Mesenchymal Chondrosarcoma Originating from the Femoral Vein

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Mesenchymal chondrosarcoma originating from the femoral vein

Geun Eun Kim, MD, Do Kyun Kim, MD, In Ja Park, MD, and Jae Yun Ro, MD, Seoul, Korea

Mesenchymal chondrosarcoma is a rare variant of chondrosarcomas characterized by a bimorphic pattern with areas of the undifferentiated malignant small cells and well differentiated cartilaginous islands.1 It occurs most commonly in the bone but can also occur in the extraskeletal soft tissues, the brain, and the meninges. This type of tumor has also been described in the eyelids, parapharyngeal space, mediastinum, and the kidney.1-5 An origin from the large vessels has not been reported in the medical literature. The authors report a case of mesenchymal chondrosarcoma originating from the femoral vein in a 28-year-old female patient, treated by the wide-margin resection. (J Vasc Surg 2003;37:202-5.)

Mesenchymal chondrosarcoma is a highly malignant Operative findings. The operation was performed tumor that follows a rapid clinical course with frequent with spinal block. A skin incision was made and dissection metastasis to the lung. The principle anatomic sites of was carried to the deep above and below the femoral vein. extraskeletal mesenchymal chondrosarcoma are the region A 10- ϫ 3.5- ϫ 3.5-cm firm mass of the superficial femoral of the head and neck, the cranial and spinal dura mater, vein was found, which was adherent to the surrounding followed by the low extremities.6-8 The thigh is the most quadriceps muscles. common site of extraskeletal mesenchymal chondrosar- The tumor encompassed the confluence of the superfi- coma in the low extremities,1,6 and the involvement of the cial and deep femoral veins, but intraluminal mass was muscle of the leg has been rarely observed. The authors movable (Fig 2, A). The mass was resected en bloc including experienced the case of a 28-year-old female patient with a the superficial femoral vein and the surrounding soft tissue. firm lump at her proximal anteromedial thigh. Preoperative The proximal and distal ends of the vein margin were free of computed tomography (CT), duplex study, and venogram tumor, which was confirmed by the frozen section evalua- were helpful in defining intraluminal and extraluminal mass tion. The proximal femoral vein was repaired by angioplasty of the femoral vein. method, allowing distal vein drainage through the greater saphenous vein (Fig 2, B). CASE REPORT Pathologic findings. The tumor measured 10 cm in greatest dimension and is mostly confined within the vas- A 28-year-old female patient presented with a 1-month his- cular lumen. It was firm, well demarcated, multilobulated, tory of lump at the anteromedial aspect of the left thigh and left leg and attached to the surrounding soft tissues. The intralu- edema. The mass was not painful. The patient denied any history of minal portion of the mass was mostly pinkish-gray and trauma and her other medical history was unremarkable. On solid. Histologically, the tumor originated from the vascu- physical examination, a firm 10- ϫ 4-cm mass was palpable deep to lar wall and showed a bimorphic pattern composing areas of the fascia in the thigh. There was no localized warmth, erythema, the undifferentiated small cells and the well-differentiated bruising, or tenderness. No lymphadenopathy was present. CT cartilaginous islands (Fig 3, B). Calcification and ossifica- (Fig 1) showed a large lobulated mass in the superficial femoral tion were present in some areas. Most of the mass was vein with scattered calcifications. The tumor extended intralumi- confined in the intima, but focal invasion to adventitia was nally beyond the orifice of the greater saphenous vein of the also noted. The undifferentiated small cells had ovoid or femoral vein. The intraluminal mass extending to the common elongated hyperchromatic nuclei and scanty cytoplasm. femoral vein was somewhat movable when examined by real-time There was a prominent hemangiopericytic pattern. The duplex study. No evidence of deep vein thrombosis was noted in cartilaginous foci were relatively well-defined islands or other veins. The maximal venous outflow test showed the normal nodules of the benign appearing chondroid tissue (Fig 3, ratio in both legs. A). By immunohistochemistry, the undifferentiated small From the Division of Vascular Surgery and Department of Pathology, tumor cells were strongly positive for CD99 but negative in University of Ulsan College of Medicine and Asan Medical Center, Seoul, the cartilaginous areas (Fig 3, C) that showed immunore- Korea. activity for S-100 protein but negative in the undifferenti- Competition of interest: nil. Reprint requests: Geun Eun Kim, MD, FACS, Chief of Vascular Surgery, ated small tumor cells. The positive reaction to CD31 was University of Ulsan College of Medicine, Director, Cardiovascular Cen- limited to the vascular endothelial cells which confirmed ter, Asan Medical Center, 388-1 Poongnap-dong, Songpa-gu, Seoul the tumor being within the vascular space. 138-736, Korea (e-mail: [email protected]). Postoperative course. The patient had an uneventful Copyright © 2003 by The Society for Vascular Surgery and The American postoperative recovery and was discharged 1 week after Association for Vascular Surgery. 0741-5214/2003/$30.00 ϩ 0 surgery. Bone scan and chest CT were performed 3 months doi:10.1067/mva.2003.106 postoperatively and showed no distant metastasis. 202 JOURNAL OF VASCULAR SURGERY Volume 37, Number 1 Kim et al 203

Fig 1. CT shows a multilobulated mass in the superficial femoral vein with scattered calcifications. The tumor extended to the orifice of the greater saphenous vein and the femoral vein.

Fig 2. A, Operative findings. The tumor encompassed the confluence of the superficial and deep femoral veins. B, Proximal femoral vein was repaired by angioplasty allowing distal vein drainage through saphenous vein. Arrow shows the resected stump. JOURNAL OF VASCULAR SURGERY 204 Kim et al January 2003

Fig 3. A, Histologically, the tumor is located in the vascular lumen. Blood between vascular wall and the tumor was also noted (hematoxylin and eosin, ϫ40). B, The tumor shows a characteristic bimorphic pattern composed of areas of undifferentiated small cells and cartilaginous island shown by arrows. C, By immunohistochemistry, the undifferentiated small tumor cells are strongly positive for CD99 (CD99, ϫ200).

DISCUSSION rate. A wide-margin excision is a preferred treatment,1 Mesenchymal chondrosarcoma is a rare tumor, first and the role of chemotherapy and radiation remains 13-15 described by Lichtenstein and Bernstein9 in 1959 as a controversial. primary bone sarcoma that comprises less than 2% of all The authors’ experience of mesenchymal chondrosar- chondrosarcomas.1 This tumor is even less commonly dis- coma originating from the femoral vein showed no evi- covered in the extraskeletal soft tissues10 with brain and dence of invasion to the surrounding structures. Salvage of meninges being involved more frequently. There appears a tributary of the superficial femoral vein and greater saphe- to be a predilection for the female sex and the young adult nous vein resulted in venous patency without graft interpo- age. Approximately 60% to 80% of mesenchymal chondro- sition after resection of the tumor. We resected the tumor sarcoma occurs in the second and third decades of life. with a sufficient margin. Louvet et al11 reported a relation between the age of the In conclusion, we are reporting a case of mesenchymal patient and the location of the tumor; the patients with chondrosarcoma originating from the femoral vein in a neural and muscular forms of the disease were younger than 28-year-old female patient who was surgically treated with those with mesenchymal chondrosarcoma arising from the a wide-margin resection. We are reporting the first case of musculature. mesenchymal chondrosarcoma arising apparently from the Histologically, it is characterized by a bimorphic major vessel. pattern with sheets of the undifferentiated round-, oval-, or spindle-shaped small cells and islands or the nodules REFERENCES of benign-appearing cartilaginous tissue, frequently with central calcification. The undifferentiated cells are ar- 1. Alder CP. Case report 788: mesenchymal chondrosarcoma of the soft ranged in small aggregated or hemangiopericytoma-like tissue of the left foot. Skeletal Radiol 1993;22:300-5. 12 2. Rohrbach JM, Steuhl KP, Pressler H, Kaiserling E, Schaumburg-Lever pattern. G, Scheel-Walter HG. Primary extraskeletal mesenchymal chondrosar- 13 Local recurrence usually precedes metastasis. Me- coma of the lid. Graefes Arch Clin Exp Ophthalmol 1991;229:172-7. tastasis of mesenchymal chondrosarcoma has been ob- 3. Gomersall LN, Needham G. Case report: mesenchymal chondrosar- served in the regional and/or distant lymph nodes, and coma occurring in the parapharyngeal space. Clin Radiol 1990;42:359- the lungs as well as in the bones. Mesenchymal chondro- 61. 8 4. Chetty R. Extraskeletal mesenchymal chondrosarcoma of the mediasti- sarcoma has a poor prognosis. Nakashima et al reported num. Histopathology 1990;17:261-3. 5- and 10-year survival rates of 54.6% and 27.3%, respec- 5. Malhotra CM, Doolittle CH, Rodil JV, Vezeridis MP. Mesenchymal tively. Huvos et al7 described a similar 10-year survival chondrosarcoma of the kidney. Cancer 1984;54:2495-9. JOURNAL OF VASCULAR SURGERY Volume 37, Number 1 Kim et al 205

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