Abstract Malignant pigmented villonodular synovitis is an ex- tremely rare and controversial dis- ease. We describe malignant change in pigmented villonodular ...
Skeletal Radiol (1998) 27:392±395 International Skeletal Society 1998
CASE REPORT
Malignancy in pigmented villonodular synovitis
Ricardo K. Kalil K. Krishnan Unni
R.K. Kalil, M.D. Department of Pathology, Sarah Network of Hospitals for the Locomotor System, Brasilia, D.F., Brazil
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K.K. Unni, M.B., B.S. ( ) Department of Laboratory Medicine and Pathology, Mayo Clinic and Mayo Foundation, 200 First Street SW, Rochester, MN 55905, USA
Introduction Pigmented villonodular synovitis (PVNS), a well-known disease of joints and tendon sheaths, is diffuse or localized, may involve any synovial surface, and may be aggressive. The probable neoplastic quality of PVNS is supported by findings of aneuploidy [1] and cytogenetic abnormalities [2, 3]. Its even more controversial malignant transformation was documented in three cases recently reported by Bertoni et al. [4]. Recurrences in these cases were frequent, in one instance appearing 44 years after the initial excision, with excision of 11 recurrent tumors in between. About half the patients described so far died of extensive and metastatic disease between 21 months and 27 years after initial admission.
Abstract Malignant pigmented villonodular synovitis is an extremely rare and controversial disease. We describe malignant change in pigmented villonodular synovitis of the ankle in a patient with an unusually long clinical history. Symptoms began at age 21, metastatic disease developed at age 85, and the patient died 1 year later. The histologic appearance of the malignant tumor differed from that in most reported cases, in that spindle-shaped cells predominated.
Lymph node metastasis also developed, a feature uncommon to soft tissue sarcomas.
We report an instance of malignant PVNS in a patient who was 21 years old when the process began, had surgical treatment at age 25, and underwent reoperation 3 years later. Malignant transformation was diagnosed when the patient returned at age 85; she was treated with an above-knee amputation, had a recurrence, and died 1 year later.
of the periosteum.º Six months later, two tumors were resected from her ankle. Histopathologic examination at the time reported ªno cancer.º After surgery, the ankle became progressively painful and swollen, with radiation of the pain to the knee and hip. Three years later, at age 28, she was seen at our institution. At this time, although in good general health, she had diffuse soft tissue swelling and heat at the ankle, most pronounced over the lateral malleolus. Movements were normal. Radiographs at the time were reported as negative, but a soft tissue mass was seen at the posterior aspect of the right ankle. A biopsy and subtotal removal of the lesion were done. The surgeon described the lesion as ªvery cellularº and darkly pigmented. It had infiltrated soft tissue and ex-
Case report A 21-year-old woman had pain and swelling of her right ankle after torsion. The pain subsided, but the ankle did not return to normal size. Two years later, the pain returned and persisted. A gradual swelling was also noticed. She was treated continuously with casts for 3 more years, when a radiograph showed ªrupture
Key words Pigmented villonodular synovitis ´ Giant cell tumor of tendon sheath ´ Malignant tumor ´ Synovium
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1B Fig. 1A, B Pigmented villonodular synovitis. A Low-power view, showing the surface of the lesions, with villous projections (hematoxylin and eosin, 50). B High-power view. The nodules are composed of round to oval mononuclear cells without pleomorphism divided by fibrous tissue, giving rise to clefts. Many cells contain hemosiderin pigment (hematoxylin and eosin, 100) Fig. 2A, B Radiographs of the right ankle at age 85. A Anteroposterior and oblique views. An aggressive lytic and infiltrative lesion involves the soft tissues around the ankle, destroying the lower third of the tibia and fibula and the tarsal bones. B Lateral view. A large, ill-defined lytic lesion infiltrates soft tissues around the ankle, the lower third of the tibia and fibula, and multiple tarsal bones
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tended to the calcaneus and calcaneocuboid joint. The histologic diagnosis was PVNS (Fig. 1). Less than 1 year later, she underwent another procedure to remove tissue from the calcaneus. Postoperative radiotherapy was administered (230 min/140 kV) both times. The patient was next seen at age 85 at another institution. She had a large destructive tumor (Fig. 2) of the distal tibia. An above-knee amputation was done, but metastasis to the lungs and inguinal lymph nodes sub-
sequently developed. At the time of her death 1 year later, she had a recurrent lesion in the stump and a tumor in the groin. Histologic examination of the amputated specimen showed a cellular, sheetlike, solid, infiltrative lesion. Spindle cells were predominant, with moderate to large nuclei, prominent nucleoli, and eosinophilic cytoplasm. Typical and atypical mitoses were frequent (eight per ten high-power fields). There were focal areas of necrosis. Foam cells were variably present in many
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Fig. 3A, B Recurrent tumor. A Low-power view. Sheets of spindle cells are seen with some collagen bundles at the right and a collection of xanthoma cells at the left (hematoxylin and eosin, 50) B High-power view. Bundles of spindle cells with moderate atypia and numerous mitoses (hematoxylin and eosin, 100) Fig. 4 High-power view of recurrent tumor. A myxoid area is seen occasionally (hematoxylin and eosin, 200)
fields, and there were occasional giant cells (Fig. 3). Collagen bands, which could be seen in many fields, were occasionally reminiscent of osteoid. Myxoid areas were sometimes noted (Fig. 4). Radiographs obtained at ages 83 and 85 were available.
Discussion Enzinger and Weiss [5] defined malignant PVNS as a malignant lesion occurring with concomitant or previously documented PVNS at the same site. They stressed that some of the cases of malignant PVNS reported in the literature do not fulfill these criteria [6±8] and have histologic fea-
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tures suggestive of a variety of other neoplastic malignant lesions, whereas other reports appear to be true cases of malignant PVNS [9±11]. Bertoni et al. [4] analyzed eight examples of malignant PVNS, defining common histologic features and documenting, in three cases, histologic evolution from what was considered benign PVNS ± in which it was not possible, even retrospectively, to identify malignant features ± to a clinically and histologically more aggressive malignant tumor. They included five other cases that, although lacking documented PVNS, had the same histologic features. These were characterized by the progressive predominance in all mi-
croscopic fields of one type of cell ± large, round to oval, with large hyperchromatic nuclei and prominent nucleoli. Atypical mitosis and necrosis were frequent. Polymorphism of the lesion, xanthomatous cells, giant cells, clefts and lacunae, and maturation from the center to the periphery of nodules all tended to decrease or even disappear. The authors also described three cases in which radiotherapy as adjuvant treatment did not lead to the highly malignant appearance expected in postradiation sarcomas. In the present case, the descriptions of the radiologic image and the histologic character of the tumor resected at age 28 were consistent with
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References
Fig. 5 Lateral and oblique radiographs of the right ankle at age 83. Ankle shows marked joint space narrowing and extensive spotty demineralization, with areas of cystic rarefaction on both sides of the joint
PVNS. Even now, it is impossible to find histologic evidence of malignant change in the original slides. The radiograph obtained at the patient's recent admission showed a highly malignant tumor, and the histologic findings were also clearly those of a malignant neoplasm. Unlike most reported cases, the present one was notable for a predominance of spindle-shaped cells. Although the patient underwent postoperative radiotherapy, the cytologic features were not those of a high-grade neoplasm and thus were not typical of postradiation sarcomas. However, we cannot exclude a relationship between the radiotherapy and the subsequent neoplasm. It is noteworthy that three patients in the series described by Bertoni et al. [4] also had prior radiotherapy. The authors emphasized that these tumors did not have the high-grade malignant appearance of the usual postradiation sarcoma.
We do not have information on what occurred in the unusually long, 58-year, span between the two admissions, but the radiograph obtained 2 years before her final admission did not show evidence of recurrence (Fig. 5). Another interesting and unusual finding in this case was lymph node metastasis, a feature uncommon to soft tissue sarcomas. Although the present case complies with one of the criteria defined by Enzinger and Weiss [5] for the diagnosis of malignant PVNS, in that documented PVNS was previously diagnosed at the site of the malignant tumor, some cases lack histologic evidence of (or a history of) previous or concomitant PVNS, but nevertheless have morphologic features of malignant PVNS. Care should be taken to exclude other synovium-centered tissue lesions previously confused with malignant PVNS, such as fibrosarcoma, malignant fibrous histiocytoma, clear cell sarcoma, epithelioid sarcoma, and, occasionally, osteosarcoma.
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