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Extraskeletal osteosarcoma located to the gallbladder
HPB, 2006; 8: 65 /66
CASE REPORTS
Extraskeletal osteosarcoma located to the gallbladder
´ ZSEF KOCSIS2, ´ BOR OLGYAI1, VIKTOR HORVA ´ TH1, PE´TER BANGA1, JO GA ´ LIA BUZA3 & ATTILA OLA ´ H1 NATA Departments of 1Surgery and 2Pathology, Petz Alada´r Teaching Hospital, Gyo¨r and Experimental Tumour Pathology, National Institute of Oncology, Budapest, Hungary
3
Department of Human and
Abstract Extraskeletal osteosarcoma is a rare malignant soft tissue tumour. At open cholecystectomy performed for gallstones, a 61-year-old woman was found to have osseous tissue in the wall of the gallbladder. Histopathological examination of the specimen revealed a focus of extraskeletal osteosarcoma. The patient developed widespread intra-abdominal metastases 5 months after the operation, and died of pulmonary deposits at 9 months. Although osteosarcoma has rarely been reported at other extraskeletal sites, this appears to be the first case of a primary tumour in the gallbladder.
Key Words: Gallbladder, extraskeletal osteosarcoma
Introduction Extraskeletal osteosarcoma is a high-grade malignant mesenchymal neoplasm which accounts for about 1% of malignant soft tissue tumours. In a review of the literature there are no previous reports of extraskeletal osteosarcoma occurring in the gallbladder. Case history A 61-year-old woman presented with a 2-month history of right upper abdominal discomfort, nausea and moderate subcostal tenderness. Full investigation revealed gallbladder stones, colonic diverticulosis and a sliding hiatus hernia, i.e. Saint’s triad. At open cholecystectomy a lump of bony tissue measuring 30 /45 mm was seen within the wall of the gallbladder (Figure 1). The specimen was sent for frozen section, but no malignancy was confirmed. The patient recovered uneventfully and was discharged in good condition. Microscopically, there was a tumour arising from the serosa and infiltrating the muscular wall of the gallbladder. There was calcificied neoplastic osteoid among the tumour cells and osteoclastic giant cells (Figure 2) that showed intensive positive reaction with CD 68 (histiocyte marker), although the tumour cells were negative. The immunohistochemical reac-
tions for cytokeratin AE1/AE3 and epithelial membrane antigen were completely negative. The final histological diagnosis was extraskeletal osteosarcoma. Bone scintigraphy was negative. After multidisciplinary discussion it was decided to give no further treatment. The patient remained symptom-free for 5 months, when she developed vague abdominal pain and fever and lost 5 kg in weight. A right upper abdominal mass was now palpable. CT scan detected complex cystic-calcareous metastases in the abdominal wall and peritoneal cavity as well the liver, the wall of the intestine, the lesser and greater omentum and the mesentery. She was readmitted with small bowel obstruction and underwent further laparatomy, but the extent of tumour recurrence prevented any relief of the obstruction. Further biopsies confirmed ossified lesions. Four weeks later the patient underwent four cycles of chemotherapy, but she died of pulmonary metastases 9 months after her initial treatment. Discussion Extraskeletal osteosarcoma consists of neoplastic osseous tissue that is disconnected from the skeleton [1]. From a histological point of view, the tumour
Correspondence: Dr Ga´bor Olgyai, Department of Surgery, Petz Alada´r Teaching Hospital, Vasva´ri P. str. 2-4, Gyo¨r 9024, Hungary. Tel: / 36 96 507 936. E-mail: [email protected]
ISSN 1365-182X print/ISSN 1477-2574 online # 2006 Taylor & Francis DOI: 10.1080/13651820600573204
66
G. Olgyai et al. contains varying amounts of neoplastic osteoid, osseous and cartilaginous tissue with osteo-fibro-chondroblastic or giant (osteoclast) cells; the different representation of the types of cells defines the variety of the neoplasm [2 /4]. The aetiology of extraskeletal osteosarcoma is essentially unknown. The diagnosis is generally delayed because symptoms are often absent or vague. The tumour occurs principally as a soft tissue mass in the lower extremity (47%), upper extremity (21%) and retroperitoneum (17%) [5]. The prevailing sites of metastases are the lung, the regional lymph nodes and bone. Radical resection appears to be the best therapeutic option; resection of a pulmonary metastasis will occasionally produce a cure [6]. Radiotherapy may provide temporary palliation, while adjuvant chemotherapy is usually ineffective [7]. There is no survival difference between the three main tumour subtypes. Tumour size and the proliferation index are the main prognostic factors [8]. Local recurrence develops in about one-third of patients and pulmonary metastases in 60%. Median survival time is 24 months, and the cause-specific survival rate at 5 years is less than 25% [9].
References Figure 1. Photomicrograph showing giant cells (osteoclasts) and neoplastic osteoid among the tumour cells (H&E /100).
[1] Vulpio C, Desito C, Carlucci I, Mercuri M, Ruffini MV, Wiel Marin A. Extraskeletal retroperitoneal osteosarcoma. A case report and review of the literature. Minerva Chir 1996;5:1129 / 33. [2] Oonuma M, Hatori M, Hosaka M, Kokubun S. Extraskeletal osteosarcoma arising in the buttock. Ups J Med Sci 2001;106: 211 /5. [3] Chapman AD, Pritchard SC, Yap WW, Rooney PH, Cockburn JS, Hutcheon AW, et al. Primary pulmonary osteosarcoma: case report and molecular analysis. Cancer 2001;91:779 /84. [4] Pillay P, Simango S, Govender D. Extraskeletal osteosarcoma of the scalp. Pathology 2000;32:154 /7. [5] Lee JS, Fetsch JF, Wasdhal DA, Lee BP, Pritchard DJ, Nascimento AG. A review of 40 patients with extraskeletal osteosarcoma. Cancer 1995;76:2253 /9. [6] Ahmad SA, Patel SR, Ballo MT, Baker TP, Yasko AW, Wang X, et al. Extraosseous osteosarcoma: response to treatment and long-term outcome. J Clin Oncol 2002;20:521 /7. [7] Bane BL, Evans HL, Ro JY, Carrasco CH, Grignon DJ, Benjamin RS, et al. Extraskeletal osteosarcoma. A clinicopathologic review of 26 cases. Cancer 1990;65:2762 /70. [8] Chung EB, Enzinger FM. Extraskeletal osteosarcoma. Cancer 1987;60:1132 /42. [9] Lidang JM, Schumacher B, Myhre JO, Steen NO, Keller J. Extraskeletal osteosarcomas: a clinicopathologic study of 25 cases. Am J Surg Pathol 1998;22:588 /94. /
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Figure 2. Operative photograph of metastasis showing focus of bony tissue.
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CASE REPORTS
Extraskeletal osteosarcoma located to the gallbladder
´ ZSEF KOCSIS2, ´ BOR OLGYAI1, VIKTOR HORVA ´ TH1, PE´TER BANGA1, JO GA ´ LIA BUZA3 & ATTILA OLA ´ H1 NATA Departments of 1Surgery and 2Pathology, Petz Alada´r Teaching Hospital, Gyo¨r and Experimental Tumour Pathology, National Institute of Oncology, Budapest, Hungary
3
Department of Human and
Abstract Extraskeletal osteosarcoma is a rare malignant soft tissue tumour. At open cholecystectomy performed for gallstones, a 61-year-old woman was found to have osseous tissue in the wall of the gallbladder. Histopathological examination of the specimen revealed a focus of extraskeletal osteosarcoma. The patient developed widespread intra-abdominal metastases 5 months after the operation, and died of pulmonary deposits at 9 months. Although osteosarcoma has rarely been reported at other extraskeletal sites, this appears to be the first case of a primary tumour in the gallbladder.
Key Words: Gallbladder, extraskeletal osteosarcoma
Introduction Extraskeletal osteosarcoma is a high-grade malignant mesenchymal neoplasm which accounts for about 1% of malignant soft tissue tumours. In a review of the literature there are no previous reports of extraskeletal osteosarcoma occurring in the gallbladder. Case history A 61-year-old woman presented with a 2-month history of right upper abdominal discomfort, nausea and moderate subcostal tenderness. Full investigation revealed gallbladder stones, colonic diverticulosis and a sliding hiatus hernia, i.e. Saint’s triad. At open cholecystectomy a lump of bony tissue measuring 30 /45 mm was seen within the wall of the gallbladder (Figure 1). The specimen was sent for frozen section, but no malignancy was confirmed. The patient recovered uneventfully and was discharged in good condition. Microscopically, there was a tumour arising from the serosa and infiltrating the muscular wall of the gallbladder. There was calcificied neoplastic osteoid among the tumour cells and osteoclastic giant cells (Figure 2) that showed intensive positive reaction with CD 68 (histiocyte marker), although the tumour cells were negative. The immunohistochemical reac-
tions for cytokeratin AE1/AE3 and epithelial membrane antigen were completely negative. The final histological diagnosis was extraskeletal osteosarcoma. Bone scintigraphy was negative. After multidisciplinary discussion it was decided to give no further treatment. The patient remained symptom-free for 5 months, when she developed vague abdominal pain and fever and lost 5 kg in weight. A right upper abdominal mass was now palpable. CT scan detected complex cystic-calcareous metastases in the abdominal wall and peritoneal cavity as well the liver, the wall of the intestine, the lesser and greater omentum and the mesentery. She was readmitted with small bowel obstruction and underwent further laparatomy, but the extent of tumour recurrence prevented any relief of the obstruction. Further biopsies confirmed ossified lesions. Four weeks later the patient underwent four cycles of chemotherapy, but she died of pulmonary metastases 9 months after her initial treatment. Discussion Extraskeletal osteosarcoma consists of neoplastic osseous tissue that is disconnected from the skeleton [1]. From a histological point of view, the tumour
Correspondence: Dr Ga´bor Olgyai, Department of Surgery, Petz Alada´r Teaching Hospital, Vasva´ri P. str. 2-4, Gyo¨r 9024, Hungary. Tel: / 36 96 507 936. E-mail: [email protected]
ISSN 1365-182X print/ISSN 1477-2574 online # 2006 Taylor & Francis DOI: 10.1080/13651820600573204
66
G. Olgyai et al. contains varying amounts of neoplastic osteoid, osseous and cartilaginous tissue with osteo-fibro-chondroblastic or giant (osteoclast) cells; the different representation of the types of cells defines the variety of the neoplasm [2 /4]. The aetiology of extraskeletal osteosarcoma is essentially unknown. The diagnosis is generally delayed because symptoms are often absent or vague. The tumour occurs principally as a soft tissue mass in the lower extremity (47%), upper extremity (21%) and retroperitoneum (17%) [5]. The prevailing sites of metastases are the lung, the regional lymph nodes and bone. Radical resection appears to be the best therapeutic option; resection of a pulmonary metastasis will occasionally produce a cure [6]. Radiotherapy may provide temporary palliation, while adjuvant chemotherapy is usually ineffective [7]. There is no survival difference between the three main tumour subtypes. Tumour size and the proliferation index are the main prognostic factors [8]. Local recurrence develops in about one-third of patients and pulmonary metastases in 60%. Median survival time is 24 months, and the cause-specific survival rate at 5 years is less than 25% [9].
References Figure 1. Photomicrograph showing giant cells (osteoclasts) and neoplastic osteoid among the tumour cells (H&E /100).
[1] Vulpio C, Desito C, Carlucci I, Mercuri M, Ruffini MV, Wiel Marin A. Extraskeletal retroperitoneal osteosarcoma. A case report and review of the literature. Minerva Chir 1996;5:1129 / 33. [2] Oonuma M, Hatori M, Hosaka M, Kokubun S. Extraskeletal osteosarcoma arising in the buttock. Ups J Med Sci 2001;106: 211 /5. [3] Chapman AD, Pritchard SC, Yap WW, Rooney PH, Cockburn JS, Hutcheon AW, et al. Primary pulmonary osteosarcoma: case report and molecular analysis. Cancer 2001;91:779 /84. [4] Pillay P, Simango S, Govender D. Extraskeletal osteosarcoma of the scalp. Pathology 2000;32:154 /7. [5] Lee JS, Fetsch JF, Wasdhal DA, Lee BP, Pritchard DJ, Nascimento AG. A review of 40 patients with extraskeletal osteosarcoma. Cancer 1995;76:2253 /9. [6] Ahmad SA, Patel SR, Ballo MT, Baker TP, Yasko AW, Wang X, et al. Extraosseous osteosarcoma: response to treatment and long-term outcome. J Clin Oncol 2002;20:521 /7. [7] Bane BL, Evans HL, Ro JY, Carrasco CH, Grignon DJ, Benjamin RS, et al. Extraskeletal osteosarcoma. A clinicopathologic review of 26 cases. Cancer 1990;65:2762 /70. [8] Chung EB, Enzinger FM. Extraskeletal osteosarcoma. Cancer 1987;60:1132 /42. [9] Lidang JM, Schumacher B, Myhre JO, Steen NO, Keller J. Extraskeletal osteosarcomas: a clinicopathologic study of 25 cases. Am J Surg Pathol 1998;22:588 /94. /
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Figure 2. Operative photograph of metastasis showing focus of bony tissue.
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