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Extraskeletal Mesenchymal Chondrosarcoma of the Labium Majus
GYNECOLOGIC ONCOLOGY ARTICLE NO.
60, 492–493 (1996)
0080
CASE REPORT Extraskeletal Mesenchymal Chondrosarcoma of the Labium Majus J. LIN,1 K. M. H. YIP, N. MAFFULLI,
AND
L. T. C. CHOW*
Department of Clinical Oncology and *Department of Anatomical and Cellular Pathology, Prince of Wales Hospital, Shatin, New Territories, Hong Kong Received June 12, 1995
Extraskeletal mesenchymal chondrosarcoma is a rare tumor. It has been noted to occur most commonly in muscle and the central nervous system. It has never been reported to occur in the soft tissues of the anogenital region. We report the first such case, occurring in a 40-year-old woman who presented with a 1-year history of a mass originating in the left labium majus. After extensive local resection and lymph node dissection she remains disease free after 40 months follow-up. q 1996 Academic Press, Inc.
INTRODUCTION
Mesenchymal chondrosarcoma was first described in bone by Lichtenstein and Bernstein [1] in 1959 and in soft tissue by Dowling [2] in 1964. It is a rare tumor, with less than 200 documented cases. It occurs two to three times more commonly in bones than in extraskeletal locations [3, 4]. Of the extraskeletal tumors reported in the literature, the majority have occurred in the muscles of the lower limb and meninges or brain [4, 5]. There have been no reports occurring in the anogenital region. We report such a case, occurring in the labium majus.
An excisional biopsy showed a large, fairly well circumscribed tannish nodular mass measuring 9 cm at its greatest diameter. Cut surface of the mass revealed tannish firm tumor tissue with a hemorrhagic center. Microscopically, the tumor was located mainly in the subcutaneous fat with infiltration into the overlying dermis. The tumor consisted mostly of undifferentiated round cells with hyperchromatic vesicular nuclei, prominent nucleoli, and scanty cytoplasm. Numerous mitotic figures were seen. Focally, these tumor cells were arranged in a hemangiopericytic pattern around ectatic vascular clefts. There were areas of poorly circumscribed islets of tumor cells showing features of chondroid differentiation (Fig. 1). No calcification or ossification was noted. The histological features coupled with the clinical findings pinpointed the diagnosis of extraskeletal mesenchymal chondrosarcoma. Because the deep resection margin was approximately 2 mm, it was decided that the patient should undergo a more radical operation. A radical vulvectomy and deep lymph node dissection was therefore performed 2 weeks after the initial surgery. There was no residual tumor in the subsequent specimen and all lymph nodes resected were clear of tumor. No adjuvant treatment was given. At a followup period of 40 months the patient remains well and free of disease.
CASE REPORT
A previously healthy 40-year-old woman presented with a 1-year history of a mass in the left suprapubic region. The mass had started as a small nodule in the left labium majus, and had progressively enlarged to the size of a fist, now causing some discomfort. There was never any discharge or bleeding from the mass. The patient was otherwise well. Clinical examination revealed a large mass occupying the left labium majus extending proximally toward the pubic symphysis. The mass was slightly tender and was not adherent to the underlying tissues. There was no ulceration or skin involvement. A full systemic workup showed negative findings. 1 To whom correspondence and reprint requests should be addressed. Fax: 852-26497426.
0090-8258/96 $18.00 Copyright q 1996 by Academic Press, Inc. All rights of reproduction in any form reserved.
/ m4716$4194
02-09-96 19:57:54
DISCUSSION
Extraskeletal mesenchymal chondrosarcoma is a rare tumor with less than 100 cases reported in the literature up to 1985 [5]. In that review, 54% of cases involved muscles, predominantly of the lower limb, and 46% of cases involved the central nervous system (CNS), meninges, brain, or orbit. The age at the time of diagnosis was significantly greater in the patients with extraskeletal mesenchymal chondrosarcoma involving the muscles (43.9 in years in the patients with tumors of the muscle, and 23.5 years in the patients with CNS involvement). Histologically, extraskeletal mesenchymal chondrosarcoma closely resembles hemangiopericytoma or poorly differentiated synovial sarcoma [3, 6]. The distinguishing fea-
492
goal
AP: Gyn Onc
493
CASE REPORT
FIG. 1. Photomicrograph showing undifferentiated tumor cells with areas of chondroid differentiation. Hematoxylin and eosin stain. Magnification, 125.
ture is the presence of areas of cartilaginous differentiation within the tumor [6]. Huvos et al. [3] histologically subclassified extraskeletal mesenchymal chondrosarcomas into two groups, the better differentiated hemangiopericytoid variant and the less well differentiated small cell type. The long-term prognosis of mesenchymal chondrosarcomas, when all sites are considered, is poor. Local recurrences or metastases can occur after more than 20 years. In a Mayo Clinic study of 23 patients with long-term follow-up, 74% died of disease 6 months to 23 years after the initial diagnosis, with an average survival of 6.7 years [4]. Six patients died after 5 to 10 years, and 3 patients died after more than 10 years’ follow-up. The 5-year survival rate was 54.6% and the 10-year survival rate was 27.3%. In the series reported by Huvos et al. at the Memorial Hospital, the 10-year survival rate was 28% [3]. We were not able to locate any other reports of a case of extraskeletal mesenchymal chondrosarcoma occurring in the soft tissues of the anogenital region. The histology in the case presented shows features of undifferentiated cells as well as hemangiopericytoid areas with focal chondroid differentiation, therefore making it difficult to subclassify according to Huvos et al. Forty months after the initial diagno-
/ m4716$4194
02-09-96 19:57:54
goal
sis, the patient remains well and free of disease. We await longer term follow-up of this patient. ACKNOWLEDGMENT The authors thank Dr. S. C. Luk for making the histological slides available for the present report.
REFERENCES 1. Lichtenstein, L., and Bernstein, D. Unusual benign and malignant chondroid tumors of bone, Cancer 12, 1142–1157 (1959). 2. Dowling, E. A. Mesenchymal chondrosarcoma, J. Bone Joint Surg. 46A, 747–754 (1964). 3. Huvos, A. G., Rosen, G., Dabska, M., and Marcove, R. C. Mesenchymal chondrosarcoma: A clinicopathologic analysis of 35 patients with emphasis on treatment, Cancer 51, 1230–1237 (1983). 4. Nakashima, Y., Unni, K. K., Shives, T. C., Swee, R. G., and Dahlin, D. C. Mesenchymal chondrosarcoma of bone and soft tissue: A review of 111 cases, Cancer 57, 2444–2453 (1986). 5. Louvet, C., de Gramont, A., Krulik, M., Jagueux, M., Hubert, D., Brissaud, P., Sirinelli, A., Augereau, B., Tubiana, J. M., and Debray, J. Extraskeletal mesenchymal chondrosarcoma: Case report and review of the literature, J. Clin. Oncol. 3, 858–863 (1985). 6. Enzinger, F. M., and Weiss, S. W. Cartilaginous tumors and tumorlike lesions of soft tissue, in Soft tissue tumors (F. M. Enzinger and S. W. Weiss, Eds.), Mosby, St. Louis, pp. 698–710 (1983).
AP: Gyn Onc
60, 492–493 (1996)
0080
CASE REPORT Extraskeletal Mesenchymal Chondrosarcoma of the Labium Majus J. LIN,1 K. M. H. YIP, N. MAFFULLI,
AND
L. T. C. CHOW*
Department of Clinical Oncology and *Department of Anatomical and Cellular Pathology, Prince of Wales Hospital, Shatin, New Territories, Hong Kong Received June 12, 1995
Extraskeletal mesenchymal chondrosarcoma is a rare tumor. It has been noted to occur most commonly in muscle and the central nervous system. It has never been reported to occur in the soft tissues of the anogenital region. We report the first such case, occurring in a 40-year-old woman who presented with a 1-year history of a mass originating in the left labium majus. After extensive local resection and lymph node dissection she remains disease free after 40 months follow-up. q 1996 Academic Press, Inc.
INTRODUCTION
Mesenchymal chondrosarcoma was first described in bone by Lichtenstein and Bernstein [1] in 1959 and in soft tissue by Dowling [2] in 1964. It is a rare tumor, with less than 200 documented cases. It occurs two to three times more commonly in bones than in extraskeletal locations [3, 4]. Of the extraskeletal tumors reported in the literature, the majority have occurred in the muscles of the lower limb and meninges or brain [4, 5]. There have been no reports occurring in the anogenital region. We report such a case, occurring in the labium majus.
An excisional biopsy showed a large, fairly well circumscribed tannish nodular mass measuring 9 cm at its greatest diameter. Cut surface of the mass revealed tannish firm tumor tissue with a hemorrhagic center. Microscopically, the tumor was located mainly in the subcutaneous fat with infiltration into the overlying dermis. The tumor consisted mostly of undifferentiated round cells with hyperchromatic vesicular nuclei, prominent nucleoli, and scanty cytoplasm. Numerous mitotic figures were seen. Focally, these tumor cells were arranged in a hemangiopericytic pattern around ectatic vascular clefts. There were areas of poorly circumscribed islets of tumor cells showing features of chondroid differentiation (Fig. 1). No calcification or ossification was noted. The histological features coupled with the clinical findings pinpointed the diagnosis of extraskeletal mesenchymal chondrosarcoma. Because the deep resection margin was approximately 2 mm, it was decided that the patient should undergo a more radical operation. A radical vulvectomy and deep lymph node dissection was therefore performed 2 weeks after the initial surgery. There was no residual tumor in the subsequent specimen and all lymph nodes resected were clear of tumor. No adjuvant treatment was given. At a followup period of 40 months the patient remains well and free of disease.
CASE REPORT
A previously healthy 40-year-old woman presented with a 1-year history of a mass in the left suprapubic region. The mass had started as a small nodule in the left labium majus, and had progressively enlarged to the size of a fist, now causing some discomfort. There was never any discharge or bleeding from the mass. The patient was otherwise well. Clinical examination revealed a large mass occupying the left labium majus extending proximally toward the pubic symphysis. The mass was slightly tender and was not adherent to the underlying tissues. There was no ulceration or skin involvement. A full systemic workup showed negative findings. 1 To whom correspondence and reprint requests should be addressed. Fax: 852-26497426.
0090-8258/96 $18.00 Copyright q 1996 by Academic Press, Inc. All rights of reproduction in any form reserved.
/ m4716$4194
02-09-96 19:57:54
DISCUSSION
Extraskeletal mesenchymal chondrosarcoma is a rare tumor with less than 100 cases reported in the literature up to 1985 [5]. In that review, 54% of cases involved muscles, predominantly of the lower limb, and 46% of cases involved the central nervous system (CNS), meninges, brain, or orbit. The age at the time of diagnosis was significantly greater in the patients with extraskeletal mesenchymal chondrosarcoma involving the muscles (43.9 in years in the patients with tumors of the muscle, and 23.5 years in the patients with CNS involvement). Histologically, extraskeletal mesenchymal chondrosarcoma closely resembles hemangiopericytoma or poorly differentiated synovial sarcoma [3, 6]. The distinguishing fea-
492
goal
AP: Gyn Onc
493
CASE REPORT
FIG. 1. Photomicrograph showing undifferentiated tumor cells with areas of chondroid differentiation. Hematoxylin and eosin stain. Magnification, 125.
ture is the presence of areas of cartilaginous differentiation within the tumor [6]. Huvos et al. [3] histologically subclassified extraskeletal mesenchymal chondrosarcomas into two groups, the better differentiated hemangiopericytoid variant and the less well differentiated small cell type. The long-term prognosis of mesenchymal chondrosarcomas, when all sites are considered, is poor. Local recurrences or metastases can occur after more than 20 years. In a Mayo Clinic study of 23 patients with long-term follow-up, 74% died of disease 6 months to 23 years after the initial diagnosis, with an average survival of 6.7 years [4]. Six patients died after 5 to 10 years, and 3 patients died after more than 10 years’ follow-up. The 5-year survival rate was 54.6% and the 10-year survival rate was 27.3%. In the series reported by Huvos et al. at the Memorial Hospital, the 10-year survival rate was 28% [3]. We were not able to locate any other reports of a case of extraskeletal mesenchymal chondrosarcoma occurring in the soft tissues of the anogenital region. The histology in the case presented shows features of undifferentiated cells as well as hemangiopericytoid areas with focal chondroid differentiation, therefore making it difficult to subclassify according to Huvos et al. Forty months after the initial diagno-
/ m4716$4194
02-09-96 19:57:54
goal
sis, the patient remains well and free of disease. We await longer term follow-up of this patient. ACKNOWLEDGMENT The authors thank Dr. S. C. Luk for making the histological slides available for the present report.
REFERENCES 1. Lichtenstein, L., and Bernstein, D. Unusual benign and malignant chondroid tumors of bone, Cancer 12, 1142–1157 (1959). 2. Dowling, E. A. Mesenchymal chondrosarcoma, J. Bone Joint Surg. 46A, 747–754 (1964). 3. Huvos, A. G., Rosen, G., Dabska, M., and Marcove, R. C. Mesenchymal chondrosarcoma: A clinicopathologic analysis of 35 patients with emphasis on treatment, Cancer 51, 1230–1237 (1983). 4. Nakashima, Y., Unni, K. K., Shives, T. C., Swee, R. G., and Dahlin, D. C. Mesenchymal chondrosarcoma of bone and soft tissue: A review of 111 cases, Cancer 57, 2444–2453 (1986). 5. Louvet, C., de Gramont, A., Krulik, M., Jagueux, M., Hubert, D., Brissaud, P., Sirinelli, A., Augereau, B., Tubiana, J. M., and Debray, J. Extraskeletal mesenchymal chondrosarcoma: Case report and review of the literature, J. Clin. Oncol. 3, 858–863 (1985). 6. Enzinger, F. M., and Weiss, S. W. Cartilaginous tumors and tumorlike lesions of soft tissue, in Soft tissue tumors (F. M. Enzinger and S. W. Weiss, Eds.), Mosby, St. Louis, pp. 698–710 (1983).
AP: Gyn Onc