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Extraskeletal Chondrosarcoma Between Chest Wall and Liver
Image of the Month Extraskeletal Chondrosarcoma Between Chest Wall and Liver DIANBO CAO,* ABHISHEK SHAH,* and YAHUI LIU‡ *Department of Radiology, ‡Department of Surgery, The First Hospital of JiLin University, Chang Chun, China
Figure 1.
A
71-year-old man presented with a 1-year history of swelling and pain in the right upper abdomen. He was admitted to our hospital because of shortness of breath for 1 month. He had suffered from chronic bronchitis and emphysema for 20 years and hepatic hemangiomas for 40 years. The physical examination was unremarkable except for mild tenderness in the right upper abdomen. The hematologic and biochemical profiles were normal, including carcinoembryonic antigen, carbohydrate antigen 199, and ␣-fetoprotein level. Computed tomography (Figures A–D) revealed an oval-shaped, scattered, mineralized mass measuring 9.1 ⫻ 6.6 ⫻ 6.6 cm in the right upper abdomen. Ring-and-arc calcifications were seen adjacent to the inner edge of the mass. The outer margin of the mass was obscure with lateral chest wall tissue and the inner region had a well-defined arc margin with liver. Tumor feeding arteries originated from intercostal arteries. At surgery, a neoplasm originating from the intercostal soft tissue and growing between the lateral thoracic wall and liver was found. Gross cut-section was lobulated with a pearly white glistening appearance (Figure E). The histologic examination revealed the tumor was a disorderly arranged cell with large atypical nuclei in the
cartilage matrix (Figure F). A diagnosis of well-differentiated chondrosarcoma was made. The postoperative course was uneventful, and the patient refused postoperative chemotherapy. The patient was well with no recurrence at the 12-month follow-up evaluation. Chondrosarcoma is a malignant tumor of cartilaginous origin that produces cartilage matrix, and lesions that arise de novo are called primary chondrosarcoma. Primary chondrosarcoma is the second most common primary malignant tumor of bone, constituting 20% to 27% of all primary malignant osseous neoplasms. Chondrosarcoma also may develop in extraskeletal locations such as in soft tissue where cartilage normally is not found. It is unclear whether these tumors develop from mesenchymal differentiation or ectopic chondroid precursor cells.
Conflict of interest The authors disclose no conflicts. © 2011 by the AGA Institute 1542-3565/$36.00 doi:10.1016/j.cgh.2011.03.021 CLINICAL GASTROENTEROLOGY AND HEPATOLOGY 2011;9:e92– e93
September 2011
Extraskeletal chondrosarcomas are uncommon, accounting for approximately 2% of all soft-tissue sarcomas. The most common presentation is a mass; however, when in the abdomen or pelvis, these tumors can reach enormous sizes before detection, as in the case discussed. Chondrosarcomas have highly diverse features and behavior patterns, ranging from slow-growing nonmetastasizing lesions to highly aggressive metastasizing sarcomas, including conventional intramedullary, clear cell, juxtacortical, myxoid, mesenchymal, extraskeletal, and dedifferentiated. Although the pathologic appearance varies with specific lesion type, chondrosarcomas grow with
IMAGE OF THE MONTH
e93
lobular-type architecture and relatively specific calcification. Imaging features directly depict these pathologic traits, especially in identification of typical ring-and-arc chondroid matrix mineralization in well-differentiated lesions, a clue of chondrosarcoma. The degree of the matrix calcification may be correlated with the grade of tumor, but computed tomography is optimal for detecting matrix mineralization, particularly when it is subtle or when the lesion is located in anatomically complex areas. Understanding and recognizing the spectrum of primary chondrosarcoma is a prerequisite to making an accurate diagnosis before surgery. (Supplementary Figure 1).
Figure 1.
A
71-year-old man presented with a 1-year history of swelling and pain in the right upper abdomen. He was admitted to our hospital because of shortness of breath for 1 month. He had suffered from chronic bronchitis and emphysema for 20 years and hepatic hemangiomas for 40 years. The physical examination was unremarkable except for mild tenderness in the right upper abdomen. The hematologic and biochemical profiles were normal, including carcinoembryonic antigen, carbohydrate antigen 199, and ␣-fetoprotein level. Computed tomography (Figures A–D) revealed an oval-shaped, scattered, mineralized mass measuring 9.1 ⫻ 6.6 ⫻ 6.6 cm in the right upper abdomen. Ring-and-arc calcifications were seen adjacent to the inner edge of the mass. The outer margin of the mass was obscure with lateral chest wall tissue and the inner region had a well-defined arc margin with liver. Tumor feeding arteries originated from intercostal arteries. At surgery, a neoplasm originating from the intercostal soft tissue and growing between the lateral thoracic wall and liver was found. Gross cut-section was lobulated with a pearly white glistening appearance (Figure E). The histologic examination revealed the tumor was a disorderly arranged cell with large atypical nuclei in the
cartilage matrix (Figure F). A diagnosis of well-differentiated chondrosarcoma was made. The postoperative course was uneventful, and the patient refused postoperative chemotherapy. The patient was well with no recurrence at the 12-month follow-up evaluation. Chondrosarcoma is a malignant tumor of cartilaginous origin that produces cartilage matrix, and lesions that arise de novo are called primary chondrosarcoma. Primary chondrosarcoma is the second most common primary malignant tumor of bone, constituting 20% to 27% of all primary malignant osseous neoplasms. Chondrosarcoma also may develop in extraskeletal locations such as in soft tissue where cartilage normally is not found. It is unclear whether these tumors develop from mesenchymal differentiation or ectopic chondroid precursor cells.
Conflict of interest The authors disclose no conflicts. © 2011 by the AGA Institute 1542-3565/$36.00 doi:10.1016/j.cgh.2011.03.021 CLINICAL GASTROENTEROLOGY AND HEPATOLOGY 2011;9:e92– e93
September 2011
Extraskeletal chondrosarcomas are uncommon, accounting for approximately 2% of all soft-tissue sarcomas. The most common presentation is a mass; however, when in the abdomen or pelvis, these tumors can reach enormous sizes before detection, as in the case discussed. Chondrosarcomas have highly diverse features and behavior patterns, ranging from slow-growing nonmetastasizing lesions to highly aggressive metastasizing sarcomas, including conventional intramedullary, clear cell, juxtacortical, myxoid, mesenchymal, extraskeletal, and dedifferentiated. Although the pathologic appearance varies with specific lesion type, chondrosarcomas grow with
IMAGE OF THE MONTH
e93
lobular-type architecture and relatively specific calcification. Imaging features directly depict these pathologic traits, especially in identification of typical ring-and-arc chondroid matrix mineralization in well-differentiated lesions, a clue of chondrosarcoma. The degree of the matrix calcification may be correlated with the grade of tumor, but computed tomography is optimal for detecting matrix mineralization, particularly when it is subtle or when the lesion is located in anatomically complex areas. Understanding and recognizing the spectrum of primary chondrosarcoma is a prerequisite to making an accurate diagnosis before surgery. (Supplementary Figure 1).