their tumors becauseof hemorrhage. However, pelvic radiation therapy was then employedin thesepatients and produceda completeregressionin one patient and partial regression in two patients with minimal shrinkagein another patient. The latter patients were reexplored after pelvic radiation and underwent completeresectionof their disease.Two patientsdeveloped pelvic recurrencesat 2 and 9 years, respectively,and thesewere effectively resected. All four patients are all alive and free of disease5 to 18yearslater. If this lesionisunexpectedlydiscovered

at laparotomy, our experience suggests that the resection should be discontinuedand that they should be treated with pelvic radiation and delayed resectionof persistentand recurrent pelvic tumors. 0 1990 Academic Press, Inc.

INTRODUCTION Hemangiopericytomas are rare vascular neoplasms derived from pericytes that surround the capillaries and contain smooth muscle type filaments to facilitate contraction. Since the original clinico-pathologic description by Stout and Murray [l], several papers have described the histopathologic features, clinical presentation, treatment modalities, and outcome of these tumors [2-71. Pelvic hemangiopericytomas are particularly rare. Uterine hemangiopercytomas appear to behave in a more benign manner than do extrauterine tumors 141. There are no accepted histopathologic criteria that predict the subsequent behavior of these malignancy. We report five patients with pelvic hemangiopericytomas and review the world’s literature.

380 0090-8258/90$1.50 Copyright Q 1990 by Academic Press, Inc. AU rights of reproduction in any form reserved.

METHODS

AND MATERIALS

All cases of pelvic vascular tumors from the UCLA Pathology files and from the UCLA Tumor Registry were evaluated. Original hematoxylin and eosin-stained slides, as well as slides stained with trichrome and reticulum technique, were examined. Current follow-up was obtained on all five patients by personal contact. RESULTS We identified five patients with pelvic hemangiopericytomas treated over a 30-year period (1955-1984) at the UCLA Medical Center. The patient ages ranged from 27 to 67 years, with a median age of 44 years. Table 1 summarizes the clinical course of each patient. In one patient, the tumor arose from the uterus, and in four patients, the lesions were extrauterine pelvic hemangiopericytomas. The patient with a uterine hemangiopericytoma was asymptomatic. All the other four patients presented at routine gynecologic exam with mild symptoms of vague pelvic discomfort. Histologically, the neoplasms revealed irregular capillary spaces and proliferation of cells around the vessels in a whorl-like pattern (Fig. 1). Perivascular collagen deposits were seen focally. The nuclei were relatively uniform with an elongated to oval configuration. The cellularity and mitotic activity varied from tumor to tumor. The uterine tumor contained abundant stromal fibrosis and less than one mitotic figure per 10 high power fields. Other pelvic tumors were moderately to highI> cellular. The mitotic activity in one neoplasm (case 2) was less than five mitotic figures. and the rrmuining three had more than five mitotic figures per I(1 high powct fields (Table I).

PELVIC

381

HEMANGIOPERICYTOMAS

TABLE 1 Clinical presentation

Initial treatment

27

Asymptomatic/ cervical polyp

Polypectomy, 1 cm in size

2

67

3

34

S/P, TAH/ BSO nonspecific pelvic discomfort SP/TAH/ BSO vague pelvic discomfort

4

60

5

44

Case

Age

I

Additional treatment

Pathology

Follow-up

None

NED 6 years

Incomplete resection of l6-cm bladder flap mass, 1500 cc blood loss

Benign, less than one mitotic figure per 10 HPFs Highly cellular, less than five mitotic figures per 10 HPFs, no necrosis

5000 cGy pelvic radiation followed by complete resection

Omental recurrence 9 years. Surgically resected, NED I5 years

Incomplete resection of 20-cm pelvic mass, 3000 cc blood loss

More than five mitotic figures per IO HPFs, highly vascular necrosis

Pathologic fracture LS-5, at 2 years, ERT to fx NED 12 years

Vague pelvic discomfort

Incomplete resection of 25cm pelvic mass, 2500 cc blood

More than five mitotic figures per IO HPFs, vascular, necrosis

Vague pelvic discomfort

Incomplete resection of 20-cm pelvic mass, 1500 cc blood loss

More than five mitotic figures per IO HPFs, vascular, necrosis

5000 cGy pelvic radiation and Adriamycin followed by complete resection 4000 cGy pelvic radiation, no clinical shrinkage of tumor followed by complete surgical resection 6000 cGy pelvic radiation complete clinical response. Patient refused reexploration

NED I8 years

NED 5 years

Note. HPFs, high power fields; NED, no evidence of disease.

Benign and malignant tumors derived from endometrial stromal tissue were excluded from this study. They are characterized by a diffuse, monotonous, sheet-like proliferation. Cellular proliferation around the arterioles sometimes result in a whorl-like pattern. However, they lack the dilated capillary spaces and compartmentalized pattern typical of hemangiopericytoma. This pattern was maintained throughout the tumors reported here. In all four patients with extrauterine tumors, only incomplete surgical resection was possible because of excessive bleeding. All four subsequently underwent whole pelvic external radiation therapy to 5000 cGy. In two patients, reoperation was successful in totally removing residual tumor, whereas in one patient, radiation produced only minimal shrinkage, but the lesion was completely resected on reoperation. One patient had a complete clinical response to radiation therapy, and she refused further surgical intervention. The patient with uterine hemangiopericytoma had a 2-cm cervical polyp removed but she refused further treatment. Two patients developed a pelvic recurrence at 2 and 9 years, respectively. Both patients were reexplored and the tumors were completely resected. The first of these patients also had disease recurrence in the lumbosacral spine that completely regressed after 5000 cGy radiation therapy to the pelvis.

The follow-up ranged from 5 to 18 years. With a median survival of 12 years, all patients are alive and clinically free of disease. DISCUSSION In contrast to earlier studies in which vaginal bleeding and significant pelvic pain are described consistently [241; all patients but one were asymptomatic. All four of our patients with extrauterine hemangiopericytomas were not surgically resectable initially because of their extreme vascularity. One tumor with a high mitotic count, necrosis, and high vascularity, recurred in 2 years, whereas a less malignant appearing tumor recurred 9 years later. All tumors incompletely resected exhibited some degree of malignant histology, increased cellularity, vascularity, and mitotic figures. These features are described by Enzinger in his review of 106 cases of soft tissue hemangiopericytomas as those that correlated with malignant behavior [5]. These pathologic features were not substantiated in other papers related to pelvic hemangiopericytomas [2-41. The majority of hemangiopericytomas of the soft tissue with more than four mitotic figures per 10 high power fields have behaved in a malignant manner [5]. Three tumors in this series had more than

382

MUNOZ

FIG.

1.

ET AL.

Irregular capillaries are surrounded by round to oval cells with a distinct whorl-like

five mitotic figures per 10 high power fields but have not resulted in metastasis. Thus, pelvic hemangiopericytomas may be more benign than their soft tissue counterparts. Thus, the extent of tumor differentiation, as shown by light microscopy, corresponds to the clinical behavior of the tumors. Most unresectable tumors in our series showed a favorable response to radiation therapy making subsequent resection possible in all cases. One patient with recurrent disease received radiation to the spine and had a complete clinical response. In Wilbank’s review of 10 extrauterine tumors, four patients with extensive pelvic disease were incompletely resected. Two patients died at 11 and 18 months after failed attempts at resection; neither received radiation therapy. One patient who did receive radiation subsequently underwent a posterior exenteration and had free margins of resection [3]. Several authors have shown radiation to be an effective modality in the treatment of hemangiopericytomas. In a review of 11 cases at the M.D. Anderson Hospital, 8 patients were clinically free of disease when treated primarily and for recurrence. Five had pelvic hemangiopericytomas that were surgically unresectable [6]. Mira et al [7] reported that in 49 nonpelvic tumors, those treated with more than 3500 cGy had over a 50% com-

pattern. (H&E,

x

125).

plete regression rate. Thus, radiation therapy appears to be beneficial both in unresectable pelvic hemangiopericytomas and in treatment of isolated recurrences if radiation had not been employed previously. REFERENCES 1. Stout, A. P., and Murray, U. R. Hemangiopericytoma: A vascular tumor featuring Zimmerman’s pericytes, Arch. Surg. l&26 (1942). 2. Greene, R. R., Gerbie, A. B., Gerbie, U. V., and Eckwan, T. R. Hemangiopericytomas of the uterus, Amer. J. Ubstet. Gynecol. 106, 1020 (1970). 3. Wilbanks, G. D., Szywanska, Z., and Miller, A. W. Pelvic hemangiopericytomas: Report of four patients and a review of the literature, Amer. J. Obstet. Gynecol. 123, 555 (1975). 4. Buscema, J., Klein, V., Rotmenash, J., Rosenstein, N., and Woodruff, J. D. Uterine hemangiopericytomas, Obstet. Gynecol. 69, I04 (1987). 5. Enzinger, F. M., and Smith, B. H. Hemangiopericytoma: An analysis of 106 cases, Hum. Puthol. 7, 61 (1976). 6. Jha, N., McNeese, M., Barkley, H. T., and Kong, J. Does radiotherapy have a role in hemangiopericytoma management: Report of I4 new cases and a review of the literature, ht. J. Radiat. Oncol. Biol. Phys. 13, 1399 (1987). 7. Mira, J. Cl., Chu, F. C. H., and Fortner, J. G. The role of radiotherapy in the management of malignant hemangiopericytoma: Report of eleven new cases and review of the literature, Cancer 39, 1254 (1977).